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1,100 | 6038475-7 | 30,046,458 | comm/PMC006xxxxxx/PMC6038475.xml | Descriptive Study of Conjunctival Cysts: A Rare Complication after Strabismus Surgery | A 4-year-old female patient underwent surgery under general anesthesia following the diagnosis of concomitant exotropia. Ten days following the strabismus surgery, the patient showed subconjunctival cyst in the left eye, without evident symptoms (). Surgical exploration discovered thin purulent fluid in the subconjunctival cyst, with no evident capsule and an unclear boundary mostly made up of necrotic soft tissue. The broken end of medial rectus was firmly attached to the sclera surface (surgical design: 5 mm posterior insertion) and the initial muscle suture (6-0 Coated Vicryl absorbable, Ethicon, INC) was intact but loose; therefore, it was removed. A portion of necrotic tissue was extracted for bacterial and fungal culture tests, as well as a pathological test which revealed (left subconjunctival) chronic suppurative inflammation (). Postoperative bacterial culture was positive for methicillin-resistant Staphylococcus aureus (MRSA) infection. According to a drug sensitivity test, vancomycin (0.25 g, q8h) was administered via intravenous drip, combined with local antibiotic and corticosteroid eye drops (Tobradex, SA Alcon-Couvreur NV, Belgium). Five days postoperatively, scant mucous discharge was present at the conjunctival incision suture (). Therefore, the conjunctival suture was removed. At the first postoperative follow-up 1 month after the surgery, a complete recovery of conjunctival incision () with right binocular alignment was shown. | [[4.0, 'year']] | F | {'26261229': 1, '19840730': 1, '2014110': 1, '27799579': 1, '16631253': 1, '26677907': 1, '20795604': 1, '24742059': 1, '10532751': 1, '27085279': 1, '20651594': 1, '341738': 1, '12765540': 1, '17383913': 1, '6629442': 1, '23960870': 1, '27561000': 1, '26235788': 1, '28251554': 1, '1558120': 1, '7752033': 1, '9577816': 1, '22909077': 1, '24568992': 1, '11182673': 1, '30046458': 2} | {'6038475-1': 2, '6038475-2': 2, '6038475-3': 2, '6038475-4': 2, '6038475-5': 2, '6038475-6': 2} |
1,101 | 6038490-1 | 30,046,505 | comm/PMC006xxxxxx/PMC6038490.xml | Adult Bilateral Ureteroceles Presenting with Lower Urinary Tract Symptoms and Acute Urinary Retention | A 30-year-old female was referred to the urology clinic for severe lower urinary tract symptoms (LUTS) in addition to suspicion of a urethral mass. Her symptoms started two years earlier after the indwelling urethral catheter was removed following her Caesarian section. She noticed that a vestibular mass protruded at the level of her urethra and she reported increasing need to strain to void. In addition, this vestibular mass was very painful, and it required manual reduction to allow for her to void. LUTS got progressively worse over the course of the two years. In addition to the dysuria, she had intermittent hematuria, frequency of every hour as well as nocturia 3 to 4 times per night. Her personal past medical history was only positive for a remote appendectomy and a caesarean section. The patient had been previously worked up by her obstetrician since the LUTS appeared after her caesarean delivery. She had repeated urine analyses and cultures that were negative. Abdominal ultrasonography demonstrated the presence of two cystic lesions in the bladder of 2.5cm and 9mm in diameter. The kidneys did not show any hydronephrosis nor hydroureter. Her blood work, including renal function, was unremarkable.\nExamination in lithotomy position revealed a very sensitive erythematous mucosa protruding from the urethra. Patient had to manually reduce the vestibular mass in the sitting position so that she could undergo cystoscopic examination under local anesthesia. Otherwise, it was too painful for the patient. Once reduced, cystoscopy was performed and demonstrated the presence of two large ureteroceles. The left ureterocele, which was significantly larger than the right side, demonstrated erythematous and edematous mucosa indicating that the vestibular mass previously noted by the patient and on examination was likely the wall of the left ureterocele. To rule out duplex system, a triphasic CT-urogram was ordered and it confirmed the patient's bilateral single-system ureteroceles (Figures –). No urolithiasis was identified in both collecting systems. She was offered transurethral “unroofing” of her ureteroceles with placement of bilateral indwelling ureteral stents to reduce risk of ureteral obstruction postoperatively (). While the patient was waiting for her elective surgery, she presented to the emergency department in acute urinary retention in addition to significant lower abdominal pain. An indwelling urethral catheter was placed to reduce the prolapsed ureterocele, decompress the bladder, and control her severe pain. Subsequently, she underwent the planned endoscopic procedure. We started with the larger and more symptomatic left ureterocele. As soon as we unroofed the ureterocele, we identified the normal ureteral orifice and placed ureteral catheter over a guidewire (). Ureteral catheters were used to avoid injuring the back wall of the ureterocele and avoid injuring the true ureteral orifice within the ureterocele. The same procedure was repeated for the smaller right ureterocele. Once the anterior wall of the ureterocele was resected, we placed indwelling ureteral stents bilaterally. Final pathology demonstrated benign urothelium with cystitis cystica and glandularis in addition to Von Brunn's nests. Indwelling ureteral stents were removed shortly after her operation. At 3-month follow-up, the patient was completely asymptomatic with normal voiding patterns, a normal flow study and renal function. Repeat CT-urogram demonstrated resolution of her bilateral ureteroceles without any signs of ureteral stricture, nor hydronephrosis (Figures –). A voiding cystourethrogram was not performed given that the patient remained asymptomatic at 6 months postoperatively without any signs of vesico-ureteral reflux or urinary tract infections. | [[30.0, 'year']] | F | {'24692373': 1, '7260558': 1, '23417311': 1, '17333525': 1, '1543980': 1, '22089776': 1, '32565664': 1, '26738645': 1, '8646606': 1, '33848088': 1, '2359157': 1, '33102700': 1, '18271717': 1, '11018629': 1, '18631884': 1, '26067941': 1, '1029656': 1, '30046505': 2} | {} |
1,102 | 6038581-1 | 30,046,504 | comm/PMC006xxxxxx/PMC6038581.xml | Biliary Tract Abnormalities as a Cause of Distal Bowel Gas in Neonatal Duodenal Atresia | A term female was born at 39 weeks of gestation to a 32-year-old G2P1 by spontaneous vaginal delivery. Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. The pregnancy was uncomplicated. Physical examination revealed a weight of 3650 gm (70th centile), length of 51 cm (60th centile), and head circumference of 33 cm (15th centile). The infant had features of Down syndrome: flattened facies, upslanting palpebral fissures, palmar creases, and sandal gap deformities of the great and second toes. Karyotype was obtained on the first day of life. Recurrent vomiting after each feeding was noted since birth. A plain abdominal radiograph showed a nonspecific bowel gas pattern with gas noted in the stomach, duodenum, and distal bowel (). An upper gastrointestinal (UGI) series showed a complete obstruction to the flow of barium at the proximal portion of the duodenum. A small amount of contrast was also seen to exit from the proximal duodenal segment into a biliary duct structure with a retrograde filling of the biliary tree into the intrahepatic system as well as into the gallbladder through the cystic duct. The contrast was also seen in the proximal jejunum which was located in the right upper quadrant (Figures and ). The patient underwent exploratory laparotomy on the 3rd day of life. Duodenal atresia was repaired. Malrotation was identified, and a Ladd procedure and appendectomy were performed. The postoperative course was uneventful, and the infant was discharged home at 35 days of life. Karyotype confirmed the diagnosis of trisomy 21 (Down syndrome). | [[32.0, 'year']] | F | {'11381438': 1, '5577413': 1, '11477252': 1, '22152869': 1, '8337875': 1, '24557485': 1, '19021635': 1, '2286903': 1, '31662992': 1, '20551648': 1, '6028984': 1, '8809900': 1, '30046504': 2} | {} |
1,103 | 6038588-1 | 30,046,502 | comm/PMC006xxxxxx/PMC6038588.xml | Cerebral Amyloidoma Resulting from Central Nervous System Lymphoplasmacytic Lymphoma: A Case Report and Literature Review | A previously well 87-year-old Caucasian woman living in a senior assisted care center presented to the neurology clinic with complaints of six months of slowly progressing left sided weakness. Initial difficulty in ambulating and using the stairs progressed to being wheelchair bound. Neurologic exam revealed diffuse 3/5 left sided weakness, left leg drift, and left facial droop. Brain magnetic resonance imaging (MRI) revealed a large confluent white matter T2-hyperintensity in the right frontal lobe with multifocal nodular enhancement of the left cerebral hemisphere (). Foci of enhancement were also identified in the cerebellum and leptomeninges. The radiologic differential diagnosis included vasculitis, lymphoma, and CNS sarcoidosis as the most probable causes of the multifocal disease process, with glial neoplasm, demyelination, and metastases considered less likely.\nAll sample analysis described below were performed on material obtained by brain biopsy as part of clinical care. All samples were obtained with appropriate consent.\nA biopsy of the mass was performed and revealed extensive parenchymal lakes and vascular and perivascular deposition of amorphous, amyloid like material (). Congo-red positive staining and apple-green birefringence (not shown) of the amorphous material upon polarization confirmed that the amorphous material was amyloid (). Also present in the resected tissue were a number of small intraparenchymal blood vessels with perivascular lymphoplasmacytic infiltrates (). The initial histologic differential diagnoses included cerebral amyloid angiopathy-inflammatory type (CAA-I) and lymphoma associated amyloidoma. To identify the underlying etiology of the amyloid accumulation, a number of additional analyses were performed.\nLiquid chromatography tandem mass spectroscopic analysis identified the amyloid as AL λ-type and not β amyloid or an amyloid associated with a hereditary amyloidosis. Further analysis of the perivascular lymphoid populations was undertaken. Histologically, the monotonous populations of perivascular lymphoid cells demonstrated a lymphoplasmacytic appearance (). Immunohistochemical analysis demonstrated that the lymphoid cells were CD20 positive (). Tumor cells were negative for CD3, CD5, BCL1, and CD23. The tumor Ki67 proliferation index was low (3%). The more plasmacytoid appearing cells were CD138 positive and were shown to be lambda light chain restricted by kappa and lambda chromogenic in situ analysis (Figures and ). An immunoglobulin heavy chain (IgH) gene rearrangement analysis of the brain tissue from this case was positive for a clonal process with a 253-base pair peak in the FR2 region. A MYD88 L265P mutation analysis by PCR-based pyrosequencing on the brain tissue from this case was negative. A diagnosis of a low grade, lymphoplasmacytic lymphoma (LPL) was rendered. The identification of this CNS low grade lymphoplasmacytic lymphoma confirmed the cause of the amyloidoma to be a lambda light chain producing lymphoplasmacytic lymphoma.\nTo determine if an extracranial/systemic lymphoplasmacytic lymphoma was the source of the CNS neoplasm, a bone marrow biopsy was performed. The bone marrow biopsy showed normal trilineage hematopoiesis and no evidence of lymphoma, myeloma, or amyloidosis. Cytogenetics and fluorescent in situ hybridization studies on the bone marrow were negative for genetic aberrations. Urine protein and serum immunoglobulin levels were within normal limits. A biopsy of subcutaneous abdominal adipose tissue was negative for amyloid, demonstrating lack of evidence of systemic amyloid deposition. Interestingly, an IgH gene rearrangement analysis on the bone marrow was positive for a clonal gene rearrangement with two peaks: a 282-base pair peak in FR2 region and a 120-base pair peak in FR3 region in a polyclonal background, which importantly were markedly different from the IgH gene rearrangement identified in the CNS lymphoplasmacytic lymphoma. Since the two-small bone marrow clonal peaks are present in a polyclonal background, their significance is uncertain and may be age related.\nOur patient received one cycle of chemotherapy with Rituximab for Primary CNS lymphoplasmacytic lymphoma. Two months after diagnosis, she developed a hemorrhagic infarct on the left frontal white matter and was transferred to hospice care. | [[87.0, 'year']] | F | {'27018254': 1, '10394892': 1, '23947787': 2, '34934868': 1, '8006662': 1, '19654273': 1, '28160367': 1, '11838814': 1, '24155683': 2, '29103980': 1, '26476773': 1, '27559959': 2, '23791836': 1, '16009945': 1, '24729110': 1, '30046502': 2} | {'3804736-1': 1, '5400326-1': 1, '3751626-1': 1} |
1,104 | 6038594-1 | 30,046,499 | comm/PMC006xxxxxx/PMC6038594.xml | A Case of Cephalic Tetanus in an Elderly Patient with Trismus | A 77-year-old woman presented to the Emergency Room with a three-day history of oral lesions and jaw tightness. She had presented to a different hospital four days prior due to nausea, vomiting, and dysphagia which were deemed secondary to gastroparesis. She received symptomatic treatment and, once stabilized, was discharged home. However, over the next three days, she developed oral sores and jaw tightness, which prompted her to seek medical attention again. Her physical examination revealed normal vital signs, mild dehydration, trismus, and white ulcers on the visible portion of the tongue that were suspicious for oral candidiasis. Laboratory data revealed mild leukocytosis (WBC 11.33 × 10³) and moderate hyponatremia (123 mmol/L) thought to be secondary to volume depletion and low solute intake. A CT scan of the head and neck was unremarkable. The temporomandibular joints were intact. The patient was admitted to the hospital and treated with diazepam, cyclobenzaprine, nystatin, and chlorhexidine mouthwash and was given nutritional supplementation. She was discharged after two days with a plan to follow up as an outpatient. However, she returned two days later with worsening jaw tightness and inability to swallow, for which she was readmitted.\nHer past medical history included hypertension, diabetes mellitus, dyslipidemia, and a history of deep venous thrombosis. Prior to admission, medications included glipizide, atorvastatin, telmisartan, and rivaroxaban. She was a lifetime nonsmoker and denied alcohol use. She denied recent trauma or skin lacerations, and reported having completed her primary immunization series against tetanus as a child. Her most recent tetanus booster was in 1965.\nOn physical exam, blood pressure was 147/82, pulse was 75, respiratory rate was 16, and temperature was 97.9°F (36.6°C). She was euvolemic and in no apparent distress. The presence of trismus was evident—the patient was unable to open her mouth beyond 2 mm. Laboratory data were remarkable for hyponatremia (122 mmol/L), and the patient was placed on normal saline.\nThe following day, the patient developed three brief episodes of tonic-clonic seizures in the setting of hyponatremia (127 mmol/L) and was treated with intravenous (IV) lorazepam, IV levetiracetam, and 3% saline infusion. An urgent computed tomography (CT) did not reveal any acute intracranial process. The seizures resolved, and the sodium level normalized, but the patient persisted with trismus and dysphagia. As part of the workup, she underwent magnetic resonance imaging (MRI) of the brain and temporomandibular joints, as well as a laryngoscopy and triple-phase bone scan, all of which were noncontributory. She was then given an atypical antipsychotic and antidepressant for a presumptive diagnosis of depression with somatization symptoms. A lumbar puncture was performed, and cerebrospinal fluid analysis revealed 22 nucleated cells (90% lymphocytes), a protein level of 56 mg/dL, and a glucose level of 84 mg/dL. There was no bacterial growth. On the 14th day of illness, the patient developed right lid ptosis and ophthalmoparesis with a right gaze preference, which led to a working diagnosis of cephalic tetanus. She was given intramuscular (IM) tetanus immune globulin, IV metronidazole, and a booster dose of tetanus and diphtheria toxoid vaccine. Baclofen and lorazepam were used for spasm control. Pre-existing anti-tetanus toxoid antibodies were quantified below 0.05 IU/mL.\nUnfortunately, despite treatment, she developed episodes of agonal breathing, psychomotor agitation, and autonomic dysregulation characterized by bradycardia-tachycardia with labile blood pressure, which indicated possible progression to generalized tetanus. She had a cardiopulmonary arrest followed by myoclonus status epilepticus complicated with aspiration pneumonia and sepsis, which ultimately led to her death after 24 days of hospitalization.\nPostmortem exam of the brain showed moderate to severe hypoxic-ischemic changes. The cerebrum and basal ganglia exhibited vascular congestion, cytoplasmic eosinophilia, and nuclear pyknosis of neuronal cells. Extensive neuronal loss was observed in the hippocampus and cerebellum. The midbrain, pons, and medulla oblongata showed vascular congestion. Pulmonary exam revealed bilateral congestion and focal acute bronchopneumonia. | [[77.0, 'year']] | F | {'14033589': 1, '33995513': 1, '11992301': 1, '8178692': 1, '19710932': 1, '32905162': 2, '24349407': 1, '9801353': 1, '25029486': 1, '21451446': 1, '16827708': 1, '10945801': 1, '11811878': 1, '15540881': 1, '19407132': 1, '31392642': 1, '11517134': 1, '22506253': 2, '21314679': 1, '30046499': 2} | {'3309317-1': 1, '7466034-1': 1} |
1,105 | 6038650-1 | 30,046,494 | comm/PMC006xxxxxx/PMC6038650.xml | Complicated Infective Endocarditis Limited to a Chiari Network | A 61-year-old male patient presented to the emergency room (ER) on account of a 3-month history of hemoptysis as well as a 2-week history of right upper extremity weakness and numbness.\nHemoptysis was associated with pleuritic right-sided chest pain as well as orthopnea and had been previously treated with two courses of empiric antibiotics and steroids at an outside hospital without improvement prompting his presentation to our emergency room.\nWeakness and numbness of the right upper extremity were initially associated with a painful right palmar rash which was resolved with self-administered topical corticosteroids at home.\nPast medical history was significant for untreated latent tuberculosis (TB) diagnosed about forty years prior to this presentation as well as coronary artery disease requiring stent placement twice in the past.\nOn presentation, the patient was not in acute distress but was tachycardic at 100 beats per minute, with a respiratory rate of 16 cycles per minute and an oxygen saturation of 100 percent on room air. Blood pressure was 144/87 millimeters of mercury, and temperature was 99 degrees Fahrenheit.\nOn physical examination, he was afebrile and had normal breath sounds and heart sounds without murmurs. Neurological examination was notable for reduced sensation to light but not crude touch over the right hand with reduced strength of 4/5 in that in same extremity. Laboratory studies were significant for a white blood cell count of 18,400 per microliter with 76.3 percent neutrophils, an erythrocyte sedimentation rate of 90 millimeters per hour, and a C-reactive protein level of 167 milligrams per liter.\nImaging done on admission revealed multifocal lung opacities () concerning for community-acquired multifocal pneumonia (possibly secondary to a resistant organism as patient had completed courses of cefdinir and levofloxacin at the outside hospital without resolution of his symptoms). Other considerations were for possible septic emboli of undetermined source as well as reactivated pulmonary tuberculosis given his long-standing history of latent TB with multiple positive skin purified protein derivative tests in the past.\nCT scan of the head without contrast was concerning for a possible infarct of unclear age, and a follow-up MRI revealed possible abscess collection (Figures and ).\nHe was initially placed on airborne isolation with empiric first-line antituberculosis agents (which were discontinued after 6 negative acid-fast bacilli sputum samples were obtained) as well as empiric antibiotics (vancomycin and piperacillin/tazobactam) for possible multifocal pneumonia, based on recommendations of the infectious disease consultants. Two blood cultures, obtained on admission, returned positive and grew Streptococcus intermedius sensitive to ceftriaxone, levofloxacin, tetracycline, erythromycin, clindamycin, and vancomycin. A transthoracic echocardiogram showed normal left ventricular systolic function and normal valves without regurgitations, stenosis or, vegetations.\nA subsequent transesophageal echocardiogram (TEE) revealed an 8.3 × 4.6 mm oscillating dumbbell-shaped vegetation arising from the Chiari network, close to the os of the right atrial appendage (), without evidence of valvular vegetations, insufficiency, or a patent foramen ovale.\nA diagnosis of infective endocarditis was made based on the modified Duke criteria with two separate blood cultures positive for Streptococcus intermedius, echocardiographic findings of an oscillating intracardiac mass, consistent with a vegetation, and septic pulmonary and intracranial lesions []. Medical management was chosen at the time of the diagnosis of this Chiari network endocarditis with septic emboli to the lungs and brain, with plans for repeat imaging with a transesophageal echo after six weeks of appropriate antibiotic therapy.\nAntibiotic coverage was thus narrowed and tailored to the culprit organism with ceftriaxone, and gentamycin was added for endocarditis synergy per infectious disease team recommendations. Clinical and laboratory evidences of improvement including resolution of the hemoptysis and right upper extremity weakness as well as reduction in the white blood cell count from 18,400 per microliter on admission to 6400 per microliter and in the erythrocyte sedimentation rate from 90 millimeters per hour to 28 millimeters per hour as well as the C reactive protein from 167 milligrams per liter to 0.9 milligrams per liter were documented.\nInterval reduction in the size of the lung lesions but not the brain lesions was noted after four weeks of parenteral antibiotic treatment, and neurosurgical evaluation for possible evacuation of the abscess was thus recommended. The patient was transferred to an outside hospital where those services were readily available. He had an image-guided stereotactic drainage of the abscess via a left parietal burr hole, with initial gram stain of the fluid showing gram-positive cocci, but the culture had no growth after 5 days probably because the patient had been on appropriate antibiotics for four weeks prior to the drainage procedure.\nThe patient completed six weeks of antibiotic therapy at the outside hospital and was discharged home in a stable clinical state but did not follow up as recommended. | [[61.0, 'year']] | M | {'7797753': 1, '18479359': 1, '14530039': 1, '26373316': 1, '28959797': 1, '30046494': 2} | {} |
1,106 | 6038654-1 | 30,046,503 | comm/PMC006xxxxxx/PMC6038654.xml | Smoking Relapse Causing an Acute Exacerbation of Desquamative Interstitial Pneumonia with Pleural Effusions and Mediastinal Adenopathies | Our patient is a 58-year-old Caucasian male referred initially to the pulmonary clinic for an abnormal high resolution computed tomography (HRCT) of the chest () showing ground glass opacities (GGOs), thickening of the interlobular septa primarily in the bases with minimal honeycombing, mediastinal adenopathies, and small bilateral pleural effusions. He had complaints of progressive shortness of breath and an unproductive cough. Medical history was relevant for 31-pack-year smoking history, asbestos exposure, uncontrolled Diabetes Mellitus type II, and obesity. His oxygen saturation was 93% on room air. Lung auscultation revealed bibasilar crackles with poor air entry but no clubbing or cyanosis. Pulmonary function test demonstrated an obstructive lung disease with mildly decreased forced vital capacity (FVC) at 76% and forced expiratory volume in one second (FEV1) at 70%, borderline FEV1/FVC ratio at 72%, and excellent effort. Lung volumes also revealed a restrictive lung disease with moderately decreased total lung capacity (TLC) at 64%. Finally, the diffusion capacity of carbon monoxide (DLco) was severely reduced at 44%.\nThe initial outpatient workup included a complete blood count (CBC), comprehensive metabolic panel (CMP), erythrocyte sedimentation rate (ESR), N-terminal probrain natriuretic peptide (NT-proBNP), urinalysis, lactate dehydrogenase (LDH), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), creatine kinase (CK), angiotensin converting enzyme (ACE) level, antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), anti-cyclic citrullinated peptide immunoglobulin G (Anti-CCP), histoplasmosis antibodies, human leukocyte antigen B27 (HLA-B27), aspergillus galactomannan antigen, interferon-gamma release assay for tuberculosis, anti-SCL-70 antibody, beta D-glucan, and antinuclear antibodies (ANA). All were negative except a borderline nonspecific elevation in ACE, LDH, and CK levels and positive ANCA antibodies.\nTo address the interstitial lung disease pattern, a video-assisted thoracoscopic surgery (VATS) lung biopsy was performed. Right lower and right upper lobe wedge excisions revealed classic desquamative interstitial pneumonia, along with foci of respiratory bronchiolitis interstitial pneumonia, mild interstitial fibrosis, and emphysematous changes (Figures and ).\nThe patient's history of lifelong smoking along with asbestos exposure [] likely contributed to these histological changes and DIP diagnosis.\nTo treat DIP, he was started on a tapered dose of glucocorticoids (starting at 40 mg of prednisone) along with nicotine replacement therapy for smoking cessation. Over a 7-month treatment course, the prednisone dose was lowered to 10mg with improvement in his dyspnea and cough. With assistance from the nicotine replacement, the patient successfully quit smoking for 6 months. Repeat CT images showed improvement in GGOs and resolution of prior small pleural effusions.\nAfter 7 months of DIP treatment and with minimal respiratory symptoms, he restarted smoking one pack of cigarettes a day. Six weeks after restarting smoking, respiratory issues quickly developed, and he required hospitalization for acute hypoxemic respiratory failure. Upon admission the patient was tachypneic with labored breathing, worsening bibasilar crackles, decreased air entry, and an unproductive cough. Given his presentation and dropping oxygen saturation to 84% on room air, he was given 100% FiO2 (fraction of inspired oxygen) by nonrebreather mask.\nAfter discussing treatment options for respiratory failure, the patient refused intubation and mechanical ventilation with complete understanding that his condition could be life-threatening. His breathing was therefore supported with alternating 100% FiO2 by nonrebreather mask and noninvasive ventilator support to maintain oxygen saturation above 90%.\nA chest X-ray () and a chest CT angiogram () were ordered. No pulmonary emboli were found. However, imaging did show significant worsening bilateral GGOs, bibasilar consolidations, mediastinal adenopathies, and a new moderate right pleural effusion. The patient was started on broad spectrum antibiotics with vancomycin, piperacillin/tazobactam, and azithromycin. Laboratory workup was unremarkable for infection however. White blood cell count was slightly elevated at 12,600 without a left shift. Procalcitonin level was borderline at 0.14 ng/mL, C-reactive protein was elevated initially but then normalized, and the patient was afebrile at 98.4 Fahrenheit. NT-proBNP was normal which excluded congestive heart failure as a cause for the pleural effusion and GGOs. Repeated arterial blood gas measurements displayed no abnormalities in pH or carbon dioxide levels despite his labored breathing.\nAdditional negative tests included respiratory polymerase chain reaction viral panel, fungal serology, blood cultures, sputum acid-fast bacilli testing, urine streptococcus, and legionella. Sputum fungal and bacterial cultures grew Candida dubliniensis and Pseudomonas fluorescens. Although, these organisms are not generally considered pathogens in humans unless severely immunocompromised and have been found in normal oral flora [, ].\nTo further investigate a cause for the acute decompensation, the patient was offered a bronchoscopy and thoracentesis for mediastinal sampling and pleural effusion, respectively. After an extensive discussion, he refused all invasive diagnostic procedures.\nFour days passed after being given broad spectrum antibiotics and no improvement was observed. Repeat imaging showed a persistence of bilateral interstitial opacities and pleural effusion. His oxygen saturation level still required 100% FiO2 supplementation to maintain above 90%. Because initial workup for the acute respiratory failure was inconclusive, less common etiologies were considered including DIP exacerbation.\nOn the fourth hospitalization day, treatment for possible DIP exacerbation was initiated and antibiotics were stopped. He was given 40 mg methylprednisolone every 8 hours. Within hours, his shortness of breath and oxygen saturation drastically improved. All respiratory symptoms dissipated, and he was stable to be treated as an outpatient after 48 hours of starting treatment. His discharge plan included oxygen support by nasal cannula and oral prednisone. He agreed to a smoking cessation plan with nicotine patches.\nTen days after discharge, an office-visit chest X-ray () showed complete resolution of the pleural effusion and bilateral interstitial and airspace diseases markedly improved. His oxygen saturation climbed to 96% on room air indicating supplemental oxygen was no longer needed. | [[58.0, 'year']] | M | {'29138550': 1, '26210739': 1, '24954625': 2, '16523552': 1, '9412587': 1, '22851712': 1, '5039447': 1, '15653981': 1, '14338290': 1, '25278578': 1, '26138798': 1, '27134124': 1, '634315': 1, '1792426': 1, '20854464': 1, '12833843': 1, '23728865': 1, '20854463': 1, '26278697': 1, '9876799': 1, '15168465': 1, '30046503': 2} | {'4078007-1': 1} |
1,107 | 6038655-1 | 30,046,497 | comm/PMC006xxxxxx/PMC6038655.xml | Successful Treatment of Acromegaly and Associated Hypogonadism with First-Line Clomiphene Therapy | A 49-year-old male was referred for treatment acromegaly and hypogonadism secondary to a suspected GH and prolactin cosecreting pituitary adenoma. He endorsed symptoms of 4-year duration including fatigue, decreased libido, increased snoring, prognathism, increased ring, and shoe size and arthralgias. Physical exam demonstrated a mildly overweight man with normal blood pressure and minimal physical signs of acromegaly who appeared well virilized and exhibited normal testicular size. Serum IGF-I level by immunochemiluminometric assay (ICMA) was increased at 457 ng/mL (reference range 67-205 ng/mL), as well as serum prolactin 79 ng/ml (reference range 4.0–15.2 ng/ml) growth hormone (GH) level 1.9 ng/mL (reference range 0-10 ng/mL) which did not suppress 2 hours after a 75 gram oral glucose load measured at 1.8 ng/mL; while serum testosterone level was markedly decreased at 74 ng/dL (reference range 348-1197ng/dL), gonadotropin levels were normal (). MRI demonstrated a 9 x 9 mm mass lesion in the left inferior-posterior aspect of the sella turcica abutting the left carotid artery. The patient was treated initially with transsphenoidal surgery achieving a gross total resection. Histopathology revealed a pituitary adenoma staining strongly on immunohistochemistry for prolactin and weakly for GH.\nSix months after surgery, serum IGF-I level was persistently elevated at 285ng/mL; a random GH level was 0.4 ng/mL. Prolactin levels were no longer elevated and the remainder of the pituitary function tests was normal except for serum total testosterone which remained low at 255 ng/dl and low free testosterone measured by mass spectrometry at 6.0 pg/ml (reference range 6.8-21.5.pg/ml). MRI showed a partial empty sella with no evidence of tumor. The patient reported improvement of joint aches, snoring and swelling of hands yet complained of persistent fatigue and sexual dysfunction. After discussion of treatment options including somatostatin analogs, dopamine agonists, growth hormone receptor antagonist, and various forms of testosterone replacement, the patient preferred to attempt a trial of clomiphene citrate. The patient was started on oral clomiphene citrate (CC), 25 mg/day.\nSix months after clomiphene initiation, the patient reported resolution of fatigue and improved libido which correlated with an improvement on total testosterone levels 355 ng/dL and free testosterone level 8.6 pg/ml. 11 months after clomiphene therapy, laboratory evaluation reported normalization of both serum IGF-I (134 ng/ml) and a peak serum total testosterone (535ng/dl) and free testosterone (12.6 pg/ml), while GH 0.5 ng/ml and prolactin levels (5.5 ng/ml) remained controlled (). Free testosterone levels increased into the normal range with CC treatment, paralleling the increase of total testosterone levels (). After a brief trial of four months of discontinuing clomiphene treatment, total testosterone, free testosterone, and IGF-I levels returned to near pretreatment values and the patient had recurrence of hypogonadal symptoms. Upon resuming clomiphene therapy, testosterone and IGF-I levels returned to target, fulfilling “Koch's postulate” providing evidence of effectiveness of therapy. | [[49.0, 'year']] | M | {'20535640': 1, '25738590': 1, '15466938': 1, '12656665': 1, '15080777': 1, '11207632': 1, '25356808': 1, '30046497': 2} | {} |
1,108 | 6038659-1 | 30,046,496 | comm/PMC006xxxxxx/PMC6038659.xml | Hair Loss: Evidence to Thallium Poisoning | A 53-year-old man was locally hospitalized for persistent aching pain in the abdomen and lower extremities for nearly 20 days. He was sent to our clinic because of acute and severe hair loss which occurred for 10 days. According to his self-reported history, he was healthy in the past and did not take any medications. He also denied the possibility of accidental poisoning. Physical examination revealed diffuse alopecia of scalp (). His liver function was damaged (ALT 154.8u/l and AST 49.2 u/l), but the levels of urine mercury, hair arsenic, and blood lead were all normal. The scalp hair was completely lost () 1 week later. The diagnosis of thallium poisoning gradually came to our mind and was eventually confirmed by the elevated levels of the thallium ion in urine (4677.0μg/l, normal range 5μg/l) and blood (312.1μg/l, normal range 0μg/l). Therefore, ten cycles of hemoperfusion and hemodialysis were performed, each lasting for 5 to 6 hours daily. As hemoperfusion and hemodialysis were conducted, the blood and urine thallium levels decreased. After hemoperfusion and hemodialysis, there was improvement in neurological manifestations and liver function parameters. At the same time, he was given Prussian blue 2.64 g (0.33 g ∗ 8), Q6h, forced diuresis and 20 mmol potassium chloride twice a day, and intravenous B complex. His pain disappeared slowly, and hair regrowth started 10 days later and was completed during his 6-month follow-up (). The reason for poisoning remains unknown despite an investigation conducted by police authorities. | [[53.0, 'year']] | M | {'17224548': 1, '24580057': 1, '28889276': 1, '18824857': 1, '26402428': 1, '28571191': 1, '15664276': 1, '30813198': 2, '12941306': 1, '1101377': 1, '8518684': 1, '23811578': 1, '14579545': 1, '23900304': 1, '22865288': 1, '28761262': 2, '12612328': 1, '30046496': 2} | {'5514793-1': 1, '6407929-1': 1} |
1,109 | 6038669-1 | 30,046,506 | comm/PMC006xxxxxx/PMC6038669.xml | Severe Case of Chronic Pelvic Pain Syndrome: Recovery after Injection of Procaine into the Vesicoprostatic Plexus—Case Report and Discussion of Pathophysiology and Mechanisms of Action | We report on a 55-year-old man who was diagnosed with CPPS by urology specialists from the university hospital and referred to us for pain treatment with local anesthetics (neural therapy).\nAt his first consultation with us, the patient reported pain and other ailments that began 35 years prior, after a party in a damp basement, without vanishing ever since. In the same night, pollakisuria and dysuria occurred, and the patient noted a permanently painful foreign body sensation in the areas of the prostate and anus, as well as perineally. Furthermore, he complained of a burning sensation in the urethra, a slightly reduced urinary stream, and nocturia of varying frequency. In addition to the ongoing chronic pain, the patient suffered from week- to month-long episodes of increased pain with no discernible trigger. Overall, the pain and other symptoms progressed over time.\nOver the years, various specialist urological examinations were carried out and several attempts at treatment with various empirical antibiotic therapies and analgesics were made. Also, nerve stimulation therapy was applied, and a probatory surgical removal of both seminal vesicles and an extension surgery on the anus were performed. None of these measures resulted in any improvement in pain or other symptoms. The patient was then referred to us by the urologists for a probatory pain treatment with LA.\nAt the first consultation with us the patient was in a particularly severe pain phase. He complained of permanent pain and discomfort perineally and in the areas of the prostate, anus, and urethra, associated with pollakisuria, dysuria, and nocturia (more than ten times per night). Due to this the quality of life was impaired to a large degree. The patient was desperate and did not believe that he could be helped anymore.\nFor nine years he was taking an analgesically effective antiepileptic drug, Gabapentin, as well as the nonsteroidal anti-inflammatory drug Diclofenac, the opioid Oxycodone, and the pain-modifying tricyclic antidepressant, Amitriptyline.\nThe National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI) resulted in 39 points (pain: 18; urinary symptoms: 10; quality of life impact: 11). In rectal palpation, the patient felt pain in the lesser pelvis while the prostate was inconspicuous, as was the case in the recently performed sonography, in which 50 ml of residual urine had been detected. The PSA value was found to be 0.4 ng/ml.\nOur treatment consisted of suprapubic injection of 5 ml each of 1% procaine on the right and the left with infiltration of the vesicoprostatic plexus (in line with neural therapy). In this injection, the puncture site is directly behind the pectineal line (pecten ossis pubis), 5 cm laterally to the center of the symphysis. The puncture direction is 45° both medially and caudally. The needle point needs always to remain extraperitoneal in the paravesical connective tissue, in which the vegetative nerve fibers are located. The penetration depth in the described patient was 7 cm, with the needle gauge being 23.5 (0.6 mm).\nJust a few minutes after the first injection, the pain decreased significantly and persistently to a level the patient had not experienced in years (in his own terms: 90% improvement of all symptoms). In the following days, the patient experienced a further and lasting significant reduction in pain and other symptoms. At the next consultation after two weeks NIH-CPSI resulted in 11 points (pain: 3; urinary symptoms: 4; quality of life impact: 4). To maintain and further increase the effect, we performed the suprapubic injection of procaine a total of six more times, initially once a month, and bimonthly later. With each injection, the pains continued to decrease, up to freedom from pain and discomfort. Relapses occurred less frequently, were significantly lower in intensity and the duration was significantly shorter with an average of three days. Even the symptom-free intervals were getting longer. The patient reduced Diclofenac and Oxycodone on his own initiative and completely discontinued both drugs after the fifth consultation. After seven treatments, he was also able to stop Gabapentin and Amitriptyline and remained free of discomfort (NIH-CPSI: 0 points), which also had a positive effect on his mental and social integrity.\nNo adverse effects were observed. | [[55.0, 'year']] | M | {'16480459': 1, '11793144': 1, '14694543': 1, '11960642': 1, '17513637': 1, '3917554': 1, '17632164': 1, '9585814': 1, '11121511': 1, '9009734': 1, '26642069': 1, '14651434': 1, '16428543': 1, '14572737': 1, '9150299': 1, '21205969': 1, '1707535': 1, '10519505': 1, '93706': 1, '10537081': 1, '2061833': 1, '19363165': 1, '15784301': 1, '32357440': 1, '11050365': 1, '5320816': 1, '24811377': 1, '9655475': 1, '1884106': 1, '12712363': 1, '15082136': 1, '30046506': 2} | {} |
1,110 | 6038973-1 | 29,998,151 | comm/PMC006xxxxxx/PMC6038973.xml | Sagliker Syndrome in a Patient with Secondary Hyperparathyroidism and Chronic Renal Insufficiency: A Case Report | A 44-year-old male patient referred to our center for the parathyroid scan. The patient had a history of renal insufficiency since 1999. Despite renal transplantation, he re-experienced renal failure after 5 years. In addition to SHPT and CKD, the patient suffered from severe maxillary and mandibular deformities, dental abnormalities, and a prominent forehead. Short neck and short stature were significant, as well as the barrel like chest ().\nMoreover, subtotal parathyroidectomy was performed for the patient in 2008. The patient had experienced two episodes of hypocalcemia along with weakness, dyspnea and neck bulging in the past 6 months.\n99mTc-sestamibi dual phase protocol was used for this purpose. Immediately after IV injection of 740 MBq (20 mCi) of 99mTc-sestamibi, patients underwent early imaging of the neck and mediastinum using a dual-head E-CAM SPECT camera equipped with low-energy and high-resolution collimator. Immediately after early 99mTc-sestamibi imaging (10 minutes post-injection), SPECT was performed. SPECT images (128×128 matrix using 64 projections over 3600 with 20 seconds per step). SPECT images were reconstructed by an iterative method (OSEM, number of iterations 8 subsets 4). One hundred and eighty min after injection delayed SPECT imaging of the neck was repeated and data were acquired and processed with the same protocol.\nThe scan pattern on early phase images showed radiotracer uptake in the thyroid region, as well as a focal zone of increased tracer uptake in the left lower part of the neck inferior to the thyroid (). Delayed images revealed tracer washout from the neck region with a focal area of retained activity in the left lower part of the neck inferior to the thyroid. The SPECT images also proved the above findings ().\nThe patient underwent another parathyroidectomy and an abnormal parathyroid tissue was resected from the same location shown in our scan. PTH levels declined to a normal level postoperatively. | [[44.0, 'year']] | M | {'24413727': 1, '25784153': 1, '31656444': 1, '25355264': 1, '18446747': 1, '22200434': 1, '33133856': 2, '15490409': 1, '28263480': 1, '34164302': 1, '24575953': 1, '23026501': 1, '25661637': 1, '29998151': 2} | {'7593211-1': 1} |
1,111 | 6038974-1 | 29,998,153 | comm/PMC006xxxxxx/PMC6038974.xml | Adrenocortical Oncocytic Carcinoma and Papillary Thyroid Carcinoma Incidentally Detected in an Asymptomatic Patient by F-18 FDG PET/CT | A 37-year-old man who had a recent history of inguinal hernioraphy and surgery for varicocele referred for follow up. He had no history of previous major medical illness and had an uneventful previous surgery. A high resolution diagnostic Computed Tomography (HRCT) showed a large isodense mass in the left upper abdomen measuring 19 cm in short axis diameter with areas of hypo-attenuation inside ().\nThe initial blood and biochemistry investigations such as full blood count, renal, liver function tests results and blood tests results for adrenal hormones including Cortisol, Aldosterone, DHEA and Androgenic Steroids, Epinephrine (Adrenaline) and Norepinephrine (Noradrenaline), Thyroid function tests and Calcitonin level are shown in .\nBiochemical blood tests results were nearly normal and with a bulky adrenal mass the possibility of adrenal carcinoma was raised and accordingly F-18 FDG PET/CT was requested for detection of possible additional site of involvement.\nThe FDG-PET-scan was performed after 6 hours fasting and serum glucose level was 68 mg/dl prior to the scan procedures. The PET scanning was started 60 minutes after intravenous administration of 370 MBq (10 mCi) of F-18 FDG. CT scan was obtained without oral contrast and no IV contrast was given. The non-contrast CT scans was used for attenuation correction and localization. Images were acquired with 85 mAp on a GE discovery MI-DR 64 slice LYSO-crystal PET/CT scanner. Transaxial, coronal and sagittal PET images were reviewed in conjunction with fused noncontrast CT.\nThe maximum intensity projection-MIP-PET and PET-CT fused images in coronal and transaxial projections showed left bulky retroperitoneal mass with heterogeneous intense FDG uptake (SUVmax 13.0) and central photopenic areas within the mass ().\nIn addition, the PET and PET-CT fused images in transaxial projections demonstrated an intense focal FGD uptake within the right thyroid lobe with (SUVmax 10.0) (). There was a low-grade FDG uptake in the distal portion of esophagus with SUVmax 3.0 suggestive of gastroesophageal reflux disease. The left kidney was dislocated and was identified in horizontal position immediately below the inferior pole of the suprarenal mass. Otherwise, the scan showed no other abnormal FDG uptake throughout of the body.\nHe underwent laparotomy and total left adrenalectomy was done and histopathological results revealed adrenocortical carcinoma, oncocytic type (pT2), (). Subsequently fine needle aspiration biopsy of the thyroid nodule showed papillary thyroid cancer, and total thyroidectomy was performed. Pathology specimen revealed well-differentiated papillary thyroid cancer, classic type (pT1N0), (). | [[37.0, 'year']] | M | {'26071763': 1, '27094616': 1, '29403439': 1, '25722719': 1, '26793559': 1, '22355500': 1, '22385288': 1, '11752075': 1, '26279997': 1, '23179777': 1, '7666591': 1, '33578929': 1, '29310349': 1, '11762719': 1, '29998153': 2} | {} |
1,112 | 6038976-1 | 29,998,152 | comm/PMC006xxxxxx/PMC6038976.xml | Utility of 99mTc-Sestamibi SPECT/CT in the Early Localization of Metastatic Parathyroid Carcinoma | A 63-year-old female was admitted at our institution due to generalized body weakness and anorexia. She is a known hypertensive with chronic kidney disease. Upon further work-up, she was assessed to have severe hyponatremia (116 mmol/L), hypokalemia (2.8 mmol/L) and hypomagnesemia (1.2 mg/dL). Ionized calcium was 1.82 mmol/L (reference range: 1.00–1.30 mmol/L), serum PTH was 3071.90 pg/mL (reference range: 14.0–72.0 pg/mL), and serum vitamin D was 35.52 ng/mL (reference range: ≥ 30 ng/mL). Because of the clinical suspicion for primary hyperparathyroidism, a parathyroid scan was requested for further evaluation.\nParathyroid scintigraphy was performed using a dual-tracer protocol and with SPECT/CT as per institutional standard procedure. Planar imaging of the neck and thorax was done 15 minutes after intravenous injection of 173.9 MBq of 99mTc-pertechnetate. Planar and SPECT/CT images of the neck and thorax were acquired 15 minutes after injection of 769.6 MBq of 99mTc-sestamibi. Delayed 2- and 4-hour planar images were also obtained.\nStatic and subtraction images are illustrated in The 99mTc-pertechnetate image showed decreased tracer uptake in the inferior half of the right thyroid lobe but fairly homogeneous tracer uptake in the left lobe. The initial 99mTc-sestamibi planar image revealed a large focus of increased tracer activity in the inferior pole of the right lobe. The said focus showed retained activity on subtraction images and was found to arise from a solid mass with calcification on SPECT/CT (). Incidentally, a fainter focus of tracer uptake was noted in a soft tissue mass on the lateral aspect of the 7th right rib ().\nDelayed 2- and 4-hour planar images () showed tracer retention in the inferior pole of the right lobe. Symmetric tracer clearance was seen in the rest of the thyroid parenchyma. Physiologic tracer uptake was seen in the naso- and oropharynx, salivary glands, myocardium and liver dome.\nHistopathologic correlation was recommended for the sestamibi-avid mass with calcification in the inferior pole of the right thyroid lobe. This was attributed to a parathyroid carcinoma or adenoma, although a thyroid pathology was also possible. A similar recommendation was made for the incidental sestamibi-avid soft tissue mass in the lateral aspect of the 7th right rib, as it was highly suspicious for metastasis.\nThe patient was subsequently given intravenous furosemide for calcium correction. Intravenous saline solution was titrated for sodium correction. Intravenous potassium chloride, then later oral potassium citrate, was given for potassium correction. Magnesium sulfate infusion was given to correct for magnesium. After 10 days of admission, the patient was deemed to have acceptable electrolyte levels (ionized calcium 1.39 mmol/L; sodium 136.9 mmol/L; potassium 4.4 mmol/L; magnesium 2.2 mg/dL) and no subjective complaints. She was thus discharged and started on Cinacalcet, a calcimimetic drug, to address the patient’s hyperparathyroid state.\nShe underwent total thyroidectomy in another institution two months post-discharge. The final histopathologic diagnosis was as follows:\nFavor a parathyroid carcinoma, right lobe. Lymphovascular space invasion seen. Negative for tumor: surgical resection margins. Recommend immunohistochemical staining.\nPapillary microcarcinoma (0.2 cm greatest diameter), limited to the left lobe. Negative for extrathyroidal extension.\nMultinodular colloid goiter with hyperplastic features, isthmus and pyramidal lobe.\nHistopathologic slide review was done in our institution 7 months later. The final assessment showed an enlarged hypercellular parathyroid consistent with parathyroid carcinoma. The pathologist remarked that while the lesion is not mitotically active nor is there the presence of more recognizable definitive criteria for malignancy such as vascular and perineural invasion in the submitted material, the lesion shows extension beyond the capsule and encroaching on sizable extraglandular vessels.\nImmunohistochemical staining was also performed on slide review and was said to substantiate the above findings. The specimen was positive for chromogranin and negative for synaptophysin, thyroglobulin and calcitonin. Ki-67 was found to be less than 5% (low index of proliferation).\nThe patient was lost to follow-up after surgery. Seven months later, she was re-admitted at our institution due to alleged behavioral changes. Serum sodium, potassium and calcium were within normal limits. Repeat PTH was noted to be 438.70 pg/mL. Once she was stabilized, she was referred to the Medical Oncology service for possible chemotherapy initiation. A bone scan was subsequently requested for metastatic work-up.\nWhole body bone scintigraphy in the anterior and posterior projections, with additional views of the head, thorax and pelvis, was performed 2.5 hours after intravenous injection of 1054.5 MBq of 99mTc-methylene diphosphonate (MDP). SPECT/CT of the head and chest was also done.\nPlanar and SPECT/CT images are shown in and , respectively. The kidneys appeared faint on planar images. Foci of increased tracer uptake were noted in the following areas:\nright side of the sternum (mild focus corresponding to mild bone erosion)\n4th left rib\n7th right rib (corresponding to a soft tissue mass on SPECT/CT, previously noted to be sestamibi-avid on parathyroid scintigraphy)\n9th to 11th right ribs (uptake in the 10th rib corresponding to another soft tissue mass)\n8th left costovertebral junction\nsacroiliac junctions (photopenic foci surrounded by a tracer-avid rim) and left iliac bone\nright lesser trochanter\nThe above findings were assessed to be consistent with metastatic bone disease. | [[63.0, 'year']] | F | {'26354493': 2, '22043238': 1, '22327883': 1, '22022662': 1, '11157996': 1, '34302628': 1, '28644425': 1, '15565599': 1, '20510594': 1, '29998152': 2} | {'4722419-1': 1} |
1,113 | 6039156-1 | 30,013,861 | comm/PMC006xxxxxx/PMC6039156.xml | Wernicke’s Encephalopathy as a Rare Complication of Hyperemesis Gravidarum: A Case Report and Review of Literature | A 36-year-old Caucasian female, 16 weeks into her pregnancy, presented with intractable nausea, non-bloody emesis, and poor oral intake for the prior two months. She initially presented to an outside facility for an evaluation of loss of consciousness (LOC) and mild abdominal pain. The LOC was found to be secondary to syncopal episode. The initial blood workup was significant for leukocytosis (11.54L), hyponatremia (130 mmol /L), and hypokalemia (2.5 mmol /L). Her aspartate aminotransferase (AST) was 496 U/L, alanine aminotransferase (ALT) was 1280 U/L, and alkaline phosphatase (ALP) was 76 U/L. Lipase levels were also elevated to 83 U/L. Urine analysis was significant for ketonuria. The patient was transferred to our tertiary care facility for the management of electrolyte imbalance and acute pancreatitis with a presumed diagnosis of HG. She had an ultrasound (US) of the abdomen, which revealed mild hepatic steatosis. The patient was treated symptomatically with antiemetics, pain medications, and intravenous (IV) fluids. She had poor oral intake during the hospital stay, and her blood sugar was in the range of 50-70 mg/dL. She was started with intravenous (IV) dextrose to treat her hypoglycemia. Within 48 hours after starting IV dextrose, she developed confusion and started having problems with learned memories. She also complained of blurry vision and horizontal double vision that was worse when she turned her head toward the right side. She also had a subjective sensation of constant somersaulting and reported well-formed visual hallucinations. Her physical examination showed spontaneous upbeat nystagmus (Video ), gait instability, and ataxia with a tendency to lean to the left side while walking.\nMagnetic resonance imaging (MRI) of the brain, as shown in Figure , revealed symmetrical T2/fluid attenuated inversion recovery (FLAIR) hyperintense signals on bilateral medial thalamic regions, more pronounced on the left along with a diffusion restriction in the same regions. Figure shows an axial view of the diffusion-weighted imaging (DWI) sequence, which revealed cytotoxic edema in regions of the bilateral thalamus with corresponding apparent diffusion coefficient (ADC) changes.\nShe also underwent a magnetic resonance venogram (MRV) that did not show venous sinus thrombosis. Blood was drawn for thiamine levels before the replacement, which showed a low thiamine level of 40 nmol/L (normal range: 70-180 nmol/L). She was treated with high dose IV thiamine 400 mg for three days followed by 100 mg per day. At the time of discharge, her memory and gait instability improved significantly. However, the patient had persistent upbeating nystagmus. She was discharged to inpatient rehabilitation with daily oral thiamine replacement. The patient was re-evaluated from three months of diagnosis in our outpatient clinic and was noted to have a persistent upbeating nystagmus (Video ). She no longer had any major deficits in memory and/or gait.\nWe also obtained a repeat MRI of the brain during this follow-up that showed a significant resolution of previously identified bilateral hyperintense signals in bilateral medial thalamic regions on T2/FLAIR sequence (Figure ). As shown in Video , at six months follow-up, her spontaneous vertical nystagmus had resolved, but she continued to have gaze-evoked horizontal and vertical nystagmus. | [[36.0, 'year']] | F | {'8933197': 1, '27597910': 2, '28641304': 1, '32983708': 2, '19151161': 1, '27763485': 1, '20642790': 1, '7596325': 1, '25564426': 1, '21601782': 1, '30013861': 2} | {'4997071-1': 1, '7515211-1': 1} |
1,114 | 6039213-1 | 30,013,869 | comm/PMC006xxxxxx/PMC6039213.xml | Acute Psychosis After Treatment of Epididymitis with Ciprofloxacin | The patient is a 36-year-old African American male who initially presented to the emergency department with a chief complaint of feeling confused for the past day. He also complained of depression, anxiety, and paranoia. The patient declined psychiatric evaluation and medical admission at that time and was discharged home. The patient was urged to come back to the hospital by his wife and mother approximately 14 hours later due to disorientation, confusion, and agitation. At that time, he was admitted for an altered mental status workup with the working diagnosis of medication-induced delirium. Psychiatry was consulted to the medical floor and one-to-one observation was recommended. The patient underwent a full neurologic workup, including a computed tomography (CT) scan of the head and a lumbar puncture, which were unremarkable. The patient elected to leave against medical advice after two days. He presented to the emergency department two days later with similar complaints. At that time, all labs, including urinalysis, complete metabolic panel, urine drug screen, complete blood count, and vital signs, were within normal limits, and he was medically cleared and evaluated by psychiatry in the emergency department.\nDuring this evaluation, the patient was calm and cooperative. He reported being diagnosed with a “testicular infection” and was prescribed a course of ciprofloxacin, 500 mg for 10 days. He reported that he was not thinking clearly after starting the antibiotic and stated that he had “some side effects” but was too paranoid to elaborate. The medication was discontinued during his previous admission. He was unable to provide any details regarding the reason for admission and stated that it was his wife’s idea to come to the hospital. The patient appeared internally preoccupied, guarded, and paranoid throughout the evaluation process. He denied any auditory or visual hallucinations. The patient once again requested to sign out of the hospital against medical advice. The patient was paranoid about what the medical staff was doing with his blood and stated he did not feel safe in the hospital. Collateral information was obtained from the patient’s wife who reported that the patient's behavior changed abruptly after starting the ciprofloxacin. She reported that the patient was very paranoid and accused her of attempting to poison him.\nThe patient had no previous psychiatric history. The patient reported marijuana use since age 17. He recently stopped using marijuana two weeks prior to treatment for the diagnosis of epididymitis; he had used approximately one gram every other day prior to stopping. Social history revealed the patient is married with three children and employed as a construction worker. The patient denied any previous medical or surgical history.\nThe differential diagnosis included medication-induced delirium, drug-induced psychosis, cannabis-induced psychotic disorder, and brief psychotic disorder. The patient was admitted to the psychiatric inpatient unit. After two days of hospitalization and five days after discontinuing ciprofloxacin, the patient’s paranoia resolved and he was discharged to outpatient follow-up. | [[36.0, 'year']] | M | {'1504404': 1, '1452578': 1, '9293187': 1, '2686432': 1, '12927330': 1, '16871474': 1, '2012096': 1, '7604468': 1, '17478598': 1, '23570031': 1, '30013869': 2} | {} |
1,115 | 6039215-1 | 30,013,867 | comm/PMC006xxxxxx/PMC6039215.xml | Brachial Artery Coiling: Report of a Rare Case | An 81-years-old female presented in the emergency room of our hospital with clinical and electrocardiography signs of non-STEMI. A coronary angiogram was planned. Allen's test was performed and the TR approach was chosen. The radial artery was successfully accessed with a 6F radial sheath. A 5F Tiger catheter (Terumo Corporation, Somerset, New Jersey) was introduced through a 0.035 guide wire, but we felt resistance in the wire progression at the level of the cubital region. The wire was removed and a retrograde contrast injection was given to visualize the obstruction. We found a loop of 360 degrees of the BA just proximal of the bifurcation with initial spasm (Figure ).\nA 0,035 hydrophilic coated guidewire passed through, but the catheter advancement was difficult and accompanied with pain. Thereafter, we used an alternative transfemoral (TF) access with a successful outcome. A critical left anterior descending artery stenosis was successfully treated with a drug-eluting stent implantation. | [[81.0, 'year']] | F | {'16229800': 1, '27278701': 1, '13877204': 1, '11117624': 1, '4704042': 1, '24123805': 1, '30013867': 2} | {} |
1,116 | 6039216-1 | 30,013,874 | comm/PMC006xxxxxx/PMC6039216.xml | A Left-sided Azygos Vein in a Cadaver: Anatomical and Surgical Considerations | During a routine dissection in our Department of Anatomy and Surgical Anatomy, we encountered the presence of the AV on the left side of the vertebral column (Figure and Figure ].\nThe dissection was conducted on an 80-year-old formalin-fixed female cadaver, used for educational and research purposes, whose death was unrelated to the present case report. Specifically, after the meticulous dissection of the thorax region and the mediastinum, and after the excision of both lungs and the heart by means of the classical method of anatomical dissection, we detected an enlarged left-sided AV with maximum diameter 11.05 mm approximately at its midportion. The specific cadaver displayed hemiazygos and accessory hemiazygos veins in the expected course in the mediastinum, whereas the thoracic duct was noted ascending normally through the posterior mediastinum between the azygos vein and the thoracic aorta. In particular, at the level of tenth, ninth, and eighth thoracic vertebra the AV lay on the left side of the spine and the distance between the vessel and the midline of the vertebral column was 3.91 mm at the tenth, 5.75 mm at the ninth and 2.69 mm at the eighth thoracic vertebra. From the twelfth to tenth thoracic vertebra the AV lay on the midline of the spine, whilst from the seventh thoracic vertebra to its end in the superior vena cava, the AV was found as expected at the right side of the vertebral column. Our finding was documented by several photographs taken using a Nikon D3400 digital camera, and the measurements were made using a digital vernier caliper with an accuracy of 0.01 mm. No other congenital anomalies, variations or pathological conditions, or evidence of previous surgical interventions in the region were present. | [[80.0, 'year']] | F | {'13727912': 1, '26448373': 1, '31028449': 1, '24442867': 1, '18998005': 1, '14655133': 1, '30013874': 2} | {} |
1,117 | 6039219-1 | 30,013,871 | comm/PMC006xxxxxx/PMC6039219.xml | Long-term Survival After Treating Cardiac Metastasis With Radiation and Immune Therapy: A Case Report | A 51-year-old male was diagnosed with muscle-invasive urothelial cell carcinoma in May 2015. Standard neoadjuvant chemotherapy was given followed by radical cystoprostatectomy. Final pathology showed pT3 N0 M0, stage III disease. He remained disease free until April 2016, at which point he developed new exertional dyspnea and a small lung nodule was noted on imaging. During cardiac clearance for a biopsy, a Mobitz 2 heart block with bradycardia was noted. Transthoracic echocardiogram (ECG) demonstrated a mass in the right ventricular outflow tract, which was additionally found to be fluorodeoxyglucose (FDG)-avid on positron emission tomography (PET) computed tomography (CT) (Figure , upper panel).\nThe patient developed progressive dyspnea with minimal exertion and then experienced a syncopal episode prompting hospitalization. Biopsy of the right ventricular mass demonstrated poorly differentiated carcinoma, consistent with urothelial origin (Figure ).\nThe mass was not felt to be respectable and chemotherapy was not felt to offer rapid disease control in the setting of progressive symptoms. He was offered palliative radiotherapy. His bradycardia progressed to a complete heart block, likely due to the growth of the mass, necessitating placement of a dual chamber pacemaker. The right ventricle mass was treated with 45 Gy in 18 fractions (3D conformal photons for 5 fractions, followed by intensity-modulated radiotherapy for the remaining 13 fractions (to reduce dose to the left ventricle). Figure demonstrates the dose distribution.\nProton radiation was considered to spare the uninvolved myocardium. He had substantial tumor thromboembolic disease to his lungs during radiotherapy, requiring medical intensive care unit (MICU) admission for symptom management. He ultimately completed the full course of radiotherapy which improved his functional status.\nFollowing radiotherapy, he began immune checkpoint therapy with the PD-L1 antagonist atezolizumab (1200 mg every three weeks), which was well tolerated. A PET/CT scan seven months after completing radiation demonstrated a complete metabolic response in the right ventricle mass and no other sites of progression (Figure , lower panel). The previously noted pulmonary nodules were stable. He resumed normal activity and returned to work. Eight months after radiotherapy and six months into his immune therapy, he developed progressive shortness of breath. He had heart failure and the ejection fraction reduced to 20%-30%. Cardiac magnetic resonance imaging (MRI) revealed a focal area of subendocardial delayed enhancement, potentially representing myocarditis. Cardiac catheterization revealed extensive coronary artery disease and complete occlusion of the right coronary artery; however, the left to right collaterals were intact. Atezolizumab was held with an initiation of high dose steroids to treat potential immune-mediated myocarditis with minimal effect. Electrophysiology testing demonstrated severe cardiac dyssynchrony. Cardiac resynchronization therapy was recommended and a biventricular pacer was placed with rapid symptom relief. He returned to work with overall minimal symptoms. Atezolizumab was resumed, and a total of 18 cycles (54 weeks) of therapy were completed without issue.\nIn the months that followed the placement of his biventricular pacer, he was evaluated several times for shortness of breath and atypical chest pain, however with no clear etiology. PET/CT imaging in August 2017 demonstrated increased FDG-avidity in his right ventricle concerning for progression. An MRI was ordered and in early September 2017, he was admitted for workup of an exacerbation of chest pain. A contrast-enhanced CT of the chest showed significant disease progression in the area of prior treatment, extension toward the left ventricle and along the heart wall leading to increased right heart pressure (Figure ), and probable encasement of his left anterior descending (LAD) artery by disease progression (Figure ). He had acute progression of chest pain associated with an increased oxygen requirement, shortly after being admitted. His ECG showing ST elevations in the anterior leads, consistent with an LAD infarct (Figure ). The cardiology service was consulted to consider palliative LAD stenting; however, the patient underwent cardiac arrest in the interim and did not wish to be resuscitated. He died 17 months after his disease recurrence. | [[51.0, 'year']] | M | {'33546060': 2, '33445506': 2, '14998838': 1, '21247968': 1, '25036983': 1, '28212060': 1, '33204858': 1, '20669802': 1, '27939400': 1, '26952546': 1, '28081914': 1, '18704641': 1, '20231682': 1, '26880683': 2, '30013871': 2} | {'4924355-1': 1, '4924355-2': 1, '4924355-3': 1, '7903268-1': 1, '7837973-1': 1} |
1,118 | 6039220-1 | 30,013,866 | comm/PMC006xxxxxx/PMC6039220.xml | Probable Lewy Body Dementia with a Predilection for Auditory Hallucinations | The patient is a 60-year-old male who initially presented to the emergency room in 2016 with a long history of perceptual disturbances, mood symptoms, and religious preoccupation. He was brought to the emergency department due to extreme agitation aggravated by internal stimuli of women professing their love to him. The voices were so overwhelming that, at times, he was unable to sleep. He also presented with visual hallucinations. He was very talkative and grandiose. He described that he will build a church for the homeless and put them to work. His speech was circumstantial and poorly goal-directed. His mini mental exam was a 30/30. His temperature was 97.7°F; pulse was 86/min; respiratory rate was 18/min; blood pressure was 100/55 mm Hg. His initial labs showed a white blood cell (WBC) count of 5.92 THOU/uL; hemoglobin of 15.4 g/dL; hematocrit of 43.6%; platelet count of 203 THOU/uL; sodium of 137 mmol/L; potassium of 4.1 mmol/L; creatinine of 1.0 mg/dL; glucose of 96 mg/dL; calcium of 9.7 mg/dL; aspartate aminotransferase (AST) of 12 U/L; alanine aminotransferase (ALT) of 11 U/L; thyroid-stimulating hormone (TSH) of 1.55 uIU/mL. The urine toxicology screen was negative and rapid plasma reagin (RPR) was nonreactive. The physical exam was unremarkable besides the mood, hallucination, and paranoia symptoms. He was believed to have unspecified schizophrenia, bipolar disorder with psychotic features, or schizotypal personality disorder. He was started on risperidone 0.5 mg BID and was increased to 2 mg BID. Trazodone was initiated for sleep and lithium 300 mg BID for mood stabilization was added throughout the course. His condition improved and he was discharged.\nApproximately 16 months later, he was brought in by emergency petition because he was trying to enter a stranger’s car. He mistook the stranger’s car for his own. Upon admission, the patient was unable to provide an accurate history due to confusion and memory impairment. He reported no new visual or auditory hallucinations after being discharged in 2016. He also responded to most questions with “I don't remember.” Vitals at the time were temperature: 97.6 °F; pulse: 96/min; respiratory rate: 18/min; and blood pressure: 97/62 mm Hg. Initial laboratory tests showed WBC count: 6.7 THOU/uL; hemoglobin: 14.3 g/dL; hematocrit: 40.7%; platelet count: 215 THOU/uL; sodium: 128 mmol/L; potassium: 2.8 mmol/L and 4.2 mmol/L when repeated; creatinine: 0.7 mg/dL; glucose: 95 mg/dL; calcium: 8.7 mg/dL; AST: 12 U/L; ALT: 10 U/L; TSH: 1.9 uIU/mL; urine toxicology screen: normal; and RPR: negative. He presented with reduced psychomotor activity and organized thinking as opposed to last years' visit. His prior discharge medication was started and 1 mg of Lorazepam PRN was added for anxiety. During this hospital stay, confusion and psychomotor retardation were noted. In response, his risperidone dose was decreased with no change in psychotic symptoms; however, he began developing extrapyramidal side effects. He displayed involuntary movements of the mouth, flailing movements, restlessness, and a patting of his head. Risperidone was replaced by zyprexa due to the decreased likelihood of extrapyramidal symptoms. After observation, the patient was discharged from the hospital.\nApproximately one and a half months later, he was found unresponsive and catatonic in his car and readmitted to the hospital. His vital signs were temperature: 99 °F; pulse: 119/min; respiratory rate: 16/min; blood pressure: 112/85 mm Hg; and pulse oximetry on room air: 97%. His labs were WBC: 14.8 THOU/uL; hemoglobin: 17.2 g/dl; hematocrit: 52.3%; platelet count: 276 THOU/uL; sodium: 143 mmol/L; potassium: 3.9 mmol/L; creatinine: 0.7 mg/dL; glucose: 164 mg/dL; calcium: 9.6 mg/dL; AST: 22 U/L; and ALT: 14 U/L. During this stay, the patient appeared to be having latency in speech before responding to inquiries, but some days were significantly worse than others. He would be aware of his location but unable to explain why or for how long. He had a difficult time remembering names. He was still exhibiting abnormal involuntary movements around the mouth and extremities. Magnetic resonance imaging (MRI) was ordered to rule out stroke and vascular dementia; however, there was no acute intracranial pathology, as seen in Figure , and an unremarkable magnetic resonance angiogram (MRA) of the Circle of Willis, as seen in Figure .\nHis carotid ultrasound was unremarkable as well (Figure ).\nHis history of late-onset auditory and visual hallucinations, sensitivity to neuroleptics, extrapyramidal symptoms, limited judgment, fluctuating cognition, and frequent emergency room (ER) visits due to altered mental status and deficiencies in the visual-spatial cognitive function may suggest Lewy body dementia. The patient was observed for a few days and was determined to have capacity before being discharged on zyprexa, ativan, and lithium. | [[60.0, 'year']] | M | {'22033743': 1, '11841138': 1, '15264967': 1, '30013866': 2} | {} |
1,119 | 6039222-1 | 30,013,872 | comm/PMC006xxxxxx/PMC6039222.xml | The Emergence of a Forgotten Entity: Dermatomyositis-like Presentation of Lyme Disease in Rural Wisconsin | A 76-year-old Caucasian female presented to the emergency department (ED) with an acute exacerbation of left-sided shoulder pain. Her history was significant for chronic degenerative joint disease of shoulder and hip joints. She denied prior autoimmune disorders including rheumatologic or inflammatory myopathies. Physical exam was unremarkable and preliminary workup was negative. Myocardial infarction was ruled out and she was discharged home after intra-articular steroid injection. Over the next two weeks, she developed fatigue, malaise and progressive proximal muscle weakness. She reported a 5-pound weight loss over one-week and had mild dyspnea both at rest and exertion but no functional limitations. She denied cough, paroxysmal nocturnal dyspnea or orthopnea. She noticed upper eyelid edema and a periorbital violaceous rash, prompting her to visit a walk-in clinic. She lived in rural WI but reported no tick bites or prior LD. She had no neurological complaints and there was no evidence of EM. She was discharged with oral prednisone 20 mg daily. After few days, she returned to the ED with persistent complaints despite steroid treatment. Vitals were stable upon arrival, but she appeared lethargic. There were no signs of distress and oxygen saturation was normal on room air. The physical exam showed a positive heliotrope rash and a positive malar rash involving both nasolabial folds. She had a macular erythematous rash of extensor surface of forearms. Moreover, a widely distributed erythema of the upper neck, extending to the upper back and upper shoulders indicating a “Shawl sign” was noted. A generalized cutaneous erythema with edema involving the abdominal wall was also present. The musculoskeletal exam revealed symmetric pelvic and shoulder girdle weakness; examination of shoulder joints was unremarkable. Nervous system exam was negative for focal neurological deficits. Labs showed leukocytosis, elevated erythrocyte sedimentation rate and elevated C-reactive protein (Table ).\nAlanine aminotransferase was elevated; creatine kinase was normal. Antibodies including anti-cyclic citrulline peptide antibody, antimitochondrial antibody and anticardiolipin IgA and IgM were positive; anti-JO-1 antibody was negative. Chest radiograph showed no lung pathology. Computed tomography (CT) of the chest revealed interstitial lung disease but no malignancy. Electrocardiogram showed sinus rhythm with few premature atrial complexes. The patient’s clinical presentation mimicked DM. Moreover, there was a suspicion of LD due to its increased prevalence in WI. Therefore, an enzyme-linked immunosorbent assay (ELISA) was ordered, and the patient was started on IV ceftriaxone as empiric treatment. After receiving empiric antibiotic, the patient appeared toxic, became hypotensive and developed high-grade fever. However, the worsening of cutaneous lesions was not observed. Given clinical deterioration, blood cultures were drawn, and she received fluid resuscitation. She was transferred to the intensive care unit (ICU) for close monitoring. While in ICU, two target-shaped lesions were noted on her left scapula, highly suggestive of LD-related EM; they were not previously reported or observed. The suspicion of LD became paramount leading to the addition of IV doxycycline given the patient’s deteriorating condition. After few doses of antibiotics, the patient started showing signs of clinical improvement; fever and hypotension resolved. She required no further antibiotic adjustments given her prompt response to antiborrelial treatment. She had positive ELISA for LD, later confirmed with western blot (WB). Blood cultures showed no growth. She received a total of five days treatment with IV ceftriaxone and IV doxycycline. Her DM-like presentation showed an effective response to antimicrobial treatment, and therefore, required no steroids or immunosuppressants. Her skin lesions were resolved, and muscle strength was significantly improved. Given her recovery with antiborrelial treatment, DM workup including EMG, skin biopsy (SB) or muscle biopsy (MB), was not performed. DM-like features were attributed to the LD. She was discharged home in a stable condition with oral doxycycline for two weeks. On her post-discharge follow-up, she showed no recurrent signs or symptoms of LD or DM-like clinical features. Six months after the completion of treatment, she remained in remission without recurrence of her symptoms. | [[76.0, 'year']] | F | {'9385698': 1, '8161622': 1, '17337098': 1, '26516401': 1, '29090371': 1, '20565951': 2, '31108976': 1, '1395222': 1, '32793606': 1, '9674723': 1, '7639814': 1, '30013872': 2} | {'2898701-1': 1} |
1,120 | 6039223-1 | 30,018,866 | comm/PMC006xxxxxx/PMC6039223.xml | Paradoxical Embolism in a Patient with Aortic Valve Endocarditis: A Case Report | A 35-year-old male with a past medical record of interventricular communication presented with progressive dyspnea for one month. He was admitted to the emergency department with vomiting and fever for the past five days. At physical examination, the patient exhibited tachycardia (122 bpm), blood pressure of 110/70 mmHg and an aortic systo-diastolic murmur, grade IV/VI. Chest X-ray revealed bilateral nodular infiltrates and laboratory exams showed the increase of nitrogenous compounds and metabolic acidosis. Transthoracic echocardiogram revealed left ventricular enlargement and aortic insufficiency, but no signs of endocarditis. Therefore, a transesophageal echocardiogram was performed (Figure ), showing aortic valve vegetation, ascending aorta dilatation, aortic regurgitation, and the interventricular communication. Blood cultures were drawn and empirical treatment for infective endocarditis with Meropenem and Linezolid initiated. Cultures turned out positive for oxacillin-sensitive Staphylococcus aureus.\nThe patient developed signs of peripheral embolization (Figure -). Computed tomography (CT) scans showed evidence of lungs, brain, and splenic emboli (Figure -). Cranial tomography revealed 2-3 cm hypodense cortico-subcortical areas at the left parieto-occipital and right occipital regions, consistent with embolic infarctions. Abdominal tomography revealed peripheral hypodense areas in the spleen and a subcapsular hypodense area at the inferior pole of the right kidney, both images consistent with ischemic lesions.\nContrast-enhanced CT of the chest revealed subpleural cuneiform consolidation areas, some with central excavation, findings consistent with septic embolism. Moderate left pleural effusion was also seen.\nThe patient was submitted to cardiac surgery. During the procedure, a vegetation at the ventricular side of the aortic valve and an abscess along the aortic ring were found. Extracorporeal circulation was used for 3 hours and 10 minutes and multiple blood transfusions were taken. The patient arrived at the intensive care unit hemodynamically unstable under high doses of noradrenalin and dobutamine. Unfortunately, the patient died at the fifth post-operatory day due to refractory shock. | [[35.0, 'year']] | M | {'10430983': 1, '27364708': 1, '18064995': 1, '16460540': 1, '16294827': 1, '25060377': 1, '30018866': 2} | {} |
1,121 | 6039542-1 | 30,022,980 | comm/PMC006xxxxxx/PMC6039542.xml | A Case of Adult-Onset Still’s Disease Caused by a Novel Splicing Mutation in TNFAIP3 Successfully Treated With Tocilizumab | A 32-year-old female patient initially presented at the age of 16 with high fever and severe abdominal pain associated with anorexia, but not diarrhea and vomiting. Over the period of the next 4 years, she had multiple hospital admission with similar presentation, which was always accompanied with raised C-reactive protein (CRP). On few occasions her urine dipstick showed traces of protein, and consequently she was treated for presumed urinary sepsis. However, multiple blood and urinary cultures were negative, other investigations including abdominal ultrasound showed no obvious intraabdominal pathology, and the diagnosis remained inconclusive. At the age of 21, she developed sudden onset polyarthritis, fever, and wide spread erythematous skin rash. The routine investigations at the time showed highly elevated acute phase response with CRP > 200 mg/L and hyperferritinemia. Additional investigations including antinuclear antibody (ANA), rheumatoid factor (RF), and anti-citrullinated peptide antibodies (ACPA) were all negative. Diagnosis of AOSD was at that point established, based on the typical clinical features associated with hyperferritinemia and exclusion other autoimmune rheumatological diagnoses. Her condition improved following initial treatment with oral corticosteroids (prednisolone 40 mg daily), but she remained dependant on prednisolone for the disease control despite attempts to introduce disease-modifying antirheumatic drugs (DMARDs) such as hydroxychloroquine, azathioprine, and methotrexate, which were all largely ineffective. Over the next several years, she continued to suffer from inflammatory polyarthritis affecting predominantly the large joints (hips and knees). Prior to establishing her genetic diagnosis, the patient was eventually treated with tocilizumab to which she made an excellent response. She discontinued long-term corticosteroid treatment and remains well controlled on monotherapy with tocilizumab (IV 8 mg/kg monthly) for the last 2 years.\nSubsequent enquiries into her family history revealed that she has two children, a boy aged 6 and a half and a girl of age 4 years. Both children had history of unexplained inflammatory symptoms. The girl was symptomatic from the age of 8 months, at which point she begun having recurrent episodes of feeling generally unwell, with malaise and high fever up to 40°C. The frequency of these episodes varied between 2 and 4 weeks, and each episode was relatively short-lived, lasting anywhere between 2 and 3 days. On few occasions, the episodes of fever were associated with erythematous macular rash and mouth ulcers. Subsequently, she also developed episodic abdominal pain as well as difficulty walking due to the pain in her knees. All these symptoms would disappear when the fevers resolved and she would fully recover in between these episodes. Since commencing regular colchicine, she has not had any further inflammatory episodes or symptoms. Her brother was well until age 5 when, like his sister he started with monthly episodes of high fever lasting between several hours to several days. On few occasions, these episodes were associated with vomiting, mouth ulcers, and interestingly, salmon-pink skin rash. He was also commenced on colchicine, which in his case has not been as effective, and he is currently awaiting therapeutic trial of tocilizumab. Finally, the father of the index case also appears to have relevant clinical history. He was diagnosed with early onset RA, but the details of symptoms and investigations leading to his diagnosis are lacking since he is looked after by another hospital and he has minimal contact with his daughter with who he has not shared any further details regarding his health. | [[32.0, 'year']] | F | {'20530205': 1, '28469620': 1, '23059429': 1, '30766537': 1, '31769858': 1, '33745335': 1, '21841782': 1, '25607459': 1, '29362479': 1, '32107482': 1, '21341261': 1, '34030699': 1, '19643665': 1, '26642243': 1, '31625129': 1, '32733444': 1, '29163546': 2, '31164164': 2, '34899744': 1, '29515565': 1, '29317407': 1, '11009421': 1, '27535533': 1, '24246475': 1, '31427375': 1, '25043000': 1, '34262681': 1, '29241730': 1, '28659290': 1, '29678940': 1, '29163488': 1, '21088135': 1, '30619251': 1, '27845235': 1, '30022980': 2} | {'6549368-1': 1, '6549368-2': 1, '5681747-1': 1} |
1,122 | 6039581-1 | 29,953,023 | comm/PMC006xxxxxx/PMC6039581.xml | Abdominal pain as the presenting symptom of Takayasu arteritis in an adolescent male | On May 3, 2018, a 13-year-old boy presented to the general surgery outpatient department of our hospital with a history of intermittent right abdominal pain for one week. He had no history of fever or emesis. Physical examination revealed no abdominal distension or tenderness, and muscle tension was not palpated. Abdominal ultrasonography showed that the right lower quadrant and urinary system were normal. No special treatment was performed, and follow-up was recommended. After 5 days, the boy presented to the general surgery emergency department for aggravating abdominal pain with emesis. Physical examination revealed abdominal tenderness in the left upper abdomen; however, muscle tension was not palpated.\nRoutine blood examination showed a white cell count of 14.71 × 109/L (reference range 4–10 × 109/L) with 67.2% neutrophils and C-reactive protein of 59.65 mg/L (reference range < 8 mg/L). An abdominal computed tomography (CT) scan depicted a retroperitoneal occupying lesion in the upper abdomen accompanied by an abdominal aorta dilatation (Fig. ). The child was then admitted to the department of surgical oncology. On admission, the boy's medical history was found to be unremarkable, and his family history contained nothing of significance. Blood pressure (BP) measurement showed that BP in the right upper limb was 178/94 mm Hg. Laboratory tests revealed the following: white cell count, 9400/μL with 62.8% neutrophils; CRP, 46 mg/L; erythrocyte sedimentation rate (ESR), 23 mm/h (reference range 0–21 mm/h). Computed tomography angiography (CTA) of the aorta showed an aortic pseudoaneurysm (Fig. ). The boy was transferred to the pediatric intensive care unit (PICU) for further treatment. An urgent consultation was held with the department of vascular surgery of Zhongshan Hospital of Fudan University. An emergency aortic stent implantation and aneurysm embolization were performed the next morning, and the intraoperative diagnosis was aortic pseudoaneurysm (Fig. ).\nAfter the operation, nicardipine hydrochloride and metoprolol were introduced to lower the patient's BP. A consultation with the department of rheumatism determined the diagnosis to be TA, based on the boy's medical history, physical examination, and auxiliary examination results. The patient was then transferred to the department of rheumatism for treatment on May 9. Treatment with prednisolone was initiated (48 mg daily). Five days after the boy received intravenous prednisolone, cyclophosphamide was administered at a dose of 600 mg. Six months after the initial presentation, he was doing well clinically with no additional vascular involvement, and his BP was stable with oral antihypertensive drugs. | [[13.0, 'year']] | M | {'34506410': 2, '1975175': 1, '25879697': 1, '20413568': 1, '34050525': 1, '24955077': 1, '19720487': 1, '20562196': 1, '32424657': 1, '26585174': 1, '7909656': 1, '30985685': 1, '9119518': 1, '20541299': 1, '18413441': 1, '23626437': 1, '22640655': 1, '29953023': 2} | {'7775015-1': 1} |
1,123 | 6039600-1 | 29,952,977 | comm/PMC006xxxxxx/PMC6039600.xml | Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult | A 41-year-old man was accidently detected with a mass without tenderness on the right lobe of the thyroid. B-ultrasound showed that the morphology and echo of thyroid were abnormal though without nodules. Magnetic resonance imaging (MRI) showed significantly enlarged right lobe of the thyroid with a clear boundary and uneven density of mass, partly involving the isthmus of the thyroid, which was suspected as being thyroid adenoma (Fig. ). The thyroid function testing and emission computed tomography (ECT) scan were normal. Physical examination showed the following: right lobe of the thyroid showed no-fixed II degree swelling with no tender and obvious nodules. The liver and spleen were normal. Bone marrow biopsy was normal and the percentage of eosinophils in peripheral blood was 2.97% (normal range, 0.5%–5%). The patient had a history of hypertension for 7 years and hepatitis for 10 years. There was no family history of thyroid diseases and radiation ray contact. The patient underwent a right thyroid resection and dissection considered the possibility of thyroid adenoma. However, the pathology after surgery indicated LCH because immunohistochemistry staining confirmed CD1a (+), S100 (+), CD68 (+), Valentine (+), and thyroglobulin (+). The thyroid lesion gradually improved after the patient further received radiotherapy of 16 regimens combined with interleukin-2 therapy. The patient regularly monitored the function of thyroid and began to take 50 μg Euthyrox (levothyroxine) per day since being diagnosed with hypothyroidism 3 years after subtotal thyroidectomy. In 2007, the patient was again admitted to our hospital with the complaint of thirst and polyuria for several months. Urine osmolality was obviously lower than blood osmolality and urine specific gravity was less than 1.005. Further, fluid restriction test was positive and MRI of the pituitary revealed loss of the pituitary posterior lobe signal and the pituitary stalk was slightly thicker (Fig. ). We considered the diagnosis of central diabetes insipidus (DI) and the patient received 3 tablets of desmopressin acetate (0.3 mg/day) with improvement of the symptoms. However, the thyroid and total bone ECT scan were both normal.\nIn 2008, the patient felt pain and distension in the upper abdominal area and was admitted to the hospital. Abdominal computed tomography (CT) showed diffuse fine nodules on the right hepatic with partly fusion. Immunohistochemical staining showed there were Langerhans cells with ovoid to reniform nuclei with grooves by percutaneous liver biopsy (Fig. ), meanwhile in which the histiocyte-like cells S-100 (+) and CD1a (+) diagnosed as liver Langerhans cell. Liver function testing revealed that alkaline phosphatase (ALP) was 233 U/L (normal range, 40–160 U/L), gamma-glutamyl transaminase was 220 U/L (normal range, <50 U/L) and total bilirubin, liver transaminase and alpha-fetoprotein were in the normal range. Further, the patient received chemotherapy of CHOP regime (CTX1350 mg, VCR2 mg, prednisone 100 mg for 5 days,) and radiation therapy of 11 regimens including 16.5 Gy dose. Surprisingly, we found that the hepatic local region in the patient had remarkably improved and finally was normal during 5 years of follow-up. There was no lesion found in the right lobe during the T1-weighted MR images in 2012 and enhanced MR images of the liver in 2017 (Fig. ). We considered the patient in clinical remission while the examination of thyroid and pituitary was favorable. | [[41.0, 'year']] | M | {'9561904': 1, '17600725': 1, '24436311': 1, '20188480': 1, '33140265': 1, '2334209': 1, '17455311': 1, '17111302': 1, '9251735': 1, '10770168': 1, '17458874': 1, '22198822': 1, '12847329': 1, '34093427': 2, '25329766': 1, '24147236': 1, '26500997': 1, '9561899': 1, '16047354': 1, '31061179': 1, '16804933': 1, '32185056': 2, '14764769': 1, '29952977': 2} | {'7069888-1': 1, '8170468-1': 1} |
1,124 | 6039602-1 | 29,952,960 | comm/PMC006xxxxxx/PMC6039602.xml | Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease | A 50-year-old woman was admitted to our hospital on October 2016 complaining of sicca syndrome, slight pain, and bilateral enlargement of parotid glands for 18 months; afterwards, a progressive bilateral periorbital swelling began. Patient did not report fever, cough, or any respiratory symptom. In 2015, Sjogren syndrome was diagnosed, according to clinical and histopathological American College of Rheumatology/European League Against Rheumatism classification criteria.[ Nevertheless, serum anti-nuclear, anti-SSA/Ro, anti-SSB/La antibodies and rheumatoid factor were persistently negative. Intermittent courses of low-doses steroids (prednisone 5–12.5 mg/d) and hydroxychloroquine 200 mg/d were ineffective. Therapy had been stopped 6 months before our observation due to inefficacy. In her past medical history a moderate asthma for few years, requiring treatment with long acting beta2 agents and inhaled steroids, was noteworthy. Physical examination revealed evident swelling of salivary and lacrimal glands and bilateral yellow eyelids xanthelasmas (Fig. A and B). In the suspicion of an underlying indolent lymphoproliferative disease a magnetic resonance imaging (MRI) exam was performed, showing patchy diffuse salivary and lacrimal glands enlargement together with the presence of multiple intra-glandular lymph nodes, without focal lesions (Fig. C). Moreover, a high-resolution computed tomography (HRCT) scan of the chest revealed a nodular lesion (diameter 3 cm) at the inferior right pulmonary lobe, with irregular margins and air bronchogram in the context (Fig. C). Bronchoalveolar lavage did not show pathogenic microorganisms, nor atypical cells; mixed mononuclear population (macrophages and lymphocytes) emerged at microscopic examination (quantitative analysis not done). An endoscopic biopsy failed to obtain diagnostic material, due to the scarce opacity of the lesion at the fluoroscopic guide. A whole-body positron emission tomography (18FDG-PET-CT) showed a modest increase in fludeoxyglucose up-take of lymph nodes (right axillary [SUVmax = 2.0], right upper paratracheal [SUVmax = 3.1], Barety loggia [SUVmax = 2.8], and subcarinal [SUVmax = 4.5]), parotid glands, and oral-rhino pharyngeal mucosa (Fig. A). Laboratory findings confirm the absence of serologic markers of connective tissue diseases, as well as signs of systemic inflammation. Serum IgG4 level was high, almost 4 folds UNL (794 mg/dL). Peripheral blood flow cytometry showed elevated CD19+ CD38bright CD27+ CD20- plasmablasts concentration (2713 cells/mL). Complete laboratory features are summarized in Table . Histological examination of an intra-parotid lymph node confirmed clinical hypothesis of IgG4-related disease (Fig. E–J), according to the comprehensive diagnostic criteria 2012.[ After microbiological screening and informed consent, 1 cycle of 2 g rituximab (RTX) (1000 mg each 15 days apart together with methylprednisolone 100 mg single shot premedication) was administered. No chronic therapy was prescribed and no adverse effects were observed. Patient referred a rapid and complete improvement of exocrine glands swelling, and resolution of asthma, allowing bronchodilator therapy discontinuation, whereas xanthelasmas were still visible and unchanged. Remission persisted at the 12-months follow-up visit and was confirmed by MRI. Furthermore, neither the pulmonary lesion was visible at HRCT (Fig. D), or significant intra-thoracic 18FDG-uptake was detectable at PET control (Fig. B). IgG4-RD responder index[ (without IgG4 serum level) stepped down from 14 to 6, serum IgG4 levels roughly halved and plasmablasts concentration became undetectable (Table , Fig. ). | [[50.0, 'year']] | F | {'27152364': 1, '22218969': 1, '22316447': 1, '34305927': 1, '25481618': 1, '22611406': 1, '29381959': 1, '26512617': 2, '29279491': 1, '29253269': 1, '26683924': 1, '28701054': 1, '27789466': 1, '28091934': 1, '24817416': 1, '28915275': 1, '28684557': 1, '27368010': 1, '33869889': 1, '28856463': 1, '28614220': 1, '34448063': 1, '28165852': 1, '17198199': 1, '28858963': 1, '31392499': 1, '8140711': 1, '29952960': 2} | {'4985430-1': 1, '4985430-2': 1, '4985430-3': 1} |
1,125 | 6039603-1 | 29,953,016 | comm/PMC006xxxxxx/PMC6039603.xml | IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjögren's syndrome | A 69-year-old male farmer presented to our hospital for evaluation of mild left lower abdominal pain. The onset of symptoms occurred ten months prior to his admission to our hospital. The patient was previously admitted to another provincial hospital and misdiagnosed with abdominal aortic dissection. His symptoms were not improved following the administration of oral antibiotics and proton pump inhibitors.\nPhysical examination was unremarkable except for upper right abdomen and periumbilical mild tenderness. Complete blood counts revealed mild anemia (Hb 10.2 g/dL) with normal leukocyte and platelet counts. Biochemistry showed elevated levels of γ-glutamyltransferase (103.0 U/L, range: 7–45 U/L), alkaline phosphatase (144 U/L, range: 50–135 U/L), renal dysfunction [serum creatinine (113.99 μmol/L, range: 45–84 μmol/L)], and elevated levels of serum amylase (138 U/L, range: 15–125 U/L). The erythrocyte sedimentation rate was elevated at 84 mm/h (range: 0–20 mm/h). Total serum IgG levels were extremely high (18.9 g/L, range: 6.0–16.0 g/L), while IgG4 was approximately 6 times the normal limit (9.0 g/L, range: 0.08–1.4 g/L). Antinuclear antibody titer was 1:320, and his anti-SS-A antibody and antimitochondrial type 2 (M2) antibody were positive. Urinalysis showed a high level of beta-microglobulin, while proteinuria, occult blood, white blood cells and casts were within normal ranges. Other blood tests, including fecal occult blood, C-reactive protein, carbohydrate antigen, carcinoembryonic antigen, and alpha-fetoprotein, were all within normal ranges. No abnormalities were found in the complement system. The T-SPOT.TB test was negative.\nRadiographs of the chest were unremarkable. Contrast-enhanced abdominal computed tomography (CT) revealed left hydronephrosis and a periaortic mass (Fig. ). The mass surrounding the aorta appeared to be soft tissue rather than lymph nodes or tumor, suggesting left ureteral stenosis due to RPF, leading to hydronephrosis of the left kidney. Magnetic resonance cholangiopancreatography (MRCP) revealed no significant dilation of the common bile duct or the extra- and intra-hepatic bile ducts.\nThe patient underwent biopsy of the retroperitoneal mass using an autobiopsy gun under CT-guidance for further diagnosis of RPF. Penetrating tissue specimens stained with hematoxylin and eosin revealed significant infiltration of lymphocytoplasma cells, fibrosis and fibrin accumulation (Fig. A). However, immunohistochemical staining did not show IgG4-positive plasmacytes in the retroperitoneal mass (Fig. B). Despite this, IgG4-related RPF was suspected, and with patient agreement we performed a less invasive lip biopsy in an attempt to obtain pathologic evidence. Although his labial salivary gland appeared normal, and the patient exhibited mild clinical features such as xerostomia and xerophthalmia, histological examination revealed a decreased number of gland bubbles and heavy infiltration of lymphocytes (100/HFP) (Fig. C). IgG4 staining was again negative (Fig. D). Together with positive expression of anti-SS-A antibody and positive Saxon and Schirmer tests, all findings fulfilled the American–European Consensus Group classification criteria for pSS.[ In addition, the patient had intrahepatic cholestasis as well as the high serum level of antimitochondrial M2, and MRCP revealed neither strictures of the lower common bile duct nor a segmental stricture. Therefore, the presence of IgG4-SC was not determined. The patient was suspected to have underlying PBC.\nAfter ruling out of other possibilities, the patient was finally diagnosed with IgG4-related RPF and overlapping PBC complicated with pSS. With the patient's consent, 250 mg ursodeoxycholic acid was administered twice daily and 40 mg/d (1 mg/kg) of prednisone was administered for 30 days, which was then reduced to 30 mg/d for 15 days followed by 25 mg/d. At 45 days follow-up, the patient exhibited no signs of abdominal pain. A follow-up contrast-enhanced CT scan confirmed that the retroperitoneal soft tissue lesion was markedly reduced (Fig. ). The ESR decreased to 29 mm/h, and IgG4 reduced to 3.18 g/L. Antinuclear antibody titer was significantly decreased to 1:100, and other laboratory data, including serum creatinine and biliary enzymes, were almost normal. The patient then received a long-term maintenance dose of 10 mg/d prednisone after steroid tapering. At six-month follow-up, the patient had no disease recurrence. | [[69.0, 'year']] | M | {'21245628': 1, '18701557': 1, '19593223': 1, '22316447': 1, '22536256': 1, '11236777': 1, '25809420': 1, '15893711': 1, '21168884': 1, '32581168': 1, '22654917': 1, '10955606': 1, '12006334': 1, '21971969': 1, '26469909': 1, '25590841': 1, '24593168': 1, '22198501': 1, '19950407': 1, '21953287': 1, '19580797': 1, '27091321': 1, '11978339': 1, '23479712': 1, '23429355': 1, '21400555': 1, '26106212': 1, '23146570': 1, '12169990': 1, '22717980': 1, '29953016': 2} | {} |
1,126 | 6039606-1 | 29,953,008 | comm/PMC006xxxxxx/PMC6039606.xml | Concomitant tonsillar cyst and papilloma in larynx | A 34-year-old man was referred with abdominal pain, abdominal distension, and loss of appetite for 6 months. At his first visit to the Department of Gastroenterology, he was evaluated by diagnostic electronic gastroscopy, which detected a mass in his throat. He had no history of dyspnea, dysphagia, odynophagia, foreign body feeling, or hoarseness. He subsequently visited the Department of Otolaryngology for throat discomfort after his last endoscopic examination. Physical examination showed that the patient was healthy-looking in appearance, with other characteristics, including his tonsils, thyroid gland, and lymph nodes, being unremarkable. Computed tomography (CT) imaging showed a mass bulging out of his pharyngolaryngeal cavity (Fig. ) and flexible fiberoptic laryngoscopy revealed a round laryngeal cyst (Fig. ).\nThe patient underwent endoscopic excision of the mass. Transoral fiberoptic intubation was performed under general anesthesia. A 2 × 2 cm mass was observed on the laryngeal surface and the right edge of the epiglottis, which appeared to be a cyst. Following suction to remove the cyst at its base, however, another mass was found to emanate from the underside of the cyst. This mass had the appearance of a papilloma, measuring about 1 × 1 cm (Figs. and ). Hence the patient was diagnosed as having concomitant tonsillar cyst and papilloma of the larynx, and the lesion was completely excised microsurgically using a bipolar radiofrequency plasma ablation (RFA) device. The entire lesion was sent to the pathologist and the pathologic findings showed that the squamous epithelium was in a papillary arrangement, and that under the squamous epithelium there was loose connective tissue with diffuse lymphoid infiltration, which confirmed the diagnosis (Fig. ). The patient's postoperative course was uneventful, with no evidence of recurrence observed after 6 months. The patient has provided written informed consent for publication of this case report. | [[34.0, 'year']] | M | {'22398119': 1, '26507793': 1, '10547496': 1, '28695764': 1, '5411821': 1, '6731351': 1, '24782443': 1, '15179225': 1, '17129695': 1, '10652415': 1, '29953008': 2} | {} |
1,127 | 6039608-1 | 29,952,994 | comm/PMC006xxxxxx/PMC6039608.xml | The diagnosis and management of rare cystic liver metastases from nasopharyngeal carcinoma | A 40-year-old female, with 3-month history of nasal obstruction and tinnitus was admitted in August 2012. Nasopharyngeal endoscopy and biopsy already had been performed in another hospital, showing nonkeratinizing undifferentiated NPC. This histopathologic diagnosis was confirmed in our center. Magnetic resonance imaging (MRI) of the nasopharynx and neck revealed the tumor was confined to the nasopharynx and the bilateral locoregional cervical lymph nodes enlarged with its greatest dimension of 2 cm. Chest computed tomography (CT) scan, ultrasound of abdomen, and whole-body bone scan ruled out distant metastases. So clinical staging was determined to be T1N2M0, IIIA according to American Joint Committee on Cancer TNM Staging System for NPC (7th ed, 2010).\nThe patient was treated with definitive IMRT to 7050 cGy for primary tumor and 6600 cGy for infiltrated regional lymph nodes. Concurrent chemotherapy based on cisplatin and 5-flurorouracil was administrated for 2 cycles and then 2-cycle chemotherapy was given subsequently to consolidate the efficiency with the same regimen. At the end of therapy, she obtained clinical complete response by nasopharynx and neck MRI.\nIn the initial therapy, the patient had undergone abdominal ultrasonography for 4 times, and no hepatic lesions were noted during this period. Nevertheless, when she came to our hospital for 3-month conventional follow-up in April 2013, abdominal sonogram revealed a liver cystic lesion with thin wall and smooth margin of 18 × 16 mm in the right liver lobe, and the lesion was interpreted as a simple liver cyst (Fig. ). Therefore, intense follow-up was suggested. Five months later, the cystic lesion enlarged to be 59 × 46 mm, with thick wall, but no signal of blood flow. Further CT presented a low density and heterogeneous lesion taking irregular wall and incomplete septa with strong contrast enhancement, indicating liver abscess (Fig. ). However, the patient was asymptomatic, with no fever, no right up abdominal pain, and no palpable mass by physical examination. On the contrary, laboratory findings were negative, liver function was normal, the white blood cell count, C-reactive protein, and cancer-related antigen including α-fetoprotein were within the normal limits. Afterward the patient was transferred to another hospital for abscess drainage, and fluid culture was negative, but fine-needle aspiration was not performed. After drainage, the mass shrank significantly to one-third of the original size. However, it returned to previous size by CT 2 months later. Thus, the liver lesion was suspected to be malignant. Work-up examination including chest CT, pelvic MRI, emission CT for bones, gastroscopy, colonoscopy, and brain MRI excluded other lesion that may account for another primary tumor or extra-hepatic distance metastases from NPC. In addition, nasopharynx MRI showed no evidence of local relapse. On November 21, 2013, surgical resection was administrated both for histologic diagnosis and treatment, and the surgical margin was negative. Histopathologic examination definitely confirmed that the metastasis originated from NPC, since the cells of the surgical segment were similar to primary NPC on the morphology and they were positive in Epstein–Barr virus (EBV) encoded RNAs (EBERs) (Fig. ). No adjuvant chemotherapy was done after resection of the liver metastasis.\nHowever, relapse-free survival time lasted only for 8 months. In July 2014, 2 small cystic lesions were found on abdominal CT scan again, which were extremely similar to simple cysts (Fig. ). Further contrast-enhanced ultrasonography provided no sign of “fast in and fast out,” that is, a characteristic appearance in malignant carcinoma. As a result of multidiscipline team discussion, “watch and wait” strategy was recommended. However, the number of hepatic lesions increased to 4, and the size of previous 2 cysts enlarged to be 3 cm within 3 months. The multidiscipline team members reached a consensus that the cystic lesions were metastases from NPC and suggested a palliative systematic chemotherapy. Then the patient received chemotherapy with paclitaxel and cisplatin for 6 cycles. During the first 3 cycles, partial lesions diminished in size, but stayed stable within the later 3 cycles. Nevertheless, the intraliver metastases exhibited significant progress soon after the chemotherapy. Subsequently, she underwent chemotherapy with gemcitabine plus targeted therapy with nimotuzumab, then single navelbine both for 3 cycles, but neither protocols showed notable effects on hepatic lesions. In addition, during the phase of chemotherapy with navelbine, the patient complained of chest pain, an irregular and fixed lump was found on her chest wall, approximately 3 cm in diameter. After local surgical resection was performed, the lump histologically was demonstrated to be an extra-hepatic metastasis from NPC. Considering the patient's good performance status score and grade A liver function according to the Child-pugh grading system, transcatheter hepatic artery chemoembolization (TACE) was administrated for 5 times from December 2015 to April 2016. After the TACE treatment, all the lesions were obviously decreased in size, with the largest metastasis decreasing from 64 × 53 to 33 × 32 cm (Fig. ). Meanwhile, carbohydrate antigen 125 (CA125) decreased from 125 to 60.8 U/mL, and squamous cell carcinoma antigen decreased from 2.8 to 0.9 ng/mL. Then 4 cycles of gemcitabine plus cisplatin were offered to consolidate the clinical effects. In the next 6 months, her liver metastases maintained stable, but lung metastases were noted. In October 2016, the CT showed the liver metastases progressed. At last, she died of liver failure in March 2017. | [[40.0, 'year']] | F | {'20716628': 1, '17085352': 1, '18595127': 1, '22687948': 1, '1709423': 1, '10757381': 1, '19145100': 1, '12676255': 1, '25376591': 1, '16998361': 1, '11452064': 1, '26351355': 1, '20922367': 1, '15650637': 1, '26321262': 1, '34083637': 1, '3773537': 1, '25415696': 1, '10758991': 1, '26181570': 2, '24669569': 1, '17909810': 1, '10493478': 1, '29952994': 2} | {'4617094-1': 1, '4617094-2': 1, '4617094-3': 1, '4617094-4': 1} |
1,128 | 6039615-1 | 29,952,950 | comm/PMC006xxxxxx/PMC6039615.xml | Kidney Transplantation from an extracorporeal membrane oxygenation-supported brain-dead donor | The donor, a 39-year-old male with a history of schizophrenia, fell from a fifth floor window and was taken to Tri-Service General Hospital, Taipei, Taiwan. Computed tomography scan revealed multiple rib fractures, pelvic fracture with active bleeding, and dissection of the descending aorta with intramural hematoma. Consequently, the patient underwent bilateral anterior thoracotomies and transcatheter embolization of the pelvic vessels for acute resuscitation.\nAfter 14 days, the patient underwent thoracic endovascular aortic repair of the descending aortic aneurysm. During the procedure, the patient experienced oxygen desaturation and cardiac arrest, and advanced cardiac life support was performed immediately. After return of spontaneous circulation, ECMO was set up due to persistent bradycardia and poor tissue perfusion, possibly caused by pulmonary embolism.\nHypoxic-ischemic encephalopathy was not improved after 1 week; therefore, the diagnosis of brain death was made by 2 senior doctors, and the family decided to enter the critical hospice pathway and donate his organs.\nOrgan donation was made at 21 days after the patient's trauma (7 days after the ECMO was placed). The patient's initial SCr on admission was 0.9 mg/dL and rose to 4.7 mg/dL at organ harvesting. During kidney procurement, we discovered an extremely abnormal appearance of the kidneys, which were “very dark blue” in color (Fig. ). Rather than discarding the kidneys, we performed an on-table biopsy. Microscopically, this section showed only minimal interstitial fibrosis of parenchyma. The glomeruli, tubules, and vessels were intact (Fig. ). The final grade of pretransplant biopsy based on the Remuzzi Score System was 1 of 12, indicating single kidney transplantation.\nAfter informed consent, 2 candidates received grafts; one was a 47-year-old female with lupus nephritis who developed end-stage renal disease (ESRD) and underwent hemodialysis and the other was a 58-year-old female with immunoglobulin A nephropathy leading to ESRD who underwent peritoneal dialysis (Table ). Both recipients received dialysis within the first postoperative week due to delayed graft function.\nWith the use of immunosuppressive therapy, the SCr of these 2 patients decreased from 8.5 and 17.6 mg/dL to 1.1 and 1.8 mg/dL, respectively, at the 1-month postoperative follow-up, and these 2 grafts remained functional at the 8-month follow-up (Table ).\nWritten informed consent to publish the case report was provided by the patients, and the consent procedure was approved by the Ethics Committee of Tri-Service General Hospital. | [[39.0, 'year']] | M | {'18785957': 1, '20102398': 1, '24815128': 1, '19692267': 1, '34357195': 1, '16436766': 1, '17692626': 1, '16799171': 1, '21911111': 1, '27194996': 1, '25155294': 1, '20111046': 1, '24928419': 1, '29952950': 2} | {} |
1,129 | 6039618-1 | 29,952,975 | comm/PMC006xxxxxx/PMC6039618.xml | A case report | A 55-year-old male farmer presented to the Outpatient Department with persistent left epigastric dull pain accompanied by chills and febrile (Tmax 39 °C). He did not report a clear cause of the abdominal pain. An abdominal exam revealed no distended abdomen, normoactive bowel sounds, no rebound tenderness, no tenderness in the left upper quadrant, no palpable mass. He lost 10 kg within 6 months unintentionally. He visited a gastroenterologist at a health clinic and received anti-infection therapy, which relieved the abdominal pain. However, he recently suffered from intermittent abdominal pain. He was subsequently referred to our hospital for further evaluation and treatment.\nThe patient presented with fever and left upper abdominal pain. In a blood sample, the white blood cell count was 17.37 × 109 cells/L (neutrophils = 93.9%, lymphocytes = 3.1%, monocytes = 3.0%, eosinophils = 0.00%, basophils = 0.00%) and the platelet count was 289 × 109 cells/L when he first came to the outpatient department. The results of laboratory tests, including urine test, liver function tests, alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19–9, were all normal. Highly sensitive C-reactive protein was 56.6 mg/L (Table ). He had a 1-year history of type 2 diabetes mellitus and took medicine irregularly. He had no hepatitis or tuberculosis, and a human immunodeficiency virus serological test was negative. Upper abdominal contrast-enhanced computed tomography (CT) showed diffuse hepatic disease, spleen abscess with inflammatory exudate, and spleen calcification (Fig. A). A chest radiograph and CT scan showed chronic inflammation of the inferior lobe of the left lung, a small amount of fluid in the left chest, and thickening of the wall of the gastric fundus.\nThe patient was subsequently subjected to laparoscopic exploration and splenectomy. A histological examination of biopsies demonstrated obvious spleen enlargement, fibrosis, and necrosis of spleen parenchyma, presenting as “frozen” lesions. Additionally, the tail of the pancreas and greater curvature of the stomach adhered to the spleen. A peritoneal cavity drainage tube was inserted in the splenic recess for the drainage of pus. A spleen specimen of 12 × 8 × 6 cm exhibited a number of divergent pus cavities, the largest of which had a diameter of 2 cm (Fig. B). Two days after surgery, the patient developed an effusion in the splenic fossa, which was successfully drained.\nWe suspected tuberculosis according to a histological analysis of HE-stained spleen sections. Typical granulomatous inflammation was observed in splenic pathological sections (Fig. C). However, the specific acid-fast staining test for tuberculosis was negative (data not shown). Immunohistochemical staining was performed using a B pseudomallei antibody preserved in our laboratory, and B pseudomallei in the splenic tissue sections were observed (Fig. D). Bacterial culture of the spleen pus for 72 hours revealed small, dry colonies with a typical wrinkled surface on blood agar (Fig. E). Bacteria isolated from the pus culture were identified as B pseudomallei using the commercial BD Phoenix-100 Automated Microbiology System (BD Biosciences, Franklin Lakes, NJ). Based on analyses of the minimum inhibitory concentration (MIC), the isolated strain was susceptible to ceftazidime (MIC, 4 μg/mL), meropenem (MIC, 2 μg/mL), and trimethoprim-sulfamethoxazole (TMP-SMX) (MIC, 0.5 μg/mL).\nThe patient was treated with antibiotics intravenously for 15 days. On the day of admission, he initially received empirical anti-infective intravenous cefmenoxime and piperacillin-tazobactam. Owing to the lack of a clinical response, the treatment was changed to intravenous ceftazidime for 2 days, followed by oral TMP-SMX according to the results of a drug sensitivity test, and the patient exhibited a gradual improvement in clinical condition. Upper abdominal CT showed minor pneumoperitoneum and a reduction in pleural fluid in the left lung lobe. The pigtail was removed after no further abscess drainage was necessary. The patient was asymptomatic for the next 5 days, and the wound was clean and granulating. Hence, he was discharged with oral TMP-SMX for 3 months for eradication therapy. | [[55.0, 'year']] | M | {'25531547': 1, '26458779': 1, '26803404': 1, '25643275': 1, '26972397': 1, '32509481': 1, '20578524': 1, '29046753': 2, '21734135': 1, '33911455': 2, '27571754': 1, '26276499': 1, '33975989': 1, '25531912': 1, '29952975': 2} | {'5637701-1': 1, '8054788-1': 1} |
1,130 | 6039626-1 | 29,953,018 | comm/PMC006xxxxxx/PMC6039626.xml | Partial molar pregnancy in the cesarean scar | On March 16, 2016, a 28-year-old woman (gravida 3, para 1) was admitted to our hospital because of amenorrhea for 48 days and vaginal bleeding for 3 days. She had a cesarean section 1 year ago. On admission, she complained of irregular vaginal bleeding and mild abdominal pain. A pelvic examination showed a closed external cervical os and a normal uterus with tenderness. Her hemoglobin and serum human chorionic gonadotropin (hCG) levels were 110 g/L and 7894 IU/L, respectively. A transvaginal sonogram showed a 1.2 × 1.4 × 1.5-cm-sized gestational sac implanted near the previous cesarean scar in the anterior wall of the uterine corpus (Fig. ). The gestational sac was bulging toward the serosa, with a 5-mm-thin layer of overlying myometrium. A pelvic magnetic resonance imaging (MRI) scan showed a group of abnormal cystic component signal on the anterior wall of the uterine isthmus incision, measuring approximately 1.6 × 2.0 × 1.5 cm (Fig. ). The lesions, low signal on T1-weighted image (T1WI), and mixed with high signal on T2WI, were prominent in the uterine cavity. The myometrium of the anterior wall of the uterine isthmus incision was not continuous, with the thinnest area at approximately 0.6 cm, whereas the uterine serosa was continuous. Based on these findings, she was suspected of having CSP.\nOn day 1, considering the high risk of hemorrhage, bilateral uterine arterial embolization was performed. Her serum hCG level was 10,940 IU/L. The ultrasound revealed decreased vascularity. On day 2, careful suction evacuation under ultrasound guidance was performed. A 10-g tissue without chronic villi or cystic mole was obtained, which was sent for histologic examination. At the end of the procedure, the ultrasound showed no evidence of the previous lesion. The total blood loss was 5 mL, and oxytocin was intravenously administered to reduce the risk of bleeding. On day 3, vaginal bleeding was not observed, and her serum hCG level decreased to 4488 IU/L, and the patient was discharged. Surprisingly, the histologic examination of the tissue confirmed a partial hydatidiform mole. Chorionic villi with focal trophoblastic proliferation and hydropic change were observed (Fig. ). The immunohistochemical results showed P57(+) and proliferation index of trophocyte Ki67 of 10%. The patient had a partial molar CSP instead of normal CSP, which we considered preoperatively. The patient's serum hCG level was monitored weekly. Her serum hCG level gradually returned to normal, and she had no vaginal spotting for 9 weeks. Menstruation restarted in the ninth week. | [[28.0, 'year']] | F | {'19780724': 1, '30235783': 1, '30383708': 1, '16273594': 1, '22640013': 1, '32368549': 2, '26522484': 1, '27648323': 2, '20728069': 1, '17175496': 1, '7566847': 1, '24185003': 1, '17655732': 1, '32509606': 1, '22270435': 1, '29953018': 2} | {'7190942-1': 1, '5014949-1': 1} |
1,131 | 6039648-1 | 29,952,940 | comm/PMC006xxxxxx/PMC6039648.xml | Recurrence of anti-MDA5 antibody-positive clinically amyopathic dermatomyositis after long-term remission | In October 2010, a 70-year-old Japanese woman was diagnosed with ILD and CADM based on the findings of rash on the fingers of both hands, interstitial pneumonia, and a high titer of anti-MDA5 Ab (148 index). We administered 1000 mg of methyl-prednisolone (mPSL) pulse therapy and intravenous cyclophosphamide therapy (IVCY) followed by prednisolone (PSL) 50 mg/day with tapering and cyclosporine (CyA), and the symptoms were improved by a total of 5 IVCY continuations. After that, the patient maintained long-term remission for approximately 7 years, and she was treated with oral PSL 3 mg/day and CyA 100 mg/day. However, in November 2017, she suffered from nasal discharge, feeling heaviness of her head, and fatigue. In December 2017, she also presented with rash on both her fingers and toes, and she was then admitted to our department.\nOn admission, her body temperature was 37.1°C; her blood pressure was 141/76 mm Hg, the heart rate was 86 beats/min, and the pulse oximetric saturation (SpO2) was 95% (room air). On physical examination, fine crackles were audible on the dorsal side of the bilateral lower lung regions, and she had erythemas on the nail circumference and both dorsal and palm sides around the proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints, suggesting Gottron's sign and inverse Gottron's sign, respectively (Fig. ). She had no muscle pain, and a manual muscle test showed no abnormalities in the upper and lower limbs.\nLaboratory investigations showed the following results: partial pressure of arterial oxygen (PaO2) 73.5 mm Hg, white blood cell count (WBC) 9400 /μL (neutrophils 89.5%, lymphocytes 9.3%), hemoglobin (Hb) 12.1 g/dL, platelet (PLT) 27.0 × 104/μL, C-reactive protein (CRP) 3.4 mg/dL, lactate dehydrogenase (LDH) 231 IU/mL (normal range 124–222), ferritin 319 ng/mL (normal range 6.0–138). The levels of creatinine kinase and aldolase were 79 IU/L and 3.6 IU/L, respectively (= within the normal range). Although the serum Krebs von den lungen (KL)-6 level was 274 U/mL (within the normal range), the levels of surfactant protein (SP)-A and SP-D were 49.3 and 140 ng/mL in slightly high titers. No abnormalities were revealed by a urinalysis, and no liver or renal dysfunction was detected.\nThe following immunological and serological results were all negative (the exception is anti-MDA5 antibody): rheumatoid factor (RF), antinuclear antibody (ANA), proteinase-3 anti-neutrophil cytoplasmic autoantibodies (PR3-ANCAs), myeloperoxidase anti-neutrophil cytoplasmic autoantibodies (MPO-ANCAs), anti-ARS antibody, anti-transcription intermediary factor 1-gamma (TIF1-γ) antibody, and angiotensin converting enzyme (ACE). The anti-MDA5 antibody titer index was 109 (normal range < 32). The results of assays of β-D-glucan, T-SPOT. TB Legionella pneumophila, Streptococcus pneumonia, Mycoplasma pneumonia, Clamydia psittaci, and Clamydia pneumonia were all negative. A chest computed tomography (CT) examination showed the expression of invasive shadows on lung field under the pleura and on the dorsal side of the bilateral lower lobes (Fig. A), suggesting an exacerbation of interstitial pneumonia.\nWe diagnosed the patient as having recurrent CADM complicated with ILD based on the findings of the typical skin symptoms, the exacerbation of interstitial pneumonia, and a high titer of anti-MDA5 Ab. We then administered 1,000 mg of mPSL pulse therapy and IVCY followed by PSL 50 mg/day with tapering and an increase of CyA from 100 mg to 150 mg. After that treatment, the patient's skin symptoms and interstitial pneumonia were relieved (Fig. B). All laboratory investigations such as ferritin, the serum markers of interstitial pneumonia (i.e., SP-A, SP-D), and the titer of anti-MDA5 Ab showed signs of improvement. We administered a total of 2 sessions of IVCY continuation, and the patient's remission has now been maintained for over 1 months as of this writing (Fig. ). We measured the antisplicing factor proline/glutamine-rich protein antibody (anti-SFPQ Ab) using her preserved serum and found that anti-SFPQ Ab at the initial diagnosis was negative, but it turned positive at the recurrence. | [[70.0, 'year']] | F | {'19444719': 1, '28711882': 1, '22589330': 1, '31923118': 1, '21482889': 1, '8449401': 1, '28451794': 1, '29106035': 1, '19565506': 1, '15880816': 1, '22886382': 1, '20875136': 1, '16625202': 1, '23436757': 1, '20015976': 1, '32033996': 2, '11907524': 1, '16142867': 1, '18272355': 1, '21531040': 1, '23908005': 1, '22623119': 1, '22644102': 1, '16116171': 1, '32925726': 2, '27452897': 1, '34745149': 1, '22192091': 1, '22198664': 1, '22378718': 1, '27919567': 1, '20498012': 1, '20501546': 1, '29952940': 2} | {'7021146-1': 1, '7489601-1': 1} |
1,132 | 6039649-1 | 29,953,015 | comm/PMC006xxxxxx/PMC6039649.xml | Extrapyramidal signs occurring after sympathetic block for complex regional pain syndrome responding to diphenhydramine | This is a 22-year-old female patient diagnosed with lower extremity CRPS type-I in the left ankle and foot based on the International Association of the Study of Pain (IASP) Budapest Criteria.[ The patient did not have any history of psychological disorder, seizure disorder or neurological abnormality. Physical examination in the first presentation also included the contracture of the left foot in plantar-flexed position (Fig. ), and the only motor presentation of the CRPS was a reproducible tremor of the ipsilateral quadriceps muscles when the left knee was fully extended. The patient did not have any other visible or reproducible involuntary motor movement in her history or physical examination. The patient underwent a diagnostic left lumbar sympathetic block (LSB), followed by 5 additional LSBs with the same technique and medications (Fig. ). All the procedures were performed under local anesthesia and the patient was given lorazepam 1.5 mg orally as a sedative prior to procedures. In addition, for the 6th block, the patient was given DPH 50 mg IV prophylactically. The patient was placed prone and ipsilateral 30° oblique view was obtained with fluoroscopy. The needle entry point was marked on the skin at the level of L3. After sterile preparation and draping, 3 mL of Lidocaine 1% was used to anesthetize the entry point. 20-gauge 3.5-inch introducer and 25-gauge 6-inch spinal needles were used. The spinal needle was directed to the anterolateral aspect of the L3 vertebral body. There was no paresthesia during advancement of the needles. After negative aspiration for blood and cerebrospinal fluid, 4 mL of Iohexol (180 mgI/mL) contrast was injected under live fluoroscopy to rule out intravascular injection. Appropriate distribution of the contrast in the anterolateral aspect of the L3 vertebral body was verified. Thereafter, 10 mL Bupivacaine 0.5% was injected at 1 mL increments after negative aspiration. Temperature measurements of bilateral plantar skin as well as pulse amplitude of the ipsilateral big toe with pulse oximetry were monitored continuously. Adequate sympathetic blockade was achieved after each block with confirmation of at least 2°C increase from the baseline temperature. Symptomatically, the patient responded well to the series of 6 LSBs with improvement of pain and other CRPS-related signs in the left ankle and foot. However, within 5-minutes of the completion of dose for each first 5 LSBs, the patient developed intense muscle spasms of the entire ipsilateral lower extremity (Video 1). After the first occurrence, various medications were tried to abort these intense spasms including postprocedure IV midazolam, oral cyclobenzaprine and oral baclofen, but they were not effective. The patient eventually responded immediately to IV DPH 50 mg. In the next 4 blocks, the patient was treated similarly with DPH 50 mg IV postprocedure each time with abrupt resolution of muscle spasms. For the sixth LSB, prophylactic treatment of DPH 50 mg IV was given before the procedure and the patient did not show any motor response on the 6th block. | [[22.0, 'year']] | F | {'10349117': 1, '20693883': 1, '7556545': 1, '34809486': 2, '21180636': 1, '10639611': 1, '22336720': 1, '27467116': 1, '8748051': 1, '7710376': 1, '20493633': 1, '2296383': 1, '29953015': 2} | {'6039649-2': 2, '8610071-1': 1, '8610071-2': 1, '8610071-3': 1} |
1,133 | 6039649-2 | 29,953,015 | comm/PMC006xxxxxx/PMC6039649.xml | Extrapyramidal signs occurring after sympathetic block for complex regional pain syndrome responding to diphenhydramine | This is a 40-year-old female patient diagnosed with lower extremity CRPS type-I in the right ankle and foot, based on the IASP Budapest Criteria.[ The patient did not have any history of psychological disorder, seizure disorder or neurological abnormality. Physical examination in the first presentation showed the presence of well-healed incision scars from the previous surgeries in the dorsum of the right ankle and foot. There were mild color changes. There were moderate skin texture changes and trophic changes in the nails. The patient had excessive sweating both on inspection and palpation in the entire right foot. There was hyperesthesia and hyperalgesia of the entire dorsum of the foot (Fig. ). Right foot skin temperature was 3°C colder than the left foot. The patient had 4/5 weakness of the right foot extensors and toe flexors. Right ankle range of motion was moderately limited secondary to pain. The patient did not have any visible or reproducible involuntary motor movement in her history or physical examination. The patient underwent a first LSB with the same technique and medications as described in Case 1, but on the right side. LSB was performed under local anesthesia and the patient was given lorazepam 1.5 mg orally as a sedative prior to procedure. Temperature measurements of bilateral plantar skin as well as pulse amplitude of the right big toe with pulse oximetry were monitored continuously. Adequate sympathetic blockade was achieved after the block with confirmation of at least 2°C increase from the baseline temperature. The patient developed muscle spasms of the entire ipsilateral lower extremity in the recovery room 15 minutes after the completion of the first LSB (Video 2). These muscle spasms were not as intense as the Case-1, but significant enough that the patient was complaining of increased pain and was unable to stand. The patient was initially treated with 2 mg of midazolam IV. The patient did not respond to treatment with midazolam. As the presentation was very similar to Case-1, the patient was treated with IV DPH 50 mg. The patient responded immediately with abrupt resolution of muscle spasms. | [[40.0, 'year']] | F | {'10349117': 1, '20693883': 1, '7556545': 1, '34809486': 2, '21180636': 1, '10639611': 1, '22336720': 1, '27467116': 1, '8748051': 1, '7710376': 1, '20493633': 1, '2296383': 1, '29953015': 2} | {'6039649-1': 2, '8610071-1': 1, '8610071-2': 1, '8610071-3': 1} |
1,134 | 6039681-1 | 29,952,988 | comm/PMC006xxxxxx/PMC6039681.xml | Uncommon foreign body in the hypopharynx | A 9-month-old boy presented to Department of Otolaryngology for an hour of crying associated with having congee. He was suspected of foreign body aspiration. However, there were no suggestive symptoms, such as dyspnea, stridor, croupy cough, hoarseness, or vomiting. In physical examination, no foreign body was found in the oropharynx. Differences in the intensity, phase, and quality of bilateral breath sounds were uncertain for disturbance of baby crying. Therefore, cervicothoracic computed tomography (CT) scan with virtual endoscopy was taken for excluding foreign bodies of the pharynx, larynx, trachea, bronchi, and esophagus. However, no abnormality was revealed in the CT scan, as shown in Fig. . Then, flexible laryngoscopy was applied for viewing the hypopharynx and larynx. A transparency film, exhibited in Fig. , was visualized on the posterior hypopharyngeal wall, as shown in Fig. . The removal of the foreign body was proceeded immediately. The tongue was depressed for better exposure of the foreign body. Under direct visualization it was removed with forceps. | [[9.0, 'month']] | M | {'34934574': 2, '26782802': 1, '3251696': 1, '27598617': 1, '15952575': 1, '1395399': 1, '29952988': 2} | {'8684363-1': 1} |
1,135 | 6039684-1 | 29,952,979 | comm/PMC006xxxxxx/PMC6039684.xml | Small cell variant of ALK-positive anaplastic large cell lymphoma with primary subcutaneous presentation | An 11-year-old Taiwanese girl was admitted to our hospital with fever, dyspnea, and impending respiratory failure in May 2013. Physical examination revealed an enlarged nodular lesion over the right shoulder and several smaller nodular lesions on the abdomen. A complete blood count showed leukocytosis at 19.4 × 109 cells/L with 78% segmented neutrophils, 2% band form, 11% lymphocytes, and 9% monocytes. C-reactive protein level was 132.26 mg/L (normal: <5 mg/L), and serum lactate dehydrogenase level was 392 U/L (normal: 135–260 U/L). High-resolution computed tomography showed multiple lung opacities and mediastinal, cervical, and bilateral axillary lymphadenopathies. Bone marrow aspiration and biopsy revealed no lymphoma cells.\nLymph node biopsies confirmed ALK+ ALCL diagnosis. Lymphoma cells were positive for CD2, CD3, CD4, CD30, ALK1, Bcl-6, MUM1, and TIA-1, but were negative for CD20, CD5, cyclin D1, CD10, TdT, CD8, and PD1. Conventional cytogenetic analysis showed a normal karyotype. Lymph nodes also displayed a relatively small number of small-to-large hallmark cells with reniform nuclei. Because of the rate small cell ALCL variant morphology resembling classic ALCL, it was misdiagnosed initially as ALK+ ALCL. Our patient achieved complete remission 4 months after diagnosis. Treatment involved an initial course of intravenous dexamethasone and cyclophosphamide, and intrathecal administration of methotrexate, cytarabine, and hydrocortisone, followed by 3 alternating cycles of A and B regimens every 3 weeks (A: dexamethasone, high-dose methotrexate, cytarabine, etoposide, and ifosfamide; B: dexamethasone, cyclophosphamide, doxorubicin, and high-dose methotrexate). New skin lesions were later noted in the lower back, and ALCL relapse was confirmed by skin biopsy 28 months after the initial treatment. Based on these findings we reviewed the histology of subcutaneous nodules biopsy performing additional immunohistochemistry for the ALK protein which revealed positivity in some of the CD3+ small lymphocytes as well as in rare dispersed previously unrecognized atypical large cells which also turned out to be CD30+. This prompted a diagnosis of subcutaneous nodule involvement by a small cell component of an ALK+ ALCL of the composite variant. Small cell variant of ALCL was confirmed by the reviewing pathologist.\nFurther treatment consisted of chemotherapy with 2 courses of high-dose CHOP (cyclophosphamide 2000 mg/m2 [day 1], hydroxydaunorubicin 90 mg/m2 [day 1], oncovin 2 mg/d [day 1], prednisolone 60 mg/m2 [days 1–5], mesnum [150% cyclophosphamide dose]), alternating with one course of standard ESHAP ([etoposide 40 mg/m2 [days 1–4]; cisplatin 25 mg/m2 [days 1–4], cytarabine 2000 mg/m2 per day [day 5], and prednisolone 250 mg [days 1–4]). After completion, the patient underwent allogeneic peripheral blood stem cell transplantation from her human leukocyte antigen-identical sister in December 2015. The timeframe from initial diagnosis to transplantation was 32 months. Before transplantation, the patient had residual skin lesions suggesting a partial remission, and bone marrow biopsy revealed no residual lymphoma cells. The patient received a conditioning regimen consisting of total body irradiation (13.2 Gy in 8 fractions on days −8 to −5), and cyclophosphamide (60 mg/kg on days −3 to −2). Infused cells and CD34+ cells were 10.14 × 108/kg and 10.65 × 106/kg, respectively.\nGraft-versus-host disease prophylaxis consisted of intravenous cyclosporine (5 mg/kg per day) beginning on day −3 and short-term methotrexate at 15 mg/m2 on day +1 and 10 mg/m2 on day +3 and +6. Rapid engraftment was obtained. Neutrophil count of >0.5 × 109/L and platelet count of >20 × 109/L were achieved on days 12 and 15, respectively, and complete donor chimerism was observed in a bone marrow sample obtained on day 42. After HSCT, a follow-up positron emission tomography–computed tomography showed complete metabolic remission. As of April 2018, the patient had achieved 28 months of continuous complete remission with a Lansky score of 100.\nA chest wall skin-punch biopsy demonstrated diffuse subdermal tumor cells infiltrates. Infiltrated lymphocytes consisted of small-to medium-sized cells with irregular nuclear contours, condensed chromatin, and moderately clear cytoplasm (Fig. A). Lymphocytes infiltrated the vascular wall with prominent rimming of individual endothelial cells (Fig. B). Immunohistochemical analysis showed the infiltrating cells were positive for CD2, CD3 (Fig. A), CD4, CD30 (Fig. B), ALK (Fig. C), Bcl-6, MUM1, and TIA-1. | [[11.0, 'year']] | F | {'8394652': 1, '25156886': 1, '23264126': 1, '18385450': 1, '28570392': 1, '9736036': 1, '28591420': 1, '26458652': 1, '22084369': 1, '19074096': 1, '11090048': 1, '10911802': 1, '21763080': 1, '16611309': 1, '3876124': 1, '23758281': 1, '21043827': 1, '29952979': 2} | {} |
1,136 | 6040003-1 | 30,402,619 | comm/PMC006xxxxxx/PMC6040003.xml | Hyperresponsiveness to antihistamines in spontaneous urticaria and heat urticaria | We report the case of a 28-year-old female patient who presented with a 4-year history of almost daily episodes of urticarial skin lesions. She reported to not yet be suffering from angioedemas. The patient suspected that the skin reactions were triggered by exposure to the sun. Furthermore, the intake of cetirizine tablets had led to a worsening of the urticaria in the past, and sometimes she had experienced episodes of vertigo and nausea after intake. Thus, the patient had self-tested the tolerability of several antihistamines during symptom-free periods. She reported always having developed urticarial lesions approximately 2 hours after intake. The triggering drugs were Rupafin® (rupatadine), Telfast® (fexofenadine), Atarax® (hydroxyzine), Lorano® (loratadine), Wick Medi Nait® (among others, doxylamine succinate), and Grippostad® (among others chlorpheniramine maleat). Furthermore, the patient indicated that Livocab® (levocabastine) eye and nose drops, used for the treatment of rhinoconjunctival conditions, had also led to urticarial lesions in the entire integumentary system, mostly in the face. Her patient history included neurodermitis in childhood, rhinoconjunctival symptoms in the months of April to October, oral allergy syndrome after ingestion of apples and various nuts as well as perennial bronchial asthma. Due to migraines, she took acetylsalicylic acid and ibuprofen as needed, the latter twice a week. Due to relapsing tonsillitis, a tonsillectomy had been carried out, but after that, increased CRP levels had still repeatedly been measured. On the day of first presentation, the skin symptoms were weak, no urticarial lesions or red dermographism were detected. The physical examination revealed remnants of the tonsils at the soft palate. | [[28.0, 'year']] | F | {'19639729': 1, '19348661': 1, '18205837': 1, '9722235': 1, '20128233': 1, '15153902': 1, '12911412': 1, '15548781': 1, '15480348': 1, '10759435': 1, '11991289': 1, '30402619': 2} | {} |
1,137 | 6040024-1 | 30,013,752 | comm/PMC006xxxxxx/PMC6040024.xml | Detection of asymptomatic celiac disease in two siblings from a mother with non-celiac gluten sensitivity | A 43-year old woman was referred to our outpatient clinic due to gastrointestinal symptoms (i.e. abdominal pain and bloating, diarrhea, gastro-esophageal reflux) and extra-intestinal manifestations (weakness, headache, foggy mind and limb numbness, skin rash, fibromyalgia-like symptoms and anemia) triggered by gluten and wheat ingestion. Symptoms started three years before, when the patient was 40 years old. IgA tTGA and EMA tested negative as well as duodenal biopsy showed a normal mucosal architecture on a gluten containing diet, thus ruling out CD diagnosis. Wheat allergy was excluded by means of IgE to gluten and wheat as well as by skin prick tests. As part of a thorough diagnostic work-up, the patient was found to be positive for HLA-DR7 and -DQ2 haplotype. Other laboratory data revealed positivity for antibodies to native gliadin of IgG class (AGA IgG, twice the upper normal limit; conversely, deamidated gliadin peptide IgG antibodies were negative) and low levels of folic acid, ferritin and vitamin D. Thyroid function tests disclosed a condition of autoimmune thyroiditis without hypothyroidism. An open 6-week trial with gluten-free diet (GFD) led to a significant symptomatic improvement in a few days and the patient remained symptom-free on GFD. The diagnosis of NCGS was validated by means of a double-blind placebo-controlled cross-over trial as previously described (). The patient was advised to follow a strict GFD which led to a significant improvement of her clinical picture along with disappearance of IgG AGA. Notably, following GFD a significant improvement of folic acid, ferritin and vitamin D levels was observed at 6-month follow-up.\nConcerning the family history, the patient had two children, a 12-year old daughter and a 9-year-old son. None of them complained of gastrointestinal and extra-intestinal symptoms and they showed a normal growth without signs of short stature and weight loss. Laboratory data of these two children were unremarkable with normal value of hemoglobin, red blood cells, white cells and platelets. Values of vitamin D3, ferritin and folic acid were in the normal range in both children.\nCD antibody screening turned to be positive in both children, despite they were asymptomatic and with normal laboratory data. The 12-year old girl showed positivity for tTGA of IgA class at a very high titer (>10 times the upper normal limit) associated with EMA of IgA class. The genetic haplotype of this girl was positive for HLA-DR3 and -DQ2, corroborating the diagnosis of CD. Duodenal biopsy confirmed an active CD by showing a subtotal villous flattening (Marsh III) (, ) (). The 9-year old boy was positive at a low titer for tTGA of IgA class (1.5 times the upper normal limit) associated with a weak IgA EMA positivity.\nGenetic testing highlighted the same genetic pattern of the sister. Duodenal biopsy revealed the presence of normal villi, with a villous/crypt ratio >3:1, but with an increased number of intraepithelial lymphocytes (IELs) (55 IELs/100 epithelial cells), consistent with a diagnosis of potential CD (). Both siblings were also positive for antinuclear antibodies (ANA) detected by indirect immunofluorescence on HEp2 cells. In the boy, ANA tests showed a speckled pattern at a low titer (1:80) with negativity for anti-extractable nuclear antigen antibodies (ENA). The girl showed ANA positivity with homogeneous pattern at a very high titer (1:640) associated with ENA positivity (SSA-Ro52 +++). Both children underwent a rheumatological evaluation which was unremarkable for connective tissue disease. Due to the condition of active CD with the typical villous flattening, the girl started a GFD, which led to a significant decrease of antibody titer after 6 months. In contrast, the boy was left on a gluten containing diet as suggested for asymptomatic potential CD (). Patients involved in this case report signed informed consent to grant permission of the inclusion of their data in the study in an anonymous form. | [[43.0, 'year']] | F | {'25753138': 1, '26538207': 1, '24619056': 1, '24885375': 1, '25701700': 1, '10419909': 1, '29160841': 1, '22371218': 1, '24216570': 1, '22313950': 1, '27852116': 1, '22825366': 1, '33483854': 1, '24834212': 1, '25926934': 1, '25404189': 1, '30013752': 2} | {} |
1,138 | 6040032-1 | 30,013,753 | comm/PMC006xxxxxx/PMC6040032.xml | Post pancreatitis/cholecystectomy gluten intolerance | A 41-year-old lady presented with several years history of abdominal pain and reflux. Her past medical history includes anti-TPO positive hypothyroidism treated with thyroxine, heavy menstrual bleeding and she had a BMI of 27kg/m2. In January 2013, two years prior to her clinic encounter, she developed sudden-onset abdominal pain and was diagnosed with cholecystitis which was investigated by ERCP. Two weeks later she developed pancreatitis from which she recovered well. She went on to have a laparoscopic cholecystectomy in February 2013. Since the procedures, she developed severe gastro-oesophageal reflux, abdominal pain, bloating and diarrhoea alternating with constipation. Clinical examination was mostly unremarkable except mild tenderness in both the right and left iliac fossae without organomegaly or palpable masses.\nHer investigations in the gastroenterology clinic were negative for coeliac disease with negative endomysial antibodies. The gastroscopy revealed only reactive gastritis negative for Helicobacter pylori and more importantly, normal duodenal histology. The colonoscopy and liver ultrasound detected no abnormalities except multiple liver cysts. Her blood results were all within normal limits, including full blood count, urea and electrolytes, liver function testing, inflammatory markers, vitamin D, haematinics and thyroid hormones.\nShe was given a clinical diagnosis of IBS, but pharmacological treatment for this with Mebeverine and Hyoscine Butyl bromide did not produce much symptomatic change. After multiple follow-ups with several gastroenterologists and a dietician, she was given a trial of a gluten-free diet (GFD) and exclusion of lactose. These measures gave the patient significant symptomatic control, albeit without becoming completely asymptomatic. Re-introduction of gluten caused symptomatic flares. | [[41.0, 'year']] | F | {'26096570': 1, '26056920': 1, '24077239': 1, '26370139': 1, '27852116': 1, '26528408': 1, '27799885': 1, '25970665': 1, '16445300': 1, '33868604': 1, '27747460': 1, '16392101': 1, '22345659': 1, '25584178': 2, '27086886': 1, '28839818': 1, '25759526': 1, '30013753': 2} | {'4285934-1': 1} |
1,139 | 6040056-1 | 29,991,348 | comm/PMC006xxxxxx/PMC6040056.xml | Walking hyponatremia syndrome of inappropriate antidiuretic hormone secretion secondary to carbamazepine use: a case report | A 44-year-old Guatemalan woman presented to our outpatient clinic with a chief complaint of left knee pain for the last 6 months. One month prior our patient had consulted with an outside physician, who prescribed her with 300 mg of carbamazepine, 5 mg of prednisone every 24 hours, and ibuprofen every 8 hours as needed. The symptoms did not resolve and our patient increased the dose to 600 mg of carbamazepine and 20 mg of prednisone 7 days before consulting. She suddenly interrupted prednisone 3 days before consulting, because she felt this was making her pain worse. At the consultation, our patient complained of left knee pain, fatigue, and bilateral lower limb cramps. No pertinent medical, surgical, allergic, family, and psychosocial history was recorded and her vital signs were within normal limits (blood pressure of 118/64 mmHg; heart rate of 75/minute; respiratory rate of 14 beats/minute; temperature at 98.9 °Fahrenheit). Our patient was on day 12 of her menstrual cycle. A physical examination revealed mild facial edema, multiple port-wine stains on her upper and lower extremities associated with mild hypertrophy of the calves (Fig. ), more prominent on the right side with mild edema, the rest of the physical examination was non-contributory. Imaging and routine blood studies were requested. Our patient was seen the next day at the office with the laboratory results (Table ).\nOur patient presented with only a complaint of fatigue at this visit, however, no neurologic or physical disabilities were recorded, and her vital signs were within normal limits.\nOur patient was sent to the emergency department (ED) for electrolyte imbalance correction and for other diagnostic studies. In the ED, a 12-lead electrocardiogram did not show any electric abnormalities, her urinary Na+ (UNa+) was 164 mmol/L (normal 40–220 mmol/L), and osmolality 328 mmol/kg (50–1200 mmol/kg). No carbamazepine serum levels were recorded due to lack of reactive in the hospital. Our patient was diagnosed with syndrome of inappropriate antidiuretic hormone secretion (SIADH) secondary to carbamazepine use, hypokalemia secondary to corticosteroid therapy, and Klippel-Trénaunay-Weber (KTW) syndrome. Patient orders were to discontinue carbamazepine, fluid restriction to 1.5 L/day and intravenous potassium (K+) slow correction. She was discharged 3 days later with Na+ 128 mmol/L, K+ 4.6 mmol/L, and serum osmolality 265 mmol/kg. Our patient was seen in the office 1 week after discharge: she no longer felt fatigued, her cramps were not present and her left knee pain had mildly improved with acetaminophen use and local nonsteroidal anti-inflammatory drug (NSAID) cream. Her left knee pain was attributed to the KTW syndrome. She was not taking carbamazepine and prednisone at the time of the follow-up. Electrolyte studies revealed Na+ 138 mmol/L, K 4.6 mmol/L, and serum osmolality at 276 mmol/kg. | [[44.0, 'year']] | F | {'24569125': 1, '24074529': 1, '10824078': 1, '25822386': 1, '33019727': 1, '12170391': 1, '16189283': 1, '6327253': 1, '15168958': 1, '20525972': 1, '27737595': 1, '21777511': 1, '29991348': 2} | {} |
1,140 | 6040079-1 | 30,002,912 | comm/PMC006xxxxxx/PMC6040079.xml | Idiopathic full thickness macular hole in a 10-year-old girl | A 10-year-old girl presented with reduced vision in the right eye. The vision had deteriorated from 20/17 1 year previously, to 20/60. She was otherwise well, with no history of trauma or inflammation of either eye and no other significant medical or drug history. Her grandfather, and grandfather’s brother had a history of retinal detachment.\nVisual acuity (VA) was 20/60 in the right eye and 20/20 in the left eye. Ocular examination was unremarkable except for the presence of a FTMH in the right eye (Fig. ). There was no evidence of trauma, inflammation or signs of retinal dystrophy. Optical coherence tomography (OCT) showed a 365 μm FTMH with no vitreomacular traction or posterior vitreous detachment (Fig. ).\nFollowing informed consent of her parents, we carried out a 23-gauge transconjunctival pars plana vitrectomy. Induction of a posterior vitreous detachment (PVD) was completed using triamcinolone, followed by ILM blue-assisted internal limiting membrane (ILM) peel, fluid–air exchange and air-26% sulfur hexafluoride (SF6) exchange. She was positioned face down for 3 days postoperatively. At postoperative week 1, VA was 20/60 with OCT evidence of hole closure (Fig. ). At postoperative month 1, VA was 20/20, and at 4 months postoperative, the macular hole remained closed with remodeling of the outer retina on OCT (Fig. ). There was still a small defect at the photoreceptor level. | [[10.0, 'year']] | F | {'22241015': 1, '11320012': 1, '20180132': 1, '33099567': 1, '15734006': 1, '16365800': 1, '24094528': 1, '28433125': 1, '16038666': 1, '31856539': 2, '15642812': 1, '21210570': 1, '18465733': 1, '21914147': 1, '25709274': 2, '30002912': 2} | {'6951201-1': 1, '4333543-1': 1} |
1,141 | 6040207-1 | 30,069,255 | comm/PMC006xxxxxx/PMC6040207.xml | False Positive Troponin I Rendering Two Admissions for “Recurrent Acute Myopericarditis” | We report a case of a 18-year-old female patient with previous history of recurrent myopericarditis in 2015 and 2016. In both episodes, troponin I was elevated (maximum value 3.1 ng/mL) and slowly declined. In the last episode she took ibuprofen 600 mg every 8h for 2 weeks and colchicine 0.5 mg twice a day for 6 months. Physical activity was restricted for 6 months. Transthoracic echocardiography was normal but cardiac Magnetic Resonance Imaging (MRI) performed latter demonstrated residual fibrosis in the lateral wall of left ventricle.\nThe patient remained asymptomatic until June 2017 when she complained of palpitations and mild chest pain. Physical examination, Electrocardiogram (ECG) and transthoracic echocardiography were normal but troponin I was positive (1.04 ng/mL; normal range < 0.04 ng/mL) so myopericarditis was assumed and the patient was admitted\nto the hospital and ibuprofen and colchicines were initiated. The next day and until discharge, troponin I remained negative and the patient was free from symptoms. Serology for virus, interferon gamma release assay and clinical or laboratory tests for autoimmune diseases were all negative. The possibility of a false positive test on admission was thought due to the immediate fall in troponin level. However, owing to the relief of symptoms with medication, myopericarditis could not be excluded and the patient was discharged on ibuprofen and colchicine at the same dose as before.\nThree weeks later she returned to the hospital complaining of similar symptoms. Once again, initial measurement of troponin I was positive (1.31 ng/mL) and incessant myopericarditis was assumed. Physical exam remained unremarkable as well as the ECG and transthoracic echocardiography. During hospitalization, measurements of troponin I remained always negative. She had a cardiac MRI once again but no signals of active myocarditis were detected and residual fibrosis remained as previously described (Fig. ). Once more, a false positive result was a possibility since the first measurements were performed by the emergency laboratory (Beckman Coulter Access AccuTnI+3®) and the next ones by the routine laboratory (Abbott Architect STAT high sensitive TnI®). To rule out a false positive result in the first measurement, performed by emergency laboratory, two samples of blood were collected at the same time and sent to both laboratories. The result was positive in the emergency laboratory (1.03 ng/mL) and negative in the routine laboratory (0.00 ng/mL). Although alkaline phosphatase activity was within the normal range (58 U/L; normal range 40-150 U/L), alkaline phosphatase interference was proved in the Beckman Coulter central laboratory. To demonstrate that, beta human chorionic gonadotropin (βhCG) was measured using the Beckman Coulter Dxl 800 Total β-hCG® (Marseilles, France) assay [] (which is also an alkaline phosphatase dependent immunoassay) and the βhCG was 1.1 IU/L (age-specific reference interval 0-0.6 IU/L []) while using other assay [Siemen´s Vista hCG (Erlanger, Germany)] was 0 IU/L.\nRetrospectively, we confirmed that in the first two admissions, troponin I was elevated in both laboratories (and not substantially higher in one assay relatively to the other) so myopericarditis indeed existed. In order to avoid future hospital admissions, information regarding alkaline phosphatase interference with troponin I and other assays was provided to the patient and added to the patient´s files. | [[18.0, 'year']] | F | {'26320112': 1, '25684454': 1, '26345181': 1, '24897400': 1, '34113117': 1, '22752944': 1, '31538105': 1, '31223267': 2, '11436198': 1, '12008180': 1, '20632814': 1, '27317886': 1, '34833051': 1, '26346942': 2, '26391123': 1, '25283872': 1, '30069255': 2} | {'6559625-1': 1, '4554225-1': 1} |
1,142 | 6040243-1 | 30,050,710 | comm/PMC006xxxxxx/PMC6040243.xml | A Novel Adverse Event Associated with Olaparib Therapy in a Patient with Metastatic Breast Cancer | A 45-year-old female with a history of metastatic breast cancer presented with an erythematous rash in her bilateral lower extremities. She was diagnosed approximately four years previously with estrogen and progesterone receptor positive, HER-2-negative breast cancer with involvement of twelve axillary lymph nodes. At the time of diagnosis, she was also found to have bony metastatic disease, and genetic testing revealed a deleterious 3036del4 germline BRCA2 mutation. After multiple lines of therapy, including most recently progressing on palbociclib and fulvestrant, the patient was switched to monotherapy with olaparib. Her rash began approximately three days after starting olaparib capsules at a dose of 300 mg twice daily. She subsequently developed progression of the erythematous nodules which became painful and limited her ambulation, bilateral lower extremity edema, fevers to 101.7°F (degrees Fahrenheit), and rigors. She tried diphenhydramine without any improvement in her symptoms, and patient then presented to the emergency department for evaluation. Her other home medications included levothyroxine, omeprazole, and cholecalciferol.\nHer vital signs were within normal limits. Her physical examination revealed multiple erythematous nodules over the bilateral distal lower extremities which were markedly tender to palpation, along with trace edema in her bilateral lower extremities (). Her basic metabolic profile was unremarkable, and her complete blood count demonstrated white blood count of 1.80 with absolute neutrophil count of 1200, hemoglobin of 11.2, and platelet count of 114. Her urinalysis was unremarkable, chest X-ray was normal, and blood cultures were sent. The patient was then admitted to inpatient Hematology-Oncology service for further evaluation of neutropenic fever. However, her infectious evaluation was unrevealing, and she then remained afebrile off antibiotics. Since this patient's symptoms and clinical examination were consistent with erythema nodosum, her olaparib was held. She was treated with nonsteroidal anti-inflammatory medications as well as acetaminophen as needed for ongoing pain. She was discharged with close outpatient follow-up. Her nodules improved dramatically within 24 hours of stopping olaparib and completely resolved within a week of withholding olaparib.\nAt her subsequent outpatient follow-up appointment, the patient was resumed on olaparib with a slow titration of dose to her prior regimen of 300 mg twice daily. The first day she took the 300 mg BID dose, the patient developed recurrence of her erythema nodosum, and she was started on a course of prednisone with a resolution of her symptoms (). The patient was then restarted on olaparib at a reduced dose of 250 mg twice daily, but afterwards developed erythema nodosum again (). Her symptoms resolved after completing another short course of prednisone therapy. At her next follow-up appointment, the patient was later resumed on olaparib capsules at a dose of 200 mg twice daily. She has tolerated this therapy at a reduced dose without any significant recurrence of erythema nodosum requiring cessation of olaparib or use of prednisone therapy. This patient had a complete metabolic response to treatment in the previously described metastatic bone lesions, and the previously described focal liver FDG avidity was no longer seen; in addition, her CA27.29 normalized. Once olaparib tablets were commercially available, she was subsequently changed to therapy with olaparib tablets 300 mg twice daily. Since then, she similarly has continued to have intermittent but attenuated episodes of erythema that have not required treatment or cessation of therapy. | [[45.0, 'year']] | F | {'28611314': 1, '12202973': 1, '7294570': 1, '26187614': 1, '17888212': 1, '28706762': 2, '9762816': 1, '28578601': 1, '28754483': 1, '18793974': 1, '24746312': 1, '17560306': 1, '22452356': 1, '30050710': 2} | {'5507666-1': 1} |
1,143 | 6040249-1 | 30,079,258 | comm/PMC006xxxxxx/PMC6040249.xml | Successful Renal Transplantation between Identical Twins with Very Brief Immunosuppression | A 60-year-old Caucasian female with a history of ESRD secondary to hypertension and type 1 diabetes mellitus received a living related renal transplant from her HLA identical twin sister. At the time of transplant, monozygosity had yet to be determined. Therefore, she received a 3-day course of steroids, consisting of methylprednisolone 500 mg intraoperatively, followed by 250 mg and 125 mg on postoperative days (POD) 1 and 2, respectively. She also received maintenance immunosuppression of tacrolimus with a target trough level of 6 ng/mL and mycophenolic acid (Myfortic®) 360 mg twice daily. Postoperatively, blood samples from the donor and recipient were sent for short tandem repeat (STR) analysis in order to determine monozygosity. Given the use of maintenance immunosuppression, opportunistic infection prophylaxis was provided with oral sulfamethoxazole-trimethoprim 400-80 mg daily for Pneumocystis jirovecii pneumonia, fungal prophylaxis with oral clotrimazole 10 mg four times daily and cytomegalovirus (CMV) prophylaxis with valganciclovir 900 mg daily. She experienced immediate graft function and was discharged with no complications on postoperative day (POD) 5 with a serum creatinine (SCr) of approximately 1.0 mg/dL. The STR analysis occurred one week after transplant and demonstrated allelic identity at all 16 loci tested, consistent with monozygosity. Therefore, the transplant team decided to discontinue maintenance immunosuppression. Tacrolimus was stopped 12 days after transplant, and mycophenolate was stopped one week later. Infection prophylaxis was also discontinued once maintenance immunosuppression was stopped. She continues to have stable graft function at 5 years after transplant, with a baseline SCr of approximately 1.5 mg/dL (). She has experienced several episodes of mild acute kidney injury, which were attributed to her poorly controlled diabetes mellitus. She underwent a protocol allograft biopsy at 3 months after transplant, which showed no significant interstitial fibrosis and tubular atrophy, minimal arterial sclerosis, and mild arteriolar hyalinosis, with no evidence of acute allograft rejection. At 17 months after transplant, she underwent a second biopsy due to an elevation in SCr. The second biopsy showed mild tubular injury, without evidence of allograft rejection. No interventions were indicated based on the results of the 2 allograft biopsies, beside attempts to optimize her glycemic control. She has had no infectious or neoplastic complications from short-term immunosuppression. | [[60.0, 'year']] | F | {'13278189': 1, '10620213': 1, '27686224': 1, '12270364': 1, '17186463': 1, '17391129': 1, '17295642': 1, '8826810': 1, '19624493': 1, '8066655': 1, '25102303': 1, '12711830': 1, '20121740': 1, '18808409': 1, '11477152': 1, '20977497': 1, '16539620': 1, '21929642': 1, '12216942': 1, '16772284': 1, '18844834': 1, '7676492': 1, '19363805': 1, '11440823': 1, '19077892': 1, '9632172': 1, '3524026': 1, '30079258': 2} | {'6040249-2': 2} |
1,144 | 6040249-2 | 30,079,258 | comm/PMC006xxxxxx/PMC6040249.xml | Successful Renal Transplantation between Identical Twins with Very Brief Immunosuppression | A 71-year-old Caucasian female received a living related renal transplant from her identical twin sister. She developed ESRD due to chronic cystitis related to ureteral obstruction. Prior to transplantation, she made normal amounts of urine and had been on hemodialysis for 6 months. Her past surgical history included an ileal loop conduit urinary diversion and ileostomy drainage bag placement. Given the recipient's structural bladder anomaly, a ureteral anastomosis was made to the small bowel over a 6-French 30 cm double-J stent which extended from the renal pelvis out to the ileal conduit. Prior to transplant, monozygosity was confirmed via an STR analysis, which revealed that she was identical to her sister in all 16 polymorphic gene loci that were evaluated. Additionally, she was found to be a six-antigen match on HLA typing and had a negative anti-human globulin (AHG) crossmatch. Interestingly, a class II donor specific antibody (DSA) to DPB1∗05:01/DPA1∗02:02 with a mean fluorescence intensity (MFI) of 1,359 was identified four months prior to transplant. The antibody screening was repeated 10 days prior to transplant and revealed an MFI of 1,458. Based on the confirmation of monozygosity, the transplant team proceeded with transplantation without maintenance immunosuppression. She received a 3-day steroid taper course consisting of methylprednisolone 500 mg intraoperatively, followed by 250 mg and 125 mg intravenously on POD 1 and 2, respectively. She did not receive antibody induction therapy or further maintenance immunosuppression beyond the corticosteroids. Due to rapid immunosuppression withdrawal, she did not receive prophylaxis against opportunistic infections. She experienced immediate graft function, although her SCr was initially slow to decline (). Her hospital course was uncomplicated, and she was discharged home on POD 4 with a SCr of 2.3 mg/dL. She experienced a urinary tract infection (UTI) approximately 1 month after transplant, which resolved after ureteral stent removal and treatment with a 7-day course of oral ciprofloxacin. At 1 year after transplant, she continues to have excellent graft function with a baseline serum creatinine of 1.0 ng/mL (). A protocol kidney biopsy was attempted approximately 3 months after transplant, which however could not be performed due to lack of a clear window. Repeat posttransplant surveillance DSA screening with high resolution typing at the time of the attempted biopsy revealed persistence of Class II DPB1∗05:01/DPA1∗02:02 antibody, with a stable MFI of 1,030. She remains free from long-term complications due to brief immunosuppression, including infections, malignancy, or metabolic complications. | [[71.0, 'year']] | F | {'13278189': 1, '10620213': 1, '27686224': 1, '12270364': 1, '17186463': 1, '17391129': 1, '17295642': 1, '8826810': 1, '19624493': 1, '8066655': 1, '25102303': 1, '12711830': 1, '20121740': 1, '18808409': 1, '11477152': 1, '20977497': 1, '16539620': 1, '21929642': 1, '12216942': 1, '16772284': 1, '18844834': 1, '7676492': 1, '19363805': 1, '11440823': 1, '19077892': 1, '9632172': 1, '3524026': 1, '30079258': 2} | {'6040249-1': 2} |
1,145 | 6040262-1 | 30,050,706 | comm/PMC006xxxxxx/PMC6040262.xml | A Blind Spot in the Diagnostic Field: The Challenging Diagnosis of Tumefactive Multiple Sclerosis | A 29-year-old female with no medical history presented to the hospital for acute left-sided vision loss. Associated symptoms included photophobia, floaters, and bifrontal headache. On examination, she had left homonymous hemianopia, but no other neurologic deficits. Computed tomography revealed acute ischemia involving the right parieto-occipital lobe with vasogenic edema. Magnetic resonance imaging () revealed a mass in the aforementioned region. Initial differential was ischemia versus neoplasm. She was started on dexamethasone and underwent brain biopsy. Pathology revealed white matter infiltration by macrophages intermixed with reactive astrocytes with loss of myelin in the white matter. Myelin was seen within the macrophages. Axons were preserved. Overall the findings were consistent with active demyelination with no findings to suggest neoplasm, most consistent with tumefactive multiple sclerosis. Cerebral spinal fluid (CSF) analysis supported the diagnosis with elevated immunoglobulin G, immunoglobulin G/albumin ratio, and immunoglobulin G index. CSF analysis for oligoclonal bands which is positive in up to 30% of patients with TMS had a negative result in our patient []. Despite this, given the patient's clinical presentation and definitive findings on biopsy she was diagnosed with tumefactive multiple sclerosis. She received one gram of intravenous methylprednisolone daily for a total of five days and was then transitioned to prednisone taper. She was discharged with stable neurologic status to inpatient rehabilitation with plans to start immunomodulatory therapy as an outpatient. A four-month chart review of the patient's record revealed that she is doing well. She is currently completing speech therapy with goals to improve mild language deficits including auditory comprehension and integration, verbal expression, and thought organization. | [[29.0, 'year']] | F | {'26472944': 1, '14663037': 1, '12082058': 1, '19181773': 1, '21387374': 1, '23035065': 1, '30800548': 2, '20100418': 1, '23334629': 1, '27603348': 2, '18535080': 1, '1481644': 1, '27531859': 1, '28090055': 1, '30050706': 2} | {'6384036-1': 1, '5023870-1': 1} |
1,146 | 6040269-1 | 30,050,707 | comm/PMC006xxxxxx/PMC6040269.xml | Congenital Uterovaginal Prolapse in a Newborn | A 6-hour-old female neonate born from a 22-year-old Para 1 mother after term pregnancy. The newborn had protrusion of mass per vagina since birth; she also has a defect at the lower back with no discharge since the time of birth. Additionally the mother has noticed deformity on both legs and feet which barely move. The baby was active since the time of delivery and is sucking well and she passed meconium. The mother had two ANC visits and was vaccinated according to the national schedule and she reported the whole course of the pregnancy as uneventful. She did not have any known medical illness and has never taken any medication during the entire course of the index pregnancy except iron folate which was prescribed during the ANC visit.\nShe had spontaneous onset of labor and the membrane was ruptured spontaneously intrapartum. She gave birth to a 2330-gram female neonate after 5 hours of labor at a local health center. Baby had good APGAR score. Health care providers at the health center noticed defect at the back of the baby and referred her to Hawassa University Comprehensive Specialized Hospital (HUCSH) with the diagnosis of spinal bifida.\nDuring the initial evaluation at HUCSH the baby was active, vital signs were in the normal limits, and all neonatal reflexes were intact. There was 4x4 cm pink mass protruding through the introitus, cervical os is noted at the tip of the mass, the external genitalia appears normal, no discharge or bleeding from the mass, and the mass was reducible digitally and increases in size when the baby cries (see ). There was also 4x4 cm defect at the lumbosacral region the major portion of which is covered with skin while the lower edge is open, no discharge from the mass (see ). Additionally the newborn had bilateral club foot deformity (see ).\nOn investigation, the complete blood count was normal, creatinine level was 0.4mg/dl, and transfontanelle ultrasound scan shows mild dilatation of the lateral and third ventricles with an index of “mild hydrocephalus” and abdominal ultrasound was normal.\nAfter obtaining consent from the parents, under aseptic technique, the baby was catheterized, the vaginal mass was reduced digitally, and bandage was applied from the lower abdomen, both buttocks and legs were strapped in the bandage to the level of the mid-thigh leaving an opening at the anal orifice for passage of stool (see ). The bandage was removed after 72 hours at which time the mass was completely reduced and there was no recurrence of the prolapse afterwards (see ). Unfortunately one day after the removal of the bandage the baby started to shoot fever and had difficulty of sucking, on examination she had tachycardia and tachypnea and was febrile, she had depressed reflexes, the lower border of the meningocele got ruptured, her fontanels were bulged, and she was diagnosed with ruptured myelomeningocele and meningitis. The plan was to start her on antimeningitis drugs and repair the spinal cord defect after improvement but the parents insisted and went home against medical advice frustrated by her multiple anomalies. We communicated with the parents after her discharge and were informed that the baby died 7 days after she went home, but there was no recurrence of the prolapse throughout her stay. | [[6.0, 'hour']] | F | {'3895085': 1, '16481269': 1, '23543808': 1, '26023398': 1, '21508572': 1, '15009585': 1, '6726586': 1, '8657402': 1, '24741427': 2, '30050707': 2} | {'3977332-1': 1} |
1,147 | 6040285-1 | 30,050,704 | comm/PMC006xxxxxx/PMC6040285.xml | Auditory Hallucinations as a Rare Presentation of Occipital Infarcts | A 56 year-old female was transported to the emergency department by EMS after physically threatening her husband with a knife. According to the patient, she experienced altered sensorium for one week prior to presentation, primarily resulting in suicidal and homicidal command hallucinations instructing her to overdose on NSAIDs and kill her husband. She denied any headaches, vertigo, fevers, head trauma, urinary symptoms, or use of illicit substances. There was no history of psychosis, schizophrenia, mania, or depression and a review of her medication list for any potential hallucinogenic agents did not indicate a pharmacologic etiology. Her past medical history was comprised of two prior strokes, the most recent being two years ago that involved the right frontal lobe. MRI of the brain at the time also showed evidence of an old right parietal lobe infarct. An echocardiogram during that hospitalization revealed a severely decreased left ventricular function and the patient was initiated on warfarin for anticoagulation. Since the two prior cerebral infarcts, the patient and her husband denied noting any behavioral changes, cognitive impairment, or any focal neurologic deficits. On presentation, she appeared disheveled and exhibited a flat affect with minimal verbalization. Physical examination revealed only a left hemianopia without hemiplegia. All cranial nerves were evaluated as well as gait, cerebellar function, and proprioception, which were all found to be normal. The patient was alert and oriented with intact mentation.\nShe was initially admitted to the psychiatric unit for further evaluation where the patient participated in daily activities and reported no symptoms. Given the acute onset of her symptoms, laboratory studies and brain images were obtained in order to rule out an organic etiology. A complete blood count was normal and a basic metabolic panel revealed normal electrolytes and renal function. Additional labs included liver function tests, lipid panel, cardiolipin antibody, and TSH, which were all normal and RPR was nonreactive. A urine sample was negative for urinary tract infection and 8-panel urine drug screen was negative. An MRI of the brain was obtained, which revealed old ischemic infarcts within the right parietal and frontal lobes along with a new acute right occipital lobe infarct with hemorrhagic transformation (). The patient was subsequently transferred to the medical unit for further workup and management.\nThe patient was placed on telemetry, which showed normal sinus rhythm. Given the involvement of multiple brain territories and circulations, a cardioembolic source of stroke was highly suspected. A cardiologist evaluated the patient and performed a transesophageal echocardiogram that revealed a low ejection fraction of less than 20%. In addition, a bubble study was performed that was positive for a patent foramen ovale. Given her significantly low ejection fraction, the patient underwent placement of an automated implantable cardioverter-defibrillator. The patient's presenting symptom of psychosis, primarily in the form of auditory hallucinations, was self-limited and resolved on day two of hospitalization without requiring the use of antipsychotic medications. | [[56.0, 'year']] | F | {'20662758': 1, '16518011': 1, '19927238': 1, '26243819': 1, '21691873': 1, '21686449': 1, '25401133': 1, '10986195': 1, '27733031': 1, '11122995': 1, '7996290': 1, '26289573': 1, '19641245': 1, '28658884': 1, '29403140': 2, '29209479': 2, '26166588': 1, '30050704': 2} | {'5795689-1': 1, '5711658-1': 1} |
1,148 | 6040287-1 | 30,050,701 | comm/PMC006xxxxxx/PMC6040287.xml | Report of Four Cases with Equestrian Injury: Therapeutic Approach and Outcome | A 20-year-old female fell and was kicked several times by the horse on the thoracoabdominal area. The patient was transferred to the emergency room (ER). Vital signs included blood pressure: 122/82 mmHg; heart rate: 72 beats per minute (bpm); body temperature: 36.3°C; respiratory rate: 18/min; and SpO2: 100% (ambient air). The patient complained of epigastralgia and tenderness was observed at the same site. Focused assessment with sonography for trauma (FAST) was positive at the Morison's pouch, perisplenic and pericystic regions. Laboratory data revealed elevated levels of hepatic enzymes (aspartate aminotransferase: 174 U/l; alanine aminotransferase: 149 U/l). Enhanced abdominal computed tomography (CT) showed a hepatic injury (abbreviated injury scale [AIS]: 4; revised trauma score [RTS]: 7.8408; probability of survival [Ps]: 0.94702; Figures -). Enhanced CT did not show obvious extravasation and the patient's general condition was stable. Thus, nonoperative management (NOM) was selected. Transcutaneous drainage of the biloma was performed () and the drainage tube was removed () on days 17 and 34 of hospitalization, respectively. The clinical course was stable and the patient was discharged on day 37 of hospitalization. Forty months after injury the patient has fully recovered. | [[20.0, 'year']] | F | {'10027058': 1, '29123740': 1, '16247251': 1, '24767580': 1, '17049524': 1, '25177363': 1, '30050701': 2} | {'6040287-2': 2, '6040287-3': 2, '6040287-4': 2} |
1,149 | 6040287-2 | 30,050,701 | comm/PMC006xxxxxx/PMC6040287.xml | Report of Four Cases with Equestrian Injury: Therapeutic Approach and Outcome | A 60-year-old female fell and was trampled on the left side of the chest by the horse. The patient was transferred to the ER. Vital signs included blood pressure: 156/116 mmHg; heart rate: 113 bpm; body temperature: 36.5°C; respiratory rate: 30/min; and SpO2: 96% (ambient air). An abrasion on the left chest without flail chest was observed. Chest roentgenography and CT showed left hemopneumothorax and pulmonary contusion with multiple rib fractures (AIS: 3; RTS: 7.55; Ps: 0.95590; Figures -). A chest tube was placed to the left chest and NOM was selected for the rib fractures due to the little deviation. The patient's respiratory condition was stable, and endotracheal intubation and mechanical ventilation were not required during the course of treatment. The chest tube was removed on day 6 and the patient was discharged on day 8 of hospitalization. Twenty-seven months after injury the patient has fully recovered. | [[60.0, 'year']] | F | {'10027058': 1, '29123740': 1, '16247251': 1, '24767580': 1, '17049524': 1, '25177363': 1, '30050701': 2} | {'6040287-1': 2, '6040287-3': 2, '6040287-4': 2} |
1,150 | 6040287-3 | 30,050,701 | comm/PMC006xxxxxx/PMC6040287.xml | Report of Four Cases with Equestrian Injury: Therapeutic Approach and Outcome | A 30-year-old female fell and was injured in the lumbar area. The patient was transferred to the ER. Vital signs included blood pressure: 159/91 mmHg; heart rate: 77 bpm; body temperature: 36.5°C; respiratory rate: 12/min; and SpO2: 100% (ambient air). The patient complained of lumbago. Roentgenography and CT showed a compression fracture of Th12 (AIS: 3; RTS: 7.8408; Ps: 0.99416; Figures -). Conservative therapy using a medical corset was advised, and the patient was discharged on day 17 of hospitalization. Thirty-seven months after injury the patient has fully recovered. | [[30.0, 'year']] | F | {'10027058': 1, '29123740': 1, '16247251': 1, '24767580': 1, '17049524': 1, '25177363': 1, '30050701': 2} | {'6040287-1': 2, '6040287-2': 2, '6040287-4': 2} |
1,151 | 6040287-4 | 30,050,701 | comm/PMC006xxxxxx/PMC6040287.xml | Report of Four Cases with Equestrian Injury: Therapeutic Approach and Outcome | A 50-year-old male fell and was bitten on the crotch by the horse. The patient was transferred to the ER. Vital signs included blood pressure: 161/118 mmHg; heart rate: 71 bpm; and body temperature: 36.3°C. There was a 5-cm laceration on the right scrotum (AIS: 1; RTS: 7.8408; Ps: 0.99700). The tunica vaginalis of the right testis was macroscopically intact, while ultrasonography did not reveal abnormal findings in the right testis. The laceration was sutured after cleansing with normal saline under local anesthesia. The clinical course was uneventful and the patient was discharged from the hospital the following day. | [[50.0, 'year']] | M | {'10027058': 1, '29123740': 1, '16247251': 1, '24767580': 1, '17049524': 1, '25177363': 1, '30050701': 2} | {'6040287-1': 2, '6040287-2': 2, '6040287-3': 2} |
1,152 | 6040289-1 | 30,050,709 | comm/PMC006xxxxxx/PMC6040289.xml | Malignant Phyllodes Tumor of the Breast with Metastasis to the Pancreas: A Case Report and Review of Literature | A 34-year-old female who has no previous clinical illness presented in 2000 with a large irregular mass, estimated clinically to be around 5 × 6 cm by physical examination, involving the right breast while she was lactating. There were no other signs or symptoms. FNA was done to rule out breast cancer, and the specimen showed few foamy macrophages and rare clusters of ductal cells, with focal secretory lactational changes. Based on that, it was determined that the specimen was negative for malignancy. The tumor continued to grow, and in June 2004, FNA of the right breast was repeated and showed large staghorn-shaped sheets of uniform ductal cells with no cytologic atypia along with myoepithelial cells. There were fragments of fibrotic stroma and bare nuclei scattered in a bloody background, and so, a diagnosis of fibroadenoma was made. Two months later, the patient underwent excision of the tumor in another healthcare facility. Examination of the slides showed an overgrowth of epithelial and stromal components in pericanalicular and intracanalicular patterns with irregular large cystic spaces showing protruding leaf-like structures (). The stromal cells showed plump elongated slightly dysmorphic nuclei with occasional prominent nucleoli. Other areas showed significant cellular growth of the stromal spindle cells which appeared to be arranged in long intersecting fascicles and growing in herringbone pattern (). Within the spindle cell growth, extracellular mucin was noted. One focus showed an exclusive spindle cell growth which was moderately atypical with plump elongated hyperchromatic nuclei. Numerous mitosis was found, up to 20 in 10 high power fields in this focus. Entrapped epithelial ductal elements were occasionally seen in other areas exhibiting ductal epithelial hyperplasia. These features were consistent with malignant phyllodes tumor with stromal fibrosarcomatous overgrowth. The patient subsequently underwent mastectomy as the margins were focally involved. During the procedure, a mass measuring around 3-4 cm was found in the most posterior aspect of the breast and there was some indication that it may have invaded the pectoralis major muscle. All the breast tissue, the tumor, and some of the muscle fibers were removed. On gross examination, there were multiple tumor masses in the inner lower and outer upper quadrants, the largest being 3 cm. On microscopy, there was proliferation of atypical spindle and elongated plump cells with pleomorphic nuclei and occasional prominent nucleoli. Extracellular mucin was also identified. The atypical stromal cells formed herringbone fascicular growth pattern reminiscent to fibrosarcoma and numerous mitosis averaging around 14 mitosis in 10 high power fields. No residual phyllodes tumor elements were identified. The tumor nodules had a well-demarcated margin with focal infiltration of surrounding breast and adipose tissue. No lymphovascular invasion, necrosis, or heterologous differentiation was seen. All surgical margins and muscle fibers were not involved by the tumor. The skin and nipple did not show any involvement either. All these histological features were identical to those identified in her previous lumpectomy except that there was more pronounced atypia and no residual epithelial component of phyllodes tumor identified. No lymph nodes were identified. A year later, the patient began to suffer from acute bouts of pancreatitis and was admitted several times for this. In March of 2006, abdominal CT was performed and showed a heterogenous low attenuation soft tissue mass involving the head and body of the pancreas and was extending upwards. The celiac vessels and its branches were going through this mass but did not show any significant narrowing. There was obvious atrophy of the pancreatic tail and dilatation of the pancreatic duct. The mass was in contact with the anterior aspect of the inferior vena cava (IVC) with no clear fat plane in between (). Biopsy of the pancreatic mass showed uniform proliferation of elongated spindly cells which had coarse chromatin and mild to moderate nuclear pleomorphism. Some cells had plump hyperchromatic nuclei. Numerous mitotic figures were identified (). The background showed variable amounts of collagen and stroma with focal areas of myxoid appearance. Immunohistochemical staining showed strong positivity for vimentin; however, the cells were negative for actin, S100, and cytokeratin (). These findings were consistent with metastatic fibrosarcoma of the pancreas secondary to her primary breast lesion. Due to the location, extent, and nature of the condition, the case was deemed unresectable. Chemotherapy was initiated; and in the little chance that the tumor shrunk enough, the possibility of resecting the metastasis would be entertained. In January 2007, the patient presented to the ER with severe epigastric pain, where she collapsed, was hypotensive, and subsequently admitted. On examination, a tender mass in the epigastric area was felt. Urgent CT of the abdomen and pelvis with and without contrast showed a pseudoaneurysm in the splenic artery measuring around 3.5 cm. There was also expansion of the retroperitoneal mass and blood in the intraperitoneal cavity. There was heterogenous enhancement in the liver most probably indicating liver infarct. In addition, there were areas of the IVC which had markedly thinned wall and areas suspicious for active bleeding, especially from the proximal splenic artery (). The pancreatic fibrosarcoma had grown and eroded branches of the celiac artery with bleeding pseudoaneurysm along with infarct of the liver, spleen, and adjacent organs. The patient continued to be hypotensive with abdominal distention due to severe intra-abdominal bleeding and developed multiorgan failure and hemorrhagic shock leading to her death. | [[34.0, 'year']] | F | {'8608483': 1, '8659370': 1, '25931913': 1, '26287414': 1, '29147192': 1, '19329316': 1, '8522284': 1, '17549503': 1, '33282161': 1, '12540427': 1, '17562113': 1, '11423590': 1, '33242383': 2, '24522677': 1, '23577269': 1, '30050709': 2} | {'7787903-1': 1} |
1,153 | 6040295-1 | 30,050,719 | comm/PMC006xxxxxx/PMC6040295.xml | Those Who Hear Music: Three Cases on Musical Hallucinations | A 29-year-old female was referred by the neurosurgical team as she complained of hearing music for one-week duration. She was admitted with the complaints of severe headache of sudden onset and was found to have intraventricular and intracranial haemorrhages. Following the surgical evacuation of the haemorrhages she made a full recovery. Hearing of music started one week following the surgery. She was hearing familiar songs in increased volume with distorted sounds. She had no hallucinations of any other modalities and nor there were any delusions. Her consciousness was clear and she was oriented in time, place, and person. There were impairments in her short-term memory and long-term memory along with frontal lobar impairment. She had no neurological deficits in the physical examination. A Noncontrast CT (NCCT) brain following the onset of musical hallucinations revealed no abnormalities. She was started on quetiapine 25 mg and was gradually titrated up to 150mg for which she responded. One year following the initiation of treatments she remains symptom free. | [[29.0, 'year']] | F | {'23530017': 1, '3607711': 1, '33628561': 1, '26446167': 1, '2180526': 1, '19864669': 1, '15546592': 1, '4841585': 1, '29213612': 1, '8290053': 1, '30050719': 2} | {'6040295-2': 2} |
1,154 | 6040295-2 | 30,050,719 | comm/PMC006xxxxxx/PMC6040295.xml | Those Who Hear Music: Three Cases on Musical Hallucinations | An 86-year-old lady complained of being persecuted by her neighbours for 3-month duration. She reported that 2 males are living inside her body controlling her activities. She heard persistent voices chanting “pirith” (a form of religious sermon) for a duration of 2 years. The sounds were slow and rhythmic in nature. She found great relief with the voices and believed that she has achieved a higher spiritual status. In addition, she had persecutory delusions, delusions of control, and somatic hallucinations. Her cognitive functions were normal. She was diagnosed with very late onset schizophrenia and was commenced on risperidone 2mg in the night which was gradually increase to 5mg in the night. Even though her symptoms gradually improved, she regretted it as she no longer heard “pirith” chanting. She had no hearing impairment. One year following the initiation of treatments she has no symptoms. | [[86.0, 'year']] | F | {'23530017': 1, '3607711': 1, '33628561': 1, '26446167': 1, '2180526': 1, '19864669': 1, '15546592': 1, '4841585': 1, '29213612': 1, '8290053': 1, '30050719': 2} | {'6040295-1': 2} |
1,155 | 6040299-1 | 30,050,702 | comm/PMC006xxxxxx/PMC6040299.xml | Diagnostic Dilemma in Two Cases of Hyperandrogenism | A 60-year-old woman came to the endocrinology clinic with a complaint of rapidly progressive signs and symptoms of hyperandrogenism over 6 months. She mentioned hirsutism noticed especially in the face and chin, hair loss that took a male-pattern baldness in all over the head, deepening voice, and increased libido.\nShe had no galactorrhea, muscle weakness, hyperpigmentation, bruising, weight loss, or anorexia.\nShe was married, housewife, and smoker (5 pack-years), got 6 children, did not consume alcohol; she had regular menses since puberty until she had amenorrhea 22 years ago after hysterectomy (because of leiomyoma). She was diagnosed with hypothyroidism 15 years ago treated with L-Thyroxine (700 μg∖weekly) and osteoporosis 7 years ago treated with Calcium supplements + alendronate 70 mg weekly. She denied the use of any drugs that may cause hyperandrogenism.\nOn examination, the patient seemed well. The blood pressure was 120/80 mm Hg, the pulse 83 beats per minute, the height 154 cm, the weight 72 kg, and the body mass index (BMI; the weight in kilograms divided by the square of the height in meters) 30.2 (obesity class I). The Ferriman-Gallwey score for hirsutism estimation was 6 (4 in the chin, 2 in upper lip); she had acanthosis nigricans, frontal baldness, and clitoromegaly (2 cm by 3 cm) as shown in (). Except for a cesarean scar in the abdomen, the rest of examination was unremarkable.\nLaboratory studies revealed a hemoglobin concentration of 15 g/dL, serum sodium level of 141 mEq/L, and potassium level of 4.5 mEq/L. An automated chemistry panel showed normal findings. Hormonal studies were as in .\nTransvaginal ultrasonography showed that uterine and left ovary was removed, right ovary measured 2.1 cm by 2 cm by 4.5 cm with a volume of 7.8 cm3. Abdominal computed tomography (CT) showed bilateral adrenal nodular hyperplasia as in (). All adrenal function tests (hypo- and hypersecretion) were proved to be normal: 24-hour urine normetanephrine 47.3 μ/24hours (up to 600), metanephrine 107 μ/24hours (up to 350), 8 A.M cortisol 13.02 μg/dL, adrenocorticotrophic hormone ACTH 19.69 pg/mL (7-63), and 11 P.M cortisol 1.66 μg/dL\nIn order to distinguish ACTH-dependent hyperandrogenism from other causes of hyperandrogenism, a 48-h low-dose (2mg) dexamethasone-suppression test was carried out [], without a decrease in testosterone value (10.94 ng/mL) though enough cortisol suppression at the end of the test 0.58 μg/dL.\nIn such cases the catheterization of the adrenal and ovarian veins may be useful in identifying the source of hyperandrogenism but it was not available at our center. Since the lack of dexamethasone-induced inhibition of testosterone was suggestive of an ACTH-independent etiology mainly ovarian, and the patient was postmenopausal, the decision of laparoscopic oophorectomy was made. The pathology report confirmed the diagnosis of 2.8 cm Leydig cell tumor (). Testosterone was performed 72-hour postsurgery and it was 0.03 ng/mL. 17 hydroxy-progesterone and testosterone were performed 1 month later and they were in normal limits. | [[60.0, 'year']] | F | {'22335316': 1, '12788867': 1, '10685333': 1, '29132723': 1, '28492032': 1, '22802276': 1, '25225480': 1, '21167382': 1, '16263820': 1, '14668717': 1, '24849517': 1, '22669303': 1, '24188447': 1, '12007895': 1, '30050702': 2} | {'6040299-2': 2} |
1,156 | 6040299-2 | 30,050,702 | comm/PMC006xxxxxx/PMC6040299.xml | Diagnostic Dilemma in Two Cases of Hyperandrogenism | A 39-year-old woman came to endocrinology clinic with a complaint of hirsutism started 4 years ago, alongside with oligomenorrhea followed by amenorrhea two years ago. There was no temporal baldness or deepening voice.\nThe patient was treated for a period of 3 months with combined oral contraceptive pills COCP and cyproterone acetate without improvement in symptoms, 6 months earlier to admission.\nShe was married, got 3 children and was nonsmoking or alcohol consuming. She was diagnosed 5 years ago with rheumatoid arthritis and treated for only one month with prednisolone and methotrexate.\nOn examination, she seemed well. The blood pressure was 120/80 mm Hg, the height 155 cm, the weight 65 kg, and the body mass index BMI 27.1 (overweight). The Ferriman-Gallwey score for hirsutism estimation was 16 (4 points for each chin, upper lip, low abdomen, and medial thigh), clitoromegaly (1 cm by 0.5 cm); she had no acanthosis nigricans or frontal baldness. Otherwise, she had normal findings.\nLaboratory studies revealed a hemoglobin concentration of 10.3 g/dL, ferritin 10 ng/mL, serum sodium level of 138 mEq/L, and potassium level of 4.15 mEq/L. An automated chemistry panel showed normal findings except for fasting glucose 119 mg/dL. She started metformin therapy and ferrous replacement. Hormonal studies were as in .\nTransvaginal ultrasonography showed that ovaries measured 3.3 by 2 cm and 3.2 by 2 cm for right and left ovary, respectively, without masses. CT scan for adrenals was within normal also and right ovary measured 3.8 by 2.3cm as shown in .\nSince catheterization of the adrenal and ovarian veins was not available, the diagnostic and therapeutic options were explained to the patient and giving that she was not interested in future fertility, she underwent laparoscopic exploration for oophorectomy.\nPathologic report sowed 2.5 cm of Leydig cell tumor in the right ovary, while the left ovary was within normal as shown in (). Testosterone was normalized after surgery. | [[39.0, 'year']] | F | {'22335316': 1, '12788867': 1, '10685333': 1, '29132723': 1, '28492032': 1, '22802276': 1, '25225480': 1, '21167382': 1, '16263820': 1, '14668717': 1, '24849517': 1, '22669303': 1, '24188447': 1, '12007895': 1, '30050702': 2} | {'6040299-1': 2} |
1,157 | 6040606-1 | 30,009,190 | comm/PMC006xxxxxx/PMC6040606.xml | Free-style puzzle flap as a cross-leg pedicled flap: the concept of re-using a flap in acute burns, a case report | A 55-year-old woman without relevant medical history was referred to our burn center with 35% total body surface area full thickness thermal burns (third degree) from flames on her lower limbs, hands and face (UBS 110, ABSI 9) (Fig. ). The burns on her right ankle and foot and left knee seemed to be deeper (fourth degree). Initial surgical treatment occurred 2 h after the burns (Day 0) and included releasing incisions from knee to toes in both her lower limbs. The initial dressing was composed of silver sulfadiazine 1% and general resuscitation included fluid resuscitation and invasive monitoring. Then, three surgical procedures were required to excise the devitalized tissues, including muscles and tendons of both lower limbs. Thighs and upper third of her legs were covered with skin autografts. Tibia and fibula were exposed on the right limb. Toes were amputated through the metatarso-phalangeal joints. Ankle, mediotarsal and tarso-metatarsal capsules were burnt, some joints were open (Fig. ) and vasculo-nervous pedicle was at high risk of exposure. Consequently, the lower extremity required covering with vascularized tissues. Local pedicled flap was not an option for the ankle and foot; therefore, we opted for a latissimus dorsi musculo-cutaneous flap with vertical skin paddle (4 × 20 cm) centered on the main perforator of the anterior branch of the thoraco-dorsal pedicle, identified with an echo-doppler. The flap was performed on day 27 post-burn. On the pre-operative CT-angiogram of the lower limb, we noticed that the anterior tibial artery was thrombosed just at the second third of the leg. The posterior tibial pedicle was preserved to ensure the vascularization of the remaining tissues of the foot. The thoraco-dorsal pedicle was end-to-end anastomosed to the anterior tibial artery. The anastomoses were difficult to perform because inflammatory tissues, full of oedema, surrounded the vessels. The skin paddle of the latissimus dorsi flap was originally intended for flap monitoring (Fig. ). The free flap was a success, and 3 weeks later, the right limb was almost totally covered either with skin graft or with the flap. Nevertheless, the left patellar tendon was still exposed and needed to be covered with a flap (Fig. ). The options discussed were to perform a pedicled flap, to perform a second free flap or to recycle tissues from the previous flap. We performed a vascular mapping of the flap with the use of an echo-doppler. The main pedicle of the flap was divided into two branches. The skin paddle was vascularized by this anterior branch from which a main perforator was identified. The choice between a musculo-cutaneous flap and a perforator flap was discussed. As a cross-leg had to be performed, the risks of rupture due to excessive tension or of thrombosis due to desiccation of the perforator were considered as too high. Consequently, a musculo-cutaneous cross-leg flap was harvested from the previous flap. The flap was partially raised from the right limb including the anterior branch of the thoraco-dorsal pedicle and a cross leg was performed to cover the left patellar tendon. Both legs were immobilized together using an external fixation (Hoffmann apparatus, Fig. ). The donor site was covered with a skin graft. After a period of 3 weeks, the skin paddle was progressively cut from the previous flap after partial occlusion clamps were tested. One week later, the patient was discharged to a rehabilitation center for further treatment. The wounds were completely healed 9 weeks after the burns. At 6 months post-burns, the patient was able to walk and left the rehabilitation centre (Fig. ). | [[55.0, 'year']] | F | {'24522174': 1, '8884009': 1, '18281793': 1, '24518305': 1, '8115502': 1, '21492067': 1, '21811967': 1, '25983220': 1, '2734390': 1, '21703953': 1, '21354884': 1, '11525856': 1, '23800463': 1, '26668561': 1, '27673542': 1, '23357987': 1, '25692987': 1, '27574621': 2, '30009190': 2} | {'4994499-1': 1} |
1,158 | 6041124-1 | 30,009,031 | comm/PMC006xxxxxx/PMC6041124.xml | Use of low-cost three-dimensional printer to simulate grasping of bronchial foreign body | An 89-year-old man with dementia was hospitalized in our department with severe pneumonia. Chest computed tomography (CT) showed left lower lobe consolidation and a small high-density lesion suspected to be a foreign body in the left main bronchus. We performed bronchoscopy one week after initiation of antibiotic treatment, and his oxygen demand decreased from 7 to 4 L/min.\nBefore bronchoscopy, we moulded a 3D model of the bronchial foreign body using a low-cost 3D printer (UP! Plus2; OPT, Tokyo, Japan) with an acrylonitrile butadiene styrene (ABS) resin filament. It took approximately 1 h from uptake of the Digital Imaging and Communications in Medicine chest CT images to the completion of the 3D model. We found that the model had the shape of a molar tooth. Because this 3D printer utilizes a fused filament fabrication (FFF) prototyping system, the surface of the model becomes moderately rough. Therefore, we dipped the model in the ABS solvent eSolve (Kaneko Chemical, Saitama, Japan), a halogen alkylate, to make the surface smoother, resembling a real tooth .\nUsing this 3D model, we simulated grasping of the foreign body with all available types of grasping forceps. Although we failed to grasp it with several types of biopsy forceps, an alligator forceps, and a five-pronged grasping forceps, we succeeded using a basket-type forceps, a retrieval net, and a shark-tooth forceps, especially when grasping the recess site of the 3D model (Fig. ).\nBecause space was present around the foreign body in the left main bronchus on chest CT, we initially planned to use a basket-type forceps or a retrieval net. However, several days after planning, a chest radiograph showed that the foreign body had moved to the right bronchus. When inserting the bronchoscope, a dental foreign body covered with mucinous sputum was stuck in the right upper bronchus. Because the recess site of the foreign body was turned to the peripheral side, we scraped it out to the truncus intermedius using a retrieval net. We could recognize the recess site and thus grasped and removed it with a shark-tooth forceps as performed in the simulation (Fig. ). It took just 9 min from bronchoscope insertion to foreign body removal, without adverse events. | [[89.0, 'year']] | M | {'25862113': 1, '28712105': 1, '27976687': 1, '25969517': 1, '30009031': 2} | {} |
1,159 | 6041125-1 | 30,009,030 | comm/PMC006xxxxxx/PMC6041125.xml | Tracheobronchial involvement of mantle cell lymphoma | Our patient is a 65-year-old male who presented with a two-year history of productive cough and progressive dyspnoea. He had no prior medical history and was a smoker of 40 pack-years. On physical exam, he had mild fine bibasilar crackles on lung exam without wheezing. His laboratory values were all normal, with the exception of his arterial blood gas analysis, which showed a lower PaO2 of 74.4 mmHg on room air. His serum soluble interleukin-2 receptor (sIL-2R) level was high (1655 U/mL). Pulmonary function testing revealed severe irreversible obstructive ventilatory dysfunction with normal diffusing capacity of the lung (Fig. ). A computed tomography (CT) scan of the chest revealed irregular wall thickening of the trachea and bilateral bronchi (Fig. ). In addition, there was bilateral bronchiectasis, and mediastinal and both hilar lymphadenopathy were noted. Flexible bronchoscopy demonstrated a diffuse irregular surface of the tracheal and bilateral bronchial mucosa and multiple macroscopic submucosal nodules involving the trachea, the distal trachea above the major carina, and throughout the left and right main bronchi (Fig. ). Endobronchial biopsy of the mucosa on the major carina showed a population of small atypical lymphocytes with scant cytoplasm and hyperchromatic nuclei of irregular nuclear contours. These atypical lymphocytes were CD20 positive B-cells co-expressing CD5, blc-2, and Cyclin D1, leading to the diagnosis of MCL (Fig. ). Further positron emission tomography (PET)-CT imaging showed the persistence of FDG-avid lymphadenopathy within the chest (SUVmax 4.2) and FDG-avid wall thickening of the trachea and bilateral main bronchi (SUVmax 2.8), as well as FDG-avid nasopharyngeal soft tissue thickening (SUVmax 6.8). Biopsies of the nasopharyngeal mucosa further demonstrated B-cells with the same immunophenotype as the endobronchial biopsy, and 11;14 translocation was confirmed by fluorescent in situ hybridization (FISH) analysis using a CCND1/IGH probe. Bone marrow biopsy showed an increased lymphocyte percentage of 23%, with 1% of immature lymphocytes, and a positive immunoglobulin rearrangement (FR1-JH(+), FR2-JH(+), FR3-JH(+), Vk-Jk(+), Vk-Kde + intronKde(−)). Bone marrow flow cytometry demonstrated a surface kappa clonal B-cell population with CD5, CD20, and FMC-7 expression. Taken together, these findings were consistent with an MCL involving the tracheobronchial wall, nasopharyngeal soft tissue, and bone marrow. | [[65.0, 'year']] | M | {'22532909': 1, '27536549': 1, '19075279': 1, '27469362': 1, '18175356': 1, '21658653': 1, '17409613': 1, '22576406': 1, '12752899': 1, '25724714': 1, '20940415': 1, '30009030': 2} | {} |
1,160 | 6041220-1 | 29,995,255 | comm/PMC006xxxxxx/PMC6041220.xml | A rare mechanism of delayed splenic rupture following the nonoperative management of blunt splenic injury in a child | A 9-year-old boy (height: 133 cm, weight: 25.8 kg) with severe abdominal pain was transferred to our hospital. Twenty-one hours before admission, he had been kicked in the region of his left lateral abdomen. On admission, the patient’s face was pale, and his Glasgow coma scale was recorded at 13/15 (E: 3, V: 4, M: 6). A physical examination revealed severe tenderness on the left lateral abdomen with moderate abdominal distension. His vital signs were as follows: blood pressure 80/50 mmHg, heart rate 110 bpm, and a respiratory rate of 30/min, respectively.\nContrast-enhanced abdominal computed tomography revealed a severe intraparenchymal hematoma and multiple lacerations of the spleen with a large amount of hemoperitoneum (Fig. a, b). The splenic hilar vessels were not injured (Fig. c), and there were no signs of active bleeding. His injury was diagnosed as grade III on the American Association for the Surgery of Trauma (AAST) splenic injury scale. The patient’s laboratory data showed his red blood cell count 251 × 104/ml, hemoglobin (Hb) 9.1 g/dl, hematocrit (Ht) 27.1%, and platelets 33.6 × 104/μl. All of his other laboratory data were within normal limits. After fluid resuscitation, his blood Hb and Ht levels fell to 7.6 g/dl and 22.3%, respectively. He required a transfusion of 1000 ml red blood cells in order to maintain his blood pressure. His vital signs became stable, and he regained clear consciousness.\nWe treated him by NOM in our intensive care unit. His abdominal pain decreased gradually while resting in bed. His hemodynamic conditions were stable, and further blood transfusion was not necessary. At 70 h after the injury, he started drinking water. Two hours after drinking water (72 h post-injury), he discharged feces. After defecation, he suddenly started complaining of severe abdominal pain and left shoulder pain. His blood pressure dropped to 70/35 mmHg, and he started to lose consciousness. Abdominal ultrasonography (US) revealed increased ascites. Fluid resuscitation and blood transfusion were performed. His symptoms and abdominal US findings suggested that splenic re-bleeding had caused delayed splenic rupture to occur. An emergency splenectomy was performed. The resected spleen was enlarged with a large parenchymal hematoma. The posterior-lateral side of the splenic capsule was torn (Fig. ). The splenocolic ligament was resected during the operation. There were no anatomical anomalies between the ligament and the spleen.\nThe post-operative course was uneventful, and the patient was discharged 9 days after splenectomy. | [[9.0, 'year']] | M | {'19639310': 1, '24139835': 1, '34741862': 2, '29079311': 1, '21616255': 1, '19524710': 1, '28188036': 1, '20485153': 1, '19741402': 1, '28184357': 2, '19342266': 1, '10693659': 1, '28611565': 2, '29995255': 2} | {'5292018-1': 1, '5465718-1': 1, '8581505-1': 1} |
1,161 | 6041221-1 | 29,998,373 | comm/PMC006xxxxxx/PMC6041221.xml | Icterus and abdominal pain: an unexpected, rare sonographic finding in a Peruvian Emergency Department | A 27-year-old female from the Peruvian Highlands presented to the emergency department in Lima with intermittent diffuse abdominal pain and jaundice. There was no reported history of hepatitis or change in stool color. Physical examination revealed scleral icterus and mild tenderness at the right upper quadrant.\nGiven the patient’s region of origin and clinical picture, the initial working diagnosis was a hydatid cyst. A point-of-care ultrasound was performed, which revealed a large multi-locular cyst, with multiple surrounding anechoic structures (Fig. , Additional file : Video 1). These findings were not typical for hydatid disease, and ultimately the surgical pathology confirmed a choledochal cyst. | [[27.0, 'year']] | F | {'18258119': 1, '25442379': 1, '12606104': 1, '7220891': 1, '25923827': 1, '29998373': 2} | {} |
1,162 | 6041387-1 | 30,026,742 | comm/PMC006xxxxxx/PMC6041387.xml | Combination of Cytokine-Induced Killer Cells and Programmed Cell Death-1 Blockade Works Synergistically to Enhance Therapeutic Efficacy in Metastatic Renal Cell Carcinoma and Non-Small Cell Lung Cancer | An 80-year-old man (Patient 1) received a diagnosis of clear cell carcinoma of the right kidney after partial nephrectomy. One year later, computed tomography showed a right lumbar mass, and surgical specimens revealed clear cell carcinoma. The patient subsequently underwent afatinib treatment. However, therapy was discontinued as a result of intolerable adverse effects. Eighteen months later, PET-CT showed multiple metastases, including to the right pleural tubercle, thoracic vertebra, lumbar vertebra, left ilium, and humerus. In consultation with the radiation therapist, he received stereotactic radiotherapy to the right lung; however, a new left upper gingival soft tissue mass was found during the radiotherapy, and tumor metastasis was confirmed by biopsy. He was then treated with pembrolizumab combined with CIK cell transfer. CIK cells were prepared as described previously (). Briefly, peripheral blood mononuclear cells (PBMC) were separated and cultured under sterile conditions in 1640 medium containing anti-CD3 monoclonal antibody, interferon γ, interleukin-2, and RetroNectin. After culturing the cells for 10–14 days, a target dose of about 6 × 109 CIK cells with over 95% viability was obtained and tested for biological contaminants. Cells were then prepared in sodium chloride solution containing 2% albumin before transfusion. He achieved a complete response following treatment with four cycles of pembrolizumab combined with eight cycles of CIK cell transfer (Table S1 in Supplementary Material) and continues to be in remission on day 537 of the first dose of pembrolizumab treatment (Figure A). The patient had gingivitis after first cycle of pembrolizumab and pneumonia after second cycle of pembrolizumab, for which he received systematic antibiotic treatment. He did not receive glucocorticoids or other immunomodulating agents during his treatment with pembrolizumab and CIK cells.\nA 63-year-old man (Patient 2) received a diagnosis of squamous cell carcinoma after biopsy of a right lower lobe lung mass. CT scans showed that this patient had developed multiple metastases, including mediastinal, right hilar, and anterior superior phrenic lymph nodes, and also to the sternum. His disease progressed following first-line platinum-based doublet chemotherapy and second-line S-1, a second-generation oral fluoropyrimidine composed of tegafur, gimeracil, and oteracil (). He was then treated with pembrolizumab combined with CIK cell transfer as third-line therapy. Follow-up imaging showed a near-complete response after treatment with eight cycles of pembrolizumab in combination with seven cycles of CIK cell transfer (Table S1 in Supplementary Material), and the patient continues to be in remission 185 days posttreatment (Figure B). No adverse events occurred during treatment in this patient.\nGiven that PD-1 pathway blockade induces a clinical response in subsets of cancer patients, defining biomarkers that predict therapeutic effects and adverse events is crucial. Recent studies have suggested an association between clinical responses and several promising biomarkers, including PD-1, PD-L1, CD3, tumor mutation burden (TMB), deficient DNA mismatch repair status (dMMR), and insertions and deletions (indels) (). To examine the relationship between PD-1, PD-L1, or CD3 expression in the tumors and antitumor responses elicited by anti-PD-1 antibodies combined with CIK cell transfer, we evaluated pretreatment tumor biopsies from both patients using immunohistochemistry and found that the tumor biopsy from Patient 1 exhibited moderate CD3+ T cell infiltration, but no PD-1 or PD-L1 expression (Figure A). The tumor cells from Patient 2 strongly expressed PD-L1, and there was extensive tumor infiltration by CD3+ T cells; however, no PD-1 staining was seen (Figure B). It has been reported that frameshift indels are more prevalent in renal cell carcinoma compared with other tumor types and are associated with enhanced T-cell activation (). Across all tumor types, clear cell renal carcinoma was found to have the highest proportion of coding indels, 0.12, a 2.4-fold increase when compared with the pan-cancer average, 0.05 (). We analyzed the whole-exome sequences of pretreatment tumors and patient-matched blood samples. We observed an indel proportion value of 0.08 and an indel count of 4 in patient 1, while these values were 0.04 and 5, respectively, in patient 2. We also found that tumors from both patients harbored non-synonymous single nucleotide variant (nsSNV) mutations. The TMB in patient 2 was higher than that in patient 1 (2.4 versus 0.8/Mb, respectively); however, no mutations in MMR-associated genes occurred in either patient (Figure ). Based on these data, we hypothesize that nsSNVs, along with higher indel mutations, in patient 1 with renal cell carcinoma and nsSNVs along with higher TMB in patient 2 with lung cancer correlate with tumor-infiltrating CD3+ lymphocyte density. Therefore, CD3+ T cell infiltration in baseline tumor biopsies may be a potential biomarker to predict which patients will benefit most from PD-1 blockade in combination with CIK cell therapy, although this will need to be confirmed in larger numbers of patients. | [[80.0, 'year']] | M | {'26787823': 1, '15070686': 1, '28653677': 1, '22581306': 1, '34123792': 2, '33150733': 1, '28694034': 1, '34540187': 1, '23193418': 1, '24316555': 1, '8653704': 1, '26205340': 1, '27433478': 1, '7694868': 1, '34984004': 2, '26842696': 1, '26716894': 1, '18565854': 1, '32882824': 1, '30026742': 2} | {'8702989-1': 1, '8702989-2': 1, '8194346-1': 1} |
1,163 | 6041406-1 | 30,027,093 | comm/PMC006xxxxxx/PMC6041406.xml | Presumed Caudal Cerebellar Artery Infarction in Three Cats: Neurological Signs, MRI Findings, and Outcome | An 11-year-old male neutered domestic shorthair cat was presented for investigation of acute vestibular signs. The owner reported a left sided head tilt and loss of balance observed a few hours prior to presentation. Physical examination was unremarkable while a left-sided head tilt, broad-based pelvic limb stance, and a mild intermittent intention head tremor was observed on neurological examination. Vestibular ataxia with falling to the left, decreased postural reactions in all four limbs (paw positioning, hopping, and extensor postural trust) and bilateral positional vertical nystagmus were also recorded. A left central vestibular syndrome was suspected.\nHematology, biochemistry, fasting bile acids, thyroxine levels, and urinalysis were unremarkable. Feline leukemia virus antigen and feline immunodeficiency virus antibody tests were negative (SNAP FIV/FeLV Combo test; IDEXX, Maine). Systolic blood pressure measured upon admission using Doppler sphygmomanometry was considered within normal limits for a hospital environment (mean reading = 151 mmHg).\nAn MRI study of the brain (0.4T Hitachi Aperto Lucent MR scanner, Tokyo) was performed 24 h after the onset of neurological signs. A single, sharply-demarcated focal cerebellar lesion was identified in the caudal third of the right cerebellar hemisphere and lateral medulla oblongata (Figure ). The lesion was isotense on T1-weighted images, and hyperintense on T2-weighted images (Figures ) and T2 FLAIR images. Diffusion-weighted images (DWI) showed a well-demarcated area of uniform hyperintensity with a corresponding area of reduced diffusion (hypointensity) on an apparent diffusion coefficient (ADC) map (Figures ). No mass effect was seen. There was minimal enhancement with gadobutrol (Gadovist, Bayer, UK) administration (0.1 mmol/kg, IV). Based on the MRI appearance and presence of restricted diffusion, history, and neurological localization, a presumptive diagnosis of a non-hemorrhagic right CCA infarction, resulting in paradoxical central cerebellar syndrome, was made.\nThe cat was hospitalized for monitoring and supportive care. Oral antioxidant/multivitamin therapy (Aktivait, Vetplus, UK) was initiated as supportive trial therapy. The cat's neurological status progressively improved over 4 days and he was discharged with mild head tilt. No neurological signs were present at 1 month re-examination and antioxidant/multivitamin therapy was discontinued. | [[11.0, 'year']] | M | {'17317258': 1, '15693551': 1, '16594588': 1, '22122807': 1, '19891725': 1, '8418555': 1, '23206661': 1, '21311074': 1, '16231718': 1, '30027093': 2} | {'6041406-2': 2, '6041406-3': 2} |
1,164 | 6041406-2 | 30,027,093 | comm/PMC006xxxxxx/PMC6041406.xml | Presumed Caudal Cerebellar Artery Infarction in Three Cats: Neurological Signs, MRI Findings, and Outcome | A 4-year-old female spayed domestic short hair cat was referred with a 24 h history of acute vestibular signs. Physical examination was unremarkable. Neurological examination revealed head tremors, left-sided head tilt and generalized vestibular ataxia with a tendency to fall to the left. Mild postural reactions deficits (in hopping and extensor postural trust tests) in the left pelvic limb and positional horizontal nystagmus on left eye were observed. Neuroanatomical localization suggested a left-sided central vestibular lesion, most likely central-cerebellar localization.\nHematology and serum biochemistry revealed hypercholesterolemia (5.6 mmol/l; RI 1.9–3.9) and a marked increased creatinine kinase (2405 IU/L; RI 0–152). Thyroid testing was consistent with non-thyroidal illness (thyroxine < 12.9 nmol/l [RI 15–50]; thyroid-stimulating hormone (TSH) 0.07 ng/ml [RI 0.0–0.32]). Toxoplasma gondii serology in blood (IgM and IgG) was negative.\nBrain MRI showed a focal well-defined hyperintense lesion on T2-weighted (Figures ) and T2 FLAIR sequences in the caudal left cerebellar hemisphere. Diffusion was restricted on diffusion-weighted sequences and corresponding ADC map (Figures ). A thin rim of contrast enhancement was seen around the margin of a cerebellar folium. No mass effect was observed. Findings were compatible with a non-hemorrhagic left CCA infarct. Spinal MRI (performed due to the left pelvic limb proprioceptive deficits) revealed facet hypertrophy causing dorsal impingement at T13-L1. Cisternal cerebrospinal fluid (CSF) analysis revealed mild neutrophilic pleocytosis (nucleated cell count 12cells/ul [RI 0–5]; total proteins were normal). Echocardiography, thoracic radiography, and abdominal ultrasound were unremarkable. The cat was discharged 48 h subsequently with mild generalized vestibular ataxia only. On telephonic follow-up 3 months later the cat's vestibular signs had completely resolved. | [[4.0, 'year']] | F | {'17317258': 1, '15693551': 1, '16594588': 1, '22122807': 1, '19891725': 1, '8418555': 1, '23206661': 1, '21311074': 1, '16231718': 1, '30027093': 2} | {'6041406-1': 2, '6041406-3': 2} |
1,165 | 6041406-3 | 30,027,093 | comm/PMC006xxxxxx/PMC6041406.xml | Presumed Caudal Cerebellar Artery Infarction in Three Cats: Neurological Signs, MRI Findings, and Outcome | An 8-year-old male neutered Persian cat was presented for neurological evaluation of acute onset vestibular ataxia and abnormal mentation. Physical examination revealed a grade II/VI systolic heart murmur but was otherwise unremarkable. Neurological examination revealed generalized vestibular ataxia with marked loss of balance, generalized postural reactions deficits (hopping and paw positioning tests), absent oculocephalic reflex, and ventrolateral positional strabismus in both eyes. The neuroanatomical localization was compatible with a central vestibular lesion.\nHematology and serum biochemistry were unremarkable. On MRI, a poorly defined area (8 × 13 × 6 mm) of homogeneously increased T2 and T2 FLAIR signal hyperintensity compared to normal cerebellar gray matter, affecting the caudal aspect of the cerebellar vermis and right cerebellar hemisphere was observed (Figures ). This lesion had a low signal in T1-weighted images, and there was no abnormal contrast enhancement. There was no signal void observed on T2* weighted images. An acute non-hemorrhagic territorial cerebellar infarct affecting the perfusion area of the right CCA was suspected. Cisternal CSF analysis revealed a normal cell count and population but increased total protein at 0.68 g/l (RI < 0.25). RT-PCR for infectious diseases (Toxoplasma gondii, feline coronavirus and feline herpesvirus) in CSF was negative. Oral antioxidant/multivitamin therapy (Aktivait, Vetplus, UK) was instituted for 1 month. The cat's neurological status progressively improved over 4 days and he was discharged with markedly improved generalized ataxia and a mild head tilt. At 1 month follow-up no neurological signs were observed and therapy was discontinued. | [[8.0, 'year']] | M | {'17317258': 1, '15693551': 1, '16594588': 1, '22122807': 1, '19891725': 1, '8418555': 1, '23206661': 1, '21311074': 1, '16231718': 1, '30027093': 2} | {'6041406-1': 2, '6041406-2': 2} |
1,166 | 6041423-1 | 30,009,244 | comm/PMC006xxxxxx/PMC6041423.xml | Rapid diagnosis of mixed phenotype acute leukemia after identifying a blood histogram abnormality | A 38-year-old woman suffering from back, right arm, and ankle joint pain for several months, had consulted several orthopedic, neurological and pain clinics. She was indicated to have anemia and advised to visit an internal medicine clinic. Before visiting another clinic, she visited the emergency department of our hospital at 11:30 P.M., Friday, because of severe back pain. Upon admission to our hospital, her white blood cell (WBC) count was slightly high (11,700/µL) and low numbers of red blood cells (RBCs) (2.21 × 106/µL) and platelets (PLTs) (42,000/µL) were observed. Furthermore, high lactate dehydrogenase (2300 U/L), P-FDP (33.8 µg/mL), and d-dimer (14.22 µg/mL) were observed (). The histogram of an automated hematology analyzer (DxH 500; Beckman Coulter, CA) was abnormal. Although the RBC histogram was normal (A), the PLT histogram showed an abnormally shaped peak at around 20–30 fL (B), suggesting the presence of giant PLTs or PLT aggregation. The WBC histogram showed abnormal elevation at 35 fL (C, arrow) and around 100 fL (C, arrowhead), suggesting giant PLTs and abnormal cells including nucleated RBCs. A peripheral blood smear was prepared, and morphology was examined. As a result, blasts (4%) including many orthochromatic erythroblasts (48/100 WBCs) were observed (, ). Acute leukemia was suspected and the patient was transferred the next day to Sendai Medical Center capable of acute leukemia treatment. Bone marrow was examined, and 99% of cells were blasts () positive for CD10, CD19, CD24, CyCD22, CyCD79a, Cy µ, and myeloperoxidase as shown by flow cytometry (). The patient was diagnosed with MPAL, immediately treated by an acute lymphoid leukemia regimen (JALSG ALL202-O), and achieved remission. Genetic analysis revealed that there were no major (i.e. major, minor-bcr-abl, PML-RARa, AML1-MTG8, DEK-CAN, NUP98-HOXA9, ETV6-AML1, E2A-PBX1, SIL/TAL1, or MLL gene rearrangements or FLT3-ITD) abnormalities. However, G-banding analysis of bone marrow samples revealed that the patient had complex chromosomal aberrations (among 20 cells analyzed, three cells were A: 47, XX, +X, add(3)(q27), − 4, del(6)(q?), and +mar1, 13 cells were B: 47, idem, add(3)(q11.2), add(7)(q22), and del(7)(q?), and four cells were a type A-derived clone). Thereafter, the patient received an allogenic bone marrow transplantation. Until then, her remission was maintained and the patient is currently receiving immunosuppressive drugs as an outpatient. | [[38.0, 'year']] | F | {'26524955': 1, '27069254': 1, '29072953': 1, '8249908': 1, '29207767': 1, '31929708': 2, '28099272': 1, '30009244': 2} | {'6943870-1': 1} |
1,167 | 6041511-1 | 30,034,456 | comm/PMC006xxxxxx/PMC6041511.xml | Huge Fungating Benign Phyllodes Tumor of Breast | A 25 years old unmarried female developed a painless, firm, round, mobile swelling in right breast since one year which was growing in size progressively. It was incised by a quack under local anesthesia three month back, which started growing with a great speed since that period.\nIt was hanging from the chest, painless, fungating ( and ), discharging offensive fluid. Its size was 30x25cms. It was hindering in performing her daily routine jobs. She was grossly anemic and clinically there were no glands in axilla. X-ray chest was clear; Hb was 6g/dl. There was no history of any breast ailment in the family. Preoperative open biopsy was taken which revealed benign phyllodes tumor on histopathology. The tumor had a loose adhesion with the pectoral major. | [[25.0, 'year']] | F | {'24369215': 1, '26768026': 1, '21251125': 1, '16860620': 1, '12689668': 1, '32913869': 2, '22610081': 1, '10515335': 1, '1655234': 1, '20077881': 1, '6283677': 1, '30034456': 2} | {'7457108-1': 1} |
1,168 | 6041545-1 | 30,034,455 | comm/PMC006xxxxxx/PMC6041545.xml | Pyogenic liver abscess after laparoscopic sleeve gastrectomy | A thirty-seven-year-old female patient presented with abdominal pain and fever. Two weeks ago, she underwent LSG in a state hospital. Physical examination revealed minimal sensitivity by palpation localized to the left upper quadrant. Her body temperature was 39°C. White blood cell count was 11,600mm3/dL, and the C-reactive protein (CRP) value was 166. The chest X-ray showed blunting of the left costa-phrenic angle with minimal left sided pleural effusion (). An oral contrast given esophago-dueodenography was performed to rule out a staple line leak under scope which did not show extra-luminal contrast extravasations (). Abdominal computerized tomography (CT) revealed a unilocular pyogenic liver abscess measured 12x7cm in diameter which was localized to the left lobe (). A history of LSG from two weeks ago and a left sided pleural effusion on chest X-Ray were suggestive of pyogenic liver abscess secondary to staple line leak. The patient was hospitalized and an Ultrasound guided percutaneous drainage catheter was placed. Intravenous antibiotic therapy (piperacillin-tazobactam3x4.5gr/day) was started immediately. The course of the patient was uneventful. The liver abscess resolved and she was discharged on the 22th day with oral antibiotic therapy. | [[37.0, 'year']] | F | {'23993246': 1, '27301373': 1, '28271377': 1, '28261497': 1, '25092167': 1, '25835983': 1, '22365110': 1, '28008466': 1, '25691579': 1, '30034455': 2} | {} |
1,169 | 6041548-1 | 30,034,454 | comm/PMC006xxxxxx/PMC6041548.xml | Sealed Duodenal Perforation by nail ingestion in a Child | A 10-year-old boy presented to Pediatric Surgery Department, Mayo Hospital in October 2017 with history of accidental ingestion of metallic nail and epigastric pain for 20 days. Parents consulted a private hospital and was advised observation. The nail did not pass spontaneously and patient started having colicky upper abdominal pain and was brought to our hospital.\nThere was no previous history of foreign body ingestion and patient’s psychological evaluation was normal. On examination, there was tenderness in epigastric region and right hypochondrium. Rest of the abdominal examination was normal. Plain X-ray abdomen showed a nail in right upper quadrant and its position had not changed from first X-ray (). Laboratory reports was unremarkable.\nAfter admission and informed consent, emergency exploration was performed. Peroperatively, a metallic nail was found impacted in 2nd part of duodenum. The head was embedded in the secondpart causing a small perforation in the duodenum which was sealed by omentum (). No spillage was noted in the peritoneal cavity. Through same perforation nail was delivered (). Duodenal perforation was repaired primarily after debridement of edges and drain was placed near repair.\nPostoperative recovery was smooth and uneventful. Drain and nasogastric tube were removed on the fourth postoperative day and oral liquids were started on day 5. Patient was discharged on day 9. On subsequent follow up, he was symptom free and okay. Permission for publication was taken from father and ethical approval taken from institute. | [[10.0, 'year']] | M | {'26862844': 1, '23293561': 1, '18666336': 1, '16479337': 1, '16140701': 1, '22567210': 1, '25408769': 1, '33679957': 2, '16050452': 1, '24661696': 1, '32855789': 2, '6696536': 1, '30034454': 2} | {'7444311-1': 1, '7931289-1': 1} |
1,170 | 6042222-1 | 29,363,013 | comm/PMC006xxxxxx/PMC6042222.xml | The tibial bayonet method of wound closure | Patient was a 40-year-old male who sustained bilateral grade 3b tibia fractures (mechanism of injury: bark stripping machine at a sawmill) (Fig. ). He had no other injuries and no co-morbidities.\nFollowing initial surgical management, a temporary fixator was applied along with appropriate debridement (Hoffmann Express Stryker® comprising of four half pins and a standard two-bar configuration) (Fig. ). The soft tissue defect was equivalent to a Gustilo–Anderson IIIB, and we were unable to have a flap applied [].\nSimilar to the benchmark method of ‘fix and flap’ which usually includes two scheduled surgeries, the bayonet method too required two scheduled surgeries, however with the advantage of circumventing the need to be dependent on plastic surgeon’s availability: the first surgery for frame application, deformation and closure and the second for frame removal. In this case, there was also one unscheduled theatre visit in order to extend the frame across the ankle for the equinus correction.\nAt the 48 h, first definitive surgery a tibial bayonet procedure was performed, a technique that has been established at the Ilizarov Institute in Kurgan (AM. Cherkashin and ML. Samchukov, personal communication). Sub-muscular elevation and creation of a soft tissue envelope (no periosteal elevation or stripping) were performed (Fig. ). At this sitting, a standard two-ring/four-half-pin hexapod frame was applied (OrthoFix TL-Hex®).\nThe wound was a 270-degree circumferential skin wound, from postero-lateral to postero-medial aspect of the middle third of the leg. The underlying muscle bellies were intact and did not require repair. Sensation and motor function were present distally (the latter was decreased). The struts were kept loose in order to allow bayonetting. The distal fragment was inserted into the soft tissue pocket. There was no interposing tissue, and the bone fragments were overlapped until the skin edges could be opposed in a tension-free manner (Fig. ). The struts were locked in place, and the wound was closed primarily.\nA latent period of 6 weeks followed with the foot elevated in order to allow the skin to heal and for the oedema to settle. Initial lengthening was achieved by increasing the length of all struts sequentially and equally. The distraction rate allowed a lengthening of 1–2 mm/day. Once length was restored, the TL-Hex® software was used based on orthogonal radiographs for software referencing to allow realignment of the two fragments. Thus, the translation was corrected and the subsequent compression at the fracture site (Fig. ). There was no formal docking procedure performed. The patient was assisted by the physiotherapists and mobilized with crutches and a walker frame within the first week falling surgery to allow full weight bearing with the aim to encourage functional mobilization. The accuracy of the correction was verified with serial radiographs at one monthly intervals. | [[40.0, 'year']] | M | {'9602796': 1, '16497997': 1, '22019355': 1, '19088559': 1, '3558791': 1, '11764356': 1, '25550005': 1, '16825969': 1, '11940635': 1, '11041582': 1, '12851785': 1, '15509933': 1, '21037353': 1, '15330028': 1, '16944234': 1, '10797199': 1, '23328433': 1, '18349775': 1, '15454809': 1, '17259422': 1, '773941': 1, '29363013': 2} | {} |
1,171 | 6042236-1 | 29,996,795 | comm/PMC006xxxxxx/PMC6042236.xml | Pregnancy, child bearing and prevention of giving birth to the affected children in patients with primary immunodeficiency disease; a case-series | A 23 year-old patient with Chronic Granulomatous Disease (CGD) gave birth to a normal healthy male. During pregnancy IFNγ was discontinued and through control visits acute phase reactants were evaluated with no increase. Diagnosis of CGD was established at 8 years old due to recurrent pneumonia, empyema, oral ulcers, skin abscess and cervical mycobacterial lymphadenitis. Genetic analysis revealed p47-phox gene mutation. She was born out of consanguineous marriage and her sister had been diagnosed with CGD as well. Four weeks after delivery she went to emergency department complaining of low back pain irradiated to left gluteal, sacral and lumbosacral area. Laboratory workup showed leukocytosis (WBC = 11,700 cell/mcL) and increased levels of liver enzymes (SGPT = 131 U/L, normal:7–55, SGOT = 56 U/L normal:8–45, ALP = 1588 U/L, normal:45–115). Abdominopelvic US revealed granulomatous lesions in uterus and liver. Contrast lumbosacral MRI showed enhancement in L5 and S1 suggesting infection or inflammation. Pathology Department reported granulomatous lesion and PCR positive for Aspergillus fumigatus. She was diagnosed with fungal osteomyelitis and went under treatment with IFNγ and Voriconazole for 12 weeks with good response. In follow-up tests liver enzymes decreased (SGPT = 54 U/L, SGOT = 24 U/L, ALP = 423 U/L) and granulomatous lesions disappeared. | [[23.0, 'year']] | M | {'21958324': 1, '11332654': 1, '33134418': 1, '17152416': 1, '17566949': 1, '12099663': 1, '12592303': 1, '24378282': 1, '11600752': 1, '34580181': 1, '26413359': 2, '8781705': 1, '26856578': 1, '14732328': 1, '22538377': 1, '19220771': 1, '19268935': 1, '19639457': 1, '26097846': 1, '24976826': 1, '26380498': 1, '8297849': 1, '26125817': 1, '16449156': 1, '16595236': 1, '27836056': 1, '12149412': 1, '17298726': 1, '22527898': 1, '25204341': 1, '8014693': 1, '2117627': 1, '14663840': 1, '15512427': 1, '29996795': 2} | {'6042236-2': 2, '6042236-3': 2, '6042236-4': 2, '4564600-1': 1, '4564600-2': 1} |
1,172 | 6042236-2 | 29,996,795 | comm/PMC006xxxxxx/PMC6042236.xml | Pregnancy, child bearing and prevention of giving birth to the affected children in patients with primary immunodeficiency disease; a case-series | A 23 year-old patient with CVID whose diagnosis was done 7 years before pregnancy when she was 16 years old with clinical history of lower and upper recurrent respiratory tract infections and autoimmune thrombocytopenia. Genome analysis showed heterozygous missense variant in exon 3 of CDX1 gene. Blood tests at 5 weeks of gestation showed IgM of 0.8 g/L (0.3–2.5), a total IgG of 5.6 g/L (normal 6.0–16.0), and IgA of 0.7 g/L (normal 0.8–5). During pregnancy she received 500 mg/kg of IVIg every 3 weeks. In the 2nd trimester of pregnancy she presented idiopathic thrombocytopenia, which was managed with higher doses of IVIg (800 mg/kg) with no clinical manifestation. Platelet count dropped to 16x103platelets/mm3 abruptly before her cesarean section and need hindered urgent platelet infusion before delivery. Ultimately she gave birth to a full term, normal healthy child and few days after delivery platelet count reached normal level (Fig. ). | [[23.0, 'year']] | F | {'21958324': 1, '11332654': 1, '33134418': 1, '17152416': 1, '17566949': 1, '12099663': 1, '12592303': 1, '24378282': 1, '11600752': 1, '34580181': 1, '26413359': 2, '8781705': 1, '26856578': 1, '14732328': 1, '22538377': 1, '19220771': 1, '19268935': 1, '19639457': 1, '26097846': 1, '24976826': 1, '26380498': 1, '8297849': 1, '26125817': 1, '16449156': 1, '16595236': 1, '27836056': 1, '12149412': 1, '17298726': 1, '22527898': 1, '25204341': 1, '8014693': 1, '2117627': 1, '14663840': 1, '15512427': 1, '29996795': 2} | {'6042236-1': 2, '6042236-3': 2, '6042236-4': 2, '4564600-1': 1, '4564600-2': 1} |
1,173 | 6042236-3 | 29,996,795 | comm/PMC006xxxxxx/PMC6042236.xml | Pregnancy, child bearing and prevention of giving birth to the affected children in patients with primary immunodeficiency disease; a case-series | A 29 year-old woman, a case of CVID who was unrecognized till age 22. She had autoimmune hepatitis (AST=) with false negative markers due to immunodeficiency. Biopsy from duodenum showed celiac-like disease. She was on immunosuppressant drugs before her definite diagnosis. Her genetic result revealed NF-κB2 frameshift mutation. She had history of recurrent cold, hospitalization for pneumocystis jiroveci, atopy and allergy. Immunoglobulin levels were as follows; IgG = 7.05 g/L (normal: 6–16), IgM = 0.3 g/L (normal: 0.5–2.5), IgA = 0.5 g/L (normal: 0.8–4), IgE = 2 g/L (normal < 0.002). Flowcytometry result demonstrated CD4 = 59%, CD8 = 14%, CD16 + 56 = 14%. While she was started on IVIg therapy she showed allergic reaction to the product. IVIg was discontinued and the patient was reluctant to take it anymore. She was returned to use immunosuppressive drugs including cellcept and prednisone. She became pregnant and all drugs were discontinued. Strangely her liver enzymes became normal (AST = 20, ALT = 32). Eclampsia developed at 24w of gestation, handled under supervision of her obstetrician. She received heparin for 1 month as she had tachypnea and dyspnea and was suspicious to PTE at 30w. Her Immunoglobulin level at 34w of gestation was IgG = 5.1 g/L and IgM = 0.42 g/L and IgA = 0.49 g/L. She has born her child at 38w. The baby is well and we are waiting for her immunoglobulin level and other laboratory results to decide on vaccination. | [[29.0, 'year']] | F | {'21958324': 1, '11332654': 1, '33134418': 1, '17152416': 1, '17566949': 1, '12099663': 1, '12592303': 1, '24378282': 1, '11600752': 1, '34580181': 1, '26413359': 2, '8781705': 1, '26856578': 1, '14732328': 1, '22538377': 1, '19220771': 1, '19268935': 1, '19639457': 1, '26097846': 1, '24976826': 1, '26380498': 1, '8297849': 1, '26125817': 1, '16449156': 1, '16595236': 1, '27836056': 1, '12149412': 1, '17298726': 1, '22527898': 1, '25204341': 1, '8014693': 1, '2117627': 1, '14663840': 1, '15512427': 1, '29996795': 2} | {'6042236-1': 2, '6042236-2': 2, '6042236-4': 2, '4564600-1': 1, '4564600-2': 1} |
1,174 | 6042236-4 | 29,996,795 | comm/PMC006xxxxxx/PMC6042236.xml | Pregnancy, child bearing and prevention of giving birth to the affected children in patients with primary immunodeficiency disease; a case-series | A 26 year-old woman is recently known for CVID. She was referred to our clinic for extensive bilateral bronchiectasis. Her laboratory results showed hypogammaglobulinemia (IgM = 0.0.14 g/L normal: 0.4–2.6, IgG = 4.1 g/L normal: 6.5–18, IgA = 0.05 g/L normal: 0.7–3.6). She is getting IVIg now but she became pregnant 2 years ago while her diagnosis was not made and no immunotherapy was given. She had severe dyspnea and suffered from recurrent pneumonia during her pregnancy that was treated with antibiotic. She gave birth to a healthy boy baby. We do not have the immunoglobulin level of the child at birth but according to the mother’s statement he had no serious infection in his first 6 months of life. | [[26.0, 'year']] | F | {'21958324': 1, '11332654': 1, '33134418': 1, '17152416': 1, '17566949': 1, '12099663': 1, '12592303': 1, '24378282': 1, '11600752': 1, '34580181': 1, '26413359': 2, '8781705': 1, '26856578': 1, '14732328': 1, '22538377': 1, '19220771': 1, '19268935': 1, '19639457': 1, '26097846': 1, '24976826': 1, '26380498': 1, '8297849': 1, '26125817': 1, '16449156': 1, '16595236': 1, '27836056': 1, '12149412': 1, '17298726': 1, '22527898': 1, '25204341': 1, '8014693': 1, '2117627': 1, '14663840': 1, '15512427': 1, '29996795': 2} | {'6042236-1': 2, '6042236-2': 2, '6042236-3': 2, '4564600-1': 1, '4564600-2': 1} |
1,175 | 6042257-1 | 29,996,926 | comm/PMC006xxxxxx/PMC6042257.xml | Delayed diagnosis of spontaneous bladder rupture: a rare case report | The manuscript was approved by the Ethics Committees of Guangdong Women and Children Hosptial and the participant provided written informed consent.\nThe patient was a 23-year old pregnant woman under healthy condition, and vaginally delivered one healthy girl with term birth as the first pregnancy. Delivery was smooth, first stage of labor was 15 h and second stage was 2 h. The birth weight was 3.6 kg. This newborn was in good health. The slight abdominal bloat was complained after delivery, but without special treatment. The abdominal pain was exacerbated On the fifth day after delivery accompanied with vomiting, shivering, and high fever. The highest body temperature was 39 °C. Oliguria and edema on the bilateral lower extremities were reported. The large amount of ascites was revealed by ultrasound examination; and the venous blood biochemical assays indicated the elevated level of serum creatinine (427 umol/L), urea nitrogen (26 mmol/L) and potassium (6.6 mmol/L). The diagnosis was considered to be postpartum acute renal failure (ARF) and peritonitis The patient was treated with intravenous antibiotics, hemodialysis, peritoneal catheter drainage (2000 mL reddish ascites drained) and indwelling bladder catheter. The abdominal pain was significantly alleviated on the second day and the ascites disappeared with the serum biochemical restored normal. The patient was transferred to our hospital on the sixth day postpartum. Physical examination:Temperature 37.8 °C, Pulse 93 beats per minute, Respiratory rate 30 times per minute, and Blood Pressure 118/78 mmHg. BP118/78 mmHg, Patient was conscious and no abnormality was revealed during heart and lung auscultations. Abdominal distension, mild tenderness and rebound tenderness are positive for the whole abdomen. no percussion pain in the liver and kidney, The bottom of the uterus is at subumbilical 1.5 cm.The intraperitoneal bladder rupture and peri-bladder inflammation were diagnosed and confirmed by MR, CT, cystoradiography and cystoscopy. Exploratory laparotomy was performed to remove the lesion on the bladder wall and repair perforation. The pelvic drainage tube was indwelled. Finally, Patient made a good recovery and was discharged on the 8th day after surgery.\nThe bladder was ruptured within abdominal cavity, the rupture site was located on the right top wall of bladder with the size of 10 mm × 10 mm, the edge of rupture was irregular. The local wall of bladder was thickened and formed a mass with size of 60 mm × 30 mm. The mass was located within the serosa membrane and depressed the bladder. The broken site was at the edge of the mass. The edema and adhesion around the bladder was observed (Fig. ). The mass was proven to be inflammatory granuloma by pathological examination (Fig. ). | [[23.0, 'year']] | F | {'20221826': 1, '15217426': 1, '33343963': 2, '34679583': 2, '34796196': 2, '2718073': 1, '34122695': 1, '6845121': 1, '34812299': 2, '2711546': 1, '19457813': 1, '31211082': 1, '32049867': 2, '34693398': 1, '33100570': 1, '1519581': 1, '21095427': 1, '20438556': 1, '29996926': 2} | {'8592997-1': 1, '8592997-2': 1, '8604095-1': 1, '7035120-1': 1, '8534473-1': 1, '7725554-1': 1} |
1,176 | 6042331-1 | 30,008,805 | comm/PMC006xxxxxx/PMC6042331.xml | An uncommon t(9;11)(p24;q22) with monoallelic loss of ATM and KMT2A genes in a child with myelodysplastic syndrome/acute myeloid leukemia who evolved from Fanconi anemia | A five-year-old male patient with recurrent infections and persistent anemia was admitted at the National Cancer Institute, Rio de Janeiro, Brazil. Physical examination showed a small stature (<P2); hyperpigmentation around the eyes; enophthalmia; multiple cafe-au-lait spots; hypoplasia of the thenar eminence accompanies left thumb hypoplasia. Laboratory findings: Hb 9.1 g/dl (age-adjusted 13.5–18.0 g/dl), platelet count 40 × 109/l (150-400 × 109/l) and white blood cell count 7.6 × 109/l (age-adjusted range 4-10 × 109/l). BM findings: hypocellularity and normal karyotype by G-banding, according to the International System form Human Cytogenomic Nomenclature (ISCN 2016) []. Clinical genetic exams were done at Medical Genetic Department, Fernandes Figueira National Institute, Oswaldo Cruz Foundation, Rio de Janeiro, Brazil. Chromosome breakage test cytogenetic analysis was performed in peripheral lymphocytes during 72 h of cultures exposed to DEB (0.1 μg/ml), according to Auerbach []. This analysis demonstrated spontaneous chromosome breakage - 0.16 breaks per cell (reference 00.00–0.08) and DEB-induced chromosome breakage - 2,32 breaks per cell (reference 0.00–0.08), confirming the FA diagnosis. He was hospitalized due to the persistent anemia and progressive neutropenia. Oxymetholone (50 mg/day) was the initial treatment. A partial hematological response was achieved, even with oxymetholone dose reductions due to liver toxicity. However, the hematological parameters worsened and erythropoietin (EPO) and granulocyte-colony stimulating factor (G-CSF) were associated, as well as danazol 200 mg/day was introduced. He also received multiple blood transfusions during the treatment but no satisfactory response was achieved. At this time, BM evaluation revealed dysplastic megakaryocytes, intense dyserythropoiesis and 11% of myeloblasts. Immunophenotypic analysis of BM cells also revealed 11% of myeloblasts expressing CD34/CD13/CD11b (54.25%), HLA-DR/CD33/CD7 (31,54%), dysplastic erythropoiesis (CD36/CD71/CD235a), dysgranulopoiesis (CD13/CD16/CD11b/Cd33/CD64/CD15,CD45) and monocytic lineage expressing CD14/CD64/CD36/HLA-DR,CD45. Cytogenetic analysis with G-band technique in bone marrow cells showed an abnormal karyotype: 46,XY,der(9)t(9;11)(p24;q?22)[9]/46,XY[12] (Fig. ). The diagnosis was MDS, refractory anemia with excess of blasts (RAEB). Fluorescence in situ hybridization (FISH) was performed to analyze some genes that may be altered during chromosomal rearrangement. So, we investigated the CDKN2A gene (located in 9p) and the ATM and KMT2A genes (located in 11q22 and 11q23, respectively), due to the important role they play during leukemogenesis. The FISH analysis for the CDKN2A gene showed two normal signals (Fig. ). It was observed a monoallelic loss of KMT2A and ATM genes (Fig. and , respectively). The final karyotype with G-banded and FISH analysis, according to the ISCN 2016 [], was: 46,XY,der(9)t(9;11)(p24;q?22),der(11)t(9;11)(p24;q?22)[9].ish del(11)(q22.3q23)(ATM-)(KMT2A-)[7]. Treatment with decitabine was initiated and well tolerated, although progression to AML occurred 3 months later. He had no remission with systemic chemotherapy. The patient died 8 months after the diagnosis of RAEB-t, due to disease progression and infectious complications. Allogeneic HSCT was indicated early in the diagnosis, but non-consanguineous parents were available and no donor was found. | [[5.0, 'year']] | M | {'28474441': 1, '29167174': 1, '18179970': 1, '17036037': 1, '27760710': 1, '19508850': 1, '27248996': 1, '28637614': 1, '22517793': 1, '21554264': 1, '22675616': 1, '28977983': 1, '24859875': 1, '10814674': 1, '22693659': 1, '20023263': 1, '21325596': 1, '23161685': 1, '21156231': 1, '28240841': 1, '29198440': 1, '20691474': 1, '12086603': 1, '28701730': 1, '28212262': 1, '30008805': 2} | {} |
1,177 | 6042377-1 | 29,996,818 | comm/PMC006xxxxxx/PMC6042377.xml | Pulmonary tumor thrombotic microangiopathy and pulmonary veno-occlusive disease in a woman with cervical cancer treated with cediranib and durvalumab | A 45-year-old woman with metastatic squamous cell carcinoma of the cervix refractory to standard of care chemotherapy was referred to the National Institutes of Health (NIH) for enrollment in a Phase I clinical trial (NCT02484404) of combination therapy with daily cediranib, a VEGFR tyrosine kinase inhibitor, and once every 2 weeks of durvalumab, a PD-L1 inhibitor. Her first four treatment cycles were well tolerated. Treatment related side effects included hypertension, subclinical hypothyroidism, non-nephrotic range proteinuria and mild diarrhea.\nDuring a routine study clinic visit the patient was found to be in sinus tachycardia. Upon further questioning, the patient noted progressive dyspnea on exertion and fatigue over the previous month and was therefore admitted to the NIH Clinical Center for further evaluation. Vital signs revealed a temperature of 37°C, heart rate of 120 beats per minute, a manual blood pressure of 90/72 mmHg without orthostatic changes and oxygen saturation ranging from 93 to 97% on room air. Physical examination findings were notable for a normal jugular venous pressure, regular heart rate without a prominent P2, clear breath sounds, and warm extremities without edema. Intravenous fluid was administered for possible dehydration due to diarrhea, but symptoms did not improve. A portable chest x-ray revealed hazy bibasilar interstitial markings (Fig. ). Laboratory studies revealed a hemoglobin of 9.8 g/dL (normal range 11.2-15.7 g/dl), a platelet count of 159 k/μL (normal range 173-369 k/μL), normal coagulation indices (PT 13.8 s; aPTT 35.5 s; thrombin time 15.8 s), a D-dimer of 0.98 μg/mL (normal range 0.00–0.50 μg/mL), a fibrinogen of 517 mg/dL (normal range 177–466 mg/dL), a pro-brain natriuretic peptide of 4541 pg/mL (normal range 0–124 pg/mL) and a troponin-T of 0.022 ng/mL (normal range 0.000–0.009 ng/mL). Echocardiography demonstrated a dilated right ventricle with decreased function (tricuspid annular plane systolic excursion (TAPSE) of 8 mm; normal ≥17 mm) and an elevated right ventricular systolic pressure of 67 mmHg, new findings compared to her baseline echocardiogram completed just 4 months earlier. Cardiac MRI, performed to evaluate for possible treatment related myocarditis, demonstrated severely reduced right ventricular function (ejection fraction of 27%; normal 61±10%) with volume and pressure overload consistent with pulmonary hypertension, but no evidence of myocarditis. A CT angiogram showed no evidence of pulmonary emboli, however there was a prominence of interstitial markings (Fig. ), the main pulmonary artery was enlarged (Fig. ) and the right atrium and ventricle were both severely dilated (Fig. ). A ventilation (Fig. ) - perfusion (Fig. ) scan (VQ) demonstrated mismatched perfusion defects along the pleural margins that were interpreted as a high probability of pulmonary emboli. Doppler ultrasonography revealed no evidence of venous thrombosis in the lower extremities. Despite the equivocal findings, therapeutic anti-coagulation was started for possible pulmonary emboli.\nOver the ensuing 10 days the patient’s clinical course progressively deteriorated heralded by a syncopal event and the development of significant resting hypoxemia. The patient was referred for right and left heart catheterization which confirmed pre-capillary pulmonary hypertension with an associated low cardiac output state (Table ). Vasodilator challenge with nitric oxide (40 ppm) plus 100% oxygen paradoxically increased pulmonary artery mean and occlusion pressure, but did not result in symptomatic pulmonary edema. Balloon occlusion pulmonary venous blood sampling did not reveal any circulating tumor cells. Aggressive therapy for right heart failure was initiated including intravenous diuresis, inotropic support and pulmonary vasodilator therapy. Riociguat was initiated due to the concern for possible chronic thromboembolic pulmonary hypertension (CTEPH). Ultimately, intravenous epoprostenol was added due to persistent symptoms of severe right heart failure. Soon after starting epoprostenol the patient became severely hypoxic. As a result, pulmonary vasodilator therapy was discontinued and she was treated with diuretics and high flow oxygen. Given her advanced disease and poor functional status her protocol treatment was stopped and she was discharged home with hospice care. She died 1 day later and an autopsy was performed.\nGross examination of the lungs showed scattered white plaques, nodular and gritty on palpation, covering the pleural surface of the lower lobes. Microscopic sections of the lung showed metastatic carcinoma with lymphangitic spread (Fig. ) within the septa and subpleural spaces with associated congestion and fibrosis. These findings were associated with fibromuscular thickening of lymphatic vessels which may represent a type of desmoplastic response to lymphatic obstruction. Pulmonary arterioles (Fig. , ) were occluded by organized thrombi, some with evidence of recanalization, as well as smooth muscle proliferation and ingrowth of fibroblasts. Some arteries showed fresh thrombi with metastatic tumor foci. Pulmonary venules were also occluded with ingrowth of fibroblasts. Some of these vessels showed evidence of chronic occlusion and recanalization, (Fig. ) while others were completely occluded. No tumor cells were identified in any venules. No evidence of infection or pneumonia was identified. Collectively, these findings were most consistent with a primary diagnosis of PTTM with features of PVOD [, ]. | [[45.0, 'year']] | F | {'20008624': 1, '34379693': 1, '25497573': 1, '24334027': 1, '22837856': 1, '20472834': 1, '8404211': 1, '29320654': 1, '24932885': 1, '27387102': 1, '23536282': 1, '24951765': 1, '11673836': 1, '23168066': 1, '28471727': 1, '32257112': 1, '32675452': 2, '33950913': 1, '2471933': 1, '23774540': 1, '22216845': 1, '28559461': 1, '7094496': 1, '28084316': 1, '26206876': 1, '28782988': 1, '2163747': 1, '455208': 1, '26808628': 1, '17495297': 1, '23908119': 1, '29996818': 2} | {'7542055-1': 1, '7542055-2': 1, '7542055-3': 1, '7542055-4': 1} |
1,178 | 6042392-1 | 29,996,943 | comm/PMC006xxxxxx/PMC6042392.xml | Lethal outcome of granulomatous acanthamoebic encephalitis in a man who was human immunodeficiency virus-positive: a case report | In December 2010, a 54-year-old German man presented to our hospital with suspected cerebral toxoplasmosis. HIV infection had been diagnosed in 1995. He had received a triple-combination highly active antiretroviral therapy (HAART) of lopinavir, lamivudine, and tenofovir, which was stopped in 2009 due to intolerable side effects (diarrhea, nausea).\nOver a period of 5 days, progressive neurological deficits including sensorimotor paresis of his right leg and deterioration of alertness occurred.\nOn clinical and neurological examination, he showed high-grade flaccid paralysis of his right lower limb with preserved muscle proprioceptive reflexes and positive Babinski sign.\nMagnetic resonance imaging (MRI) on day 1 revealed a periventricular hyperintense lesion with perifocal edema in the left parieto-occipital region which continued to progress as shown in imaging on day 6 (Fig. ).\nBlood laboratory values on day 1 revealed leukopenia (3.4/nL) and thrombocytopenia (101/nL).\nNo fungi, viruses (except HIV-1), or bacteria were detected in blood and cerebrospinal fluid cultures nor in serologic tests and polymerase chain reaction (PCR; Table ). Prophylactic antibiotic treatment (antifungal, antiviral, antibacterial, and antiprotozoal) was administered as listed in Table . Immunocytology of cerebrospinal fluid on day 5 showed a reduced absolute lymphocyte count (640/μL), reduced T-helper cells (CD3, 365/μL), and a pathologic CD4/CD8 ratio.\nDue to pathologic MRI findings, a stereotactic biopsy was performed on day 9. Histopathological results obtained on day 12 showed extensive tissue necrosis with mixed inflammatory infiltrates. Cysts of Acanthamoeba species were detected in periodic acid–Schiff (PAS) and Grocott stainings of brain specimens. Mononuclear trophozoites could be identified in hematoxylin and eosin (HE) and PAS stainings (Fig. ). Furthermore, additional immunohistochemical staining was performed using an antibody specific to Acanthamoeba species (from rabbits immunized with Acanthamoeba genotype 4; Fig. ).\nElectroencephalography (EEG) on day 6 showed a lesion located in his left frontotemporal region with epileptic patterns in the left parietal lobe. He developed relapsing tonic-clonic seizures which normalized by day 8 following anticonvulsive therapy with valproic acid, methohexital, levetiracetam, and clonazepam.\nMRI on day 11 revealed a new and massive ubiquitous subarachnoid hemorrhage, a beginning compression, a generalized cerebral swelling, and, an expanding left parietal periventricular lesion.\nDue to the unfavorable prognosis, we, in agreement with his relatives, took no further intensive care measures. He died on day 12 after hospital admission.\nSince histopathology did not reveal GAE before day 12, a specific treatment against GAE (for example, miltefosine-based combination therapy) had not been initiated. | [[54.0, 'year']] | M | {'25619531': 1, '727637': 1, '20802332': 1, '11015431': 1, '23965830': 1, '19847677': 1, '27142544': 1, '12692099': 1, '15956445': 1, '11511003': 1, '11419508': 1, '19041307': 1, '17552103': 1, '33488560': 1, '18976559': 1, '16672436': 1, '25687209': 1, '11326011': 1, '15072770': 1, '31502076': 1, '25047131': 1, '25848385': 1, '29996943': 2} | {} |
1,179 | 6042427-1 | 29,996,908 | comm/PMC006xxxxxx/PMC6042427.xml | Marijuana intoxication in a cat | A 6-year-old neutered female Persian cat presented to the “Cztery Łapy i Ty” veterinary clinic, Poland due to strong psychomotor agitation turning into aggression. The owner described the animal as “absent-minded”, disoriented, not reacting when called and behaving “strangely”. The cat walked around aimlessly, tried jumping into inaccessible areas, ran around the room, then hid and attacked people. This behavior was accompanied by loud meowing and hissing. The owner observed that prior to the aggression episodes, the cat had mydriatic pupils, which she believed showed “strong fear”. The cat demonstrated increased thirst, polyuria, attacks of gluttony mixed with periods of inappetence. During the attacks, the animal was not able to eat or drink by itself. According to the owner, the behavior of the animal made the members of the household afraid of it as it clapperclawed and bit its carers during the attacks. The periods of agitation and aggression were mixed with periods of normal behavior.\nWhen the cat began to have sialorrhoea, sneezed and was not able to swallow, the owner sought veterinary assistance. The cat was examined clinically and then hospitalized for 3 weeks. A complete blood count and basic blood biochemical parameters were evaluated and found to be within normal range.\nThe cat was also clinically observed for symptoms of rabies over a 15-day hospitalisation period, but rabies was finally excluded. During the hospitalisation period, the cat behaved normally and had no attacks of unwanted behavior. The cat was then returned to its home.\nAfter taking the cat home, the owner had to leave the pet with her partner for 3 weeks. When she returned, the symptoms of agitation/aggression and attacks had reoccurred but were more frequent and stronger. The day rhythm of the cat also changed—as it was mostly agitated at night.\nVeterinary advice was sought again. Initially the cat was calm during the clinical examination, which included sampling of blood. The general condition of the cat was good. Respiration, pulse and rectal temperature were normal. The mucous membrane was pink and moist and the lymph nodes of normal size. The abdominal cavity was soft and painless on palpation and the patient showed no neurological abnormalities except for symmetrical mydriasis and scleral congestion. But during the consultation, the cat’s behavior changed diametrically. Alternate periods of agitation and apathy, each lasting several minutes, occurred. During the period of agitation, the cat jumped onto furniture, intensively explored the premises, walked into objects and stepped into a water bowl. In the quiet period, the cat sat in one place staring at one point and reacted poorly to external stimuli (mainly vocal; calling by the owner).\nIn the follow-up interview it turned out, that the aggressive behaviour commenced after longer absences of the owner of at least a couple of days, when the owner’s partner looked after the cat. The examining veterinarian asked the owner to contact her partner in order to identify possible causes of the medical condition of the cat. It turned out that the partner was marijuana-dependent and he admitted to have smoked marijuana while the cat’s owner was absent and to have exhaled marijuana smoke directly in the cat’s face “as a joke”.\nThe cat was given an isotonic solution of NaCl 2.5 and 2.5% glucose (Fresenius Kabi Polska Sp. z o.o., Warsaw, Poland) parenterally at a dose of 40 mL/kg/day for 2 days and was hospitalised in order to monitor the condition. During the 48-h hospitalisation, there were two periods of agitation and aggression. Sedatives were not applied as the attacks lasted less than a few minutes.\nThe blood sample underwent toxicological analysis. A screening enzyme-linked immunoassay analysis (ELISA) was used to screen for opiates, cannabinols, benzodiazepines, amphetamines and their derivatives as well as cocaine. A positive result was observed for cannabinols. Hence, further verification and quantitative tests were conducted with the use of gas chromatography tandem mass spectrometry (GC–MS) (Gas chromatograph: Aglient Technologies 6890N, Santa Clara, CA, USA; Mass spectrometer Aglient Technologies 5975B Santa Clara, CA, USA). The blood tests revealed the presence of delta-9-THC at 5.5 ng/mL, 11-hydroxy-delta-9-THC at a concentration of 1.2 ng/mL, and 11-carboxy-delta-9-THC at a concentration of 13.8 ng/mL.\nTaking into account the clinical signs as well as the result of the toxicological tests, marijuana intoxication was diagnosed as the cause of the cat’s disease. The animal was returned to its owner and future isolation of the animal from marijuana smoke was advised. | [[6.0, 'year']] | F | {'32017686': 1, '23216842': 1, '1248507': 1, '12142262': 1, '33769105': 1, '7402446': 1, '23796481': 1, '4787563': 1, '4746199': 1, '14748410': 1, '6289158': 1, '4199474': 1, '33531559': 1, '29996908': 2} | {} |
1,180 | 6042469-1 | 30,026,723 | comm/PMC006xxxxxx/PMC6042469.xml | Multidrug Resistant Brain Abscess Due to Acinetobacter baumannii Ventriculitis Cleared by Intraventricular and Intravenous Tigecycline Therapy: A Case Report and Review of Literature | A 55-year-old male patient, who was admitted to the local medical facility due to the sudden onset of severe headache and loss of consciousness, had a cerebellar hematoma on CT scan. He underwent an emergency placement of an external ventricular drain (EVD) on October 7, 2017. The patient was transferred to our hospital 3 days later because of high fever with Glasgow Coma Scale (GCS) score of 4. Follow-up CT indicated cerebellar hemorrhage in the bilateral and 3rd ventricles (Figure ). CSF from EVD revealed a white blood cell count of 1,280 × 106/L, total protein 4.18 g/L, and glucose 2.26 mmol/L, the simultaneous blood glucose level was 7.5 mmol/L (Table ). The sputum culture tested positive for extreme-drug (EXD)-resistant A. baumannii, which was sensitive to amikacin only. Thus, pulmonary and intracranial infections were highly suspected, and vancomycin (1 g/day twice daily via IV) and amikacin (0.4 g/day twice daily via IV) were initiated empirically (October 12, 2017). During the follow-up period, the patient's fever gradually subsided. The laboratory CSF analysis improved, and the CSF cultures tested negative. However, the bacterial load further increased and the fever recurred.\nOn October 31, 2017, magnetic resonance imaging revealed hydrocephalus and interstitial edema beside bilateral ventricles. In addition, occipital horn enhancement bilaterally suggested intracranial infection (Figure ). An endoscopic ventriculostomy was done, a large amount of pus was removed from the ventricles (Figure ), and two EVDs were placed for antibiotic therapy (bilateral occipital horn; Figure ). The CSF cultures revealed an XDR strain of A. baumannii (November 6, 2017) and a minimum inhibitory tigecycline concentration of 16 μg/mL. On November 8, 2017, the patient was administered tigecycline (100 mg twice daily via IV), cefoperazone sulbactam (2 g every time, thrice daily via IV), and CVI tigecycline (10 mg/500 mL saline twice daily, in from the right occipital horn and out from the left horn). A leakage was observed around the drainage tube 4 days later. This was probably because we had to exchange the in-out tube, due to the frequent obstruction of the drainage tube or to the rather short (< 3 cm) subcutaneous tunnel of the drainage tube. Although the dressing was changed twice daily and the wound was sutured several times, the leakage persisted. Therefore, on November 14, 2017, we removed the two occipital horn drainage tubes and replaced it with two frontal horn drainage tubes with a bigger size (12#) and a longer subcutaneous tunneling (10 cm) (Figure ). Perioperative antibiotics were administered and the leakage stopped. Twelve days from the start of the CVI tigecycline, the signs of ventriculitis disappeared, and the Acinetobacter CSF load steadily decreased until CSF sterilization. Thus, we adjusted the IVT tigecycline dosage to 50 mg twice daily, whereas CVI tigecycline was adjusted to IVT tigecycline (2 mg twice daily). Tigecycline was diluted in saline up to 4 mL and was slowly injected into the lateral ventricles via EVD. After every injection, the CSF drain was temporarily closed for 2 h to prevent the untimely washout of the drug. The tigecycline treatment was well tolerated by the patient. The ventricular drainage tubes were removed, and the antibiotic therapy was discontinued after 1 week of persistent negative CSF cultures and laboratory CSF analysis (Table ). The follow-up MRI (November 27, 2017) was negative for ventriculitis (Figure ). Thus, on November 29, 2017, 14 days after the restart of the CVI tigecycline and 3 days after the IVT tigecycline, the patient was transferred to a rehabilitation unit.\nThe GCS score of the patient was 10 (E4VM5) after 2 months of follow-up, and the symptoms of intracranial infection were not observed. Repeated CSF cultures were all negative, and no radiological signs of neuro-inflammation were found. The laboratory tests for CSF during follow-up (January 17, 2018) are shown in Table . | [[55.0, 'year']] | M | {'32963684': 1, '19324297': 1, '28767605': 2, '27436468': 1, '33656584': 1, '33553404': 2, '32349999': 1, '32518927': 1, '28614982': 1, '20210684': 1, '23507414': 1, '27365967': 1, '20445494': 1, '19962335': 1, '16040625': 1, '27540902': 1, '19022191': 1, '31372014': 1, '19162449': 1, '17270377': 1, '30026723': 2} | {'5626159-1': 1, '7829735-1': 1} |
1,181 | 6043242-1 | 30,009,264 | comm/PMC006xxxxxx/PMC6043242.xml | Bile Duct Injury in Children: Is There a Role for Early Endoscopic Retrograde Cholangiopancreatography? | A 15-year-old male sustained a grade IV liver laceration (\n) during an all-terrain vehicle accident. He was hemodynamically stable and treated nonoperatively with intensive care unit (ICU) observation for 24 hours and bed rest for a total of 5 days. On hospital day 6, he developed abdominal distension and a high-grade fever. Chest radiograph showed bilateral atelectasis and a right-sided pleural effusion. Abdominal ultrasound (US) revealed a small collection on the right lobe of the liver within the hematoma. Broad-spectrum antibiotics were started, and US-guided drainage of the pleural fluid was performed. The fluid was serous without evidence of infection or bile. The patient developed a worsening ileus and abdominal tenderness a few days later. Repeat abdominal US showed an increase in the amount of free fluid in the right upper quadrant. In addition, his serum conjugated bilirubin was elevated; therefore, suspicion of an ongoing bile leak was high. On hospital day 10 after injury, the patient underwent ERCP (\n), which demonstrated a leak from one of the secondary bile ducts in the right lobe. Sphincterotomy was performed with insertion of a transampullary stent. Two days later, his ileus resolved. He was started on oral diet, antibiotics were stopped, and then he was discharged home 5 days following the ERCP and stenting. At his 6-week follow-up, the patient had resumed normal physical activity, his bilirubin had normalized, and repeat abdominal US showed complete healing of the liver injury. Biliary stent was removed 8 weeks after placement. | [[15.0, 'year']] | M | {'32563827': 2, '15729238': 1, '17531646': 1, '17688661': 1, '7794078': 1, '16917447': 1, '16952588': 1, '11987098': 1, '12194118': 1, '16205513': 1, '16677889': 1, '25638632': 1, '12407549': 1, '24650470': 1, '10759206': 1, '30009264': 2} | {'6043242-2': 2, '6043242-3': 2, '7306525-1': 1} |
1,182 | 6043242-2 | 30,009,264 | comm/PMC006xxxxxx/PMC6043242.xml | Bile Duct Injury in Children: Is There a Role for Early Endoscopic Retrograde Cholangiopancreatography? | A 12-year-old female pedestrian suffered multisystem blunt trauma on the right side of her abdomen after being struck by a sports utility vehicle. The patient suffered a closed head injury, bilateral pulmonary contusions, and grade IV liver laceration. Because the patient was vitally stable, she was observed in the ICU. Seven days after the initial injury, the patient's abdomen became markedly distended and caused respiratory compromise requiring reintubation. Repeat computed tomography (CT) scan of the abdomen (\n) showed a significant amount of free fluid in the peritoneal cavity. An abdominal drain was inserted under US guidance, and several liters of bloody fluid and bile were removed. Despite the drain, the patient went on to develop infected bile peritonitis (peritoneal culture grew pseudomonas aeruginosa) showing signs of severe sepsis requiring laparotomy and washout, as well as extensive abdominal drainage. Ten days after the laparotomy and 4 weeks after the initial injury, bile leak was persistent and the patient underwent ERCP (\n), which demonstrated bile leakage from a left second biliary radicle. A transampullary stent was placed. Shortly thereafter, she improved dramatically and was discharged home after a 7-week hospital stay. Bilirubin had returned to normal prior to discharge. A follow-up US 6 weeks later showed no fluid collection or vascular abnormality. The stent was removed 8 weeks later. At her 12-month follow-up, the patient had resumed her normal activity with no residual symptoms. | [[12.0, 'year']] | F | {'32563827': 2, '15729238': 1, '17531646': 1, '17688661': 1, '7794078': 1, '16917447': 1, '16952588': 1, '11987098': 1, '12194118': 1, '16205513': 1, '16677889': 1, '25638632': 1, '12407549': 1, '24650470': 1, '10759206': 1, '30009264': 2} | {'6043242-1': 2, '6043242-3': 2, '7306525-1': 1} |
1,183 | 6043242-3 | 30,009,264 | comm/PMC006xxxxxx/PMC6043242.xml | Bile Duct Injury in Children: Is There a Role for Early Endoscopic Retrograde Cholangiopancreatography? | A 10-year-old boy sustained a handlebar injury while riding a snowmobile. He suffered a grade IV liver laceration with a vascular injury to segment 4 that necessitated emergency angiogram and embolization. Postembolization he developed respiratory distress secondary to increasing abdominal distention. On postinjury day 5, a drain was inserted into the peritoneal cavity which drained approximately 1.5 L of bile. This resulted in clinical improvement, but an HIDA scan postinjury day 8 demonstrated ongoing bile leak from the laceration site. And ERCP demonstrated a leak just distal to the bifurcation of the right hepatic duct. This was treated with stenting and sphincterotomy on postinjury day 10. Unfortunately, the patient continued to have abdominal distention and discomfort and on postinjury day 12, he started deteriorating clinically necessitating an urgent laparotomy and drainage of multiple infected bile and blood collections. Postoperatively he continued to improve slowly until discharge home with close follow-up.\nHis biliary stent was removed 3 months after insertion. At his 6-month follow-up, he was doing well and had resumed all of his physical activities. | [[10.0, 'year']] | M | {'32563827': 2, '15729238': 1, '17531646': 1, '17688661': 1, '7794078': 1, '16917447': 1, '16952588': 1, '11987098': 1, '12194118': 1, '16205513': 1, '16677889': 1, '25638632': 1, '12407549': 1, '24650470': 1, '10759206': 1, '30009264': 2} | {'6043242-1': 2, '6043242-2': 2, '7306525-1': 1} |
1,184 | 6043464-1 | 30,003,446 | comm/PMC006xxxxxx/PMC6043464.xml | Detection of needle tract implantation and peritoneal seeding after radiofrequency ablation using intraoperative near-infrared fluorescence system for recurrent hepatocellular carcinoma: a case report | A 76-year-old man originally underwent medial sectionectomy for HCC in 2009. When repeated intrahepatic recurrence occurred, he underwent RFA and transcatheter arterial chemoembolization (TACE) for recurrent HCC twice at segment III and once at segment IV. A 1.5-cm-diameter tumor at segment III was ablated by RFA needle twice for the first time. Four months after this treatment, a 1-cm diameter of new recurrence around the previous lesion at segment III was pointed out and ablated once by RFA needle. In 2013, the second hepatectomy for recurrent HCC at segment VIII was performed. In 2016, he had recurrent HCC at segment III around a previous RFA and TACE scar again; left lateral sectionectomy was therefore planned (Fig. ).\nThe patient had persistent infection of chronic hepatitis C and diabetes requiring insulin. Laboratory data before this operation is shown in Table . Focused on tumor markers, des-gamma carboxyprothrombin (DCP) was high while alpha-fetoprotein (AFP) and lectin-reactive alpha-fetoprotein (AFP-L3%) were within normal limits. Liver function was good, Child-Pugh score 5A. We therefore considered these tumors to be within operative indication for left lateral sectionectomy as the third operation on the clinical diagnosis of recurrent HCC of Union for International Cancer Control (UICC) TNM staging stage IB (T1bN0M0).\nWe planned to use ICG-NIRF system to observe the main intrahepatic metastasis at segment III and searched for other tumors in the remnant liver. Two days before the operation, 0.5 mg/kg ICG (Diagnogreen, Daiichi-Sankyo, Tokyo, Japan) was intravenously injected. Photodynamic eye (PDE, Hamamatsu Photonics, Hamamatsu, Japan) was used as a detector of NIRF. Intraoperative gross appearance is shown in Fig. . The recurrent tumor was located at the liver surface of segment III by fluorescent signal that could be detected by NIRF system (Fig. ). There was no signal on the surface of future remnant liver (right lobe). We incidentally observed tiny signals in the right upper abdominal wall, however (Fig. ). Another fluorescent tumor was detected in the greater omentum (Fig. ). These tumors were thought to be seeding of the previous RFA, but localized and controlled. After left lateral sectionectomy, they were also resected. Fluorescent signals were observed from all tumors in each specimen, even after resection (Fig. ). Operation time was 141 min and intraoperative bleeding was 230 ml.\nHistopathological examination revealed that the main recurrent liver tumor was moderately differentiated HCC showing the progression pattern of pseudoglandular type, including the bile duct with microvascular invasion for portal vein (vp1) (Fig. ). The main tumor included both viable and necrotic areas because of the previous TACE and RFA. Both abdominal wall and greater omentum lesions were pathologically demonstrated to be metastases of HCC (Fig. ).\nThe patient was discharged from our hospital on the eighth postoperative day without any complications. There has been no further recurrence 14 months after the operation.\nIn this study, we incidentally detected implanted and disseminated needle tract and peritoneal seeding from previous RFA by intraoperative NIRF imaging system. Detection of intrahepatic HCC was reported by Gotoh et al. [] and Ishizawa et al. []. There are several reports about HCC detection using ICG-NIRF system [, ]. Moreover, this system could also be used to detect extrahepatic HCC lesions, such as in the appendix [], lymph node, lungs, peritoneum, and adrenal glands []. The detailed mechanism of how extrahepatic tumor cells uptake ICG and emit fluorescence remains unclear. Satou et al. hypothesized that the extrahepatic HCC cells themselves also possess the capability to take up ICG from the blood stream. The absence of other adjacent cells or vessels, including the biliary system, may have caused the ICG retention in the metastatic lesions []. Further pathological examination is needed to understand this phenomenon.\nThis patient underwent repeated RFA for recurrent HCC. Percutaneous procedures have a risk of needle tract implantation, and peritoneal seeding and frequency of these complications are reported as between 0.9 and 12.5% [, –]. Llovet et al. indicated risk factors for complications include subcapsular lesions, use of cool-tip-type electrode needles, poorly differentiated HCC, and high levels of alpha-fetoprotein.\nEfficacy of resection for needle tract implantation was suggested by Noda et al. []. One-year survival rate was 85% for patients who underwent resection of extrahepatic lesions, according to Satou et al. []. Operative indications are generally restricted to selective patients, however []. Time intervals between RFA and diagnosis of needle tract implantation were between 3 weeks and 48 months []; the mean doubling time was 112 (range 22–415) days []. ICG-NIRF system might be useful for the removal of these lesions during the operation for RFA treatment.\nICG-NIRF system has some limitations, however. The system is restricted to detection of fluorescence for tumors 5–10 mm from the liver surface [, ]. There were also false-positive results for liver cysts and dysplastic tumors in severe liver cirrhosis []. In the detection of extrahepatic lesions, however, there are no such limitations. This system might be helpful in the detection of needle tract implantations or peritoneal seeding. | [[76.0, 'year']] | M | {'24254203': 1, '23179608': 1, '19326450': 1, '23794086': 1, '19301311': 1, '33653302': 2, '15064996': 1, '18219905': 1, '33718233': 1, '19086957': 1, '24828028': 1, '22797964': 1, '16037512': 1, '19432967': 1, '22200990': 1, '11343240': 1, '21994847': 1, '15892154': 1, '16120469': 1, '10648580': 1, '30003446': 2} | {'7923305-1': 1} |
1,185 | 6043705-1 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 61-year-old woman presented with a persistent headache for 6 days which started after she received an epidural steroid injection for chronic back pain. The patient had no history of any similar headaches in the past. The headache was described as severe and throbbing. The headache was worse with activity and upright position and improved in the supine position. She rated the severity as 8/10 at peak intensity. The patient underwent magnetic resonance imaging of the brain with gadolinium enhancement that showed the mild descent of cerebellar tonsils through foramen magnum but no dural enhancement. Magnetic resonance venogram was unremarkable showing no evidence of venous sinus thrombosis. The patient underwent epidural blood injection and reported improvement in severity of headaches during upright posture to 5/10 in severity. Headache frequency and intensity were decreased over next 2 days after which she was discharged on pain medication. | [[61.0, 'year']] | F | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-2': 2, '6043705-3': 2, '6043705-4': 2, '6043705-5': 2, '6043705-6': 2} |
1,186 | 6043705-2 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 25-year-old woman underwent fluoroscopic-guided lumbar puncture for new-onset occipital headaches primarily on the right side occasionally radiating to the temporal region. The initial pressure was 10 mmHg by fluid-coupled measurements and 11 cm of H2O by fluid column method. A total of 18 cc of clear CSF was obtained. The final pressure was 6 mmHg by fluid-coupled measurements and 7 cm of H2O by fluid column method. The patient developed headaches (rated as 10/10 in severity) that were exacerbated by upright posture postprocedure. The headache continued for 5 days postprocedure and had not improved with hydromorphone, acetaminophen, decadron, indomethacin, lorazepam, caffeine sodium benzoate, and hydrocodone-acetaminophen treatment. The patient underwent an occipital nerve block which did not reduce the severity of the positional headaches. Magnetic resonance imaging with gadolinium enhancement demonstrated protrusion of cerebellar tonsils through the foramen magnum, slit ventricles, and enhancement of dura. The patient underwent epidural injection of whole blood and reported complete resolution of headaches (0/10) and was able to ambulate without any difficulty. | [[25.0, 'year']] | F | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-1': 2, '6043705-3': 2, '6043705-4': 2, '6043705-5': 2, '6043705-6': 2} |
1,187 | 6043705-3 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 20-year-old woman had a progressively worsening headache since delivery and epidural anesthesia at lumbar 4 and 5 vertebral level 5 days ago. The headache was initially frontal but became diffuse and was associated with nausea and vomiting. The headache was described as 9/10 in severity and worse on standing position and interfered with activities of daily living and sleep. The patient received ibuprofen, intravenous caffeine, caffeine tablets, ketorolac without any significant relief. Magnetic resonance imaging with gadolinium enhancement demonstrates cerebellar tonsils descending through the foramen magnum, slit ventricles, and enhancement of dura. The patient underwent epidural injection of whole blood and reported complete resolution of headaches (0/10) immediately postprocedure. | [[20.0, 'year']] | F | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-1': 2, '6043705-2': 2, '6043705-4': 2, '6043705-5': 2, '6043705-6': 2} |
1,188 | 6043705-4 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 34-year-old woman with past medical history of complex regional pain syndrome developed a frontal headache which progressively worsened over a period of 7 days. She developed fever and nausea and underwent a lumbar puncture as part of the diagnostic evaluation. Multiple attempts were made to sitting position for performing a lumbar puncture and CSF pressure was not measured. She reported worsening headache with localization in the high cervical segment with extension into the occipital and frontal regions after lumbar puncture accompanied by photophobia, nausea, and chest pain. Headache was worse in an upright position with the rated severity of 10/10 and reduced to 5/10 in severity in lying position. Magnetic resonance imaging with gadolinium enhancement demonstrates cerebellar tonsils descending through the foramen magnum, slit ventricles, and enhancement of dura. The patient received oxycodone-acetaminophen, intravenous caffeine, tramadol, and butalbital-acetaminophen-caffeine without any relief. The patient underwent epidural injection of whole blood and reported an immediate complete reduction in of headache severity (4/10) in an upright position with a resolution of photophobia. | [[34.0, 'year']] | F | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-1': 2, '6043705-2': 2, '6043705-3': 2, '6043705-5': 2, '6043705-6': 2} |
1,189 | 6043705-5 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 28-year-old man underwent fluoroscopic-guided lumbar puncture as part of the evaluation of new-onset diffuse headaches for several weeks ago which developed after coitus. The initial pressure was 5 mmHg by fluid-coupled measurements and 11.5 cm of H2O by fluid column method. A total of 10 cc of clear CSF was removed. The final pressure was 2 mmHg by fluid-coupled measurements and 3.5 cm of H2O by fluid column method. After lumbar puncture, he developed a headache which was different from an original headache with clear worsening when the patient was upright. The severity was rated as 8/10 and accompanied by nausea and vomiting. The headache was persistent for 4 days and had not improved with steroids, caffeine infusion, and hydrocodone-acetaminophen treatment. Magnetic resonance imaging with gadolinium enhancement demonstrates cerebellar tonsils descending through the foramen magnum, slit ventricles, and enhancement of dura. The patient underwent epidural injection of whole blood and reported complete resolution of headaches (0/10) and was able to ambulate without any difficulty. | [[28.0, 'year']] | M | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-1': 2, '6043705-2': 2, '6043705-3': 2, '6043705-4': 2, '6043705-6': 2} |
1,190 | 6043705-6 | 29,920,982 | comm/PMC006xxxxxx/PMC6043705.xml | Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: A prospective study | A 34-year-old woman presented with severe episodic headaches that started after giving birth to a child 5 months ago which was rated 5/10 in severity. The headaches were associated with nausea, vomiting, and photophobia with no clear relationship to posture. The patient also reported visual scotomas in both the eyes. Magnetic resonance imaging demonstrated findings of empty sella syndrome but there was no distortion of optic nerves or posterior aspect of the optic globe. The patient had a fluoroscopic-guided lumbar puncture performed to measure CSF pressure to exclude intracranial hypertension. The opening CSF pressure was 7 mmHg by fluid-coupled measurements and 15 cm of H20 by fluid column method. A total of 16 cc of clear CSF was removed. The final pressure was 3 mmHg by fluid-coupled measurements and 5 cm of H2O by fluid column method. The patient reported worsening of headaches postprocedure. The patient described it as a continuous and excruciating headache that progressively worsened was rated as 10/10 in severity. A noncontrast magnetic resonance imaging demonstrated cerebellar tonsils descending into the foramen magnum. A contrast-enhanced computed tomographic scan did not demonstrate any dural enhancement. The patient received caffeine tablets, topiramate, morphine injection, intravenous hydromorphone, and caffeine infusion during admission for pain but had no significant relief. The patient underwent epidural injection of whole blood and reported complete resolution of headaches (0/10) and photophobia after the procedure and was able to ambulate without any difficulty. | [[34.0, 'year']] | F | {'9579246': 1, '2080712': 1, '26908033': 1, '30888542': 1, '17261680': 1, '28295143': 1, '28591231': 1, '28043956': 1, '28613452': 1, '15676628': 1, '21329983': 1, '17184183': 1, '25874452': 1, '27149484': 1, '25595241': 1, '28738118': 1, '2197679': 1, '20618978': 1, '21658029': 1, '18258706': 1, '11182844': 1, '10975661': 1, '27906941': 1, '23771276': 1, '22336696': 1, '28414396': 1, '28738139': 1, '7829785': 1, '28522756': 1, '16291466': 1, '29920982': 2} | {'6043705-1': 2, '6043705-2': 2, '6043705-3': 2, '6043705-4': 2, '6043705-5': 2} |
1,191 | 6043957-1 | 30,001,750 | comm/PMC006xxxxxx/PMC6043957.xml | Marked attenuation of the amplitude of transcranial motor-evoked potentials after intravenous bolus administration of ketamine: a case report | A 15-year-old Japanese girl with a diagnosis of adolescent idiopathic scoliosis was admitted to our hospital to undergo posterior spinal fusion at T4–L3. She was a high school student and did not present any symptoms at admission. She did not take any medications prior to the surgery. Her past medical, social, environmental, and family history was not appreciable. She was 147 cm tall, and her weight was 40 kg. She had a Cobb angle of 60 degrees. She had no neurological symptoms. Her temperature was 36.8 °C, her blood pressure was 118/64 mmHg, and her pulse was 92 beats per minute. Laboratory findings at admission were as follows. Her white blood cell count was 6860/μl, red blood cell count 472 × 104/μl, hemoglobin 14.3 g/dl, hematocrit 42.1%, platelets 30.8 × 104/μl, aspartate transaminase 19 IU/L, alanine transaminase 15 IU/L, total bilirubin 0.5 mg/dl, γ-glutamyl transferase 16 IU/L, alkaline phosphatase 371 IU/L, total protein 8.2 g/dl, albumin 5.2 g/dl, blood urea nitrogen 11 mg/dl, creatinine 0.39 mg/dl, sodium 141 mEq/L, potassium 4.5 mEq/L, chloride 102 mEq/L, C-reactive protein 0.01 mg/dl, urinalysis pH 6.0, no uric protein, no urinary sugar, no ketone body, and no uric blood. No microbial examination was performed.\nWe planned general anesthesia using a target-controlled infusion of propofol and a continuous infusion of remifentanil to record MEPs and somatosensory evoked potentials (SSEPs). In addition, we planned to use both bolus and continuous infusions of ketamine, followed by intravenous patient-controlled fentanyl as postoperative analgesia because we believed that ketamine did not affect MEP monitoring.\nAfter securing an intravenous line in the patient’s forearm, general anesthesia was induced using a target-controlled infusion of propofol 4 μg/ml and remifentanil 0.3 μg/kg/minute. After administration of rocuronium 20 mg, the trachea was intubated using a reinforced endotracheal tube. Thereafter, 1 g of cefazolin sodium was administered intravenously every 3 hours during the surgery and every 12 hours until postoperative day 1.\nWe started preparation for recording the MEPs and SSEPs. MEPs were evoked by transcranial electrical stimulation (a train of five pulses with an interstimulus interval of 2 milliseconds; supramaximal stimulus, 400 V) with screw electrodes fixed at 2 cm anterior to C3 and C4 (cathode and anode, respectively, following the international 10–20 system). MEPs and SSEPs were recorded using an intraoperative neurophysiologic monitoring system (Neuromaster MEE-1216; Nihon Kohden, Tokyo, Japan). MEPs were recorded bilaterally from the abductor pollicis, quadriceps femoris, tibialis anterior, and flexor hallucis brevis muscles. SSEPs were evoked by stimulation (30 mA) of the sciatic nerve and recorded from 2 cm posterior to Cz (averaging the signals generated by 200 stimulations).\nAfter the patient was turned to a prone position, we confirmed that we could record MEPs from all targeted muscles without difficulty. We maintained general anesthesia using a target-controlled infusion of propofol 3.3 μg/ml and remifentanil 0.2 μg/kg/minute. The patient’s bispectral index values were below 60, and her vital signs were stable. Several minutes before the skin incision was made, the attending anesthesiologist evaluating the MEPs discovered that they were markedly attenuated (Fig. ). The attending anesthesiologist had not changed the propofol infusion rate and had not administered any other drugs except for intravenous bolus administration of ketamine 50 mg (1.25 mg/kg). Ketamine was administered about 3 minutes before the change in MEP waveforms. The patient’s blood pressure, heart rate, peripheral oxygen saturation, end-tidal CO2, and body temperature remained stable. The surgery had not started at that time. About 6 minutes after administration of ketamine, the MEP amplitudes were recovered.\nThe surgery was performed uneventfully, and the patient had no neurologic deficit when she emerged from general anesthesia. Intravenous patient-controlled analgesia (a combination of fentanyl 25 μg/ml and ketamine 2.5 mg/ml, background infusion 1 ml/hour, bolus 1 ml, and lockout time 10 minutes) was used for postoperative pain management. The patient was discharged on postoperative day 11 without any complications. She was well without complications at 2 years after the surgery. | [[15.0, 'year']] | F | {'9239584': 1, '12505959': 1, '9681399': 1, '7885550': 1, '18090070': 1, '26659198': 1, '23392233': 1, '11883843': 1, '1356320': 1, '26079533': 1, '30001750': 2} | {} |
1,192 | 6043999-1 | 30,001,695 | comm/PMC006xxxxxx/PMC6043999.xml | Xanthine calculi in a patient with Lesch-Nyhan syndrome and factor V Leiden treated with allopurinol: case report | A 12-year-old boy (height: 51 in.; weight; 44 lbs. 1.5 oz. [20.0 kg]; BMI: 11.93 kg/m2) with a history of Lesch-Nyhan syndrome presented to our office with a 1 ½ month history of dysuria, hematuria, and pain secondary to nephrolithiasis. He suffered from a non-verbal learning disorder associated with a developmental delay, was wheelchair-dependent, and had undergone extraction of 10 teeth due to biting and grinding his teeth. Due to his self-mutilating behavior, he wore braces on his arms and had surgery of his left thumb as a result of biting himself. Two years prior to presentation, the patient underwent a cystoscopy with bilateral ureteroscopy due to xanthine stones. He was stone free following the procedure. The patient’s mother denied a family history of kidney stones, thromboembolism and gout. At the age of 18 months, the boy underwent a test for organic acids in his urine which revealed highly elevated hypoxanthine without an elevation of xanthine and with a slight elevation of uracil. He was diagnosed clinically with Lesch-Nyhan syndrome at that time based on a triad of uric acid overproduction, neurologic dysfunction, and cognitive and behavioral disturbances.\nUric acid crystals were noted intermittently in the patient’s diaper which had increased significantly in the preceding days. He had been treated with the medication allopurinol since he was 2 years old. At the time of presentation, the dose of allopurinol was 200 mg administered once per day. He had never experienced gout. The patient was prescribed potassium citrate.\nA renal ultrasound demonstrated multiple calculi in both kidneys with the largest measuring 1.8 cm in the right kidney as well as echogenic material in the medullary pyramids bilaterally suggesting nephrocalcinosis (Fig. ). There were no masses, hydronephrosis, or hydroureter. A urinalysis revealed the following: specific gravity = 1.010; pH = 7.0; leukocyte esterase = 500; trace microscopic hematuria; and negative nitrite. The urine culture was negative. The uric acid level in blood was 2.9 mg/dL (Normal range: 2.5–8.5 mg/dL). Uric acid levels in blood collected in the previous 2 years were all in the normal range, specifically, 5.0 mg/dL, 2.6 mg/dL, and 3.2 mg/dL. There was no evidence of renal failure. The patient underwent six separate urinalyses between the ages of 9 and 12, all of which demonstrated a pH of either 6 or 7.\nA CT scan of the abdomen and pelvis demonstrated staghorn calculi in both kidneys, involving all calyces.\nThe patient underwent a right percutaneous nephrolithotomy (PCNL) 2 months after presentation. During the hospital stay, the patient was noted to be anemic and received 5 days of epogen and iron supplementation. He developed a thrombus in the right cephalic vein and was diagnosed with heterozygous Factor V Leiden with elevated homocysteine (16.2 umol/L [Normal range: 6.6–14.8 umol/L]). The methylene tetrahydrofolate reductase (MTHFR) and prothrombin genes were negative. Folic acid was initiated. The Factor V Leiden mutation was detected through a polymerase chain reaction (PCR) test that utilized microarray-based oligonucleotide hybridization and signal amplification to detect the Factor V Leiden mutation.\nSeven weeks later, the patient began experiencing aspiration of liquids, gastroesophageal reflux, and markedly delayed gastric emptying, necessitating a laparoscopic Nissen fundoplication with gastrostomy tube and open pyloroplasty. The patient continued to suffer from nephrolithiasis, with visualization of a left renal calculus greater than 2 cm. A CT scan demonstrated staghorn calculi in the left kidney (Fig. ). He underwent a left PCNL and left nephrostomy tube exchange 6 months after the right-sided procedure. A nephrogram revealed evidence of a filling defect in the lower pole calyx which may represent organizing blood clot or residual stone. Renal ultrasounds performed 6 and 9 months later demonstrated extensive bilateral nephrocalcinosis without hydronephrosis or hydroureter. There was no evidence of calculi in the collecting systems.\nThe calculi that were removed during the ureteroscopies, the ones that the patient had passed while seen as an outpatient, and those that were sent for analysis following the PCNL were all consistent with xanthine calculi. | [[12.0, 'year']] | M | {'14142409': 1, '8918725': 1, '4884382': 1, '16698380': 1, '11018746': 1, '4012980': 1, '8633391': 1, '27079129': 1, '16773422': 1, '11145936': 1, '4291947': 1, '18067674': 1, '19561505': 1, '15804753': 1, '17065067': 1, '9293873': 1, '31541411': 1, '15897349': 1, '3580782': 1, '8439471': 1, '3981718': 1, '11307586': 1, '12707252': 1, '17697859': 1, '11113762': 1, '6864903': 1, '21116184': 1, '30001695': 2} | {} |
1,193 | 6044070-1 | 30,001,711 | comm/PMC006xxxxxx/PMC6044070.xml | Detecting germline BAP1 mutations in patients with peritoneal mesothelioma: benefits to patient and family members | A 45-year-old female presented with bilateral upper-quadrant abdominal pain. She is a non-smoker and had no known exposure to asbestos. Family history was significant for peritoneal mesothelioma in two family members—a sister who developed peritoneal mesothelioma at age 29, and a first degree male cousin who developed peritoneal mesothelioma at age 45. Physical examination revealed tenderness in the upper quadrants. Computerized tomography of the abdomen revealed omental stranding in the upper-quadrants (Fig. ). Laparoscopy showed studding and multiple white plaques on the diaphragm and peritoneum with adhesions (Fig. a, b). Biopsies revealed epithelioid malignant mesothelioma (Fig. ). Germline DNA was extracted from a blood sample, DNA extracted and BAP1 testing was performed by polymerase chain reaction followed by Sanger sequencing, as described [, ]. Genetic testing revealed that our patient carried the following inactivating truncating germline BAP1 mutation: chr3.52406884A > G, c.604T > C, p.Trp202Arg. This mutation inactivates the catalytic domain of BAP1, preventing autodeubiquitylation of the BAP1 protein and nuclear translocation [].\nShe was treated with cytoreductive surgery, hyperthermic intraoperative peritoneal chemotherapy with cisplatin and doxorubicin, followed by adjuvant systemic chemotherapy, specifically six cycles of cisplatin and pemetrexed. She has been followed by clinical surveillance and utilizing diffusion weighted (DW)-MRI imaging every 6 months. She is doing well without any evidence of recurrence 24 months since treatment.\nThe identification of a germline BAP1 mutation in this young lady with peritoneal mesothelioma, led to meetings with family members in which we explained the potential advantages and possible disadvantages of genetic testing for BAP1 mutations, consistent with the guidelines of the Consensus report on mesothelioma []. Eight family members elected to get tested: 6 were found to carry the same germline BAP1 mutation. The patient pedigree is illustrated in Fig. . Among them, 2 are tumor free and 1 has well differentiated papillary mesothelioma. Three of the carriers have other BAP1 related malignancies. Those who had not inherited the mutation were reassured that they did not carry a higher risk of developing mesothelioma or other cancers compared to the general population at large. Those who were found to carry germline BAP1 mutations, were informed of the increased risk for mesothelioma and other BAP1-associated cancers, and advised to reduce sun-exposure, minimize radiation exposure, including diagnostic/therapeutic radiation for the increased risk of cancer because of impaired DNA repair and apoptosis, and to avoid trades that could lead to asbestos exposure. In addition two of them had elected to enroll in a screening program, consisting of weighted (DW)-MRI imaging every 12 months utilizing diffusion weighted (DW)-MRI imaging rather than CT scans to avoid radiation exposure. All carriers are also undergoing annual detailed retinal exam by a retina specialist. | [[45.0, 'year']] | F | {'23725699': 1, '25514803': 1, '26896281': 1, '25668121': 1, '27453164': 1, '28706907': 1, '25080371': 1, '21874003': 1, '26928227': 1, '22935333': 1, '23684012': 1, '21788493': 1, '32206571': 1, '25488749': 1, '25225168': 1, '11273069': 1, '26096145': 1, '32509926': 1, '28706906': 1, '30376426': 1, '23550303': 1, '28665402': 1, '21874000': 1, '30450291': 1, '32206572': 1, '34725624': 2, '28661472': 1, '32004714': 1, '27501770': 1, '17251920': 1, '25231345': 1, '26683624': 1, '32206577': 1, '24703950': 1, '29061454': 1, '17044196': 1, '24347639': 1, '28793149': 1, '27834213': 1, '28170043': 1, '28614305': 1, '25380601': 1, '26119930': 1, '25658628': 1, '32206569': 1, '33805973': 1, '24894717': 1, '24243779': 1, '10810369': 1, '21059199': 2, '23709298': 1, '26140217': 2, '30001711': 2} | {'4488956-1': 1, '2988810-1': 1, '2988810-2': 1, '2988810-3': 1, '2988810-4': 1, '2988810-5': 1, '2988810-6': 1, '2988810-7': 1, '2988810-8': 1, '8555483-1': 1} |
1,194 | 6044379-1 | 30,018,751 | comm/PMC006xxxxxx/PMC6044379.xml | Papillary thyroid microcarcinoma (Black Ink) | A 58-year-old Caucasian woman with no personal history of thyroid disease presented herself to perform a thyroid ultrasound examination.\nRoutine laboratory tests results were normal. BMI result was 33 kg/m2.\nA sister was previously diagnosed of papillary thyroid carcinoma.\nBidimensional ultrasonography (DUS 2) using high frequency probes (13 - 15 MHz) (Toshiba Aplio 500) highlighted a highly suspicious micro-focus of the left lobe, of radial shape with markedly hypoechoic echostructure, irregular margins, size 0.3 cm (classified TI-RADS 5, very high risk lesion ATA guidelines).\n(Figure -).\nADF test showed a clear flow through newly formed tortuous vessels at the periphery of the specific micro-focus (Figure , ).\nFNAC procedure was proposed to the patient and, after informed consent had been obtained, it was performed under ultrasound guidance (Figure ).\nCytologic examination of the slides (Papanicolaou stain) allowed recognition of malignancy with cytologic pattern very suspicious for papillary tumor (TIR5 cathegory according to Italian Consensus, cathegory VI according to The Bethesda System for Reporting Thyroid Cytopathology (Figure , ).\nThe patient became candidate for thyroidectomy and subsequently underwent a Total Extracapsular Thiroidectomy procedure (TT). Some enlarged lymph nodes (ENL) were evaluated ultrasonography previous to surgical exploration, and found and removed close to the left inferior thyroid pole at the time of thyroidectomy.\nHistological examination of the lesion showed a proliferation of well differentiated epithelial cells forming an purely papillary structure surrounded by blood vessels, with irregular borders, overtly infiltranting thyroid parenchyma.\nThe neoplasm showed no relationship to thyroid capsule and no vascular or lymphatic invasion. All the lymph nodes retrieved were devoid of metastatic cells (final TNM 8th eds. staging pT1, pN0) (Figure ).\nPatient underwent hormonal and tumor markers evaluation plus ultrasonographic evaluation of the neck after 3 months. Post operative remnants therapy with Radioiodine I-131 (RAI), was not administered inasmuch the tumor was very little, contained in the thyroid and lacked vascular invasion. | [[58.0, 'year']] | F | {'3652005': 1, '20824810': 1, '1940060': 1, '23266584': 1, '22270990': 1, '18578608': 1, '20189076': 1, '7647575': 1, '3686348': 1, '9036821': 1, '24664970': 1, '29051792': 2, '2914297': 1, '18287387': 1, '22319042': 1, '27732329': 1, '19224514': 1, '15300502': 1, '1182663': 1, '23745671': 1, '19888858': 1, '5819259': 1, '25913730': 1, '23600998': 1, '19294400': 1, '23242040': 1, '26191611': 1, '26745127': 1, '22972508': 1, '1000814': 1, '6073888': 1, '21876434': 1, '16478817': 1, '32365823': 2, '26020388': 1, '22496497': 1, '8551134': 1, '2302665': 1, '16896263': 1, '24789536': 1, '30018751': 2} | {'7277915-1': 1, '5634945-1': 1} |
1,195 | 6044477-1 | 30,027,005 | comm/PMC006xxxxxx/PMC6044477.xml | Stercoral Ulcer Not Always Indolent: A Rare Complication of Fecal Impaction | An 80-year-old male patient was admitted to the emergency ward with the complaints of abdominal distention, vomiting, and constipation for three days. On examination, the patient was dehydrated with stable vital signs. The abdomen was distended with generalised guarding and rigidity. The bowel sounds were not heard. On rectal examination, rectum was empty with minimal fecal staining and no palpable mass lesion. After initial resuscitation, imaging studies, including an ultrasound of the abdomen, were done. Chest X-ray showed air under the diaphragm (Figure ).\nAbdominal x-ray showed few air-fluid levels. Ultrasound showed distended bowel loops with significant free fluid in the peritoneal cavity. The patient was diagnosed to have a hollow viscus perforation with peritonitis. Routine blood investigations and blood cultures were done. Renal parameters were deranged suggesting pre-renal failure. Broad spectrum antibiotics were started in view of the high leukocyte counts. The patient was taken up for emergency laparotomy. Intraoperatively, approximately 500 mL fecal-contaminated peritoneal fluid was cleared. There was a 1 x 1 cm2 rent in the rectosigmoid junction (Figure ).\nProximal to the perforation, large bowel loops were distended. Bowel wall thickening was present in the rectosigmoid junction. Distal to the perforation, the rectum was found to be collapsed. The liver and the rest of the intestine were normal. Pelvic deposits/growths were not found. Primary closure of the rent in two layers was done after a thorough peritoneal wash. A diverting transverse colostomy was done after manual bowel decompression. The patient recovered after one week of intensive postoperative care. He was started on oral fluids on the tenth postoperative day and discharged after three weeks of hospital stay. The patient was followed up regularly, and the colostomy closure was done six weeks after the primary surgery. | [[80.0, 'year']] | M | {'5047522': 1, '34963813': 2, '28193645': 1, '12355222': 1, '1759759': 1, '22347716': 2, '15788592': 1, '20098066': 1, '20607025': 2, '16489660': 1, '30027005': 2} | {'2890920-1': 1, '3278635-1': 1, '8710845-1': 1} |
1,196 | 6044478-1 | 30,027,013 | comm/PMC006xxxxxx/PMC6044478.xml | Acquired Hemophilia Secondary to Soft-tissue Sarcoma: Case Report from a Latin American Hospital and Literature Review | A male, 52-year-old patient visited the Instituto Nacional de Cancerología (INC) in Bogotá, Colombia, because of the development of a mass in the back region. There had been progressive growth and multiple drainage attempts due to a suspected abscess at various referral sites without relief. He was given non-oncological surgical resection in another institution, and his pathology report was compatible with high-grade sarcoma with positive margins. Additionally, he referred to bilateral vision loss that had been evolving for one month. The initial clinical exam revealed a patient with normal vital signs who was disoriented with regard to space and time though awake and had poor communication with the examiner. He presented a hyperpigmented tumor measuring 10 x 8 cm in the back region, along with a scarred postsurgical lesion without inflammatory signs. The biopsy was reviewed by the INC Pathology Department, which indicated a high-grade sarcoma compatible with myxofibrosarcoma.\nHe was examined by the Ophthalmology Department, which considered bilateral retinal detachment probable. An abdominal computed tomography (CT) scan was performed, which showed a diffuse density alteration of the subcutaneous cellular tissue, without evidence of lesions, probably due to edema. A chest computed tomography (CT) scan with contrast showed an undefined heterogeneous mass dependent on the soft tissues in the posterior thorax wall, with infiltration signs in the muscular plane and the density alteration of the subcutaneous cellular tissue and skin (See Figure ). He was examined by clinical oncology, surgical oncology, and radiation oncology services, which indicated he was not a candidate for chemotherapy, surgical treatment, or radiotherapy because of the significant extension of the tumor into the upper and lower back, the inadequate response of these tumors to chemotherapy, and the patient’s poor Zubrod performance score.\nDuring inpatient care, the patient showed substantial clinical worsening characterized by the progressive deterioration of his consciousness state and persistent oral cavity bleeding. Laboratory tests were requested, which showed the prolongation of blood clotting time due to extended partial thromboplastin time (PTT), with the test value for prothrombin time (PT) being 11.9 seconds and that for PTT being 50.1 seconds (normal value until 28 seconds). The mixing study showed PTT fixing with a 50:50 normal plasma sample that lasted for the first and second hour, suggesting intrinsic pathway coagulation factor deficiency. Even though the mixing study was not suggestive of the presence of inhibitors, the medical history of the patient, the presence of an active neoplasm, and the recent beginning of bleeding created a clinical suspicion of acquired hemophilia. For these reasons, a new mixing study, VIII factor assay, and Bethesda assay to measure factor VIII inhibitors were requested. In addition, treatment with prednisone at 1 mg/kg/day and factor VIII at 1700 international unit (IU) every 12 hours was initiated. The results of these assays showed a basal PTT of 67 seconds, which with the 50:50 sample with normal plasma, was reduced to 38,2 seconds and maintained at around normal values two hours afterwards, with a value of 36,3 seconds. Factor VIII was measured at 4% and remained at this same value (4%) 10 minutes after the injection of intravenous factor VIII. The specific factor VIII inhibitor assay reported 1.6 Bethesda units. A diagnosis of acquired hemophilia was made based on the presence of hidden low-titer inhibitors in the mixing study.\nBased on the findings above, the persistence of oral cavity bleeding, and daily transfusion support in the form of two red blood cell units, IV treatment with an activated prothrombin complex like the factor eight (VIII) inhibitor bypassing activity (FEIBA) at a dose of 3500 units/12 h (50 U/kg every 12 hours) was initiated. During the patient’s evolution, hypofibrinogenemia development, the continuous elevation of D-dimer levels, and progressive thrombocytopenia suggested a diagnosis of disseminated intravascular coagulation (DIC). Because of the unresolved bleeding, it was decided to initiate a transfusion of cryoprecipitate and tranexamic acid oral rinse while augmenting the FEIBA dose. The patient developed a low response to the established treatment, along with more profound neurological impairment (central nervous system bleeding was ruled out) and the persistence of red blood cell, platelet, and cryoprecipitate transfusion requirements. Additionally, the patient had severe hyponatremia in the form of an electrolyte imbalance. Electrolyte correction was initiated, and because of the persistence of bleeding, oral cyclophosphamide was added to the immunosuppressive treatment, with the objective of eradicating the inhibitors. Seven days afterward, because of the lack of clinical response to management and the impossibility of providing specific antineoplastic treatment, with the agreement of the patient´s family, transfusion support was suspended, and palliative care was initiated. The patient died one week after. | [[52.0, 'year']] | M | {'19644360': 1, '25359571': 1, '22618709': 1, '30510943': 2, '22321904': 1, '22517903': 1, '26679396': 1, '22682137': 1, '24750687': 1, '25564260': 1, '20529258': 1, '22969696': 1, '19336751': 1, '25525118': 1, '22941793': 1, '20407848': 1, '25623166': 1, '20606415': 1, '17047148': 1, '21115138': 1, '28960809': 1, '30027013': 2} | {'6264993-1': 1, '6264993-2': 1} |
1,197 | 6044483-1 | 30,027,023 | comm/PMC006xxxxxx/PMC6044483.xml | Hemiplegic Migraine as the Initial Presentation of Biopsy Positive Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy | The patient was a 41-year-old gentleman with a past medical history of hepatitis C, hemiplegic migraine, and diverticulitis. He was well until 2011 when he first developed a severe headache followed by right-hand weakness and numbness. He was taken to the hospital where a diagnosis of hemipalegic migraine was made. Magnetic resonance imaging (MRI) revealed some white matter changes thought to be secondary to hemiplegic migraine at that time. He recovered complete neurological function with no residual deficits.\nOn this admission, the patient initially presented to the hospital with right-sided facial droop and weakness in the right lower extremity. The initial computed tomography (CT) scan of the brain was negative for acute bleed. An MRI of the brain was ordered because the patient continued to experience focal neurological deficits including right arm weakness, dysarthria, and decreased cognitive function on neurological examination and neuropsychiatric testing. At this point, it was thought that he had suffered an ischemic stroke, and the MRI showed multiple areas of restricted diffusion suggestive of embolic disease (Figure ). These findings called into question the diagnosis of hemiplegic migraine.\nThe patient received aspirin after ruling out active bleeding on non-contrast CT of the head. The search for an embolic source included a CT-angiograph of the neck which did not reveal carotid stenosis. He then had a transthoracic echocardiograph and a trans esophageal echocardiograph both of which were unremarkable. The differential diagnosis at that time included transient ischemic attack, ischemic stroke, brain tumors, central nervous system (CNS) infection, CNS vasculitis, inherited disorders including mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), CADASIL, and hereditary telangiectasia. A lumbar puncture was performed for possible meningitis. The cytology was negative and cerebrospinal fluid (CSF) analysis was not suggestive of meningitis, neurosyphilis, or Lyme disease. Lyme serology, the Venereal Disease Research Laboratory test (VDRL), and human immunodeficiency virus (HIV) blood tests were all negative. The CSF was also screened for possible multiple sclerosis, but no oligoclonal bands were detected. At this point, there was a concern for possible vasculitis as the cause of the patient's symptoms. Genetic testing for CADASIL was ordered, but the sample had to be sent out to a lab equipped to perform the genetic test and would take several weeks to return. He was placed on a five-day course of intravenous (IV) methylprednisolone which improved his neurological symptoms. Following completion of his steroid course, it was decided that an alternative to angiogram would be brain biopsy to look for CNS vasculitis. The patient and his family were eager to have an established diagnosis by the end of the hospital course, and the differential diagnosis included CNS vasculitis for which brain biopsy would provide valuable diagnostic information. Given the recent advances in imaging and neurosurgical techniques, the complication rate for routine biopsy has been reduced significantly. The patient elected to have the procedure performed. Rheumatology was consulted and an angiogram was recommended as well to rule out CNS vasculitis or vasospasm as a possible cause for the strokes. The patient had physical therapy and his motor and cognitive function gradually improved over the course of his hospital stay. The patient was accepted into a neurological rehabilitation facility and discharged on oral steroids. The results of the brain biopsy confirmed CADASIL after the patient was discharged to the rehabilitation facility. The results of the genetic tests for mutations in the NOTCH3 gene provided further confirmation with a sensitivity approaching 100%. | [[41.0, 'year']] | M | {'21038489': 1, '10356105': 1, '11245715': 1, '8485581': 1, '11807151': 1, '7908596': 1, '21458376': 1, '9710018': 1, '18384453': 1, '24840674': 1, '9818928': 1, '32430436': 1, '12023326': 1, '8878478': 1, '31226929': 1, '17645764': 1, '19098031': 1, '10210836': 1, '12601098': 1, '33126486': 1, '11439943': 1, '17620550': 1, '30027023': 2} | {} |
1,198 | 6044484-1 | 30,027,008 | comm/PMC006xxxxxx/PMC6044484.xml | Bilateral Earlobe Crease as a Marker of Premature Coronary Artery Disease | A 44-year-old male with past medical history of hypertension, was in his usual state of health until the morning of admission, when he was awakened by a substernal pressure-like chest pain associated with shortness of breath. He had no prior history of tobacco use, alcohol use, illicit drug use or family history of premature coronary artery disease. On admission, the patient was hemodynamically stable. Physical examination revealed an overweight male with bilateral earlobe creases (Figure ) and no other relevant findings.\nA 12-lead electrocardiogram (ECG) revealed 2 mm downsloping ST depressions in the inferolateral leads along with 1 mm ST elevation in aVR. Initial cardiac enzymes included a troponin of 0.44 ng/ml, with normal creatine kinase (CK) and creatine kinase-muscle/brain (CK-MB) levels. The patient was given aspirin and intravenous heparin in the emergency room in addition to morphine and sublingual nitroglycerin. He continued to have ongoing pain despite analgesics and nitrates, thus he was transferred to the cardiac catheterization lab where angiography revealed severe triple vessel disease (Figure ). The decision was made to proceed with coronary artery bypass surgery (CABG). A transthoracic echocardiogram (TEE) revealed hypokinesis of the inferior wall of the left ventricle and ejection fraction of 45%. | [[44.0, 'year']] | M | {'26868940': 1, '28331775': 1, '22335855': 1, '34466548': 1, '26131833': 1, '31641899': 1, '11791030': 1, '30027008': 2} | {} |
1,199 | 6044485-1 | 30,027,011 | comm/PMC006xxxxxx/PMC6044485.xml | Restless Genital Syndrome: Case Report of a Rare Disorder from Pakistan | A 44-year-old previously healthy Asian female reported to the neurology clinic with complaints of episodic, persistent, uncomfortable needle-like sensations in her genitalia extending to the anal area and the tip of the coccyx. She had been suffering from these symptoms for approximately 11 years. She had difficulty describing the actual nature of this phenomenon, which, according to her, was more of an irritation and discomfort than actual pain. The discomfort was aggravated during rest and periods of inactivity, particularly at night. It responded briefly to mefenamic acid tablets for four to five hours. She also reported the pain was worse before her monthly menstrual cycle but that sexual activity did not affect her discomfort. Sometimes, the discomfort was intense and would wake her from sleep causing severe discomfort and resulting in difficulty sitting; walking would relieve her symptoms partially. She was better in the morning, but the symptoms appeared again at end of the day. She had multiple gynecology and dermatology consults with no relief or clear diagnosis. She was prescribed antifungal creams, including fluconazole and ketoconazole, topical steroids (betamethasone and hydrocortisone), and mefenamic acid for pain relief. All failed to bring relief. She also consulted a homeopathy practitioner, who labeled her as suffering from a chronic skin disease and prescribed oral and topical medication. She took it for a few months but stopped it due to lack of relief. Ultimately, due to the distress and anguish associated with the condition, lack of response to various treatments, and social embarrassment, she stopped visiting doctors for some time. However, during a recent visit, the consulting gynecologist suspected it was not a dermatological or gynecological problem and referred her for a neurology consult.\nThere was no history of back pain, sensory symptoms in the legs, urinary incontinence, or neurological disorder, such as Parkinsonism or restless leg disorder. She had a stable and happy marital life of 22 years and had three children. She has used no substance of abuse or recreational drugs. Her neurological and gynecological examinations, as well as magnetic resonance imaging (MRI) of the spine, were normal (Figure ). Similarly, her biochemical profile (including complete blood count, urine analysis, serum urea, and creatinine) and an ultrasound examination of abdomen and pelvis were normal.\nConsidering her history of circadian rhythm variations in symptoms and aggravation on rest, RGS was considered the most likely cause of her symptoms. After a detailed discussion with the patient, she was prescribed 0.5 mg of ropinirole once at night. It was increased to twice daily after seven days, to which she responded well and reported a dramatic relief of symptoms after 14 days. The dose was increased to 1 mg in the morning and 0.5 mg at night, which led to further improvement. On her third follow-up after two months, she reported taking her medication regularly, which led to almost no episodes of discomfort, with exception of one or two times. The dose of ropinirole was maintained for one month. At the three-month follow-up, she reported only one episode of pain and itching, when she missed one dose of her medicine for a few days; otherwise, she had no complaints and reported no side effects of the medication. | [[44.0, 'year']] | F | {'27570347': 2, '20955316': 1, '23157369': 1, '23970620': 1, '11441520': 1, '19912500': 1, '25285600': 1, '19138358': 1, '30027011': 2} | {'4980903-1': 1} |
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