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2,200 |
6109974-3
| 30,145,982 |
comm/PMC006xxxxxx/PMC6109974.xml
|
Chordoma: a case series and review of the literature
|
A 38-year-old Caucasian woman who had no medical history presented with a headache of 2 months’ duration in August 2012. There was a family history of malignancies. She had no psychosocial history. She did not take any medications. She was a housewife. She lived with her husband and three children in a flat in the city center of Istanbul. She never smoked tobacco and did not consume alcohol. Her vital signs were stable with blood pressure 110/70 mmHg, pulse rate 82/minute, and temperature 36.5 °C. On neurological examination, there was a limitation of temporal movement in her right eye. There were no signs of paresthesia or hypoesthesia. A general examination showed no other abnormality. Brain MRI showed 34 mm × 10 mm and 20 mm × 19 mm masses in her clivus. She was operated on and a pathology examination showed chordoma. Postoperative stereotactic radiotherapy to clivus at a total dose of 24 Gray in one fraction with gamma-knife was performed. In March 2014, she had a recurrence in her clivus and then she underwent another operation. A pathology examination revealed chordoma. She presented with diplopia for 1 month in January 2015. Brain MRI detected a recurrent mass in her clivus and invasion to the pons. She was treated with external cranial radiotherapy for palliative intent. She received a total of 30 Gray to recurrent mass in ten fractions during 10 days. In March 2015, a residual mass in her clivus was seen in MRI. She was started on daily 400 mg of imatinib in April 2015. The best response to imatinib was stable disease. Overall, imatinib was well tolerated; she reported periorbital edema, grade 1 skin rash on her legs, and nausea grade 2. She complained of visual loss in her left eye in May 2017. Brain MRI confirmed progressive disease (Fig. ). Imatinib was stopped and sunitinib 37.5 mg per day was started in June 2017. She received sunitinib until December 2017 when she had radiological and clinical progression (Fig. ). Four weeks later, her sunitinib dose was lowered to 25 mg/day due to ongoing grade 2 nausea and vomiting. Other reported symptoms included grade 2 fatigue and grade 1 hand-foot syndrome. The laboratory results are given in Table . She received a total of 25 months of imatinib therapy and 6 months of sunitinib treatment. She continued her follow-up with best supportive care until April 2018. She died on 23 April 2018.
|
[[38.0, 'year']]
|
F
|
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|
2,201 |
6109996-1
| 30,186,639 |
comm/PMC006xxxxxx/PMC6109996.xml
|
Traumatic Retropharyngeal Hematoma in a Patient Taking Clopidogrel
|
An 81-year old female with a history of coronary artery disease, hypertension, and thrombocytosis suffered a witnessed trip and fall onto a nightstand. The patient took 75 mg of clopidogrel daily in addition to an 81 mg aspirin tablet. She reported a mild headache however had no change from her baseline mentation per family members with no evidence of obvious injury aside from a small area of ecchymosis near a small forehead laceration. She remained up and ambulatory with no further complaints. Ten hours after her injury the patient presented to the Emergency Department with stridorous and agonal respirations with a profoundly decreased level of consciousness. She was noted to have developed extensive ecchymosis on the anterior portion of her neck and chest. Her symptoms had begun rapidly shortly prior to arrival while lying in bed. Family reported that she had been in the constant company of her husband with no further falls or injuries that had occurred since her fall. The patient was intubated upon hospital arrival due to respiratory extremis with obvious swelling and crepitus noted on neck examination. A noncontrast CT scan of head was unremarkable while there was demonstration of a large retropharyngeal hematoma measuring 3.6 cm by 5.3 cm by 20 cm on a CT of the cervical spine with no evidence of fracture. Her hemoglobin was 9.5 gm/dL and platelets were 1234 per deciliter, with an INR of 3 and a slightly below normal and activated partial thromboplastin time of 23.9 seconds (reference range 25-35 seconds). A CT angiogram of the neck was subsequently obtained demonstrating active bleeding from the anterior ligaments of the vertebral column that was not felt to be amenable to embolization (). Given the extent of the hematoma intraoral surgical evacuation was performed with bleeding from the anterior vertebral spine controlled with Bovie cauterization, placement of topical thrombin, and drain placement. No reaccumulation of hematoma was noted during her hospital course. The patient unfortunately expired 12 days from the date of admission from presumed aspiration pneumonia and multisystem organ failure.
|
[[81.0, 'year']]
|
F
|
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|
{'1828150-1': 1, '7788530-1': 1, '4706887-1': 1}
|
2,202 |
6110011-1
| 30,186,644 |
comm/PMC006xxxxxx/PMC6110011.xml
|
A Novel STAT3 Gene Mutation Related Hyper-IgE Syndrome Misdiagnosed as Hidradenitis Suppurativa
|
A 21-year-old white female was referred to our Allergy-Immunology Clinic for a history of multiple intractable cutaneous abscesses and cysts for several years. She had undergone multiple incision and drainage and had been treated with antibiotics as well as topical and systemic steroids intermittently with minimal relief and developed methicillin resistant staphylococcal aureus (MRSA) colonization during the same period. She was at the time clinically diagnosed as Hidradenitis Suppurativa. Her medical history was significant for hypertension, diabetes mellitus type II, hyperlipidemia, obesity, and anxiety.\nOn further questioning, she reported more than fifteen hospitalizations for pneumonias. According to her mother, she had recurrent pneumonias, upper respiratory tract infections, sinusitis, mastoiditis, and oral candidiasis since early childhood. The patient denied any history of atopic dermatitis, other types of eczema, or food allergy. Evaluation for cystic fibrosis and hypogammaglobulinemia at the time had been negative. More recently, she had been admitted for septic shock secondary to septic arthritis of left hip. She also had multiple fractures with minimal trauma since childhood and was clinically diagnosed with osteogenesis imperfecta. She also reported history of primary teeth retention for which she had underwent orthodontic surgery at the age of 12 years. She had a normal birth history with normal documented developmental milestones and was up-to-date with her immunizations. She had Penicillin and Trimethoprim-Sulfamethoxazole (TMP-SMX) listed as allergy after she developed rash with their use when she was a toddler.\nFamily history was pertinent for her paternal grandmother with recurrent pneumonias, who passed away at 40 years of age secondary to a severe lung infection. Her biological brother had a history of recurrent skin boils.\nImportant physical findings included coarse facial feature with exacerbated pore size, deep set eyes, broad nasal bridge, high arched palate, and multiple scattered healed scars on skin with some remnant cold abscesses. She had mild thoracic scoliosis. Pulmonary and cardiovascular exam were unremarkable.\nLaboratory investigations revealed a normal complete blood count with no eosinophilia. IgE level was elevated at 5,842 IU/ml, with erythrocyte sedimentation rate of 64 mm/hr and C-reactive protein of 1.56 mg/dl. IgG, IgM, and IgA levels were within normal limit. Tetanus and pneumonia titers were normal. Total complement (CH50) levels were normal and testing for chronic granulomatous disease was unrevealing. Due to history, physical findings, and these initial labs, we suspected Hyper-IgE Syndrome. A calculated HIES score was 63 (>40 required for diagnosis). With a high suspicion of AD-HIES, mutation analysis of STAT3 gene was sent which detected a novel pathogenic mutation, C.1388 T>A (pVal463Glu) at DNA-binding domain of STAT3 gene. Although this mutation in STAT3 gene has never been published as a pathogenic mutation leading to AD-HIES, missense variants in nearby residues have been reported in association to those reported in the Human Gene Mutation Database in association with HIES [].\nPenicillin allergy testing and TMP-SMX oral challenge were performed in the clinic. With a normal response, she was started on TMP-SMX (800mg-160mg) once daily prophylactically for prevention of infections (MRSA sensitive to TMP-SMX).\nPatient was advised to follow up for clinical response and monitoring tolerance to treatment. She was asked to have regular dental exam, chest imaging, bone density scan, and pulmonary function test screening. She was also advised to have genetic counselling. Genetic testing for her biological brother was offered.
|
[[21.0, 'year']]
|
F
|
{'18602572': 1, '4161105': 1, '29527033': 2, '20159255': 1, '27226025': 1, '5059313': 1, '10441580': 1, '27535530': 1, '26592211': 1, '26743515': 1, '28197791': 1, '27333819': 1, '28803389': 1, '24058807': 1, '28567072': 1, '31069200': 2, '30186644': 2}
|
{'5838762-1': 1, '6491627-1': 1}
|
2,203 |
6110037-1
| 30,186,636 |
comm/PMC006xxxxxx/PMC6110037.xml
|
Case Report of a Massive Thigh Hematoma after Adductor Canal Block in a Morbidly Obese Woman Anticoagulated with Apixaban
|
The patient involved provided written consent for reporting of this case.\nA 63-year-old woman with medical history of super morbid obesity (BMI 54) and atrial fibrillation for which she was anticoagulated with apixaban presented for an open reduction internal fixation (ORIF) of an ankle fracture. Significant medical history included diabetes mellitus type 2, obstructive sleep apnea, chronic obstructive pulmonary disease, and diastolic heart failure. The patient's last dose of apixaban was 48 hours prior to surgery. Other than moderate anemia (hemoglobin 8.8 g/dL), all laboratory studies, including a coagulation profile, were normal.\nPrior to surgery, the patient was offered a sciatic nerve catheter and an adductor canal block as part of a multimodal postoperative analgesia strategy. Because of her many, serious medical conditions, we concluded that a peripheral nerve block offered the best opportunity to provide satisfactory postoperative analgesia. Specifically, we were concerned that the postoperative pain management primarily with opioid medications would pose increased cardiopulmonary risk to the patient. We were careful to explain the risks associated with peripheral nerve blocks, including the risk of bleeding and hematoma formation, and verbal consent was obtained.\nThe surgery was performed under general anesthesia and her intraoperative course was uncomplicated. Upon arrival to the recovery room, our acute pain service was contacted to evaluate her for peripheral nerve blockade. We positioned the patient in the lateral decubitus position and placed a sciatic nerve catheter. Though technically challenging due to body habitus, this sciatic nerve block was performed successfully and without any complication. The patient was then positioned supine for the adductor canal block. The leg was externally rotated and the knee slightly flexed for optimal positioning. A high-frequency linear array ultrasound transducer was applied to the mid-thigh in short-axis and the adductor canal was identified. Imaging was again challenging given the patient's habitus, but with firm compression of the ultrasound transducer, the important anatomical structures were clearly identified. The superficial femoral artery (SFA) was visualized dorsal to the sartorius muscle and a hyperechoic structure anterolateral to the artery was identified as the adductor canal and saphenous nerve [, ]. The skin adjacent to the probe was cleansed with a chlorhexidine and alcohol solution. A 20-gauge × 4-inch beveled, echogenic needle was inserted using an in-plane technique. The needle was visualized continuously as it coursed between the vastus medialis and sartorius toward the adductor canal. The needle was positioned lateral to the SFA within the canal and a bolus of 20 ml of 0.25% bupivacaine with 5 mcg/ml epinephrine was administered with negative heme aspiration checks after every 5 ml injection. Spread of local anesthetic within the adductor canal was clearly observed under ultrasound visualization. There was no evidence of intravascular injection of epinephrine while monitoring the patient. Upon needle withdrawal, brisk bleeding was noted at the skin insertion site but with direct manual pressure for approximately 60 seconds, bleeding ceased completely.\nShortly after the blocks were performed, the patient reported complete resolution of her ankle pain and was transferred to her hospital room. Approximately 6 hours after surgery, the patient reported new anterior thigh pain on the operative leg, which was treated by her nurse with intravenous hydromorphone. Roughly 13 hours after surgery, the patient's nurse finally contacted the orthopedic surgery team due to unmanageable mid-thigh pain. The orthopedics team initially believed the pain was due to tourniquet compression pain which occurred during surgery. Upon further physical examination, a hematoma was noted in the anterolateral mid-thigh. Vital signs were within normal ranges and distal pulses were intact. A CT scan with contrast was ordered and revealed a 14-cm hematoma in the right thigh (). Lab studies showed a drop in hemoglobin from 8.8 g/dL preoperatively to 6.9 g/dL the morning of postoperative day (POD) 1. Coagulation studies at that time were within normal limits including partial thromboplastin time, prothrombin time, and international normalized ration, as well as platelet count. The patient's primary medicine service transfused 1 unit of packed red blood cells, which improved her hemoglobin to 7.6 g/dL.\nInterventional radiology was consulted on the morning of POD 1 for management recommendations for the hematoma. A CT angiogram was performed revealing active extravasation from a small superficial branch of the SFA (). Embolization with coil and gel foam was performed and compressive dressings were used to apply direct pressure. Throughout the day, the patient's hemoglobin remained stable and the hematoma showed no evidence of further expansion.\nOn POD 2, further consultation from the vascular surgery and interventional radiology teams was sought for possible hematoma evacuation versus drain placement given the massive size. Vascular surgery recommended conservative management with application of direct pressure. Interventional radiology, however, recommended placement of a drain within the hematoma. A pigtail catheter was placed and fluid cultures were obtained. Minimal output from the drain was observed, so beginning on POD 3, tissue plasminogen activator (TPA) was administered through the catheter daily to facilitate hematoma drainage. Follow-up ultrasound on POD 6 showed a persistent hematoma despite TPA administration. Hematoma cultures resulted negative for infection. On POD 7, she was discharged to a rehabilitation facility with the drain in place and care team instructions to flush 5-10 ml saline twice daily until evaluation with interventional radiology one week later.\nOn POD 14, the drain was inadvertently pulled out requiring a return visit to interventional radiology and drain replacement. On POD 19, the hematoma cavity had decreased to an acceptable size so the drain was removed. One week later, on POD 26, she returned to interventional radiology due to increased pain and swelling at the hematoma site. A recurrent fluid collection was noted on ultrasound examination so the drain was replaced a third time and aspirated fluid was sent for culture, which grew staphylococcus aureus. She was treated for hematoma superinfection with a five-day course of levofloxacin and when she returned two weeks later, the abscess had resolved and the drain was removed.
|
[[63.0, 'year']]
|
F
|
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|
{}
|
2,204 |
6110053-1
| 30,197,697 |
comm/PMC006xxxxxx/PMC6110053.xml
|
Nontypable Haemophilus influenzae Septicemia and Urinary Tract Infection Associated with Renal Stone Disease
|
A 32-year old man was admitted to a Danish hospital due to right sided flank pain of four days duration. He had no confirmed medical diagnoses, but had previously been tested for Sarcoidosis, Polycythemia vera, stroke and acute coronary syndrome. He also had a history of former steroid-use. The available medical records did not state his vaccination status or previous childhood infections. At hospitalization, he presented with intermittent right sided flank pain, turning into constant pain of VAS 7-8 and radiating to the right side groin. Additional symptoms were nausea, chills, and observation of blood in the urine. Physical examination revealed right sided abdominal and renal pain and a temperature of 38.0 degrees Celsius. His urine tested positive for leucocytes, erythrocytes, nitrite and protein 1 g/L and blood samples showed normal urate levels, elevated ionized calcium levels 1.56 mmol/L, creatinine 122 µmol/L, leukocytosis of 15.9 x 109 /L and CRP 6.4 mg/L increasing to 172 mg/L the next day. CT scan showed bilateral nephrolithiasis as well as right side ureterolithiasis causing obstruction Fig. (). Direct microscopy on three out of three blood culture bottles revealed small gram-negative pleomorphic rods within 24 hours of incubation. Mass spectrometry (Bruker Daltonics using MBT Compass software version 4.1 that contains 6903 MSP´s) identified the strain as H. influenzae with a score of 2.24. Microbiology testing of urine routinely cultured on a 5% blood agar plate and a UTI chrome agar plate showed 105 growth of H. influenzae confirmed by MALDI-TOF MS (score of 2.15). The strain was found to be a non-capsulated biotype II, susceptible to all antibiotics tested by disc diffusion: penicillin (1 unit, zone diameter: blood = 15 mm, urine = 15 mm), amoxicillin-clavulanate (3 µg), ampicillin (10 µg), ciprofloxacin (5 µg, zone diameter: blood = 35 mm, urine = 41 mm), cefuroxime (30 µg) and piperacillin-tazobactam (36 µg) using EUCAST disc diffusion recommendations. After microbiology samples had been collected, the patient started antibiotic treatment with intravenous ampicillin 1 g x 4 daily and a right sided JJ ureteric stent was surgically inserted. The patient received two doses of ampicillin, but due to subjective discomfort, treatment was changed to cefuroxime 1500 mg x 3 daily. After three days, the patient was discharged with 5 days of peroral ciprofloxacin 500 mg x 2 and a scheduled ambulant stone-removal surgery.
|
[[32.0, 'year']]
|
M
|
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|
{'4214010-1': 1}
|
2,205 |
6110074-1
| 30,197,711 |
comm/PMC006xxxxxx/PMC6110074.xml
|
Superior Capsular Release After Failed Combined Superior Labral Repair And Biceps Tenodesis For Slap Tear
|
The patient is a 46-year-old right-hand dominant male who presented for left shoulder pain, stiffness, and mechanical symptoms. He underwent arthroscopic SLAP repair and open subpectoral biceps tenodesis 2 years ago for long-standing left shoulder pain without any history of trauma. He stated that his pain and dysfunction were worse at this time than before the index surgery. Golf was his main recreational activity before the surgery, but now he has problems doing activities of daily living. He has not improved with physical therapy. He has been to two other orthopaedic surgeons and was diagnosed with subacromial impingement.\nOn physical exam, the patient’s left shoulder was slightly more protracted and he had mild scapular dyskinesia. He was tender to palpation at the acromioclavicular joint, greater tuberosity, and glenohumeral joint. He actively forward elevated to 130 degrees compared to 160 degrees on the right; same with passive elevation. He internally rotated to L1 on the left and T6 on the right. He externally rotated to 70 degrees at the side bilaterally. He had full abduction which was symmetric to the other side. Internal rotation in the scapular plane was 20 degrees compared to 60 degrees on the right. External rotation in the scapular plane was 80 degrees on the left and 100 degrees on the right. Impingement tests with Neer, Hawkins, and Kim were all positive. Strength testing of all 4 rotator cuff muscles were 5/5 and symmetric. However, he had some pain with Jobe and bear hug tests. Crossbody adduction test and O’briens were positive. Instability tests were all negative and he did not have any signs of hyperlaxity per Beighton criteria. His American Shoulder and Elbow Surgeons Shoulder (ASES) score at this time was 26.6.\nMRI prior to surgery reported a type VII SLAP (Snyder type II). Surgery report states that this was repaired with 2 suture anchors; 1 placed anterior to the biceps and another placed posteriorly and knots were tied. Additionally, there was a partial articular subscapularis tear that was debrided and decision to do the subpectoral biceps tenodesis was based on the subscapularis tear suggestive of biceps instability with compromise of the medial sling. The bursa was excised and a bursal sided rotator cuff tear was debrided of about 10%. MRI 1 year later shows intact superior labrum repair and biceps tenodesis. There was a progression of tendinosis, mild acromioclavicular (AC) joint arthrosis, and mild degenerative changes along the inferior glenoid with osseous spurring and mild chondral loss. X-rays showed a type III acromion with a large subacromial spur (Fig. ). Based on these findings, the patient was consented for left shoulder arthroscopic subacromial decompression, distal clavicle excision, possible removal of anchors, and possible capsular release.\nDuring surgery, patient was placed in lazy lateral decubitus position. Kim’s posterior portal was established. ESR and CRP were obtained pre-operatively and were negative. However, before turning on the fluid, a needle was placed in the rotator interval and intra-articular joint fluid was aspirated and sent to pathology (Fig. ). Prophylactic antibiotics were then started and fluid was turned on. An anterior portal was established in the rotator interval. The superior labrum had healed. There were no proud anchors and the knots were away from the articular surface. The rotator interval was thickened and scarred and the MGHL was thick and tight. The knots were removed using an open knot cutter. A superior capsular release was performed with an arthroscopic tissue liberator knife between the interval of the labrum and rotator cuff at the glenoid (Figs. -). The SGHL was released. The MGHL was resected with a meniscal punch (Fig. ) as well as the rotator interval and CHL. The anterior capsule had normal pliancy and was not thick and fibrotic as seen typically with adhesive capsulitis and therefore, the capsular release was not extended anteroinferiorly.\nIn the subacromial space, there was thickened bursa and a bursectomy was performed. Adhesions were removed in the anterior, lateral, and posterior gutters. A subacromial decompression (Figs. -) and distal clavicle excision were performed.\nHe was discharged home the same day with a sling for comfort and noted that he was able to raise his arm overhead on POD 0 which he was not able to do previously. The patient was given 3 weeks of oral penicillin until final cultures came back. He started immediate physical therapy with a range of motion exercises and periscapular strengthening and progressive cuff strengthening. Final cultures at 3 weeks were negative.\nOn his last follow up at 6 months post-operative, he was able to actively forward flex to 160 degrees, internally rotate to T8, externally rotate to 70 at the side, externally rotate in the scapular plane to 90 degrees, and internally rotate in the scapular plane to 60 degrees. Neer and Kim impingement tests were negative while Hawkins was mildly positive. He had symmetric strength of all four rotator cuff muscles. His final ASES score was 86.6.
|
[[46.0, 'year']]
|
M
|
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{}
|
2,206 |
6110164-1
| 30,177,912 |
comm/PMC006xxxxxx/PMC6110164.xml
|
Homozygous and Heterozygous Nuclear Lamin A p.R582C Mutation: Different Lipodystrophic Phenotypes in the Same Kindred
|
The proband (Figure ) was a 12-year-old girl, born at full-term (birth weight, 3,600 g) as the first child of consanguineous parents, referred to the Universidade Federal do Ceará Clinical Hospital, Fortaleza, Brazil for clinical assessment of short stature and learning disabilities that manifested since age 8. Physical examination revealed reduced fat in the arms, legs, and gluteal region, muscular hypertrophy, and acanthosis nigricans as well as macroglossia, dry and thickened skin, short stature, and pubertal stage Tanner 1. Proband was 118.7 cm (Z-score, −5.2) in height, weighed 27 kg, and had a body mass index (BMI) of 19.1 kg/m2. Thyroid function tests revealed severe primary hypothyroidism (TSH > 100 uU/mL and free T4 = 0.01 ng/dL) and thus the proband was started on levothyroxine resulting in catch-up growth and normal pubertal development with menarche by age 14.8. After hypothyroidism treatment, the selective loss of subcutaneous fat tissue in limbs, gluteal region, and abdomen became evident over time, leading to the diagnosis of lipodystrophy. At age 12, she presented with moderate hepatomegaly, umbilical hernia, hypertriglyceridemia (509 mg/dL), and acanthosis nigricans in the neck and axillary regions. One year later, at age 13, hypochromic and atrophic cutaneous plaques were observed distributed throughout the body. Skin biopsies revealed localized scleroderma (morphea). Some years later in adulthood, remarkable fat accumulation in the neck, face, and axillary and dorsocervical regions was observed, along with the worsening of subcutaneous fat atrophy in limbs and abdomen, indicating partial lipodystrophy diagnosis.\nAt age 23, the proband was diagnosed with diabetes and albuminuria. Currently, she is 26 years old and presents with uncontrolled diabetes, hepatomegaly (10 cm below the costal margin), and irregular menses. She is 145 cm in height, weighs 42 kg, and has a BMI of 20.0 kg/m2. A formal assessment of intelligence quotient is not available, but she shows a slight degree of intellectual impairment. Standard serum determinations are listed in Table .
|
[[12.0, 'year']]
|
F
|
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|
{'7362519-1': 1}
|
2,207 |
6110280-1
| 30,159,206 |
comm/PMC006xxxxxx/PMC6110280.xml
|
Tacrolimus-Induced Remission in Drug Resistant Inflammatory Myopathy:\nA Case Series
|
42-year-old man presented with proximal muscle weakness of 1.5 years\nduration. Muscle biopsy demonstrated polymyositis. High dose PDN and MTX were\ninitiated; however, no significant improvement was appreciated. After a year on\nPDN and MTX, the patient developed worsening proximal muscle weakness and\ndifficulty in swallowing. PDN dose was increased and azathioprine (AZA)\ncommenced. Two doses of intravenous immunoglobulin (IVIG) were administered.\nGiven the lack of response to the new regimen, the patient transferred his care\nto our hospital. Physical exam was notable for 2/5 strength for neck flexors,\ndeltoid, biceps, hand grip, hip flexors and quadriceps. His hands appeared\nswollen with muscle wasting.\nAfter comprehensive case review, PDN 75 mg/day and TAC 2 mg twice daily\nwere started. Biologics could not be prescribed due to non-medical reasons. TAC\ndose was increased to 4 mg twice daily, based on trough goal of 6–10\nng/mL []. After a month of initiating\ntacrolimus, mycophenolate mofetil (MMF) 1500 mg twice daily was added without\ncomplications. Six months after TAC and MMF combination had started, muscle\nenzymes decreased significantly (CK 4419 U/L to 732 U/L and LDH 1402 U/L to 513\nU/L) and clinical improvement was appreciated. PDN was tapered to 30 mg/day.\nBlood pressure and glycemic levels were monitored at every clinic visit. Eleven\nmonths after TAC and MMF were started, LDH and CK normalized; patient had 3/5\nstrength on neck flexors and 4/5 strength on hip flexors and quadriceps. No\nadverse effects have been reported ().
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[[42.0, 'year']]
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M
|
{'23124935': 1, '16157605': 1, '17881720': 1, '20536595': 1, '28052609': 1, '10579116': 1, '18716733': 1, '16822917': 1, '21376512': 1, '16227154': 1, '16357462': 1, '10440399': 1, '21694936': 1, '12697731': 1, '17543149': 1, '28763333': 1, '16251851': 1, '16582685': 1, '25923553': 1, '9402690': 1, '8632081': 1, '10878285': 1, '22955021': 1, '16583368': 1, '28970719': 1, '26633102': 1, '23173570': 1, '22870489': 1, '23970542': 1, '10347992': 1, '11294511': 1, '16052580': 1, '22886003': 1, '10755536': 1, '17003173': 1, '19433436': 1, '16636243': 1, '22141281': 1, '1714235': 1, '14511932': 1, '12510664': 1, '30159206': 2}
|
{'6110280-2': 2, '6110280-3': 2}
|
2,208 |
6110280-2
| 30,159,206 |
comm/PMC006xxxxxx/PMC6110280.xml
|
Tacrolimus-Induced Remission in Drug Resistant Inflammatory Myopathy:\nA Case Series
|
39-year-old man with history of dermatomyositis (DM) presented to our\nhospital. Six months before, patient had developed typical skin rash and\nproximal muscle weakness. Work-up including muscle biopsy led to a diagnosis of\nDM. Intravenous methylprednisolone had been administered. Patient was lost to\nfollow-up. On presentation to our hospital the patient had worsening proximal\nmuscle weakness, rash, dysphagia, an elevated CK of 389 5U/L, and elevated liver\nenzymes (AST 242 U/L, ALT 191 U/L, ALP 146 U/L). The acute episode was treated\nwith pulse steroids and IVIG. Patient was placed on MMF 2 g/day, PDN 70 mg/day\nwith plans for rituximab infusions.\nThree months after disease onset, the patient reported improvement of\nskin lesions on his hands but continued to have significant proximal muscle\nweakness evidenced by difficulty standing from a seated position and inability\nto lift his arms above his head. CK was 1352 U/L and LDH was 617 U/L. Despite\nMMF was increased to 3 g/day, the patient continued to have little improvement.\nRituximab was given in the interim. Five months after presentation, proximal\nmuscle weakness recurred. CK (1495 U/L), LDH (495 U/L), and ESR (42 mm/hr)\nremained elevated; TAC 2 mg/day was added to the existing regimen and PDN was\nslowly tapered.\nThree months after tacrolimus had been started, the patient was able to\nstand from a seated position. Labs revealed down trending CK (806 U/L), LDH (459\nU/L), ESR (27 mm/h). At this point, TAC level was 2.5 ng/mL, so TAC was\nincreased to 3 mg twice daily. Five months after TAC initiation, there was\nsignificant clinical and laboratory improvement. TAC dosage was adjusted to\nachieve therapeutic level and PDN was eventually tapered off. Eleven months\nafter TAC initiated, muscle weakness had significantly improved and labs\nnormalized (CK 102 U/L and LDH 183 U/L). Patient was able to return to work\n().
|
[[39.0, 'year']]
|
M
|
{'23124935': 1, '16157605': 1, '17881720': 1, '20536595': 1, '28052609': 1, '10579116': 1, '18716733': 1, '16822917': 1, '21376512': 1, '16227154': 1, '16357462': 1, '10440399': 1, '21694936': 1, '12697731': 1, '17543149': 1, '28763333': 1, '16251851': 1, '16582685': 1, '25923553': 1, '9402690': 1, '8632081': 1, '10878285': 1, '22955021': 1, '16583368': 1, '28970719': 1, '26633102': 1, '23173570': 1, '22870489': 1, '23970542': 1, '10347992': 1, '11294511': 1, '16052580': 1, '22886003': 1, '10755536': 1, '17003173': 1, '19433436': 1, '16636243': 1, '22141281': 1, '1714235': 1, '14511932': 1, '12510664': 1, '30159206': 2}
|
{'6110280-1': 2, '6110280-3': 2}
|
2,209 |
6110280-3
| 30,159,206 |
comm/PMC006xxxxxx/PMC6110280.xml
|
Tacrolimus-Induced Remission in Drug Resistant Inflammatory Myopathy:\nA Case Series
|
51-year-old woman with a history of hypertension, hyperlipidemia, and\nstroke presented with a two-month history of muscle weakness; she reported a\nremote exposure to statins. Examination revealed 2/5 strength on the left side\nand 4/5 on the right. CK was 28,885 U/L; necrotizing myositis was suspected.\nPatient was treated with IV methylprednisolone followed by PDN 60 mg/day.\nExtensive proximal muscle edema was seen on MRI. Vastus medialis biopsy showed\nnecrotizing features without inflammation. Hydroxy-Methyl-Glutaryl Coenzyme A\nreductase (HMG CoA) antibodies were strongly positive. One month after\npresentation, there was a modest improvement in motor weakness; CK had dropped\nto 5,546 U/L. IVIG (5 days) was given and MMF 1 g/day were started. Patient\ncontinued to experience weakness and CK remained elevated. Two months after\npresentation, rituximab was added to the regimen of MMF (3 g/day) and prednisone\n(60 mg/day). Two doses of IVIG were given as a bridge therapy. After an initial\nresponse, the patient’s muscle weakness returned. Three months after\npresentation, CK remained elevated (3,178 U/L). TAC 4 mg/day was added to MMF (3\ng/day) and PDN 40 mg/day. On follow up visits, TAC dose was increased to 6\nmg/day and PDN was tapered slowly. Eight months after TAC was initiated,\nweakness improved markedly and labs normalized (CK 117 U/L, LDH 251 U/L). One\nyear after tacrolimus and MMF, the physical exam was normal with full strength\nthroughout. PDN had been reduced to 5 mg daily ().
|
[[51.0, 'year']]
|
F
|
{'23124935': 1, '16157605': 1, '17881720': 1, '20536595': 1, '28052609': 1, '10579116': 1, '18716733': 1, '16822917': 1, '21376512': 1, '16227154': 1, '16357462': 1, '10440399': 1, '21694936': 1, '12697731': 1, '17543149': 1, '28763333': 1, '16251851': 1, '16582685': 1, '25923553': 1, '9402690': 1, '8632081': 1, '10878285': 1, '22955021': 1, '16583368': 1, '28970719': 1, '26633102': 1, '23173570': 1, '22870489': 1, '23970542': 1, '10347992': 1, '11294511': 1, '16052580': 1, '22886003': 1, '10755536': 1, '17003173': 1, '19433436': 1, '16636243': 1, '22141281': 1, '1714235': 1, '14511932': 1, '12510664': 1, '30159206': 2}
|
{'6110280-1': 2, '6110280-2': 2}
|
2,210 |
6110409-1
| 30,155,382 |
comm/PMC006xxxxxx/PMC6110409.xml
|
Epidural Catheter in a Child with Metastatic Rhabdomyosarcoma
|
The present case is a 12-year-old girl with progressive metastatic rhabdomyosarcoma-left forearm primary. Metastasis was identified on the right lumbar paraspinal muscles, left femur, and left jaw. Multiple magnetic resonace imaging (MRI) and computed tomography (CT) scans showed no involvement of the spinal cord. She did not receive more chemotherapy or radiation therapy. She had a very significant cancer-related pain, especially in her lower extremities and jaw, which was not amenable to hospice management at home with hydromorphone patient controlled analgesia (PCA). She was admitted to the hospital due to poor pain control and the pain team was consulted for better pain management options. Her life expectancy is very short and a decision had to be made to place a tunneled epidural catheter to send her home. Due to significant debilitating headaches and the possibility of cerebral spinal fluid leak and postdural puncture headaches during an intrathecal catheter placement, this therapy was not considered as the first option. Her parents are hoping that with the epidural she will be less sedated than with the hydromorphone PCA. The patient received a transfusion of fresh frozen plasma to get a normal prothrombin time (PT), partial thromboplastin time (PTT), and international normalized ratio (INR). She also needed a platelet transfusion, which brought her level to 109,000 during the morning time of the procedure. Her initial hemoglobin level is 9.1 g/dL on the day of the procedure. She was brought to the operating room for sedation. The epidural catheter is placed without complication; the catheter tip is confirmed at the L2-L3 level with fluoroscopy and 1 millimeter (ml) of contrast dye (Omnipaque 300, GE Healthcare, Cork, Ireland) and subcutaneously tunneled completely under the left paraspinal muscles with a Touhy 18-gauge 3.5 inches needle (B. Braun Medical, Inc., PA, USA). The needle was removed carefully keeping the catheter in place, and no signs of bleeding were observed after the needle was removed. The patient reported analgesia from epidural and PCA dose was weaned down. Overnight she started leaking from the epidural and tunneled sites; coagulation parameters were normal. A neuro check was normal after stopping the epidural infusion for two hours. On the second day, the bleeding was more pronounced, her platelets were 96,000 INR 1.2, and the surgical hemostat was applied with pressure dressings. The neuro checks continued to rule out epidural hematoma and spinal cord injury. Aspiration of the epidural catheter was bloodless, and a test dose of 3 mm of lidocaine 1.5% with epinephrine 1:200,000 was negative, ruling out migration to a vessel. On the third day, the patient had massive bleeding, which came from the tunnel site developing big clots around the dressings. Aspiration of the epidural catheter was bloodless, and a test dose of 3 mm of lidocaine 1.5% with epinephrine 1:200,000 was negative. Thrombin, Stat Seal disc (Biolife, LLC, Sarasota, FL, USA), Quick Clot (Z-MEDICA, LLC, Wallingford, CT, USA), and all hemostatic agents were applied, without any success. The patient was transfused with platelets (her count was 76,000), fresh frozen plasma, and packed red blood cells because at this point, her hemoglobin level was 5.4 g/dL. Bleeding resolved after removing the epidural catheter.
|
[[12.0, 'year']]
|
F
|
{'10919415': 1, '20052816': 1, '1493343': 1, '12520314': 1, '25140337': 1, '20890208': 1, '26994232': 1, '21199115': 1, '30155382': 2}
|
{}
|
2,211 |
6110410-1
| 30,155,389 |
comm/PMC006xxxxxx/PMC6110410.xml
|
Pylephlebitis Caused by Actinomyces Bacteremia
|
A 56-year-old female, with a history of a repaired Tetralogy of Fallot and pulmonary embolism while on warfarin, presented with epigastric pain and melena. The patient was febrile (101.2℉) but hemodynamically stable and did not appear to be septic. Labs on admission are shown in Table .\nThe patient was given 10 mg of vitamin K intravenously and six units of fresh frozen plasma. The esophagogastroduodenoscopy (EGD) showed two nonbleeding duodenal arteriovenous malformations (AVMs). Her total bilirubin level increased to 3.0 mg/dL on day three of her hospital stay. An abdominal ultrasound (US) scan and a CT scan with/without contrast (Figure ) showed acute portal vein thrombosis extending into the splenic vein and segmental branches of the right and left hepatic lobes. No abscesses or other sources of infection were noted.\nThe patient was started on 1 mg/kg enoxaparin daily (INR 1.5 on day three). The patient’s initial fever and leukocytosis were attributed to portal vein thrombosis; thus, no antibiotics were given, pending blood cultures. The next day, the blood cultures grew Gram-positive cocci and rods (Micromonas miros and Actinomyces turicensis, respectively). She was started on IV vancomycin. However, she continued to spike fevers with worsening leukocytosis (Figure ). An echocardiogram did not show any valve vegetation. A tagged WBC scan showed no evidence of infection, making infective endocarditis unlikely. Her dental evaluation showed poor oral hygiene, multiple retained roots, pulpal necrosis, and mobile teeth. Repeated blood cultures grew Actinomyces meyeri. Both the hepatology and infectious diseases teams agreed this was likely a septic pylephlebitis secondary to Actinomyces bacteremia (likely stemming from the oral cavity). She was switched to IV penicillin G, after which her WBC count improved (Figure ) and repeated blood cultures came back negative. She was discharged on IV ertapenem for six weeks followed by six weeks of oral amoxicillin and a follow-up appointment for oral surgery.
|
[[56.0, 'year']]
|
F
|
{'15615953': 1, '26160320': 1, '8589130': 1, '11721772': 1, '15728028': 1, '27592344': 1, '20735334': 1, '16545171': 1, '34684101': 2, '15906779': 1, '8942542': 1, '17853956': 1, '30155389': 2}
|
{'8537041-1': 1}
|
2,212 |
6110411-1
| 30,155,383 |
comm/PMC006xxxxxx/PMC6110411.xml
|
Acute Kidney Injury in a 31-year-old Male as a Consequence of Multiple Myeloma
|
A 31-year-old male presented to the emergency department with a five-week history of right-sided chest pain, right upper quadrant abdominal pain, and associated shortness of breath. On initial clinical review, he claimed to be otherwise healthy with no prior medical or social history. The patient denied any prior history of renal disease. He claimed to have sustained a fall at work five weeks prior to presentation and started noticing gradually worsening right-sided chest pain. On initial presentation, he was tachycardic with a pulse rate of 104 beats/minute, blood pressure 121/76 mmHg, and oxygen saturation of 100% on room air. The cardiac examination was unremarkable with no additional sounds and murmurs. He was tender over lower four right ribs. Lungs were clear to auscultation with no rales or rhonchi. The abdomen was soft and non-tender, with no evidence of organomegaly, and there was no peripheral edema.\nHis important baseline investigations are listed in Table :\nHe underwent a chest X-ray that suggested a pleural lesion in the right hemothorax (Figure ). Computed tomography (CT) showed an expansile lytic lesion corresponding to the pleural lesion (Figure ). Multiple lytic deposits were also seen on the CT scan (Figures -). A subsequent skeletal survey revealed multiple lesions on the skull, pelvic bone, and proximal right femur. The lytic lesions were investigated further and revealed normocytic anemia with hemoglobin of 113 g/L, mean corpuscular volume (MCV) of 83.6 fL and 3+ rouleaux formation on peripheral blood smear, and normal white cell and platelet count. Corrected calcium was 4.43 mmol/L, and creatinine was 621 μmol with urea of 23.6 mmol/L. Urine microscopy was positive for protein and negative for blood.\nOn serum protein electrophoresis, total protein was 114 g/L with high gamma globulin 47.5 g/L (6.0-18.0 g/L) and a monoclonal band (M-spike) 44.6 g/L. Immunoglobulin panel revealed IgG 62.40 g/L (5.52-17.24 g/L), and immunofixation electrophoresis revealed monoclonal IgG, type kappa (k). Immunofixation of urine revealed Ig G (k) of 0.24 g/d. The patient consequently underwent a bone marrow core and aspirate biopsy of the iliac crest. The aspirate was suboptimal for evaluation for flow cytometry. The biopsy revealed sheets of plasma cells and interstitial infiltrate involving approximately 50% of the biopsy cellularity. Immunohistochemical staining was positive for CD 138 (suggestive of plasma cells). The results were consistent with a diagnosis of multiple myeloma based on diagnostic criteria by International Myeloma Working Group (IMWG).\nHe was started on the cyclophosphamide-bortezomib-dexamethasone (CyBorD) regimen and received four cycles of therapy. He had an excellent renal response but a partial hematologic response and relapsed after four months of therapy. The biochemical and hematologic response is documented in Table . He is now being treated with lenalidomide and dexamethasone, and once he achieves hematologic remission, he will undergo autologous stem cell transplant.
|
[[31.0, 'year']]
|
M
|
{'8639427': 1, '9711912': 1, '12528874': 1, '30155383': 2}
|
{}
|
2,213 |
6110412-1
| 30,155,386 |
comm/PMC006xxxxxx/PMC6110412.xml
|
Hidradenocarcinoma: Five Years of Local and Systemic Control of a Rare Sweat Gland Neoplasm with Nodal Metastasis
|
A 38-year-old male presented to the otorhinolaryngology clinic with the complaint of right-sided neck swelling in February 2013. This swelling had been progressively increasing for three months. There was no associated pain, fever, or difficulty in swallowing. He also reported having a painless swelling on the scalp which had been there for 20 years. On examination, there was a firm, fixed, non-tender mass palpable on the right side of the neck at level II. It measured 3 x 3 cm in size. Another lump was appreciated on the scalp, which was soft in consistency, non-tender, mobile, and 4 x 4 cm in size.\nConsidering these clinical findings, he underwent excisional biopsy of the right nodal mass which suggested hidradenocarcinoma. Histopathologic evaluation revealed sheets of tumor cells showing pleomorphic cells and frequent mitotic figures. On immunohistochemical staining, tumor cells showed positivity for cytokeratin 7, epithelial membrane antigen (EMA), and p63 (Figure ). A panendoscopy showed no abnormality in the pharynx, nasal cavity, or larynx. The locoregional extent of the disease was evaluated by a computed tomography (CT) scan of the head and neck that showed multiple enlarged lymph nodes on the right side of the neck (Figure ), along with a well-defined lobulated cystic mass over the right side of the scalp (Figure ). CT scans of the chest and abdomen were negative for any distant metastasis (Figure ).\nThe case was discussed in the head and neck multidisciplinary tumor board meeting at our hospital. On the basis of the available evidence, the consensus was to go for a wide local excision of the scalp lesion, along with a right-sided neck dissection. The histopathology of the scalp lesion was reported as malignant hidradenocarcinoma. The size of the lesion was 4.2 x 3.5 x 2.2 cm with a closest resection (deep) margin of 0.1 cm. A total of 56 lymph nodes were recovered from the right side of the neck, out of which two were positive for tumor metastasis at level II, the largest deposit being 2.3 cm in size.\nPostoperatively, the case was again discussed in the tumor board meeting where the consensus was to offer concurrent chemoradiation. Radiation therapy was offered in two phases to a total dose of 60 Gy in 30 fractions. In phase 1, a radiation dose of 50 Gy was given to the scalp and ipsilateral neck from level II to level IV. A boost dose of 10 Gy was delivered in phase 2 to the deep margin in the scalp and level II neck only. Concurrent cisplatin was given with the radiation at a dose of 100 mg/m2 as a radiosensitizer. After completion of treatment, he was followed up with clinical examination and serial imaging of the head and neck. Currently, he has completed five years of follow-up and is disease-free, both for local and distant metastasis.
|
[[38.0, 'year']]
|
M
|
{'32328335': 2, '32226692': 2, '6297408': 1, '33884163': 2, '34925613': 1, '22666726': 2, '18410617': 1, '22540063': 2, '18867767': 1, '15355375': 1, '20441512': 1, '17043204': 1, '34094723': 2, '16882695': 1, '10727324': 1, '17083691': 1, '30155386': 2}
|
{'7171610-1': 1, '8046015-1': 1, '7096074-1': 1, '3336933-1': 1, '8169006-1': 1, '3364684-1': 1}
|
2,214 |
6110413-1
| 30,155,376 |
comm/PMC006xxxxxx/PMC6110413.xml
|
Hypertensive Crisis in Cardiac Tamponade
|
A 76-year-old female with past medical history of well-controlled hypertension, coronary artery disease presented with subacute progressive shortness of breath for two weeks. On presentation, blood pressure (BP) was 238/146 mm Hg, heart rate (HR) of 75 beats per minute (bpm), SaO2 (oxygen saturation) to 80% and was placed on 4 L nasal cannula (NC) with improvement in her oxygenation. Physical exam was remarkable for pulsus paradoxus, distant heart sounds without murmurs or gallops, marked jugular venous distension, diminished breath sounds at the bases and mild bilateral lower extremity pitting edema. Electrocardiogram (EKG) showed only low voltage (Figure ).\nChest radiograph showed enlarged cardiac silhouette and bilateral moderate pleural effusions. An echocardiogram demonstrated moderate to large pericardial effusion with tamponade physiology (Figures , , ).\nThe patient remained significantly hypertensive despite adding three antihypertensive medications requiring labetalol drip with failure to control her blood pressure. She underwent pericardiocentesis with the removal of 1200 cc bloody fluid. Right heart catheterization was also done prior and after the pericardiocentesis, which indicated severely elevated right-sided pressures and equalization of right atrial, right ventricular and pulmonary capillary wedge pressure with diminished cardiac output. There was a significant improvement in right-sided pressures following pericardial drainage, with a mean right atrial pressure of 10 mm Hg down from 21 mm Hg. Systemic blood pressure normalized after pericardiocentesis. Follow-up echocardiogram showed resolution of the pericardial effusion. Further workup was done to identify the etiology of the pericardial effusion, including fluid cytology, culture, lactate dehydrogenase (LDH), serum antinuclear antibodies (ANA), serum complement, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), anti-double-stranded DNA (dsDNA), and anti-Smith antibody. Analysis of the pericardial fluid showed exudative fluid and it was negative for malignant cells.
|
[[76.0, 'year']]
|
F
|
{'22721572': 1, '1625736': 1, '2684445': 1, '21716107': 1, '17215663': 1, '30155376': 2}
|
{}
|
2,215 |
6110414-1
| 30,155,379 |
comm/PMC006xxxxxx/PMC6110414.xml
|
A Case of Acute Pancreatitis in Hemolysis, Elevated Liver Enzymes, and Low Platelets Syndrome
|
A 32-year-old female, gravida 2 para 1 at gestational age of 36 weeks and two days, presented to the Overlook Medical Center at 2:57 am complaining of nausea and contractions, which had started earlier in the night and worsened. Prenatal records were unavailable. Her previous pregnancy was complicated by premature rupture of membranes, and she delivered vaginally a healthy infant at 36 weeks of gestational age.\nDuring intake, the patient sat upright, appearing uncomfortable, and complained of epigastric pain and vomiting. The physical examination was remarkable for a blood pressure of 202/101 mmHg. Initial laboratory results were significant for the following: white blood cell count (WBC) 13.6 x 103/µL, urine protein of 300 mg/dL, hemoglobin (HGB) 15.6 g/dL, platelets (PLT) 182 x 103/µL, lipase 200 IU/L, total bilirubin (T bili) 0.2 mg/dL, aspartate transaminase (AST) 56 IU/L, alanine transaminase (ALT) 40 IU/L, alkaline phosphatase (ALP) 162 IU/L, and albumin 2.3 g/dL.\nUpon a diagnosis of severe preeclampsia, the patient was started on intravenous (IV) magnesium sulfate for seizure prophylaxis at 2 g/h and was given IV labetalol to control blood pressure. The fetal non-stress test was reactive and category 1. The patient was scheduled for emergent cesarean section, which was carried out without complications three hours after admission, resulting in the delivery of a vigorous 1.645 kg male infant, with appearance, pulse, grimace, activity, respiration (APGAR) scores of 8/9 at one and five minutes, respectively. The placenta was delivered complete, and the blood loss during surgery was 680 mL. In the recovery room, the patient continued with high blood pressure values in the 190s/110s mmHg and was given boluses of 10 mg of hydralazine IV in addition to IV labetalol.\nTwo hours post-operation (post-op), the patient reported severe right upper quadrant pain with worsening nausea and vomiting. Laboratory data at that time revealed up-trending liver enzymes (AST of 1054, ALT of 687, ALP of 134 IU/L), a T bili that had risen but was still in the normal range of 0.8 mg/dL, worsening of her hypoalbuminemia at 1.7 g/dL, anemia of 11.2 g/dL, thrombocytopenia of 59 x103/µL, and worsening leukocytosis with neutrophilia (19.9 x103/µL and 87.3%). Prior to this point, the patient had received 1.6 L of IV fluids. The international normalized ratio (INR) was slightly elevated at 1.19, and the fibrinogen was decreased at 192 mg/dL, with fibrin degradation products ≥20 µg/mL. The patient was then transferred to the intensive care unit (ICU) with the diagnosis of HELLP syndrome. A chest x-ray to rule out pulmonary edema reported no significant findings.\nSix hours post-op, the patient still complained of right upper quadrant pain and nausea, but these were slightly improving. Her physical exam was remarkable for decreased breath sounds bilaterally at the lung bases and diffuse abdominal tenderness, which was more prominent on the left side. Subsequent laboratory values reported worsening transaminitis with AST of 1332, ALT of 663, ALP of 146 IU/L, increased T bili of 1.1 mg/dL, WBC of 20.5 x103/µL, and worsening PLT of 38 x103/µL. An elevated lactate dehydrogenase (LDH) of 1881 IU/L and a decreased haptoglobin of <8 mg/dL pointed to a hemolytic process consistent with HELLP syndrome. Although the patient was afebrile, empiric antibiotic coverage with IV cefazolin 2 g every eight hours was initiated out of concern for endometritis given her recent delivery. Her fibrinogen levels had improved, however, to 228 mg/dL. Magnesium levels were monitored, and the magnesium infusion was adjusted accordingly. Blood pressure levels showed signs of better control, with values in the 150s/105s mmHg, and she was maintained on an IV labetalol infusion. A chest x-ray was ordered, and was reported clear. Worsening thrombocytopenia and liver function tests prompted an abdominal computed tomography (CT) scan, which revealed a small volume of complex abdominal and pelvic ascites, mild bilateral pleural effusions and bibasilar atelectasis, and no evidence of subcapsular hematoma, liver, or gallstone pathology. Upon further review of the CT scan, it was felt that the pancreas appeared prominent, which prompted further workup. We include an image of the CT scan below in Figure .\nTwelve hours post-op, the patient had improved blood pressure values in the 130s/90s mmHg. Pancreatitis was confirmed with a lipase of 2365 IU/L, which had risen from 200 IU/L on admission. Laboratory findings also showed increased lactic acid of 2.9 mmol/L, bicarbonate (HCO3-) of 22 mEq/L, worsening anemia with HGB of 10.1 g/dL, down-trending liver enzymes (AST of 944, ALT of 536 IU/L), and improved leukocytosis (WBC of 17.3 x103/µL). The labetalol infusion was discontinued at this point, given her lower blood pressure values. Labetalol, as needed, was ordered to maintain a blood pressure goal of below 150s/110s mmHg.\nIn the morning of the second day after admission, the patient´s blood pressure improved to 145-118 /92-79 mmHg, her right upper quadrant pain was reduced, and her physical exam revealed decreased abdominal tenderness. Laboratory values showed down-trending transaminitis (AST of 573, ALT of 415, ALP of 111 IU/L), and decreasing LDH of 1030 IU/L. The anemia and thrombocytopenia had worsened to HGB of 9 g/dL and PLT of 36 x103/µL, with increasing WBC 15.6 x103/µL. The lipase level had increased to 2509 IU/L together with the lactic acid 3.9 mmol/L, yet her HCO3- level remained normal at 23 mEq/L. On the evening of hospital day 2, she started tolerating clear liquids with only mild nausea. Laboratory results eventually showed normalization of the lipase at 186 IU/L, and her liver enzyme levels continued trending downward, along with trends toward normalization of her other laboratory values.\nOn the third day after admission, the patient maintained normal blood pressure values (110s/80s mmHg) with oral labetalol. Her abdominal pain continued decreasing and her nausea stopped. She remained on the magnesium sulfate infusion and was transferred from the ICU back to the obstetrics floor. The patient continued to improve on her fourth day, with decreasing liver enzymes (AST of 84, ALT of 113, ALP of 99 IU/L), increasing platelets (PLT 80 x103/µL), and decreasing WBC at 13.3 x103/µL, but her HGB dropped to 7.9 g/dL. The patient was not transfused, as she was hemodynamically stable. Importantly, her creatinine levels remained below 1.1 mg/dL throughout her hospitalization. The patient was subsequently discharged on the fifth day after admission, with improved HGB of 8.5 g/dL, PLT of 127 x103/µL, WBC of 11.7 x103/µL, and normalization of her transaminitis.
|
[[32.0, 'year']]
|
F
|
{'24201165': 1, '27512470': 1, '28458836': 2, '23107053': 1, '23418608': 1, '7631678': 1, '22825263': 1, '23892538': 1, '27535735': 1, '29041829': 1, '19123930': 1, '23354015': 1, '8831599': 1, '19245695': 1, '24380504': 1, '17454215': 1, '20457564': 1, '26064725': 2, '24150027': 1, '29153664': 1, '30155379': 2}
|
{'5400475-1': 1, '4443756-1': 1}
|
2,216 |
6110417-1
| 30,155,387 |
comm/PMC006xxxxxx/PMC6110417.xml
|
Paraneoplastic Mononeuritis Multiplex: A Unique Presentation of Non-Hodgkin Lymphoma
|
A 25-year-old female with gestational amenorrhea for 32 weeks presented to our outpatient department with complaints of a recurring headache along with pain and weakness in the legs for the past seven months. The headache was described as a bilateral, dull and persistent pain that fluctuated between mild to moderate in intensity. She also described neck stiffness along with her chief complaints but denied any nausea, vomiting, and changes in gait or memory. The pain in her legs waxed and waned over time, although progressively increasing in intensity with each passing episode. At the outset of this predicament, pain was localized to her left leg, eventually became symmetrical and later progressed to afflict both arms. She denied numbness or paresthesia. She was eventually brought to our clinical setting following an aggravation of her symptoms over the previous two weeks that lead to a restriction in mobility. At the time of this presentation, she also complained of double vision that was gradually worsening. She also added that she experienced fluctuating fevers, undocumented weight loss, and episodes of night sweats for the last four months.\nInitial assessment found the patient to be alert and well-oriented, with a Glasgow Coma Scale score (GCS) of 15/15, albeit thin, emaciated, and noticeably distressed due to her clinical predicament. Her heart rate (HR) was 102/minute with a respiratory rate (RR) of 18/minute, a temperature of 98.4°F and a blood pressure (BP) of 110/175 mm Hg. A neurological examination revealed generalized weakness and a bilaterally diminished muscle tone. A strength assessment revealed that she had reduced power in her upper (right arm; 2/5, left arm; 4/5) and lower (right leg; 1/5, left leg; 3/5) extremities. There was a complete absence of all deep tendon reflexes except the biceps. A comprehensive ophthalmological exam demonstrated normal visual acuity, with notable issues in the right eye which included ptosis, fixed and dilated pupil, and diplopia which manifested with the right-sided gaze. A funduscopic examination showed normal definitions. She also had a bilateral facial nerve palsy which affected the lower half of the face, along with reduced sensation along the distribution of the maxillary and mandibular divisions of the trigeminal nerve. This deficit was more pronounced on the right side of the face. On abdominal examination, an appendectomy scar was visible on the right iliac fossa. The abdomen was protuberant, soft and non-tender. Bowel sounds were audible and inguinal lymph nodes were not palpable.\nInitial laboratory investigations were within normal limits, with the exception of an elevated erythrocyte sedimentation rate (ESR) of 128 millimeters/hour. A magnetic resonance imaging (MRI) scan of the brain ruled out any local pathology that could explain her neurological deficits. A cerebrospinal fluid (CSF) analysis following a lumbar puncture revealed a white blood cell (WBC) count of 1500 with neutrophilic predominance, which leads to the initiation of empirical therapy for bacterial meningitis. Two days after the initiation of antibiotic therapy, she complained of dull abdominal pain and fullness. She was investigated with an abdominal ultrasound which revealed a thickened descending colon with a well-defined heterogeneous lesion measuring 89 mm by 94 mm in the left adnexal region that impinged on the uterus and urinary bladder due to its mass effect. The lesion was further investigated using a computed tomography (CT) scan which disclosed a circumferential mural thickening in the distal third of the descending colon with no luminal narrowing at the site. Multiple enlarged para-aortic lymph nodes were also appreciated, with the largest measuring 17 mm by 10 mm at the level of the left renal hilum.\nFollowing patient stabilization, the adnexal mass was biopsied which showed an atypical infiltrate composed of small to medium cells exhibiting immature chromatin, irregular nuclear folds, and increased mitosis. Immunohistochemistry showed this infiltrate to be CD3 (+), TdT (+), CD99 (+), PAX-5 (-) and CD20 (-), consistent with precursor T cell lymphoblastic lymphoma (Figures -).\nIn lieu of her newly diagnosed malignancy, we suspected that her neurological deficits could be attributed to a paraneoplastic syndrome. A workup for autoimmune etiologies was unrevealing based on a negative serum electrophoresis, negative anti-ganglioside profile and a negative anti-neuronal profile (Tables -).\nThe patient eventually underwent a nerve conduction study that revealed severe sensory-motor axonal neuropathy involving the left tibial, left peroneal and right facial nerve.\nAn eventual diagnosis of paraneoplastic MM was formulated based on a combination of the physical findings and documented nerve damage that simultaneously affected two separate nerve areas. She was treated with plasmapheresis which eventually led to a resolution of her neurological discrepancies. The patient remains asymptomatic till date from a neurological standpoint.
|
[[25.0, 'year']]
|
F
|
{'17452948': 1, '24249325': 1, '17480225': 1, '34659082': 1, '22750923': 1, '23645658': 1, '24455360': 2, '21339498': 1, '23315459': 1, '14561798': 1, '24664309': 1, '15258215': 1, '7496783': 1, '30155387': 2}
|
{'3885191-1': 1}
|
2,217 |
6110419-1
| 30,155,384 |
comm/PMC006xxxxxx/PMC6110419.xml
|
New-Onset Diabetes Presenting as Monoballism Secondary to a Mixed Hyperglycemic Crisis
|
A previously healthy 41-year-old male, presented to the emergency department (ED) complaining about involuntary movements of the left arm and abrupt onset that had started 12 hours prior to the admission. He complained of asthenia, adynamia, polyuria, and hyporexia for the last three days. Upon arrival, his blood pressure was 129/82 mmHg with a heart rate of 101 beats per minute; the respiratory rate was 20 breaths per minute and the temperature was 36.4°C. The capillary glucose level was 566 mg/dL. On physical examination, his left arm had a persistent and arrhythmic violent high-amplitude movement, mainly affecting the proximal muscles, which were consistent with monoballism (Video ). The patient was alert and co-operative. Speech, cranial nerves, strength, muscle stretch reflexes, and cerebellum examination were unremarkable.\nThe patient’s initial blood workup showed a serum sodium of 145 mmol/L (normal range: 135–145) with a corrected sodium of 152 mmol/L for a glucose of 517 mg/dL; potassium 3.7 mmol/L (normal range: 3.6–5), chloride 88 mmol/L (normal range: 98-107), magnesium 0.73 mmol/L (normal range: 0.66-1.85), calcium 2.5 mmol/L (normal range: 2.15-2.5), and serum lactate 1.8 mmol/L. An arterial blood gas analysis showed a moderate metabolic acidosis with a pH of 7.4 and a bicarbonate of 8.7 mmol/L; a calculated osmolarity of 332 mOsm/L (normal range: 285–295) and a high anion gap of 48 mmol/L (normal range: 8-16). Urinalysis was relevant for glycosuria (1,000 mg/dL) and ketonuria (80 mg/dL). These findings were consistent with a mixed hyperglycemic state (ketoacidosis and hyperosmolar state). Complete blood cell count and renal function tests were within the normal range.\nThe magnetic resonance imaging (MRI) of the brain was normal (Figure ), without any evidence of ischemia or hemorrhage. In the ED, the patient was treated with normal saline and insulin infusion, which resolved the acute hyperglycemia and the acid base disorder. After resolving the latter, the patient was moved to the internal medicine department. Monoballism resolved 48 hours after correction of the metabolic abnormalities and the patient was discharged four days after admission with insulin as the treatment for his new-onset diabetes.
|
[[41.0, 'year']]
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M
|
{'20566257': 1, '19370491': 1, '14572734': 1, '13798206': 1, '19305947': 1, '32669781': 1, '23326735': 2, '21871808': 1, '23639559': 1, '32754552': 2, '21930415': 1, '24014327': 1, '30155384': 2}
|
{'3541550-1': 1, '7380802-1': 1}
|
2,218 |
6110421-1
| 30,155,388 |
comm/PMC006xxxxxx/PMC6110421.xml
|
A Rare Case of Isolated Left Ventricular Non-compaction in an Elderly Patient
|
A 61-year-old male, with a history of emphysema, obstructive sleep apnea, and hypertension, presented to the emergency room with worsening shortness of breath over a three-month period. The patient also complained of orthopnea, paroxysmal nocturnal dyspnea, and progressively worsening lower limb edema. On examination, the patient had jugular venous distension, bilateral lower extremity edema, and bibasilar crackles. The laboratory evaluation showed a B-natriuretic peptide level of 11,065 pg/ml and a troponin level of < 0.04 ng/ml. A transthoracic echocardiogram showed a reduced left ventricular ejection fraction (LVEF) of 20%-25% with prominent hyper-trabeculations noted in the left ventricle, most prominent in the lateral and apical walls. These findings were concerning for LVNC. Cardiac magnetic resonance imaging (CMRI) showed a non-compacted to compacted myocardium ratio of 5:1 at the left ventricular apex (Figure ), confirming the diagnosis of LVNC. The patient underwent left heart catheterization, which did not show obstructive coronary disease as an etiology for the cardiomyopathy. The patient was managed with guideline-directed therapy for heart failure, including carvedilol, losartan, furosemide, hydralazine, and isosorbide mononitrate. He was also started on warfarin due to the increased risk of thromboembolism associated with LVNC. He had episodes of non-sustained ventricular tachycardia during his admission and was subsequently evaluated by electrophysiology (EP). He was discharged home with a wearable cardioverter defibrillator with instructions to follow up with EP in three months for an evaluation of implantable cardioverter defibrillator (ICD) placement for primary prevention.
|
[[61.0, 'year']]
|
M
|
{'26323941': 2, '19225828': 1, '11711464': 1, '16567565': 1, '22438694': 2, '31523538': 2, '15892002': 1, '30155388': 2}
|
{'6728782-1': 1, '4817066-1': 1, '3303910-1': 1}
|
2,219 |
6110626-1
| 30,159,215 |
comm/PMC006xxxxxx/PMC6110626.xml
|
Conduction Aphasia as Initial Manifestation of Tuberculous Meningitis
|
A previously healthy right-handed 24-year-old woman developed a headache three days before admission in the left frontal region with an 8/10 intensity accompanied by retro-ocular pain and phosphenes. Twenty-four hours later, she developed a speech disorder and was presented to the emergency department. Upon arrival, her blood pressure was normal (110/70 mmHg), tachycardic with a heart rate of 94 beats per minute, normal respiratory rate (14 breaths per minute) and temperature (36.2°C). The neurological examination showed normal mental status, with fluent speech and no paraphasias. The patient had normal nomination, and she was able to understand and obey simple commands. She was able to read-out loud and write, but could not repeat simple phrases; the rest of the examination was normal. Her blood work revealed hemoglobin of 8.9 g/dL and 4,390 leukocytes mm3/mL, human immunodeficiency virus type one and two antibodies detection were negative; rest of the blood work was normal. A chest X-ray was performed and it revealed generalized symmetrical interstitial infiltrates. Acontrast-enhanced magnetic resonance image (MRI) of the brain showed multiple edematous nodular lesions in the left parietal lobe and cerebellum on the T1-weighted sequence (Figure ).\nTransthoracic echocardiogram, carotid and vertebral Doppler ultrasound examination were normal. Cerebrospinal fluid (CSF) analysis showed 88 cells mm3/mL of which 65% were mononuclear with low glucose of 36 mg/dL, a central glucose of 116 mg/dL (ratio 0.31), and elevated proteins of 201 mg/dL. The CSF smear was negative and Gene Xpert (Cepheid Inc., Sunnyvale, CA, USA) MTB/rifampicin (RIF) in the CSF was positive for MTB. She was started on first-line antituberculosis drugs (isoniazid, rifampicin, pyrazinamide and ethambutol) and dexamethasone (0.4 mg/kg/day). The patient was discharged on the 11th day of hospital stay. After her three-month follow-up, she still had CA.
|
[[24.0, 'year']]
|
F
|
{'10675209': 1, '26039845': 1, '25693576': 1, '20760593': 1, '21256582': 1, '25110618': 1, '30159215': 2}
|
{}
|
2,220 |
6110627-1
| 30,159,216 |
comm/PMC006xxxxxx/PMC6110627.xml
|
Dural Arteriovenous Fistula Associated With a Vestibular Tumor: An Unusual Case and Review of the Literature
|
A 46-year-old female had symptoms of right ear congestion accompanied by pulsatile tinnitus and mild hearing loss. Neurological examination revealed weakness of cranial nerves X and XII. Magnetic resonance imaging (MRI) identified a 4.2 x 4.7 x 4.1 cm lobulated mass centered at the cerebellopontine angle that was hypointense on T1-weighted, heterogeneous on T2-weighted, and avidly enhancing on post-contrast images (Figure ). No significant component was noted within the internal auditory canal. An Octreoscan was performed, which showed intense increased tracer uptake in the mass centered in the region of the right jugular foramen, thereby supporting the suspected diagnosis of paraganglioma (Figure ).\nDigital subtraction angiography (DSA) demonstrated a surprising lack of vascularity associated with the tumor. However, there was an incidental discovery of a Cognard IIa+b dural arteriovenous fistula in the right posterior fossa associated with an occluded right sigmoid sinus (Figure -).\nDue to the extensive volume of tumor, the patient underwent a staged surgical resection. The first surgery included a modified radical right neck dissection, right superficial parotidectomy with facial nerve dissection, as well as the initial approach to the tumor and partial extradural resection. One week later, the patient underwent the second stage surgery where the tumor was radically resected using a right transcochlear approach. The right sigmoid sinus was ligated. The facial nerve was mobilized and transposed. Postoperative MRI confirmed complete resection of the tumor (Figure ). Histological analysis of samples from both surgeries confirmed the tumor not to be a paraganglioma, but instead a schwannoma (Figure ).\nShe had a long recovery from the surgery due to the lower cranial nerve palsies but has shown gradual improvement. Her latest follow-up MRI (48 months from surgery) (Figure ) showed no evidence of recurrent tumor; however, there was some residual posterior fossa DAVF. At that time, she also reported bothersome pulsatile tinnitus that she perceives on the right despite a complete hearing loss on the right side. DSA revealed new large feeders from the right middle meningeal artery (MMA) and occipital arteries into the residual DAVF (figure ), which were not present on initial presentation (Figure ). The DAVF was embolized utilizing Onyx in the right MMA. Final right common carotid artery (CCA) angiogram demonstrated resolution in arteriovenous shunting (Figure ). Vertebral artery injection after embolization demonstrated minimal residual shunting via the right anterior inferior cerebellar artery (AICA) (Figure ). A further intervention was declined by the patient and angiographic follow-up in one year has been scheduled.
|
[[46.0, 'year']]
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F
|
{'26272635': 1, '12836076': 1, '9540333': 1, '22251512': 1, '20559067': 1, '9482191': 1, '7862961': 1, '17652919': 1, '10485631': 1, '8367056': 1, '9662588': 1, '12870551': 1, '11688695': 1, '1870670': 1, '18695896': 1, '22241393': 1, '34221759': 2, '22325476': 1, '25467205': 1, '17483974': 1, '24363145': 1, '30159216': 2}
|
{'8237924-1': 1}
|
2,221 |
6110681-1
| 30,175,009 |
comm/PMC006xxxxxx/PMC6110681.xml
|
Rare Hypothenar Myxoma Causing Ulnar Neuropathy: Histopathology and Treatment Pearls
|
A 55-year-old right-hand-dominant male with a history of acute inflammatory demyelinating process and a 3-month history of a left volar wrist and hypothenar soft tissue mass presented for evaluation. The patient was admitted to the hospital with generalized weakness, ataxia, and multiple sensory deficits, including complete lack of sensation in the left small finger and ulnar side of his hand. Although his sensory deficits were initially thought to relate to his generalized demyelinating disorder, on examination, the ulnar deficit was thought to be related to the mass in his hand. The mass was soft, nontender, and did not limit range of motion. Two-point sensation was absent in the ulnar nerve distribution distal to the wrist, and grip strength, while not measured objectively, was decreased compared with the contralateral side. There was no history of trauma, previous surgery, or other masses, and radiographs of the left upper extremity were normal. A lipoma was the suspected initial diagnosis. Magnetic resonance angiography of the left hand showed a soft-tissue mass of unclear etiology (Fig. ).\nOperative excision was performed under general anesthesia. A longitudinally oriented ulnar-sided skin incision was made over the mass, with dissection through the palmar fascia. The ulnar nerve and artery were dissected free from the mass, and Guyon’s canal was released. The mass was noted to be arising from beneath the hypothenar musculature. Grossly, it was tan-white, rubbery, and lobulated (Fig. ). Pathology confirmed the mass to be a benign myxoma (Fig. ). There were no postoperative complications. At 5 weeks postoperatively, the patient reported marked improvement in his numbness and weakness. He was found to have intact sensation in the ulnar nerve distribution distal to the site of the excised mass, and improved grip strength. The patient was asked to return in several months for repeat sensorimotor assessment, but was subsequently lost to follow-up.
|
[[55.0, 'year']]
|
M
|
{'18917127': 1, '12990622': 1, '10913223': 1, '25519827': 2, '22747359': 1, '15368634': 1, '23869282': 2, '9230966': 1, '30175009': 2}
|
{'3707327-1': 1, '4301402-1': 1}
|
2,222 |
6110811-1
| 30,186,825 |
comm/PMC006xxxxxx/PMC6110811.xml
|
A Novel PCCA Mutation in a Patient With Late-Onset Propionic Acidemia Identified by Genetic Diagnosis Panel
|
The child, a Chinese boy with birth weight of 3,450 g and gestational age of 39+6 week (his mother was 28-year-old, G1P1, with normal pregnancy), was screened in Genetics and Metabolism Department of the Obstetrics and Gynecology Hospital affiliated to the Nanjing Medical University at 3th day after birth. The results are shown in Table . MS/MS showed that he had elevated C3/C2 but C3 and 3-hydroxypropionate remained almost normal. In order to identify the etiology, the patient and his families were diagnosed by Genetic diagnosis panel in our hospital on 16 June 2016. Parents were healthy and non-consanguineous.\nGenetic diagnosis panel of genetic metabolic disease covers 51 diseases and 98 genes, of which Panel 1 covers 18 amino acid metabolism diseases and 35 genes, Panel 2 covers 17 diseases, and 42 genes of organic acid metabolic diseases and glycogen metabolism diseases, and Panel 3 covers 16 fatty acid metabolism diseases and 21 genes. Genomic DNA was extracted from the peripheral blood of the families using the OMEGA Genomic DNA Extraction Kit (OMEGA Biotech, USA). All mutations were verified by Sanger sequencing. Ion Torrent data extraction, sequence alignment and SNPs and Indels extraction were performed by using Ion Torrent Suite v3.0 software. After the resulting SNPs and indels were filtered by the dbSNP 137 database, HGMD, LOVD and other databases and Pubmed related literatures were retrieved, matching the reported pathogenic sites. Two pathogenic mutations (c.802C>T/c.827delG) were detected in the PCCA gene (Table , Figure ), among them, the variation rs774738181 (c.802C>T) was present on the dbSNP database which appeared to be “Likely pathogenic” in GenBank dbSNP (). we propose that this variation may be pathogenic. ().\nc.827delG was a frameshift mutation, leading to p.Gly276ValfsX46 mutation of amino acid sequence, which has not been reported. According to the ACMG principle, the mutation was a highly reliable pathogenic mutant type. The protein function was predicted by SIFT and PolyPhen, and the results were all harmful. The identified pathogenic mutations and suspected pathogenic mutations were confirmed by Sanger sequencing.\nAlthough two pathogenic mutations were detected in PCCA, two mutations were not reported before, and the 3-Hydroxypropionate in urine was only slightly increased. Therefore, the treatment was postponed and frequency of follow-up was increased.\nThe patient underwent 1 year of follow-up. MS/MS and GC/MS detection results were shown in Table . He remained asymptomatic and his blood ammonia and liver function were normal.\nWhen the child was 1 year old (in May of 2017), C3 and 3-Hydroxypropionate sudden elevated significantly, indicating the pathogenicity of c.802C>T and c.827delG. Thus we suggest that c.802C>T and c.827delG may be linked to late-onset PA.
|
[[28.0, 'year']]
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M
|
{'32819290': 2, '19157943': 1, '12409268': 1, '15059621': 1, '5727920': 1, '20549364': 1, '11592820': 1, '10820128': 1, '30186825': 2}
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{'7441651-1': 1}
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2,223 |
6111297-1
| 30,096,934 |
comm/PMC006xxxxxx/PMC6111297.xml
|
Epithelial-Mesenchymal Transition with Malignant Transformation Leading Multiple Metastasis from Disseminated Peritoneal Leiomyomatosis
|
This case study was approved by the Institutional Review Board (07-CR-085) of Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation.\nA 61-year-old G2P1AA1 woman presented with intermittent, progressive lower abdominal pain for three years. She had a medical history of hypertension that was controlled with medication. In 1993, she was diagnosed as having a uterine leiomyoma with leiomyomatosis of retroperitoneal tumors. Because of her desire to bear children in the future, she underwent myomectomy at that time. Subsequently, recurrent DPL developed two years later due to an enlarged palpable mass in the abdomen with intermittent abdominal pain. She underwent abdominal hysterectomy with left-sided oophorectomy and right ovary wedge resection in 1996. Results of the pathological report showed a borderline mucinous ovarian tumor of the left ovary. In 1998, recurrent DPL with left ureter involvement was noted, and the third operation involving debulking surgery and reteroneocystostomy was performed. Results of that pathological report showed no significant malignancy. She was regularly followed up at gynecologic clinics. In 1999, a 9 × 9-cm2 palpable pelvic mass was observed, and recurrent leiomyomatosis was suspected. A fourth laparotomy for tumor excision was suggested to the patient, but she refused. Hormone therapy with medroxyprogesterone (5-mg tablet daily) was performed until 2014 because of the progression of a thyroid nodular goiter and hypertension. After stopping hormone therapy, she reported intermittent, progressive lower abdominal pain, and a growing palpable mass was observed in the abdomen.\nOn admission, her temperature was 36.6 °C, blood pressure was 123/69 mmHg, heart rate was 83 beats/min, body weight was 59.8 kg, body height was 155.5 cm, and body mass index was 24.7 kg/m2. On pelvic examination, a huge tumor was palpable in the pelvis. Transvaginal ultrasonography showed huge solid tumors measuring about 15.17 × 24.48 × 10.51 cm3 in the pelvic field without free fluid in the cul-de-sac region (A–D).\nThe laboratory evaluation showed the following: white blood cell count, 13,520 µL; hemoglobin level, 7.8 g/dL; fibrin degradation product level, >10,000 ng/mL; follicle-stimulating hormone level, 15.98 mIU/mL; progesterone level, 1.58 ng/mL; and, estradiol level, 32.84 pg/mL. No significant sign of infection or internal bleeding was indicated by the laboratory data. There were no significantly increased tumor markers, such as the carbohydrate antigen 125 level (20.2 IU/mL), carbohydrate antigen 19–9 level (10.20 IU/mL), and carcinoembryonic antigen level (0.649 ng/mL). The chest X-ray showed nodular densities in the right lower lung ().\nContrast-enhanced whole-body computed tomography was performed and revealed three heterogeneous pelvic tumors, which were measuring approximately 9.4 × 8.6 × 9.3 cm3, 7.1 × 6.1 × 6.0 cm3, and 11.1 × 7.5 × 9.1 cm3, with multiple pulmonary nodules and multiple heterogeneous hepatic tumors. Therefore, distant metastasis was suspected. Severe hydronephrosis of the right kidney was obvious due to tumor compression (A–E).\nExploratory laparotomy was recommended and performed. Three individual bulky tumors were encountered in the retroperitoneal pelvic cavity, which were found by the direct compression of the urinary bladder and both ureters. A solitary tumor mass measuring about 14.5 × 12 × 9 cm3 and weighing 1400 g was excised from the left paracolic gutter with extensive abdominal peritoneal carcinomatosis. The smooth-surfaced tumor seedings were suspected to be benign myomatous lesions. The right ovary was found in the right pelvic cavity and adhered to the interbowel loops. The pelvic tumor partially invaded the urinary bladder (A–D). Nearly complete cytoreductive surgery was performed. The three individual bulky tumors were resected with the left ovary, right adnexa, paracolic gutter tumor, and cul-de-sac cells for cytology. Unlike complete staging surgery, she did not undergo omentectomy, appendectomy, and lymph node assessment.\nResults of the final pathology report showed that the largest encapsulated tumor had many spindle cells with multinucleated pleomorphic nuclei, extensive necrosis, and a mitotic index of 11/10 per high power field, which is consistent with high-grade leiomyosarcoma (differentiation score = 3, mitotic score = 2, necrotic score = 2, total score = 7; French Federation of Cancer Centers Sarcoma Group histologic grade 3). Immunohistochemistry staining showed that the tumor cells were reactive with desmin, negative with cytokeratin and CD117 (c-kit), and negative for estrogen and progesterone receptors. The immunohistochemical profile confirmed leiomyomatous differentiation in other small nodules that were seeding in the peritoneum and omentum, smaller pelvic masses with hypercellular spindle cell tumors, and minimal nuclear atypia, which were all indicative of leiomyomatosis. She was diagnosed as having a leiomyosarcoma arising from DPL at 61 years of age. Chemotherapy with doxorubicin caused the leiomyosarcoma.\nUnfortunately, she died approximately one month after the diagnosis because of rapid progression of pleural effusion due to malignancy.
|
[[61.0, 'year']]
|
F
|
{'34369191': 2, '9922202': 1, '2386147': 1, '33579891': 1, '32359372': 2, '17616942': 1, '31514467': 1, '29662568': 1, '8626146': 1, '29326430': 1, '10502446': 1, '25546607': 1, '30935077': 1, '20493472': 1, '3725244': 1, '20068330': 1, '31410732': 2, '26998373': 2, '28811330': 1, '3778588': 1, '22335043': 1, '8333475': 1, '20651304': 1, '10458552': 1, '7807463': 1, '17583244': 1, '15581996': 1, '27284430': 1, '9481573': 1, '16223449': 2, '30096934': 2}
|
{'1266363-1': 1, '8358528-1': 1, '6692793-1': 1, '4779833-1': 1, '7196235-1': 1}
|
2,224 |
6112024-1
| 30,061,514 |
comm/PMC006xxxxxx/PMC6112024.xml
|
Reverse Shoulder Arthroplasty for Primary Synovial Osteochondromatosis of the Shoulder with Massive Rotator Cuff Tear and Marked Degenerative Arthropathy
|
The patient was a 75-year-old woman, who had developed left shoulder pain five years earlier without any known precipitating factor. She presented to our department because of gradual difficulty in raising her left arm and worsening pain. Physical examination on presentation did not reveal any swelling or feeling of heat in the left shoulder. The range of motion of the left shoulder showed extremely severe restriction; namely, flexion 80°, abduction 60°, and external rotation 0°, and prominent impingement symptoms were found. On plain radiographs and computed tomography (CT), prominent shoulder arthropathic changes and numerous calcified lesions around the joint were found. On plain magnetic resonance imaging (MRI), around the shoulder an irregular hypointense region was identified in the center on T1-enhanced images and on T2-enhanced images (). Routine blood examinations did not reveal any obvious abnormalities. This patient was diagnosed with synovial osteochondromatosis associated with a massive tear of the rotator cuff and shoulder arthropathic changes, for which the treatment RSA was chosen.\nIntraoperative findings: A delto-pectoral approach was used. Full-thickness tears of the supraspinatus and infraspinatus tendons were found. When the joint capsule was incised, synovial proliferation and a whitish mass-like lesion, seemingly adherent to the synovium, were found. The intraarticular mass was resected to the extent possible, but the cartilage in the humeral head was severely damaged, while that in the glenoid cavity had almost disappeared. After loose body resection, RSA was performed.\nThe postoperative course was uneventful, with the left shoulder pain disappearing from early in the postoperative period. At one year postoperatively, there was no recurrence of pain and the left shoulder range of motion showed improvement to flexion 140°, abduction 130°, and external rotation 30°. Moreover, no complications such as recurrence of osteochondromatosis, implant loosening, or infection were seen.\nThe loose body was found to consist of a cartilage component and bone tissue with hyalinization (). No findings indicative of malignancy were evident, and since nodular cartilage arrangement was found, primary osteochondroma was diagnosed. Synovial osteochondromatosis could be diagnosed.
|
[[75.0, 'year']]
|
F
|
{'16337537': 1, '15726326': 1, '598088': 1, '18153904': 1, '25861500': 2, '10077803': 1, '468226': 1, '11195106': 1, '14673413': 1, '8542376': 1, '14814164': 1, '3180568': 1, '19213576': 1, '26005571': 1, '12522395': 1, '17957072': 1, '11043962': 1, '15891738': 1, '908703': 1, '18308184': 1, '30061514': 2}
|
{'4377398-1': 1}
|
2,225 |
6112072-1
| 30,186,640 |
comm/PMC006xxxxxx/PMC6112072.xml
|
Giant Prolactinoma of Young Onset: A Clue to Diagnosis of MEN-1 Syndrome
|
An eight-year-old boy initially presented to us in 2008 with progressive headache and visual disturbances. His imaging revealed a giant pituitary tumour (59 x 45 x 42 mm) with extrasellar extension () with initial prolactin of 91,800 μg/L confirming the diagnosis of giant prolactinoma. Initially, he responded well to high doses of cabergoline (7 mg/week) with normalization of prolactin and total tumour shrinkage. A few years later, he developed recurrence of the tumour, which was resistant to cabergoline therapy (), and underwent transcranial excision of the tumour in 2013. During the immediate postoperative period, he developed recurrent hypoglycaemic episodes, which was confirmed to be endogenous insulin dependent hypoglycaemia biochemically (insulin was 15.9 µIU/mL and C-peptide was 3.94 ng/mL when random blood glucose was less than 2.1 mmol/L). Imaging located a well circumscribed lesion (20 x 12 x 10 mm) in the head of pancreas. He underwent enucleation of the tumour, and that was confirmed as an insulinoma histologically with benign characteristics (Ki67<1%). Six months after the pituitary surgery he received three-field radiotherapy (4500 cGy) and continued on cabergoline (3.5 mg/week) resulting in declining prolactin levels. His baseline echocardiography was normal. He had normal calcium at presentation, but currently he is being evaluated for new onset primary hyperparathyroidism (total calcium 2.98 mmol/L [normal range: 2.40-2.55], intact PTH 88.2 pg/L [12-60]).
|
[[8.0, 'year']]
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M
|
{'23321498': 1, '26126205': 1, '24088550': 1, '17879353': 1, '31555208': 2, '26561015': 1, '23662975': 1, '28346715': 1, '25594862': 1, '25792376': 1, '22612670': 1, '22723327': 1, '22584705': 1, '27138902': 1, '11836268': 1, '19223454': 1, '15670192': 1, '24536090': 1, '30186640': 2}
|
{'6722186-1': 1}
|
2,226 |
6112084-1
| 30,186,638 |
comm/PMC006xxxxxx/PMC6112084.xml
|
An Adult with a Remnant Urachus Anomaly Diagnosed in the Emergency Department
|
A 21-year-old male presented to an emergency department (ED) with a two-day history of purulent drainage from his umbilicus. He denied pain. He reported that he had experienced a similar episode one year prior which was diagnosed in an ED as umbilical cellulitis and resolved with oral antibiotics. Interestingly, the patient noted that since childhood he had recurrent episodes of what he described as a “pulling sensation” in the umbilicus and suprapubic areas. The patient had seen multiple specialists regarding the sensation but the workups had been unremarkable.\nThe patient had no pertinent medical or surgical history. The family and social history were unremarkable. He did not take any medications and denied drug and alcohol use.\nVital signs on presentation were blood pressure 139/82 torr, heart rate 55 beats per minute, respiratory rate 15 breaths per minute, and temperature 98.5 degrees F. (oral temperature) with a pulse oximetry reading of 100% on room air.\nYellowish discharge was noted from center of the umbilicus. The periphery of the umbilicus was erythematous with mild tenderness to palpation. The physical examination was otherwise unremarkable.\nThe complete blood count and basic metabolic panel were within normal limits. The urinalysis was unremarkable.\nA computed tomography (CT) scan of the abdomen and pelvis with oral and intravenous contrast revealed an urachal remnant arising from the anterior/superior margin of the bladder and extending to the umbilical region. The remnant consisted of a thin fibrous band of tissue measuring up to 4.6 mm in thickness near the umbilicus. The band narrowed to a minimum of 2 mm along its course. No umbilical fluid collection was identified. The abdominal fat posterior to the umbilicus showed no inflammatory reaction (Figures and ).\nAn infected umbilical-urachal sinus was suspected. The patient was treated with oral antibiotics since the patient had success with a similar presentation a year prior to the ED visit. He was instructed to follow up with a urologist in the outpatient setting for definitive treatment. His infection was resolved. He later underwent an excision of the urachal remnant. Histology showed no evidence of malignant transformation.
|
[[21.0, 'year']]
|
M
|
{'27393484': 1, '22084806': 2, '8448315': 1, '25741124': 2, '25140240': 1, '19829803': 2, '27831842': 1, '30186638': 2}
|
{'4341378-1': 1, '2740315-1': 1, '3196200-1': 1}
|
2,227 |
6112089-1
| 30,186,645 |
comm/PMC006xxxxxx/PMC6112089.xml
|
Prosthetic Joint Infection from Carbapenemase-Resistant Klebsiella pneumoniae Successfully Treated with Ceftazidime-Avibactam
|
A 26-year-old Italian Caucasian male had a trauma from a fall on July 2014, with multiple fractures including left hemipelvis with luxation of coxofemoral joint (managed with reduction and osteosynthesis of the posterior acetabular wall), distal third of the right femur (treated with an osteosynthesis with plate and screws), and distal diaphysis of the right fibula (osteosynthesis plate and screws) along with facial skull trauma and chest trauma. All surgeries were executed in late 2014, and only perioperative antibiotic prophylaxis had been administered.\nHe also had a history of fracture of the left femur at 11 years of age treated with osteosynthesis with a rod that was subsequently removed, Von Willebrand disease, and depressive disorder.\nOn July 11, 2016, he was admitted to orthopedic surgery for redness and swelling of the right knee joint with a fistula on the right distal limb.\nMagnetic resonance imaging (MRI) of the right knee and femur showed osteomyelitis of the distal femur ().\nThe patient underwent surgery with removal of implants, a fistulectomy of the right femur was performed, biopsies were collected, sonication of the plate was performed, and a knee brace was placed.\nThe same CRKP strains were isolated both on cultures of biopsies and on prosthetic material after sonication. Bacterial identification and antimicrobial susceptibility testing were performed using the Phoenix Automated Microbiology System (Becton Dickinson Diagnostic Systems, Sparks, MD, USA). Confirmatory MIC testing for imipenem and meropenem was carried out by gradient test for MIC determination (Etest Liofilchem, Roseto degli Abruzzi, Italy) and interpreted in accordance with the European Committee on Antimicrobial Susceptibility Testing (EUCAST) breakpoints [].\nThe Klebsiella pneumoniae isolate was further evaluated for the presence of carbapenemase using a phenotypic assay (Rosco, Stamford, CT, USA) containing discs of meropenem (10 μg), meropenem + phenyl boronic acid (PBA), meropenem + dipicolinic acid (DPA), and meropenem + cloxacillin (CL). The organism was confirmed as a class A (KPC) carbapenemase enzyme producer. In detail, KPC enzymes are inhibited by phenylboronic acid.\nA real-time PCR, detecting several genes involved in carbapenem resistance (Xpert Carba-R; Cepheid, Sunnyvale, CA, USA), was performed to confirm the results of the phenotypic test. More specifically, this method allows for the detection and differentiation of the most frequent carbapenemases gene families (bla, bla, bla, bla, and bla) in Gram-negative bacteria. The real-time PCR resulted positive for KPC and negative for VIM, OXA-48, IMP-1, and NDM.\nOn July 20th, the patient was started on colistin-fosfomycin and trimethoprim-sulfamethoxazole and then transferred to the Infectious Disease (ID) ward. We switched antibiotic therapy to colistin (loading dose of 9 million IU, and then 4.5 million IU BID), fosfomycin (4 g every eight hours), and tigecycline (loading dose of 100 mg, and then 50 mg BID). After the switch, he reported frequent nausea, while renal function remained normal and acute phase reactants remained elevated. shows the kinetics of white blood cells and acute phase reactants, and the antibiotics susceptibility test.\nOn August 4th, the patient was transferred to Orthopedic Surgery for resection of the distal femur along with minimal resection of the proximal fibula with positioning of a cemented Stage one® (Zimmer Biomet, Warsaw, IN, USA) spacer with an intramedullary rod in the femur. Samples of both bones were cultured, and tissue collected during surgery was negative. On August 5, the patient was transferred back to ID ward, and because of the onset of fever, poor tolerance of antibiotic therapy, and increase of acute phase reactants, we requested susceptibility testing for ceftazidime/avibactam (C/A) to our Bacteriology Laboratory. Sensitivity to C/A was confirmed using the specific disc (BD) provided by AstraZeneca, Molndal, Sweden. Resistance to carbapenems was further confirmed with the Xpert® Carba-R molecular diagnostic system (Cepheid, Sunnyvale, CA, USA). On August 19 after approval from our ethics committee for off-label use, we started treatment with ceftazidime/avibactam at a dose of 2.5 g TID for 2 weeks. In the subsequent days, the patient's clinical condition and laboratory tests improved with healing of the wound except for a fistula in the middle of the wound (fistula and rectal swabs were negative for CRKP). On September 2, a technetium-99 bone scan was performed showing distal uptake in the site of surgical intervention (which was deemed normal given that less than 12 months had elapsed from surgery). On September 16, the patient was transferred to the Orthopedic Surgery ward for a surgical curettage of the fistula. After being transferred back to the ID ward, the patient remained afebrile and daily care of the wound showed no discharge and no fibrosis. Cultures of samples taken during the curettage were negative. On October 14, the cemented spacer was removed in the Orthopedic Surgery ward and definitive knee prosthesis was positioned.\nThe patient was discharged from the ID ward on September 20, 2016. shows a timeline reviewing the events presented in this case report. During the latest orthopedic follow-up visit on February 23, 2017, the patient had no signs and symptoms of infection, was walking with the help of crutches, and continued being treated with physical therapy.
|
[[26.0, 'year']]
|
M
|
{'22578641': 1, '24252194': 1, '26846351': 1, '28559250': 1, '28610832': 1, '24813874': 2, '29671219': 1, '30662878': 1, '34408454': 2, '34379310': 1, '18534412': 1, '21793988': 1, '20473541': 1, '27942218': 1, '23592813': 1, '33195289': 1, '28785404': 1, '24867988': 1, '30186645': 2}
|
{'4336167-1': 1, '4336167-2': 1, '4336167-3': 1, '8364839-1': 1}
|
2,228 |
6112140-1
| 30,149,805 |
comm/PMC006xxxxxx/PMC6112140.xml
|
Use of erbium laser in the treatment of persistent post-radiotherapy laryngeal edema: a case report and review of the literature
|
We report a case of a 69-year-old male patient with post-radiotherapy laryngeal edema. The patient was treated with tumor resection, right selective neck dissection of levels II to IV, and adjuvant radiotherapy due to a pT2N1M0R0 oropharyngeal squamous cell carcinoma of the right tonsil. In the 2 years following radiotherapy, the patient was treated six times as an inpatient due to acute dyspnea. The endoscopic findings of the larynx always revealed a massive edema of the arytenoid area (Fig. , upper). Treatment included corticosteroid/adrenalin inhalation with systemic corticosteroids. Each time, subjective and objective recovery were transient. The endoscopic and radiologic findings revealed no indications of tumor recurrence. As an outpatient, the patient underwent multiple sessions of lymphatic massage drainage without improvement. Treatment with proton pump inhibitors also showed neither subjective nor objective benefits.\nTwo and a half years after radiotherapy, the patient underwent transoral laser microsurgery of the arytenoid area. An erbium laser was used. The laser was set at 103 J/cm2 and 10 Hz. To prevent postoperative synechia and/or webs, only the right arytenoid was assessed. This intervention aimed to minimize the edema without causing severe thermal tissue damage, which could lead to additional edema. Therefore, the cranial surface of the right arytenoid was pulse targeted to achieve a shrinking effect. Subsequently, multiple targeted holes were made in the tissue. Edema fluid was emptied from the channels. The intraoperative effect was slightly obvious (Fig. ). The patient remained under general anesthesia. The day after the procedure, microlaryngoscopy was performed. No additional edema was observed. The right arytenoid was still shrunken, and the patient was extubated.\nThe patient was admitted 2 weeks later to our department due to acute dyspnea. However, endoscopic examination of the larynx revealed a slight edema reduction of the right arytenoid. After conservative treatment with inhalation and systemic corticosteroids, the same procedure was performed for the left arytenoid, resulting in similar intraoperative edema reduction (Fig. ). In January 2015, 2 months later, endoscopic findings revealed a slight edema reduction of the left arytenoid area, whereas the postoperative status of the right arytenoid remained stable. No synechiae, webs, or local swelling were observed. The patient also noted a slight improvement of the dyspnea. Thus, a new procedure was performed using the same surgical technique, this time in both arytenoids, with the same intraoperative findings. Again, no synechiae or webs were observed postoperatively.\nDuring the next 6 months, the patient underwent three procedures in both arytenoids using the same surgical technique, with the same intraoperative findings. The erbium laser settings varied between 100 and 200 J/cm2 and 3 and 10 Hz, depending on the precise exposure of the cranial surface of the arytenoid area. No complications were observed. Endoscopic findings of the larynx at 2 months after the final procedure revealed a massive improvement. The patient experienced no symptoms. Thirty months after the final procedure, no additional edema was observed (Fig. , bottom).
|
[[69.0, 'year']]
|
M
|
{'20171520': 1, '2032882': 1, '3624628': 1, '8263388': 1, '16671101': 1, '11360460': 1, '24114067': 1, '33361747': 1, '7055780': 1, '11074514': 1, '12516067': 1, '21217966': 2, '8427504': 1, '17151839': 1, '30149805': 2}
|
{'3010544-1': 1, '3010544-2': 1, '3010544-3': 1, '3010544-4': 1}
|
2,229 |
6112214-1
| 30,186,651 |
comm/PMC006xxxxxx/PMC6112214.xml
|
An Uncommon Case of Pyogenic Spondylodiscitis Caused by Gemella morbillorum
|
The patient was an 81-year-old man with a history of left total hip replacement, open discectomy at the L4/5 level more than 10 years prior, percutaneous coronary intervention 3 years prior, and periodontitis detected 1 month before presentation. He suffered from severe back pain of 2-day duration. Plain lumbar spine radiographs showed spondylosis but no signs of fractures (). Laboratory tests were significant for a white blood cell count of 1.2 × 104 cells/μl and C-reactive protein level of 13.8 mg/dl (). He was admitted for treatment. Two days after admission, magnetic resonance imaging of the lumbar spine revealed discitis at the L5/S level (). Punctures of the disc were performed from both the left and right side under fluoroscopy, and two samples were obtained. Two sets of blood cultures and urine cultures were collected at the same time. Empiric therapy was started with vancomycin 1 g every 12 hours and ceftriaxone 1 g every 24 hours combined with lumbosacral orthosis. The culture of the disc aspirate was positive after 6 days, with the causative agent identified as G. morbillorum based on matrix-assisted laser desorption/ionization time-of-flight mass spectrometry analysis, performed with a Bruker Daltonics Microflex LT system (Bruker Daltonics, Germany). Blood and urine cultures were negative. Transthoracic echocardiogram showed no evidence of endocarditis. We could not perform the broth microdilution method for susceptibility testing because the isolate did not grow in the wells. Instead, we used the E-test method (SYSMEX bioMérieux) for determining susceptibility to penicillin G. Susceptibility of the isolate was interpreted by applying the Clinical and Laboratory Standards Institute (CLSI) M45-ED3. The minimum inhibitory concentration (MIC) of the isolate for penicillin G was 0.012 μg/ml, which was interpreted as susceptible. Nine days from the initial treatment, antibiotic therapy was changed to ampicillin 2 g every 6 hours for 4 weeks. Then, oral amoxicillin was administered for 3 weeks. Lumbago resolved after 4 weeks of treatment. The patient was discharged from the hospital after 6 weeks of treatment. The isolate was referred to the Department of Clinical Laboratory Medicine, Kyoto University Graduate School of Medicine, for 16S ribosomal RNA sequence analysis. A BLAST search for the sequence in GenBank database gave 99.86% identity (1418/1420 bp) as G. morbillorum (GenBank accession number L14327).
|
[[81.0, 'year']]
|
M
|
{'16708233': 1, '8783715': 1, '23533214': 1, '34659942': 2, '22033610': 1, '16230205': 1, '17148095': 1, '17577836': 1, '12973290': 1, '7827381': 1, '33537207': 2, '22628599': 1, '11923320': 1, '20118833': 1, '19713361': 1, '9641197': 1, '30186651': 2}
|
{'8491562-1': 1, '7838709-1': 1}
|
2,230 |
6112268-1
| 30,186,647 |
comm/PMC006xxxxxx/PMC6112268.xml
|
A 13-Year-Old with Coexistence of Gastric Volvulus and Leprosy: A Case Report of Two Rare Entities
|
A 13-year-old female of the Marshallese origin presented to the emergency department with complaints of nausea; nonbloody, nonbilious vomiting; and abdominal pain []. The patient denied any recent fevers, and no rash was reported. Review of systems was notable for the left ear drainage. Immunization status was unknown, and she was not reported to have been previously treated for any significant illness, though the history was limited by the patient's custodial circumstances. She lived in a small house in rural North Carolina with 21 other Marshallese immigrants and was cared for by relatives who had assumed care for her at the time of her immigration five years before.\nExamination during the initial hospital visit revealed dehydration and acute otitis media with rupture of the tympanic membrane. Laboratory results revealed leukocytosis, prerenal azotemia, elevated liver enzymes, and mildly elevated lipase (). Computed tomography (CT) showed scattered focal pulmonary infiltrates, splenomegaly, and a markedly distended stomach without an obvious focus of mechanical obstruction (). Cytomegalovirus and Epstein–Barr virus serology were requested with results suggesting prior exposure. She was admitted with a presumptive diagnosis of gastroparesis secondary to a nonspecific viral infection and possible mild pancreatitis. An NG tube was placed resulting in high volume output. Her symptoms gradually improved over several days with IV fluid support and bowel rest; her NG was successfully removed, and she was discharged home tolerating a regular diet.\nTwo days after discharge, the patient returned with recurrence of her prior symptoms. The patient appeared acutely ill with dehydration. On lung exam, scattered crackles were noted. She was also noted to have diffuse small nodular lesions most apparent on her hands, feet, lower legs, and face (). An advocate placed with the family reported her concern for additional symptoms of chronic weight loss and productive cough with posttussive emesis and reported the skin changes to have been present for months. Prior exposure to or testing for tuberculosis was unknown. The patient did not report symptoms of peripheral neuropathy.\nLaboratory results revealed a relatively increased white blood cell count (21.4 × 109 per liter) with significant worsening of her renal function (creatinine of 2.45 mg/dL which had previously been normalized with rehydration) and persistent mild elevation in liver enzymes and lipase. Respiratory viral screening was positive for rhino/enterovirus. Repeat CT imaging showed persistent gastric dilation and splenomegaly ().\nThe patient was placed on empiric antibiotics for community-acquired pneumonia and put on reverse isolation with concern for tuberculosis. Skin biopsies of multiple lesions were obtained and sent for pathology and culture (). Because multiple attempts to place an NG tube failed and vomiting failed to respond to conservative measures, endogastroduodenoscopy was pursued. The scope passed into the stomach and through the pylorus easily, reaching a normal-appearing proximal duodenum. However, a feeding tube could not be passed beyond the pylorus and was left just proximal to the pylorus for decompression. Stomach mucosa was described as having a “cobblestone” appearance in places, with one area of ulceration possibly due to prior nasogastric tube. Stomach biopsies revealed Helicobacter pylori and chronic active gastritis, but were negative for AFB.\nDue to persistent evidence of gastric outlet obstruction, an upper gastrointestinal study was performed which suggested gastric volvulus. The patient proceeded to gastropexy via open gastrostomy tube placement. The surgeons reported an extremely large and patulous stomach that had twisted mesenteroaxially. Gastric aspirates were negative for AFB.\nBiopsies of the skin lesions confirmed the presence of many AFB with changes consistent with lepromatous leprosy due to M. leprae (). Additionally, the serial sputum samples were positive for AFB, and culture of the skin sample for AFB showed evidence of growth within a few days, suggesting Mycobacterium tuberculosis (MTB) pulmonary infection and possibly disseminated MTB given selectivity of the culture medium.\nIn coordination with the health department and the infectious disease service, the patient was initiated on treatment for presumed disseminated MTB, noting that this would also provide coverage for M. leprae. However, polymerase chain reaction (PCR) testing from the sputum samples and skin samples failed to demonstrate MTB, while PCR testing performed at the Hansen's Disease Center of skin samples was positive for M. leprae. Retrospectively, it was felt that growth detected on the AFB culture was due to metabolism by an unusually large inoculum.\nGradually, the patient's oral intake improved. With hydration, her renal function again returned to normal. After several weeks of inpatient treatment, the patient discharged to complete therapy through the health department. Symptoms of gastric outlet obstruction have not returned.
|
[[13.0, 'year']]
|
F
|
{'27592500': 1, '18678603': 1, '27438203': 2, '19238115': 1, '27209077': 1, '7971596': 1, '21762573': 1, '27186586': 1, '15297472': 1, '25675654': 1, '28571290': 1, '27192519': 1, '28116186': 2, '34038422': 1, '8170880': 1, '30186647': 2}
|
{'5220437-1': 1, '4938280-1': 1}
|
2,231 |
6112871-1
| 30,113,485 |
comm/PMC006xxxxxx/PMC6112871.xml
|
Late onset adrenal insufficiency after adrenalectomy due to latent nonclassical 21-hydroxylase deficiency
|
In October 2017, a 71-year-old man visited our hospital due to moving difficulties and visual hallucination after experiencing general fatigue for 3 months. He reported dizziness and increased fatigue, 3 weeks prior to presentation, followed by urinary frequency, urinary incontinence, and a fall, 2 weeks later. To investigate his symptoms, head computed tomography (CT) and blood examination were conducted, which revealed a sodium level of 119 mEq/L, and he was hospitalized. Upon admittance, the patient underwent a physical examination that revealed a height of 138.5 cm, a weight of 49.0 kg, and a body mass index of 25.5 kg/m2. His temperature was 36.2°C and blood pressure 127/67 mm Hg. Pigmentations were detected, which were more visible around the lips, on the tongue, and fingers. Although the penis and scrotum were normal in size, the testicles measured small on an ultrasound scan.\nHis medical history revealed a growth spurt at approximately 8 years of age and the appearance of pubic hair at 10. His height did not increase past 139 cm from the age of 12. In his thirties, he suspected infertility, which was not investigated. At 49 years old in January 1996, he underwent a left adrenalectomy because of a heterogenous incidentaloma, with a size of approximately 3 to 4 cm. Laboratory investigations at the time revealed adrenocorticotropic hormone (ACTH): 102.2 (7.2–63.3) pg/mL, cortisol: 14.6 (6.24–18.0) μg/dL. 17-Ketosteroids (17-KS) were also reported to be elevated. The detailed results of these tests are summarized in Table . Because malignancy could not be fully excluded, adrenalectomy was performed. The mass was pathologically diagnosed as an adenoma and not malignant. After operation, although he was prescribed prednisolone, he did not keep up with his follow-up visits at the hospital because of no symptoms.\nAbdominal CT revealed almost normal right adrenal gland (Fig. ). Laboratory evaluations revealed ACTH level of 1820 (7.2–63.3) pg/mL and cortisol level of 3.11 (6.24–18.0) μg/dL. The results of the other tests are presented in Table . Therefore, a diagnosis of primary adrenal insufficiency was suspected, and the patient immediately received a dose of 100 mg hydrocortisone intravenously. This initial dose was followed by a maintenance regimen of intravenous hydrocortisone diluted in saline as follows: 200 mg for 2 days, 150 mg for 3 days, 100 mg for 3 days, and 50 mg for 3 days. Thereafter, he received 15 mg hydrocortisone orally. During this time, the patient also received approximately a liter of physiologic saline per day. Sodium levels were monitored and reached 126 meq/L on day 2, 131 meq/L on day 3, and 138 meq/L on day 7 after hospitalization. As a result, the patient's symptoms improved after a few days. An ACTH-stimulating test was performed, with the results revealing low response of cortisol 17-hydroxyprogesterone (17-OHP). The basal levels were low in the former and high in the latter. The complete results are summarized in Table . Therefore, a diagnosis of primary adrenal insufficiency was made. No anti-adrenal cortex antibody was detected. The patient was discharged on day 34 with a daily oral dose of 15 mg of hydrocortisone.\nWe suspected nonclassical 21-OHD, and searched for the most common CYP21A2 mutations by polymerase chain reaction (PCR) as per previous studies,[ using the primers previously reported for these mutations,[ after obtaining the patient's informed consent, looking for the large gene deletion or conversion, and the following 9 micro conversion-derived mutations: P30L in exon 1, 656A/C > G in intron 2, I172N in exon 4, V281L in exon 7, Q318X in exon 8, R356W in exon 8, the cluster I236N, V237E and M239K in exon 6, L307+T in exon 7, and 8 bp-del in exon 3.[ The mutations positive in our patient were micro mutation I172N and heterozygous large gene deletion or conversion leading to the diagnosis of nonclassical 21-OHD.
|
[[71.0, 'year']]
|
M
|
{'19037697': 1, '29348269': 1, '26594092': 1, '9556656': 1, '8706282': 1, '12213672': 1, '26082286': 1, '24654754': 1, '23388220': 2, '24077358': 1, '12594318': 1, '26336836': 1, '21689130': 1, '25736066': 1, '29390437': 2, '30113485': 2}
|
{'5758139-1': 1, '3577455-1': 1}
|
2,232 |
6112881-1
| 30,142,813 |
comm/PMC006xxxxxx/PMC6112881.xml
|
Gastric varices and splenic vein obstruction during steroid treatment for autoimmune pancreatitis
|
A 50-year-old man presented to the emergency department following 2 days of lightheadedness, abdominal pain, and melena. His distant past medical history was significant for hypertension and transfusion-requiring erosive gastritis. In addition, he reported having been diagnosed with AIP 2 weeks prior at a geographically-proximate academic medical center, where a mass at the head of the pancreas was identified after he presented with abdominal pain, elevated lipase, and hyperglycemia. At that time, he was prescribed prednisone 20 mg PO b.i.d., therapy with which he was compliant. Additional medications prior to admission were diltiazem and tramadol. He has no history of drug or alcohol abuse and denies use of nonsteroidal anti-inflammatory drugs.\nOn presentation, the patient was in no apparent distress, afebrile, and not hypoxic with a blood pressure of 175/39 mm Hg, a pulse of 140, and a respiratory rate of 18. On physical examination, bowel sounds were normoactive and the abdomen was soft without guarding or rigidity, but significant for marked epigastric tenderness without rebound. There was no palpable splenomegaly, Castell sign was negative and percussion of Traub Space was tympanic. There was no appreciable jaundice on the integument, the sclera, or the oral frenulum. Melena was present on rectal examination. Complete blood count revealed a white blood cell count of 46,000/mL, a hemoglobin of 9.2 mg/dL (baseline 11), and platelets of 96,000/mL. We attributed the leukocytosis to a combination of steroid therapy and stress response to the gastrointestinal bleed. The patient's metabolic panel was significant for a creatinine of 1.8 mg/dL, aspartate aminotransferase of (AST) 40 U/L, alanine aminotransferase of (ALT) 59 U/L, a total bilirubin of 0.7 mg/dL, serum glucose of 300 mg/dL, and a lipase of 31 U/L. IgG4 levels were not drawn as they were not pertinent to the immediate management of the patient's acute gastrointestinal bleed. The patient became acutely hypotensive, at which time an electrocardiogram revealed sinus tachycardia at a rate of 120 beats per minute. Following fluid resuscitation, the hemodynamic compromise resolved. Subsequent hemoglobin measurements revealed a temporal decrement from 9.2 to 7.8 mg/dL. Following initiation of maintenance fluids and a famotidine drip, the patient was admitted to the intensive care unit, where he also received blood transfusions. Due to concerns regarding diagnostic clarity and the veracity of the AIP diagnosis in the setting of gastrointestinal bleeding, the steroid therapy was discontinued.\nOn hospital day 2, the patient had continued abdominal pain and an additional episode of transfusion-requiring melena, absent vital sign abnormalities. The remainder of laboratory studies were significant for a platelet count of 79,000/mL, a hemoglobin A1c (HbA1c) of 7.0, and a reduction in leukocyte count to 24,500/mL, as expected following discontinuation of steroid therapy. The maintenance fluids and famotidine drip were continued with morphine as needed for abdominal pain.\nOn hospital day 3, esophagogastroduodenoscopy (EGD) revealed multiple erosions in the duodenal bulb with surrounding mucosal edema and a 3.5 cm ulcer with an adjacent crater and exudate in the bulb on the anterior wall. Erosive gastritis was also present in the antrum, body, and fundus of the stomach; tissue biopsy was negative for H Pylori. Grade 2+ gastric varices were found along the gastric fundus (Fig. ) and grade 1+ nonbleeding varices were present in the distal esophagus. Abdominal ultrasound with Doppler studies demonstrated moderate splenic enlargement, but patency of the splenic, hepatic, and portal veins.\nThe patient remained in stable condition throughout hospital days 4 to 7. The remainder of his hospital course was unremarkable with the exception of intermittent melena. He was discharged on hospital day 7, tolerating a full diet. Discharge medications were pantoprazole, prednisone 20 mg PO b.i.d., propranolol, sucralfate, and tramadol as needed for abdominal pain. In the outpatient setting, his care team titrated the dose of propranolol to avert significant heart rate decrement.
|
[[50.0, 'year']]
|
M
|
{'22081918': 1, '19398440': 1, '22919259': 1, '15166967': 1, '23232048': 1, '16843735': 1, '21613442': 1, '10773149': 1, '28197205': 1, '23382638': 1, '16586561': 1, '23076542': 1, '21287714': 1, '17525092': 1, '30142813': 2}
|
{}
|
2,233 |
6112910-1
| 30,167,348 |
comm/PMC006xxxxxx/PMC6112910.xml
|
Nasal Chondromesenchymal Hamartoma with Skull Base and Orbital Involvement: Case Presentation
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A 25-month-old female patient presented with recurrent mass lesion of the sinonasal tract. According to her history, she had feeding difficulties and nasal obstruction since birth. Microphthalmia on the right side was also noticed. Examination revealed mass lesion in the right nasal cavity and maxilla, however, biopsy was noninformative. Then, in October 2015 at the age of eight months, the patient was admitted to the department of maxillofacial surgery of local pediatric hospital. Computed tomography (CT) scans were obtained demonstrating a widespread tumor in the right nasal cavity with severe dislocation of the nasal septum, involving the right maxilla, ethmoid labyrinth, orbit, and cranial base (Figure , , ). In November 2015, the lesion was resected via a lateral rhinotomy in a piecemeal fashion until the bony boundaries of the maxillary antrum were reached around the tumor mass. Postoperative CT scans showed tumor remnants along the lateral nasal wall in proximity to the orbit (Figure ). No complications occurred after surgery. Histologic examination diagnosed chondromesenchymal hamartoma.\nThe patient presented at N.N. Burdenko National Research Center for Neurosurgery (Moscow, Russia) to obtain consultations concerning the management of the remaining lesion. New MRI obtained in August 2016 (Figure , left) showed remnants of the tumor without any deficit, and further follow-up was recommended.\nThe histological specimens were examined in the pathology department, and the diagnosis of NCMH was confirmed.\nOn low magnification, the resected material showed different histological patterns. It consisted of cellular cartilaginous islands and areas that contained fibro-osseous and mesenchymal components (Figure ). The cartilaginous component was composed of cellular cartilage foci with a hyaline cartilaginous matrix. The cells of that foci had a very low level of mitotic activity, and no signs of atypia were found (Figure ).\nA mesenchymal component was represented by quite cellular zones consisting of plump fibroblast-like cells without any mitotic figures and atypia (Figure ). Multiple bone trabeculae (Figure ) and irregular osteoid matrix (blue on Masson – Figure ) were found upon the connective tissue background. Areas of preexisting bone tissue with lamellar structure were present (Figure ).\nImmunohistochemical study with antibodies to SMA, S-100 protein, vimentin, MDM2, CDK4, desmin, CD34, and Ki-67 was performed. The cells of the cartilaginous component were strongly positive for vimentin (Figure ) and S-100 protein (Figure ). The cells of the mesenchymal component are positive for vimentin and focally positive for SMA (Figures and 4D). Immunohistochemical reactions with MDM2 (Figure ) and PanCK (AE1/AE3) were negative, positive control for PanCK was present in the mucosal epithelium (Figure ). Proliferative activity was very low according to Ki67 expression (Figure ).\nIn January 2017 (Figure , right) the next follow-up MRI study showed a progression of the lesion, and surgical treatment was indicated. Since the tumor was limited to the nasal cavity, ethmoid labyrinth, maxillary sinus, and was situated extradurally, the second surgery was performed using endoscopic endonasal technique (March 2017). No distinct margins of the lesion were detectable intraoperatively, therefore the superior portion of the tumor was left in order to avoid skull base penetration. Post-operative course was uneventful, no cerebrospinal fluid leak was detected.\nThe material from the second surgery was represented by hypocellular connective tissue with reactive inflammatory infiltration (Figure ), and the same irregular osteoid matrix (Figure ). In this case, the cartilaginous component was not found.\nAt six months of follow-up, the magnetic resonance imaging (MRI) demonstrated clear nasal cavity and remaining tumor mass in the ethmoid roof and sphenoid sinus (Figure , top). At one year, after the second surgery inflammatory changes had regressed, the tumor is stable and exhibits no increase in the volume (Figure , bottom).
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[[25.0, 'month']]
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F
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{'22356457': 1, '32851313': 1, '33723758': 1, '34352902': 1, '10321780': 1, '19568473': 2, '12745162': 1, '26138824': 2, '34164613': 1, '9537469': 1, '28385583': 1, '33571989': 1, '23348387': 1, '17091268': 1, '30167348': 2}
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{'4495949-1': 1, '2702054-1': 1}
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2,234 |
6113042-1
| 30,142,810 |
comm/PMC006xxxxxx/PMC6113042.xml
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Treatment of Rosacea using acupuncture for improving the local skin microcirculation
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The patient is a 52-year-old woman, who first presented to our clinic on November 30, 2016, with complaints of redness, red rash, and increased sebum excretion of the nose. These symptoms were sometimes accompanied by repeated occurrences of itching, papules, and pustules, and had been present for 1 year. Her symptoms were triggered by unknown causes and worsened after eating any greasy food. She was diagnosed with rosacea and tried medical treatments in other hospitals with metronidazole cream, antifungal drug, and steroidal ointments, but the therapeutic effect was poor. She made the decision to try acupuncture; she ceased receiving any medical treatment 1 month before she received the acupuncture treatment on March 13, 2017.
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[[52.0, 'year']]
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F
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{'25354241': 1, '27207355': 1, '21585062': 1, '29713744': 1, '29499802': 1, '22207450': 1, '2525010': 1, '28628749': 1, '26099423': 1, '28274361': 1, '25285620': 1, '20002660': 1, '25151931': 1, '25890456': 1, '18580161': 1, '28270193': 1, '26916576': 1, '29123119': 1, '28626508': 1, '12786702': 1, '25534374': 1, '26345379': 1, '24191171': 1, '33014112': 1, '26428269': 1, '29484096': 1, '30142810': 2}
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{}
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2,235 |
6113049-1
| 30,142,787 |
comm/PMC006xxxxxx/PMC6113049.xml
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Horner syndrome as a complication after thyroid microwave ablation
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The patient was a 44-year-old female patient who had presented with thyroid nodules for at least 5 years, had a history of atrial premature beats, and who had undergone an ovariohysterectomy almost 10 years previously. There was no history of hypertension, diabetes, or other infectious disease and allergies, except for hepatitis B. Thyroid ultrasound suggested a 15 × 35 mm solid cystic nodule located in the upper dorsal side of the right lobe of the thyroid gland (Fig. ). The nodule was well defined with regular form. Streaky bloodstream signals were observed in the interior and edges of the nodule. Pre-MWA thyroid function tests showed a thyroid-stimulating hormone level of 0.912 uIU/mL, free T3 of 4.61 pmol/L, free T4 of 13.30 pmol/L, thyroglobulin antibody of 14.46 IU/mL, and thyroid peroxidase antibody of 37.61 IU/mL.\nAfter being admitted from the outpatient department, the patient completed the remaining pre-MWA examinations. The results of an electrocardiogram, laryngoscopy, and lung computed tomography scan were all normal. The patient was given a principal diagnosis of nodular goiter. We decided to perform MWA given the small volume and benign character of the nodule. We used a MWA instrument (ECO-100A1; YIGAO Microwave System Engineering Co. Ltd, Nanjing, China), matched aseptic disposable MWA needle (ECO-100AL3; 100 mm in length, 1.6 mm in diameter), and 500 mL normal saline for cold fluid circulation for the ablation procedure. The output power setting was 35 W with a frequency of 2450 MHz. Moreover, ultrasound (GE, LogiQ-E9) was used for guidance before, during, and after the ablation.\nThe patient underwent MWA in November 2017. Considering that local anesthesia would not adequately reduce pain, talking, or coughing during the MWA procedure, we injected lidocaine into the skin puncture site with the assistance of intravenous anesthesia. The patient subsequently fell asleep and therefore, we did not monitor ptosis during the procedure. After confirming the effect of the anesthesia, we set up a liquid-isolating zone by injecting 10 mL normal saline into the space between the anterior capsule of the thyroid gland and the cervical anterior muscles, between the lateral capsule of the thyroid gland and the carotid artery, between the posterior capsule of the thyroid gland and the recurrent laryngeal nerve crossing area, and between the esophagus and the parathyroid gland. Next, we performed MWA from the deep to the shallow part of thyroid gland. The procedure was completed successfully.\nSubsequently, we advised the patient to remain laying down and abstain from drinking water for at least 2 hours. About 4 hours after MWA, the patient showed mild miosis and eyelid ptosis in her right eye but no enophthalmos, anhidrosis, or vascular dilatation. Her symptoms became more serious a day later and therefore, the patient underwent brain magnetic resonance imaging and examination by a neurological physician. Along with the clinical presentation, these assessments ruled out the possibility of oncothlipsis and the patient was finally diagnosed with HS as a rare complication of MWA (Fig. ). Therefore, routine treatment with mecobalamin was administered immediately.\nAfter 42 days of MWA, the patient's nodule showed a reduction in volume with ultrasound (Fig. ). However, after 5 months of follow up, the patient's miosis and ptosis had not been completely alleviated.
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[[44.0, 'year']]
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F
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{'17697179': 1, '28539793': 1, '28298940': 1, '33441131': 2, '22447813': 1, '15856482': 1, '5782083': 1, '11110927': 1, '28203621': 1, '33769287': 1, '34095479': 1, '22940229': 1, '11742325': 1, '18466837': 1, '8158744': 1, '15768422': 1, '25369752': 1, '15191478': 1, '18677509': 1, '30142787': 2}
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{'7805074-1': 1}
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2,236 |
6113389-1
| 30,186,228 |
comm/PMC006xxxxxx/PMC6113389.xml
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Presence of Middle Cerebellar Peduncle Sign in FMR1 Premutation Carriers Without Tremor and Ataxia
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Case 1 is a 65-year-old, right-handed male with 84 cytosine-guanine-guanine (CGG) repeats, who denied tremor and ataxia. On examination his blood pressure was 177/87 mmHg and his heart rate was 62 bpm. This was consistent with reported history of and treated with metoprolol and candesartan. His body mass index (BMI) was 29.3. On neurological examination, finger-to-nose touching was without tremor and his arm movements were normal. His deep tendon reflexes were 1 to 2+ in the upper extremities, 3+ at the knees, and 2+ at the ankles. His temperature sensation was normal and his vibration sensation was absent in both great toes. Tandem walking was performed normally. No cognitive abnormalities were present on neuropsychological examination and no psychiatric symptoms were reported.\nHis MRI demonstrated the emergence of a faint MCP sign (Figure ). His CC was slightly thin with minimal hyperintensity of the splenium of the CC. There was no significant atrophy but there was a hint of white matter hyperintensity in the insula bilaterally.
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[[65.0, 'year']]
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M
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{'23602499': 1, '25900641': 1, '15052536': 1, '17296852': 1, '27089882': 1, '27259564': 1, '25620901': 1, '28568317': 1, '32494560': 1, '31456732': 1, '23077007': 1, '29389022': 1, '17427188': 1, '32089651': 1, '14747503': 1, '11912110': 1, '27108270': 1, '27287737': 1, '28233916': 1, '11445641': 1, '27340021': 1, '20513237': 1, '19367323': 1, '23867198': 1, '28444183': 1, '18057083': 1, '28707277': 1, '32466255': 2, '32711390': 1, '20056738': 1, '28065649': 1, '27555610': 1, '16332642': 1, '18165273': 1, '12427636': 1, '10631132': 1, '23478018': 1, '32575683': 1, '28391068': 1, '22528549': 1, '33709078': 1, '30805918': 1, '17724287': 1, '27414076': 1, '25436181': 1, '24610885': 1, '30186228': 2}
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{'6113389-2': 2, '6113389-3': 2, '6113389-4': 2, '6113389-5': 2, '7277845-1': 1, '7277845-2': 1, '7277845-3': 1}
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2,237 |
6113389-2
| 30,186,228 |
comm/PMC006xxxxxx/PMC6113389.xml
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Presence of Middle Cerebellar Peduncle Sign in FMR1 Premutation Carriers Without Tremor and Ataxia
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Case 2 is a 50-year-old, right-handed male carrier with 102 CGG repeats who denied tremor and ataxia. He had a history of multiple concussions from sports injuries in high school and college.\nOn examination his blood pressure was 147/82 mmHg and his heart rate was 48 bpm. His BMI was 25.8. Finger-to-nose touching was without tremor, and deep tendon reflexes were symmetrical and 1+ at the upper extremities, 2+ at the knees, and 2+ at the ankles. His vibration sense was mildly decreased in the lower extremities. His tandem gait was normal. No cognitive abnormalities were present on neuropsychological examination and no psychiatric symptoms were reported.\nHe presented with the MCP sign on MRI (Figure ). He also had deep cerebellar white matter disease adjacent to the dentate nuclei, white matter hyperintensity in the splenium of the CC, mild volume loss involving the vermis and cerebellar hemispheres, and mesencephalic changes with widened third ventricle. In addition, an indentation in the superior aspect of the CC was thought to relate to a small vascular malformation or aneurysm.
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[[50.0, 'year']]
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M
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{'23602499': 1, '25900641': 1, '15052536': 1, '17296852': 1, '27089882': 1, '27259564': 1, '25620901': 1, '28568317': 1, '32494560': 1, '31456732': 1, '23077007': 1, '29389022': 1, '17427188': 1, '32089651': 1, '14747503': 1, '11912110': 1, '27108270': 1, '27287737': 1, '28233916': 1, '11445641': 1, '27340021': 1, '20513237': 1, '19367323': 1, '23867198': 1, '28444183': 1, '18057083': 1, '28707277': 1, '32466255': 2, '32711390': 1, '20056738': 1, '28065649': 1, '27555610': 1, '16332642': 1, '18165273': 1, '12427636': 1, '10631132': 1, '23478018': 1, '32575683': 1, '28391068': 1, '22528549': 1, '33709078': 1, '30805918': 1, '17724287': 1, '27414076': 1, '25436181': 1, '24610885': 1, '30186228': 2}
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{'6113389-1': 2, '6113389-3': 2, '6113389-4': 2, '6113389-5': 2, '7277845-1': 1, '7277845-2': 1, '7277845-3': 1}
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2,238 |
6113389-3
| 30,186,228 |
comm/PMC006xxxxxx/PMC6113389.xml
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Presence of Middle Cerebellar Peduncle Sign in FMR1 Premutation Carriers Without Tremor and Ataxia
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Case 3 is a 62-year-old, right-handed male carrier with 86 CGG repeats who denied tremor and ataxia. On examination his blood pressure was 125/88 mmHg and his heart rate was 88 bpm. His blood pressure was being controlled with irbesartan and hydrochlorothiazide. His BMI was 26.9. Finger-to-nose touching showed no tremor and his tandem walk was without difficulty. He had slight increased tone on the right and left extremities with symmetrical movement. Deep tendon reflexes were normal, and he scored a 2+ in all four extremities. His vibration sense, tactile sensation, and cold sensation were also normal. No cognitive abnormalities were present on neuropsychological examination and no psychiatric symptoms were reported.\nThe MRI revealed the MCP sign (Figure ). Additional white matter changes were seen in the splenium of the CC.
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[[62.0, 'year']]
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M
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{'23602499': 1, '25900641': 1, '15052536': 1, '17296852': 1, '27089882': 1, '27259564': 1, '25620901': 1, '28568317': 1, '32494560': 1, '31456732': 1, '23077007': 1, '29389022': 1, '17427188': 1, '32089651': 1, '14747503': 1, '11912110': 1, '27108270': 1, '27287737': 1, '28233916': 1, '11445641': 1, '27340021': 1, '20513237': 1, '19367323': 1, '23867198': 1, '28444183': 1, '18057083': 1, '28707277': 1, '32466255': 2, '32711390': 1, '20056738': 1, '28065649': 1, '27555610': 1, '16332642': 1, '18165273': 1, '12427636': 1, '10631132': 1, '23478018': 1, '32575683': 1, '28391068': 1, '22528549': 1, '33709078': 1, '30805918': 1, '17724287': 1, '27414076': 1, '25436181': 1, '24610885': 1, '30186228': 2}
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{'6113389-1': 2, '6113389-2': 2, '6113389-4': 2, '6113389-5': 2, '7277845-1': 1, '7277845-2': 1, '7277845-3': 1}
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2,239 |
6113389-4
| 30,186,228 |
comm/PMC006xxxxxx/PMC6113389.xml
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Presence of Middle Cerebellar Peduncle Sign in FMR1 Premutation Carriers Without Tremor and Ataxia
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Case 4 is a 60-year-old right-handed, male carrier with 74 CGG repeats who denied tremor and ataxia. On examination he had a blood pressure of 152/86 mmHG and a heart rate of 89 bpm. He had a BMI of 27.9. His neurological examination showed decreased vibration sense in the lower extremities. Deep tendon reflexes were 1–2+ in the upper and 2+ in the lower extremities. He had no rest or action tremor, gait ataxia, or dystonia. No cognitive abnormalities were present on neuropsychological testing and no psychiatric symptoms were reported.\nThe MRI showed mild cerebellar volume loss, with MCP sign bilaterally (Figure ) and subtle inferior cerebellar white matter changes.
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[[60.0, 'year']]
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M
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{'23602499': 1, '25900641': 1, '15052536': 1, '17296852': 1, '27089882': 1, '27259564': 1, '25620901': 1, '28568317': 1, '32494560': 1, '31456732': 1, '23077007': 1, '29389022': 1, '17427188': 1, '32089651': 1, '14747503': 1, '11912110': 1, '27108270': 1, '27287737': 1, '28233916': 1, '11445641': 1, '27340021': 1, '20513237': 1, '19367323': 1, '23867198': 1, '28444183': 1, '18057083': 1, '28707277': 1, '32466255': 2, '32711390': 1, '20056738': 1, '28065649': 1, '27555610': 1, '16332642': 1, '18165273': 1, '12427636': 1, '10631132': 1, '23478018': 1, '32575683': 1, '28391068': 1, '22528549': 1, '33709078': 1, '30805918': 1, '17724287': 1, '27414076': 1, '25436181': 1, '24610885': 1, '30186228': 2}
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{'6113389-1': 2, '6113389-2': 2, '6113389-3': 2, '6113389-5': 2, '7277845-1': 1, '7277845-2': 1, '7277845-3': 1}
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2,240 |
6113389-5
| 30,186,228 |
comm/PMC006xxxxxx/PMC6113389.xml
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Presence of Middle Cerebellar Peduncle Sign in FMR1 Premutation Carriers Without Tremor and Ataxia
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Case 5 is a 61-year-old, right-handed male carrier with 89 CGG repeats, who denied any history of tremor. He noted that he had no history of falling but he experienced some instances of unsteadiness when turning around, possibly attributable to a 4 cm difference in leg lengths secondary to a congenital vascular malformation in one leg that impacted growth. On examination, his blood pressure was 107/68 mmHg and his heart rate was 55 bpm. He had a BMI of 26.5. There was no sign of tremor during finger-to-nose touching. Along with having mild balance problems while turning, he had instability on tandem walking during the first few steps. With practice, he was able to perform at least 6 steps without missteps. He had normal reflexes in his upper extremities and knees, all +2. His right ankle reflex was 1+, and there was no reflex in his left ankle. He had decreased vibration sense bilaterally in both big toes but normal vibration sense at the ankles bilaterally. Additionally, pinprick sensation was slightly decreased in the great toe. No cognitive abnormalities were present on neuropsychological examination and no psychiatric symptoms were reported.\nOn MRI, the T2 images showed the MCP sign (Figure ) and white matter hyperintensity in the splenium of the CC. He also had mild brain atrophy.
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[[61.0, 'year']]
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M
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{'23602499': 1, '25900641': 1, '15052536': 1, '17296852': 1, '27089882': 1, '27259564': 1, '25620901': 1, '28568317': 1, '32494560': 1, '31456732': 1, '23077007': 1, '29389022': 1, '17427188': 1, '32089651': 1, '14747503': 1, '11912110': 1, '27108270': 1, '27287737': 1, '28233916': 1, '11445641': 1, '27340021': 1, '20513237': 1, '19367323': 1, '23867198': 1, '28444183': 1, '18057083': 1, '28707277': 1, '32466255': 2, '32711390': 1, '20056738': 1, '28065649': 1, '27555610': 1, '16332642': 1, '18165273': 1, '12427636': 1, '10631132': 1, '23478018': 1, '32575683': 1, '28391068': 1, '22528549': 1, '33709078': 1, '30805918': 1, '17724287': 1, '27414076': 1, '25436181': 1, '24610885': 1, '30186228': 2}
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{'6113389-1': 2, '6113389-2': 2, '6113389-3': 2, '6113389-4': 2, '7277845-1': 1, '7277845-2': 1, '7277845-3': 1}
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2,241 |
6113740-1
| 30,181,894 |
comm/PMC006xxxxxx/PMC6113740.xml
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Hemophagocytic syndrome in a cat
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A 12-year-old male castrated domestic shorthair cat was referred to the\nVirginia-Maryland College of Veterinary Medicine Veterinary Teaching Hospital (VTH)\nfor weight loss of 10 months’ duration and anemia of 3 weeks’ duration.\nAbnormalities identified by the primary veterinarian 3 weeks prior to admission\nincluded a thin body condition and a grade II/VI holosystolic heart murmur. Serum\nbiochemical profile and total thyroxine were within normal limits. Complete blood\ncount (CBC) revealed a normocytic, normochromic, regenerative anemia (hematocrit\n[HCT] 17.2%, reference interval [RI] 30.3–52.3; 66,500 reticulocytes/μl, RI\n3000–50,000) and thrombocytopenia (142,000 platelets/μl [RI 151,000–600,000]). A PCR\npanel testing for Mycoplasma haemofelis (MH),\nCandidatus Mycoplasma turicensis (CMt) and\nCandidatus Mycoplasma haemomintum (CMh) was submitted. The cat\nwas administered orbifloxacin 3.4 mg/kg by mouth once daily pending results of the\nPCR panel and oral vitamin B supplementation (unknown type and dose) in the\nmeantime.\nThe CBC 1 week later revealed improved normocytic and normochromic anemia and\nreticulocytosis (21% HCT and 94,600 reticulocytes/μl, respectively) and resolved\nthrombocytopenia (159,000 platelets/μl). The PCR panel was negative. The cat\ncontinued to receive orbifloxacin and vitamin B supplementation due to clinical\nimprovement.\nOver the following 2 weeks, the packed cell volume (PCV) ranged from 20.1–22.5% and\nreticulocyte count from 58,000–80,600 reticulocytes/μl. The cat also tested positive\nfor feline immunodeficiency virus (FIV) and negative for feline leukemia virus\n(FeLV) on a lateral flow ELISA test kit (SNAP FIV/FeLV Combo) during this period of\ntime. The cat was vaccinated for FIV 7 years prior to testing. One year prior to\nvaccination, the cat had tested negative for FIV through unknown diagnostic\nmethods.\nThe cat presented to the VTH 1 week later. Abnormalities on physical examination\nconsisted of a body condition score of 4/9 and a grade II/VI systolic parasternal\nheart murmur. CBC revealed a normocytic, normochromic, mildly regenerative moderate\nanemia (19.5% [RI 33.7–47.5%]; 83,500 reticulocytes/μl [RI 13,100–71,600\nreticulocytes/μl]), marked thrombocytopenia (34,000 platelets/μl with platelet\nclumping; RI 149,000–532,000 platelets/μl), a neutrophilic left shift (185 bands/μl;\nRI 0–0 bands/μl) and lymphopenia (739 lymphocytes/μl; RI 804–9240 lymphocytes/μl)\nwith reactive lymphocytes. Mild cardiomegaly was present on thoracic radiographs.\nAbdominal ultrasound identified mild splenomegaly and hypoechoic splenic parenchyma.\nFine-needle aspirate cytology of the spleen demonstrated moderate macrophagic\ninfiltration with phagocytosis of erythrocytes and erythroid progenitors and\nextramedullary hemato poiesis. Cytological evaluation of a bone marrow aspirate\nshowed similar findings with moderately increased macrophages and phagocytosis of\nerythroid progenitors and erythrocytes. Erythroid hyperplasia was also noted. Rare\nphagocytosis of myeloid progenitors and megakaryocytic hyperplasia was also noted.\nThe macrophages were similar in appearance to those observed in the spleen. The\nmacrophages did not exceed 20% of the population. A bone marrow core biopsy was also\ncollected, but a definitive diagnosis could not be obtained with the sample owing to\nlow cellularity. Primary differentials at this point were hemophagocytic syndrome\nand hemophagocytic histiocytic sarcoma.\nIn 2 weeks, the cat’s heart murmur progressed to a grade IV/VI parasternal systolic\nmurmur. Splenomegaly was now identifiable on abdominal palpation. CBC revealed\nprogression of anemia (15.6% HCT; 46,200 reticulocytes/μl), leukopenia (3500 white\nblood cells [WBCs]/μl; RI 4250–14,610 WBCs/μl) characterized by neutropenia (2065\nneutrophils/μl; RI 2272–9639 neutrophils/μl) and a left shift (105 bands/μl) and\nmild thrombocytopenia (90,000 platelets/μl with manual estimate of 130,000\nplatelets/μl). Echocardiogram identified moderate, irregular, left ventricular\nhypertrophy with left atrial enlargement and left ventricular chamber enlargement,\nright ventricular chamber enlargement and right atrial enlargement. Small-volume\npericardial effusion with pericardial thickening and pleural effusion were also\nnoted. Primary differentials for these findings included either infiltrative disease\nof the heart secondary to infection, inflammatory disease or neoplasia, hypertension, or hypertrophic cardiomyopathy. Chronic anemia may also have played a role in the cat’s cardiomegaly as\nanemia has been associated with increased cardiac troponin 1 concentration, which is\nsuggestive of cardiac myocyte damage. Blood pressure was not evaluated. Thoracic radiographs revealed progression\nof cardiomegaly but no evidence of congestive heart failure. Tests for\nHistoplasma species antigen in urine and serum IgM and IgG to\nToxoplasma gondii were negative. Whole blood was tested using\nPCR for Anaplasma phagocytophilum, Bartonella henselae, Bartonella\nclarridgeiae, Bartonella quintana, Ehrlichia species, MH, CMh, CMt,\nRickettsia rickettsii and Rickettsia felis,\nand was negative. In order to further differentiate between hemophagocytic syndrome\nand an underlying neoplastic condition, splenectomy was recommended.\nApproximately 2 weeks later, splenectomy was performed without complication. The cat\nreceived buprenorphine 0.02 mg/kg IV q8–12h as needed for analgesia after surgery.\nLiver biopsies were collected at the time of surgery. Recheck echocardiogram prior\nto anesthesia revealed similar findings with progression of pericardial effusion and\nmild cardiac tamponade. Repeat thoracic radiographs did not reveal any evidence of\npleural effusion or pulmonary edema, making congestive heart failure unlikely. The\ncat had type A blood and was given 20 ml blood-type-compatible packed red blood\ncells prior to surgery, which resulted in an increase in PCV from 18% to 21.5%.\nPostoperative PCV was stable at 23.5%. The cat was discharged and returned 1 week\nlater for a recheck. The cat appeared more lethargic and the PCV had decreased to\n15%.\nHistopathology of the spleen revealed multifocal aggregates of mononuclear cells with\nmoderate amounts of eosinophilic cytoplasm, occasional variably sized\nintracytoplasmic vacuoles and distinct cell borders (). Occasionally, macrophages within\nthe aggregates were observed phagocytizing erythrocytes (). There were also large numbers of\nmegakaryocytes and both myeloid and erythroid precursors throughout the splenic\nparenchyma. Increased numbers of metarubricytes and rubricytes resulted in a myeloid\nto erythroid ratio of approximately 1:2. Multifocal regions of red pulp contained\ndistinct sinuses void of erythrocytes. The hemophagocytosis within the spleen,\npaired with the extramedullary hematopoiesis, was suggestive of hemophagocytic\nsyndrome. The macrophages did not exhibit characteristics of malignancy, which\nlowered the suspicion of hemophagocytic histiocytic sarcoma. The splenic\nhistopathology was reviewed by five anatomic pathologists, who agreed with a\ndiagnosis of non-neoplastic hemophagocytic histiocytosis. Histopathology of the\nliver was consistent with mild, multifocal extramedullary hematopoiesis. A diagnosis\nof hemophagocytic syndrome was made. PCR testing for FIV was performed on the\nsplenic tissue and was found to be negative.\nAfter splenectomy, there was no improvement in the anemia over the next 4 weeks, with\nPCV ranging from 15–17%. The cat was started on lomustine at 41.6 mg/ m2\norally every 3 weeks. The cat’s PCV fluctuated between 22% and 25% over the following 2 months.\nEvaluation with echocardiogram after the second lomustine treatment indicated stable\ncardiac disease. A grade 3 non-febrile neutropenia (864 neutrophils/μl) was\nencountered after the third treatment, so the treatment interval was extended to every 4 weeks. Lomustine was\notherwise well tolerated.\nThree months after initiating lomustine, disease progression characterized by\nthrombocytopenia, worsening anemia, ascites, pleural effusion, severe left atrial\nenlargement and static pericardial effusion was noted. There was concern that the\ncat may be in congestive heart failure owing to the presence of severe left atrial\nenlargement, pleural effusion and ascites. Abdominal fluid analysis was consistent\nwith a protein-poor transudate. The cat was treated with vincristine 0.5\nmg/m2 IV once and furosemide 1.5 mg/kg PO q12h. Over the following 6\ndays, a 25% reduction in body weight was noted, attributed to fluid loss and muscle\nwasting. The thrombocytopenia improved but the cat was considered too debilitated to\nreceive any further chemotherapy. Owing to poor response to treatment, the cat was\neuthanized and no necropsy was performed.
|
[[12.0, 'year']]
|
M
|
{'19578295': 1, '24792320': 1, '23278382': 1, '16937360': 1, '21828139': 1, '17331054': 1, '20559036': 1, '8965270': 1, '2808102': 1, '24645736': 1, '18045564': 1, '15922227': 1, '29730195': 1, '17338159': 1, '12239144': 1, '20582898': 1, '17215570': 1, '18366555': 1, '23953723': 1, '28530307': 1, '30181894': 2}
|
{}
|
2,242 |
6113765-1
| 29,701,015 |
comm/PMC006xxxxxx/PMC6113765.xml
|
A case series: Association of anaphylaxis with a significant decrease in platelet levels and possible secondary risk of thrombosis
|
The patient is a 49 year old man with psoriasis and idiopathic anaphylaxis—generalized frequent type who developed an upper extremity deep vein thrombosis (DVT) after recurrent and protracted episodes of anaphylaxis without clinical signs of shock. The patient denied a family or personal history of coagulation disorders or unprovoked blood clots.\nThe events leading to his DVT started weeks prior when he experienced urticaria and angioedema of his face, body, and extremities without association of additional systemic symptoms. The urticaria and angioedema was initially responsive to steroids and antihistamine treatment, but within weeks became refractory and progressive. A subsequent sudden episode of respiratory distress with hypoxia resulted in an emergent crico-thyroidotomy.\nDuring his hospitalization subject 2 had recurrent anaphylaxis. Multiple physicians reported bilateral wheezing and poor air exchange during respiratory distress episodes. The events were unresponsive to tracheotomy hygiene and albuterol, but resolved after IM epinephrine. One incident required an epinephrine drip for 12 h. Within 24 h of an anaphylaxis episode he developed an acute occlusive left upper extremity DVT. He has had a total of four hospital admission within 1 year for anaphylaxis. During his third admission he developed an acute worsening of his chronic left upper arm DVT. With each anaphylactic attack a drop in his platelet levels were seen (Table ).\nHis work-up for a trigger of anaphylaxis has been negative to date. His tryptase levels at baseline, during protracted anaphylaxis, and an hour after anaphylaxis have never been above 9. All histamine and histamine metabolites have also been negative to date. Investigations into malignancy, mast cell disorders, autoimmune diseases have been negative to date with the exception of his psoriasis.
|
[[49.0, 'year']]
|
M
|
{'26945498': 1, '31802955': 1, '12004251': 1, '6776143': 1, '24466205': 1, '915295': 1, '26505932': 1, '18974586': 1, '14633796': 1, '17493503': 1, '33943038': 1, '20167241': 1, '31849989': 1, '26123418': 1, '21436837': 1, '11476463': 1, '7540500': 1, '22702510': 1, '26716499': 1, '26051949': 1, '15786828': 1, '34733000': 1, '21216082': 1, '8885805': 1, '8219198': 1, '18172172': 1, '28973181': 1, '29701015': 2}
|
{'6113765-2': 2}
|
2,243 |
6113765-2
| 29,701,015 |
comm/PMC006xxxxxx/PMC6113765.xml
|
A case series: Association of anaphylaxis with a significant decrease in platelet levels and possible secondary risk of thrombosis
|
An 18 years old female undergoing chemotherapy for acute myeloid leukemia (AML) was found to be allergic to her pentamidine treatments. She developed nonocclusive thrombus involving the celiac trunk, proximal hepatic, and splenic arteries shorty after her first pentamidine administration. She originally started intravenous (IV) pentamidine after developing a nonspecific rash (without mucosal involvement) initially attributed to trimethoprim/sulfamethoxazole.\nTwenty minutes into her first administration of IV pentamidine she developed severe crippling abdominal pain, emesis, diarrhea, and light headiness followed by near-syncope. Abdominal X-ray showed non-obstructive gas patterns with small bowel wall thickening. After resolution of her abdominal pain she was given a platelet transfusion for thrombocytopenia (Table ). During the platelet infusion, she developed intense abdominal pain, hypotension, and poor perfusion. Treated with intramuscular epinephrine yielded a positive response.\nAn abdominal CT scan showed colonic wall thickening with surrounding inflammatory changes and concern for ischemic colitis. An MR enterography revealed a nonocclusive thrombus involving the celiac trunk, proximal hepatic, and splenic arteries. The patient underwent a partial colectomy from the mid transverse colon to mid distal colon. The pathology results showed patchy transmural ischemic colitis without evidence of local thrombosis or emboli. Continued treatments of IV pentamidine every 2 weeks were noted to have immediate production of a range of symptoms mostly commonly: abdominal pain, emesis, pruritus (originally urticaria was rarely noted), diarrhea and headaches. Pentamidine allergy was established and desensitization protocol was subsequently used. Isolated urticarial flares with each desensitization was reported without any other clinical systemic involvement.\nCombined using a paired two tailed Wilcoxon test on the Pre- and Post-anaphylactic platelets levels of all 3 case's showed the overall P < 0.0001(Figure ). A Spearman test on the pairing had a rs value of 0.9344 with a P < 0.0001.\nFor Case 3, her platelet count post BMT remained in the low 30–50's. Laboratory analysis of known specific antibodies to platelets were negative. With spacing out her pentamidine treatments to every 4 weeks her counts marginally improved to the 40–60's. Once off pentamidine, trimethoprim/sulfamethoxazole (TMP-SMX) was restarted. Her thrombocytopenia again incrementally improved to the 80–100's. A Kruskal-Wallis test on her platelet levels with respect to the frequency of her pentamidine infusions and off pentamidine showed a P < 0.0001 (Figure ).
|
[[18.0, 'year']]
|
F
|
{'26945498': 1, '31802955': 1, '12004251': 1, '6776143': 1, '24466205': 1, '915295': 1, '26505932': 1, '18974586': 1, '14633796': 1, '17493503': 1, '33943038': 1, '20167241': 1, '31849989': 1, '26123418': 1, '21436837': 1, '11476463': 1, '7540500': 1, '22702510': 1, '26716499': 1, '26051949': 1, '15786828': 1, '34733000': 1, '21216082': 1, '8885805': 1, '8219198': 1, '18172172': 1, '28973181': 1, '29701015': 2}
|
{'6113765-1': 2}
|
2,244 |
6113981-1
| 30,174,718 |
comm/PMC006xxxxxx/PMC6113981.xml
|
Two cases of white globe appearance in non-cancerous stomach
|
An 82-year-old Japanese man underwent esophagogastroduodenoscopy for a routine health checkup. The patient had been taking vonoprazan, dimethicone, acotiamide, sitagliptin, candesartan, dutasteride, etizolam and zolpidem for reflux esophagitis, functional dyspepsia, diabetes, hypertension, benign prostatic hyperplasia and insomnia. Physical examination revealed no abnormalities in his abdomen. All laboratory findings were within the normal ranges, except for elevation of plasma glucose (256 mg/dL), haemoglobin A1c (7.1%) and gastrin levels (844 pg/mL, normal range: 42–200 pg/mL). He tested negative for Helicobacter pylori (H. pylori) IgG antibody.\nEndoscopy revealed multiple white spots in the fornix () and body () of the stomach. Magnifying endoscopy observation () and blue laser imaging () showed a slightly elevated, round, white substance. Microvasculature was also seen on its surface, suggesting deposition of the white substance within the mucosa. Atrophic gastritis was also observed during esophagogastroduodenoscopy. No inflammation was observed in the oesophageal mucosa. Biopsy from the gastric mucosa that contained white spots revealed cystic dilation of the gastric fundal gland with a 400 μm diameter (, ). Parietal cell protrusion was also noted (, arrows). Xanthoma cells were absent in the biopsied specimen.
|
[[82.0, 'year']]
|
M
|
{'33250690': 2, '33025345': 1, '1553828': 1, '26227666': 1, '27803398': 1, '30510814': 2, '33993429': 1, '27915451': 1, '26135651': 1, '11436167': 1, '10872661': 1, '30174718': 2}
|
{'6113981-2': 2, '6232817-1': 1, '6232817-2': 1, '7670350-1': 1}
|
2,245 |
6113981-2
| 30,174,718 |
comm/PMC006xxxxxx/PMC6113981.xml
|
Two cases of white globe appearance in non-cancerous stomach
|
A 74-year-old Japanese woman underwent esophagogastroduodenoscopy for investigation of epigastric pain and throat discomfort. She had been taking esomeprazole, rebamipide, sitagliptin, candesartan, ezetimibe, mirabegron, levocetirizine, zolpidem and lactobacillus preparation for reflux esophagitis, diabetes, hypertension, hyperlipidaemia, urticaria and insomnia. The patient had also been using flurbiprofen poultice for chronic lumbar pain. She is allergic to multiple medications, including antibiotics. Although the patient underwent eradication treatment for H. pylori 2 years previously, she discontinued taking the medication due to epigastric discomfort, and eradication failed. Physical examination revealed no abnormalities in her abdomen. Laboratory findings revealed elevated levels of total cholesterol (254 mg/dL), triglyceride (130 mg/dL), haemoglobin A1c (7.7%) and immunoglobulin E (598 IU/mL, normal range: 0–170 IU/mL). Gastrin levels were not measured in this patient. H. pylori IgG antibody was positive.\nEsophagogastroduodenoscopy revealed multiple white spots in the gastric fornix (, arrows), body (, post-indigo carmine spraying) and antrum. Magnifying endoscopy observation () and blue laser imaging () showed small, round, white deposits that were similar to the substances observed in case 1. Other endoscopic findings included atrophic gastritis and oesophageal hiatal hernia. Cystic dilation of the gastric fundal gland was identified in the biopsied specimen obtained from the white spots, which contained debris in the dilated duct (, ). The dilated duct was approximately 600 μm in diameter. In addition, parietal cell protrusions and dilated glands forming microcysts were present ().
|
[[74.0, 'year']]
|
F
|
{'33250690': 2, '33025345': 1, '1553828': 1, '26227666': 1, '27803398': 1, '30510814': 2, '33993429': 1, '27915451': 1, '26135651': 1, '11436167': 1, '10872661': 1, '30174718': 2}
|
{'6113981-1': 2, '6232817-1': 1, '6232817-2': 1, '7670350-1': 1}
|
2,246 |
6113983-1
| 30,174,722 |
comm/PMC006xxxxxx/PMC6113983.xml
|
A cancer-mimicking diagnosis of peritoneal carcinosis: report of a case of abdominal non-tuberculous mycobacterial infection
|
A 53-year-old Caucasian woman was referred to our institution because of the suspicion of peritoneal carcinomatosis, raised by the findings of ascites at a transvaginal ultrasound performed as a yearly routine exam; a pre-surgical staging exam with computed tomography (CT) scan show thickening of the gastric walls, multiple omental nodules and ascites ().\nHer previous personal history was unremarkable and she denied any clinical symptom or cancer history.\nAt our hospital, she underwent an esophagogastroduodenoscopy and colonoscopy, with results negative for gastric/colon cancer.\nHer comprehensive metabolic profile revealed mild liver dysfunction with an alanine transaminase of 77 U/L and aspartate transaminase of 71 U/L. When tumour markers were assessed, CA125 demonstrated increased levels of 290 U/mL (normal values <35 U/mL), whereas carcinoembryonic antigen, CA 19.9 and other immunohistochemical markers were within the normal ranges. Serological assessment of HIV, hepatitis C virus and hepatitis B virus were negative.\nTen days later, the patient underwent an ultrasound-guided biopsy () with a diagnosis of suspicious carcinoma from the an unknown primary site.\nAfter 2 weeks, the patient received a CT scan of the thorax (to complete pre-operative staging), demonstrating a spontaneous (with no therapy) dimensional and numerical reduction of peritoneal lesions in the upper abdomen, partially included in the chest CT scan, as well as resolution of peri-hepatic and peri-splenic ascites ().\nSince there was no evidence of primary cancer at pre-operative examinations and the second CT scan revealed a partial resolution of peritoneal implants and ascites without therapy, the suspicion of an infectious disease was raised.\nThe pathological evaluation of the biopsies performed on the omentum and peritoneum revealed the presence of lymphoid aggregates with a central core of epithelioid cells with large eosinophilic cytoplasm, without atypia or mitosis () and with no immunohistochemical marker of oncologic malignancy. Due to the presence of necrotic nodules with histiocytes and giant cells, a Ziehl–Neelsen stain was performed to identify bacilli (), whose presence was then confirmed with molecular assays.\nBecause of the uncertain result of the biopsy and the conflicting results of the 2 CT scans, in order to rule out malignancy with certainty, the patient underwent a laparoscopic surgery in 2 weeks.\nDefinitive histological diagnosis excluded the presence of malignant cells and reported a necrotising inflammation caused by non-tuberculous mycobacteria. Hence, the patient was sent to a hospital with expertise in infectious diseases.\nA follow-up CT scan performed 1 year later confirmed a complete recovery of peritoneal findings.
|
[[53.0, 'year']]
|
F
|
{'24631039': 1, '12625084': 1, '23460008': 1, '15534923': 1, '15520483': 1, '16183939': 1, '22131651': 1, '16040924': 1, '12640160': 1, '11346208': 1, '9683690': 1, '20392359': 1, '24624285': 1, '25426446': 1, '20452173': 1, '30174722': 2}
|
{}
|
2,247 |
6113986-1
| 30,174,723 |
comm/PMC006xxxxxx/PMC6113986.xml
|
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases
|
A 4-year-old male Caucasian patient with no prior family history of neoplasms presented in fair general condition with weight loss and loss of appetite, which, according to his parents, had developed over 2 months. Clinical evaluation showed evidence of abdominal pain and distention in addition to hepatomegaly. A full work-up was requested, significant findings were anaemia, very high levels of α-fetoprotein (352,050 ng/mL, normal range: <20 ng/mL), low levels of chorionic gonadotropin subunit beta (1.9 mIU/mL) and elevated levels of lactate dehydrogenase (1,615 IU/L). Tests on the levels of catecholamines in the blood and urine were negative. An abdominal ultrasound was performed, which showed irregular hepatomegaly with a heterogeneous echotexture due to the presence of multiple, randomly distributed echogenic nodules, suggestive of metastasis. The pancreas could not be evaluated. This was followed by an abdominal and pelvic CT scan with and without contrast dye: this showed a heterogeneous tumour in the body and tail of the pancreas that had defined borders, hypodense areas inside (suggestive of necrosis) and an apparent pseudocapsule. This measured: anteroposterior diameter 75 × transverse diameter 57 × longitude 101 mm, displacing the left kidney and the splenic artery in the head and rear directions, the left ureter in the dorsal direction and the bowel in the caudal direction. Enlarged heterogeneous liver, due to the presence of multiple hypodense nodules. The full body bone scan pathology did not show focal uptake. The adrenal glands showed no significant changes.\nThe patient’s condition deteriorated: he presented an acute abdomen requiring surgery, for which an exploratory laparotomy was performed. This revealed a hepatic nodular lesion in the left lobe, with surface erosion and bleeding, from which biopsies were taken. The histopathological study with Haematoxylin–Eosin (H & E) staining showed limited liver trabeculae, infiltrated by a poorly differentiated malignant neoplastic proliferation consisting of medium-sized round or polygonal cells with large cytoplasma, with areas of necrosis and haemorrhage. Immunohistochemistry procedures were run, revealing neoplastic cells: positive for vimentin, Cytokeratin (AE1–AE3), β-catenin, Ki-67 (in 80% of the neoplastic nuclei) and, focally, carcinoembryonic antigen. In contrast, the tests were negative for neuron-specific enolase, Chromogranin and Hep Par-1. The earlier findings, together with the patient’s clinical context, suggested liver metastasis due to pancreatoblastoma.\nThe patient went into intensive care and, upon returning to the ward, started a course of chemotherapy, using cisplatin 56 mg/m2 and doxorubicin 21 mg/m2.\nFollowing neoadjuvant treatment, the patient underwent schedule surgery for excision of a pancreatic tumour. The surgical approach was through the lesser sac, locating it in the tail and body of the pancreas. We proceeded to the lower margin, releasing the relevant vessels, to the posterior for unaffected pancreatic tissue and, finally, to the upper margin respecting the splenic vessels. A circumscribed, nodular lesion of 7 × 6 cm and weighing 150 g was obtained in the aforementioned procedure (pancreatectomy of the body and tail). The site of incision had a yellowish surface with solid areas and a friable central section. Histological sections showed the presence of a pancreatoblastoma, with large areas of necrosis and two residual peripheral nodules. Additional immunohistochemical techniques demonstrated the membrane positivity for E-cadherin and vascular endothelial growth factor (VEGF).\nAt present, the patient is receiving medical follow-up and is in a liver transplant programme.
|
[[4.0, 'year']]
|
M
|
{'22584784': 1, '29235221': 1, '24212642': 1, '15632705': 1, '20057322': 1, '10671596': 1, '18090935': 1, '21696948': 1, '27926361': 1, '188539': 1, '17228135': 1, '16844942': 1, '17198189': 1, '21768235': 1, '17959228': 1, '16633003': 1, '22525406': 1, '7503360': 1, '28693851': 1, '5549499': 1, '15256806': 1, '30174723': 2}
|
{}
|
2,248 |
6114031-1
| 30,157,782 |
comm/PMC006xxxxxx/PMC6114031.xml
|
Multimodal computed tomography-guided intravenous rtPA for aborted stroke in a HIV-infected young man: a case report
|
A 19-year-old male presented to the emergency department at 10:42 a.m.,42 min after the sudden onset of slurred speech with weakness of his right upper and lower extremities. He reported no headache, dizziness, nausea, vomiting, fever, or convulsions. He denied any significant medical history, drug abuse, or high-risk sexual behaviors. He had no history of migraines, trauma, insect bites, exposure to chemicals, or use of medications. Apart from cigarette smoking for 1 year, he denied other risk factors for stroke. There was no history of early cardiovascular disease in the family.\nOn physical examination, the patient’s vital signs were normal. His weight was 65 kg (69 kg, 3 weeks ago), body mass index (BMI) 21.47 kg/m2. His chest examination was clear, and no additional murmurs were detected upon cardiac examinations. The liver, spleen and cervical lymph nodes were not enlarged; no skin or mucosal lesions were seen.He was alert and oriented to person, place, and time. The pupils were equal and reactive to light and accommodation. He had mild right hemiplegia with strength of 4:5 in the right upper and lower extremities; slight dysarthria and right lower facial paresis were also noted. The neurologic examination was otherwise unremarkable. The National Institutes of Health Stroke Scale (NIHSS) score was 3.\nRapid blood glucose was in the normal range (6.3 mmol/L). Complete blood count results showed white blood cell (WBC) count 3.0 × 109/L, hemoglobin 11.9 g/dL, and platelets 273 × 1012/L. Stroke was first considered. As he was then in the 4.5-h time window for IV-rtPA, an urgent brain CT with computed tomography angiography (CTA) of intra–extracranial vessels and whole-brain computed tomography perfusion (CTP) imaging were performed in the emergency department. The nonenhanced CT (NECT) scan (Fig. ) and CTA (Fig. ) were normal. CTP showed hypoperfusion in the left hemisphere with prolonged mean transit time (MTT) and time-to-peak (TTP), slightly increased cerebral blood volume (CBV), and relatively preserved cerebral blood flow (CBF) (Fig. -). A diagnosis of acute ischemic stroke was made. After excluding absolute contraindications for intravenous thrombolysis, 58.5 mg (0.9 mg/kg) rtPA was given at 12:30 a.m., immediately after the patient signed the informed consent. After 1 h of rtPA, his symptoms were alleviated, and the NIHSS score was decreased to 1, with slight asymmetry of the nasolabial sulcus. The patient was then admitted to the department of neurology for further investigations and treatment. Within 60 min after admission, the clinical neurological examination had completely normalized.\nOn the next day, the patient had no complaints of discomfort. The NIHSS score was 0. Cerebral magnetic resonance imaging (MRI) was performed, and no acute lesion was seen in the diffusion-weighted image (DWI) sequences (Fig. ). Doppler ultrasonography of carotidal and intracranial arteries showed no abnormality. Electrocardiogram, electroencephalogram (EEG) and chest CT scan were all normal. Echocardiography showed that the left and right heart chambers were within normal size and function(left ventricle ejection fraction 70%). His laboratory tests showed WBC count 2.2 × 109/L, hemoglobin 10.4 g/dL, platelets 210 × 1012/L, ESR 18 mm/h (normal 0–15.0), CRP 0.66 mg/L (normal 0–8.0), homocystein 10.4 umol/L (normal < 15.0), and mild impairment of liver function (ALT 132 U/L (normal 5–40), AST 106 U/L (normal 8–40), GGT 257 U/L (normal 11–50), ALP 127 U/L (normal 40–150)). Serologies showed the following: rapid plasma reagin, negative; antinuclear antibody and antineutrophil cytoplasmic antibody, negative; complement level including C3, C4 levels, normal; anticardiolipin immunoglobulin G(ACL-IgG), ACL-IgM, and ACL-IgA antibody levels, normal; β2 glycoprotein I immunoglobulin G (β2-GP1- IgG) and β2-GP1- IgM antibody levels, normal; high β2-GP I-IgA antibody level, 67.16 units/mL (normal 0–18). HIV enzyme-linked immunosorbent assay and Western blot confirmed HIV infection with CD4 cell count of 7 cells/uL; the CD4:CD8 T cell ratio was 0.04 (7/173). Hepatitis B virus surface antigen and hepatitis C antibody were unremarkable.\nOn the third day of admission, the patient received a lumbar puncture with pressure of 140 mmH2O. Cerebrospinal fluid (CSF) studies showed normal-range white cell and red cell counts but a high protein level at 1185 mg/L. The CSF glucose and chloride were in the normal range. CSF viral PCRs (including herpes simplex virus, varicella zoster, Epstein Barr, cytomegalovirus and JC virus), cryptococcal antigen, and bacterial and fungal cultures were all negative. CSF syphilis TRUST and TPPA tests were also negative. There was no meninges or brain parenchymal enhancement on his brain contrast-enhanced MRI (Fig. ).\nThe patient was diagnosed with aborted stroke and HIV infection. Oral aspirin 100 mg and atorvastatin calcium 20 mg daily were given. For further treatment, the patient was transferred to the HIV/AIDS ward on the fourth day of admission. On follow up 2 months later, he reported no similar symptoms.
|
[[19.0, 'year']]
|
M
|
{'25280902': 1, '15827890': 1, '22995692': 1, '15469799': 1, '12740462': 1, '29273597': 1, '19893313': 1, '26219650': 1, '7994900': 1, '29604976': 1, '28727732': 1, '28493892': 1, '23983008': 1, '23946297': 1, '2110431': 1, '14595478': 1, '22554568': 1, '17138253': 1, '16514093': 1, '25150187': 1, '11296952': 1, '30157782': 2}
|
{}
|
2,249 |
6114032-1
| 30,181,930 |
comm/PMC006xxxxxx/PMC6114032.xml
|
Metastatic squamous cell carcinoma of the skin with clinical response to lapatinib
|
A man in his 80s presented with 2 years of recurrent cutaneous squamous cell carcinoma of the left temple (Fig. ) with zygomatic bone metastasis. He also had significant unilateral hearing loss secondary to perineural involvement. The 2 years of therapy preceding evaluation in our oncodermatology clinic is described below. In addition to Mohs micrographic surgery, the patient had also received two rounds of adjuvant radiotherapy. In the first round of radiotherapy, the patient received a total dose of 5000 cGy in 25 fractions delivered with 3D conformation irradiation to the tumor bed and facial nodal basins. Eight months later, a bony metastasis of the mandible led to another 5000 cGy dose, which was delivered in 25 fractions using intensity-modulated irradiation tracking along the V2 branch of the trigeminal nerve to the ipsilateral skull base and encompassing the cavernous sinus. Yet another bony metastasis was discovered 5 months later, at which time he consented to 5 cycles of off-label, palliative, compassionate-use nivolumab monotherapy. However, following 2 months of nivolumab treatment, repeat MRI showed continued tumor progression. At this time he presented to our clinic complaining of a 3 week history of a rapidly enlarging painful nodule over his left zygoma. Tumor genomic analysis of the nodule using next-generation sequencing (FoundationOne®, Cambridge, MA) revealed a somatic missense (R135C) mutation in the ERBB3/HER3 gene, as well as multiple other mutations (Table ) and a high tumor mutation burden (75 mutations per megabase).\nIn an effort to target the ERBB3/HER3 mutation, therapy with 1,250 mg of lapatinib daily in combination with 240 mg nivolumab every 2 weeks was initiated. Additional tumor debulking in conjunction with cryotherapy to the base of the lesion was performed by our Mohs surgeon. Significant improvement in the clinical size of the lesion was noted after 2 months of lapatinib therapy. After 6 months, there was continued clinical improvement (Fig. ) and MRI showed significant regression of muscle, nerve, and bone involvement (Fig. ). The patient experienced a significant decrease in narcotic pain medication dependence and improvement in hearing of the ipsilateral ear. Other than fatigue, he experienced no side effects from this therapy.
|
[[85.0, 'year']]
|
M
|
{'26194695': 1, '21406472': 1, '16953240': 1, '23680147': 1, '16247014': 1, '11166154': 1, '25531942': 1, '7538656': 1, '34877077': 1, '1607418': 1, '8665853': 1, '30181930': 2}
|
{}
|
2,250 |
6114043-1
| 30,153,836 |
comm/PMC006xxxxxx/PMC6114043.xml
|
Cytopathological findings of proliferating pilomatricoma misdiagnosed as a malignant parotid gland tumor
|
A 64-year-old man noticed an acne-like nodule in the left parotid region 2 years prior to this presentation. It was painless, but it increased up to a maximum diameter of 4.5 cm. Clinically, left parotid gland carcinoma was suspected, and FNA cytology was performed from the left parotid region. Clusters of epithelial cells were observed in a necrotic and hemorrhagic background. These cell clusters had a sheet-like arrangement and high nuclear-cytoplasmic ratio. The nuclear shape was ovoid with hyperchromasia. Neither nuclear membrane thickening nor irregular-shaped nuclei were noted. One obvious nucleolus was observed in the central portion of the cytoplasm (Fig. ). Small lymphocytes, histiocytes and multinucleated giant cells were also seen. Malignant epithelial cells derived from salivary glands, including squamous cell carcinoma, myoepithelial carcinoma and carcinoma ex pleomorphic adenoma, were suspected. Magnetic resonance imaging (MRI) revealed a well-defined multilocular tumor located close to the outside of the left parotid gland. On T1- and T2-weighted imaging, low-intensity and heterogeneous gadolinium enhancement was seen (Fig. ). Radiologically, parotid gland cancer was suspected.\nOne month later, tumor resection of the left parotid region and superficial parotidectomy were performed. The cut surface showed a well-defined lobulated tumor containing yellowish-muddy materials (Fig. ). Histologically, the resected tumor was diagnosed as proliferating pilomatricoma composed of basophilic cells and shadow cells apart from the left parotid gland. The tumor was encapsulated by fibrous tissue without stromal invasion. Approximately 60% of the tumor cells consisted of shadow cells, and basophilic cells were confirmed at the periphery of the tumor. The basophilic cells were oval-shaped with a high nuclear cytoplasm ratio and had an obvious nucleolus. Two mitoses were observed per high-powered field. Focal squamous metaplasia, coagulative necrosis and apoptotic cells were also observed. Eosinophilic-stained shadow cells showed nuclear concentration and disappearance. Transitional histological findings were identified between basophilic cells and shadow cells, and supramatrical cells characterized by incomplete nuclear disappearance were also seen (Fig. ).\nImmunohistochemically, basophilic cells and shadow cells were negative for anti-pan cytokeratin antibody (AE1/AE3, diluted 1:800; Leica) and high-molecular-weight keratin (34βE12, diluted 1:200; DAKO), but squamous metaplastic cells were positive. β-catenin (3-caten, diluted 1:400; DAKO) was positive for basophilic cells with nuclear and cytoplasmic staining. Ki-67 (MIB-1, diluted 1:30; Biogenex) labeling index for basophilic cells and shadow cells were 46.2% and 0%, respectively, and the p53 (Bp53–12, diluted 1:200; IBL) labeling index were 94.8% and 0%, respectively. S-100 protein (2A10, diluted 1:400; IBL), HMB-45 (HMB-45, diluted 1:200; DACO) and Ber-EP4 (Ber-EP4, diluted 1:400; DAKO) were negative for basophilic cells and shadow cells. Fibrosis, calcification, foreign body granulomatous reaction, foamy macrophage aggregation, and lymphocyte infiltration were observed in the tumor stroma. The tumor was completely resected. There was no metastasis to the lymph nodes around the parotid gland.\nOn a re-evaluation of the cytological specimens, the ovoid-shaped epithelial cells were considered to be basophilic cells. Shadow cells with nuclear disappearance were also confirmed. Keratin fibers were found in the cytoplasm of the shadow cells (Fig. ). Ultimately, we concluded that these cytological findings were consistent with pilomatricoma.
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[[64.0, 'year']]
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M
|
{'26991211': 1, '17718128': 1, '9138114': 1, '8879294': 1, '15185259': 1, '25478356': 1, '17635163': 1, '30153836': 2}
|
{}
|
2,251 |
6114064-1
| 30,186,652 |
comm/PMC006xxxxxx/PMC6114064.xml
|
Sextuple Tumors in Head and Neck Area: Evidence of Field Cancerization
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A 63-year-old Caucasian male patient sought an otorhinolaryngology treatment in São José do Rio Preto, Brazil, complaining of continuous hoarseness. Patient reported being smoker and alcoholic for 40 years and having stopped smoking for five years and denied any systemic or local diseases. Clinical examination showed lesions in the piriform sinus during nasofibroscopy. Lesions were biopsied and the pathological diagnosis was moderately differentiated and invasive squamous cell carcinoma (SCC). Within a month, a laryngectomy was performed with a selective right cervical dissection followed by radiotherapy.\nAt the first routine monitoring, 12 months later, patient presented a vegetating lesion on soft palate, diagnosed as moderately differentiated, and invasive SCC, a second neoplasm. The excision of the soft palate and complementary radiotherapy were performed. Twenty-four months after the first diagnostic, patient presented erythematous lesions on the soft palate and left tonsillar pillar, both identified as moderately differentiated SCC, third and fourth malignant tumors. Thirty-six months after the first diagnostic, patient had an ulcerative-infiltrative lesion in right tonsillar pillar, diagnosed as SCC, which is the fifth malignant tumor. Forty-six months after the diagnostic, the first malignancy the patient developed symptomatic lesions in base of tongue was diagnosed as nonspecific chronic glossitis. The tissue adjacent to the lesion was evaluated with immunohistochemical staining for p53 () with some focal areas in the basal and suprabasal layer with weak nuclear staining and Ki-67 () with the positivity of basal and suprabasal layer.\nA month later, another surgery was executed to remove a lesion located in the uvula, also diagnosed as moderately differentiated SCC, being the sixth malignancy. Likewise, immunohistochemical investigation was performed for p53 and Ki-67, contiguous to the lesion of the uvula and all lesions that preceded it. The tissue adjacent to the uvula expressed diffuse immunoreactivity for p53 () and Ki-67 () with strong nuclear staining in the basal and suprabasal layer. None of the neoplasms was accompanied by lymph node metastases. At the patient's return, 60 months after the first diagnostic, there was no evidence of other malignancies.
|
[[63.0, 'year']]
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M
|
{'22842165': 1, '24959211': 1, '25920765': 1, '13094644': 1, '12584590': 1, '25674170': 1, '28528739': 1, '2202902': 1, '25519014': 1, '23511666': 1, '25494606': 1, '25992232': 1, '25328294': 1, '25709366': 1, '29138574': 1, '26619122': 1, '21234721': 1, '9234095': 1, '30186652': 2}
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{}
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2,252 |
6114066-1
| 30,186,657 |
comm/PMC006xxxxxx/PMC6114066.xml
|
Laparoscopic Resection of Appendiceal Schwannoma
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A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase (). No abnormal findings were found in the root to the middle of the appendix. Abdominal ultrasonography (US) revealed a well-demarcated hypoechoic tumor. The tumor size was 22 mm × 18 mm × 18 mm, with some cystic area and blood flow (). Colonoscopy findings were normal. The patient's symptoms naturally alleviated during examination period.\nPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.\nPathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein (), which confirmed the schwannoma of the appendix. The patient was discharged on the 9th day after surgery without any complication requiring medical treatment. The patient is presently doing well without any evidence of recurrence at 3 months after surgery.
|
[[75.0, 'year']]
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M
|
{'15156378': 1, '26324037': 1, '16988762': 1, '12740466': 1, '17114072': 1, '3126126': 1, '25759634': 2, '20395722': 1, '11686568': 1, '20651976': 1, '28265705': 1, '20173452': 1, '22587439': 2, '22778564': 1, '19332945': 1, '29992004': 1, '28868377': 1, '21773906': 1, '21152234': 2, '25360169': 2, '9438854': 1, '11684962': 1, '30186657': 2}
|
{'4214396-1': 1, '2998005-1': 1, '4327572-1': 1, '3488541-1': 1}
|
2,253 |
6114068-1
| 30,186,656 |
comm/PMC006xxxxxx/PMC6114068.xml
|
Eltrombopag Improves Refractory Thrombocytopenia in a Patient with Systemic Lupus Erythematosus
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A 42-year-old Japanese woman was admitted to our hospital for evaluation of severe thrombocytopenia. At the age of 34, SLE was diagnosed on the basis of a malar rash, oral ulcers, arthritis, and positivity for anti-double-stranded- (ds-) DNA antibody. Prednisolone was started at a dose of 30 mg/day, after which tacrolimus was added at 3 mg daily. Her SLE improved, and prednisolone was tapered to 3 mg/day. One month before admission, proteinuria increased to 2.34 g daily. Serum albumin was 2.0 g/dL (normal: 3.9–5.2), and creatinine was 2.3 mg/dL (normal: 0.46–0.78). Anti-ds-DNA antibody showed an increase to 90 IU/mL (normal: <12). The platelet count was decreased to 4.4 × 104/μL, and CH50 was 5 U/mL (normal: >30 U/mL) (). Antiplatelet drugs or any other drugs that could adversely affect platelet numbers were not taken. Intravenous methylprednisolone pulse therapy (1000 mg/day for three days) was initiated, followed by methylprednisolone at 48 mg/day. She also received five monthly cycles of intravenous cyclophosphamide (IVCY) pulse therapy (500 mg per cycle). Although her renal function, complement levels, and the anti-ds-DNA antibody titer all improved, thrombocytopenia became worse and the patient was admitted to our hospital ().\nOn admission, she had diffuse purpura on the anterior chest and limbs. The lungs were clear on auscultation. The liver and spleen were not palpable, and there was no evidence of arthritis or pathological bleeding such as epistaxis. Pitting edema of both legs was noted.\nLaboratory findings were as follows (): the white blood cell count was 6,300/μL (86.5% neutrophils and 7.5% lymphocytes), hemoglobin was 8.7 g/dL, reticulocyte count was 9.3 × 104/μL (3.5%), and platelet count was 0.8 × 104/μL (10.5% immature platelets). The direct Coombs test was negative. Anemia secondary to chronic inflammation caused by SLE was considered. In addition, serum albumin was 3.1 g/dL, blood urea nitrogen was 33 mg/dL (normal: 8–21), creatinine was 1.2 mg/dL, and eGFR was 38.8 ml/min/1.73 m2. C-reactive protein was 0.0 mg/dL. Antinuclear antibody (ANA) was positive at 1:40 with a speckled pattern, while anti-ds-DNA antibody was elevated to 18 U/mL (normal: <12). The lupus anticoagulant assay was consistently negative, and anticardiolipin antibodies were not detected throughout the clinical course. ADAMTS13 activity was 87% (normal: >10%), and inhibitors were not detected. Total urinary protein excretion was 1.55 g/day. The urine sediment contained 6–10 erythrocytes per high-power field (HPF). Bone marrow examination showed normocellular marrow, and megakaryocyte number was within normal range with 12 (normal range: 10 to 50) number/mm2. There was no hepatosplenomegaly on computed tomography of the abdomen.
|
[[42.0, 'year']]
|
F
|
{'28423296': 1, '24740463': 1, '21325604': 1, '19324405': 1, '33606126': 1, '25416695': 1, '20823093': 1, '25179983': 1, '30186656': 2}
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{}
|
2,254 |
6114227-1
| 30,186,648 |
comm/PMC006xxxxxx/PMC6114227.xml
|
Capnocytophaga Induced Acute Necrotizing and Exudative Pericarditis with Abscess Formation
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A 55-year-old gentleman with a history of diabetes mellitus, hypertension, cocaine, and marijuana use presented to the emergency department (ED) with complains of chest pain and dyspnea for past 6–8 months, as well as lower extremity edema and weight loss. On admission to the hospital, his vital signs were stable. Physical examination revealed obesity, decreased breath sounds bilaterally, and mild tachycardia, and point of maximal impulse was enlarged and displaced at the presence of edema on bilateral lower extremities. The patient has poor dentition with cavity in the left second molar tooth. The rest of the examination was otherwise unremarkable. Complete blood count revealed a hematocrit of 33.1%, hemoglobin 9.7 g/dL, platelet count 232,000/mL, and white blood count 8.6 × 103. Blood chemistry was unremarkable. B-type natriuretic peptide was 613 pg/mL.\nBecause of his chest pain and associated signs, the patient underwent a computed tomography (CT) scan of the chest with intravenous contrast, which revealed a very large pericardial effusion, compressing the right and left ventricles and the right atrium (). In addition, there was consolidation in the left mid lung, bilateral pleural effusions, and bilateral pulmonary embolism. A 2D echocardiogram revealed cardiac tamponade with right ventricular diastolic collapse, with a large fibrinous exudative pericardial effusion (). The patient underwent an emergent pericardial window due to his clinical signs and symptoms consistent with cardiac tamponade. The pericardial drainage showed a significant amount of yellow creamy pus with thickened pericardium. Anaerobic culture reported the presence of Capnocytophaga species. The pathology specimen showed acute necrotizing and exudative changes including frank abscess formation with no specific organism detected and no evidence of malignancy (Figures and ).\nThe patient's condition improved postoperatively and was placed on piperacillin and tazobactam for four weeks. His pulmonary embolism and acute deep vein thrombosis were treated with systemic anticoagulation. The patient was discharged home on apixaban and has been seen on the follow-up visit with significant improvement in his symptoms.
|
[[55.0, 'year']]
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M
|
{'1600004': 1, '34659942': 2, '29181783': 1, '12622586': 1, '17205476': 1, '19307219': 1, '19352300': 1, '34062778': 1, '24695601': 1, '29703650': 1, '29390603': 1, '518235': 1, '25098798': 1, '18052017': 1, '20934560': 1, '30186648': 2}
|
{'8491562-1': 1}
|
2,255 |
6114237-1
| 30,186,649 |
comm/PMC006xxxxxx/PMC6114237.xml
|
Noninvasive Positive-Pressure Ventilation for Preeclampsia-Induced Pulmonary Edema: 3 Case Reports and a Literature Review
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A 21-year-old woman pregnant with twins at 29 weeks of gestation was admitted to the previous hospital for preterm labor. After one week of tocolysis with intravenous ritodrine, she developed acute dyspnea and was referred to our hospital. Ritodrine was stopped immediately, and computed tomography of the chest revealed no pulmonary embolus, but bilateral pleural effusion was present. On admission, she also presented with hypertension (152/112 mmHg) and proteinuria (3.8 g/day). She was diagnosed with severe preeclampsia, and magnesium sulfate was initiated, and betamethasone was administered for accelerating fetal lung maturation. After starting magnesium sulfate, her systolic blood pressure did not exceed 140 mmHg, and no further antihypertensive agent was necessary. On day 3 of admission, her SpO2 fell to 95% with 5 liters of supplemental oxygen, and NPPV was initiated. After implementation of NPPV, her subjective dyspnea improved, and her SpO2 rose to 99% on room air. Pulmonary edema was also ameliorated on her chest X-ray. However, her serum creatinine level was increased to 1.0 mg/dl at 33 weeks of gestation, indicating reduced kidney function. Other symptoms, such as increase in liver enzymes, platelet reduction, and gastrointestinal or neurological symptoms, were not detected. Fetal conditions in utero were favorable. She underwent a cesarean section at 33 weeks and 1 day of gestation due to initiation of labor. The patient delivered healthy male twin infants weighing 1496 g and 1876 g. NPPV was continued intermittently after delivery until she was successfully weaned off of it.
|
[[21.0, 'year']]
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F
|
{'15599160': 1, '20702082': 1, '33743806': 2, '17067334': 1, '24591671': 1, '28560149': 1, '28282780': 1, '26094569': 1, '21514554': 1, '21354656': 1, '26342729': 1, '23288967': 1, '14974057': 1, '23728654': 1, '28282767': 1, '3578433': 1, '27512467': 1, '23235608': 1, '24150027': 1, '22292815': 1, '27651980': 1, '19558746': 1, '30186649': 2}
|
{'6114237-2': 2, '6114237-3': 2, '7981838-1': 1}
|
2,256 |
6114237-2
| 30,186,649 |
comm/PMC006xxxxxx/PMC6114237.xml
|
Noninvasive Positive-Pressure Ventilation for Preeclampsia-Induced Pulmonary Edema: 3 Case Reports and a Literature Review
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A 36-year-old primigravida at 17 weeks and 4 days of gestation was admitted for hypertension (152/99 mmHg), proteinuria (1.8 g/day), and elevated liver enzymes (AST 75 U/L, ALT 121 U/L). Careful examination revealed no evidence of secondary hypertension or primary renal disease. Furthermore, serum levels of soluble fms-like tyrosine kinase 1 (sFlt1) were very high (8.41 ng/mL) at 18 weeks of gestation. Subsequently, we classified this case as extremely early onset preeclampsia []. Nifedipine and magnesium sulfate were administered. Ascites, pleural effusion, and pulmonary edema were detected at 19 weeks of gestation. NPPV was initiated for worsening pleural effusion at 20 weeks of gestation due to desaturation (94% SpO2 on room air). After NPPV implementation, the patient's SpO2 rose to 99% with 1 liter of supplemental oxygen. Chest X-ray showed no progression of pulmonary edema, although ascites gradually increased, resulting in an emergency cesarean section at 23 weeks and 3 days of gestation due to deteriorating dyspnea, and nonreassuring fetal status, specifically reversed end-diastolic umbilical artery flow and absence of atrial-flow in ductus venosus. A 285 g male infant was delivered. NPPV was discontinued on day 2 after delivery.
|
[[36.0, 'year']]
|
F
|
{'15599160': 1, '20702082': 1, '33743806': 2, '17067334': 1, '24591671': 1, '28560149': 1, '28282780': 1, '26094569': 1, '21514554': 1, '21354656': 1, '26342729': 1, '23288967': 1, '14974057': 1, '23728654': 1, '28282767': 1, '3578433': 1, '27512467': 1, '23235608': 1, '24150027': 1, '22292815': 1, '27651980': 1, '19558746': 1, '30186649': 2}
|
{'6114237-1': 2, '6114237-3': 2, '7981838-1': 1}
|
2,257 |
6114237-3
| 30,186,649 |
comm/PMC006xxxxxx/PMC6114237.xml
|
Noninvasive Positive-Pressure Ventilation for Preeclampsia-Induced Pulmonary Edema: 3 Case Reports and a Literature Review
|
A 40-year-old primigravida at 24 weeks of gestation was referred to our hospital for severe hypertension (170/95 mmHg) and proteinuria (8.8 g/day). On admission, she received magnesium sulfate, methyldopa, and nifedipine. On day 2 of admission, she developed respiratory distress with mild desaturation (95% SpO2 on room air), and chest X-ray showed bilateral pleural effusion. Blood exam revealed elevation of liver enzymes (AST 133 U/L, ALT 161 U/L), and partial HELLP syndrome was diagnosed. Corticosteroids were administered intravenously, and NPPV was initiated. The patient's SpO2 rose to 99%, and pleural effusion did not increase further. However, ascites gradually increased, and her general fatigue became intolerable. As a result, a cesarean section was performed at 25 weeks and 2 days of gestation. Before delivery, the fetal condition in utero was reassuring, in terms of fetal heart rate monitoring and biophysical profile score. A 532 g female baby was delivered. We applied NPPV postoperatively, and she was discharged on day 12 after delivery without any complications.
|
[[40.0, 'year']]
|
F
|
{'15599160': 1, '20702082': 1, '33743806': 2, '17067334': 1, '24591671': 1, '28560149': 1, '28282780': 1, '26094569': 1, '21514554': 1, '21354656': 1, '26342729': 1, '23288967': 1, '14974057': 1, '23728654': 1, '28282767': 1, '3578433': 1, '27512467': 1, '23235608': 1, '24150027': 1, '22292815': 1, '27651980': 1, '19558746': 1, '30186649': 2}
|
{'6114237-1': 2, '6114237-2': 2, '7981838-1': 1}
|
2,258 |
6114244-1
| 30,186,654 |
comm/PMC006xxxxxx/PMC6114244.xml
|
Successful Localization of the Source of Hemorrhage in Patient with Post-Whipple Surgery by 99mTc-Labelled Red Blood Cell Scintigraphy
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A 53-year-old patient was recently diagnosed with pancreatic cancer with obstructive jaundice, for which he underwent a Whipple procedure. Unfortunately, the procedure was complicated with a pancreatojejunostomy anastomosis leak, deep vein thrombosis deep vein thrombosis (DVT), and postoperative-bleeding. He was taken for exploratory laparotomy and a revision of gastrojejunostomy anastomosis without the successful localization of the source of the bleeding; later the same day, the patient underwent diagnostic DSA, which also failed to localize the source of the bleeding. GIBS was requested for better localization of the GI bleeding source.\nThe study was positive for active bleeding, which had started primarily high in the right upper abdomen, supposedly from the region of the hepaticojejunostomy (Figures and ). Therefore, the patient was transferred to the angiography suite for another diagnostic and therapeutic DSA. Selective catheterization of the superior mesenteric artery was performed followed by an angiogram, which showed no active contrast extravasation and identified no abnormality. Then selective catheterization of the celiac trunk was followed by an angiogram, which showed small contrast extravasation originating from the proximal common hepatic artery, most likely from the gastroduodenal artery stump (). Using a coaxial microcatheter/microwire utilizing an Echelon catheter and synchro-wire, contrast was injected for the selective catheterization of the small arterial branches originating from the proximal main common hepatic artery. A small extravasation was confirmed, and while the catheter remained in the same position, coil embolization was performed utilizing three coils measuring 2 mm. After that, an angiogram was performed that showed no extravasation and no abnormality (). And upper abdominal angiogram was also performed, again demonstrating no abnormalities. No immediate complications were encountered.
|
[[53.0, 'year']]
|
M
|
{'7916530': 1, '27101433': 1, '22393223': 1, '8122642': 1, '6331732': 1, '3876189': 1, '9106699': 1, '8372742': 1, '2334015': 1, '26678616': 1, '18613444': 1, '12029216': 1, '21178067': 1, '16332485': 1, '30186654': 2}
|
{}
|
2,259 |
6114246-1
| 30,186,653 |
comm/PMC006xxxxxx/PMC6114246.xml
|
Scapular Bronchogenic Cyst in a Girl Presenting as Recurrent Cellulitis: A Case Report and Review of the Literature
|
Our patient is a 5-year-old female who was referred for evaluation and management of recurrent episodes of cellulitis in the left scapular region. A small cystic lesion had first been noted at the age of 2 years. While this had initially been an asymptomatic, small lump that grew in size over time, it first became symptomatic when the patient was 4 years old. The lesion developed surrounding induration and erythema, as well as purulent drainage and tenderness. Over the next 1-year period, she went on to develop 3 such episodes of cellulitis. This was treated with a 10-day course of cephalexin by the patient's family physician, and it was due to these recurrent episodes of infection that we saw the patient in consultation.\nPhysical examination revealed a playful and well-appearing 5-year-old female, weighing 24.8 kg and measuring 114.5 cm in height. Review of systems and cardiorespiratory examinations were unremarkable. On inspection of the left scapular region, approximately 7 × 5 cm area of cellulitis was noted, with a small opening and associated purulent drainage. The surrounding skin was tender to palpation, but there was no appreciable fluctuance. Thus, the initial working diagnosis was infected epidermoid cyst. An ultrasound of the affected area showed a complex cystic mass measuring 3.9 × 2.9 × 3.7 cm within the subcutaneous fat (). Deeper margins of the mass were poorly demarcated due to the degree of inflammation, and the lesion appeared to abut the underlying musculature. Since these findings were nonspecific, an MRI was obtained to further characterize the lesion. The MRI revealed the presence of a subcutaneous cystic lesion just superior to the scapula measuring 1.6 × 3.5 × 2.8 cm. While the mass abutted underlying muscular fascia, there was no extension into the underlying trapezius muscle itself (Figures and ). Thus, the decision was made to pursue surgical excision of the lesion. This was performed under general anesthesia, which the patient tolerated well. Intraoperatively, we noted that the mass was quite soft, cystic, irregular in shape, and not well circumscribed. Nonetheless, we were able to excise the lesion in its entirety. Histopathology from this specimen revealed a benign cyst lined with ciliated columnar epithelium (). In addition, the cyst wall itself contained smooth muscle, small mucous glands, and clusters of lymphocytes. Thus, these findings were in keeping with a bronchogenic cyst and not an epidermoid cyst as was initially suspected. On 1-year postoperative follow-up, the patient is doing well, with no recurrence of infection or other symptoms.
|
[[5.0, 'year']]
|
F
|
{'25515809': 1, '12661817': 1, '8902460': 1, '16180006': 1, '8008684': 1, '17859095': 1, '21866472': 1, '4056165': 1, '15058837': 1, '28767594': 1, '20011500': 2, '11807461': 1, '26512714': 1, '11732728': 1, '22496944': 1, '12809250': 1, '24441234': 1, '34376164': 2, '2809973': 1, '5567731': 1, '28144783': 1, '8021357': 1, '8747573': 1, '30186653': 2}
|
{'2788465-1': 1, '8353838-1': 1}
|
2,260 |
6114280-1
| 30,157,806 |
comm/PMC006xxxxxx/PMC6114280.xml
|
Successful use of combined blood purification techniques in splenectomised patient with septic shock in streptococcus pneumoniae infection – a case report
|
52-year old woman with a history of chemotherapy for coat cell lymphoma in 2011, splenectomy in 2013 and autologous bone marrow transplantation in 2014 was admitted to the medical intensive care unit (ICU) after having fever up to 38.7 °C and malaise for 24 h. On admission, she was somnolent; the skin was cold, wet and pale; body temperature was 38 °C, blood pressure 50/40 mmHg and puls 120/min. She was eupnoeic with oxygen saturation (SatO2) of 100% by pulse oximetry, inspiring 2 L of oxygen by nasal cannula. Clinical examination revealed rales over both lungs and tachycardia without heart murmurs. Abdomen was soft and painless with audible peristalsis. Standard electrocardiogram (ECG) showed sinus tachycardia of 125/min.\nOn admission, we started continuous ECG monitoring, pulse oximetry, non-invasive blood pressure measurements and inserted central venous, arterial and urine catheters to measure central venous pressure intermittently, arterial blood pressure continuously and diuresis per hour.\nWe suspected sepsis with septic shock and immediately started treatment of shock and diagnostic procedures for sepsis. We managed shock initially by rapid infusion of crystalloids until we confirmed fluid unresponsiveness by ultrasound of inferior vena cava, demonstrating its diameter of 2.2 cm, that did not change with inspiration. Therefore, we started noradrenalin infusion within the first 15 min and up titrated it to 66μg/min. In addition, bedside echocardiography showed decreased ejection fraction (EF) of the left ventricle to 20%. We added dobutamine infusion, but also glucocorticoids and later on vasopressin to reach normotension.\nFrom the very start we suspected pneumonia on clinical grounds and confirmed it by bilateral infiltrates on chest rentgenograph. Among admission laboratory data we observed lactacidosis (arterial pH 7.24, bicarb 13.4 mmol/l, pCO2 4.24 kPa, pO2 13 kPa, lactate 7.5 mmol/l), thrombocytopenia (62 × 103/μL), leucocytosis, increase of procalcitonin to 100 ng/ml, C-reactive protein (CRP) to 166 mg/l, N-terminal-pro brain natriuretic peptide (NT-proBNP) to 2114 pmol/l, myoglobin to 482μg/l, and serum creatinine to 288 μg/l. Admission SOFA score was eight. We collected hemocultures, urinoculture and aspirates as soon as possible and after that immediately administered imipenem 500 mg/6 h IV.\nAfter the first 24 h positive pneumococcal urine antigen confirmed streptococcal pneumonia. We continued imipenem therapy and adjusted the dose to renal failure. Other microbiological cultures remained negative. Together with the specialist for infectious disease we decided to continue imipenem therapy due to prior disease, including splenectomy.\nAfter 24 h of ICU-stay the patient needed 40% oxygen by mask to achieve satisfactory blood gases (pH 7.2, bicarb 15 mmol/l, paCO2 5.35 kPa, paO2 8.5 kPa), her body temperature was 38 °C. SatcvO2 was 76.1%. Luckily, the patient did not need neither non-invasive, nor invasive ventilation during the entire ICU stay.\nIn spite of all treatments, after the first 24 h multiorgan failure syndrome persisted, including severe systolic myocardial dysfunction with left ventricular EF of 20%, measured by echocardiography. SOFA score at that time was 12.\nAfter 36 h of ICU stay resistant septic shock with high-dose catecholamine support, left ventricular dysfunction with EF of 20% persited and renal failure (serum creatinine 379μmol/l, daily urine output < 500 ml) worsened. SatcvO2 was 78%, body temperature 37 °C and SOFA score increased to 13.\nIn addition to echocardiography, Pulse Contour Cardiac Output (PiCCO) catheter was inserted to improve hemodynamic monitoring and demonstrated cardiac index (CI) of 3.3 l/min/m2) with stroke volume (SV) of 50 ml, increased global end-diastolic index (GEDI) to 1023 ml/m2 and extra vascular lung water index (ELWI) to 13.3 ml/kg and decreased systemic vascular resistance index (SVRI) of 1672 dyn.s.cm− 5.m2.\nPersistant hemodynamic instability and worsening renal failure led to the decision to start continuous veno-venous hemofiltration (CVVH) combined with hemoadsoption treatment by CytoSorb® membrane for the next 24 h. The goal was to improve hemodynamic situation and modulate the inflammatory response in our splenectomised septic patient. Before the start of blood purification therapy, we measured serum IL-6 level, which was 114 pg/ml.\nAfter only 24 h of CVVH with concomitant use of a single CytoSorb® membrane EF increased to 45%. PiCCO measurements improved as follows: GEDI changed to 805 ml/m2, ELWI to 11.2 ml/kg, SVR to 1888 dyn.s.cm− 5.m2 and CI to 3.95 min/m2 and SV to 61 ml. The patient’s temperature was 37 °C and SOFA score 11. IL-6 dropped from 114 pg/ml to 14,2 pg/ml after termination of hemoadsoption therapy.\nWe could stop the use of dobutamine, norepinephrine and vasopressin. The next day SOFA score was seven. Serum lactate and arterial pH turned to normal within few days, as well as CRP, procalcitonin (Fig. ), leucocyte and platelet count after 14 days (Fig. ). Table presents the course of the treatment.\nFor regeneration of the kidney function the patient received CVVH intermittently for another 21 days. She was discharged from ICU after 10 days and from the hospital after 76 days.
|
[[52.0, 'year']]
|
F
|
{'15812624': 1, '22136372': 1, '25186837': 1, '23866032': 1, '26543526': 1, '32195065': 1, '26247933': 1, '18651035': 1, '28343448': 1, '26566443': 1, '31571940': 1, '23971020': 1, '12720560': 1, '28101605': 1, '24292568': 1, '21906334': 1, '18434884': 1, '24249974': 1, '32194424': 1, '30157806': 2}
|
{}
|
2,261 |
6114482-1
| 30,153,804 |
comm/PMC006xxxxxx/PMC6114482.xml
|
Recurrent orbital bone sub-periosteal hematoma in sickle cell disease: a case study
|
A 12-year-old boy with SCD was presented with fever, periocular pain, and diplopia after returning from Taif, Jeddah. Taif (means “encompassing”) is located in the Hejaz Mountains of Saudi Arabia. It is considered as a high-altitude area because it is 6000 ft above the sea level []. The patient had a previous history of similar attacks that resolved after conservative management at another hospital in the same city few years ago (Fig. ).\nOn admission, the patient looked sick, drowsy, and pale. The temperature of patient was 38.2 °C, heart rate was 115/min, respiratory rate was 25/min, blood pressure was 100/65, and oxygen saturation was 90% on room air. The patient weighted 38 kg. Ocular examination showed right eyelid edema, peri-ocular soft tissue swelling, proptosis, and limitation in elevation of the right eye. On admission, the visual acuity of right eye was 20/30 and left eye was 20/20. Color vision was evaluated using the color plates that came out to be normal. The pupils were equal in size and reactive to light. Swinging light Reflex showed normal reaction of both pupils. There was no afferent pupillary light reflex defect (APD). The intra-ocular pressure was normal in both eyes. Fundus examination revealed normal disc, blood vessels, and macula. A complete systemic evaluation was conducted.\nThe systemic evaluation revealed hemolytic anemia, thrombocytopenia, stable coagulation profile, and negative blood culture. Laboratory results showed hemoglobin level of 89 g/ L, mean cell volume was 84.2 FL, white blood cell count was 24.04X109/L with neutrophils 21.81X109/L, and mean platelets volume 10.30 FL. Serum bilirubin was measured to be 95.5 mmol/L, albumin was 26 g/L, blood urea was 3.8 mmol/L, and serum creatinine was 39 mmol/L. The erythrocyte sedimentation rate was 40 mm/h (normal < 15 mm/h), and C-reactive protein was 8.2 mg/dL (normal < 0.5 mg/dL) (Table ).\nThe coagulation parameters revealed a prothrombin time (PT) of 14 S (normal 10–12.8), International Normalization Ratio (INR) 1.2 (normal 0.9–1.2) and activated partial prothrombin time (aPTT) 33.4 S (normal 25.3–38.4). Hemoglobin electrophoresis showed HbS 58%, HbA 36%, HbF 2%, and HbA2 4% (consistent with sickle b thalassemia). Urine analysis was normal, and the culture report was negative. Magnetic Resonance Imaging (MRI) of the right orbit demonstrated peri-orbital edema and a mass adjacent to the right orbital wall. This condition was identified as a superior subperiosteal haematoma with evidence of orbital bone and bone marrow abnormal signals consistent with orbital wall infarction (Fig. ).\nThe bone abnormality was further investigated to explore the possibility of the presence of primary or metastatic tumors that are susceptible to bleeding. Therefore, CT-imagery was utilized to explore this area of interest. However, as may be seen from the CT images, there was no evidence of primary or metastatic bone tumors (Fig. ).\nThe patient received intravenous fluids, analgesics, broad spectrum antibiotics, and pulse methylprednisolone immediately. The patient responded well to medical management with complete recovery and was discharged after the condition was stabilized. This case has highlighted the importance of considering orbital wall infarction in the differential diagnosis of orbitopathy among the patients with SCD, along with osteomyelitis and orbital abscess. Careful evaluation, diagnosis, and the prompt initiation of the appropriate supportive care are highly recommended in order to prevent permanent visual loss.
|
[[12.0, 'year']]
|
M
|
{'26790559': 1, '11827001': 1, '34532046': 2, '33163693': 1, '34765338': 2, '22476181': 1, '23813607': 1, '27642150': 1, '27677923': 1, '20577135': 1, '18946577': 1, '16214655': 1, '7340130': 1, '18520831': 1, '20631366': 1, '5130214': 1, '26157663': 1, '27979860': 1, '30153804': 2}
|
{'8435225-1': 1, '8575328-1': 1}
|
2,262 |
6114485-1
| 30,181,777 |
comm/PMC006xxxxxx/PMC6114485.xml
|
Stable transmission of an unbalanced chromosome 21 derived from chromoanasynthesis in a patient with a SYNGAP1 likely pathogenic variant
|
The patient was a two-year eleven-month old boy born to non-consanguineous 21-year-old primigravida mother of Ashkenazi Jewish descent and 25-year-old father of Ashkenazi Jewish and Irish descent. Pregnancy was uncomplicated and he was born at 41 + 4 weeks gestation via spontaneous vaginal delivery. Apgar scores were 9 at 1 and 5 min. Birth weight was 3.834 kg (50-75th centile). Neonatal course was unremarkable.\nDevelopmental concerns arose in early infancy. He sat unsupported after twelve months of age and walked independently at twenty-two months of age. At 2 years, 3 months of age, he could go upstairs with two-hand support and climb furniture. Gait was ataxic. He had a palmer grasp, could hold objects at the midline for thirty seconds, and could not transfer objects. He did not have any words but had recently started using gestures. Reception was limited to one step commands. He was easily excitable but demonstrated good socialization attempts with other children.\nMedical history was significant for myoclonic seizures starting between 2 to 3 years of age, requiring anti-epileptic medications. He preferred pureed foods, with occasional choking episodes. He also displayed preference for specific textures, and a fascination for water. Sleep was disrupted with frequent awakenings, thought to be behavioural. Echocardiogram, abdominal ultrasonography, brain magnetic resonance imaging (MRI), and genetic testing for Angelman syndrome were normal.\nAt 2 years, 3 months of age, weight was 12.1 kg (15-50th centile), height was 87.5 cm (15-50th centile) and head circumference was 48 cm (− 1 SD). He had deep-set eyes, down slanting palpebral fissures, prominent nasal root and tip, prominent ears, with a myopathic expression (Fig. ). An intention tremor and ataxic gait were noted.\nFamily history was notable for maternal anxiety, and attention deficit disorder in two maternal cousins. His mother and maternal grandfather had completed college and post-graduate professional education respectively. There was no family history of GDD, ataxia, other neurological disorders, infertility or multiple miscarriages. As the father was no longer involved in the child’s care, further detailed paternal history was unavailable.
|
[[21.0, 'year']]
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M
|
{'24143197': 1, '25799107': 1, '32010222': 1, '23478216': 1, '23708187': 1, '28260531': 1, '26936114': 1, '21925314': 1, '23161826': 1, '21215367': 1, '26989088': 1, '30951674': 1, '19196676': 1, '33802828': 1, '23135524': 1, '33426154': 1, '26079862': 1, '25439810': 1, '26312826': 1, '22388000': 1, '30181777': 2}
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{}
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2,263 |
6114493-1
| 30,153,845 |
comm/PMC006xxxxxx/PMC6114493.xml
|
Pleomorphic lobular carcinoma of the breast with osteoclast-like giant cells: a case report and review of the literature
|
A 72-year-old woman was referred to our hospital with one-month history of a palpable mass with burning sensation in her left breast. Mammography revealed a nodular increased density of the upper inner quadrant of the left breast considered to be suspicious of malignancy, Breast Imaging Reporting and Data System category 5 (BI-RADS-5).\nUltrasound revealed a hypoechoic mass with irregular and poorly defined margins measuring 23 mm × 14 mm. The ipsilateral axillary lymph nodes were normal. After a diagnosis of malignancy on core needle biopsy, the patient underwent simple mastectomy of the left breast and sentinel lymph node biopsy.\nOn gross examination, two neighboring foci were found measuring together 28 mm × 17 mm. There were ill-defined whitish lesions with soft red-brown areas inside. No nipple and periareolar lesion were seen. Histologically, both tumor foci were identical, and similar features were observed in the 6 sections examined. The tumor showed high cellularity arranged in sheet of discohesive cells with large cytoplasm and marked nuclear atypia. The tumour cells showed 15 mitosis per high power microscopic field. The lesion included numerous osteoclast-like giant cells containing many small uniform nuclei and hemosiderin-laden macrophages. The stroma was loose, highly vascular with hemorrhagic areas and foci of chronic inflammatory infiltration. Some carcinomas in situ foci were also identified at the periphery of the infiltrating tumour (Fig. ).\nOne of the three sentinel lymph nodes analyzed using routine intraoperative One-Step Nucleic Acid Amplification (OSNA) assay showed metastasis (17,000 copies/uL). Subsequently, ipsilateral axillary dissection was performed and no additional metastases were found in 14 additional lymph nodes resected.\nThe immunohistochemical study (see Additional file : Table S1) demonstrated the epithelial nature of the neoplasia, since the tumour cells expressed both cytokeratins AE1/AE3 and CK19 that were positive. Due to the discohesive nature of the cells, immunostaining for E-cadherin was performed and demonstrated complete absence of expression in both, the in situ and the invasive components. On the contrary, giant cells were negative for cytokeratin expression but were strong positive for the histiocytic marker CD68. With these features, the diagnosis was of invasive pleomorphic lobular carcinoma (histological grade 3) with OGCs (Fig. ).\nBiomarker analysis demonstrated that the carcinoma was estrogen receptor (ER) positive (strong positivity in 90% of tumor cells); progesterone receptor (PR) negative (complete absence of expression); and demonstrated lack of overexpression of human epidermal growth factor receptor type 2 (HERCEPTEST 1+). The Ki67 cellular proliferation index was 18%. The analysis of immune related markers demonstrated that, after counting at least 10HPF, the tumors has a mean of 34 CD3+ lymphocytes per 1HPF, 22 CD8+ lymphocytes per 1HPF, 2 CD4+ lymphocytes per 1HPF, and 1 CD20+ lymphocyte per 1HPF. Only occasional tumor cells (less than 1%) were PDL-1 + .\nThe tumor was subjected to molecular analysis by targeted next generation sequencing. DNA was extracted from a punch focused on the area with greater tumour cell density by using QIAamp® DNA FFPE Tissue Kit (QIAGEN). Quantification (447 ng/μl) and qualification (DIN = 4.6) of the DNA was performed using a Tape Station 2200 (Agilent) and the Genomic DNA kit. We used an in-house panel based on hybrid capture for sequence enrichment including the 34 genes frequently mutated in breast cancer (AKT1, ARID1A, ARID1B, BRCA1, BRCA2, CASP8, CCND1, CDH1, ERBB2, ESR1, FGFR1, GATA3, GRB7, GSDMB, MAP2K4, KRAS, MAP3K1, MLL3, MYC, NCOR1, NF1, PGAP, PIK3CA3, PNMT, PTEN, RB1, SF3B1, STARD3, TBX3, TCAP, TP53, VGLL1, ZNF217, ZNF703) and regions in chromosome 8 (targeting amplification of FGFR1 and MYC), chromosome 11 (targeting amplification of CCND1), chromosome 17 (targeting amplification of ERBB2) and chromosome 20 (targeting amplification of ZNF217). With this technique, we identified a deleterious mutation (C.del866C) in CDH1 (the gene coding for E-cadherin protein) (Fig. ).\nThe patient was enrolled in a trial that studies giving tamoxifen with or without combination chemotherapy in postmenopausal women who have undergone surgery for breast cancer. The patient was randomized on the arm for receiving only hormone treatment. The patient remains well without evidence of recurrence or metastases two years after surgery.
|
[[72.0, 'year']]
|
F
|
{'12861063': 1, '30641862': 1, '24728734': 1, '6704923': 1, '18668643': 1, '32716010': 1, '33672165': 2, '1398644': 1, '26926684': 1, '16138376': 1, '32723564': 1, '26451490': 1, '33203195': 1, '11348418': 1, '18473330': 1, '11117786': 1, '2985488': 1, '1592388': 1, '8127635': 1, '2813192': 1, '19154262': 1, '26729235': 1, '474518': 1, '9622697': 1, '25773929': 1, '30153845': 2}
|
{'7927002-1': 1}
|
2,264 |
6114516-1
| 30,181,904 |
comm/PMC006xxxxxx/PMC6114516.xml
|
Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization
|
A 65-year-old white male presented 10 days after the onset of a central scotoma in the left eye (LE). BCVA at presentation was 20/20 in the right eye (RE) and 20/32 in the LE. Fundus examination of the LE revealed an area of RPE atrophy in the inferonasal macula with foveal sparing (Fig. ). FAF in the left eye revealed a trizonal pattern and a demarcating hyper-FAF line between the involved and uninvolved retina (Fig. ); these findings were consistent with AZOOR [].\nTen days later, he returned complaining of distortion. Fundus examination revealed zonal lesion expansion, which was confirmed by FAF. The OCT revealed mild subretinal fluid (SRF) which explained his metamorphopsia. The patient returned a week later. At this time, visual acuity had reduced to 20/60, and two perilesional haemorrhages were observed along with a hypo-FAF zonal lesion expansion which included the fovea (Fig. ). The OCT showed a significant increase of subretinal fluid. These findings were consistent with a type 2 (subretinal) CNV which was confirmed by fluorescein angiography. Consequently he received 3 monthly intravitreal bevacizumab injections. One month after the first injection, FAF revealed zonal progression (Fig. ) and OCT revealed a worsening of SRF (Fig. ). One month after the third injection, BCVA in the LE was 20/70 and the patient reported stabilization of his central scotoma. At month 5, BCVA was 20/60; OCT scan showed resolution of SRF, while FAF revealed minimal advancement of zonal lesion size. During follow-up the zonal lesions stabilized; the patient received 11 intravitreal bevacizumab injections on a treat and extend regimen for 4 years and maintained a BCVA of 20/60 in the LE.
|
[[65.0, 'year']]
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M
|
{'18978936': 1, '17389511': 1, '22105506': 1, '17467524': 1, '21031022': 1, '18003800': 1, '23135525': 1, '8340485': 1, '20691421': 1, '20661173': 1, '23591538': 1, '27243784': 1, '12614757': 1, '7977571': 1, '12208243': 1, '11078842': 1, '15531341': 1, '21056448': 1, '17846358': 1, '26731213': 1, '24945598': 1, '7872395': 1, '18705680': 1, '18547281': 1, '25390984': 1, '30181904': 2}
|
{'6114516-2': 2, '6114516-3': 2, '6114516-4': 2}
|
2,265 |
6114516-2
| 30,181,904 |
comm/PMC006xxxxxx/PMC6114516.xml
|
Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization
|
A 69-year-old white male was referred for sudden onset scotoma with blurriness and photopsia in the LE. BCVA was 20/20 in the RE, and 20/40 in the LE. Fundus examination revealed bilateral peripapillary atrophy and retinal swelling at the macula of the LE (Fig. ). OCT scan and FA revealed a juxtafoveal type 2 CNV in the LE. One intravitreal bevacizumab injection was administered in the LE. Four 4 weeks after the injection, BCVA was stable; in the RE the peripapillary lesion extended and new zonal lesions were visible temporal to the fovea (Fig. ); in the LE the peripapillary and the macular defect extended and merged appearing as a single, large zonal defect involving the fovea. FAF in both eyes revealed a trizonal pattern and a demarcating hyper-FAF line between the involved and uninvolved retina (Fig. ); these findings were consistent with AZOOR [].\nFA showed persistence of leakage from the CNV and two additional intravitreal bevacizumab injections were administered. One month after the third injection, further progression of the zonal lesions was observed in both eyes (Fig. ). SD-OCT scan showed complete resolution of the subretinal fluid with persistent well-defined subretinal hyperreflective material (Fig. ). Since then, the patient has maintained a BCVA of 20/50 in the LE (and 20/25 in the RE) with no evidence of progression of the zonal lesions and CNV stabilization during 2 years of follow-up.
|
[[69.0, 'year']]
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M
|
{'18978936': 1, '17389511': 1, '22105506': 1, '17467524': 1, '21031022': 1, '18003800': 1, '23135525': 1, '8340485': 1, '20691421': 1, '20661173': 1, '23591538': 1, '27243784': 1, '12614757': 1, '7977571': 1, '12208243': 1, '11078842': 1, '15531341': 1, '21056448': 1, '17846358': 1, '26731213': 1, '24945598': 1, '7872395': 1, '18705680': 1, '18547281': 1, '25390984': 1, '30181904': 2}
|
{'6114516-1': 2, '6114516-3': 2, '6114516-4': 2}
|
2,266 |
6114516-3
| 30,181,904 |
comm/PMC006xxxxxx/PMC6114516.xml
|
Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization
|
A 33 year-old white female presented with central scotoma and distortion in the RE for 2 months. She was 3 months post-partum and nursing at the time of presentation. BCVA was 20/30 in the RE and 20/20 in the LE. Dilated fundus examination showed multiple, well-demarcated zonal areas of outer retinal atrophy at the posterior pole and at the mid periphery of both eyes (Fig. ). There were pigmented brownish dots located mainly at the margins of the atrophic areas but also within the lesions bilaterally. FAF in both eyes revealed a trizonal pattern and a demarcating hyper-FAF line between the involved and uninvolved retina (Fig. ); these findings were suggestive of AZOOR []. After 2 months, a progression of the zonal lesions was found in both eyes; in the RE, the progression occurred around the disc and towards the fovea and development of a subfoveal type 2 CNV was noted (Fig. ). Over a course of 3 years the patient underwent intravitreal anti-VEGF injections on a treat-and-extend regimen (12 ranibizumab and 5 aflibercept injections) and her vision has stabilized to 20/40 in the RE. During treatment there was zonal progression at the right macula (Fig. ). In the LE the zonal lesion at the posterior pole remained stable with fovea sparing and patient remained asymptomatic with a BCVA of 20/20.
|
[[33.0, 'year']]
|
F
|
{'18978936': 1, '17389511': 1, '22105506': 1, '17467524': 1, '21031022': 1, '18003800': 1, '23135525': 1, '8340485': 1, '20691421': 1, '20661173': 1, '23591538': 1, '27243784': 1, '12614757': 1, '7977571': 1, '12208243': 1, '11078842': 1, '15531341': 1, '21056448': 1, '17846358': 1, '26731213': 1, '24945598': 1, '7872395': 1, '18705680': 1, '18547281': 1, '25390984': 1, '30181904': 2}
|
{'6114516-1': 2, '6114516-2': 2, '6114516-4': 2}
|
2,267 |
6114516-4
| 30,181,904 |
comm/PMC006xxxxxx/PMC6114516.xml
|
Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization
|
In 2004 a 47 year-old white male presented with 2 months history of blurred vision in the RE, associated with photopsias. BCVA was 20/50 in the RE and 20/20 in the LE. Fundus examination of the RE showed a peripapillary lesion. Fundus examination of the LE was unremarkable. FAF of the right peripapillary lesion showed a trizonal pattern and a hyperautofluorescent border between the involved and uninvolved retina []. Upon consultation of the recent literature at the time [], the diagnosis of AZOOR was made. Small haemorrhages were observed at the temporal side of the lesion and fluorescein angiography showed the presence of a choroidal neovascularization. Decision was made to perform a standard PDT with a single spot of 2.5 mm focused on the temporal part of the zonal lesion. Three months after treatment no improvement of visual acuity was recorded. The PDT was not repeated and during follow-up the CNV enlarged resulting in macular scarring. In 2005 there was a central fibrotic pattern with persistent peripheral haemorrhages and subretinal fluid. In the RE BCVA improved from 20/100 to 20/40, with an eccentric viewing; this eye had no longer a reading ability. From 2005 to 2015, a progressive enlargement of the scar was noted in the RE (Fig. ). In 2009 small peripapillary lesions were found in the LE. FAF features of these lesions were consistent with AZOOR []. These zonal lesions increased in size during follow-up (Fig. ) and remained stable thereafter. At the last follow-up visit in 2015 the left eye eye maintained a BCVA of 20/20, without any symptoms.
|
[[47.0, 'year']]
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M
|
{'18978936': 1, '17389511': 1, '22105506': 1, '17467524': 1, '21031022': 1, '18003800': 1, '23135525': 1, '8340485': 1, '20691421': 1, '20661173': 1, '23591538': 1, '27243784': 1, '12614757': 1, '7977571': 1, '12208243': 1, '11078842': 1, '15531341': 1, '21056448': 1, '17846358': 1, '26731213': 1, '24945598': 1, '7872395': 1, '18705680': 1, '18547281': 1, '25390984': 1, '30181904': 2}
|
{'6114516-1': 2, '6114516-2': 2, '6114516-3': 2}
|
2,268 |
6114535-1
| 30,157,786 |
comm/PMC006xxxxxx/PMC6114535.xml
|
Eye damage due to cosmetic ultrasound treatment: a case report
|
A healthy 32-year-old Asian woman presented to the emergency department with pain, photophobia and blurred vision in the right eye. There were no associated illnesses, history of retinotoxic exposures (medications, light), or family history of eye disorders. Written informed consent was obtained; the procedures adhered to the Declaration of Helsinki, and the study was approved by the institutional review board of the Wuhan University Renmin Hospital.\nHer uncorrected visual acuity was 20/200 in the right eye, 20/25 in the left eye. Intraocular pressure (IOP) was 31 mmHg in the right eye and 16 mmHg in the left. Neuro-ophthalmic examination was RAPD positive in the right eye. Mid-dilated fixed pupil in the right eye (Φ ≈ 4 mm). Tyndall phenomena were observed, vitreous were normal without cells; fundus examination results were also normal. The anterior segment image present with iris pigment detachment at 9 o’clock in the right eye (Fig. -). Results of anterior segment optical coherence tomography (ASOCT) showed slightly shallow anterior chamber in right eyes (Fig. -). The unharmed left eye also show slightly shallow anterior chamber (Additional file : Figure S1). On subsequent questioning, the patient disclosed that she received an intense-focused ultrasound (IFUS) in a cosmetic surgery center to lift and tighten the upper eyelid. Ultrasonic probe was applied at the eyebrow area. She immediately complained of painful blurry vision, the treatment was stopped and she was transferred to hospital.\nAfter anti-glaucoma treatment for 1 day, IOP of the right eye dropped to normal range (21 mmHg). Uncorrected visual acuity in the right eye was improved to 20/160. Best-corrected visual acuity (BCVA) of right eye was 20/20 with refraction of − 1.50 DS/− 1.0 DC × 165. At 3 days follow-up, spectral-domain optical coherence tomography (SD-OCT) was performed using an SD-OCT/scanning laser ophthalmoscope system (Heidelberg Engineering). Automated light-adapted static perimetry (HFA II-I; Carl Zeiss Meditec) using a 30–2 protocol confirmed a pericentral scotoma corresponding to the abnormality in the right eye (Fig. ), which could be causal by acute increase of IOP induced optic nerve edema; the left eye finding was unremarkable. Standard full-field flash electroretinography (ERG), and visual evoked potential (VEP) was normal, ruling out retina and brain disease. Optical Quality Analysis System (OQAS) exam showed that the objective scatter index (OSI) was 1.0 in the right eye and 0.7 in the left, modulation transfer function (MTF) cut-off was 23.831 in the right eye and 28.694 in the left; indicating comparable worse vision quality in the right eye (Additional file : Table S1), at 1 month’s follow-up.\nAt presentation to us on the 3 months of her symptoms, the patient still complained with headache, blur vision with photophobia in the right eye. Her iris damage was remained, with IOP measurement in normal range. Acquired myopia became her major complain. A comprehensive eye exam was further performed by an optometrist. Negative and positive relative accommodation (NRA/PRA), and accommodation amplitude was measured. Clearing additional plus lens power was difficult for this patient, indicating accommodation spasm in the right eye.\nAccommodative spasm is a rare condition occurring in children, adolescents, and young adults. It can be caused by trauma [], emotional problems, and other causes. In our case, further exam confirmed her accommodative spasm which was partially reversed by cycloplegia drops and bifocals.The patient was put on 1% tropicamide eye drops once a day and flipper lenses training was applied to treat her accommodation spasm. After 1 month, the patient achieved better uncorrected vision acuity at distance in the right eye (20/40) (Additional file : Table S2).\nSafety and efficacy of IFUS in the aging eyelids have been studied and reported in the previous study []. Tightening of infraorbital laxity and skin can be achieved using the IFUS, is performed by heating the dermis and underlying tissue, where protein around the focal point will reach over 65 °C and denatured within milliseconds []. After the initial heat effects, the skin initiates a wound healing response, resulting in the formation of new collagen, which provides longer-term tightening of the skin []. In this case, the acoustic energy rays might have threaded through the eyelid, caused dysfunction of the ciliary muscle, which affected the zonular tension, causing acute increase of IOP and acquired myopia. The excessive curvature of refractive lens surface leaded to curvature myopia and accommodation spam (Fig. ). Heat-caused injuries may be associated with a transient IOP increase due to acute trabecular meshwork changes.
|
[[32.0, 'year']]
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F
|
{'17300599': 1, '18094311': 1, '17372061': 1, '34765802': 1, '22092848': 1, '15617937': 1, '26257234': 1, '25099495': 1, '23057597': 1, '30157786': 2}
|
{}
|
2,269 |
6114698-1
| 30,157,795 |
comm/PMC006xxxxxx/PMC6114698.xml
|
Optical coherence tomography findings of falciform retinal detachment complicated with persistent fetal vasculature
|
This present case involved a 22-year-old female who became aware of leukocoria in both eyes. Upon examination at another eye clinic, a vitreous strand was detected in her left eye, with a suspected diagnosis of PFV, and she was referred to the Department of Ophthalmology at Osaka Medical College Hospital, Takatsuki-City, Japan for a more detailed diagnosis and subsequent treatment.\nThe patient had previously been diagnosed with strabismus when she was 2 years of age. She was delivered at full term, with a birth weight of 3320 g, and she had no history of oxygen administration. We did not perform genetic investigation (i.e., sequencing) on the patient in order to diagnose PFV. In addition, her relatives had no previous history of visual impairment.\nAt initial visit, the clinical findings of a slit-lamp examination revealed a shallow anterior chamber in both eyes. In her right eye and left eye, the diameter of the cornea was 8 mm and 9 mm and the axial length was 15 mm and 19 mm, respectively, and microphthalmus was observed in both eyes. In her right eye, the fundus was not visible due to a cataract, and ultrasonic B-mode examination revealed total retinal detachment (Fig. ). A magnetic resonance imaging scan of the patient’s head revealed no calcification in the right eye and no abnormalities in her brain. In the left eye, retrolental fibrovascular proliferation was found around the temporal side. The fundus exhibited FRD from the optic disc to the inferior-temporal side (Fig. ). Most of the peripapillary retinal vessels were involved in the retinal folds, and a part of the nasal retina covered-over the optic disc. In the periphery of the fundus, retinal avascular area was observed over the entire circumference, and pigmentation was also observed in a wide range on the temporal side. An oscillating nystagmus was observed in both eyes, and was found to be prominent in the left gaze and less conspicuous in the right gaze when her face was turned to the left.\nDuring the clinical course, cataract and corneal opacification progressed, ultimately becoming phthisis bulbi in her right eye. On the other hand, from the age of 6 to 22 years, her left eye retained a corrected visual acuity of 0.08, and no significant change of the fundus was observed during that 16-year period (Fig. ). OCT images obtained when she was 22 years of age revealed bundle shading at the optic disc, combined with the finding that the nasal retina was overlaid on the optic disc (Fig. ). However, the layer structure of the surrounding retina was well preserved (Fig. ). On the temporal side of the optic disc, the elevated stalk of the fold protruding into the vitreous was observed at the site of the FRD, yet the upper and lower retinal layered structures were relatively well retained (Fig. ). However, on the temporal peripheral side, the retina was remarkable thinned, the layered structure was unclear, and the ellipsoid zone could not be clearly identified (Fig. ).\nGoldman kinetic visual field examination findings, with an isopter of V− 4, obtained when the patient was 22 years of age exhibited 50-degrees upwards, 40-degrees to the nasal side, 60-degrees downward, 75-degrees to the temporal side, and 80-degrees to the inferior-temporal side (Fig. ). The patient is currently undergoing yearly follow-up observations (i.e., once per year).
|
[[22.0, 'year']]
|
F
|
{'21872202': 1, '21868098': 1, '26477845': 1, '9372715': 1, '29190238': 1, '100893': 1, '33688352': 2, '26299697': 1, '1180303': 1, '33335385': 1, '30157795': 2}
|
{'7920703-1': 1}
|
2,270 |
6114735-1
| 30,153,864 |
comm/PMC006xxxxxx/PMC6114735.xml
|
Prenatal diagnosis and implications of microphthalmia and anophthalmia with a review of current ultrasound guidelines: two case reports
|
Patient A was a 36-year-old woman of Mauritanian ethnicity who presented for an initial hospital-booking visit at 13 weeks’ gestation. She had no known medical conditions and a non-consanguineous partner of Nepalese descent. Her obstetric history included a 35-week morphologically normal stillbirth of unknown etiology. The pregnancy with which she presented had a low-risk result for the first-trimester aneuploidy antenatal screening in the nuchal translucency program. A fetal morphology scan attended at 19 weeks identified potential fetal anomalies, leading to a tertiary referral for review. A detailed sonogram at 21 weeks’ gestation confirmed left microphthalmia (Fig. ) and a small biparietal diameter (< fifth centile).\nA range of investigations and management options were offered and consented to, including: genetic counseling, amniocentesis, single nucleotide polymorphisms (SNP) array testing, placental histopathological testing, preservation of cell line, and a full postmortem. A magnetic resonance imaging (MRI) examination was declined by the parents. Amniocentesis and chromosomal microarray showed a chromosomally normal male and genetic counseling was organized. The couple had significant concerns regarding the uncertain prognosis, leading to a decision for an elective termination. The fetal postmortem showed left-sided microphthalmia (Fig. ), with associated persistent hyperplastic primary vitreous, probable hypoplasia to the left side of the face, and a thin left optic nerve compared to the right. Placental histopathological results were normal.
|
[[36.0, 'year']]
|
F
|
{'23701296': 1, '23276567': 1, '22267908': 1, '27552085': 1, '31856529': 1, '20842655': 1, '15517557': 1, '27601422': 1, '24502600': 1, '20003547': 1, '18039390': 1, '23312594': 1, '23815237': 1, '1992414': 1, '12905522': 1, '34603993': 1, '16007635': 1, '21285886': 1, '18570241': 1, '20451288': 1, '15619313': 1, '24568872': 1, '7612513': 1, '18781617': 1, '23318894': 1, '23820083': 1, '11113891': 1, '16543359': 1, '30153864': 2}
|
{'6114735-2': 2}
|
2,271 |
6114735-2
| 30,153,864 |
comm/PMC006xxxxxx/PMC6114735.xml
|
Prenatal diagnosis and implications of microphthalmia and anophthalmia with a review of current ultrasound guidelines: two case reports
|
Patient B was a 31-year-old Caucasian woman with a non-consanguineous Caucasian partner and a history of a term normal birth followed by a first trimester miscarriage. She had no significant medical or family history and stated no illicit substance use. She presented with an uncomplicated pregnancy with a low-risk screening result on nuchal translucency for aneuploidy. At the 20-week fetal anomaly morphology scan, an absent right globe was identified (Fig. ) with mild bilateral ventriculomegaly. Fetal MRI at 20 weeks further delineated the absent right globe, dysplastic ventricular system (Figs. and ), and confirmed diagnosis.\nA screen for toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, listeria, parvovirus, and human immunodeficiency virus (HIV) (TORCH screen) completed at the time of diagnosis was negative. Our patient had no family history of fetal anomalies. Amniocentesis and microarray results showed no chromosomal anomalies in a male fetus. Our patient chose not to continue the pregnancy and a termination was performed without complication. An autopsy revealed right-sided anophthalmia with right optic nerve atrophy and mild bilateral ventriculomegaly. Placental histopathological results were normal. Genetic counseling and testing was organized.
|
[[31.0, 'year']]
|
F
|
{'23701296': 1, '23276567': 1, '22267908': 1, '27552085': 1, '31856529': 1, '20842655': 1, '15517557': 1, '27601422': 1, '24502600': 1, '20003547': 1, '18039390': 1, '23312594': 1, '23815237': 1, '1992414': 1, '12905522': 1, '34603993': 1, '16007635': 1, '21285886': 1, '18570241': 1, '20451288': 1, '15619313': 1, '24568872': 1, '7612513': 1, '18781617': 1, '23318894': 1, '23820083': 1, '11113891': 1, '16543359': 1, '30153864': 2}
|
{'6114735-1': 2}
|
2,272 |
6114737-1
| 30,181,717 |
comm/PMC006xxxxxx/PMC6114737.xml
|
Arteriolar vs. valvular thrombosis: Pick your evil!
|
A 72-year-old male with a past medical history of ESRD, chronic atrial fibrillation (AF) and rheumatic heart disease (RHD) status post mechanical mitral and aortic valve replacements presented to his primary care doctor complaining of left thigh erythema with skin induration. The patient had been on warfarin therapy for anticoagulation of his mechanical heart valves as well as prophylactically for underlying AF for greater than 20 years. He was initially diagnosed with cellulitis and treated accordingly with antibiotics. Despite multiple antibiotic regimens, the skin lesions did not improve and instead progressed into painful, necrotic ulcers. The lesions were evaluated by his nephrologist 2 months later who deemed cellulitis to be a misdiagnosis and recommended a biopsy of the skin lesions. The biopsy revealed pathology consistent with calciphylaxis, a lethal disease typically associated with ESRD.\nThe disease is known to be exacerbated by certain medications including warfarin, vitamin D analogs, calcium-based binders and glucocorticoids []. Other risk factors in ESRD patients include diabetes, hyperphosphatemia, obesity, hyperparathyroidism and hypercalcemia. The patient was on warfarin therapy and vitamin D analogs. He was also taking sevelamer, a non-calcium-containing phosphate binder, to prevent hyperphosphatemia.The vitamin D supplementation was discontinued but the cessation of warfarin was controversial.\nSince warfarin contributes to the development of calciphylaxis, it should have been exchanged for another form of anticoagulation to avoid progression of the non-healing, necrotic ulcerations. It was not accomplishable in this circumstance since LMWH is not Food and Drug Administration (FDA) approved in ESRD and is associated with serious bleeding and the need for frequent dose adjustments and monitoring []. The only other option for long-term anticoagulation was UFH administered subcutaneously but this approach was not taken. It is difficult to maintain therapeutic levels with UFH as it requires massive doses to do so. Because it was believed that the risk of two mechanical valve thromboses outweighed the risk of the discontinuation of anticoagulation, the warfarin was continued. Unfortunately, the lesions progressed over 1 year and the patient suffered from the sequelae of calciphylaxis despite the addition of sodium thiosulfate infusions to each hemodialysis session. With the advancement of the ischemic ulcerations, the patient developed superinfections and debridement was not sufficient to control the sepsis. He eventually expired due to septic shock.
|
[[72.0, 'year']]
|
M
|
{'23989356': 1, '23677243': 1, '22121234': 1, '10647757': 1, '16670137': 1, '11422768': 1, '28698808': 1, '23520041': 1, '3377615': 1, '22315264': 1, '24939033': 1, '22516723': 1, '28329059': 1, '23991661': 1, '30181717': 2}
|
{}
|
2,273 |
6114742-1
| 30,157,798 |
comm/PMC006xxxxxx/PMC6114742.xml
|
Toric IOL implantation in a patient with keratoconus and previous penetrating keratoplasty: a case report and review of literature
|
A 71-year-old man with previously known keratoconus presented with bilateral cataract (Fig. ). In the left eye, PKP had been performed when the patient was 25 years old because of keratoconus (Fig. ). No surgery had been done in the right eye. Because of discomfort with contact lenses, the patient wore glasses both for near and far distance. The patient had a medical history of a transient ischemic attack and medicated with acetylsalicylic acid. The right eye presented with advanced keratoconus including Vogt striae (Fig. ) in the cornea and moderate senile nuclear cataract but no other pathology. The left eye presented with a clear corneal graft and moderate senile nuclear cataract but no other pathology. First surgery was only planned in the left eye. After more than 1.5 years surgery was also performed in the right eye. Written informed consent was acquired from the patient.\nPreoperatively, best corrected visual acuity (BCVA) was 20/40, with − 0.25 sph − 5.0 cyl 50°. The cornea exhibited regular astigmatism (K1 44.5 D, K2 48.5 D, astigmatism 3.9 D) (Fig. ) based on corneal tomography performed with Scheimpflug imaging (Pentacam, Oculus, Germany). The toric IOL AcrySof IQ Toric SN6AT8 (Alcon, USA), 22 D was implanted with target refraction − 2.26 D. The target refraction was chosen to match the more myopic right eye. Biometry was performed with the IOLMaster (Carl Zeiss Meditec, Germany) and Haigis formula was used. Preoperative marking of the toric IOL axis was performed with the patient in upright position to avoid misalignment due to cyclotorsion, using the RoboMarker (Surgilum, USA). Phacoemulsification and lens implantation were performed through a 2.2 mm limbal incision. One day postoperatively, BCVA was 20/40 with − 2.0 cyl 90°. Five weeks postoperatively BCVA was 20/30 with + 0.5 sph − 2.75 cyl 71°. Nine months postoperatively BCVA had improved to 20/25 with − 3.25 cyl 90° and the astigmatism was still regular (Fig. ) based on corneal tomography performed with Scheimpflug imaging. Two years postoperatively BCVA was still 20/25 with − 0.5 sph − 3.25 cyl 80°. The spherical equivalent 2 years postoperatively only differed − 0.135 D from the intended target refraction. During all postoperatively controls the toric IOL only misaligned 1° (from 139° to 140°) from the implanted axis and the corneal graft remained clear. The patient was very satisfied with the visual result from day one postoperatively.\nPreoperatively, BCVA was 20/150, with − 5.75 sph − 9.75 cyl 72° and the cornea had irregular astigmatism (K1 53 D, K2 57.7 D, astigmatism 4.7 D) (Fig. ) based on corneal tomography performed with Scheimpflug imaging. In the right eye, the astigmatism was judged as being too irregular for toric IOL implantation and cataract surgery was performed with the spherical IOL Acrysof Multipiece MN60MA (Alcon, USA), 5 D. Conventional biometry (IOLMaster) and Haigis formula was used to calculate the power of the spherical IOL. Two months postoperatively, BCVA was 20/80 with + 1.25 sph − 3 cyl 65°, spherical equivalent − 0.25. Target refraction prior to surgery was − 2.33 D, however the patient was pleased with the obtained result. He continues to wear glasses for far distance but does not require glasses for near distance.
|
[[71.0, 'year']]
|
M
|
{'11084265': 1, '17873627': 1, '25283612': 1, '23966369': 1, '23079317': 1, '26795410': 1, '9493273': 1, '10434860': 1, '16765789': 1, '2272682': 1, '24321143': 1, '8127564': 1, '17251799': 1, '8053464': 1, '17159449': 1, '19672198': 1, '30157798': 2}
|
{}
|
2,274 |
6114781-1
| 30,153,795 |
comm/PMC006xxxxxx/PMC6114781.xml
|
A case report of dengue haemorrhagic fever during the peripartum period: challenges in management and a case of vertical dengue transmission
|
A 33-year-old previously healthy mother in her 2nd pregnancy, was admitted at 38 weeks of gestation with a one-day history of high fever with chills, myalgia, arthralgia, and headache. Her first pregnancy ended up as lower segment caesarian section (LSCS) due to the unfavorable cervix. On admission, she was febrile (102 °F). Her pulse rate (PR) and blood pressure (BP) were 88 beats per minute and 94/60 mmHg respectively. Rest of the cardiac and respiratory examination was unremarkable. Abdominal examination revealed a soft abdomen with a single live fetus and symphysiofundal height was compatible with gestational age. Vaginal examination revealed an unfavorable cervix. Laboratory investigation results were as follows: total white cell count (WBC) 7100/mm3, platelet count (PLT) 112,000/mm3, haemoglobin (Hb) 11.5 g/dl, packed cell volume (PCV) 30%, C reactive protein (CRP) 31 mg/l and positive NS 1 antigen. Liver function and renal function tests were normal. Cardiotocograph (CTG) was normal and the fetal biophysical profile was compatible with the period of gestation (POG). There was no ultrasound evidence of free fluid in the abdomen or pelvis. The diagnosis was made of uncomplicated dengue and managed according to current national dengue management guidelines.\nOn the 2nd day, she developed two episodes of vomiting and had mild intermittent abdominal pain. Her PR was 98 beats per minute and blood pressure was 90/64 mmHg. There was mild right hypochondriac tenderness. Complete blood count (CBC) showed WBC 6900/mm3, PLT 72000mm3 and PCV 32%. Ultrasonically, there was no free in abdomen or chest. A multidisciplinary meeting was convened and a decision made to deliver her baby by urgent LSCS. Blood, fresh frozen plasma, and platelets were preserved and LSCS was performed under spinal anaesthesia. A healthy male baby weighing 3.0 kg was delivered with an uneventful intraoperative period. Although there was no significant bleeding as a prophylactic measure B lynch sutures were applied and an abdominal drain was also inserted. She was then transferred to intensive care unit (ICU) for further management.\nOn the 3rd day, fever was persisting and she continued to complain of right hypochondriac pain. She became tachycardic with PR of 105 beats per minute. BP was 92/66 mmHg. CBC revealed WBC of 5300/mm3 with PLT of 63,000/mm3 and PCV of 35%. Ultrasound (USS) examination showed pericholecystic oedema with a thin layer of free fluid in the hepatorenal pouch. The diagnosis of dengue hemorrhagic fever was made and observations conducted according to the observation chart for management of dengue in adult patients with fluid leakage. A 3 hourly PCV monitoring, hourly capillary refill time (CRFT), PR, supine BP and urine output was conducted. CBCs were performed twice daily with daily alanine transaminase (ALT) and aspartate transaminase (AST). Serum albumin was found to be low and 2D ECHO was normal. To cover unexpected nosocomial infections, IV of 4.5 g Piperazillin tazobactam, 8 hourly, was started. The summary of investigations is shown below (Table ).\nDuring the first 12 h of the critical phase (CP), vital parameters were stable and a flat rate of fluid administration was continued. Towards the peak of the leaking phase, her PR started to rise with a narrowing of pulse pressure. As there was a sudden drop in PCV, possible intra abdominal bleeding was suspected and 500 ml of packed cells was transfused. Although the abdominal draining was absent, USS confirmed the high possibility of the presence of blood in the peritoneal cavity. Haemodynamic parameters were improved following transfusion. At the same time, intravenous (IV) tranexamic acid 1 g was also given. Although there was a further drop in platelets, clotting studies were well within the normal range. At the beginning of the second half of the critical phase, she had developed pedal edema, mild ascites with right-sided mild pleural effusion without respiratory compromise. Around the 36th hour of CP, urine output dropped to less than 0.5 ml/kg/hour. Due to the high possibility of fluid overload, IV dextran 350 ml was given. Thereafter she had an uneventful recovery from rest of CP. She was then transferred to the high dependency unit and later to the postnatal ward, for further care. During the recovery phase, she became fever free and went into polyuric phase. Her CBC showed WBC 10000/mm3, PLT 238000/mm3 and Hb 9.8 g/dl. IV antibiotics were stopped after completion of the one week course following the negative urine and blood culture reports. Throughout the illness, her clotting studies and renal functions were normal. Later, she was discharged and underwent an uneventful recovery.\nDuring her ICU stay, the baby was kept in the special baby unit (SBU) for the continuation of feeding and observation. He was never taken out of SBU and had no history of mosquito bites. The neonate was well until the 5th day of life but suddenly developed a high fever with jaundice. Sepsis or congenital dengue infection was suspected. Laboratory investigation results were as follows: WBC 5800/mm3, Hb 13.8 g/dl, PLT 126000/mm3, positive dengue NS 1 Antigen, AST 87 U/L, ALT 23 U/L, CRP 5.8 mg/l with normal liver and renal functions with the exception of an elevated indirect bilirubin of 35 micro mol/L. Diagnosis of dengue fever was made and managed according to current guidelines. Later, on the 7th day of life, his PLT count dropped from 86,000/mm3 to 43,000/mm3 and he developed malena. However, bedside USS revealed no evidence of fluid leakage or intracranial bleed. Haemodynamic parameters were within normal ranges except tachycardia of 170 bpm during the period of malena. 30 ml of PLT and 45 ml of packed red cell transfusion were done. With close monitoring and judicious fluid balance, the baby improved and was discharged from the SBU with PLT of 146,000/mm3 and Hb of 12 g/dl on the 11th day of life. However, his body weight had dropped to 2.580 kg upon discharge. On the 25th day of life, clinic review revealed weight gain up to 2.780 kg with age-appropriate milestone levels.
|
[[33.0, 'year']]
|
F
|
{'27982356': 2, '33147733': 2, '11747474': 1, '15115090': 1, '31616923': 1, '27213856': 1, '25299383': 1, '30153795': 2}
|
{'7768497-1': 1, '7768497-2': 1, '7768497-3': 1, '7768497-4': 1, '5147720-1': 1}
|
2,275 |
6114782-1
| 30,157,783 |
comm/PMC006xxxxxx/PMC6114782.xml
|
A case report of mucocutaneous tuberculosis after orthotopic liver transplantation: a challenging diagnosis
|
A 59-year-old Italian male, weighting 69 kg and 173 cm tall, came to our attention for an ulcerative lesion of the left lower lip (Fig. , Panel A). He had already received antibiotic treatment with amoxicillin/clavulanate plus antiviral acyclovir for 10 days in other outpatient facilities without any clinical improvement. His clinical history was remarkable for hepatitis B (HBV) and Genotype 3 hepatitis C (HCV) co-infection, which led to OLT due to HCC, and several years spent in foreign countries. In fact, when he was in his late 40 he had spent 6 years in Nigeria and one and a half year in the South of China where he worked at sea as a kitchen supervisor. The patient was HIV negative. Six months before the OLT he had received treatment with daclatasvir (60 mg/die), sofosbuvir (400 mg/die) and ribavirin (1000 mg/die) for HCV, successfully reaching sustained virological response (SVR) 12 weeks after the end of treatment. A QuantiFERON®-TB Gold In-Tube (QFT-G) was performed among the pre-transplant screening and resulted positive.\nNeither before nor after OLT, latent TB infection (LTBI) therapy was administered.\nPatient received OLT and 14 months post-transplant presented with a lower lip lesion. At the time of presentation patient was on the following medications: entecavir1000 mg daily for chronic HBV with lamivudine resistance, tacrolimus 3 mg daily and everolimus 1 mg twice a day for immunosuppression.\nA punch biopsy of the lower lip lesion was performed and submitted for extended microbiology and histological examination.\nThe histological examination suggested chronic granulomatous inflammation (Fig. , Panel B).\nReal time PCR (Xpert MTB/Rif™– Cepheid Sunnyvale, CA United States) was positive for MTB by high grading, implying a high bacterial load in the analysed specimen. No rpo-B mutation, affecting rifampicin resistance, was detected. Conventional microbiological investigations were also carried out: smear microscopy and automated liquid cultures (Bactec MGIT960™– Becton and Dickinson Franklin Lakes, NJ) were positive and the subsequent susceptibility testing showed sensitivity to all first-line drugs tested. A total body CT scan was performed to rule out presence of granuloma or signs of pulmonary or other extra-pulmonary site involvement. Moreover, Xpert MTB/Rif™–, Ziehl Neelsen and MTB colture on sputum resulted negative.\nA treatment with rifabutin (450 mg/daily), isoniazid (300 mg/daily), ethambutol (1200 mg/daily), pyrazinamide (1500 mg/daily) and daily supplementation of B6 vitamin was started for the intensive phase of 2 months. The therapeutic regimen was then simplified to rifabutin (300 mg/daily) plus isoniazid (300 mg/daily) for the following 4 months.\nLiver function and level of immune-suppressive treatment were monitored weekly. No increase in transaminases was observed and only a slight decrease in both tacrolimus (from5 μg/L to 3; normal value 5–7 μg/L) and everolimus (from 3 μg/L to 1.9 μg/L; normal value 2.5–3 μg/L) was noticed after 1 month of treatment; therefore, to achieve satisfactory blood-level concentrations, tacrolimus dosage was increased to 6 mg/ daily and everolimus was progressively titrated to 2.75 mg/ daily in two doses. After 3 weeks of therapy, a dramatic clinical improvement was observed and after 6 months of treatment the lesion was cured (Fig. , Panel D).
|
[[59.0, 'year']]
|
M
|
{'28761946': 1, '28260424': 1, '18277121': 1, '26616847': 1, '22496318': 1, '29226732': 1, '22008761': 1, '33536799': 1, '22849726': 1, '26671024': 1, '16300038': 1, '27838445': 1, '24696113': 1, '22994607': 1, '25211360': 1, '28579606': 1, '18564245': 1, '17067920': 1, '26405286': 1, '28153873': 1, '30157783': 2}
|
{}
|
2,276 |
6114836-1
| 30,153,816 |
comm/PMC006xxxxxx/PMC6114836.xml
|
Femtosecond laser-assisted removal of an intracorneal chestnut, a case report
|
A 32-year-old man presented with the symptoms of foreign body sensation and blurred vision in the left eye 3 days before presentation. The best-corrected visual acuity (BCVA, in decimal values) was 1.2 in the right eye and 0.6 in the left eye. Slit-lamp biomicroscopy (BX-900, Haag-Streit AG, Koeniz, Switzerland) of the left eye revealed an intracorneal foreign body, localized at the paracentral region, obliquely protruding to Descemet membrane with no penetration into the anterior chamber (Fig. ). The original entry path of the foreign body had sealed and epithelialized, leaving a sub-epithelial opacity and edematous stroma (Fig. ). The shadow effect shown in the anterior segment optical coherence tomography (AS-OCT, RTVue XR, Optovue, Inc., Fremont, CA) corresponded to the location of the intracorneal chestnut (Fig. ). The corneal thickness was approximate 755 μm at the site of lesion, of which 152 μm distance from the sealed corneal epithelium to the chestnut (Fig. ). The white ulcers with feathery edges or satellite infiltrates were not observed. The intraocular pressure, anterior chamber, lenses and the fundi appeared normal. No signs of systemic disorders were found in the presented case.\nThis study conformed to the principles of the Declaration of Helsinki and was approved by the Institutional Ethics Committee of Guangdong General Hospital and Guangdong Academy of Medical Sciences. After discussing with the patient and informed consent was obtained, femtosecond laser was applied using the protocols of IntraLase Enabled Keratoplasty (IEK, iFS™ Advanced Femtosecond Laser System). Following parameters were used: 300 μm lamellar depth, 7.5 mm diameter, 1.20 μJ energy, and cut angle with 180 degrees from 12 o’clock to 6 o’clock in the left eye (Fig. ). Under strict aseptic precautions, IEK was performed to create an anterior lamellar flap according to the routine procedures. The lamellar flap was easily separated with a flap lifter to expose the superior side of the chestnut. As shown in Fig. , the chestnut was then removed entirely with a pair of forceps under a surgical microscope (OPMI LUMERA 700, Carl Zeiss Meditec, Jena, Germany). After removal of the chestnut, no fluorescein leakage was found with the Seidel test and the wound was washed with 250 mL normal saline containing 40 mg gentamycin. The edges were dried for 3 min using a surgical sponge, and a soft contact lens (Extended wear, PureVision. Bausch and Lomb, NY) was applied over the surface. No suture was applied. The chestnut, shown in Fig. , was inoculated onto Sabouraud glucose agar and chocolate agar to detect potential growth of fungi and bacteria.\nBefore the surgery, Levofloxacin (Santen Pharmaceutical Co., Ltd. Japan) was prescribed for four times per day, and postoperatively, TobraDex (tobramycin 0.3% and dexamethasone 0.1%, s.a. Alcon-Couvreur n.v. Puurs, Belgium) was included and tapered weekly over a month. In 3 days after surgery, the patient complained of mild pain and blurred vision. These symptoms were relieved after treatment with the eyedrops. At three-month follow-up, a dot-like haze was noted in the cornea, which was corresponded to the scarring formation at the site of foreign body removal (Fig. ). As shown in Fig. , assessment with corneal topography (Oculus Pentacam Typ 70,700, Topcon, Tokyo, Japan) demonstrated that there was no surgical induction of corneal astigmatism compared to the preoperative astigmatism (Pre-Op, K1 = 42.1D, K2 = 42.9D, Axis: 166.7o vs 3-month Post-Op, K1 = 41.4D, K2 = 42.7D, Axis: 160.6o), and the Post-Op decimal BCVA in the left eye was improved gradually from 0.3 to 1.2. The results in microbiological culture of fungi and bacteria were negative.
|
[[32.0, 'year']]
|
M
|
{'27652132': 2, '34296044': 1, '19421030': 1, '23231742': 1, '8782262': 1, '12429259': 1, '10926993': 1, '20869117': 1, '25433753': 1, '25479452': 1, '31238077': 1, '30153816': 2}
|
{'5021655-1': 1}
|
2,277 |
6114853-1
| 30,153,860 |
comm/PMC006xxxxxx/PMC6114853.xml
|
Ankylosing spondylitis, chronic fatigue and depression improved after stromal vascular fraction treatment for osteoarthritis: a case report
|
Our patient was a 27-year-old Australian woman with grade IV OA confirmed by X-ray images of her pelvis; ultrasound scans showed right knee joint effusion, enthesitis, and synovitis; a CT scan of her spine indicated annulus bulges at L3/4 and L4/5, and bilateral grade 2 sacroiliitis changes; a background of AS (human leukocyte antigen-B27 negative) confirmed by MRI imaging; chronic pain syndrome with pain amplification; and post-traumatic stress disorder. Her body mass index (BMI) was 39.4 kg/m2. She did not have any: infection with hepatitis B, hepatitis C, or human immunodeficiency virus (HIV); malignancy; previous history of allergic reaction to any component of our therapeutic measure; active cardiac, respiratory, neurologic or endocrine disease necessitating receipt of medication. She was not pregnant or in lactating condition. A written and informed consent was obtained from our patient. Arthritic symptoms were measured using Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and Hip Disability and Osteoarthritis Outcome Score (HOOS) by scoring for pain intensity, walking ability (distance), joint stiffness, physical function, sports and recreation, and quality of life. Changes to her AS symptoms were measured using the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. For liposuction and stem cell treatment, she was admitted to Macquarie Stem Cells. Under light sedation and using aseptic technique, 450 ml of fat was harvested from her abdomen. Cell isolation was performed in PC II safety cabinet. Cells were isolated using collagenase digestion using Liberase GMP grade (enzyme blend).\nOur patient’s preoperative HOOS score (baseline score) for both hips was 122 (range 0–168), WOMAC for her right knee was 70 (range 0–90), and the baseline ASQoL questionnaire was 18 (range 0–18). We obtained 2.058 billion nucleated cells with a viability of 89.10% using Muse® Cell Analyzer. A total of 738 million cells were injected on the day: 100 million cells injected into each hip and right knee intra-articular under ultrasound guidance, and 438 million cells were administered as an intravenous infusion. The remaining 1.320 billion cells were cryogenically frozen into four separate vials of 330 million cells following the protocols of Thirumala et al. []. Follow-up intravenous infusions of 330 million cells were provided at 3 months, 12 months, and 36 months. Our patient’s follow-up intervals were performed at 1 day, 3 months, 6 months, 12 months, 24 months, and 36 months respectively. Neither local nor systemic adverse events were observed during the follow-up and she was satisfied with the therapy after 3 months with an increasing trend over the period. At 3-month post-treatment, she exhibited increased mobility. Her HOOS and WOMAC scores decreased to 82 and 37, respectively from her baseline scores. She also noted that pain in her spine, hips, and right knee associated with OA and AS had decreased. Interestingly, in addition to her decreased pain and increased mobility, she was feeling more energetic. Within 6 months after the first SVF infusion, her HOOS and WOMAC questionnaire scores had decreased to 79 and 31, respectively. She showed dramatic improvements over 2 years after her first SVF infusion and presented with decreased dependency on a wheelchair or walking stick (HOOS and WOMAC scores not available). Her dependency on pain relief and anti-depressant medications was found to be decreased as is evident from Table . At the 36-month follow-up, she presented significant improvements overall. She remained free from NSAIDs and her pain levels were minimal. Follow-up HOOS and WOMAC scores had decreased to 32 and 20, respectively. Her pre-treatment to post-treatment ASQoL score had decreased to 3 signifying increased quality of life. She still presents some symptoms of depression; however, her anxiety appears to have resolved almost completely. Her progressive improvement is observed over 3 years with WOMAC, HOOS, and ASQoL (Fig. ).
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[[27.0, 'year']]
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F
|
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{}
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2,278 |
6114880-1
| 30,181,879 |
comm/PMC006xxxxxx/PMC6114880.xml
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Pericardial injury from chest compression: a case report of incidental release of cardiac tamponade
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A 67-year-old woman, in good health other than systemic hypertension, lost consciousness soon after complaining of severe epigastric pain at her workplace. The ambulance crew found the patient in cardiopulmonary arrest and paramedics immediately started CPR by manual chest compressions; return of spontaneous circulation and recovery of consciousness occurred 4 min later. On arrival at the emergency room, the patient’s level of consciousness was 14 on the Glasgow Coma Scale, blood pressure was 102/74 mmHg, and pulse rate was 103/min. No cardiac murmur was detected, but vesicular breath sounds were moderately diminished in the left lung field. Cardiac enzyme studies were not consistent with a diagnosis of myocardial infarction. An ECG showed a normal sinus rhythm, and no arrhythmias or signs of myocardial ischemia were observed. A chest X-ray revealed massive left pleural effusion with no right pleural effusion, while cardiomegaly and pneumothorax were not identified. Transthoracic echocardiography demonstrated normally functioning ventricles and valves, and mild pericardial effusion. Computed tomography (CT) showed a type A acute aortic dissection (AAD) with thrombotic occlusion of the false lumen and an ulcer-like projection in the proximal arch, along with mild pericardial effusion and massive left pleural effusion (Fig. ). Occlusion of the branch vessels of the aortic arch and pulmonary emboli were not detected. Immediately after the CT, the patient fell into circulatory collapse. After drainage of bloody effusion from the left pleural space, an emergency operation was begun through a median sternotomy. No sternal fracture and bleeding in the mediastinum were found. When the pericardium was opened, a small amount of bloody effusion was present, but cardiac injury was not observed. In addition, a large laceration (10 cm) was found in the left posterolateral pericardium at the phrenico-pleural junction, through which the pericardial cavity communicated to the left pleural space (Fig. ). Neither injuries of other intra-thoracic organs such as the lung, vessels, or chest wall causing the hemothorax nor external rupture of the dissection were detected. Under cardiopulmonary bypass and cardiac arrest, the aorta was opened. The dissection with the thrombosed false lumen extended from the aortic root to the aortic arch. A primary tear was present on the lesser curvature of the proximal arch. Hemiarch replacement including the primary tear was performed without difficulty, but a large amount of inotropic agents was necessary for weaning off cardiopulmonary bypass. The postoperative course was complicated with severe low cardiac output syndrome, and the patient eventually died of multi-organ failure on postoperative day 30. An autopsy was not permitted.
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[[67.0, 'year']]
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F
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{'19327436': 1, '20494080': 1, '4757227': 1, '27095124': 1, '34608420': 2, '24525116': 1, '25533925': 1, '22966000': 1, '27889903': 2, '8912053': 1, '18807013': 1, '30181879': 2}
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{'8487396-1': 1, '5124430-1': 1}
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2,279 |
6114890-1
| 30,157,930 |
comm/PMC006xxxxxx/PMC6114890.xml
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Case report: lady with bone pains for 5 years—parathyroid carcinoma
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A 53 year old Pakistani lady presented to the Medicine clinic of a local hospital in 2004 with a history of heel pain and lower back pain for 5 months. In this period, the patient had sustained a rib fracture and left humeral fracture. There was no history of diabetes, hypertension or any other chronic disease. She had not been on any form of medication, including steroids and traditional drugs widely available and prescribed in the region, prior to the onset of pain. At the time of the fractures, she had been placed on non steroidal anti inflammatory agents, acetaminophen and tramadol. There was no history of illicit drug use and she was a non smoker. Family history was unremarkable, particularly in the context of bone disease, and malignancy.\nInitial laboratory investigations had shown a mildly elevated total calcium level of 10.8 mg/dL {2.7 mmol/L}-(no albumin level result available from that time for correction). Parathormone levels (PTH) had not been determined. There was no vitamin d or renal function report available from that time. X-Ray pelvis revealed lytic lesions in the right iliac bone (Fig. ). A magnetic resonance imaging (MRI) of the lumbosacral spine showed some signal changes. The differentials based on the MRI were metastatic bone disease or multiple myeloma.\nSerum protein electrophoresis was normal. The patient then got lost to follow-up. Her work up was resumed 4 years later when her bone pains had started flaring up. Bone marrow examinations done back in 2007, and later in 2009, were negative for multiple myeloma. A bone scan in November 2009 showed generalized increased tracer uptake over the skull and both the axial and appendicular skeletons- findings in favor of metabolic bone disease (Fig. a). An initial planar parathyroid sestamibi scan requested by a general practitioner in November 2009 was negative for any functioning parathyroid adenoma in the neck or superior mediastinum. No serum PTH report was available from this time either. Following this workup, the patient was treated empirically for bone pains with calcium supplements, an empiric vitamin d injection, and intravenous zoledronic acid 5 mg (without prior bone mineral density assessment via DXA scan). This empiric treatment was instituted by an orthopedic surgeon whom she had been referred to. The patient experienced only a slight improvement in bone pains with this treatment and also developed nausea, vomiting and anorexia. Subsequently, she sought care at the National Institute of Diabetes and Endocrinology, Dow University Health Sciences, Karachi, Pakistan.\nAt presentation, the patient was well oriented and of functional class 3 (wheel chair bound, able to walk only with support). Her blood pressure was 110/70 mmHg. Neck examination revealed no mass or lymphadenopathy. She had a significant proximal myopathy as well as curved thighs. She had shortened fingers, and spinal scoliosis was evident. Severe generalized bone tenderness was elicited. There was no focal deficit. Laboratory investigations at this time showed a calcium level of 15.1 mg/dL{3.775 mmol/L}, (corrected for albumin of 3.6 mg/dL{36 g/L}); Vitamin D3 level of 33.92 ng/mL{84.664 nmol/L}; phosphorus 2.3 mg/dL {0.743 mmol/L}and alkaline phosphatase of 1298 IU/L {21.633 µkat/L}. Her 24 h urine calcium was 155 mg/day {3.875 mmol/day}, with urine calcium to creatinine ratio of 0.02. Her creatinine level was 1.3 mg/dL {114.92 µmol/L}(Table ). The estimated glomerular filtration rate (calculated through Cockcroft-Gault equation) was 50 mL/min {0.835 mL/second).\nFollowing these tests, the patient’s PTH level was ordered and determined to be 2105 pg/mL {2105 ng/L} [Table ]. Ultrasonography of the neck showed a solid hypo echoic, well-circumscribed mass lesion, measuring 1.8 × 1.2 cm at the lower pole of the right lobe of thyroid. There were no calcifications or lymphadenopathy. Appearances were suggestive of parathyroid adenoma. Both lobes of the thyroid appeared normal. A repeat planar sestamibi scan, (requested from a different institute in the city), revealed areas of tracer retention over upper and lower poles of the right lobe of thyroid. The intensity of retained tracer was more over the right inferior parathyroid gland. The findings were highly suggestive of hyperparathyroidism (Fig. ).\nA bone mineral density scan showed a T score of − 2.9 in the spine, − 3.8 in the hip and − 4.5 in the distal forearm, consistent with severe osteoporosis. The Z scores at the spine, hip and distal forearm were − 2.0, − 3.1 and − 3.6, respectively (Table ).\nUltrasonography of the kidneys revealed a single renal stone (0.6 cm) and no neprocalcinosis.\nBased on the biochemistry results of hypercalcemia, associated with elevated PTH levels, a diagnosis of primary hyperparathyroidism was made. Subsequent sestamibi scan and neck imaging facilitated us to localize the abnormal parathyroid gland. The DXA scan was useful for evaluation of the bone mineral density. In view of the phenomenally high levels of parathyroid hormone, (more than 10 times upper limit of normal), the pre-operative suspicion of parathyroid cancer was high [, ]. The patient was rehydrated with intravenous fluids. Subcutaneous calcitonin injections at a dose of 4 units/kg every 12 h were administered to tide her over until the surgery. Once her calcium levels had come down to 10.5 mg/dL {2.625 nmol/L}L, she was operated upon. At surgery, right hemithyroidectomy and inferior parathyroidectomy with level six lymph node resection was done. The lymphadenectomy was performed as there was evidence of enlarged lymph nodes at neck exploration. The size of the lesion was measured as 2.5 × 1.5 × 1 cm. Histopathology showed features consistent with parathyroid cancer (Fig. a–d). Capsular invasion and focal vascular invasion were noted. However, margins of excision were tumor free. The excised lymph nodes did not show evidence of tumour infiltration. The patient was not given external radiation therapy postoperatively. Literature review revealed that post operative adjuvant radiation therapy may only have a role in the management of patients with a histologically positive margin following en bloc resection, or in those with lymph node metastases [, , ].\nPostoperative PTH level, performed on the second day of surgery, was 59 pg/mL {59 ng/L} (16–87). On the third postoperative day, the patient’s serum corrected calcium declined to 6 mg/dL {1.5 mmol/L}. This was associated with paresthesias around her mouth and carpo-pedal spasm. There were no seizures, although there was some confusion in terms of time and place. Intravenous calcium (2 g calcium gluconate, equivalent to 180 mg elemental calcium, in 50 mL 5% dextrose water) was infused over 20 min. Re-monitoring of calcium levels revealed persistent hypocalcemia. A slow infusion of calcium was initiated at an initial rate of 50 mL/h. This was prepared by adding 100 mL of 10% calcium gluconate (equivalent to 900 mg elemental calcium) to 1000 mL 5% dextrose water. The infusion rate was adjusted, with a goal to maintain calcium levels at lower end of normal range. On the fifth post-operative day, the calcium level had risen to 9.0 mg/dL {2.25 nmol/L}. Neurologic examination was normal and she was tolerating oral diet. Oral calcium supplementation was initiated (Qalsan D four times daily-equivalent to 2 g elemental calcium per day). She was discharged on oral calcium and vitamin D supplementation with active vitamin D, (calcitriol) 0.25 µg twice daily, in a stable condition.\nAt follow-up, her appetite and mobility had improved significantly, although she continued to experience bone pains. Corrected calcium was 9.5 mg/dL {2.375 nmol/L}. A repeat skeletal scintigraphy done 3 months after parathyroidectomy did not demonstrate a significant change in the lytic lesions (Fig. a, b). A repeat DXA scan 2 years down the line revealed a significant improvement in bone mineral density at all sites, though more so at the spine and hip, than at the forearm (Table ). Thereafter, we followed her clinically, as she was not keen to have further radiologic testing done. We have been monitoring her calcium and PTH levels on an annual basis. They have remained within their normal range till date (2018). She is now functional class 2, (no longer wheel chair bound), and on regular calcium and vitamin D supplements (patient perspective, attached as Additional file ).
|
[[53.0, 'year']]
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F
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|
{'8719313-1': 1, '3462668-1': 1}
|
2,280 |
6115318-1
| 30,159,830 |
comm/PMC006xxxxxx/PMC6115318.xml
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A neuroendocrine carcinoma with a well-differentiated adenocarcinoma component arising in Barrett’s esophagus: a case report and literature review
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A 71-year-old man was admitted to our hospital because of the detection of an esophagogastric (EG) junction tumor on regular upper endoscopy screening. He had no symptoms, such as dysphagia, epigastric fullness, and gastroesophageal reflux. His medical history included hepatolithiasis, and he had undergone hepatic left lateral segmentectomy at 50 years of age. Physical examination showed no remarkable findings, and laboratory examinations, including assessment of serum tumor markers, such as carcinoembryonic antigen and carbohydrate antigen 19-9, were normal. Endoscopy revealed a sliding hiatal hernia and an approximately 10 mm elevated mass at the EG junction (Fig. ). Endoscopic ultrasonography showed a mass having mixed echogenicity in the esophageal wall, with partial invasion of the submucosal layer (Fig. ). Upper gastrointestinal imaging showed an elevated lesion at the EG junction (Fig. ). A biopsy specimen was obtained, and the pathological diagnosis on analysis of the specimen was a differentiated tubular adenocarcinoma. Computed tomography did not indicate lymph node metastasis or distant metastasis. The clinical diagnosis was esophageal cancer (cT1bN0M0 cStage I according to the eighth edition of the Union for International Cancer Control classification) []. Proximal gastrectomy with D1 lymph node dissection was performed along with jejunal interposition.\nMacroscopically, the surgical specimen showed an elevated mass (10 × 8 mm) in the EG junction (Fig. ). Microscopic examination revealed a carcinoma associated with BE. The carcinoma, Barrett’s epithelium, and stratified squamous epithelium are indicated in Fig. . Hematoxylin-eosin staining showed that the tumor was composed of small-to-intermediate cells with scant cytoplasm and irregular hyperchromatic nuclei and was growing with nuclear palisading and tubular structures. A well-differentiated adenocarcinoma component was present independently. The neoplasm arose in Barrett’s epithelium (Fig. and ). Infiltration of the submucosal layer to a depth of < 200 μm was noted. Lymphovascular invasion was not identified. The margins of the specimen were free of tumor cells. On immunohistochemical staining, the tumor was positive for chromogranin A and synaptophysin (Fig. and ). Ki-67 was positive in 40% of the tumor cells. Thus, the histological diagnosis was an NEC with a well-differentiated adenocarcinoma component arising in BE. No metastasis in the lymph nodes was noted on histological examination. The pathological diagnosis was esophageal cancer (pT1bN0M0 pStage I). The resection margins were free of tumor cells (R0 resection). The patient’s postoperative course was good, and he was discharged on the twentieth postoperative day. He has remained free of the disease at 36 months postoperatively.
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[[71.0, 'year']]
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M
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{'12507278': 1, '10705396': 1, '15534932': 1, '16887481': 1, '23426118': 1, '21573743': 1, '18670347': 1, '10945905': 1, '18008084': 1, '7832278': 1, '28288180': 1, '32519961': 1, '9077732': 1, '27796514': 1, '10029452': 1, '14652811': 1, '1590313': 1, '20169569': 1, '3002582': 1, '23089450': 1, '27293844': 1, '19850156': 1, '10539923': 1, '18264827': 1, '6327495': 1, '31852479': 1, '18211708': 2, '30159830': 2}
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{'2263060-1': 1}
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2,281 |
6115321-1
| 30,159,641 |
comm/PMC006xxxxxx/PMC6115321.xml
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Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
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A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus (HBV) infection was referred as a candidate for LDLT. She had been diagnosed with hepatitis B 20 years before, but it had not been actively treated. She had received best supportive care, but she and her family chose to proceed with LDLT. Laboratory findings before LDLT were as follows: serum total bilirubin, 8.4 mg/dL; serum albumin, 2.5 g/dL; prothrombin time, 40%; platelet count, 84,000/μL; and Model for End-stage Liver Disease score, 17. A large amount of ascites, liver atrophy, and collaterals were observed on computed tomography scan. At the time of admission, her urine volume was decreased to 50 mL/day, and continuous hemodiafiltration treatment was started for renal failure. The predictive risk score [] was 0.80, which was lower than the score of 1.3 which predicts a poor prognosis, and the risk of postoperative mortality was therefore expected to be high. After obtaining full informed consent from both the donor and the recipient and approval from the Liver Transplantation Committee of Kyushu University, the patient was prepared for LDLT using a right posterior section graft.\nThe donor was the patient’s husband, who was 63 years old and had an identical blood type B. The surgical techniques were carried out as described previously []. The graft weight was 581 g, which was equivalent to 56.8% of the recipient’s standard liver volume (graft–recipient weight ratio, 1.12%). The hepatic arterial flow in the RHA was 87 mL/min, and the portal venous flow was 510 mL/min after reperfusion. The portal system pressure was 18 mmHg at the end of surgery, and splenectomy was not performed. The anhepatic time, and cold and warm ischemic times were 158 min, 92 min, and 49 min, respectively. The surgical time was 10 h, and the estimated blood loss was 4440 g. Ten units of red blood cells, 16 units of frozen plasma, and 40 units of platelets were transfused during surgery.\nThe post-transplant course is shown in Fig. . The patient received post-transplant immunosuppression with tacrolimus, steroid tapering, and mycophenolate mofetil. On postoperative day (POD) 7, the patient developed SFSS with serum total bilirubin 20.0 mg/dL and abdominal ascites 2000 mL/day [], indicating prolonged cholestasis and intractable ascites []. Tests for HBV DNA and cytomegalovirus (CMV) were negative, and repeated blood cultures were also negative until POD 24. There were no signs of abnormal hepatic flow or surgical complications detected by daily Doppler echo until POD 14 and by routine computed tomography scan on POD 7. There was also no notable elevation of hepatobiliary enzymes, thus ruling out the possibility of graft rejection.\nThe patient developed a fever above 39 °C on POD 9, and laboratory findings showed a white blood cell (WBC) count of 1120/μL and a platelet count of 40,000/μL, which fell to 300/μL (neutrophils, 30/μL) and 25,000/μL, respectively, on POD 11. She was treated with a combination of G-CSF and intravenous immunoglobulin, but her leukocytopenia/neutropenia failed to improve. Bone marrow aspiration was performed on POD 12, and histology revealed many macrophages and phagocytosis of hematopoietic cells (Fig. ). The patient was therefore diagnosed with HPS following LDLT. Steroid pulse therapy with 1000 mg/day methylprednisolone was initiated on the same day and continued for 3 days. Her WBC count increased to 4290/μL on POD 15, suggesting an improvement in her peripheral blood leukocytes. However, the patient developed sepsis with a fever above 39 °C on POD 24, and blood cultures were positive for Enterococcus faecium and Escherichia coli infections. She recovered rapidly with empiric antibiotic therapy including carbapenem and vancomycin, but her graft dysfunction was prolonged with a consistent serum bilirubin value of around 30 mg/dL, and her renal failure never improved. She also had several episodes of bacterial pneumonia. Splenic artery embolism was performed on POD 84 but failed to improve her graft dysfunction. Regarding immunosuppressive treatment to control infections, mycophenolate mofetil was stopped on POD 8 and not restarted, and the minimum dose of tacrolimus was administered to maintain a trough blood concentration of 3–5 ng/mL. The patient underwent a tracheotomy, and her liver function was expected to improve with long-term management; however, she developed bacterial sepsis and died of liver failure on POD 146.\nHPS, also referred to as hemophagocytic lymphohistiocytosis, is a rare and often fatal disease despite treatment [], characterized by a variety of symptoms including fever, lymphadenopathy, hepatosplenomegaly, jaundice, and skin rash []. We report a rare case of a 63-year-old woman who developed HPS 2 weeks after LDLT with a right posterior section graft, who was treated with steroid pulse therapy following an early diagnosis via biopsy. This case may help to shed light on the relationship between HPS and graft dysfunction, including prolonged SFSS.\nHPS is defined as a proliferation of phagocytic macrophages in the bone marrow, spleen, or lymph nodes, with clinical findings including fever for ≥ 7 days with peaks ≥ 38.5 °C, cytopenia (at least two of three lineages), and splenomegaly []. However, this definition is based on infant HPS and may be difficult to apply in adults and patients with various basic diseases such as liver cirrhosis or after LDLT. It is therefore necessary to perform bone marrow aspiration immediately to reach an early diagnosis of HPS in these patients []. The laboratory findings in the current patient showed WBC counts of 1120/μL on POD 9 and 300/μL on POD 11. Apart from HPS, other differential diagnoses such as medicine-induced disease, infections such as sepsis, hypersplenism, leukemia, and lack of vitamin B12 were unlikely and bone marrow aspiration on POD 12 showed typical HPS characteristics of phagocytic hematopoietic cells. This rapid diagnosis allowed early treatment with steroid pulse therapy, which contributed to an improvement in the patient’s peripheral blood leukocytes.\nHPS is a rare complication after liver transplantation with a prognosis for patient survival of 50% [, ]. Fifteen patients, including the present case, have been reported to date (Table ) [, , –], only four of whom survived. About a third of secondary HPS cases are associated with virus infection, half with lymphoma, and the remainder with bacterial and fungal infections. Given that HBV DNA and CMV tests on POD 7 were negative and blood cultures were also negative until POD 24, it was considered highly unlikely that the secondary HPS in the current patient was associated with a viral or bacterial infection. The combination of anemia and thrombopenia also added the possibly of thrombotic microangiopathy as a differential diagnosis, but no typical hemolytic anemia was detected in the bone marrow biopsy and there was no sign of encephalopathy. Thrombotic microangiopathy was thus discounted as a possible cause of HPS, and leukopenia was therefore considered the most likely cause. However, liver biopsy after LDLT and measurement of ADAMTS-13 were not performed in the present patient.\nIn general, patients may develop secondary HPS via hypercytokinemia [] due to systemic inflammatory response syndrome associated with a viral or bacterial infection. The current LDLT recipient had SFSS, which might have been associated with the donor’s older age (63 years) []. SFSS can also induce oxidative stress and hypercytokinemia [].\nBased on this hypercytokinemia theory, 10 of the past 15 cases (66.7%) were treated with steroids, 10 (66.7%) with intravenous immunoglobulin, and four (26.7%) with plasma exchange. The present patient started combination therapy with intravenous immunoglobulin, G-CSF, calcineurin inhibitor conversion, and steroid pulse therapy on POD 12, with subsequent improvement in her peripheral blood leukemia. However, her course was complicated by bacterial sepsis on POD 24. The causal relationship between steroid pulse therapy and sepsis could not be clarified, but the possibility of a septic attack is always present.\nIn addition, the relationship between SFSS and HPS was not unknown in the present case, and the possible causative effect of SPSS remains speculative. To reduce the risk of HPS, it is necessary to avoid developing viral and bacterial infections and hypercytokinemia, which induce secondary HPS, while the use of elderly donors might also increase the risk of SFSS. However, it may not be possible to avoid these factors in the setting of LDLT. Further studies on the mechanism of hypercytokinemia-induced HPS and more basic studies are needed to confirm the optimal treatment for HPS.
|
[[63.0, 'year']]
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F
|
{'15052381': 1, '12826200': 1, '15966233': 1, '22494784': 1, '16737484': 1, '22955229': 1, '16937360': 1, '28866302': 1, '18581462': 1, '24114830': 1, '19100492': 1, '24890095': 1, '16797322': 1, '18613366': 1, '19843295': 1, '21969156': 1, '11740400': 1, '15468305': 1, '16611337': 1, '28944163': 1, '30159641': 2}
|
{}
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2,282 |
6115322-1
| 30,159,613 |
comm/PMC006xxxxxx/PMC6115322.xml
|
Complete remission of advanced hepatocellular carcinoma following transient chemoembolization and portal vein ligation
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A 54-year-old man with a history of diabetes mellitus and hypertension was admitted to a regional hospital because of high fever and right hypochondriac pain. Hepatitis B virus surface antigen and hepatitis C virus antibody were both found to be negative, but he showed evidence of an excessive inflammatory reaction. A diagnosis of liver abscess was carried out that was managed by immediately performing a percutaneous puncture with drainage. Laboratory evaluation (Table ) found poor liver function and very high levels of alpha-fetoprotein (AFP, 45,928 ng/ml; normal, ≤ 20 ng/ml), protein induced by vitamin K absence or antagonist-II (PIVKA-II, 125,350 mAU/ml; normal, ≤ 40 mAU/ml), and AFP-L3 (38.3%, normal, ≤ 10%). The patient was diagnosed with HCC and with the triple-positive tumor marker status indicating highly malignant disease [, ]. The patient was also found to have a portal vein tumor thrombosis in the right posterior branch of the portal vein (Fig. ). Although a right hepatectomy was indicated for curative resection, residual liver function of the remnant volume was estimated to be insufficient [, ].\nThe patient was initially treated with chemoembolization (Table ) using a HAIC of cisplatin (50 mg/100 ml/10 min) and 5-FU (1000 mg/100 ml/10 min), followed by cisplatin (50 mg) suspended in lipiodol (5 ml) and starch microspheres (300 mg) containing mitomycin C (4 mg) [, ]. After the first round of chemoembolization, examination showed incomplete lipiodol accumulation within the tumor. Additionally, as the PVTT progressed to the right main portal vein, surgical PVL was performed to avoid involvement of the left portal vein. Three disseminated peritoneally nodules were also removed. Three additional rounds of transient chemoembolization were performed after the initial surgical procedure.\nAt the time of the fourth chemoembolization, the tumors responded to the treatment and markedly reduced in size without enhancement (Fig. ). Further, no new tumors were found in the liver, and the tumor markers returned to their normal levels (Fig. ). A suspicious lesion (2 cm in diameter) recurred at15 months after the initial treatment, which was treated with percutaneous radiofrequency ablation. The patient is alive at 2-year post-procedure and shows complete remission, as defined by the modified response evaluation criteria in solid tumor criteria.\nThis patient achieved complete remission after chemoembolization, surgical PVL, and extirpation of peritoneally disseminated nodules. The case was complicated by the poor prognostic factors, including the macroscopic diffuse-type classification, a macroscopic PVTT, the peritoneal dissemination, and triple-positive tumor marker status [–]. A tumor biopsy was not performed, but the presence of a poorly differentiated HCC was strongly suggested by the tumor marker status and diagnostic imaging [–].\nIn patients with HCC and macroscopic PVTT, multidisciplinary treatment, including liver resection, provides an excellent prognosis []. Moreover, a recent nationwide survey in Japan indicated that liver resection was more effective than non-surgical treatment in cases with a PVTT that is limited to the first- or second-order branches []. Multiple measurements of the liver function and functional liver volume after PVL [, , ] in our patient indicated that liver resection was not a viable option. For such HCC patients, other treatment options such as HAIC with chemoembolization and sorafenib also result in poor median survival times of 3.5–10.2 and 8.1–8.9 months, respectively [, ]. However, right portal vein occlusion can prevent both progression of the right PVTT into the left or main portal vein and intrahepatic metastasis into the left liver [, , ], and it may also enhance the effectiveness of HAIC because capsular invasion and satellite nodules could be supplied by the portal vein with hepatic artery [, ]. While formulating the treatment strategy, we also considered the fact that PVE is not indicated in patients with a PVTT that is in close proximity to the bifurcation.\nPeritoneal dissemination of HCC can occur after tumor rupture or due to therapeutic interventions. The standard treatment for dissemination of HCC would be systemic chemotherapy, and if dissemination is localized to abdominal cavity or abdominal wall, then the surgical removal for dissemination of HCC might be a challenging option [, ]. In this patient, iatrogenic seeding may have occurred by tumor puncture when drainage was started. However, the spread was limited, and all lesions could be isolated and surgically removed.\nOur patient was treated by HAIC followed by transient chemoembolization. Cisplatin and 5-FU are effective for HCC, evidently in intra-arterial infusion [, ]. In fact, some patients with advanced HCC and PVTT have reportedly shown complete clinical remission or pathological response after this regimen [–]. Cisplatin modulates 5-FU activity, and the two drugs seem to have a synergistic effect. Further, as cisplatin infused via the hepatic artery is not trapped in the liver parenchyma, it would also be effective as systemic chemotherapy. Essentially, cisplatin suspended in lipiodol is a highly effective embolic material that is also used in HCC treatment [, , ]. Mitomycin-C and degradable starch microspheres provide temporary occlusion, which may also increase drug concentration [].\nSorafenib is effective in HCC patients with macroscopic vascular invasions, extrahepatic spread, or both, but a recent trial has reported a response rate of 2% and a median survival time of only 10.7 months []. However, a few cases of complete remission after sorafenib therapy have been reported [, ]. In our patient, dynamic imaging detected no viable HCC and persisting normalization of the three tumor markers. Previous reports suggest that HAIC with a cisplatin–lipiodol suspension combined with 5-FU can lead to better response rates and overall survival rates (without extrahepatic metastasis) compared to only sorafenib in patients with advanced HCC and PVTT []. Thus, it would be possible to administer additional chemoembolization or radiofrequency ablation for intrahepatic recurrence and sorafenib therapy for extrahepatic metastasis. It has similarly been reported that sorafenib is effective in patients with HCC refractory to chemoembolization [] and that sorafenib and HAIC with cisplatin may have synergistic effects [].\nThe maintenance of liver function is the key to achieving longer survival in advanced HCC patients, and it is known that effective treatment for advanced HCC can improve liver function []. Further, it has been reported that a Child–Pugh score of ≤ 7 shows a better response to HAIC with better prognosis compared with Child–Pugh score of 8 or 9 []. However, our patient had a Child–Pugh score of 8 at admission, which improved to 6 after multidisciplinary treatment, indicating that the treatment regimen was effective.
|
[[54.0, 'year']]
|
M
|
{'27266618': 1, '23957810': 1, '26754678': 1, '23793266': 1, '23591833': 1, '22244181': 1, '21523415': 1, '12572874': 1, '12417507': 1, '24245496': 1, '22727733': 1, '1657361': 1, '23435678': 1, '20616599': 1, '25704417': 1, '27573564': 1, '15305412': 1, '19095497': 1, '8063553': 1, '18772170': 1, '15150897': 1, '27549776': 1, '18650514': 1, '9731568': 1, '21263091': 1, '27315783': 1, '21606837': 1, '27107976': 1, '34095718': 1, '25482462': 1, '20559534': 1, '24686196': 1, '22353520': 1, '29285366': 1, '23020312': 1, '16565970': 1, '10982622': 1, '30159613': 2}
|
{}
|
2,283 |
6115325-1
| 30,159,818 |
comm/PMC006xxxxxx/PMC6115325.xml
|
Mitral valve nonbacterial thrombotic endocarditis: a rare multi-surgery-tolerant survivor of Trousseau’s syndrome
|
A 69-year-old male was admitted to a hospital in June 2016 because of right arm asthenia and dysarthria and was diagnosed as having cerebral infarction in the left middle cerebral artery area along with deep vein thrombosis. ECG demonstrated normal sinus rhythm and echocardiography revealed no intra-cardiac thrombus or vegetation. The patient was discharged from the hospital following administration of apixaban.\nIn August 2016, the patient was readmitted to the hospital because of recurrent right arm asthenia and dysarthria. MRI revealed multiple cerebral infarctions in not only the bilateral cerebral hemispheres but also the cerebellum. Trousseau’s syndrome was suspected at this time. Apixaban administration was stopped and an intravenous drip of heparin was started. Echocardiography revealed mild mitral regurgitation with vegetation on the mitral valve. Although the laboratory data suggested no evidence of infection, ceftriaxone and gentamicin were added as a precaution against infective endocarditis. The patient was then referred to our hospital for surgery.\nA CT scan revealed a left renal infarction and multiple swollen lymph nodes around both the abdominal aorta and stomach with antral hypertrophy, suggesting an advanced gastric cancer or lymphoma. As the vegetation showed no change despite the heparin and antibiotics therapy, cardiac surgery was performed on day 5 after referral. Extracorporeal circulation was instituted employing aortic and bicaval cannulation. After aortic cross-clamping, the mitral valve was exposed via a left atriotomy. Both mitral leaflets had vegetation on the surface, and major vegetation 15 mm in width was evident on the anterior leaflet (Fig. ). These were resected in their entirety and replaced with a 25-mm Epic bioprosthesis (Abbott). Continuous intravenous heparin administration was resumed on the following day, aiming for an activated partial thromboplastin time of between 40 and 50 s. Histologic analysis revealed that the vegetations were thrombi covered with vascular endothelium and that the mitral leaflet tissue was not damaged (Fig. ). On the basis of these findings, the patient was diagnosed as having NBTE.\nAn endoscopic stomach biopsy was performed on the seventh postoperative day, and histologic analysis revealed non-solid poorly differentiated adenocarcinoma with components of signet-ring cell carcinoma and moderately differentiated tubular adenocarcinoma. The patient was definitively diagnosed as having Trousseau’s syndrome and, subsequently, transferred to the department of surgery. A Billroth I distal gastrectomy was performed, and a continuous intravenous heparin drip was employed during the operation. Histologic analysis revealed poorly differentiated adenocarcinoma with a component of moderately differentiated tubular adenocarcinoma and metastatic tumor cells in the dissected lymph nodes (T4aN3bM0; stage IIIb). Further histologic analysis using alcian blue staining confirmed the presence of mucin in the tumor.\nSubcutaneous heparin injection was introduced on day 8 after the gastric surgery, and the patient was discharged from our hospital in October after acquisition of the self-injection technique. During this long hospitalization, no thromboembolic events were observed. Chemotherapy was started in November. The patient has survived for 18 months after the diagnosis of Trousseau’s syndrome without any recurrence of thromboembolism.
|
[[69.0, 'year']]
|
M
|
{'661751': 1, '19154931': 1, '17496204': 1, '12975470': 1, '15184609': 1, '32307602': 2, '4050830': 1, '28477331': 2, '17881239': 1, '19285272': 1, '26320109': 1, '17522239': 1, '24560251': 1, '30159818': 2}
|
{'7167392-1': 1, '5419953-1': 1}
|
2,284 |
6115549-1
| 30,190,775 |
comm/PMC006xxxxxx/PMC6115549.xml
|
Fournier gangrene due to Rhizobium Radiobacter
|
A 47-year-old man presented with a 10-day history of right scrotal pain, swelling and erythema. He had malodorous drainage from right scrotum for two days. He had no diabetes mellitus (DM), hypertension or any other co-morbid diseases; also there were no any risk factors including drug-use, immunodeficiency, genito-urinary or anorectal trauma and infection in his medical history. Massive edema in both of two hemiscrotum and black necrotizing area with malodorous pus drainage in the bottom of the right hemiscrotum was detected in his physical examination (). His anorectal examination was normal. Laboratory analysis revealed as a serum creatinin 0.9 mg/dl, hemoglobin 14.9 g/dl, glucose 486 mg/dL, CRP 156 mg/L, WBC14.5x106 cells/mL, sodium 132 mmol/L. There was no infection sign in bilateral testis, but their sizes were found smaller in scrotal ultrasound (right testis 25x20 mm, left testis 20x20 mm). Intravenous crystallized insulin therapy was given for decreasing serum glucose levels and intravenous imipenem 4x500 mg and Clindamycin 4x600 mg were started prophylactically to the patient according to the infectious disease consultation. All necrotizing tissues were debrided in right scrotum. Right hemiscrotectomy was performed and right testis had a normal blood supply appearance in operation (). Open wound dressing with the nitrofurazone and rifamycin was performed in first three days after operation. The vacuum-assisted closure technique (VAC) (a technique that keeps the wound environment under sterile condition and decreases the frequency of changing protective covers of wound) was performed to the patient in postoperative 3rd day to postoperative 15th day. VAC dressing makes its function by creating mechanical stress with negative pressure of the vacuum system; wound edges diminish, granulation formation accelerates, cellular proliferation and neoangiogenesis increases.\nPathological result revealed as Fournier gangrene and Rhizobium radiobacter was isolated from both of the tissue and abscess cultures. Rhizobium radiobacter was only resistant to trimethoprim sulfamethoxazole and sensitive to ceftazidime, cefepime, imipenem, meropenem, ertapenem, amikacin, gentamycin and tetracycline; therefore same antibiotics were continued to the patient. Control tissue culture was done at postoperative 13th day and by the results revealed as negative wound site was sutured primarily at postoperative 15th day. Patient was discharged at postoperative 18th day with the suggestion of endocrinology consultation for a new diagnosed diabetes mellitus. Patient had come to third month control, he had no symptoms and his scrotum was normal.
|
[[47.0, 'year']]
|
M
|
{'23578806': 1, '18289729': 1, '26557521': 1, '25503448': 1, '21421546': 1, '12904441': 1, '20451745': 1, '22259203': 1, '31934634': 2, '30190775': 2}
|
{'6947763-1': 1}
|
2,285 |
6115556-1
| 30,190,774 |
comm/PMC006xxxxxx/PMC6115556.xml
|
An elusive case of digital ischemia in a patient with Rheumatoid Arthritis
|
We report a case of 38 year old lady, diagnosed case of Rheumatoid arthritis (RA) for two years. Apart from the symptoms of joint pains, there was history of Raynaud’s phenomenon as well. There were no systemic complaints and she remained well on treatment for RA, i.e. Hydroxychloroquin, Leflonamide and NSAID as per need. She presented to us, for the first time in June 2016, with complaints of blackish discoloration of right big toe and left 4th toe for 1.5 years. She initially developed a small ulcerated lesion over one of her toes 1.5 year back, that was attributed to RA associated vasculitis and her treatment was modified in accordance with the suspected diagnosis, which included Aspirin, Nefidipine, and Prednisolone. There was no complaint of pain, itching or temperature change at that time and she remained static until a week prior to her presentation to us. This time she had moderate intensity pain in her toes, specifically right big toe and left 4th toe which turned blackish in color. () She was admitted in ward on this occasion. Examination showed a young lady, BMI 21 Kg/m2, Pulse- 90bpm, BP - 150/80 mmHg, temperature – 99°F, RR- 18bpm. There was mild pallor, but no jaundice, rash or joint deformity. There was blackish discoloration of her big toe with partially healed necrotic ulcer on top of it, while the toe of left foot showed blackish discoloration but no ulceration. Both were tender to touch. Peripheral pulses were palpable in both upper limbs and lower limbs, although the posterior tibial and dorsalis pedis arteries had low volume. There was no temperature change and sensations were intact. Her systemic examination was entirely unremarkable. DAS-28 was calculated which was 1.8, i.e. remission. Initial labs are shown in . Doppler ultrasound of legs showed reduced blood flow in left posterior tibial and dorsalis pedis arteries with reduced peak systolic velocities. Due to her persistently increased platelets further work-up was done to evaluate the cause of thrombocytosis. JAK2 V617 mutation analysis was done, which turned out to be positive. Furthermore her bone marrow trephine biopsy was done which showed clusters of mature megakaryocytes with multi-lobulated staghorn nucleoli, suggestive of Essential thrombocytosis. Hence, this patient was diagnosed as a case of RA with associated ET. Treatment was modified in accordance with the revised diagnosis, which included Aspirin 75mg and Hydroxyurea 1g per day, in addition to her RA treatment. Patient responded well to the treatment with regard to clinical as well as laboratory parameters and is on regular follow-up visits, however, she developed gangrene of toe, for which amputation of distal phalanx of toe and nail excision was done, later in the disease course. Anagleride was started with the given treatment due to a surge in her platelet counts. Her follow-up platelet counts are shown in .
|
[[38.0, 'year']]
|
F
|
{'26604428': 1, '23410586': 1, '9324011': 1, '21243496': 1, '17210076': 1, '16508929': 1, '19259097': 1, '20384432': 1, '11703328': 1, '26538823': 1, '30190774': 2}
|
{}
|
2,286 |
6115580-1
| 30,190,776 |
comm/PMC006xxxxxx/PMC6115580.xml
|
Germ cell tumor and Takotsubo Cardiomyopathy: A treatment dilemma
|
A 32 years old male presented with painless and gradually enlarging right testicular swelling of 3 months duration. Right inguinal orchiectomy was performed and 3 weeks post Op B-hCG was 2900IU, AFP was 890 ng/ml while LDH was 560mg/ml. Histopathology showed yolk sac tumor (65%), immature teratoma (20%) and seminoma (15%). Baseline CT scan showed a 1.8cm right para-aortic lymph node with no other metastatic disease. Patient was staged as Stage IIA-S1, Good risk Mixed Germ Cell Tumor (MGCT) depending upon the markers & CT findings. Chemotherapy was planned within 3 weeks of surgery; however patient presented after 2nd week of orchiectomy to ER with severe chest pain. At presentation, cardiac enzymes were elevated and his Trop-I was 6.2 (normal < 0.4), while EKG showed 1.8mm ST segment elevation in leads V1-V3. Cardiology team was consulted immediately & echocardiogram was performed which showed apical hypokinesis with EF of 49%. His cardiac catheterization was reported to be normal with normal coronaries and without any flow restriction (). Depending upon the presentation, EKG changes, elevated Trop-I with absence of any flow restriction and normal coronary angiogram, TCM diagnosis was established, based upon Mayo clinic diagnostic criteria (). Patient was started on angiotensin converting enzyme inhibitors and beta-blockers, and his chemotherapy was delayed by 3 weeks.\nLater his chemotherapy regimen was modified to EC x 4 (Etoposide 100 mg/m2IV Day 1-5 / Carboplatin AUC-5 IV Day 1 only) instead of EP (Etoposide / Cisplatin). Though there is scarce data regarding the use of Carboplatin in MGCT and Cisplatin has been shown to be superior to Carboplatin, however due to a risk of Cisplatin induced vasculitis or acute thrombosis, the treatment regimen was modified with informed consent. Patient was treated with 4 cycles of EC and after 2nd cycle his markers normalized. At this time decision was reviewed to cut down his chemotherapy to only 3 cycles of EC but it was considered that he is already getting sub-optimal regimen, so he was continued with complete 4 cycles. He tolerated his treatment well, achieved complete biochemical and radiological response and placed on surveillance. Patient has completed 4.6 years of follow-up according to National Comprehensive Cancer Network guidelines and remains well without any evidence of relapse. After 6 months of his treatment with ACEi and BBs, both were also stopped as he had normal EF (62%) on echocardiogram.
|
[[32.0, 'year']]
|
M
|
{'8386751': 1, '20582391': 1, '17573507': 1, '19627433': 1, '15703419': 1, '23548823': 1, '9164194': 1, '22732314': 1, '9215824': 1, '25024068': 1, '29096885': 1, '30190776': 2}
|
{}
|
2,287 |
6116288-1
| 30,181,838 |
comm/PMC006xxxxxx/PMC6116288.xml
|
Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery
|
A 28-year-old Thai male with past medical history of E-beta-thalassemia and splenectomy presented to emergency department with a 3-month progressive tightness in bilateral flank region, weakness, and difficulty walking requiring crutches. Review of system was also significant for unintentional 10-pound weight loss due to poor appetite. Further review of history revealed that he was diagnosed with E-beta-thalassemia at the age of 13 months old. The condition was managed with intermittent transfusion, deferoxamine for iron overload, and hydroxyurea until he turned 21 years old when he stopped following up with his hematologist. He reported that, since then, his baseline hemoglobin was 6 g/dL. Vital signs at admission were within normal limits. Physical exam was significant for frontal bossing with depression of nasal bridge, bilateral costovertebral angle tenderness, slow broad-based gait with ambulation, decreased light touch sensation of the thorax at the level T7-T10, at left medial thigh, and in bilateral lower extremities below the knees. Further neurological exam revealed knee and Achilles hyperreflexia in addition to positive bilateral Babinski, clonus, and Romberg.\nLaboratory studies were significant for leukocytosis of 72.5 × 109/L, hemoglobin 6.8 g/dL, platelet 732 × 109/L, and reticulocyte count 44.59%. Hemoglobin electrophoresis showed fetal hemoglobin of 49% and hemoglobin E of 59%. Total bilirubin was elevated at 5.3 mg/dL; the rest of the comprehensive metabolic panel was otherwise unremarkable. Further investigations showed that zinc, copper, folate, and vitamin B12 levels were within normal limits. Two-view chest, kidney/ureter/bladder, thoracic, lumbar, and pelvic spine X-rays showed prominence of ribs anteriorly, hepatomegaly, right paraspinal soft tissue prominence surrounding mid-thoracic spines, degenerative joint disease with osteopenia. Magnetic resonance imaging (MRI) of the brain with/without contrast did not show any acute findings but did demonstrate diffuse enhancement involving the clivus and medial occipital bone with mass extension into the bilateral maxillary/sphenoid sinuses, posterior aspect of ethmoid cells, bilateral maxillary bones, inferolateral orbits, and parietal calvarium. MRI of the thoracic spine with/without contrast showed extension of enhancing masses into the spinal canal at the levels of T2-T12 (). MRI of the lumbar spine with/without contrast showed prominent medullary expansion of the visualized bony pelvis with associated extension of enhancing mass into the lumbar spinal canal at L5 level causing severe central spinal stenosis and neural foramen narrowing at levels of L5-S1 (). These masses were associated with intermediate increased T1 and T2 signal mass-like abnormality suggestive of EMH.\nGiven the history of untreated chronic severe anemia in the past 8 years, the current physical exam, abnormal laboratory findings, and imaging evidence of paraspinal masses, it was concluded that the masses were extramedullary hematopoietic elements without the need of biopsy risking excessive bleeding. Given the extent and the chronicity of cord compression and severe anemia, the neurosurgical team agreed with hematology to initiate medical management rather than surgical intervention or radiation. Therefore, packed red blood cell (PRBC) transfusion was initiated to gradually increase baseline hemoglobin to above 10 g/dL along with hydroxyurea 15 mg/kg/day. Hydroxyurea dose then gradually was increased to 2000 mg daily to achieve maximum possible hematopoiesis suppression. Surprisingly, 2 days later, the patient regained gross sensation in his lower extremities. After 1 week of hospitalization, gait was noted to be increasingly sturdy, clonus and Romberg improved, and gross sensation of the thoracic area returned. The patient subsequently graduated from physical therapy. By hospitalization day-14 when he was discharged, the patient received a total of 6 units of PRBC. Hydroxyurea was maintained at 2000 mg daily without causing neutropenia or thrombocytopenia. MRI of the thoracic/lumbar spine repeated 2 weeks after the initial imaging showed significant reduction in mass effect on spinal cord and decreased spinal canal stenosis ( and ). After discharge, he was maintained on hydroxyurea 2000 mg daily with blood transfusion to maintain hemoglobin above 10 g/dL. Seven weeks after discharge, neurological symptoms were completely resolved except for patellar hyperreflexia. At this point, hydroxyurea was discontinued due to recurrent pancytopenia. Nine weeks after discharge, repeat MRI of the thoracic/lumbar spines showed no cord compression or spinal stenosis ( and ).
|
[[28.0, 'year']]
|
M
|
{'20204423': 1, '10901597': 1, '10870303': 1, '10844371': 1, '17405752': 1, '1385278': 1, '12224008': 1, '19046318': 1, '9883960': 1, '33102361': 2, '9633880': 1, '27462228': 2, '16462720': 1, '24717020': 1, '30181838': 2}
|
{'7567292-1': 1, '4939688-1': 1}
|
2,288 |
6116300-1
| 30,181,829 |
comm/PMC006xxxxxx/PMC6116300.xml
|
Brain metastasis in colorectal cancer presenting as refractory hypertension
|
Fifty-seven-year-old Caucasian female with a past medical history significant for hypertension and recently diagnosed stage IV moderately differentiated distal rectal adenocarcinoma with liver and lung metastasis status post second cycle of FOLFOX palliative chemotherapy 1 week ago was admitted with the primary complaint of hypertensive urgency with a severe headache, intractable nausea and vomiting, and diarrhea. At presentation, her blood pressure was 191/68. Examination did not show any focal neurological deficits. She was alert, awake, and oriented to time, place, and person; cranial nerves II–XII were intact; muscle power was five out of five bilaterally in upper and lower extremities; coordination was intact bilaterally; reflexes were 2+ bilaterally in upper and lower extremities; sensation was intact; and gait was normal. The case was discussed with oncologists who were of the view that symptoms may be due to hypertensive urgency versus BM (which are quite rare for CRC) or possible opiate withdrawal as the patient has been on high-dose opiates for her cancer-related pain. Computed tomography (CT) scan of the abdomen-pelvis did not show any evidence of bowel obstruction. Imaging of the brain would be considered if the patient did not improve with medical therapy. The patient was started initially on IV hydralazine but over the next 8 h patient blood pressure remained uncontrolled despite successive antihypertensives (IV labetalol, PO amlodipine, PO clonidine, IV enalaprilat, transdermal clonidine, IV metoprolol, and eventually IV nicardipine drip), ranging from 185/98 to 230/111. Brain imaging was ordered due to continuous severe headache and refractory hypertension. CT scan of the brain without contrast showed 3.3 × 2.3 × 2.8 cm hyperdense rounded mass in the region of the left cerebellum with surrounding vasogenic edema and a 5–6-mm shift of the posterior midline toward the right. Brain magnetic resonance imaging (MRI) with and without contrast showed left cerebellar lesion measuring 3.6 × 3.2 × 2.9 cm with high T1 signal intensity, low T2 signal intensity, and low gradient echo sequence signal intensity with peripheral and mild internal enhancement, and a significant amount of surrounding vasogenic edema, ipsilateral transtentorial herniation, and obliteration of the fourth ventricle (). After discussion with the patient, the decision was made for urgent neurosurgical resection of the mass and subsequent whole-brain radiation therapy (WBRT). This decision was made as this was a solitary lesion which was symptomatic with a high degree of mass effect and edema in a patient with a good baseline functional status. The patient was started on IV dexamethasone 6 mg every 6 h, and transferred for an urgent neurosurgical intervention at a tertiary care center. The tumor was resected using an operative microscope, and a combination of suction and cautery. Postoperatively patient recovered well from the surgery and was discharged. Patient followed up outpatient and had WBRT in 4 weeks post discharge. On her 1-year follow-up, patient reported no new neurological deficits, and her repeat MRI brain () did not show any recurrence of her metastatic lesion or new metastasis.
|
[[57.0, 'year']]
|
F
|
{'25220842': 1, '24621620': 1, '1637135': 1, '25905042': 1, '12774949': 1, '8414011': 1, '2405271': 1, '19801201': 1, '12062592': 1, '27037031': 1, '25491172': 1, '16757720': 1, '21109154': 1, '27603406': 1, '8498838': 1, '19957011': 1, '22012633': 1, '27447703': 1, '9809735': 1, '11578731': 1, '33669974': 1, '29333421': 1, '8839553': 1, '28245881': 1, '21319152': 1, '23144573': 1, '30181829': 2}
|
{}
|
2,289 |
6116378-1
| 30,157,954 |
comm/PMC006xxxxxx/PMC6116378.xml
|
An unusual cause of a breast mass in a 13-year-old girl: a case report
|
A 13-year-old girl of African ancestry was referred to our breast clinic for evaluation of a left breast mass. She had been complaining of the left breast lump for 2 years. The lump was gradually increasing in size and it was tender. There was no history of skin changes, nipple discharge, fever, or trauma. Furthermore, there was no family history of similar conditions, no history of traveling abroad, and no contact with a person with tuberculosis. Her medical history revealed history of rheumatic heart disease. She underwent mitral and tricuspid valve repair more than 2 years prior to presentation at our breast clinic. She was a student in primary school living with her parents and siblings.\nOn examination she was hemodynamically stable. She had a normal body build for her age. She was not pale or jaundiced. A breast examination revealed an irregular left breast mass that was palpable at six o’clock position. The mass was approximately 4 cm in maximal diameter; it was hard, tender, and fixed on the posteromedial side. There were no inflammatory skin changes or any nipple changes. Her right breast was unremarkable. There were no palpable bilateral axillary lymph nodes. Abdomen, chest, and neurological examinations were unremarkable. Her blood work, including complete blood count, liver function test, urea and electrolytes, and coagulation profile, was within normal ranges. Ultrasound of her left breast (Fig. ) showed a large, irregular, complex, heterogeneous mass measuring 4.3 × 2.7 × 3.5 cm at 6 o’clock position. There were central cystic changes but no significant intrinsic vascular flow. There was significant associated skin and subcutaneous edema and thickening with fluid seen tracking within subcutaneous tissue. The surrounding fat appeared more echogenic, consistent with the inflammatory and infectious changes seen in breast abscesses. Given the echogenicity of the mass, an infectious cause was suspected and malignancy was less likely but could not be excluded. An ultrasound-guided biopsy was recommended. A left axillary lymph node appeared prominent with a cortical thickness of 5 mm.\nAn ultrasound-guided aspiration of the cystic portion was attempted with an 18-gauge needle. Minimal yellowish fluid was retrieved and sent for aerobic bacteria, anaerobic bacteria, and fungi culture and sensitivity analysis. A biopsy of the mass was performed. The culture showed moderate growth of Staphylococcus aureus. An acid-fast bacilli stain was negative. Microscopic tuberculosis bacilli were not detected. Histopathology sections revealed cores of breast tissue heavily infiltrated with mixed acute and chronic inflammatory cells. The diagnosis was consistent with chronic abscess. She received one gram of amoxicillin and clavulanate potassium every 12 hours for 10 days. However, she remained symptomatic, and the mass did not decrease in size. Therefore, we proceeded to surgical excision.\nExcision of the breast lump through a peri-areolar incision was performed. During the operation, we found a circumscribed semi-cystic lesion that was not typical of fibroadenoma; the mass was fixed to the underlying rib. The cystic part was opened, and the turbid fluid that emerged was sent for culture. A long, thin, metallic wire was found inside the cavity; it emerged from a small opening above the rib and moved synchronously with her heartbeat (Fig. ).\nA cardiothoracic surgeon was consulted intraoperatively. The wire was removed; it was a retained TEPW that was inserted during the tricuspid and mitral valve repair procedure and had migrated to the breast. The mass was excised and sent for histopathology, which revealed mammary tissue with acute and chronic inflammatory cells.\nShe was discharged to home on the same day and was followed up at our out-patient clinic 2 weeks later. Her wound site was clean and dry. There were no palpable masses and no signs of inflammation. She was later seen at our out-patient clinic for a 1-year post-surgery follow-up. She had no complaints, and a breast examination revealed no palpable masses.
|
[[13.0, 'year']]
|
F
|
{'22963021': 1, '27684869': 1, '17948093': 1, '33025306': 2, '23152445': 1, '19448124': 1, '24034193': 1, '27716700': 1, '25958188': 1, '24872733': 1, '22962323': 1, '8347638': 1, '24872736': 1, '17066523': 2, '24438085': 1, '30157954': 2}
|
{'7538471-1': 1, '2687765-1': 1, '2687765-2': 1}
|
2,290 |
6116383-1
| 30,157,820 |
comm/PMC006xxxxxx/PMC6116383.xml
|
Gastric volvulus through Morgagni hernia and intestinal diverticulosis in an adult patient: a case report
|
A 30-year-old female presented with complaints of epigastric burning and indigestion for 1 year, which was occasionally associated with pain and vomiting. On a previous oesophago-gastroduodenoscopy, multiple oesophageal ulcers were noted, located from 30 to 35 cm and the mucosa was seen to be circumferentially hyperaemic. Upon investigation, chest and abdominal X-ray showed abnormal air-fluid level at right hemithorax as shown in Fig. . Computed Tomography (CT) scan demonstrated organo-axial gastric volvulus accompanied with right hemi-diaphragm elevation and a slight mediastinal shift to the left, with gastric bubble above the diaphragm (Fig. ), and sections through the lower chest showed mild bilateral pleural effusion with basal atelectasis of the right lower lobe. The small and large bowel loops were unremarkable and there was no evidence of bowel obstruction.\nAn elective laparotomy was performed through a midline incision. Stomach was not seen in the abdominal cavity, but pull on the gastrocolic ligament revealed the greater curvature of the stomach through foramen of Morgagni in the right hemi-diaphragm, with the defect measuring 4 × 5 cm (Fig. ). The hernia was reduced, with excision of the hernial sac and the defect was repaired using a size zero non-absorbable polypropylene suture—no mesh was placed. Since there was a high jejunal repair, a gastrostomy was created which served to secure the stomach in place. Following the reduction of hernia, on further exploration, multiple diverticuli were observed in the small and large intestine (Fig. ). Interestingly, these were unremarkable on CT scan. Only the largest and most proximal jejunal diverticulum (Fig. ), which was about 6 cm in size, was resected using a linear stapler as it had a narrow neck. A pelvic drain was placed and the wound was closed in layers using absorbable polyglactin suture. Figure exhibits the normal anatomy and a schematic diagram of this case depicting the presence of gastric volvulus through MH on the right side. The postoperative period was uneventful and the patient was discharged on the 10th post-operative day. The patient was stable and asymptomatic on follow up after one month, and she is doing well as of writing of this report. ‘Timeline for Case Report’ in Additional file represents the events of this case in a chronologic order.
|
[[30.0, 'year']]
|
F
|
{'25829669': 1, '21170239': 2, '23917752': 1, '24898515': 1, '15172245': 1, '9101170': 1, '2239876': 1, '17465510': 1, '31952551': 2, '23602148': 1, '4016626': 1, '17701234': 1, '17180292': 1, '12956800': 1, '25973243': 1, '30157820': 2}
|
{'6969475-1': 1, '2997277-1': 1}
|
2,291 |
6116405-1
| 30,186,637 |
comm/PMC006xxxxxx/PMC6116405.xml
|
Streptococcus pyogenes Pericarditis with Resultant Pulmonary Trunk Compression Secondary to Mycotic Pseudoaneurysm
|
An 18-year-old female with an allergy to penicillin and a past medical history of migraines presented to the emergency department with pleuritic chest pain and dyspnea on exertion. Three months prior she had an upper respiratory infection. Her initial workup was unrevealing, including negative troponin and normal complete blood count. Her electrocardiogram (ECG) revealed normal sinus rhythm. A computed tomography angiogram (CTA) chest had no significant findings. She was diagnosed with atypical chest pain and discharged home.\nShe returned three days later with worsening chest pain. ECG () was significant for sinus tachycardia, PR depressions, and diffuse ST elevations, consistent with pericarditis. Significant findings at that time included a Troponin-I of 0.28 ng/ml and white blood cell count (WBC) of 16.2 (16,200). Shortly after admission, she was transferred to the intensive care unit (ICU) for hypotension and tachycardia in the 150 s. Echocardiography demonstrated a moderate pericardial effusion with evidence of tamponade. Pericardiocentesis yielded 300 ml of serous fluid and established hemodynamic stability. She was initiated on empiric antibiotic therapy with vancomycin and meropenem. Later that same day, she underwent emergent intubation and vasopressor support after two separate episodes of ventricular fibrillation and pulseless electrical activity, requiring multiple rounds of advanced cardiac life support. After stabilization, fluid aspiration from the pericardial drain revealed 130 ml of purulent fluid. Her final pericardial fluid cultures and blood cultures grew Streptococcus pyogenes. Antibiotic coverage was weaned to intravenous cefazolin monotherapy. She continued to drain 240–360 milliliters of purulent pericardial fluid daily and was on norepinephrine for pressure support. Post resuscitation, she developed acute renal failure that required intermittent renal replacement therapy secondary to acute tubular necrosis. Antibiotic coverage was broadened to vancomycin and cefepime after the development of acute respiratory distress syndrome (ARDS) secondary to multifocal pneumonia. She was gradually weaned from the ventilator and subsequently extubated. The remaining hospital course was uneventful, and she was discharged home on oral levofloxacin for completion of her antibiotic course.\nThe patient returned to the ED several days later with shortness of breath and worsening back pain that radiated to her chest. The ECG () at that time was significant for sinus tachycardia and an S1Q3T3 phenomenon. She was admitted for severe sepsis and started on ceftriaxone for concern of recurrent bacteremia. Repeat transthoracic echocardiography demonstrated right ventricular strain and what appeared to be a near to total occlusion of her pulmonary trunk (). Computed tomography (CT) angiography was significant for a 4.8 cm small-necked pseudoaneurysm arising off the anterolateral aspect of the aorta and was found to be compressing the pulmonary trunk and right pulmonary artery ().\nOur patient was again transferred to the ICU. A subsequent CT chest with contrast demonstrated pseudoaneurysm expansion to 5.3 cm. Emergent cardiothoracic surgery with circulatory arrest was performed. The operation revealed a 2 cm × 1 cm wall defect in the distal ascending aorta, extending into the arch of the aorta. Intraoperative transesophageal echocardiography revealed right ventricular remodeling resulting in a severely strained D-shaped ventricle and severely dilated RVOT of 4 cm (). Additionally, there was significant clot burden in the anterior mediastinum encasing the ascending aorta and the pulmonary artery. A CorMatrix patch was used to close the defect, and a specimen of the anterior mediastinal mass was sent for pathological analysis, confirming our diagnosis of a mycotic pseudoaneurysm. The patient tolerated the surgical procedure well and did not require additional operations during her inpatient hospital recovery.
|
[[18.0, 'year']]
|
F
|
{'11700493': 1, '28690902': 1, '24470961': 2, '16520038': 1, '2690043': 1, '930941': 1, '3096236': 1, '8952771': 1, '10818188': 1, '3898463': 1, '8227835': 1, '9104897': 1, '1138554': 1, '17098542': 1, '2657621': 1, '18573788': 1, '5006794': 1, '22230680': 1, '21618000': 1, '17215986': 1, '12923044': 1, '30186637': 2}
|
{'3892607-1': 1}
|
2,292 |
6116406-1
| 30,186,658 |
comm/PMC006xxxxxx/PMC6116406.xml
|
Recurrent Cardiac Myxoma Treated by Orthotopic Heart Transplantation: A Case Report and Literature Review of Heart Transplantation for Primary Cardiac Tumor
|
A 17-year-old girl with a recurrent cardiac tumor presented for the heart transplant evaluation. Initially, at the age of 10, she first presented with dyspnea and holosystolic murmur at apex. She did not have any significant medical history, other than a first-degree relative family history of CM. Echocardiography revealed a 3.5 × 4.8-cm LA mass. The mass was mobile, heterogeneous, and protruded from atrial septum into LA, suspected for CM and causing mitral regurgitation (). The patient underwent tumor removal surgery via midline sternotomy. The pathology showed myxoid stroma with clusters of spindle cells and small blood vessels confirming the diagnosis of CM.\nA year after the surgery, at the age of 12, she presented to the emergency department with sudden abdominal and right leg pain. The abdominal examination showed left upper abdominal tenderness and guarding. There were also signs of arterial occlusion in her right leg. Echocardiogram showed multiple cardiac masses in LA (2.5 × 1.9 cm) and left ventricular (LV) (1.8 × 1.0 cm) (). A computed tomography of the abdomen and lower extremities confirmed the diagnosis of splenic infarction and femoral arterial embolism. She underwent splenectomy and embolectomy. An open heart operation revealed 5 cardiac masses (3 in LA, and 2 in LV) which all were removed. All specimens including the tissue from embolectomy were reported as a CM. The surgery was uneventful and echocardiography afterward did not show tumor residual. The repeat physical examination did not reveal signs of Carney complex.\nUnfortunately, at the age of 13, a right ventricular (RV) mass sized 1.7 × 1.6 cm was detected on the echocardiography. She was asymptomatic but with the follow-up echocardiogram showing increasing in size of the mass to 2.8 × 4.7 cm extended into RV outflow tract causing obstruction (peak RV outflow velocity of 3.8 m/s) (). After discussion, she underwent a third sternotomy for tumor resection and specimens were reported as CM. The surveillance echocardiography was performed every 6 months and at the age of 15. There were recurrent of cardiac masses in LA, LV, and RV, sized 2.4 cm, 2.1 cm, and 2.4 cm, respectively ().\nEven though CM is considered benign, due to the infrequent recurrence, fast growing, and the complications from the neoplasm including valve regurgitation and obstruction, and embolic event, the heart transplant was proposed with the fourth recurrence of CM. Other than elevated panel reactive antibody with multiple significant anti-human leukocyte antibodies, there were no other contraindications. She was on the waiting list for 11 months. She was treated with warfarin while on the waitlist without new events. Of note, at the time of heart transplant operation, she was 17 years old. The heart transplant was performed with a bicaval anastomosis. Attention was given to maximally excise the recipient cardiac tissue. The gross examination of the excision heart showed 4, 1, and 1 masses in RV, LA, and LV (). These masses showed homogenous tan white, gelatinous, rubbery cut surface. The masses were reported as CM.\nThe clinical course was uneventful. Regarding immunosuppression, she was given standard regimen of our institution for high risk patient including perioperative plasmapheresis and induction therapy with basiliximab, then maintenance regimen with a combination of cyclosporine, mycophenolate, and prednisone. Now, 6 months after the surgery, the patient is doing well with New York Heart Association functional class I. The steroid was weaned off. There was no episode of treated rejection. The physical examination and echocardiogram did not reveal signs of recurrent masses. The echo was planned in a 6-month interval.\nThe details of each episode of tumor presentation and operations are summarized in .
|
[[17.0, 'year']]
|
F
|
{'9344319': 1, '24789151': 1, '20227299': 1, '8329433': 1, '22014449': 1, '8061032': 1, '2635939': 1, '7818349': 1, '1776247': 1, '14667616': 1, '26542780': 1, '24933197': 1, '8246562': 1, '14611833': 1, '2207997': 1, '22754666': 1, '7803440': 1, '23618320': 2, '10963150': 1, '22626745': 1, '6261046': 1, '23462142': 1, '26832806': 1, '25688375': 1, '22447042': 1, '9539829': 1, '18192811': 1, '11383779': 1, '22011311': 1, '24297716': 1, '7574960': 1, '9036481': 1, '23119110': 1, '12447180': 1, '28784324': 1, '2155359': 1, '23284099': 1, '8239823': 1, '30186658': 2}
|
{'3654903-1': 1}
|
2,293 |
6116445-1
| 30,165,878 |
comm/PMC006xxxxxx/PMC6116445.xml
|
A case of ovarian clear cell carcinoma arising from ovarian mature cystic teratoma
|
A 71-year old female, gravida 2, para 2 presented with abdominal distention which had worsened over the previous year. Her past medical history was appendicitis, and an appendectomy was performed in her thirties. Her past family history was unremarkable. A pelvic examination identified a very large mass in both hypochondrium which was hardly movable. Pelvic and abdominal ultrasonography showed a huge cystic mass with a solid component. Serum tumor marker levels were carcinoembryonic antigen (CEA): 2.0 ng/mL (normal < 37.0), CA19–9: 459.2 U/mL (normal < 37.0), SCC: 18.9 ng/mL (normal < 1.5), and CA125: 329.9 U/mL (normal < 35.0). Other blood examination results were unremarkable. A pelvic MRI showed a huge cystic mass with a nodular component which was enhanced and under diffused in diffusion weighted image (Fig. ). Computed tomography (CT) showed a 3 cm mass in the liver, and fluorodeoxyglucose-positron emission tomography (FDG-PET) showed FDG uptake not only in the pelvic tumor (SUVmax = 22.9) but also in the hepatic nodule (SUVmax = 13.7), thus suggesting metastases (Fig. ).\nOvarian cancer and liver metastasis was suspected on these data, and a subsequent percutaneous liver biopsy was performed. The pathology showed metastatic cells in normal hepatocytes, and a diagnosis of poorly differentiated carcinoma was made (Fig. ). Immunohistochemical staining showed that p40, p63, and hepatocytes were all negative, thus denying primary hepatocellular carcinoma. Therefore, a clinical diagnosis of ovarian cancer stage IVB with malignant transformation of the MCT was made.\nA total abdominal hysterectomy, bilateral salpingo-oophorectomy and partial omentectomy was performed. The left ovary was enlarged (about 300 mm) and filled with 13,000 ml of yellowish fluid. Adhesion was not particular, and the uterus and right ovary appeared to have no remarkable changes (Fig. ). The cytology of ascites was negative. The patient’s postoperative course was uneventful.\nThe pathological findings revealed that most of the ovary consisted of an MCT with squamous cell epithelium, cutaneous appendage and cartilaginous tissue (Fig. ); however, some parts consisted of papillary or glandular lesion with hobnail-like atypical cells (Fig. ). We made a diagnosis of left ovarian clear cell carcinoma and malignant transformation of MCT based upon the proof of transition from simple squamous epithelium via simple glandular epithelium to papillary change with atypia (Fig. ). Histopathology showed no endometriosis in either ovary and, therefore, endometriosis-associated malignancy was denied. However, the uterus and omentum showed no signs of malignancy. The left fimbria of the fallopian tube had a lymphovascular space of invasion with calcifying carcinoma cells (Fig. ). However, the uterus and omentum showed no signs of malignancy. Immunohistochemical analysis of the left ovary indicated that AE1/AE3, CK7, PAX-8 and Napsin A were all positive, and that 34βE12 and P53 were slightly positive (Fig. ). Although CK20 and vimentin were negative, Ki-67 (MIB-1) index was 25% positive in the carcinomatous part. Moreover, PAS was positive in the stromal part, and PAS and ALB were both positive in some glandular lesions. This was classified as stage IVB (pT2aNXM1) according to the International Federation of Gynecology and Obstetrics (FIGO) 2014 classifications.\nThe patient subsequently underwent chemotherapy with 3 courses of paclitaxel and carboplatin. Afterward, CT scanning revealed a vanished hepatic metastasis and emerged swelling of the para-aorta lymph node. Although 3 courses of doxorubicin were administered, the para-aorta lymph node continued to enlarge. As chemotherapy was not effective due to her progressive disease, the patient began to receive the best possible supportive care. After 17 months from the operation, she passed away.
|
[[71.0, 'year']]
|
F
|
{'23026196': 1, '16445624': 1, '25990936': 1, '19480263': 1, '12836022': 1, '23091901': 1, '14566099': 1, '27098182': 2, '14720146': 1, '12911740': 1, '26137048': 1, '14614249': 1, '9788270': 1, '28787321': 1, '19521124': 1, '26352547': 1, '19038764': 1, '12893196': 1, '24009635': 2, '10861437': 1, '13493921': 1, '8381377': 1, '26384067': 1, '27987343': 1, '17240432': 1, '30165878': 2}
|
{'4839142-1': 1, '3759639-1': 1}
|
2,294 |
6116566-1
| 30,157,943 |
comm/PMC006xxxxxx/PMC6116566.xml
|
A left-sided cystic pancreatic incidentaloma with sigmoid colon adenocarcinoma: a case report
|
A 65-year-old man from the Indian subcontinent presented to our hospital with complaints of a mild, dull, aching left-sided abdominal pain for the past year, loss of weight and appetite for 6 months. There was a history of per rectum bleeding and recent history of altered bowel habits. He had no history of malignancy in the family. There was no other clinically significant history.\nA general examination of our patient was within normal limits. There was no significant lymphadenopathy.\nOn abdominal examination, a 7 cm × 6 cm size lump was palpable in the epigastric region extending up to the left hypochondrium; it was nodular, nontender, firm in consistency, with a well-defined border, and not moving with respiration. The rest of his abdomen was unremarkable. A rectal examination was normal.\nRoutine laboratory investigations including a complete blood count, an international normalized ratio, liver function tests, and renal function tests were within normal limits. Ultrasonography of his abdomen and pelvis was performed, and a multicystic lesion in the distal body and tail of the pancreas measuring about 7 × 7 cms was seen; no lymph nodes were seen. The rest of his pancreas was normal, and the proximal pancreatic duct was dilated, his liver was normal, and no free fluid was seen. Abdominal and pelvic contrast-enhanced computed tomography (CECT) was performed and revealed a multicystic lobulated mass arising from the distal body and tail of the pancreas with proximal pancreatic duct dilatation, a normal liver, no free fluid, an irregular mass in the sigmoid colon with mild narrowing of the lumen, with no proximal dilatation of the colon. Tumor marker tests showed carcinoembryonic antigen (CEA) test results of 10.48 ng/ml (normal value. < or = 3.0 ng/mL, in smokers: < or = 5.0 ng/mL), and a CA19–9 test result of 7.19 U/mL (< 37 U/mL). A colonoscopy revealed ulceroproliferative, nonobstructing growth in the distal sigmoid colon with small polyps nearby. Colonoscopic biopsy result showed well-differentiated adenocarcinoma. A positron emission tomography/computed tomography (PET-CT) scan was performed and showed metabolically active circumferential thickening in the distal sigmoid colon with a maximum standard uptake value (SUV max) 7.0. and a multicystic lobulated mass with low metabolic activity arising from the distal body and tail of pancreas.\nEndoscopic ultrasound confirmed the picture of microcystic serous cystadenoma with no vessel involvement or lymph nodes. An endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS- FNA) was done, his fluid amylase levels were within normal limits, and his fluid CEA levels were not elevated. Cytology showed only benign cells (Figs. and ).\nDistal pancreaticosplenectomy and radical sigmoidectomy with end-to-end colorectal anastomosis was performed.\nHis postoperative course was uneventful. The histopathology report showed microcystic (benign) serous cystadenoma of the pancreas, and moderately differentiated adenocarcinoma of the sigmoid colon with lymph node metastasis 0/37. Angiolymphatic invasion was positive, and perineural invasion was absent.\nOur patient was followed up every third month for 1 year and was evaluated. Blood reports and imaging studies were unremarkable and did not detect any recurrence (Figs. , and ).
|
[[65.0, 'year']]
|
M
|
{'11785413': 1, '21600553': 1, '19844152': 1, '8626891': 1, '10587182': 1, '19858708': 1, '15623441': 1, '23022726': 1, '10450728': 1, '27487847': 1, '18784959': 1, '16135927': 1, '18499542': 1, '18853975': 1, '30157943': 2}
|
{}
|
2,295 |
6117226-1
| 30,167,990 |
comm/PMC006xxxxxx/PMC6117226.xml
|
Laparoscopic treatment of intestinal obstruction due to a vitelline vascular remnant and simultaneous appendicitis: a case report
|
A 14-year-old male was admitted to our hospital with right lower abdominal pain and vomiting. He had no history of abdominal surgery or trauma. Physical examination revealed deep tenderness at McBurney’s point without abdominal distension. A blood test on admission revealed slight leukocytosis (9840/μl) without elevation of the C-reactive protein level (0.01 mg/dl). Enhanced computed tomography scan showed a slightly enhanced, thickened appendiceal wall (Fig. ). Although a slightly dilated ileum and ascites at the recto-vesical pouch were also observed (Fig. ), intestinal obstruction was not diagnosed by these imaging studies. These abnormalities were thought to be inflammatory changes due to appendicitis. The initial diagnosis was mild acute appendicitis based on the physical examination findings and blood test and imaging results, and appendectomy with small laparotomy was therefore planned. However, the patient’s abdominal pain was so severe that analgesics were completely ineffective; continuous vomiting was also observed. Additionally, the ascites at the recto-vesical pouch was unusual considering the mild appendicitis. We performed laparoscopic surgery to explore the abdominal cavity and obtain a definitive diagnosis.\nLaparoscopic surgery with three trocars was performed (12-mm camera trocar in the infra-umbilical position and two 5-mm trocars in the left lower quadrant and lower median abdomen). Strangulated small bowel obstruction caused by trapping of ileal bowel loops by a band was observed (Fig. ). After reduction, the band was found to be connecting the right medial umbilical fold to the ileal mesentery (Fig. ,) and was resected using laparoscopic coagulation shears. The band was connected to the ileal mesentery, 30 cm proximal to the ileocecal valve, and neither Meckel’s diverticulum nor ischemic change of the trapped ileum was detected (Fig. ). The appendix showed slight inflammatory change (Fig. ), and appendectomy was also performed. Serous ascites was found at the recto-vesical pouch (Fig. ) and was thought to be caused by strangulated small bowel obstruction. The patient was discharged without complications on postoperative day 4.\nPathological examination revealed that the band consisted of blood vessels, and it was diagnosed as a vitelline vascular remnant (Fig. , ). The macroscopic view of the resected appendix is shown in Fig. . Fecal stones were found on the proximal side of the appendix, and the wall was slightly thickened. Pathological examination revealed diffuse infiltration of lymphocytes and eosinophils throughout the muscularis propria, and the patient was diagnosed with mild chronic appendicitis (Fig. ).
|
[[14.0, 'year']]
|
M
|
{'27995004': 2, '17618811': 1, '17859000': 1, '17210482': 1, '3495250': 1, '14625729': 1, '15959710': 1, '16521206': 1, '21024698': 1, '22920945': 1, '30923950': 2, '27999702': 2, '25823549': 1, '13031688': 1, '9138710': 1, '25680534': 1, '5295737': 1, '30167990': 2}
|
{'6439070-1': 1, '5143717-1': 1, '5126029-1': 1}
|
2,296 |
6117228-1
| 30,168,002 |
comm/PMC006xxxxxx/PMC6117228.xml
|
Overexpression of IGF2 and IGF2 receptor in malignant solitary fibrous tumor with hypoglycemia: a case report
|
An 81-year-old man presented with abdominal discomfort. Computed tomography imaging revealed a large tumor with intermediate signal intensity, showing heterogeneous contrast enhancement in the subphrenic area (Fig. a) and the feeding artery originated from the diaphragm (Fig. b). Within several days, he suddenly experienced loss of consciousness. Serum examination indicated hypoglycemia (glucose levels, 18 mg/dL). Insulin (1.05 μIU/mL), C-peptide (0.71 ng/ml), and IGF1 (39 ng/mL) levels were all relatively low but still within the physiological range. Western blot analysis of the patient’s serum revealed overexpression of high-molecular-weight IGF-2 designated “big IGF-2” (Fig. ). Along with glucose compensation, the patient underwent surgery for total tumor resection. In the operative view, the large tumor appeared to compress the right lobe of the liver without invasion. The feeding artery originating from the diaphragm was ligated and divided. A part of the diaphragm was resected with autosuture owing to firm adherence of the diaphragm to the artery. The tumor was then dissected along the liver surface without simultaneous resection of any other organs, and a tumor-free margin was achieved macroscopically. The tumor measured 34 cm at the major axis and weighed 1350 g and was well demarcated by a fibrous membrane. The resected surface was elastic, firm, had an ivory-like appearance, and was multilobulated with trabeculation. The solid component was predominant, and a small myxoid component was also noted (Fig. ). Microscopically, spindle cells generally constituted the tumor without specific cellular arrangement in the solid component (Fig. a). A few areas in this component demonstrated keloid-like collagenous stroma (Fig. b) and stag horn-like vessels. However, some areas with myxoid appearance upon gross examination revealed a multi-cystic formation by cavernous hemangioma-like septa. Karyokinesis was detected in more than 0/10 high-power fields in the hypercellular area. Necrosis was obscure. Immunohistochemically, the tumor cells expressed STAT6 (Fig. c) and CD34. The highest Ki-67 labeling index was 15%. These results confirmed that the tumor was a malignant SFT. In addition, the tumor cells exhibited cytoplasmic IGF2 expression (Fig. d), specifically with paranuclear dot-like reactivity. The tumor cells were positive for IGF2R (Fig. e) but negative for IGF1R. Since surgery, the patient has been free from tumor recurrence and hypoglycemia for more than 24 months.
|
[[81.0, 'year']]
|
M
|
{'23313952': 1, '24030747': 1, '17409923': 1, '25113505': 2, '27061182': 2, '24891941': 2, '8631597': 1, '17620336': 1, '19407853': 1, '24934576': 2, '18040628': 1, '34200924': 1, '21540285': 1, '19646808': 1, '34239677': 1, '24080445': 1, '20924377': 1, '26861122': 1, '24360568': 1, '30168002': 2}
|
{'4067084-1': 1, '4267432-1': 1, '4031926-1': 1, '4826510-1': 1}
|
2,297 |
6117531-1
| 30,197,592 |
comm/PMC006xxxxxx/PMC6117531.xml
|
Cathodal tDCS Over Motor Cortex Does Not Improve Tourette Syndrome: Lessons Learned From a Case Series
|
The third patient (P3), an 18 year old female, presented with frequent simple and complex motor as well as vocal tics (age at onset: 14 years). She was also suffering from OCD symptoms. Motor tics included hitting own thorax and pelvis with her fists, flipping, grimacing, jerking of the head, shoulders and hands, gesturing (Russian roulette), saluting and locking her feet while walking. Vocal tics included harrumphing, whistling, caterwauling, uttering syllables, words, limited sentences and echolalia. Tic-free sequences were short (max. 1 min), urges to perform tics were rated very high by the patient. OCD symptoms included compulsive counting, repeating, checking and arranging/collocating. Current treatment consisted of risperidone 6 mg/day and biperiden 4 mg/day in a stable dose for 3 weeks. Previous treatment included antidepressants and atypical antipsychotics (fluoxetine 30 mg/day, aripiprazole 10 mg/day, tiapride 600 mg/day, and quetiapine in unknown dosage) and was discontinued more than 1 year before due to symptom-alleviation (fluoxetine) or lack in positive therapeutic outcome or inacceptable side effects (aripiprazole, tiapride, quetiapine).
|
[[18.0, 'year']]
|
F
|
{'34356182': 1, '25616936': 1, '32710772': 1, '27056623': 1, '21955933': 1, '2684084': 1, '20107115': 1, '31508215': 1, '18785641': 1, '28657865': 1, '25912296': 1, '22037126': 1, '22869014': 1, '27372845': 1, '23339180': 1, '28289393': 1, '16393016': 1, '26842422': 1, '29317605': 1, '2768151': 1, '31463531': 1, '33462641': 1, '15982444': 1, '26875502': 1, '21146614': 1, '25852494': 1, '28913915': 1, '34643763': 1, '25476818': 1, '11723286': 1, '33244309': 1, '29292730': 1, '23389323': 1, '14557563': 1, '14580622': 1, '26880181': 1, '18823015': 1, '29053637': 1, '11417610': 1, '27802585': 1, '23238046': 1, '9651407': 1, '22031874': 1, '30197592': 2}
|
{}
|
2,298 |
6117874-1
| 30,165,900 |
comm/PMC006xxxxxx/PMC6117874.xml
|
Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: a case report
|
Our patient was a 45-year-old white man. He was a farmer, former alcoholic, and former smoker. He had had high blood pressure for the past 3 years, which was treated with losartan. He had been followed at Barretos Cancer Hospital (BCH) since November 2015 because he had a diagnosis of Binet stage B CLL. Three months after receiving the diagnosis, he developed stage B symptoms and a significant increase of lymph nodes. Because rituximab is not available in our public health system, the patient was treated with fludarabine and cyclophosphamide. He received six cycles of chemotherapy, achieving a partial response. He was followed up, and after 8 months, his disease relapsed, which led to the indication of ibrutinib. However, before the new treatment could be started, he returned to BCH in April 2017, reporting fever and lesions in the oropharynx and skin that had begun 2 weeks prior to this consultation. He reported that he had used penicillin 3 weeks before because of an unrelated condition.\nHe also said that, in the beginning, the skin lesions were formed by bullae that burst and caused erosions, crusts, and hemorrhagic surface with bloody exudation. He also had oral mucosa and lips lesions. The patient complained of pain in the affected areas and difficulty eating because of the oral lesions. He also reported episodes of a small amount of anal bleeding.\nThe patient’s physical examination showed lesions in ocular, oral, and urogenital mucosae; chest; scalp; back; and hand palms. The lesions in the oral mucosa were painful erosions, and hematic crusts were present in the lips. The cutaneous lesions were polymorphic, with bullous erosions, ulcerations, and hemorrhagic crusts involving many areas of the body (Fig. and ). The patient’s Nikolsky sign was positive.\nInitially, the main diagnostic hypothesis was Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis based on the previous report of penicillin use []. The patient was directed to the intensive care unit for supportive treatment. Intravenous immunoglobulin was administered for SJS treatment, the lesions were dressed daily, and meropenem and fluconazole were prescribed for a possible secondary skin infection. Although these measures had been taken, the skin and mucosal lesions worsened progressively. The patient’s clinical status deteriorated significantly, which led to the need for oxygen supplementation.\nGiven the unsatisfactory evolution, biopsies of the skin lesions were performed. The histological analysis showed keratinocyte apoptosis and acantholysis suggestive of PNP (Fig. ). Direct immunofluorescence was not performed, owing to unavailability, but based on the clinical-pathological correlation, the diagnosis of PNP was established.\nPulse therapy with methylprednisolone was prescribed for 5 days. There was a discreet initial improvement, but new bubbles appeared in the patient’s face, chest, and limbs, accompanied by intense pain. Four days after the diagnostic confirmation, the patient died of respiratory failure.
|
[[45.0, 'year']]
|
M
|
{'23796242': 1, '10918557': 1, '33942776': 1, '21246308': 1, '20180886': 1, '22801794': 1, '28295171': 1, '28801011': 1, '17683379': 1, '2247105': 1, '23517368': 1, '12366419': 1, '32607299': 2, '24599767': 1, '30165900': 2}
|
{'7320649-1': 1}
|
2,299 |
6117877-1
| 30,165,866 |
comm/PMC006xxxxxx/PMC6117877.xml
|
Video-assisted thoracoscopic right upper lobectomy in a patient with a right-sided aortic arch and Kommerell diverticulum
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A 77-year-old woman was referred to our division for a mixed ground-glass opacity lesion in the right upper lung. The patient was symptom-free and in good performance status. There were no remarkable findings on physical examination. Chest computed tomography revealed a 34 mm × 20 mm partial solid nodule with spicule formation and pleural indentation at the right S3 (Fig. ). The consolidation tumor ratio was 30%. Preoperative positron emission tomography-computed tomography (PET-CT) revealed that the standard uptake value (SUV) of the lesion was 1.9, and there was no suspicious metastasis. Kommerell diverticulum at the origin of the left subclavian artery and a right-sided aortic arch were detected through preoperative three-dimensional computed tomography (3D CT) reconstruction (Fig. , ). Echocardiography revealed no cardiac abnormalities.\nA right upper lobectomy was performed because the lesion was considered to be malignant. A three-port VATS approach with no rib spreading (no soft tissue retractor or direct visualization) was used. A 3-cm incision in the fourth intercostal space (ICS) at the anterior axillary line was made as the main manipulation port, and a 1-cm incision in the seventh ICS at the scapular line was made as an assisted manipulation port. A 1-cm thoracoscopic port was made in the seventh ICS at the middle axillary line. After dividing the interlobar fissure with a linear stapler, we detached and divided the ascending A2, right superior pulmonary vein (RSPV), A1 + A3, and right upper lobe bronchus sequentially. There were no anatomic variations of pulmonary vessels or bronchi (Fig. ). After histologically confirming the invasive carcinoma, radical mediastinal lymph node dissection was performed. After the routine dissection of the pulmonary ligament lymph node (#9) and the paraesophageal lymph node (#8), we dissected the subcarinal lymph node (#7). It was difficult to expose the left main bronchus, as the esophagus could not be suspended easily due to the obstruction of the right descending aorta (Fig. ). When dissecting the paratracheal lymph node, we identified the right vagus nerve above the azygos vein by blunt separation, and we then proceeded with the dissection in the cranial direction (Fig. ). The right recurrent laryngeal nerve (RLN) was observed to branch from the right vagus nerve and hook around the right-sided aortic arch (Fig. ). No upper paratracheal lymph node was dissected because of the obstruction of the right-sided aortic arch. The total operative time was 170 min with an estimated blood loss of 100 cc.\nThe postoperative histological diagnosis was moderately differentiated adenocarcinoma (80% lepidic and 20% acinar). There were five lower paratracheal lymph nodes, five subcarinal lymph nodes, two paraesophageal lymph nodes, two pulmonary ligament lymph nodes, one #10 lymph node, and two #11 lymph nodes retrieved. No lymph node metastasis was detected. The patient was discharged on postoperative day 7 without any complications such as hoarseness.
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[[77.0, 'year']]
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F
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{'23334985': 1, '14718830': 1, '14529170': 1, '5944202': 1, '30165866': 2}
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{}
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