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pmc-6131739-1 | In October 2014, a 53 year old male Caucasian administration officer was referred to a tertiary renal outpatient clinic for assessment of nephrotic range proteinuria. On review, he described occasional ankle oedema over the past year but otherwise felt well. His past medical history was significant for type 2 diabetes mellitus which was treated in 2007 with 15 kg of weight loss (glycosylated haemoglobin < 6% since 2013). He denied symptoms of macrovascular or microvascular complications. The patient also had hypertension (diagnosed > 10 years prior to review), obesity (body mass index: 47.6 g/m2) and degenerative spine disease. His medications were irbesartan 300 mg once daily, hydrochlorothiazide 25 mg once daily, tramadol 50 mg three times a day and meloxicam 7.5 mg as needed. His family history was significant for type 1 diabetes mellitus in his father and paternal uncle. On examination, his blood pressure was 140/80 mmHg and heart sounds were dual with no murmurs. There was bipedal oedema up to the bottom third of his shins, his jugular venous pressure was not elevated and his chest was clear to auscultation.
Initial laboratory investigation demonstrated haemoglobin 149 g/L, white cell count 7.20 × 109/L, platelets 235 × 109/L, blood urea nitrogen 6.0 mmol/L, serum creatinine 70 μmol/L and serum albumin 32 g/L. Serum electrolytes, liver enzymes, calcium and phosphate were within normal range. Repeated urinalysis did not demonstrate any haematuria, leukocyturia or casts. Urine protein creatinine ratio was 662 mg/mmol (normal in August, 2012, Fig. ). Serum levels of antinuclear antibody, antineutrophil cytoplasmic antibody, immunoglobulin (Ig) A, cryoglobulins, complement factor C3 and C4 were normal. Human immunodeficiency virus screen, Hepatitis B antigen and hepatitis C antibodies were negative. Serum κ free light chains (FLC) was 18 mg/L (7–22), serum λ FLC was 60 mg/L (8–27) and κ/λ ratio was 0.30 (0.31–1.56). Repeated serum electrophoreses over three months showed persistent κ and λ IgG bands in trace amounts consistent with an inflammatory or reactive pattern. The repeated abnormal serum electrophoreses in the absence of inflammation (normal erythrocyte sedimentation rate and C-reactive protein levels) prompted referral to haematology for further assessment.
Bone marrow biopsy showed normocellular marrow with mild lymphocytosis but no morphological evidence of plasma cell myeloma. Flow cytometry identified a clonal B-cell population suggesting a low grade B-cell lymphoma. Staging computer tomography scans of his neck, chest, abdomen and pelvis found some borderline mesenteric lymphadenopathy (clinically stage 1).
The first renal biopsy demonstrated 4 glomeruli with minor mesangial hypercellularity but not expansion of the mesangial matrix. However, there was insufficient tissue to determine the cause of the patient’s glomerular disease. The second renal biopsy was performed a month later, yielding 11 glomeruli (Fig. ). There was mild mesangial expansion but no mesangial hypercellularity, endocapillary proliferation nor crescent formation. One glomerulus was globally sclerosed. Three glomeruli showed areas of segmental sclerosis and adhesion to Bowman’s capsule. The lesions of segmental sclerosis were not perihilar in location. There was mild tubular atrophy involving 5% of the cortex and there was mild arteriolar hyalinosis. Immunofluorescence examination showed mesangial reactivity for IgA (moderate intensity), IgM (trace) and both kappa and lambda light chains (weak intensity). There was no reactivity for IgG, C3 or C1q. Electron microscopy showed extensive epithelial foot process effacement with microvillous change. A few small electron-dense deposits were found in the mesangium but not in glomerular capillary walls. Together, these findings indicated IgAN with associated FSGS.
In June 2015, the patient began 6 cycles of chemotherapy with cyclophosphamide (day 1: 750 mg/m2), rituximab (day 1: 375 mg/m2) and prednisolone (days 1–5: 50 mg/day). The patient did not demonstrate any side effects or complications from treatment. At the end of chemotherapy, the patient was in complete remission haematologically and urine protein creatinine ratio had decreased substantially to 241 mg/mmol creatinine. Over the next 14 months, his urine protein creatinine ratio continued to fall. His serum creatinine was 104 μmol/L, serum albumin was 38 g/L and urine protein excretion was 14 mg/mmol at his most recent follow-up, 26 months post-chemotherapy. |
pmc-6131756-1 | A 60-year-old woman sought medical attention after a 2-month history of minor rectal bleeding and an anal nodule. On physical examination, a 3-cm mobile anterior ulcerative mass in the anal canal was palpable, beginning at 1 cm from the anal verge with no extension to the anorectal junction. No nodes were appreciated in the inguinal regions. A biopsy revealed an invasive squamous cell carcinoma, well-differentiated (Fig. ). Staging computerized tomography (CT) of the thorax/abdomen/pelvis did not show any lymphadenopathy or distant metastatic disease. Pelvic magnetic resonance imaging (MRI) demonstrated a 3-cm mass in the anal canal extending to the anorectal junction (Fig. and ). There was no pelvic or inguinal lymphadenopathy. Her laboratory investigations including HIV-1 and HIV-2 serology were negative. A recent Papanicolau smear of the cervix was reported to be negative for intraepithelial lesion or malignancy.
The patient had a past medical history remarkable for an in-situ cervical carcinoma treated with laser therapy over 20 years ago with no subsequent recurrence. She also underwent a right salpingo-oophorectomy for an endometriotic cyst of the right ovary 8 years prior to her anal canal cancer diagnosis. She is a life-time non-smoker and social alcohol drinker. There was no past history of any autoimmune disorders.
For her T2N0M0 anal cancer, the patient underwent a course of chemo-radiotherapy as per institutional protocol. The total dose was 54 Gy in 30 daily fractions to the primary and elective nodal irradiation consisted of 36 Gy in 20 fractions to the inguinal/femoral, mesorectal, presacral and external/internal iliac nodal regions. The radiation treatment was delivered using volumetric modulated arch therapy (VMAT) technique. The chemotherapy regimen consisted of mitomycin C (10 mg/m2 on day 1) and infusional 5-fluorouracil (1000 mg/m2/day for 4 days), given concurrently on week 1 and week 5 of radiation. The patient developed RTOG grade 3 skin and perineal reactions. She had an episode of fever without neutropenia managed by oral antibiotics. |
pmc-6131799-1 | The cell line was derived from a liver metastasis of colon cancer patient who was a 69-year-old woman in Jiangsu province hospital. 2014 the patient was carried out the surgery of colon cancer. In 2016 Dec, laboratory examination results showed CEA 64.4 ng/ml, CA199 24.4 U/ml, CA724 29.9 U/ml, NSE 32.4 ng/ml; Colonoscopy biopsy showed Sigmoid adenocarcinoma. MRI examination demonstrated a space-occupying lesion in the right lobe. The resected liver tumor was approximately 5 × 5 × 2.5 cm, pathological results showed hepatic adenocarcinoma, II–III stage. |
pmc-6131835-1 | Baby G arrived at ITL in 2015 when he was one-day-old weighing 1.9 kg. His mother had concealed her pregnancy. The baby boy was fed with DHM from the day of arrival. He was switched to infant formula in November and December 2015, when supplies of DHM ran out; however, he did not tolerate the artificial substitute well and began vomiting after feeds. When DHM was available again in January 2016, his condition improved, the vomiting stopped and he gained weight once again. He was adopted at six months weighing 7.4 kg (Fig. ). |
pmc-6131839-1 | A 75-year-old man from the South-West Region of Cameroon (an endemic zone for onchocerciasis) and of Bamileke ancestry presented to our clinic with skin lesions that had been evolving for over a year. The eruptions were first noticed a few hours after he took 12 mg of ivermectin (Mectizan) during mass drug administration (MDA) campaigns carried out every 3 months (as part of the public health strategy and in line with a recommendation from the World Health Organization’s African program for control of onchocerciasis [] and to fight against filariasis in endemic parts of Cameroon). The initial eruptions were dark, itchy discolorations with occasional burning and appeared as single isolated rashes on his groin, genital, and neck regions.
On further inquiry, he described similar symptoms in the past whenever he took ivermectin which disappeared after he stopped the drug. Further consumption of ivermectin (2 months prior to consultation) during the ensuing campaign resulted in worsening of the old lesions with development of multiple new lesions over his face, back, and extremities. His family and medical history were not remarkable for any previous drug or cross-reactivity reactions.
On physical examination, he looked well with vital signs within normal limits. There were multiple well-defined circular erythematous hyperpigmented plaque lesions of sizes ranging from 1 × 3 cm to 7 × 10 cm on his face, neck, groin area, and both extremities (Fig. ) occupying approximately two-thirds of his total body surface area (TBSA). Other systemic examinations were normal.
A laboratory work-up including full blood count, human immunodeficiency virus (HIV) serology, urine analysis, and biochemistry (liver and kidney function tests) were normal. Erythrocyte sedimentation rate was at 65 mm/hour after the first hour, while punch biopsy of the skin, and antinuclear antibodies (ANA)/antineutrophil cytoplasmic autoantibody (ANCA) were requested but were unavailable.
A working diagnosis of FDE was made based on clinical signs and patient history despite the lack of histopathological findings.
Discontinuation of ivermectin (plus counselling on avoidance of other possible culprits), a short course of systemic corticosteroids (prednisone 60 mg daily for a week), and orally administered antihistamines (hydroxyzine 75 mg daily) were employed as treatment modalities. Close patient follow-up revealed marked regression of lesions within a fortnight with residual hyperpigmentation (Figs. and ). |
pmc-6131847-1 | A 10-day-old male crossbred (Frisian x local indigenous) calf presented with a severely distended abdomen (Fig. ). Due to the distention the paralumbar fossa, especially on the left, was not visible. The calf was reluctant to suckle from the dam, unable to walk, exhibited rapid and shallow breathing, and had visibly congested mucus membranes. Percussion of the left abdomen revealed a drum-like gaseous sound. On auscultation of the left abdomen, a dull fluid sound was detected. The anamnesis indicated that the calf had been dribbling urine continuously, unable to defecate, or had irregularly voided very little, hard, and pasty feces. General physical examination revealed no esophageal obstruction, but the calf was weak and with an abnormal gait. The calf was suckling its dam twice in a 12 h interval (at morning and evening) and had not started feeding the hay/roughage/concentrate or the calf starter at the moment. The physiological parameters of the calf were as follows: Rectal temperature = 39.8 degree Centigrade (°C), Pulse =175 beats/minute, Respiration =60 breaths/minute.
Abomasal bloat and choke.
In order to release trapped gases and check the patency of the esophagus, a flexible stomach tube coated with mineral oil was inserted into the esophagus, and advanced down into the rumen. A fermented watery-like fluid accompanied by some clots of milk and gases was released from the rumen through the stomach tube. Procaine penicillin (Pen Aqueous; Zoetis Canada), 10 ml (ml), 10,000 international unit per milliliter (Iu/ml of solution) mixed with 0.25Liter (L) of mineral oil was administered orally for 3 days, while milk was withheld to reduce the microbial burden and coalescence of gas. An isotonic solution containing 0.9% Sodium Chloride (Jiangsu HFQ Bio-Technology Co., Ltd), 8.4% Sodium Bicarbonate (Vet One, Nova-Tech, Grand Island, USA) and 5% Dextrose in water 1000 ml injection (Addis Pharmaceutical factory) was administered intravenously (IV) at a rate of 100 ml/kilogram (kg) over 3–5 h for 2 days. Before administration of IV fluid, the calf was sedated using Xylazine hydrochloride, 20 mg/ml (xylazine® immunological LTD, Hyderabad, India) intramuscularly (IM). This was administered during every fluid therapy, and the calf was tied up with rope in a lateral recumbent position. The hair around the jugular groove of the neck was clipped and the area was cleaned and disinfected using diluted 70% Ethanol (Addis Pharmaceutical factory). The superficial jugular vein was catheterized using 20 Gage, 0.8 in. butterfly catheter (Unolok, Hindustan syringe, Medical device LTD Faridabad, India) and secured with adhesive tape around the neck.
After 3 days of treatment, bloat reoccurred. Treatment was initiated a second time by giving antibiotic pen strep (Pen & Strep@, 100 ml, York Vet, USA): 5 ml, (IM), every 24 h (q24hrs) for 2 days while the calf had been fastening. Additional supportive therapy of 40% glucose (100 ml/kg/day IV), isotonic saline solution (10 ml/kg/hrs IV) and a multivitamin (Multivitamin injection 100 ml, Norbrook Laboratories Limited, Ireland), was administered 10 ml IM once at a time (Stat.) during the time that the milk was withheld. After 2 days of treatment, the calf was allowed to suckle milk from the dam; however, the calf exhibited bloat again 5 h after milk consumption.
Ruminal fistulation (rumenostomy) was conducted to prevent recurrence according to a procedure described by Turner and Mcilwraith []. Before the surgical procedure milk was withheld from the calf overnight while IV fluids and glucose were administered at the dose rate explained above. The left paralumbar fossa was prepared by shaving the hair and washing skin aseptically using 7.5% povidone-iodine surgical scrub (Povidone-iodine cleansing solution, Wockhard LTD, Mumbai, India) while the calf was standing. A circular area of 6 cm (cm) in diameter just below the transverse process of the lumbar vertebrae was marked and infiltrated with local anesthetic, 2% Lidocaine (Zoetis Canada, Kirkland,Quebec), at the concentration of 20 mg per milliliter (mg/ml). Approximately a 2 cm diameter circular incision was made to remove the skin. After skin removal the abdominal muscles were dissected bluntly to expose the rumen. The rumen was grasped using sponge forceps and pulled to the exterior. The rumen wall was then tacked to the edge of the skin by four horizontal mattress sutures at “quarter hour” positions (12, 3, 6 and 9 o’clock). These sutures acted as stay sutures using a non-absorbable suture (Sofsilk™ 6–0 Black, Medtronic, USA). The rumen wall was incised carefully at one half centimeter from the wound margin/apposing skin. As the contents of the rumen came out during the procedure, we observed a high amount of milk that had entered into the rumen (Fig. ).
The calf was separated from the herd for 10 days to maintain close observation. Since rumenostomy is considered a clean contaminated surgery, we had to give parenteral antibiotic, penstrep, (Penstrep-400, Metaalweg, 85,804 CG Venray, Netherlands) 5 ml for 4 days, q24hrs IM to reduce the risks of peritonitis. A dexamethasone injection at 2 mg/ml (Sparhawk laboratories Inc., Lemexa, KS66215, USA) was given every eight hours (q8hrs) IM, and also served as an anti-inflammatory agent. Moreover, Deltamethrin 1% (w/v) pour-on ready-for-use formulation (Appropriate Applications Ltd., USA) at a dose rate of 10 ml per 100 kg body weight was used to prevent insect infestation and miyiasis. The surgical wound was examined and monitored every day until closure for any complications such as wound dehiscence or rumen attachment to the skin. Rumen contents leaking out onto the flank area and outer surgical site were cleaned by using antiseptic solution (Chlorhexidine) and clean towels. The rumen was repeatedly flushed through the fistula with 0.5–1 l of warm tap water adjusted to the calf’s body temperature. This flushing helped to prevent desiccation and was used for buffering purpose. The calf was allowed to suckle milk from its dam twice a day during the follow-up period. After 10 days post-operation, the calf was provided with some hay and fresh grasses to stimulate rumen function. Bloat resolved by the time the calf started solid feeds and the wound was closed surgically just after a week of feeding grasses and hay.
The calf was followed for 6 months after the procedure. Shortly after wound closure, the amount of milk that the calf was getting was reduced to encourage the intake of hay and grasses. During this time, bloat did not occur as it had been observed prior to surgery. Although ruminal contents spilled onto the flank post-operatively, this did not appear to upset the calf, and its general condition improved gradually. We recommended that the owner reintroduce the calf with the existing herd 6 months after the surgery, and advised the owner to inform us of any observable complications. We promised the owner that we would visit the calf at one year; however, the owner had sold the calf at 9 months of age to a beef farmer in another area of the country. |
pmc-6131849-1 | A 61-year-old woman with a past medical history of type II diabetes, breast cancer, and major depression presented to the emergency department after an intentional overdose with fluoxetine (139 tablets of 20 mg), risperidone (6 tablets of 1 mg), bromazepam (90 tablets of 3 mg), zolpidem (40 tablets of 10 mg), naproxen (20 tablets of 500 mg), and clemastine (5 tablets of 1 mg). Quantities were determined by counting the remaining pills in the blister packaging. Upon arrival, the patient was somnolent but able to open her eyes on request (E3M6V5). Vital signs showed a blood pressure of 146/57 mmHg, a regular heart rate of 55/min with strong peripheral pulsations, a respiratory rate of 16/min, oxygen saturation levels varying between 95 and 100% at room air, and a body temperature of 36.2 °C. Her husband suggested that the pills must have been ingested 3 to 8 h prior to hospital admission. Electrocardiography (ECG) showed a sinus rhythm of 61 beats per minute with a prolonged corrected QT interval (QTc) of 503 ms as shown in Fig. . During admission, all ECGs were performed using a GE MAC 5500 HD electrocardiograph. The tangent method was used in order to define the end of the T-wave in the lead with the longest QT interval. All measured QTc intervals were corrected for cardiac frequency using Bazett’s formula [].
Initial laboratory findings showed a microcytic anemia (Hb 5.4 mmol/l, MCV 77 fl) with low serum ferritin (6 μg/l), along with a mild leukocytosis of 11.2/nl. Serum electrolytes, as well as liver and kidney function, were normal (Na+ 135 mmol/l, K+ 4.3 mmol/l, creatinine 89 μmol/l, corrected calcium 2.37 mmol/l, PO43− 1.41 mmol/l, magnesium 0.83 mmol/l). Arterial blood gas analysis showed a base excess of − 4.9 mmol/l (pH 7.37, pCO2 4.6 kPa, pO2 12.1 kPa, HCO3− 20 mmol/l). A chest X-ray displayed no aspiration pneumonia nor other cardiopulmonary anomalies. The patient was admitted to the ICU for observation, where she received intravenous fluids along with 40 mg of pantoprazole for the prevention of peptic ulcer disease due to the substantial ingestion of naproxen. This was administered only once due to the risk of further QTc interval prolongation. Initially, our patient remained hemodynamically stable and showed improved neurological function. Twenty-two hours after hospital admission, a second ECG showed normalization of the QTc interval to 458 ms, suggesting peak serum levels of the ingested drugs had passed. After psychiatric evaluation had taken place, our patient had fallen to the floor in search of the restroom. On examination, both her muscle strength and coordination were slightly disturbed. Due to the overall severity of the intoxication and the persistence of neurological symptoms, it was decided to observe the patient for one more night at the ICU. Flumazenil was not administered since the patient was fully conscious and already in a monitored environment and due to the risk of adverse effects, especially with chronic benzodiazepine use [].
That night, 36 h after admission, our patient developed recurrent short episodes of Torsades de Pointes (TdP) with intermittent loss of cardiac output, as shown in Fig. . Two grams of magnesium sulphate was administered intravenously, after which sinus rhythm was restored. Four hours later, she had complete loss of circulation, after which cardiopulmonary resuscitation was initiated according to protocol. A 150-J biphasic shock was delivered using an automated external defibrillator (Zoll R-Series ALS). ECG findings consistent with TdP were again observed. Spontaneous circulation was restored after intravenous administration of magnesium sulphate. Our patient was awake and immediately able to maintain a patent airway following the incident. Serum levels of fluoxetine and risperidone and their metabolites were measured in retrospect, as shown in Table and Fig. , . Laboratory findings at the time of the incident showed a mild hypocalcaemia of 2.10 mmol/l with a hypermagnesaemia of 1.72 mmol/l/, most likely caused due to the prior administration of magnesium sulphate. No other electrolyte abnormalities were identified (Na+ 137 mmol/l, K+ 3.8 mmol/l, PO43− 1.24 mmol/l). Subsequent ECGs showed progressive prolongation of the QTc interval up to 565 ms, as shown in Fig. . The patient remained hemodynamically stable for the remainder of the night on continuous infusion of magnesium sulphate (1 g/h). The following day, echocardiography was performed in order to exclude structural cardiac defects, which showed normal heart dimensions as well as a normal left and right ventricular function. |
pmc-6131859-1 | In August 2010, a 57-year-old Chinese male presented with epistaxis and decreased hearing for 1 month. No additional symptoms, such as a neck mass, nasal obstruction, headache, diplopia or other cranial nerve palsies, were noted. The patient had no history of previous or synchronous tumours or any family history of cancer. Nasopharyngoscopy revealed a large exophytic tumour that was covered by smooth mucosa, which grew from the right posterolateral nasopharyngeal wall in the right posterior naris. Magnetic resonance imaging (MRI) scans of the nasopharynx and neck using gadolinium enhancement demonstrated a 2.0 × 1.5 × 2.0 cm well enhanced mass over the right posterior nasopharynx with right retropharyngeal node enlargement. The tumour extended across the right parapharyngeal space and infiltrated into the medial pterygoid muscle. In addition, skull base erosion was detected with right alar lamina involvement (Fig. ). Cervical lymph node metastasis was not observed. Hematologic, hepatic and renal function tests revealed no abnormalities. The patients underwent chest and abdomen computed tomography (CT) as well as a bone scintigram, and no distant metastasis was found. A biopsy of the nasopharynx was performed.
In the biopsy specimen, normal salivary tissue was not present. The tumours were ill demarcated without encapsulation. Tumour cells were arranged in nests and nodules. Two morphologic patterns of the tumour cells were observed. Some small round cells exhibited dark nuclei and scant cytoplasm. Other large cells contained round to oval pale nuclei and eosinophilic to amphophilic cytoplasm. In the central region of the tumour cell nests, large cells displayed a solid growth pattern. Small dark cells were clustered at the periphery of the tumour cell nests and appeared palisaded. Prominent nucleoli and mitosis can be observed, and an average of three mitotic figures were observed per 10 high-power fields (original magnification × 400).
In the immunohistochemical analysis, the tumour cells were immunoreactive with P63, vimentin, and cytokeratin (CK7 and CK14) antibodies and focally immunoreactive with a calponin antibody. This case of BCAC was not positive for smooth muscle actin or CD117. The proliferative index as demonstrated by Ki-67 was approximately 10%. Based on the immunohistochemistry results and the pathological findings, which included tumour islands with solid proliferation, basaloid-like cells containing large pale and small dark cells, an infiltrative margin, cellular and nuclear pleomorphism, and prominent mitosis, the patient was diagnosed with a solid-type minor salivary gland BCAC (Fig. ).
Based on the 2002 American Joint Committee on Cancer (AJCC) Tumor, Node, Metastasis (TNM) staging system [], the tumour was classified as stage III (T3N0M0).
In our case, the patient received intensity-modulated radiation therapy (IMRT) with 6 MV X-rays. The delineation of the gross tumour volume (GTV) was based on the primary tumour volume determined from the physical and imaging examinations. The clinical target volume (CTV) was defined as the whole nasopharyngeal cavity, the clivus, the skull base, the pterygoid plates, the parapharyngeal space, the sphenoid sinus, the posterior one-third of the nasal cavity, the maxillary sinus, and the drainage of the upper neck (levels II, III, and Va. A total dose of 70.4 Gy/32 F/6.2 W was administered based on the planning target volume (PTVg) (GTV with 0.5 cm margin). The PTV60 was defined as 60 Gy/30 F (CTV with 0.5 cm margin) (Fig. ). After radiotherapy, MRI and nasopharyngoscopy revealed complete disappearance of the tumour (Fig. ). The patient was followed up every 3 months for the first 2 years, every 6 months for another 3 years, and then every 12 months. A follow-up at 72 months did not detect any evidence of disease recurrence. The patient developed moderate mucositis as an acute adverse event. However, he did not exhibit any grade 3/4 late adverse events, such as xerostomia, dysgeusia, or hearing impairment. |
pmc-6131875-1 | A 62-year-old man with coronary artery disease underwent four-vessel CABG including left internal thoracic artery (ITA) to left anterior descending artery. The patient was discharged on postoperative day (POD) 6 after an uneventful postoperative course with low chest tube output and trace pleural effusions.
On POD 13, the patient was readmitted after four days of moderate chest pain and exertional dyspnea. Diminished respiratory sounds were noted over the full left lung field. Chest radiograph confirmed a large left pleural effusion (Fig. ). A left pigtail catheter drained 2.3 l of milky fluid with a triglyceride level of 1604 mg/dL, diagnosing chylothorax.
Conservative management was attempted with continued catheter drainage and initiation of a low-fat diet with medium-chain triglycerides on POD 14. Drainage gradually decreased over the next three days to < 100 ml/day, but by POD 18, drainage increased to 350 mL/day that continued for two days despite attempts to readjust the catheter position. On POD 20, the pigtail catheter was replaced with a chest tube, and the patient was transitioned to total parenteral nutrition (TPN) and octreotide while nil per os, but chest tube drainage persisted at 200–360 ml/day. On POD 28, the patient noted new, bilateral yellow toenail discoloration (Fig. ). The yellow nail discoloration was not associated with any disfiguring features such as nail thickening or separation from nail bed. No lymphedema was found. Presence of yellow nails and chylothorax resulted in diagnosis of YNS.
Lymphangiogram on POD 32 noted well-opacified pelvic lymphatic channels, but cisterna chyli and thoracic duct were not identified (Fig. ), precluding embolization. Tiny droplets of lipiodol were present in the left pleural space, but the leak location could not be identified.
On POD 33, thoracoscopic left pleurodesis was performed using aerosolized talc (4 g). Prior to talc administration, half-and-half (300 mL) was administered via nasogastric tube, but no chyle leak was found on thoracoscopic examination after two hours. Since lymphangiogram did not identify a thoracic duct, thoracic duct ligation was not attempted. Five days after pleurodesis, since chest tube output remained high (> 200–300 ml/day), doxycycline pleurodesis was performed via existing chest tube. Output subsequently decreased, allowing transition to low-fat diet seven days later and discharge home two days after that.
At 2- and 8-week follow-ups, chest radiograph showed trace pleural effusion with no recurrence of chylothorax. Although still on a low-fat diet at both visits, the patient was gradually increasing fat intake. Yellow toenail discoloration had resolved by the 8-week follow-up. |
pmc-6132087-1 | The patient was a 2-year-old boy from Russian republic of Ingushetia. Both parents and younger sibling (female) were asymptomatic. Family history is not burdened. Parents may be related, as the mother of the child and the grandmother (father's line) are from neighboring villages of Malgobek region of Ingushetia.
The patient is the first child of healthy parents. Pregnancy proceeded against the background of gestosis in the first and second trimester, threats of interruption for a period of 12 weeks, anemia, and lack of hydration. The child was born as a result of fast unassisted childbirth. His birth weight was 3570 g and height was 55 cm. Apgar scores were 6/7. Early motor development slightly delays: He attained head holding at 3 months, sitting without support at 6 months, by the year the boy got up and walked with support. From the age of 8 months, the baby had a weight loss of 2 kg for 2 months with episodes of repeated vomiting. At the age of 1 year 2 months against a background of acute respiratory viral infection, there was a regress in development—the boy stopped walking. Subsequently, rachitic skeletal changes, an increase in the volume of the abdomen appeared, and a loss of motor skills progressed. Magnetic resonance imaging (MRI) of brain revealed a mildly expressed leukopathy in white matter of parietal lobes. Proteinuria and glucosuria appeared at the age of 1 year 8 months.
At the age of 2 years 2 months, he was admitted to Russian children's clinical hospital with psychomotor retardation and treatment resistance. On physical examination, he was observed to have a pronounced delay in psychomotor development (he did not get up, walk, crawl; he could only keep his head and turn over). Neurological examination revealed muscular hypotrophy, tendency to hypotension in axial musculature, and uniform decrease in tendon reflexes from hands and feet. MRI of brain revealed subatrophic changes, moderate expansion of the lateral ventricles within the subatrophy with compensated liquorodynamics, and the presence of hypomyelinization zones in the periventricular white matter of the posterolateral regions.
Clinical exome sequencing was carried out by Genotek Ltd. Genomic DNA from peripheral blood sample was extracted using QIAamp DNA Mini Kit (Qiagen, Hilden, Germany). DNA libraries were prepared using NEBNext Ultra DNA Library Prep Kit for Illumina (New England Biolabs, Ipswich, Massachusetts, USA) with adapters for sequencing on Illumina platform. Double barcoding was performed by PCR with NEBNext Multiplex Oligos for Illumina kit. Quality control of DNA libraries was carried out using Bioanalyzer 2100 (Agilent Technologies, Santa Clara, California, USA). We used SureSelect XT2 (Agilent Technologies, Santa Clara, California, USA) for target enrichment. Enriched samples were sequenced on Illumina HiSeq 2500 using pair-end 100 base pairs reads. After sequencing, we trimmed 3′-nucleotides with read quality below 10 using Cutadapt. Raw reads were aligned to reference genome hg19 (GRCh37.p13) using BWA MEM. Deduplication of reads was carried out using SAMtools rmdup. FastQC was used for data quality control. We called short variants using GATK HaplotypeCaller according to GATK Best Practices DNA-seq. The effect of each mutation was assessed using snpEff. To assess pathogenicity and conservatism, the data were extracted from the dbNSFP, ClinVar, OMIM database, and HGMD, as well as using the SIFT and PolyPhen-2 utilities to predict pathogenicity of the mutation. Information on the frequency of mutations was taken from 1000 Genomes project, ExAC, and Genotek frequency data. Description of mutations and their pathogenicity was predicted according to the Standards and Guidelines developed by ACMG (American College of Medical Genetics and Genomics), AMP (Association for Molecular Pathology), and CAP (College of American Pathologists). Copy number alterations were determined using CNVkit. CTNS variant identified by exome sequencing was confirmed by Sanger sequencing of patient, parental, and sibling DNA samples.
After exome sequencing, on admission in Veltischchev Research and Clinical Institute for Pediatrics, patient was 9 kg in weight and 80 cm in height, with low weight-for-height proportion and disharmonious physical development. Deficiency of subcutaneous fat was noted. Attention was paid to rachitic changes in skeleton: expansion of metaphyses, keel-like deformation of thorax, rachitic “rosary,” varus deformity of lower limbs. Hepatosplenomegaly was noted: liver and spleen protruded from under edge of the costal arch by 2.5 cm. Clinical signs include polyuria and polydipsia (consumed liquid per day 1300 mL, daily diuresis—1300 mL). Blood tests showed mild anemia, hypokalemia, decrease in uric acid, increase in alkaline phosphatase activity, hypophosphatemia, increase in triglycerides, and creatinine level at the lower limit of the norm. The study of equilibrium of acids and bases of blood revealed metabolic acidosis. Test of thyroid gland hormonal profile showed subclinical hypothyroidism. Level of cystine in leukocytes was normal. Glomerular filtration rate (GFR) (97 mL/min) was within the normal range (80-120 mL/min). Urine test showed phosphaturia, glucosuria, and low molecular weight proteinuria. Cystine was detected in urine test. Ultrasound examination showed hepatosplenomegaly, multiple foci of increased echogenicity in liver, nephromegaly with thickening, and diffuse changes in renal parenchyma. Radiologic examination revealed pronounced osteoporosis, lagging behind bone age. Ophthalmoscopy revealed cystine crystals in the cornea. Thus, the child has signs of infantile form of nephrotic cystinosis with preserved kidney function.
The patient was commenced on cysteamine (Cystagon), initially 50 mg orally four times daily, to increase gradually to 100 mg four times daily (0.8 g/m2/d). Also he was commenced on ophthalmic solution Cystadrops (one drop six times daily) and potassium, phosphorus, vitamin D, energotropic agents. After 6-month therapy, the patient's condition remains stable and serious with slight positive dynamics. The child gained 1 kg in weight and grew by 0.5 cm. Polydipsia/polyuria decreased to 1000-1200 mL. Hemoglobin and red blood cell levels, and thyroid-stimulating hormone and T4 levels were normalized. Minimal phenomena of metabolic acidosis persist. Also, filtration function of the kidneys was preserved, and no signs of nephrocalcinosis were detected.
At the last examination, patient had elevated cystine level in the granulocytes (2.32 nmol) and nephrocalcinosis of both kidneys. Cysteamine dose was increased to 1.25 g/m2/d. |
pmc-6132088-1 | A 42-year-old healthy man, with a previous episode of food impaction and increased number of eosinophils in esophageal biopsies taken during an index upper endoscopy 6 weeks earlier, underwent outpatient control gastroscopy after receiving proton pump inhibitor treatment. The upper endoscopy was performed under conscious sedation with midazolam, with a standard video endoscope and the air pressure setting on the video processor at “medium.” Biopsies from distal, middle, and proximal esophagus were taken according to standard protocol. At the end of the 7-minute-long procedure, just after biopsies from proximal esophagus was taken, the patient's heart rate decreased to 46, the oxygen saturation decreased to 90%, and he developed generalized tonic-clonic seizures. The endoscope was rapidly withdrawn, and the oxygen saturation and heart rate normalized spontaneously. However, the patient was unresponsive despite reversal with flumazenil. While hemodynamically and respiratory stable, he remained unresponsive, Glasgow Coma Scale (GCS 3), with pupils midsize, equal, and reactive to light, and a negative Babinski sign.
An immediate computed tomography (CT) scan 30 minutes postinitial symptoms revealed massive air embolism in the right hemisphere. (Figure A). The radiologists first suspected infarctions, which was later modified. A complementing CT angiography (CT-AI) was performed 70 minutes after the initial CT scan to rule out differential diagnoses, and the delay was due to recurrent seizure and intubation. The most striking finding was complete resolution of air in the vessels. (Figure B).
Simultaneously with the CT-AI, a CT of the thorax was performed showing air around the whole length of the esophagus and small amounts of free air in the mediastinum behind the left atrium. (Figure ).
The neurologist on call contacted the hyperbaric unit, and the patient was transported to the hyperbaric unit, arriving three hours after initial symptoms. HBOT with United States Navy (USN) (Table 6 (Figure A)) was commenced immediately. The patient remained completely unresponsive during the initial 20 minutes of compression despite a confirmed gas embolism, and to maximize the therapeutic effect, the treatment table was extended to 8 hours and 5 minutes. (Figure B). After initial HBOT, the patient underwent a second CT scan, 12 hours after the initial CT, which to our surprise showed normalization, with only slight reduced attenuation in the right parietal area (Figure A). As the patient remained unconscious and there was a concern of much more damage to the brain than evident on the CT, a magnetic resonance imaging (MRI) was performed. The indication for early MRI performed 14 hours after air embolism was to make an estimation of putative ischemic lesions with diffusion weighted imaging.
Diffusion weighted MRI indicated cytotoxic edema, predominantly in the areas previously showing the greatest amount of air embolism on the initial CT scan (Figure B). The findings should be interpreted with caution and could be partly reversible.
A wake-up examination was performed, but since the patient remained GCS 3, neurosurgical monitoring was commenced with intracranial pressure (ICP) monitoring, microdialysis, and continuous electroencephalography (EEG). The patient was continuously monitored and received additional daily HBOT 2.8 ATA, 113 minutes for 4 days. ICP was stable until day 5, but then, ICP instability and suspicion of status epilepticus required treatment with pentothal at burst suppression until day 10. As the patient presented with seizures, levetiracetam was prescribed with an initial dose of 1000 mg 12 hourly. Levetiracetam concentration was checked and dose increased to 1250 mg 12 hourly due to low serum concentrations and suspected epileptic activity on EEG. After 2 weeks, the patient had no seizures, and during the rehabilitation phase, levetiracetam was discontinued.
A second MRI was performed after 1 week showing progression with severe swelling bilaterally, but most pronounced in the white matter of the right hemisphere (Figure ). During the hospital stay of 3 weeks, the patient was examined with extensive radiology. A series of four brain CT scans, one CT angiography of the brain and neck arteries, five CT of the thorax/esophagus/pulmonary arteries, and three MRI scans of the brain was performed together with a few chest X-rays and ultrasounds. The initial CT finding with small artery and capillary air mimicking infarcts developed into large areas of reversible edema with swelling, and finally limited cortical infarcts with laminar necrosis and gliosis in the white matter.
Despite initial poor neurological presentation, the patient underwent a remarkably good recovery with a favorable outcome. ICP improved after 9 days; and pentothal infusion was stopped on day 11; neurology improved slowly; and on day 13, he was responsive and obeyed commands but with left hemiplegia. He was extubated on day 15 and then continuously improved until discharge from neurorehabilitation 3 months after the insult. The third and final MRI was performed after 3 weeks, when the patient was fully awake with a GCS score of 14. The finding was resolution of swelling and regression of edema (Figure ). At discharge, he displayed only mild disability, slight neglect, and slight fine motor disability in the left hand. Eight months after the incident, he was back to work with minimal left spatial disability. |
pmc-6132093-1 | A 48-year-old woman was diagnosed with acute myeloid leukemia (AML) in 2013,who fortunately achieved a complete remission of bone marrow after two courses of chemotherapy (IA regimen: idarubicin hydrochloride + cytarabine). Allogeneic HSCT deriving from her HLA-identical brother was performed in February 2014 after a reduced intensity conditioning regimen (Ara-c/Bu/Cy/Me-CCNU). Graft-versus-host disease prophylaxis included cyclosporine(50 mg BID, and then gradually reduced the dose to 25 mg BID)and dexamethasone (5 mg QD). Ten months later, she began to complain limb weakness and mild dyspnea after walking. No myalgia, skin rash, or obvious dysphagia was documented. She stopped taking cyclosporine in May 2015. The muscle weakness and dyspnea were significantly aggravated after a fever in July 2015. The proximal muscles [Medical Research Council (MRC)graded 3] were more severely affected than the distal ones(MRC graded 4). Obvious muscle atrophy was evident on the proximal muscles. Deep tendon reflexes were slightly decreased. The somatic sensations were normal.
Blood analyses disclosed that the levels of creatine kinase (CK), lactate dehydrogenase (LDH),aspartate aminotransferase (AST), alanine aminotransferase (ALT), and myoglobin were elevated. C-reactive protein (CRP), blood routines, and thyroid glands were in normal ranges and the antinuclear antibodies, and myositis-specific autoantibodies were not detected (Table ). Electromyography (EMG) displayed the myopathic changes. Magnetic resonance imaging (MRI) showed areas of abnormally high signal intensity in the muscles of the left arm on fat-suppressed T2-weighed image, especially the biceps, deltoids, triceps, and subscapular muscles (Figure A,B), suggesting muscle inflammation and edema.
Pathological study of the biopsy specimens from the left biceps demonstrated prominent inflammatory cell infiltration with degenerated and necrotic myofibers. Immunohistochemical staining of the sections revealed that the infiltrating lymphocytes were predominantly T cells (mainly CD8+ T cells with a few CD4+ cells), while B lymphocytes were scarce (no CD20+ cells). Major histocompatibility complex class I (MHC-1) was upregulated in some myofibers. In addition, the membrane attack complex (MAC) was deposited in the necrotic myofibers (Figure ).
Based on the clinical presentation and the pathologic findings on the biopsied muscle, the polymyositis after allo-HSCT was considered. The patient was initially treated with an increased dosages of prednisone and cyclosporin A (CsA), but no significant improvement was achieved. Thus, rituximab (100 mg) plus a low dose of intravenous immunoglobulin (IVIG, 5-10 g) weekly was applied for consecutive 6 weeks, the patient finally achieved a remarkable improvement of her symptoms with a normal CK level. Until now, the patient has been followed up for one and a half years, and she remained symptom-free on oral prednisone and CsA. |
pmc-6132093-2 | A 31-year-old woman was diagnosed with AML 20 months ago. A first complete remission of AML was achieved with two courses of chemotherapy (DA regimen: pirarubicin + cytarabine; cytarabine + methylprednisolone). Fifteen months ago, she received allo-HSCT after a reduced intensity conditioning regimen with IDA/Bu/Fu/Me-CCNU. The postoperative regimen was consisted of cyclosporine (25 mg BID) and methylprednisolone (40 mg QD and then gradually reduced the dose to 20 mg QD) for GVHD prophylaxis. She was hospitalized because she had limb weakness and myalgia for 5 months. Five months ago, she felt myalgia in both the lower limbs (MRC graded 4) and later the upper limbs (MRC graded 4-). The muscles on the four extremities were generally tender and slight atrophic. Deep tendon reflexes were slightly decreased. The sensation was normal.
Laboratory tests showed the normal ranges of CK and myoglobin levels. The myositis-specific autoantibodies were not positively detected. The antinuclear antibody (ANA) titer was weakly positive at 1:320 (Table ). MRI showed high intensities in fat-suppressed T2-weighed image in the supraspinatus, scapular muscles, arm muscle group, as well as the leg muscle group and fascia (Figure ,C,D). EMG detected myopathic changes. Muscle biopsy of the quadriceps femoris revealed that the degeneration and necrosis of myofibers were evident, with a large number of inflammatory cell infiltrates in the endomysium, similar to those described in the biopsied muscle from the case one.
The therapy was shifted from the previous regimen consisting of oral prednisone and CsA to tacrolimus (3 mg QD) combined with oral methylprednisolone (40 mg QD). The symptoms began to relieve in 2 weeks and had disappeared at about 3 months after the new regimen applied. She had been followed up for over 1 year and was in a good condition on the daily dosage of tacrolimus at 3 mg with methylprednisolone at 12 mg. |
pmc-6132095-1 | A 22-year-old woman was admitted to our hospital with a two-day history of intermittent epigastric pain and nausea without any evidence of trauma. Her past medical history showed an episode of hypertriglyceridemia 2 years before admission. One of her cousins had a history of acute pancreatitis, but the details were unclear. She reported that she did not consume alcohol. She had not been taking any drugs, dietary supplements, or herbal medicines. She had a normal constitution (height, 159 cm; weight, 58.8 kg; BMI, 23.26). On examination, she had severe epigastric pain with symptoms of mild tetany. Her blood pressure was 116/80 mm Hg, her heart rate was 72 beats/min, and her body temperature was 36.5°C. Her blood test results (Table ) were as follows: amylase (AMY), 230 U/L; aspartate aminotransferase (AST), 36 U/L; alanine aminotransferase (ALT), 46 U/L; total cholesterol (T-Cho), 1225 mg/dL; triglyceride (TG), 8595 mg/dL, low-density lipoprotein cholesterol (LDL-C), 58.5 mg/dL; high-density lipoprotein cholesterol (HDL-C), 19.5 mg/dL; and serum calcium (Ca), 7.39 mg/dL. Abdominal enhanced CT revealed swelling of the pancreas with surrounding fat stranding and fluid accumulation that resulted in the thickening of the left renal fascia (Figure A). Furthermore, plain abdominal CT revealed diffuse and large low-density areas in the liver, suggestive of moderate to severe fatty liver (Figure B). No stones, tumors, or congenital anomalies were found in the bile duct or pancreatic duct by magnetic resonance cholangiopancreatography. Based on these findings, she was diagnosed with severe acute pancreatitis and treatment was initiated with gabexate mesilate (2000 mg/d) and meropenem (1000 mg/d) under fasting conditions. On day 15, her CRP levels and serum amylase levels normalized and she was observed to have recovered from pancreatitis; thus, the oral intake was started under calorie and fat restriction. The patient had an uneventful clinical course and was discharged on day 24. The day before discharge, her LPL and HTGL levels were examined using postheparin plasma (Table ). Her LPL activity was lower than the minimal level of the normal range, whereas her LPL mass was almost at the minimal level of the normal range. Similarly, although the HTGL mass was not measured in this study, her HTGL activity was lower than the minimal level of the normal range. After receiving informed consent from the patient and her parents, we performed a genetic analysis to search for an LPL gene mutation. The DNA fragments encoding each of the nine exons of the LPL gene were amplified by a polymerase chain reaction (PCR) and sequenced as previously described., The patient had no specific mutations in exons 1-9 of the LPL gene. After discharge, bezafibrate (400 mg/d) was started to treat hypertriglyceridemia, and her TG level gradually declined. However, her transaminase level did not normalize. Liver biopsy on day 1008 after the 1st admission showed moderate fibrosis (Figure A), and hepatic steatosis and inflammation with hepatocyte ballooning, consistent with a diagnosis of NASH (Non-alcoholic fatty liver disease activity score, 3; Matteoni classification, type 4; Brunt classification, Grade 1/Stage 2) (Figure B). At that point, her serum Wisteria floribunda agglutinin-positive Mac-2-binding protein (WFA+-M2BP) level was 2.33 COI (normal; 0-0.99). Finally, the patient was treated with fenofibrate (160 mg/d) and omega-3 fatty acid (2000 mg/d). At the time of this report, she is doing well with normal TG and transaminase levels. |
pmc-6132096-1 | This 32-year-old woman presented to Mutoyi Hospital (Burundi) outpatient clinic complaining of persistent abdominal pain and purulent PV discharge. She had undergone a cesarean section in another hospital 3 months before. To that date, the Pfannenstiel incision had been allegedly resutured twice. Unfortunately, as often in these settings, no medical records of the previous admissions were available.
She presented to our attention afebrile and tachycardic (95), otherwise stable with no rigor. Clinical examination revealed a soft abdomen, tender to palpation in the mesogastric region but without signs of peritonism, and with a 4 × 3 cm open wound in the suprapubic area (Fig. ).
The blood tests available showed an increased WCC of 14 and Hb of 9 mg/dL, to consider normal in these settings.
The US scan revealed no intra-abdominal fluid, but some thick tissue (possible a solid viscous) underneath the wound.
On the first day after admission, she delivered from that same wound what we understood could only be a held infected placenta, measuring >15 × 10 × 2 cm (Fig. ).
As we realized the uterus was communicating directly and largely with the outside, we opted to perform an emergency laparotomy.
The decision was taken to treat the uterocutaneous fistula with a hysterectomy, as the chronically inflamed uterine tissue was unsuitable for repair without tension and would have exposed the patient to a high risk of ruptured uterus in the future.
The patient subsequently underwent a successful emergency hysterectomy and was discharged 10 days later in the absence of complications. |
pmc-6132107-1 | A 65-year-old gentleman presented to the emergency department (ED) at night with a history of acute-onset atraumatic bilateral knee pain and swelling. His past medical history includes bicuspid aortic valve replacement and subsequent revision of his aortic valve replacement 4 weeks prior to the onset of knee pains. He was diagnosed with sternal surgical wound infection (clinically erythematous skin with possible discharge) 4 days prior to hospital attendance with associated left knee pain and swelling. A diagnosis of wound infection with reactive arthritis was made by his general practitioner (GP), and the patient was commenced on a course of clarithromycin due to penicillin allergy. The wound infection appeared to be responsive to oral antibiotic treatment. However, his left knee symptoms gradually worsened and greatly affected his mobility; 12 hours before ED attendance, he developed acute right knee pain and swelling with associated generalized fatigue. Clinical observation raised concerns of sepsis with a pyrexia of 39°C and tachycardia of 100 beats per minute. Both of his knees were diffusely swollen, warm, and extremely tender to palpate. He did not tolerate any range of movement of his knees. Hip and ankle joints were normal, and there was no appearance of cellulitis. He was also reviewed by the medical team, and other common sources of sepsis including chest or urine infections were ruled out.
On admission, his blood test showed raised inflammatory markers, with white blood cells of 15.9 × 109 L and C-reactive protein (CRP) of 288 mg/L. His knee X-rays revealed no bony pathology otherwise. Aspiration of both knees was performed under sterile technique on the ward prior to commencing antibiotics—cloudy thick pus was drained from both knees (Figures and ). Urgent microscopy and gram stain did not show any organisms.
The patient's initial presentation to his general practice with acute unilateral atraumatic knee pain and swelling raised suspicion of reactive arthritis secondary to a wound infection. His subsequent presentation to the emergency department with bilateral knee symptoms and inability to mobilize associated with systemic symptoms including pyrexia raised the clinical suspicion of bilateral SA. Raised white blood cell and C-reactive protein suggest SA as the most likely diagnosis, although both knee aspirations were negative.
This patient was started on combination of antibiotics treatment according to the local hospital guidelines. He then proceeded to have bilateral knee arthroscopies and washout which revealed more pus collections and active synovitis (Figure ). Synovial fluid samples were obtained and sent for culture. He was treated with a total 3-week course of intravenous antibiotics and underwent a total of four knee joint washouts. A total of six synovial fluids and two intra-articular soft tissues were sent for culture—none of which grew any active microorganisms. The possibility of infective endocarditis was also considered in view of the recent revision of his prosthetic valve. He had a total of four blood cultures collected at separate times, all of which were negative. Inpatient echocardiogram and subsequent transesophageal echocardiogram (TOE) showed no vegetation on his prosthetic heart valve.
After repeated washouts and a prolonged course of IV antibiotics, the patient demonstrated full clinical recovery also evident with improvements in his inflammatory markers. He was then discharged with a further 2-week course of oral antibiotics along with an outpatient clinic follow-up. He underwent subsequent reviews by a cardiothoracic surgeon and cardiologist in view of his recent prosthetic heart valve revision surgery which was satisfactory. |
pmc-6132108-1 | A four-month-old male infant was referred to an emergency department from his pediatrician's office with fever, irritability, and bullous skin lesions covering a third of the body surface area without mucosal involvement. In the emergency department, he decompensated acutely and developed cardiac arrest requiring cardiopulmonary resuscitation and hemodynamic support with dobutamine, dopamine, milrinone, and norepinephrine. He required mechanical ventilation for acute respiratory distress syndrome and right ventricular heart failure with evidence of pulmonary hypertension, and emergent hemodialysis for acute kidney failure. Due to continued concerns for disseminated infection, he received vancomycin, ceftriaxone, acyclovir, and clindamycin. He developed disseminated intravascular coagulopathy and received multiple transfusions of packed red blood cells, fresh frozen plasma, and cryoprecipitate. |
pmc-6132110-1 | The patient is an 84-year-old woman with a long-standing history of mild leukopenia attributed to possible myelodysplastic syndrome, who presented with marked right leg swelling and hypercalcemia. Positron emission tomography (PET) scan showed FDG-avid extensive axillary, left hilar, pelvic and inguinal lymphadenopathy. She was admitted and a lymph node biopsy from the left inguinal region was performed. |
pmc-6132130-1 | A 70-year-old man, with no previous medical history, was admitted to our hospital due to impaired consciousness. On arrival at our emergency department, his airway was patent and the respiratory rate was 30 times/min. His eyes were open but appearing horizontal nystagmus. The oculocephalic reflex was negative at both sides. He did not react to any induced, painful stimulation and flexed his limbs involuntarily. His elbow and knee joints were spastic, but he did not have obvious paralysis or any sensory disturbance. His blood pressure was 230/165 mm Hg upon admission. A blood chemistry analysis showed hypernatremia (160 mEq/L) and severe kidney injury with azotemia (blood urea nitrogen was 198.9 mg/dL). An arterial blood gas analysis showed metabolic acidosis with respiratory compensation. An echocardiogram revealed concentric hypertrophy of the left ventricle with normal wall motion with hypovolemia. A cranial computed tomography (CT) image obtained on the day of admission demonstrated abnormal low-density areas in the basal ganglia, midbrain, thalamus, and both sides of the cerebral white matter. He was treated with drip infusion of a calcium channel blocker, and his blood pressure was controlled to 149/88 mm Hg within 5 hours.
A brain MRI obtained on day 2 demonstrated hyperintense lesions in the cerebral white matter, cerebellum, and brainstem. These lesions were hyperintense on apparent diffusion coefficient (ADC) maps. Diffusion-weighted imaging (DWI) indicated no remarkable alteration (Figure ). On day 3, sodium concentration was normalized, but the patient's consciousness level was E4V2M4 as determined by the Glasgow Coma Scale. We started hemodialysis that same day and continued nine sessions until azotemia recovered. After weaning from hemodialysis, disturbed consciousness persisted. As noted on an electroencephalogram (EEG), slow waves were detected at all channels without any spike waves. In follow-up MRI performed on day 10, DWI demonstrated several microhyperintense signal alterations in the bilateral basal ganglia and cerebral white matter, showing low signal intensity on the ADC map (Figure ). These images verified acute brain infarction. T2-weighted and FLAIR images showed that previous white matter lesions were constant. Given the diffuse and severely abnormal aspects of the white matter and brain ischemia, discontinuation of treatment was discussed. Eventually, we decided to continue treatment. The patient's level of consciousness did not change significantly, and he presented with an incidental, recurrent urinary tract infection and tympanitis. During the seventh week after his admission, the patient was able to nonverbally answer simple questions (eg, via a head nod). His consciousness gradually returned, and within 3 months, he was able to follow commands more easily. The patient had regained almost full consciousness by the time he was transferred to a rehabilitation hospital after 5 months. However, follow-up MRI performed during this same period did not indicate the significant resolution of hyperintense alteration in the cerebral white matter, cerebellum, and also brainstem. |
pmc-6132133-1 | An 84-year-old man with a previous history of cerebral infarction, dementia, and symptomatic epilepsy was admitted to our hospital's surgery department due to small bowel obstruction. Decompression via the nasogastric route was successful, but due to poor oral intake and recurrent aspiration pneumonia, he was transferred to our department (Internal Medicine) for further treatment on day 49. During presentation, his body temperature was 39.5°C, heart rate was 120 beats per minute, blood pressure was 101/71 mm Hg, and peripheral oxygen saturation (SpO2) was 87% with oxygen administered at 5 L/min by reservoir mask. Coarse crackles were audible on bilateral lung fields (left > right). Laboratory finding revealed leukocytosis (16, 780/μL) with neutrophilia (89%), elevated levels of blood urea nitrogen (64.2 mg/dL), and a high C-reactive protein level (18.47 mg/dL). Chest radiograph showed pulmonary infiltrates in the left lung (Figure ).
He was treated with antibiotics (meropenem hydrate 1.5 g/d), which led to some improvement in his fever and SpO2. A nasogastric tube was inserted on the 1st day of transfer, and enteral nutrition (EN) with a polymeric liquid formula (1.5 kcal/mL; 40% carbohydrate, 44% lipid, and 16% protein) was initiated on the following day at 40 mL/h (total 375 mL/d). A follow-up chest radiograph 2 days (3rd day after transfer) after commencing EN did not show any remarkable changes. On the 5th day, however, the patient developed a fever of 38°C and his SpO2 decreased to between 70% and 80%. EN was discontinued, and he was placed on parenteral nutrition alone. Chest radiograph (Figure A) and CT scan (Figure B) revealed complete atelectasis of the left lung with large amount of foreign body (aspirate) blocking the left main bronchus.
Aspiration due to the regurgitation of liquid enteral feed was suspected, but conventional nasal or oral suction was ineffective. On the 7th day, suction was performed by inserting a 15 Fr size nasogastric tube orally into the left main bronchus with the aid of fluoroscopy (Figure A). This led to significant improvement in his atelectasis as confirmed by a chest radiograph on the 8th day (Figure B). EN was resumed from the 8th day using a novel semi-solidifying liquid formula (HINE E-GELⓇ, Otsuka Pharmaceutical Factory, Inc., Tokushima, Japan) via the nasogastric route. HINE E-GELⓇ is a polymeric formula in liquid form with a caloric density of 0.8 kcal/mL (64.2% carbohydrate, 19.8% lipid, and 16% protein) and a dynamic viscosity of about 10 mPa·s (cP), enabling it to be administered easily via a regular nasogastric tube. This formula was administered using gravity control infusion starting at 375 mL/d (300 kcal/d). HINE E-GELⓇ contains 0.9 g of low-methoxyl pectin (LMP) per 100 kcal and calcium phosphate. In an acidic environment (gastric lumen), the calcium phosphate ionizes to Ca2+ which in turn binds to the LMP. The gelation process causes the dynamic viscosity to increase a hundredfold to more than 1000 mPa·s, giving it a semi-solid like texture, just like yogurt.
The novel formula was well tolerated, and EN was gradually increased to 900 kcal/d by the 16th day. Unable to achieve adequate oral intake, the patient received PEG tube placement on the 19th day after transfer and was discharged to a long-term care hospital 3 weeks later without further complications. After PEG, the patient was fed using a regular semi-solid feed (PG Soft AceⓇ, Terumo Corporation, Tokyo, Japan) through his gastrostomy tube. Chest radiograph before discharge showed marked improvement of his pneumonia and atelectasis (Figure ). Table summarizes the differences (improvement) in various nutritional biomarkers between the 8th day (resumption of EN) and the 33rd day (before discharge). |
pmc-6132136-1 | A 19-year-old male of Caucasian origin was admitted to our center as a polytrauma after a road traffic accident. He was previously fit and well, a nonsmoker with an alcohol intake of approximately 10 units per month. The accident, in which his motorcycle collided with an oncoming vehicle, caused him to sustain multiple significant injuries including unstable pelvic fractures and femoral fractures. He had bilateral pneumothoraces, extensive pulmonary contusion, and a splenic hemorrhage. He presented in extremis with signs of hypovolemic shock. He was intubated and resuscitated using local major hemorrhage protocols to achieve a blood pressure of 159/93, receiving ten units of packed red cells and four units of fresh frozen plasma in the emergency department.
He underwent an emergency laparotomy and splenectomy and was subsequently transferred to the intensive care unit, where he became increasingly hypoxic with features of adult respiratory distress syndrome (ARDS). This culminated in him receiving veno-venous extracorporeal membrane oxygenation (ECMO) from day 15 of his admission for 21 days. He returned to theater on day 21 for a massive haemothorax which required an emergency thoracotomy. After being decannulated from the ECMO circuit, he was stepped down to the general intensive care unit on day 36 and was transferred to the ward on day 55 before being discharged after a 4-month admission including a prolonged rehabilitation and recovery period.
After presentation and commencement of ECMO, there was a relatively modest change in liver function tests. Alkaline phosphatase (ALP) increased from 55 to 143 IU/L between day 1 and day 6 of hospital admission and no persistent alanine transaminase (ALT) rise until after decannulation. Proceeding decannulation, there was a sequential increase in ALP peaking at 2335 IU/L on day 113. ALT rose to a lesser extent, peaking at 781 IU/L on day 52. The bilirubin did not rise above 57 μmol/L. The pattern of liver function tests is summarized in Figure . Autoantibody screen, immunoglobulins, and viral hepatitis serology were negative.
Serial ultrasound, computerized tomography (CT), and magnetic resonance imaging excluded biliary stones and sludge. The liver on CT at day 1 showed normal liver and biliary structure (Figure ), and it was not until 10 months after the admission, the repeat magnetic resonance cholangiopancreatography (MRCP) demonstrated a multistenotic pattern of disease within the intrahepatic ducts (Figure ).
In the absence of significant casts within the biliary tree and no evidence to suggest biliary sepsis, endoscopic retrograde cholangiopancreatography (ERCP) was felt not to be helpful. A conservative management approach was taken, and the patient was instigated on ursodeoxycholic acid to help improve cholestasis. With bilirubin improving, the patient was closely monitored as an outpatient upon discharge for progressive liver disease and dysfunction. Repeat imaging and noninvasive fibrosis assessments were undertaken. Despite having evidence of SSC, the synthetic liver function has remained excellent, cholestasis markers have improved, and there have been no episodes of cholangitis or biliary sepsis for over 1 year. |
pmc-6132141-1 | A 64-year-old Caucasian woman with a history of acquired FX deficiency from Amyloid light-chain (AL) amyloidosis, PAF, metastatic lung adenocarcinoma on erlotinib, hypertrophic cardiomyopathy (HCM), hypertension, and type 2 diabetes mellitus was seen in the hematology clinic for routine follow-up.
In 2013, she presented to the hematology clinic for self-limiting epistaxis and easy bruising. Initial outpatient laboratory tests were significant for abnormal coagulation profile (prothrombin time (PT) of 12.4 seconds, INR of 1.2, and activated partial thromboplastin time (aPTT) of 38 seconds), elevated alkaline phosphatase (160 U/L), and creatinine (1.16 mg/dL) levels. Further investigations revealed proteinuria (882 mg/24 h), and abdominal ultrasound demonstrated hepatomegaly with the right lobe measuring 21 cm in craniocaudal dimension. Serum and urine electrophoreses were remarkable for the presence of a paraprotein (immunoglobulin G-lambda). Abdominal fat pad biopsy was unrevealing, and liver biopsy (Figure ) showed extensive amyloid deposition. Immunohistochemical staining of the liver tissue revealed the presence of lambda light chain. A bone marrow core biopsy demonstrated extensive amyloid deposition (Figure ), and it was positive for Congo red staining with classical apple-green birefringence under polarized light. Fluorescence in situ hybridization assay was positive for lambda light chains. Further work-up of the abnormal coagulation study revealed a decreased FX coagulation activity of 45% (reference 70%-150%). Screening for inhibitor to FX was negative. Hence, a diagnosis of acquired FX deficiency secondary to AL amyloidosis was made. In view of her recurrent lung adenocarcinoma, she was deemed a poor candidate for autologous stem cell transplantation and she was started on melphalan and dexamethasone chemotherapy.
In 2015, she developed an episode of symptomatic PAF. Her coagulation profile showed PT of 16.3 seconds, aPTT of 33 seconds, and FX activity of 34%. As she has HCM, her CHA2DS2-VASc score was 4 with a significant risk for thromboembolism. Hence, she was started on apixaban 5 mg twice daily with close outpatient follow-up.
Three months later, she was found to have progression of her lung adenocarcinoma as she sustained a left pathologic subtrochanteric fracture requiring an urgent surgical intervention. In view of the need for her to undergo an open reduction and internal fixation of the left hip, her apixaban was temporarily withheld for close to 30 hours prior to the surgery. Preoperatively, her laboratory values were as follows: hemoglobin of 9.7 g/dL, PT of 20.3 seconds, and aPTT of 36 seconds. She had an uneventful open reduction and internal fixation of her left hip requiring one unit of packed red blood cell transfusion. Postoperatively, her laboratory values were as follows: hemoglobin of 8.1-8.4 g/dL, PT of 20.9 seconds, aPTT of 33 seconds, mixing studies showed correction (indicated underlying factor deficiency), fibrinogen level of 466 mg/dL, and FX coagulation activity of 37%. She was started on enoxaparin 30 mg subcutaneously every 12 hours for deep vein thrombosis prophylaxis. Two weeks later, she was restarted on her usual dose of apixaban for her paroxysmal AF. Her recovery was uneventful. A summary of her laboratory data is shown in Table . |
pmc-6132148-1 | A 58-year-old nonsmoking male was referred with a 1-week history of cough. He had been on peritoneal dialysis since 3 years due to hypertensive nephrosclerosis. Despite having sustained an intracranial hemorrhage 8 years prior, he had no residual neurologic deficits. A chest radiograph revealed a radiopaque foreign body in the right hilar region (Figure A). The presence of a bronchial foreign body was suspected, and flexible bronchoscopy was performed. Balloon catheter was dilated at the periphery of the foreign body and withdrawn together with the bronchoscope (Figure B). After confirming the history of the prescribed medicines, the removed pill was found to be of lanthanum carbonate. After the procedure, the patient's respiratory symptoms resolved.
The nature of airway foreign bodies is highly variable, ranging from organic to inorganic. It is estimated that approximately 7% of all foreign bodies are medicinal pills. Although most pills are radiolucent, lanthanum carbonate is radiopaque and may be identified on chest X-rays. Because the removed pill was a chewable tablet, it was not crushed during the procedure. |
pmc-6132149-1 | A 22-year-old female patient reported to us with a vague pain on the left side of face which was throbbing in nature and radiating to the eye on the same side since 18 months. She was apparently in a good health and her medical history was unremarkable. She reported a transient diplopia in an upward gaze and epiphora occasionally. The epiphora could be ascribed to the tumor expansion causing compression of the nasolacrimal duct. On examination, there was vestibular obliteration extending from the left first premolar up to the second molar. Crepitations were palpated over the premolar region signifying a cystic lesion. Aspiration yielded cheesy material consisting of keratin flakes. Surprisingly, no symptoms of sinusitis were present. Orthopantomogram revealed a high posterior impacted third molar in the maxilla abutting the floor of the orbit. (Figure ) Further radiological investigations included a CT scan. (Figures , and ) The Caldwell-Luc operation involves creating an opening into the maxillary antrum through the canine fossa. (Figures and ) A trapezoidal flap was raised to gain access to the underlying pathology. (Figure ) Fenestration on the bony wall exposed the keratin cheesy lining which was enucleated along with peripheral ostectomy that aids in the removal of any traces of epithelial remnants. The intrinsic bony erosion enabled us to trace and remove the third molar. The antrum was copiously irrigated with an antral pack in place, removed subsequently. A middle meatus nasal antrostomy was performed to attain a physiological sinus drainage. To assure total removal of the cyst lining, an endoscopy was performed along with a clinical and radiological follow-up on a regular basis. Histopathology of the excised specimen read as parakeratinized variant of OKC. (Figures , and ) The wound healing was uneventful, with remission of epiphora, and diplopia following a 1-year follow-up. |
pmc-6132151-1 | A 29-year old female diagnosed with SLE for 4 years complicated with grade II lupus nephritis presented with status epilepticus. She denied a history of fever on admission, but was treated with cyclophosphamide 1 month prior for an episode of cerebral lupus. She had noticed a papule over the left deltoid region which progressed to an ulcer over 1 week. Fever was noted following several days of hospital admission and the ulcer site became painful. She had worked in paddy fields several months prior to the admission when she was in good health. However, she could not recall any precipitating injury at the affected site during working. She is a mother of two and both pregnancies were uncomplicated. She denied history of alcohol abuse or smoking.
On examination she was emaciated and had a GCS score of 15/15 following recovery of status epilepticus. There was no obvious lymphadenopathy. At presentation, the size of the ulcer was about a 3 cm lesion and it gradually developed in to an ulcer with a necrotic center with surrounding erythema. A tentative diagnosis of pyoderma gangrenosum was made with the appearance of the ulcer (Figure ). It gradually advanced into the underlying muscle over 3 weeks of onset despite the antibiotic treatment. Examination of the cardiovascular, respiratory systems, and the abdomen was normal.
Her full blood count, blood picture, and other supportive investigations showed evidence of microangiopathic hemolytic anaemia, which was suggestive of thrombotic thrombocytopenic purpura which resolved following plasmapheresis. Her ESR was persistently normal. Renal functions were stable during hospital stay, so were the liver profile. Chest radiography revealed evidence of bilateral mild pleural effusions and echocardiography revealed a thin rim of pericardial effusion and good cardiac function. MRI, MRA brain showed evidence of Posterior Reversible Encephalopathy Syndrome. Repeat imaging showed resolved changes.
A punch biopsy of the skin was done from the lesion and sent for fungal studies and histopathological studies. The direct microscopy examination revealed wide and irregular ribbon-like nonseptate hyphae with right-angle branching suggestive for Mucormycete fungi. Culture was done on Sabouraud dextrose agar with chloramphenicol (at 26°C and 37°C) yielded a white aerial mold, which covered the entire surface of the agar and came up to the lid of the culture bottles after 4 days of incubation (Figure ).
The lactophenol cotton blue mount of the growth revealed broad, nonseptate hyaline sterile hyphae. The slide culture test has been attempted with the hope of sporulation, however it was not successful. They only resulted in broad, nonseptate hyaline sterile hyphae without spores. Then the isolate was subcultured on to potato dextrose agar (PDA) and Rose Bengal (RB) agar for induction of sporulation. However, they yielded only sterile mycelia.
The isolate was inoculated on nutritionally deficient medium, tap water agar and incubated for 14 days at 37°C. It provided a hazy view of flask shaped sporangium with rhizoids in lactophenol cotton blue mount. Then floating agar method was used and it yielded characteristic flask-shaped sporangium in short sporangeophore with rhizoids after 10 days of incubation (Figure ).The sporangia had a long neck and the apex of the neck closed with a mucilaginous plug. The sporangiospores were cylindrical, with rounded ends. Those morphological features were suggestive for S. vasiformis and the isolate was identified as S. vasiformis.
The histopathology of the punch biopsy of the skin also reveled broad aseptate hyphae suggestive of Mucormycetes group of fungi.
Based on the histopathological evidence of broad aseptate hyphae, suggestive of Mucormycete fungi, the patient was started on IV amphotericin B deoxycholate. Repeated surgical debridement was done and samples were sent for fungal studies. However, local application of antifungals was not included in the management. Her second tissue biopsy, which was taken during debridement after 5 days of IV amphotericin B also had similar direct microscopy findings and yielded S. vasiformis. However third tissue sample which was obtained after 10 days after IV amphotericine B deoxycholate became negative for fungal studies. Following the confirmation of sterile cultures from the subcutaneous biopsies, superficial skin grafting was done which was completely accepted from the wound site. She was treated with intravenous conventional amphotericin B for 28 days and she was asymptomatic when she was discharged from the ward. |
pmc-6132153-1 | We present a 67-year-old man with permanent atrial fibrillation and triple mechanical prosthetic valve replacements (All bileaflet mechanical valves, St Jude Medical Inc, St Paul, MN, USA) at the aortic, mitral, and tricuspid positions since 1999 for rheumatic valvular disease. He subsequently developed progressive nonischemic cardiomyopathy with a residual left ventricular ejection fraction of 15% for which a subcutaneous implantable cardioverter defibrillator (S-ICD) was recommended but he declined.
He presented to the emergency department in October 2017 for increasing dyspnea and orthopnea. Physical examination revealed significant bradycardia of 35-40 bpm with signs of congestive cardiac failure. An electrocardiogram (ECG) performed showed atrial fibrillation with intermittent rate regularization and variable wide complex QRS morphology (Figure A,B), which was suggestive of high-grade atrioventricular (AV) block with variable ventricular escape rhythm. We counseled the patient and scheduled for an urgent cardiac resynchronization therapy with defibrillator (CRT-D) device implantation via the coronary sinus.
Under aseptic conditions, extrathoracic subclavian venous punctures were made using fluoroscopic guidance. The guidewires were easily maneuvered into a large coronary sinus (CS). An occlusive CS-venogram demonstrated an ectatic CS with tortuous takeoffs at the posterolateral branches. The middle cardiac vein (MCV) had a separate ostium which was also ectatic at its proximal segment tapering into a much smaller vessel (Figure A,B).
Using an 0.014″ guidewire (Acuity Whisper View EDS 190 cm, Boston Scientific Corp, St Paul, MN, USA) through an extended-hook outer sheath (Acuity Pro 9F Guiding Catheter Extended Hook 45 cm, Boston Scientific Corp) and inner catheter (Acuity Pro 7F Inner Guiding Catheter CS-IC130 60 cm, Boston Scientific Corp), we were able to advance the wire into the MCV after numerous attempts. However, the outer sheath could not follow through distally, and even if it did, it would not accommodate the larger caliber defibrillator lead. Hence, we proceeded to advance a straight quadripolar lead (Acuity X4 straight 86 cm, Boston Scientific Corp) into the MCV, going around to the anterior interventricular vein (AIV) to achieve a stable position. (Figure C,D) We then directed a defibrillator lead (Endotak Reliance SG 64 cm DF4, Boston Scientific Corp) into the CS just using a curved stylet and advanced it into an anterolateral branch for stability without helix deployment. During interrogation of the defibrillator lead, however, we found that there were poor R-wave sensing and unacceptably high thresholds and we could not use the quadripolar lead in the MCV for tachycardia sensing.
Hence, we removed the quadripolar lead in the MCV and advanced a bipolar lead (Acuity Steerable 90 cm IS1, Boston Scientific Corp) into the MCV, but we could not advance it to the AIV due to an increase lead caliber. Hence, we advanced it to an anterolateral branch of the CS. We also successfully switched the defibrillator lead to one with a DF1 connection (Endotak Reliance SG 64 cm DF1, Boston Scientific Corp) and readvanced it into another anterolateral branch of the CS. (Figure E,F) The leads were then connected to a generator (Inogen EL ICD, Boston Scientific Corp) with the atrial port plugged.
We then proceeded with defibrillation testing. We made five attempts at induction of ventricular tachyarrhythmia using shock-on-T as well as 50 Hz burst pacing. For three attempts, we did not induce any tachyarrhythmia. For two other attempts, we induced a rapid monomorphic ventricular tachycardia (MMVT) that was nonsustained. We decided not to persist with defibrillation testing. Postprocedure device check showed satisfactory R waves of 14 mV, a pacing threshold of 0.8 V at 0.4 ms, and stable impedance of 765 ohms. A 12-lead ECG pacing pattern was consistent with baso-anterolateral LV pacing. (Figure C).
The patient was reviewed in the outpatient clinic 1 month later, and device interrogation showed stable parameters with recorded episodes of nonsustained ventricular tachycardia (NSVT). The patient agreed to repeat defibrillation testing and was readmitted for this. After moderate sedation, rapid MMVT was induced with 50 Hz burst pacing through the device, and an initial programmed defibrillation at 26J degenerated the rhythm into ventricular fibrillation (VF), while the next programmed defibrillation at 41J successfully cardioverted the patient back to ventricular paced rhythm. We reinduced MMVT with 50 Hz burst pacing, and again the first programmed defibrillation at 36J degenerated the rhythm into VF, while the next programmed defibrillation at 41J successfully cardioverted the patient back to ventricular paced rhythm. (Figure ) A long discussion was made with the patient and family, and a decision was made not to pursue with further lead repositionings nor additional defibrillator coils or arrays in a bid to reduce the defibrillation threshold. |
pmc-6132154-1 | A 48-year-old male patient attended the clinic complaining of pain in the gum behind the lower second molar. The consent form was obtained from the patient to use clinical information and photographic material of the treatment. The patient did not report any significant medical history, except for a dental history of amalgam fillings 15 years ago. The patient brushed his teeth once daily using a medium bristled brush and a horizontal tooth brushing technique. Upon examination of the oral cavity, the upper third molar opposing the site of the pain appeared to be supra-erupted, forming abnormal contact with the soft tissue. Radiographic examination (bitewing radiographs in Figure and panoramic radiographs in Figure ) confirmed the clinical findings. The supra-erupted upper third molar that formed abnormal contact with the lower gum was determined as the cause of pain in the area. The oral examination also revealed generalized erosion on the facial, cervical (Figure ), incisal and occlusal (Figure ) surfaces of the teeth. Heat and cold sensitivity tests revealed normal results indicating that the patient experienced no dentin hypersensitivity. The medical history did not reveal usual suspected causes of erosion.
The patient was asked to make a diet log for 1 week until the next appointment to determine the extrinsic source of erosion as intrinsic factors were ruled out due to negative medical history (Table ). Salivary tests were carried out to measure salivary flow, pH, and buffering capacity (Figure ), revealing normal salivary flow with a decrease in salivary pH and buffering capacity. On examining the diet log at the next appointment, the only dietary agent that was consistently consumed by the patient that had the potential to cause such erosion was Granny Smith apples, which the patient consumed 3-4 for lunch for the last 10 years. |
pmc-6132158-1 | A 40-year-old man presented with a four-month history of progressive lower limb weakness and sensory disturbance. He reported occasional fecal incontinence, with associated 8 kg weight loss, and occasional night sweats. He had a past history type 2 diabetes mellitus, but was otherwise well.
Examination revealed proximal lower limb weakness, with a sensory level to L1. Lower limb reflexes were brisk, with flexor plantars. Anal tone and perineal sensation were preserved. The remainder of the neurological examination was unremarkable. Eye examination was normal. General examination was normal with no palpable lymph nodes or skin rashes.
Magnetic resonance imaging (MRI) of the spine demonstrated diffuse nodular and linear leptomeningeal enhancement along the spinal cord and cauda equina (Figure ). A plain chest film demonstrated bilateral hilar adenopathy, confirmed by high-resolution CT chest. 18F-FDG PET scanning demonstrated multifocal, nodular FDG-avid uptake in the mediastinum, hila, liver, and spinal canal (Figure ).
Cerebrospinal fluid (CSF) testing revealed a moderate reactive pleocytosis, with a CD4:CD8 ratio of 3.56, protein 3.99 g/L, and glucose 1.7 mmol/L (paired serum glucose 12.6 mmol/L). Cytology was negative for malignant cells. CSF was negative for acid-fast bacilli, culture, and mycobacterial PCR, and CSF cryptococcal antigen testing was negative. Serum ACE and 24-hour urinary calcium were normal. Endoscopic bronchial ultrasound of the hilar nodes did not yield adequate tissue for analysis. Ultrasound-guided percutaneous biopsy of a liver lesion yielded a sample demonstrating multiple granulomas and multinucleate giant cells consistent with sarcoidosis, with staining for acid-fast bacilli and fungal organisms negative. A diagnosis of systemic sarcoidosis with probable neurosarcoidosis affecting the spinal cord was made.
Following diagnosis, he was treated with pulsed intravenous methylprednisolone, followed by high-dose oral steroids with slow wean. Azathioprine was started as a steroid-sparing agent. He responded rapidly with normalization of power and sensation; however, he relapsed repeatedly during steroid wean, and his blood glucose proved difficult to manage while treated with glucocorticoids. He was switched to infliximab with good and sustained response. |
pmc-6132159-1 | A 32-year-old lady, gravida 5, para 4 (G5P4) was accepted as a referred case in the Department of Obstetrics and Gynecology due to abnormal placentation diagnosed during a routine ultrasound late in pregnancy at 29 weeks of gestation. She had a 3-year medical history of previous cesarean delivery due to placenta previa. We followed a high index of precision for the clinical diagnosis of invasive placentation. A trans-abdominal ultrasound examination using a 6-1.9 MHz trans-abdominal probe (Toshiba Artida PVT-375BT, Inc, Yokohama, Japan) revealed a viable pregnancy with normal amniotic fluid and appropriate fetal sonographic biometric parameters. We assessed the grade and number of placenta lacunae in accordance with Feinberg's criteria. Transabdominal ultrasonography revealed the presence of intraplacental lacunae along with loss of retroplacental clear zone and irregular disruption of bladder–uterine serosa interface with least myometrial thickness (<1 mm) Figure . The bladder was moderately filled to evaluate its involvement better. The patient was informed about all treatment options, and their possible consequences including hysterectomy as uterine preservation is a fertility-sparing option but feasible only in selected cases. Following this, an elective cesarean section was scheduled at 36 weeks of gestation. On the scheduled due date of the delivery, the patient was taken to the operating theatre after preoperative preparation and blood arrangements. After exploratory laparotomy, intra-abdominal findings included an array of invasive placentation within the lower uterine segment. After initial displacement of the bladder had been achieved, a transverse uterine incision was proceeded above the lower section of the uterus by avoiding the placental bed. A healthy baby girl of 5 pounds, 8 ounces was delivered. Subsequently, there was a severe hemorrhage, and a cesarean hysterectomy was decided upon, to be performed immediately. The placenta only penetrated up to the myometrium and reached the serosa to some extent, along with the formation of several hematomas on the bladder mucosa while dissecting which required stitching. The total estimated blood loss was 6 L. Intraoperatively, we transfused six units of whole blood and 1500 mL free-frozen plasma (FFPs). The postoperative recovery was smooth, and Foley's catheter was retained for 5 days. The patient was discharged on the sixth postoperative day in favorable recovery conditions. |
pmc-6132159-2 | A healthy 35-year-old woman with a history of 2 elective cesarean sections and one miscarriage (G4 P2 A1) was accepted as a referred case to our hospital. The patient was admitted for the elective cesarean section at 37 weeks of gestation. She was referred from the rural primary care hospital where routine ultrasonography revealed low-lying placentation. A Doppler ultrasound at 35 weeks showed complete covering of the cervical os which was attached to the scar with incessant hemorrhagic lesions in the inner anterior myometrium, indicative signs of PA (Figure ). She had diagnosed placentation in the lower uterine segment and had given a history of irregular, painless bleeding of the vagina during the fifth and seventh months of gestation. Further, magnetic resonance imaging (MRI) was performed, images still revealed that the placenta was completely covering the cervical os and suspected implantation of placental villi penetrating the full thickness of the myometrium, which further extending posteriorly up to the bladder wall and anteriorly displayed vascular engorgement (Figure ). On the day of surgery, an expert urologic surgeon had been involved along with the interventional cardiology team. During the cesarean, the uterus was opened along the midline in upper segment of uterus, and a baby of the 3 kg was delivered with a good Apgar score. Due to invasive placentation and bleeding, cesarean hysterectomy was proceeded immediately after the birth of the baby. The lower uterine segment was noted to be relatively thin but was covered by a plexus of deep vessels which spread over the bladder near the broad ligament which was covered by peritoneum. The bladder was opened and repaired due to continuous bleeding as the placenta extended to the bladder. Left-sided salpingo-oophorectomy was also performed due to constant bleeding from the left tubo-ovarian ligament. The estimated blood loss was approximately 3500 mL during the procedure. Afterward, three units of whole blood were transfused, and two hemaccel infusions were given. The patient was kept on inotropic support in postoperative ICU for 24 hours. Later, the patient was moved to the surgical ward and discharged from the hospital on the 5th postoperative day. Foley's catheter was retained for 2 weeks, and a smooth postpartum recovery was observed. |
pmc-6132159-3 | A 38-year-old patient, gravida 4, para 2 (G4P2 +1) with two cesarean sections and a history of tubal pregnancy was admitted for the elective cesarean section at 36 + 2 weeks, ultrasound from an outside private hospital revealed full placenta previa and suspected placenta accreta (Figure ). PA was further diagnosed on MRI. The patient had strongly wished for future fertility, and we informed him and her husband about the expected risks associated with this conservative treatment. After full preparation in theatre, the uterus was opened in the upper segment, sparing the placenta. A baby of 2.4 kg with a good Apgar score was delivered. A portion of the placenta was delivered immediately after the birth of the baby. However, there was excessive bleeding in the lower segment with a part of placenta adhered to the uterine wall. Several stitches were taken in lower uterine segment, and the B-Lynch suture was applied after which bleeding stopped. The estimated blood loss was 2 L. Two units of whole blood and 1-unit of FFP were transfused during surgery while 1-unit whole blood was given postoperatively. A single dose of MTX (Methotrexate) injection (25 mg/mL) was given commencing from the first day after the cesarean sections (C-sections) along with broad spectrum antibiotic cover. Postoperative recovery was satisfactory, and the patient was discharged on the 4th postnatal day. On the 30th postoperative day, the patient was re-admitted due to vaginal bleeding, an immediate D & C (dilation and curettage) was performed, and the residual placental mass was completely removed. After 1 week, ultrasonography confirmed an empty uterine cavity and no further complications were noted. |
pmc-6132165-1 | A 57-year-old man with smoking history and untreated hypertension developed sudden lower abdominal pain. Although abdominal pain disappeared half a day later, abdominal bloating and nausea appeared and got worse gradually. Physical examination showed epigastric tenderness and a pulsatile, fist-size mass under the umbilicus. Upper endoscopy showed severe edematous stenosis of the duodenum (Figure ). Contrast-enhanced CT revealed severe stenosis at the origin of the celiac artery caused by the midline arcuate ligament (Figure A-C), a low-density retroperitoneal mass lesion (Figure D), and dilatation of the branches of the pancreaticoduodenal artery (PDA; Figure E). Accordingly, we made the diagnosis of duodenal stenosis caused by a retroperitoneal hematoma secondary to rupture of the PDA aneurysm.
Pancreaticoduodenal artery aneurysms account for <2% of all visceral aneurysms. The mechanism of occurrence of true PDA aneurysms has been attributed to occlusion or constriction of the celiac artery caused by the median arcuate ligament, arteriosclerosis, or fibromuscular hyperplasia. We performed midline arcuate ligamentotomy to improve the stenosis at celiac artery, decreasing hematoma and clearing duodenal obstruction. The elevated pressure of the PDA might have caused formation of the aneurysm, which ruptured into the retroperitoneum, subsequently producing a hematoma. |
pmc-6132166-1 | A 72-year-old Caucasian man was admitted to our department with a pain in the left posterior mandible and periodontal hyperplasia associated with the left mandibular second molar tooth. He also complained about the ongoing pain for 2 months and spontaneous hemorrhage within the lesion region.
According to his medical history, the patient had suffered from coronary angioplasty 6 years ago. He also suffered from malaise and fatigue for last 1 year and also inappetence for last 6 months.
Extraoral examination of the patient showed no visible swelling, tenderness or pus discharge. Skin color and temperature were normal. In the intraoral examination of the relevant region, oral hygiene level was not good and gingival tissue around the second molar was hyperplastic and had a tendency to spontaneous bleeding.
On the other hand, the panoramic radiograph (PANO) showed a radiolucent lesion with irregular margins located the periapical area and also extended coronally that led into the serious mobility of mandibular left second molar tooth (Figure ).
Considering the patient's medical history and after an exhaustively clinical and radiographical examination, we decided to perform an incisional biopsy under local anesthesia. As expected, the result of the pathological examination was peripheral giant cell granuloma (PGCG). We did not consider to take an initial photography before the surgical procedures, however, with regard to the pathological results, we decided to perform another surgery under local anesthesia 1 week later, including extraction of the tooth and a wide curettage of the lesion in the left posterior mandible. Despite the anesthetic procedures were performed properly and adequately, the patient was still suffering from pain but no severe hemorrhage during the curettage was observed from the surgical area. After pathological assessment of the second biopsy, the lesion was diagnosed as DLBCL (Figure ).
Even though the patient was relieved and healing was uneventful, we did several consultations and asked for PET/CT scan of entire body because of metastatic nature of DLBCL. After all of these scanning procedures, we doubted the patient may also have had DLBCL in his thyroid gland, gastric system, and prostate either.
On the other hand, 2 weeks later from the second biopsy, our patient had satisfactory outcomes, such as; no pain, hemorrhage, or swelling in the operated area. He stated that he is totally relieved and he is ready for the prosthetic procedures.
Mandibular biopsy results showed sheets of medium to large lymphoid cells with hyperchromatic nuclei and scanty cytoplasm. These cells stained positive for CD3, CD20, LCA1, and LCA2 for Cyclin D1 (clone Polyclonal). Ki67 proliferation index was %90. The tumor was also positive for MUM-1 and BCL 2 and BCL 6 (Figure ). Other blood tests and bone marrow investigations did not reveal any abnormality. However, the gastrointestinal tract biopsy resulted in almost the same outcome, with a slight difference of %80 Ki67 proliferation index, positive CD5 (Clone 4C7), and CD138 (clone MI-15) and negative Cyclin D1 results. Attributed to these results, the lesion in the gastrointestinal tract was diagnosed with DLBCL (Figure ).
Even though radiological findings were not satisfactory enough in our second and the third follow-up (Figure ) and intraoral healing of the patient was still uneventful after 3 months (Figure ), the patient was decided to have several courses of systemic chemotherapy by the department of oncology as further treatment protocol of DLBCL.
Nevertheless, the patient had a stroke after chemotherapy finished (6 months later) and he passed away within 2 years after diagnosis due to DLBCL. |
pmc-6132167-1 | A 78-year-old man visited our emergency room with unconsciousness. According to his family, he was poisoned with pesticide (glyphosate) for self-injury purpose. Two years ago, he had a history of cerebral infarction, and he was taking aspirin and medication for hypertension. During gastric lavage in the local clinic emergency room, his heart rate and respiration decreased. Then, he was transferred to the intensive care unit after intubation.
Intensive care such as continuous renal replacement therapy and mechanical ventilation was performed in the intensive care unit, and colistin and minocycline were administered for pneumonia. Pulmonary thromboembolism was confirmed by chest computed tomography (CT), and anticoagulant therapy was performed. Acute acalculous cholecystitis occurred simultaneously, and a percutaneous transhepatic GB drainage tube was inserted.
After 2 months of intensive treatment, the patient's condition improved, and cholecystectomy was planned. He underwent laparoscopic cholecystectomy, and there were no remarkable details of the surgery.
On the first day postoperatively, the drainage changed to bile. The patient's vital signs were as follows: blood pressure, 160/105 mm Hg; pulse rate, 143 beats/min; respiratory rate, 37 breaths/min; and body temperature, 39°C. There was tenderness and rebound tenderness in the whole abdomen, and CT showed pneumoperitoneum and diffuse fluid collection in the right perihepatic space (Figure ). We diagnosed him as having bile peritonitis, and reoperation was decided.
On relaparoscopic examination, there was a large amount of bile juice around the GB bed and perihepatic space, and suction and irrigation were performed. The cystic duct stump was confirmed, but there was no leakage. In the GB bed, a leak was observed in a small duct-like structure, which was regarded as an aberrant duct, and the operation was terminated after primary repair (Figure ).
Until 6 days postoperatively, hemoserous fluid was drained from the drainage tube. However, from postoperative day 7, the fluid changed to bile, and a percutaneous drainage (PCD) catheter was inserted into the GB bed area after performing CT.
On postoperative day 9, endoscopic retrograde cholangiopancreatography (ERCP) was performed, and a leak was detected in the branch of the right posterior hepatic duct, which was regarded as an aberrant subvesical bile duct (Figure ). Endoscopic sphincterotomy (EST) and insertion of an endoscopic retrograde biliary drainage (ERBD) stent (7 French, 5 cm) were performed (Figure ).
One month after ERBD stent insertion, ERCP was performed again to confirm that there was no leak, and then, the ERBD stent was removed (Figure ). |
pmc-6132168-1 | A 35-year-old male patient presented to the East Riyadh dental center in Riyadh city, Saudi Arabia. He was referred from a prosthodontic clinic to correct his previous treatment. The patient was in good health with no apparent systemic disease. On clinical examination, the teeth 26 and 27 were asymptomatic with large previous restorations in both teeth. A preoperative periapical radiograph revealed the presence of four roots in tooth 27. This tooth had previous endodontic treatment with poor obturation and missing untreated canals (Figure ).
A diagnosis of asymptomatic apical periodontitis was made, and nonsurgical retreatment for teeth 26 and 27 was planned. The old restoration of tooth 27 was removed following local anesthesia and rubber dam application. Rhomboidal outlined access cavity was then prepared to obtain a straight-line access to all canals. There were four canal orifices, two buccal and two palatal canals located on the floor of the pulp chamber (Figure ).
Old gutta-percha filling was removed using a Gates Glidden drills and H file (Mani, Inc., Japan) and chloroform as solvent. Working length was determined by the apex locator (Root ZX, J. Morita Corp., Tokyo, Japan) and confirmed radiographically (Figure ). Canal preparation was performed using ProTaper Universal (Dentsply-maillefer, Ballaigues Germany) files up to F3 in mesiopalatal and in F4 distopalatal by while other canals prepared up to F2. The canal was irrigated between each instrument with 5.25% NaOCl, and final irrigation was done using 17% EDTA (Meta Biomed Co. Ltd., Cheongju City, Chungbuk, Korea). Patency was kept by recapitulation with a No.10 file.
A master cone radiograph was taken (Figure ), and canals were obturated with gutta-percha and AH plus sealer (Dentsply, Maillefer, Germany). Gutta-percha was filled using continuous wave of condensation technique with a system B heat source (SybronEndo, Orange, CA, USA), and a cordless obturation gun with gutta-percha pellets (Meta Biomed Co. Ltd.) was used for back fill (Figure ). The access cavity was sealed with a temporary filling material (Cavit™ G, 3M ESPE, Germany), postoperative radiographs were taken (Figure ), and the patient was then referred back to the prosthodontist. Tooth #26 was retreatment as the same manner as #27. We tried to locate the MB2 by careful examination of the pulpal floor under dental operating microscope and trephination using ultrasonic tip but we could not find it. |
pmc-6132171-1 | A 71-year-old male presented to the ENT outpatient clinic with a 2-year history of mild inspiratory stridor at rest, worsened markedly by exertion but not limiting exercise tolerance. He did not report any voice change, swallowing difficulties, or weight loss. Full examination including flexible nasolaryngoscopy revealed a thickened epiglottis, bulky arytenoids, and aryepiglottic folds only. The patient was a nonsmoker and was taking regular cardiac medications; however, he denied any recent medication changes or new inhalers. There was medical history of cardiac stenting, benign prostatic hypertrophy, and a transient ischemic attack. A first-degree relative had a previous diagnosis of sarcoidosis.
Initial differential diagnoses included amyloidosis and sarcoidosis, and the patient was commenced on a trial course of oral prednisolone.
Routine bloods were carried out along with immunoglobulins, creatinine kinase, ANCA, ACE, ANA, and serum amyloid A. The results were unremarkable, with the exception of speckled ANA which returned with a positive titer of 40. Following rheumatology review, this was thought to be clinically insignificant.
A contrast CT scan of the neck and chest revealed subtle asymmetric thickening of the soft tissue of the epiglottis into the right aryepiglottic fold. There was no lymphadenopathy and no other significant pathology demonstrated. (Figure ).
The patient was also referred to the respiratory team for an opinion. Pulmonary function tests were essentially normal, with an FEV1 and FVC above 90%.
Ongoing laryngeal changes with an increasingly thickened epiglottis were noted at ENT follow-up, and the patient was booked for microlaryngoscopy and biopsy. This revealed a grossly thickened epiglottis, with marked bilateral vocal cord edema and a generalized cobblestone appearance of the mucosa. There was also evidence of supraglottic narrowing. Biopsies were taken from the epiglottis and supraglottic mucosa. (Figure ).
Histopathological examination revealed moderately hyperplastic stratified squamous epithelium, with elongation of the rete pegs but without cytological atypia. The lamina propria was populated by large numbers of plasma cells in confluent sheets, accompanied by lymphocytes and some neutrophils. The features described, that is, epithelial hyperplasia accompanied by plasmacytosis, were strongly suggestive of a diagnosis of laryngeal plasma cell mucositis. (Figure ).
The patient was initially commenced on a Pulmicort inhaler; however, this did not lead to any clinical improvement and was subsequently stopped after a number of weeks. He then received a 1-week course of 40 mg once daily oral prednisolone, followed by a dose decrease of 10 mg every 3 days until stopping completely. This led to partial resolution of symptoms, with a reduction in stridor at rest and on exertion. He is currently not receiving any steroid therapy and is being reviewed on a 6-monthly basis. This is likely to continue for at least 1 year. The patient has been advised that should his condition deteriorate acutely, a suitable treatment regime would be either a course of oral prednisolone 30 mg or intravenous dexamethasone, depending on the degree of airway compromise. |
pmc-6132340-1 | A 28-year-old man presented at a traumatic surgery unit in an outlying hospital, where an external rotation of the left leg and a patellar dislocation were detected. Examination also revealed clinical signs of a neurofibromatosis-1 (Figure ). The patient had stumbled doing his work as a cook and had fallen on his flexed left knee. Conventional radiography of the knee demonstrated an osteochondral flake near the medial patellar margin, whereas the femoral fracture remained unnoticed (Figure ). After reposition of the patellar dislocation, the joint was stabilized with an orthosis, and the patient was sent home.
Two days later, because of increasing pain and swelling of the left knee, his family doctor arranged further radiographic examinations with MRI and CT scans, revealing the Hoffa fracture. The patient was then referred to a medical center for traumatic surgery. Surgical treatment consisted of diagnostic arthroscopy, open reduction of the fracture, and internal fixation with three 40-mm headless compression screws (Figure ). The screws were placed in posterior to anterior and caudal to cranial directions. The anterior horn of the lateral meniscus was fixed to the joint capsule using FiberWire 2-0, and the ruptured retinaculum was fixed to the medial patellar margin with two Mitek anchors. A 3 × 3 cm tumor close to the articular capsule turned out to be a lipoma and not, as initially suspected, a neurofibroma.
After surgery, the knee was stabilized with a knee orthosis, and partial weight-bearing was prescribed for 10 weeks. Fracture healing was regular. In <4 months after the accident, the patient returned to work without any orthopaedic therapeutic appliances. In this case, the occurrence of a femoral fracture after a low-velocity trauma was probably influenced by reduced bone quality because of an underlying NF1, but NF1 did not influence fracture healing. Two years after the operation, the range of motion of both knees was unlimited, and no osteoarthrotic changes were noticed. |
pmc-6132374-1 | Mia is a 10-year-old African American female living with a family member in an urban area. Mia is diagnosed with PTSD due to abuse and neglect experienced while living with a foster family. Mia was born addicted to substances and consequently taken into custody by the Department of Children and Families (DCF) and placed with a foster family who fled to another state. During this period, Mia was subject to extensive abuse and neglect by her foster parents, including severe sexual abuse. Mia was subsequently brought back to the area in which she was born, and placed with a family member with whom she currently lives. Mia presents with sexualized behaviors which she has acted out towards family members and the family dog. She also has a history of isolated incidents during which she plays with her feces.
Mia is an intelligent and bubbly girl that can present with a high rate of distractibility and impulsivity. Mia demonstrates oppositional behaviors when teachers or providers attempt to redirect her, but she is often able to reflect on/has insight around her actions. Mia has been receptive to trauma-informed interventions, which is why she was initially referred for equine therapy. Mia is motivated by working with animals and gravitates towards “hands on” activities.
Before the start of EFT-CT, Mia was highly symptomatic across a range of clinical domains. On the CBCL, her primary caregiver indicated that Mia exhibited a number of behavior problems on the (CBCL Total Problems T-score = 68, clinical range) both internalizing and externalizing in nature, including clinically significant social problems (T = 72), thought problems (T = 74), and attention problems (T = 77). Her caregiver also reported broadly impaired executive functioning on the BRIEF-P, indicated by a Global Executive Composite score in the clinically significant range (T = 71, 98th percentile), and with the most significant problems in the domain of metacognition (T = 75, 98th percentile, clinically significant range). In addition to significant difficulty with getting started on tasks, organizing her thinking, or strategizing, self-monitoring and working memory, Mia exhibited significant difficulties with behavioral inhibition (T = 78, 98th percentile). Regarding trauma-specific symptomatology, Mia endorsed clinically significant problems in two of the three symptom DSM-IV-TR symptoms clusters on the PTSD-RI (avoidance, T = 80; arousal, T = 92), as well as subclinical problems with trauma-related intrusions (T = 63), and a posttraumatic stress total score also in the clinically significant range (T = 86). She also endorsed clinically significant problems with dissociation (T = 75), and sexual concerns (T = 99), related to Mia’s trauma exposure. Mia herself did not endorse any significant symptoms on a self-report of depression (CDI-2 total problems T-score = 54; 66th percentile).
Mia was able to form a strong and positive attachment with the therapy pony during EFT-CT sessions. Because of her motivation to work closely with the pony, she successfully followed instruction and safety protocols. Much of the work with Mia at Bear Spot Farm focused on riding and helping her gain an understanding and awareness of her body. For instance, her clinician carried out rhythm and balance-based exercises with Mia while she rode, to help her gain an effective riding position, and thus communicate with therapy pony better. These exercises set the stage for Mia to be in her optimal window of arousal, evidenced by her decreased impulsivity and heightened sense of control over her body. Mia demonstrated improved functioning and was able to express feeling “free” and “calm” while she rode.
Due to difficulties with transportation and scheduling, Mia was unable to continue her participation in the pilot program. While she continued to receive other services, Mia demonstrated setbacks after exiting the pilot program. Increased impulsivity and oppositional and unsafe behaviors have created challenges for Mia and her family across multiple domains. After treatment, Mia also played with and hid her feces on a few occasions. Mia did not complete enough treatment to reach a second clinical assessment period, thus, no data on her clinical functioning post-treatment is available. |
pmc-6132374-2 | Kari is an 11-year-old Caucasian female who resides in an urban area with her adoptive parents and sibling. Kari has been diagnosed with PTSD, Reactive Attachment Disorder, and intellectual impairment. Prior to adoption, Kari lived with her biological mother and father during which time she was subjected to multiple forms of abuse and neglect. Kari’s biological mother used substances while pregnant, both of Kari’s biological parents were frequent substance users, and her father was incarcerated when Kari was three years old. Prior to adoption, Kari was briefly involved in the foster care system after living in a shelter with her biological mother. Kari’s ongoing trauma symptoms are indicative of severe sexual abuse.
Kari is a kind and nurturing girl who presents with a high rate of distractibility and hyperarousal. Kari becomes dysregulated easily, evidenced by sudden increases in energy, difficulties with boundaries and bodily awareness, and oppositional behavior. Kari often seems unaware of bodily sensations, which inhibits her ability to self-regulate. Though she has an established support network at school, she struggles to initiate and maintain healthy peer relationships. Kari exhibits significant difficulties with interpersonal engagement and communication across multiple life domains. She often becomes oppositional towards family, peers, and providers, and these behaviors have increased with age. Kari has a history of physically assaultive and sexualized behaviors directed specifically towards one of her adoptive parents; however, family reports no reoccurrence of these behaviors in the three years prior to treatment onset.
Prior to the start of treatment, Kari’s parents reported problems at the borderline clinical or subclinical level in several domains of the CBCL, including social problems (T = 68); anxiety Problems (T = 68); oppositional defiant problems (T = 63); anxious/depressed Problems (T = 62); and affective Problems (T = 60). These subscale elevations fell mostly in the internalizing domain (CBCL Internalizing scale T = 60, subclinical range), and were consistent with her clinical presentation of chronic low self-esteem, social difficulties and anxiety. With respect to executive functioning, her parents endorsed on the BRIEF-P clinically significant disruptions in inhibition (T = 70; 97th percentile), as well as subclinical difficulties with monitoring (T = 64; 92nd percentile), or the ability to keep track of one owns behavior and progress toward task completion.
Although Kari did not meet full DSM criteria for PTSD at baseline, on the PTSD-RI, her parents endorsed moderate PTSD symptom levels (total symptom score = 20), with the most symptoms in Cluster D (alterations in cognitions and mood) and Cluster E (alterations in arousal and reactivity). Kari’s parents denied the presence of dissociative symptoms on the CDC-3, and rated Kari as possessing normative ability to reflect upon and discuss her own emotions on the CAM. However, despite her pronounced clinical difficulties in these areas, Kari denied problems with either somatic sensory processes or affective/behavior regulation on two self-report measures (SAM and ADI). This discrepancy between parental appraisal of Kari’s capacity for reflection and Kari’s denial of known emotional and sensory difficulties raised questions about the actual extent of Kari’s emotional awareness or self-insight.
Kari made significant gains while participating in EFT-CT for two years. For approximately the first 18 months, sessions involved Kari retrieving the therapy horse from his paddock, leading him into the barn, and carrying out a grooming routine. Effective grooming requires rhythmic and consistent physical engagement using a series of brushes. While grooming, Kari became regulated and able to engage conversations with her therapist at a level of clarity, organization and fluency beyond that which she was typical capable in her traditional in-home or clinic-based psychotherapy sessions.
Over time, Kari’s tolerance for more difficult conversations about her emotions, social difficulties and energy level also increased during these sessions. This ground work also resulted in a strong attachment between Kari and the therapy horse. In-home and outpatient providers worked with Kari to generalize the relational skills Kari has acquired through this bond to her social engagement and interactions with peers, teachers and family.
Eventually, Kari expressed feeling comfortable enough to begin riding. It took roughly one and a half years of consistent ground work with a therapy horse before she felt safe enough to ride. Through her riding, Kari is able to experience a variety of rhythms as the horse transitions between gaits (walk/trot). This has allowed her to gain an understanding of her body as it pertains to effective riding position and further communication with the therapy horse. Kari has also been able to practice energy modulation in the moment, by asking the horse to move faster or slower. These cues are effectively transmitted to the therapy horse through different types of physical contact initiated by the rider. Kari has had great success with riding, and continues to gain a sense of empowerment from EFT-CT sessions.
The clinician symptom scores collected throughout the duration of Kari’s course of treatment reflect the clinically observed gains described above, but also indicate a non-linear pattern of improvement across a number of domains. The first notable change that occurred was in regard to Kari’s own capacity to reflect on her affective and behavioral functioning. For example, although Kari initially denied having difficulty with behavioral, emotional, or cognitive regulation on the ADI, after three months, she endorsed significant difficulties with both affective regulation (i.e., the ability to modulate the expression and intensity of her emotions) and behavioral regulation (i.e., the ability to control one’s own behavior). Similarly, she initially denied having difficulties with body awareness on the SAM, but by the second assessment period, Kari endorsed significant problems recognizing how her emotions manifest in her body (SAM Body Awareness raw score change from 12 to 3). Recalling that higher scores on the Body Awareness scale of the SAM are indicative of higher functioning and better overall awareness of how one’s emotions manifest in their body, these shifts could be interpreted as reflecting an actual increase in distress or decrease in psychological functioning, perhaps in response to a situational stressor. However, given the consistency of these self-reported ratings with caregiver and clinician reports at baseline, it is more likely these changes reflect increased insight into self, or a new willingness in Kari to acknowledge her difficulties with bodily awareness. As the affective and behavioral dysregulation problems began to decrease, and Kari became more regulated during treatment, she was better able to notice her difficulties in the area of cognitive regulation, or the ability to plan, organize, anticipate outcomes, and learn from experience (ADI cognitive dysregulation symptom count change from 2 to 6).
In addition, there was a subtle linear change in alexithymia and behavioral problems. Throughout the duration of the intervention, Kari demonstrated consistent improvement in her ability to identify and discuss her emotions (CAM scores changed from 13 to 6 from pre-to post-treatment). Regarding her CBCL scores, almost all of the domains in which Kari had subclinical problems at baseline showed improvement throughout the course of treatment, as well. Despite these improvements, there was a notable elevation in Kari’s self-reported depression scores across all domains (CDI-2 Total symptoms T = 73; 99th percentile) after six months of EFT-CT treatment. However, these scores all dropped below the clinical range by the final assessment period. During this same period, there was a similar spike in self-reported PTSD symptoms (PTSD-RI total symptom score change from 20 to 48) and executive functioning difficulties, with new problems emerging in attentional shifting (T = 63; 92nd percentile; subclinical range) and Working Memory (T = 61; 88th percentile; subclinical range). Notably, these too, returned to baseline levels by the last assessment period. The pattern of change in Kari’s clinical symptoms throughout the course of treatment is illustrated in Fig. . |
pmc-6132374-3 | Susan is a 12-year-old Latina female living with her adoptive mother and father in an urban community. Susan is diagnosed with PTSD and several learning disabilities. Susan’s biological mother is a non-biological relative of her adoptive mother. Susan was placed with her adoptive mother after being removed from her biological mother’s care at age 5 due to her biological mother’s substance abuse and prostitution in the home. On occasion, Susan was made to dance for her mother’s male clients. Susan’s adoptive family appears to be supportive, however there is ongoing tension in the home because of her behaviors.
Susan is an expressive, sociable girl that is motivated and willing to try new things. She was referred for equine therapy services to help her develop coping skills for impulsivity, and to solidify positive relationships. Susan has made incredible strides in safely learning the proper ways to handle, manage, ride, and care for the therapy horse. Susan struggles both academically and socially in school. She has made mean and derogatory statements towards peers and has become physically assaultive towards peers and staff on the school bus. In addition, she sometimes demonstrates extreme attention seeking behaviors, which ultimately result in her feeling isolated. Finally, Susan is especially tall for her age, which has resulted in bullying as well as being perceived as intimidating by her peers.
Susan’s baseline clinical measures prior to the start of treatment indicated a wide range of behavior problems as reported on the CBCL (CBC Total Problems T-score = 73), with most problems falling along the externalizing spectrum (T = 74). However, subclinical internalizing problems (T = 62) were present on the CBCL as well. Per parent report, Susan had clinically significant scores on measures of CBCL aggressive behavior (T = 80), social problems (T = 77), attention problems (T = 73), and thought problems (T = 71); and borderline clinical scores on rule-breaking behavior (T = 69); and anxious/depressed problems (T = 67). Her mother also reported a number of clinically significant problems related to executive functioning on the BRIEF-P prior to treatment. These included problems of equal concern across both the metacognition and behavioral regulation domains, resulting in a Global Executive Functioning composite score in the clinically significant range (T = 75; 98th percentile). Specifically, Susan exhibited the most difficulty with inhibition (T = 90; 99th percentile) and monitoring (T = 88; 99th percentile). Notably, her mother also endorsed clinically significant symptoms of dissociation on the CDC-3, with her scores consistent with a dissociative disorder. However, Susan evidenced relative strength in terms of her ability to identify, reflect on, and discuss her emotions, as indicated by the CAM.
Despite her observed difficulties with aggression, prior to treatment Susan did not endorse problems in this domain. For example, while she endorsed problems with cognitive regulation on the ADI (i.e., problems with planning, organizing, and learning from her experiences), she denied difficulties with emotion and behavior regulation on this same measure. Susan also reported some difficulties with body awareness and sensitivity on the SAM. In addition, Susan denied clinically significant depressive symptoms, but did acknowledge difficulties in two domains of the CDI-2, including ineffectiveness (T = 67; 96th percentile) and functional Problems (T = 66; 95th percentile). This indicates that while Susan was not experiencing depression as a clinical syndrome at the start of treatment, she was experiencing significantly negative views of her own abilities and academic performance, as well as difficulty effectively engaging in daily tasks. Lastly, Susan endorsed some trauma-related difficulties on the PTSD-RI, including elevated problems with altered cognitions and mood and some problems with altered arousal and reactivity. She indicated that some of her predominant concerns were that the world feels dangerous, she feels that some part of her trauma was her fault, feelings of shame, difficulty sleeping, feeling jumpy and frightened, and startling easily. However, Susan did not meet criteria for a diagnosis of PTSD at the start of her treatment.
EFT-CT sessions at Bear Spot Farm with Susan have strongly emphasized safety. Through her grooming routine and riding, she has gained insight on how her behavior impacts the therapy horse and vice versa. Susan is very motivated to ride but becomes anxious and doubtful upon mounting the therapy horse. With coaching and support, she has been able to recognize that leading the therapy horse around the arena a few times prior to getting on is helpful in reducing anxiety. Building routines and rituals specific to Susan’s needs has further allowed her to excel, providing her with a sense of competency and empowerment. With these supports, she has been able to express her feelings openly and process emotions related to her trauma history.
This was especially pertinent after a trip she took with her adoptive parents to visit her biological parents. While on the trip, Susan spent time with her biological mother and father, who were dismissive of her. Upon return to EFT-CT sessions, she disclosed this during the riding portion of her session. Once mounted on the therapy horse and comfortably walking around the arena with close support from clinician, Susan stated that she was able to see her biological parents while away, then added that it wasn’t a good time. She was able to engage in a discussion linking her unsafe and anxious feelings with her interactions with her biological parents. In this moment on the horse, it became evident that Susan is more regulated and seemingly better able to discuss difficult experiences. Susan noted that she is aware that her biological parents were never able to do a good job of keeping her safe, and that feeling unsafe exacerbates her anxiety. Susan went on to express a newfound sense of connection and appreciation for her adoptive mother who she notes is better able to keep her safe. Susan continues to show receptivity to exploring the linkage between her history and how it is affecting her today.
Clinical measures completed throughout the course of treatment indicate that Susan demonstrated some notable patterns of improvement. For example, Susan’s behavior problems steadily decreased throughout the period of intervention, as reported by her mother on the CBCL. In several domains of behavior on the CBCL, her scores dropped by over one standard deviation and at times dropped out of the clinical range, indicating significant clinical improvement. These include anxious/depressed problems (T = 51); aggressive behavior (T = 65) and anxiety problems (T = 55). She also made gradual but notable improvement in social problems (T = 68), thought problems (T = 64) and conduct problems (T = 65) on the CBCL, all of which moved from the clinically significant range to the subclinical range. In addition, her scores on scales measuring CBCL somatic problems (T = 59) and oppositional defiant problems (T = 59) moved from the subclinical range to the nonclinical range. Attention problems remained a current and significant concern, with current scores similar to those observed at baseline (T = 73).
Similar gradual but consistent improvement was also observed throughout the course of treatment with regard to Susan’s executive functioning. Two scores on the BRIEF-P moved below the clinical range (Shift, T = 57; 79th percentile, and Emotional Control, T = 56; 75th percentile) and many of her scores on this measure dropped one standard deviation or more over the treatment period, including inhibition (T = 75; 98th percentile), working memory (T = 64; 90th percentile), and monitoring (T = 64; 92nd percentile). As such, Susan’s overall Global Executive Functioning Composite improved by one standard deviation, though it remained in the subclinical range, suggesting that while she made significant and broad gains in her executive functioning abilities there is a need for continued work in this domain.
The trend of gradual and consistent improvement was also observed in symptoms of specific clinical syndromes, with self-reported problems of ineffectiveness and functional problems related to depression both dropping out of the clinical range on the CDI-2 (T-scores of 53 and 52 respectively; 61st and 58th percentiles respectively). In addition, there were self-reported decreases in total PTSD symptoms on the PTSD-RI (PTSD total symptom score change from 34 to 22) and dissociative symptoms on CDC-3 (raw score change from 24 to 7). Areas in which little change was observed throughout the course of treatment included attention problems (as indicated above), as well self-reported problems with cognitive regulation on the ADI scale, and somatic sensory sensitivity on the SAM. The pattern of change in Susan’s clinical symptoms throughout the course of treatment is illustrated in Fig. . |
pmc-6132594-1 | Here, we present a case of a 55-year-old Caucasian female with a history of substance use disorder and a comorbid bipolar disorder, who presented to the local general hospital with a history of the fragmentation of a single personality into different personalities under emotional stress and under the influence of a drug. Multiple aspects of her personalities were reported, including the following: a personality of a seven-year-old child, a personality that would behave as a teenager, and another that acted like a male person in addition to her normal 55-year-old personality. She reported that she had been constantly dominated by her alternate personalities and became aware of their existence when people around her informed her, usually after a situation ended. She reported that stressful situations and substance abuse could aggravate the fragmentation of her personality. This was found to be mostly an involuntary phenomenon with seldom memory of the event.
While transitioning between these personalities, she was found to be violent even to people who were close to her. This could range from being suicidal to homicidal for which she was arrested twice in the past. She had to be isolated and restrained by being locked in a room and calling the police. As a result, she was hospitalized in a mental institution for a significant period at least two to three times in the past. Under the influence of stress or substances like marijuana or cocaine, her personality would split into various personalities. These states were very different from one another in terms of age or gender.
One of her alternate personalities behaved as a seven-year-old child and would show the same interests and choices that included becoming moody or a self-arrogant personality. While in these states, she could hurt herself or had weeping spells if her wants were not met.
Another personality acted as a teenager with some sharp choices and dressing. Increase in substance abuse, alcohol use, and smoking would lead to multiple cases of fights or homicidal attacks, with some incidents of self-harming events. Multiple scars were found on the dorsal side of her right hand. Her speech was found to be pressured and she would repeat the same words/ conversations.
The next personality was diagnosed to be a temporary transition to the opposite gender (a male). There was a change in voice and behavior. This included male dressing, language, a perception of male body parts, choices of friends, and attraction towards females, including sexual behavior.
The normal state of a 55-year aged female was the default personality that made her feel most comfortable. She reported that she had anxiety during a personality state transition, as it could occur at any time, and involuntarily, but mostly in stressful situations and during substance abuse. More violent and harmful events were reported when someone tried to meet the patient alone rather than in a group.
The treatment included psychotherapy with cognitive behavioral therapy addressing stress and substance use disorder. The psychotherapeutic treatment lasted for at least six months. The dual treatment of drug therapy was also involved to calm her down. The patient was prescribed escitalopram to reduce her anxiety symptoms. She believed that the anxiety pills were really helpful. After six months, the patient's condition was not drastically different. However, she believed her stress was getting better. The patient was further followed up for the next one year and the treatment continues to date. |
pmc-6132596-1 | A 10-year-old boy presented to our outpatient department with complaints of multiple swellings in the back along with swellings in both the arms since last eight years. He was born out of a normal vaginal delivery at term and the perinatal period was uneventful. His parents noticed deformity in both great toes at birth, without any other associated anomalies. The child attained all his developmental milestones without any developmental delay and was apparently alright till two years of age. At two years, his parents noticed a gradually increasing painless swelling on the posterior aspect of his left proximal arm associated with restricted extension of the left elbow joint. The child was operated for the swelling by an orthopaedic specialist, but the restriction in movements persisted and the swelling recurred.
Six months later, a gradually increasing painless swelling was noticed in the right arm, followed subsequently by restricted movements of the right elbow. Gradually, multiple swellings appeared over bilateral infrascapular regions. His shoulder movements also became restricted with time leading to severe limitation of his activities of daily living. At the presentation, the child had difficulty in feeding himself along with an inability to dress and undress. On examination, multiple small, irregular, non-tender, bony hard swellings were present over bilateral parascapular and infrascapular regions extending up to the lower dorsal spine (Figure ).
All the shoulder movements were found to be severely restricted bilaterally with 10° flexion and abduction movements. Non-tender bony hard swellings could be palpated in both the arms primarily on medial and posterior aspects, which were associated with fixed flexion deformity of 90° at both the elbow joints with a further 10° flexion movement possible (Figure ).
Also, there was a severe restriction of the neck flexion and extension movements (Figure ).
The hip movements were also severely restricted on both sides allowing just up to 60° flexion causing restriction in sitting properly on a chair and inability to squat and sit cross-legged. Besides, there was a small, irregular, bony hard swelling palpable on the lateral aspect of the right distal thigh. On examination of the feet, there was hallux valgus present bilaterally along with microdactyly of the great toes (Figure ).
Routine laboratory investigations including complete blood counts, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and all other biochemical parameters were unremarkable.
On plain radiographs of the chest with bilateral arms, heterotopic ossification was seen in the soft tissues around humerus on both sides, extending through the axilla to the chest wall (Figure ).
Heterotopic ossification was also noted along left side of the neck on radiograph of cervical spine (Figure ).
Radiograph of the pelvis with bilateral hips revealed broadening of femoral neck with bridge-like heterotopic ossifications extending across both the hip joints as well as lateral to the right iliac bone (Figure ).
Plain radiographs of the knees showed bony outgrowth like appearance due to ossification along ligamentous insertion on right lateral distal femoral metaphyses as well as bilateral proximal medial tibial metaphyses producing pseudoexostoses (Figure ).
Radiographs of both the foot revealed bilateral hallux valgus with monophalangism of the great toes (Figure ).
Based on the presence of congenital bilateral great toe anomalies and progressive widespread heterotopic ossification in the characteristic anatomic pattern, a clinical diagnosis of FOP was arrived at. The patient’s relatives were counselled about the prognosis and lack of definite treatment and cure for the disease. They were also informed about the presence of support groups like International Fibrodysplasia Ossificans Progressiva Association (IFOPA) to advocate, support and connect patients, and their families, afflicted with this debilitating disorder. |
pmc-6132678-1 | A 63-year-old man presented with a 2 x 2 cm mass on the lateral side of his right elbow. Previously, he had visited a nearby doctor, and was then referred to us for a surgical consultation. After his surgical consultation, the patient underwent a marginal resection of the mass (Figure ). The histological analysis revealed an atypical lipomatous tumor (data not shown). After the resection, the patient was assessed with magnetic resonance imaging (MRI) which showed no remains of the tumor (Figure , ).
Approximately 10 months following this resection, tumor recurrence was identified on MRI (Figure , ). He was then referred to our hospital for a surgical consultation. We performed a wide resection. The patient had received no targeted therapy, chemotherapy prior to resection or after.
The resected specimen was yellow-white in color and had a hard, elastic texture (Figure ). A histological analysis of the resected specimen revealed an atypical lipomatous tumor (Figure ), and the tumor margin was negative.
One year following this surgery, the tumor recurred as indicated on MRI imaging (Figure , ). Again, we treated the patient by performing an extended resection. This time, the histological analysis revealed a proliferation of atypical lipomatous cells and high-grade spindle cells (Figure ).
We diagnosed dedifferentiated liposarcoma based on the histological findings. So far, two years have passed, and no recurrence has been observed. |
pmc-6132802-1 | A 75-year-old Iraqi male presented to the surgery clinic with chronic abdominal and right inguinal pain. He did not have a history of any surgery or trauma and was taking amlodipine 10 mg for hypertension. On examination, the patient’s blood pressure was 135/95 mm Hg, heart rate was 62 bpm, and body temperature was 36.8°C. His abdominal examination did not reveal any signs of rigidity, rebound, or pulsatile mass. The laboratory examination findings were: leukocyte: 7300/mm3(4600-10200/mm3), c-reactive protein: 2.7mg/dL (0-5 mg/dL), potassium: 3.8 mmol/dL (3.5-5.1 mmol/dL), aspartate aminotransferase: 34U/L (5-34 U/L), and alanine aminotransferase: 205 U/L (0-55 U/L). Plain abdominal X-ray and ultrasonography did not reveal any abnormality. Intravenous contrast-enhanced computed tomography (CECT) scan revealed a calcified mass that was 60x52 mm in size and localized between the right internal iliac artery and urinary bladder (Figure ). The Tru-cut biopsy of the mass was undiagnostic and did not reveal if the mass was benign or malignant. It was decided to take the surgical approach. An explorative laparotomy was performed with a vertical midline incision. A lesion that was 5x4 cm in diameter and localized in the retroperitoneal area, between the right internal iliac artery and the urinary bladder, was excised (Figures -). The frozen section pathological examination of the mass revealed that it was a pelvic hydatid cyst. Definitive pathological results also confirmed the results of the frozen section. On the first day after the surgery, a liquid diet was given. His vital signs were normal on follow-up, and he was discharged uneventfully on the fourth day of the operation. Albendazole 200 mg/day was prescribed to the patient. |
pmc-6132972-1 | A 16 year old girl presented with severe headache and visual loss, upon further questioning she also had primary amenorrhoea and galactorrhoea. An MRI [Fig. ] identified an apoplectic suprasellar tumour with a prolactin of 10,919 miU/L. After a week of treatment with cabergoline her vision had not improved so endoscopic trans-sphenoidal surgery (ETSS) was performed. Post-operatively, her vision returned to normal and she had no further galactorrhoea. 6 years later she has had no recurrence and has a normal prolactin. Histology confirmed pituitary apoplexy with expression of prolactin in a diffuse pattern. The Ki67 proliferation index was low. |
pmc-6132972-2 | A 14 year old boy was admitted with headaches and weight gain. At presentation he had only perception of light in his left eye and hand movements in the right eye. His MRI showed a pituitary tumour extending suprasellarly to compress the chiasm with features of apoplexy. The initial prolactin was 10,626 miU/L. Given his very poor visual function, he underwent emergency ETSS. Post-operatively his vision improved but he remains with significant visual impairment. Histology confirmed a prolactinoma with a moderate Ki67 (5%) and some mitotic activity. He remains on cabergoline although his subsequent MR imaging shows minimal residual disease. |
pmc-6132972-3 | A 15 year old boy was admitted with severe headache, reduced visual acuity and a sixth nerve palsy. Following an MRI he was diagnosed with a large pituitary macroadenoma with features of apoplexy. His prolactin was raised at 47,173 miU/L and he was commenced on cabergoline. His acuity had some improvement, however his sixth nerve palsy remained. Given his on-going visual symptoms, he underwent ETSS 6 weeks later. Post-operatively, his vision returned to normal with no ophthalmoplegia. Histology confirmed a prolactinoma with a low Ki67 and no up-regulation of p53. |
pmc-6132972-4 | A 15 year old boy initially presented with visual deterioration, galactorrhoea and weight gain to another neurosurgical unit. MRI showed a large pituitary tumour. He had a prolactin of 67,000 miU/L and was managed conservatively with cabergoline. His follow-up showed evidence of tumour reduction and prolactin reduced to ~ 10,000 miU/L. He presented to our unit almost a year later with sudden onset severe frontal headache and significant bi-temporal hemianopia. His MRI showed an enlarged apoplectic pituitary tumour with considerable chiasmal compression. Given the severity of his visual deterioration he underwent emergency ETSS. Post operatively his vision improved but recovery was complicated by transient diabetes insipidus, which settled over a few days. Histology confirmed pituitary apoplexy within a prolactinoma. No mitotic activity was seen. |
pmc-6132972-5 | A 13 year old girl presented with a 6 month history of headache and right temporal hemianopia. Her family were also concerned that this previously very studious girl was struggling at school. Her MRI showed a suprasellar apoplectic tumour with a prolactin of 30,824 miU/L. She was started on a dopamine agonist and monitored as an outpatient. During this time her prolactin returned to normal and she was monitored with regular visual field testing and monitoring of her prolactin. After a year she presented again with deteriorating vision. MRI showed an enlarging apoplectic tumour despite a prolactin of 63 miU/L. She therefore underwent ETSS to prevent any further visual loss. Post-operatively, both her vision and her academic performance have returned to normal. Histology confirmed a prolactinoma with apoplexy. The Ki67 and p53 labelling were not elevated. |
pmc-6133044-1 | A 3-year-old boy (body weight 17 kg) presented to a regional hospital with a 5-day history of cough and febrile illness. He was diagnosed with left-sided pneumonia and started on intravenous antibiotics (Ampicillin). The boy was previously healthy and up to date with his immunizations (including Streptococcus pneumonia). Due to worsening respiratory distress he was transferred to our pediatric intensive care unit (PICU) the next day. He developed acute respiratory failure, followed by endotracheal intubation and invasive ventilation. The chest X-ray (CXR) showed an effusion and pneumothorax on the left side which required the placement of two chest drains. Streptococcus pneumonia was isolated from the chest drain fluid and Influenza H1N1 from a tracheal aspirate via polymerase chain reaction (PCR). A chest computer tomography (CT) showed a severe necrotizing pneumonia of the left lung with destruction of lung parenchyma and formation of a large abscess cavity in the lower lobe, as well as consolidation of the right lung (Fig. ). A trial of high frequency ventilation and inhaled nitric oxide did not improve oxygenation and was abandoned. Despite ventilation with high peak inspiratory pressure (PIP = 40 cm H2O), high positive end-expiratory pressure (PEEP = 10 cm H2O) and FIO2 100%, hypoxemia and hypercapnia became worse (oxygen saturation (SpO2) = 75–80%, arterial blood gas: pH = 7.28, paO2 = 38 mmHg, paCO2 = 127 mmHg), thus fulfilling the criteria for severe ARDS (PaO2/FiO2 = 38 mmHg, oxygenation index = 52.6) after excluding acute cardiac dysfunction. Consequently, venovenous extracorporeal membrane oxygenation (VV-ECMO) was implemented on day 19 of mechanical ventilation. A 15 French access cannula was placed into the left femoral vein and a 14 French return cannula into the right internal jugular vein. Initial ECMO settings were blood flow of 2 L/min, RPM 3000 and gas sweep of 1 L/min which immediately increased his SpO2 to 93%. Mechanical ventilation was reduced to lung rest settings (PIP 20 cm H2O, PEEP 10 cm H2O, respiratory rate 10/min, FIO2 30%). Over the next 4 weeks our patient showed basically no lung aeration, depending completely on extracorporeal support. His tidal volumes (TV) were 5–8 mL, which is less than 0.5 mL/kg (Fig. ). Additionally, due to insufficiency of the venous cannula we had to reduce flows and accepted SpO2 around 75% while keeping hemoglobin levels over 14 g/dL. Next, we started several measures to promote lung recovery, namely daily prone positioning for 12–16 h, twice daily inhalation with DNase and 2–3 toilet bronchoscopies per week to remove necrotic endobronchial material and to reduce its viscosity. This led to an increase in TV to 35 mL (2 mL/kg) by week five, but finally to 100 mL (6 mL/kg) on day 41, together with areas of re-aeration on CXR. Forty-eight hours later our patient was successfully decannulated (total VV-ECMO time 43 days). During his entire ECMO run, our patient was in single-organ system respiratory failure without exhibiting any serious complications. Over the next 3 weeks, ventilation was slowly weaned as his native lung function continued to improve (total mechanical ventilation time 88 days). He was subsequently discharged from PICU and home 4 weeks later without additional oxygen. Because we hesitated to expose him to the risk of a both general anesthesia and extended surgery, we decided not to proceed to a surgical resection of the necrotic area, although the large abscess was still visible on repeat chest CT. Our patient is now 1 year post discharge and has normal oxygen saturation in room air and has fully recovered. His latest CXR showed only small residual parenchymal changes and a thickening of the pleura of the left lung (Fig. ). |
pmc-6133394-1 | A 48-year-old woman was found to have a solid mass in the right kidney, via ultrasonography during a regular physical examination. She did not complain about backache, abdominal pain, urinary irritation, hematuria, or dysuria. She had no history of tuberous sclerosis (TSC). The physical examination showed no eminence or tenderness in the costovertebral angle, hypochondriac point, or ureteral point. Laboratory examination did not show any abnormality.
The computed tomography (CT) scan revealed a well-defined solid tissue mass in the right kidney that suggested renal cell carcinoma (Fig. A). The patient received a radical right nephrectomy without any radiochemotherapy.
After the nephrectomy, gross examination showed that the kidney parenchyma was partially replaced by a tumor with a volume of 7.5 × 6 × 4 cm3. The dissected surface had a solid and soft texture with vague boundary, and a colorful appearance due to necrosis and hemorrhage. Histochemical staining showed that the tumor contained a large portion of necrotic tissue and atypical epithelioid cells with abundant eosinophilic or granular cytoplasm (Fig. B). These epithelioid cells were scattered within the tumor, or organized closely in nests separated by glassy collagen fibrils (Fig. A). The tumor cells possessed more than 1 round-to-oval atypical nuclei, with irregularly distributed coarse chromatin and prominent nucleoli (Fig. C). The mitotic count was about 2 in 50, under high power field (HPF; Fig. D). Regretfully, the tumor cells were found infiltrating into the surrounding renal parenchyma.
Immunohistochemical staining showed that the tumor cells tested positive for MelanA (Fig. F), were focally positive for HMB-45 (Fig. E) and vimentin, and 10% positive for Ki67. Tests for the following were negative: SOX-10, S-100, RCC, CD10, PAX8, PAX2, SMA, desmin, caldesmon, CK, TFE3, CD56, Syn, CgA, P53, and E-cadherin. Based on these findings, malignant EAML was diagnosed.
Thirteen months after the nephrectomy, CT detected a mass in the liver that displayed the same features as the previous tumor in the right kidney. It was speculated that the liver mass was a metastasis from the kidney lesion. No pathological examination was performed for the liver metastasis because the patient refused to provide a biopsy. She also refused to receive any more treatment due to economic stress. |
pmc-6133394-2 | A 62-year-old man presented with an untreated left backache of 1 year's duration. The regular physical examination revealed, on ultrasonography, a cystic lesion in the left kidney. A CT scan revealed a cystic solid tumor in the left kidney (Fig. B). The kidneys were not palpable under the rib; and no percussion pain or tenderness was detected in the kidney region or ureteral point. He also had no history of TSC. The laboratory examination did not show any abnormality. As requested by the patient, he was treated with a radical left nephrectomy without adjuvant therapy. Ten months after surgery, the patient was living well without any signs of disease.
Gross examination of the kidney after the nephrectomy showed that the tumor was a well-circumscribed mass with a volume of 7 × 5 × 3.5 cm3. The tumor was composed of multiple cysts that contained hemorrhagic necrotic tissue wrapped by a thick cystic membrane. Histology showed cavities of various size in the tumor, surrounded by thick membrane composed predominantly of atypical epithelioid cells with abundant eosinophilic cytoplasm, irregular nuclei, marginal aggregation of chromatin, and prominent nucleoli (Fig. A and B). Mitotic figures were counted as 2 per 50 under HPF. In some areas, there were frequent spindle cells arranged in bands, and a large number of slender vessels wrapped by thin membrane were observed in the stroma of the tumor (Fig. C). Lymphovascular invasion was also seen in the cystic wall (Fig. D).
Immunohistochemical staining confirmed that the epithelioid cells were strongly positive for MelanA (Fig. E), and focally positive for HMB-45 and vimentin. Tumor cells were negative for SOX-10, S-100, RCC, CD10, PAX8, PAX2, CK, TFE3, CD56, Syn, CgA, P53, and E-cadherin. Ki67 was positive in about 10% of epithelioid cells. In addition, spindle cells were positive for SMA (Fig. F) and focally positive for caldesmon and desmin. Therefore, the final histopathological diagnosis was malignant EAML. |
pmc-6133411-1 | A 30-year-old woman without significant history underwent ultrasonography, which revealed 2 benign-appearing masses in the left breast. VAR of the masses was performed using an 8-G needle under ultrasonic guidance. Histology confirmed fibroadenomas.
Three months later, the patient returned for a follow-up visit, complaining of a painful, enlarging lump in her left breast. Clinical examination revealed a pulsatile mass with a continuous machinery murmur in the outer upper quadrant of the left breast. Color Doppler imaging demonstrated an anechoic lump (45 × 26 × 33 mm) fed by an afferent artery; the lump showed a yin-yang appearance and biphasic “to-and-fro” pattern (Fig. ). A fistula was situated 3-mm proximal to the anechoic lump, connecting the afferent artery and a concomitant vein (Fig. ). Based on these findings, the patient was diagnosed with breast PA-AVF.
Multidisciplinary consultation concluded that the lesion posed a risk of rupture and should be excised and ligated under local anesthesia as soon as possible. A 3-mm operative incision was made along the direction of the vascular route under ultrasonography guidance. The incision was at 1 o’clock position, 7-cm from the nipple, and was concealed to prevent a cosmetic defect. The proximal and distal artery and vein of the PA-AVF were ligated. The PA-AVF was excised. The entire operation was very simple and time-efficient. The mean bleeding volume was only 5 mL. At the 1-month follow-up, the wound was found to have healed well, and ultrasonography confirmed disappearance of the breast PA-AVF. |
pmc-6133429-1 | A 19-year-old, previously healthy, female was accidentally crushed by truck resulting in left hip hemipelvectomy, left hip stump bleeding on April 27, 2013. She was treated with compression bandage in the emergency department of the local hospital and transferred immediately to our hospital, it took postaccidental 3 hours to reach our hospital. Initial examination on arrival showed she was in shock with indifferent consciousness, her left leg was mangled and nonviable with left hip stump dressing. Immediate antishock treatment initiated with wound compression bandage, hemostasis was achieved, iv fluids and blood transfusion initiated, oxygen given, the patient was under continuous electrocardiography monitoring and other symptomatic treatment. She was resuscitated from shock and emergency consultation was done with gastrointestinal surgery, vascular surgery, burns and plastic surgery, and orthopedic surgery (Fig. ).
Systemic examination was normal except her left lower abdominal wound margin extending to intestine and bladder. Her orthopedic injuries included amputated left pelvis, left lower pelvic organs were protruded out and wrapped by peritoneum, all of the vulva and anus were torn, the wound area was contaminated and actively bleeding, the right groin and perineal skin contusion with extensive skin abrasions, right knee and medial part of first great toe skin abrasion. The right hip joint, knee joint, and ankle joint with no obvious deformity but limited passive activity (Fig. ).
Emergency radiograph and abdominal CT showed left sciatic iliopectineal and left lower limb loss, rough cutting edge, local skin tear with multiple dense punctuate shadow. The left middle upper abdominal wall was swollen and accumulating. L3-5 left transverse process fracture, L5 vertebral right transverse process fracture, bilateral sacral wing fracture, right acetabulum fracture, comminuted fracture of a superior, and inferior branch of the right pubic ramus. There were contusion and laceration of the soft tissue of the pelvic floor, with the pelvic wall and the pelvic cavity scattered in the gas accumulation. Small compact shadow beside the caudal vertebra, ruled out the fracture of the caudal vertebra (Figs. and ).
The patient and her family were explained about patient condition, the treatment modality, the possible complication, and encouraged for their active participation. After resuscitation from shock and emergency consultation with gastrointestinal surgery, vascular surgery, burns and plastic surgery, and department of orthopedics, considering her current critical condition, consent was taken for current treatment of stump wound debridement and suture drainage + laparotomy transverse colostomy, as a part of multistage surgical treatment for her condition. In operating theater, after successful general anesthesia, she was positioned in right lateral position. After drapping with aseptic technique, the wound was rinsed with hydrogen peroxide and saline to remove the contamination. After emergency ligation of the left internal and external iliac artery, excision of necrotic wound edge was done than intermittent suture for anal and perineal tear, and the continuous suture was applied for left abdominal wall large defect. There was minimal bleeding during this procedure. Then the patient was positioned supine, after confirming adequate anesthesia drapping was done with aseptic technique, the abdominal cavity was explored through a midline incision. Liver, spleen jejunum, ileum, and cecum were explored, that showed no obvious abdominal injury. The transverse colostomy was done and the abdominal cavity was closed. There was also minimal bleeding during this procedure. She received an intraoperative and postoperative blood transfusion. Considering her critical condition she was transferred to intensive care unit (ICU) for postoperative care and ventilator support. She was taken over by physician and team for ICU management (Figs. and ).
Department of Orthopedics, gastrointestinal surgery, burns and plastic surgery, urosurgery, obstetrics, and gynecology were involved for proper monitoring of wound dressing, colostomy care, prevention of complication, and to strengthen patient communication. Parental nutrition was started with adequate electrolyte supplement. She developed a fever on the first postoperative day than bedside chest X-ray was taken that showed normal pleural condition, blood coagulation report and lactic acid status evaluated were normal, and the specialist was consulted to evaluate wound and stoma. Gentamycin, cefuroxime, and metronidazole were discontinued and piperacillin-tazobactam combined with the hepatoprotective drug was initiated. Fever subsided in days, nutritional support was started and increased gradually in days from parental than small oral glucose + small calorie parental than the addition of fat emulsion as tolerated. She was weaned from the ventilator and finally extubated. She was transferred to the orthopedic ward for further management. Excision of necrotic tissue and dressing were continued and when wound showed some granulation, it was assisted by burn and plastic surgery team. Meanwhile, the patient developed abdominal pain resolved by temporary fasting, antacid, and rehydration and ontrast enhanced computed tomography scan taken after gastrointestinal surgery consultation was normal. On the postoperative 22nd day, she developed intermittent fever subsided with antipyretic and oral cotrimoxazole, wound examination showed minimal bleeding and more exudates. Blood examination showed increase in alanine transaminase (178 IU/L), aspartate transaminase (126 IU/L), decrease albumin (27.9 g/L), total protein (47.3 g/L), hemoglobin (HB; 105 g/L), and hematocrit (0.23 L/L) with other in normal range. Now the patient was coordinated with infectious disease department, hematology department, and burn and plastic surgery department. Piperacillin-tazobactam was discontinued, and cefoperazone-sulbactam was enabled with a hepatoprotective drug to reduce glutathione and electrolyte correction continued.
Next day, blood transfusion was planned as patient appeared pale but the patient was transferred to ICU after worsening of condition: decrease blood pressure, increase pulse: 123 bpm, respiratory rate: 22 to 43/min, the wound dressing of left perineum were percolated. Emergency blood gas analysis showed: HB 4.0 g/L, partial pressure of oxygen 54 mm Hg, sodium 132.7 mmol/L, chlorine 113.3 mmol/L, lactate 4mmol/L, whole blood base 3.0 mmol/L. With positive transfusion therapy and noninvasive ventilator-assisted ventilation, antibiotic, electrolyte correction, nutritional support, rehydration, daily dressing, and other symptomatic and supportive treatment, the patient condition was stabilized and transferred back to the orthopedic department. She complained of phantom limb pain which was managed with gabapentin and methylcobalamin. The regular dressing was continued and when wound appeared fresh burn and plastic surgery department were consulted.
In the postoperative 39th day after abundant granulation tissue was seen, the case was discussed with the department of burn and plastic surgery and a skin graft was planned. Next day, free skin graft transferred to left lower abdomen from right anterior thigh (Figs. –). Examination showed the left lower abdominal wound as 20 cm × 10 cm and right groin had 6.0 cm × 4.0 cm granulation wound that was healthy with minimal secretion. There was perineal secretion for few days which was dry after perineal hygiene. Eighteen days later, the patient was transferred to physiotherapy and rehabilitation department. Therapy was initiated to improve the strength of right lower extremity and improve the ability of adductor longus, enhanced upper limb muscle strength training. Tramadol and Rui Calgary music therapy were given for phantom limb pain.
With 6 months of interval rehabilitation therapy patient daily life ability score improved from 35 to 96 out of 100, examination showed the amputation site wound of 15 cm × 10 cm with little yellowish graft exudates, on the lateral side of the right thigh, there was about 10 cm × 10 cm of scab wound with limited function of right hip and knee joint. Right groin had 20 cm × 5 cm abrasion which was dry and healthy, there was artificial stoma in the abdomen with colostomy margin red and humid, no break and covered with pocket, with surrounding skin clean and dry. The patient complains of occasional phantom limb pain significantly affecting sleep which was treated symptomatically and with methylcobalamin, occupational therapy, and rehabilitation training. She could wear prosthetic limbs and walk with the aid of walking aids. Her colostomy was anastomosed with anus a few months later by the department of gastrointestinal surgery.
Recently she is unemployed, unmarried stays with her parents who take care of her. She complains of phantom limb pain not responsive to analgesic, so she does not use it. She has a prosthetic leg but does not prefer it as it is inconvenient to walk with it. She usually walks with single crutch up to 100 m. |
pmc-6133563-1 | A 37-year-old woman physiatrist presented with 8-week history of persistent low back and left buttock pain with difficulty in walking and sitting. Symptoms had started at 34 weeks of pregnancy. She reported similar spontaneously resolving complaints during her previous pregnancy which were attributed to sciatica. Both pregnancies were delivered with cesarean section. Neither history of trauma nor strenuous physical activity was noticed. On examination, the patient was apyretic, 164 cm tall and weighed 48 kg. There were pain and mild tenderness on palpation of the left superior gluteal area. Pain was aggravated during provocative sacroiliac joint maneuvers. Lumbar spine was slightly painful at extension and lateral bending. Neurologic examination was normal. Laboratory investigations were unrevealing with normal values of erythrocyte sedimentation rate, blood calcium and phosphorus levels and kidney and thyroid tests.
Pelvic and lumbar x-rays revealed no bone abnormalities. Pelvic CT scan revealed degenerative changes concordant with a left mechanical sacroiliac joint (SIJ) disease (Fig. A). A subsequent MRI showed a hypointense oblique line with marrow edema on the left side of the sacrum consistent with a stress fracture (Fig. B). Treatment consisted in relative rest and pain killers and resulted in favorable outcomes. Our case was waived from ethical approval according to our institutional ethical committee. An informed consent was obtained from the patient. |
pmc-6133579-1 | Informed consent was obtained from the individual participant in the study.
A 40-year-old woman who worked as a peasant farmer in Shandong province, 300 miles from Beijing, China, was referred to a dermatology clinic within that province because she had 3 patchy rashes (Fig. A–C). One year earlier, she had 3 large patchy rashes—1 on the hip and 2 on her lower limbs. The patch on the hip was wide, raised, and erythematous with well-defined margins sloping toward the center of the lesion. The erythematous patch on the dorsum of her foot had sharp edges with thick granular margins and small satellite lesions. A skin biopsy was performed and revealed a noncaseating epitheloid granuloma (Fig. A). On the basis of this observation, we diagnosed the patient as having cutaneous sarcoidosis. She was prescribed some herbal medicine for 1 month, after which she felt aggravated and visited the hospital again. Then a skin biopsy from the patch on her right leg was obtained. Similar to the previous biopsy, the result showed numerous noncaseating epithelioid granuloma cells consisting of histiocytes and giant cells surrounded by lymphocytes infiltrating the adnexa structures (Fig. B, C). Results of the Ziehl-Neelsen staining and polymerase chain reaction (PCR) test for Mycobacterium tuberculosis were negative, and routine laboratory screening test results for the hemoglobin level, leukocyte count, and liver and renal parameters were all within normal range. Because of the possibility of sarcoidosis, plasma dipeptidyltransferase and calcium levels were measured, and chest radiography was performed. All these test results were also normal. Consequently, leprosy was suspected, and the patient was referred to Beijing Tropical Medicine Research Institute (BTMRI) for a formal assessment.
By the time of referral to BTMRI, the 3 patches became asymmetrically distributed on the patient's hip and lower limbs, while no patches were founded on her trunk or scalp. All the patches were not sensitive to light touch, and the patient was unable to discriminate between hot and cold temperatures in these areas. The eye examination was normal, and there was no enlargement of the nervus auricularis magnus. Subsequently, SSS with acid-fast staining was performed at 5 different sites: both earlobes, both eyebrows, and the chin. Results of the microscopic evaluation were negative. Therefore, M leprae-specific real-time PCR and enzyme-linked immunosorbent assay were also performed to detect serum antibodies against phenolic glycolipid (PGL)-I and leprosy IDRI diagnostic (LID)-1. The results of real-time PCR for an M leprae-specific target was positive, whereas the antibody responses against LID-1 and PGL-I mimetic NDO-BSA were positive but weak (OD >0.05) (Table ). On the basis of the patient's clinical presentation and results of the assays, we diagnosed the patient as having BT leprosy. Accordingly, we prescribed her with dapsone (100 mg daily) and rifampicin (600 mg monthly) for 6 months in accordance with the WHO recommendation for treating PB leprosy.
The skin lesion was markedly improved by completion of the 6-month multidrug therapy (MDT) (Figs. and A, B), providing a retrospective conformation of the diagnosis of BT leprosy. |
pmc-6133587-1 | We present the case of a man in his 60s who experienced atypical docetaxel-induced RRD. Although his medical history comprised a surgical intervention for a duodenum ulcer, he had no significant history of collagen vascular diseases. He was administered radiotherapy for high-risk prostate cancer after 1-year hormonal therapy (T1cN0M0 stage I; TNM 7th edition). Using 10-MV photons, he received 74 Gy in 37 fractions, 7 portals, and all coplanar irradiation. Although he experienced cystitis grade 1 (Common Terminology Criteria for Adverse Events 4.0.) as an adverse event, we observed no other adverse event. He continued receiving hormonal therapy for 2 years because of being in a high-risk group and became prostate-specific antigen (PSA) nadir. Six months since the completion of hormonal therapy, his PSA level increased again. Despite receiving hormonal therapy again, his PSA level continued increasing gradually. After 10 months of restarting hormonal therapy, he presented with dry cough and dyspnea. Based on the radiological examination, he was diagnosed with multiple lung, bone, and lymph node metastases. To distinguish primary lung cancer, biopsy was made from lung disease by bronchoscopy and established metastasis from prostate cancer. Accordingly, we started docetaxel (75 mg/m2) every 5 weeks for hormone-resistant multiple metastases. After one cycle of docetaxel, radiotherapy for the thoracic and lumbar vertebrae was performed for back pain and lumbago. In addition, irradiation was performed using 10-MV photon beams (anterior) and 10-MV (posterior) photon beams, 30 Gy in 10 fractions, and the anterior:posterior dose weight was approximately 1:2 (Fig. ). Owing to a large irradiation field, we divided the radiation field into 2 parts: thoracic and lumbar vertebrae. Meanwhile, the same dose of docetaxel was concurrently restarted at the time of 18 Gy. We observed no adverse event during radiotherapy, and soon relief was achieved for back pain and lumbago. In addition, the PSA level decreased after the administration of docetaxel. On day 21, at the end of radiotherapy, the same dose of docetaxel was administrated for the third time. On day 7, after third docetaxel administration (day 28 from the end of radiotherapy), erythema appeared in an irradiated field of the thoracic and lumbar vertebra (Fig. ). Erythema primarily appeared on the anterior side of the body, and no skin reaction was noted on the posterior part of the thoracic irradiated area. Notably, no skin reaction was observed in the previously irradiated field for prostate cancer. In fact, conservative treatment for RRD was performed using a skin care cream demonstrated better results by improving dermatitis. Subsequently, the patient underwent fourth docetaxel treatment without corticosteroid on day 28 after the third docetaxel administration; however, a less vivid skin reaction was noted this time compared with that noted during the previous session.
This retrospective study evaluated patient characteristics, diagnosis, and treatment retrieved from electronic medical records. All procedures followed the ethical guidelines of the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Written patient's consent to participate and ethical approval were waived for the retrospective study. Patients were informed before the start of treatment that data might be used in future investigations even if the outcome was death and all consented to the use of data. |
pmc-6133595-1 | A 28-year-old man without any underlying diseases had suffered from left side weakness and clonic–tonic seizures for 12 days. Computed tomography (CT) of the patient's head revealed a hypodensed mass with the size of 3 × 3 × 2.4 cm of the right temporal lobe. He was then admitted, and the magnetic resonance imaging (MRI) of his brain showed cystic astrocytoma in the right parietal lobe without midline shifting. A craniotomy was performed, and the pathology showed astrocytoma (World Health Organization (WHO) grade III). After operation, he received 2-dimensional conformal RT with a total dose of 45 Gy in 25 fractions to the whole brain. Mild skin erythematous change was noted after the completion of RT.
During a regular follow-up of 14 years after postoperative adjuvant RT, a small red nodule was noted at the patient's right scalp. The size of the nodule increased overtime and the color eventually turned to black within a year. This gray-colored skin papule with a diameter of 2.5 cm has an ulcerative center. He received skin tumor excision at the age of 42. The skin tumor was totally removed with an adequate margin. The pathology revealed BCC. The morphology of the tumor featured basaloid cells which extended into the dermis with elongated nuclei and little cytoplasm. The peripheral cell layer of the tumor masses showed a palisade arrangement of the nuclei (Fig. ). No skin tumor recurrence has been noted to date.
Two years later, the patient developed headache, left side weakness with unsteady gait at the age of 44. His brain MRI revealed a huge cystic lesion with fluid-fluid level in the patient's right frontal lobe. There were hemorrhage, perifocal edema, mass effect causing mild midline shift. Second craniotomy was performed, and there was presence of microvascular of festoons gliosis, microcalcification and tiny foci of necrosis in the specimen, which favored the diagnosis of glioblastoma (WHO grade IV). He received chemotherapy with temozolomide.
Four years later, there was recurrent astrocytoma in the right frontal lobe with calcification with worsening perifocal edema and mass effect in the follow-up MRI of his brain. Third craniotomy was performed at the age of 48. The pathology revealed anaplastic oligodendroglioma (WHO grade III), as the morphology of tumor showed moderate to high cellularity with dense network of branching capillaries and microvascular proliferation. Adjuvant 3-dimensional conformal RT (3D-CRT) to brain tumor surgical bed was performed with 50 Gy in 25 fractions (Fig. ). After following for 5 years, MRI of brain showed worsening recurrent tumor in the right frontal lobe and the body of right caudate nucleus. Fourth craniotomy was performed. The pathology revealed anaplastic oligodendroglioma (WHO grade III). The latest follow-up brain image revealed that the disease is under control (Fig. ). He receives regular follow-ups at our outpatient department. Currently, he has survived for more than 26 years since his initial diagnosis of anaplastic astrocytoma, and more than 12 years from the diagnosis of skin BCC. |
pmc-6134320-1 | In December 2015 a previously healthy 61-year-old woman started complaining of slowly progressing unsteadiness of gait due to left limb coordination impairment. In January 2016 she was referred to our Emergency Department for the subacute onset of unintentional sharp movements of left limbs, initially causing repeated falls and, later on, impossibility to reach and maintain the standing position. Neurological examination showed left limb proximal hyperkinetic-hemiballic movements with mild distal dystonic posture, mild asymmetric left-sided plastic rigidity and ipsilateral pyramidal signs. Cognitive function was normal (Mini-Mental Status Examination, MMSE 30/30), without behavioral, language, or psychiatric abnormalities.
Laboratory tests were in range except for severe hypercalcemia (15.1 mg/dl) related to primary hyperparathyroidism (PTH 422 pg/ml), which was gradually corrected with oral administration of cinacalcet, intravenous hydration, and zoledronic acid, without any improvement of signs and symptoms.
Brain computed tomography (CT) scan was normal, while brain magnetic resonance imaging (MRI) showed faint hyperintensity on Fluid Attenuated Inversion Recovery (FLAIR) images and positive diffusion-weighted (DWI) signal in the right lenticular and caudate nuclei, posterior insular and fronto-parietal cortex, without cortical atrophy or gadolinum enhancement (Figure ). Total body CT scan with administration of iodinated contrast was unremarkable.
Autoimmune screening, tumor markers, ceruloplasmin with seric and urinary copper dosage, paraneoplastic antibodies (Hu, Yo, Ri, Ma1-2, CV2/CRMP5, amphiphysin, GAD) on both blood and cerebrospinal fluid (CSF) were performed, with negative results. CSF screening for fungal, bacterial or viral infections, as well as Rickettsiosis, Borreliosis and HTLV1-2 infection (performed because of the patient's history of an insect bite during a trip in Madagascar 2 weeks before symptoms onset), yielded negative results. Despite her normal cognitive profile confirmed at 3 months after clincal onset, on the basis of clinical manifestations and brain MRI images, a prion disorder was suspected. CSF Tau protein was significantly increased (2229 pg/ml; normal values 51–70) and 14.3.3 protein was weakly positive, with normal P-tau and β-amyloid values, thus confirming the hypothesis of a neurodegenerative process. Notably, repeated electroencephalography (EEG) showed nonperiodic right-prevailing abnormal slow waves in fronto-temporal-parietal regions (Figure ) without typical periodic triphasic sharp waves. DNA sequencing for PrP gene mutations of inherited CJD on blood samples showed no alterations, while analysis of codon 129 detected a MM polymorphism.
In the following weeks, the patient experimented rapid worsening of symptoms, with neck dystonia, diffuse hypertonic rigidity, startle reaction, myoclonus at rest on the left limbs, dysarthria, hypophonia, dysphagia, and inappetence. Myoclonus polygraphic recording was not performed in the clinical workup since neurophysiologic data would not have modified the degree of diagnostic certainty according to current criteria (–). A second brain MRI was repeated, resulting unchanged. Clonazepam oral drops 2.5 mg/mL were started, with benefit only on myoclonic jerks. To note, a specific neuropsychological (NPS) panel including MMSE, Frontal Assessment Battery (FAB), Clock Drawing Test (CDT), Digit Span (DS), Corsi Block-Tapping Test (CBTT), Story Recall Test (SRT), Trail Making Test (TMT), Attentional Matrices (AM), Controlled Oral Word Association Test (COWAT), Semantic Fluency Test (SFT), Arm Ideomotor Apraxia Test and Rey-Osterrieth Complex Figure Test (ROCF), performed in March 2016 was substantially within the range of normative scores (Table ). Due to poor clinical conditions and concurrent pneumonia, the patient died in April 2016, 4 months after disease onset. Post-mortem immunoblotting for PrP protein on nervous tissue sampled from right brain emisphere confirmed the diagnosis of sCJD, by the detection of type 1 abnormal isoform of the prion protein (Figure ). To note, immunohistochemistry and histology were not performed since fixed brain tissue samples were not available. |
pmc-6134473-1 | A 79-year-old woman had been seeing her family doctor for hypertension and had been diagnosed with liver dysfunction for about 10 years. She continued to attend follow-ups because of her drinking habit. Two months before her visiting our institution, further elevation of hepatobiliary enzymes was noted, and abdominal ultrasonography showed a hepatic tumor of 4 cm in diameter in the lateral segment, so she was referred to our hospital. HCC was suspected because AFP (102 ng/ml) and L3 fraction (85.4%) were elevated, and the appearance on enhanced computed tomography (CT) was not inconsistent with HCC. Thus, she was hospitalized for surgery. The patient was a non-smoker, had a history of habitual alcohol consumption, and reported a medical history of hypertension and hyperlipidemia. Her father and one of her brothers had had esophagus cancers, two of her brothers had liver cirrhosis, and one of her brothers received dialysis.
With regard to complete blood count, platelets decreased to 131,000/μl. Leukocyte elevation and anemia were not observed. Blood biochemistry showed aspartate transaminase, alanine transaminase, alkaline phosphatase, and gamma-glutamyltranspeptidase were elevated to 51 U/l, 42 U/l, 478 U/l, and 136 U/l, respectively. Blood urea nitrogen and creatinine were elevated to 20.2 mg/dl and 1.04 mg/dl, respectively. Total protein, albumin, and bilirubin were within the normal range, and C-reactive protein was not significantly increased. Coagulation was normal, and hepatitis virus tests were negative. The tumor markers AFP and L3 fraction were elevated to 102 ng/ml and 85.4%, respectively. Carcinoembryonic antigen, carbohydrate antigen 19-9 (CA 19-9), and protein induced by vitamin K absence II were within normal limits. Indocyanine green 15 min retention rate was elevated to 16.0%. As for liver fibrosis markers, Mac-2 binding protein glycosylation isomer and type IV collagen 7S were elevated to 2.12 COI and 8.3 ng/ml, respectively. Anti-mitochondrial antibody, immunoglobulin G, and antinuclear antibody were elevated to 1:147, 2093 mg/dl, and 1:320, respectively.
Contrast CT was performed at our institution (Fig. ). It showed right lobe atrophy, left lobe enlargement, and irregularities on the surface, suggesting liver cirrhosis. A tumor 39 mm in diameter was growing on the outside of the ventral liver segment three. This tumor showed slightly low absorption before contrasting, non-uniform slight contrast in the arterial phase, heterogeneous but mostly lower absorption than the surrounding liver parenchyma in the portal vein phase, and equally distributed areas of the same and lower absorption than the surrounding liver parenchyma in the delayed phase. We considered it likely that this tumor was HCC because of its growth pattern, contrast in the arterial phase, and tumor marker elevation. Non-uniform contrast in the tumor might suggest degeneration or fibrosis. Ultrasonography showed a hypoechoic lesion 34 mm in diameter, the appearance of which was not inconsistent with HCC (Fig. ). Therefore, laparoscopic hepatectomy was performed under the preoperative diagnosis of HCC without magnetic resonance imaging, positron emission tomography-CT, or reexamination of tumor markers.
When observed with a laparoscope, the yellow tumor was growing on the ventral liver segment three. After the omentum adhering to the tumor was dissected at a sufficient distance from the tumor, we confirmed the tumor by ultrasonography and marked a hepatectomy line 2 cm in diameter around the margin of the tumor edge. The hepatectomy was started from the right caudal side, and the segment three Glisson branch and left hepatic vein were dissected after clipping. After completing the hepatectomy along the marked line, we confirmed that there was no bleeding or bile leak on the hepatectomy surface. A drain was placed in the dorsal side of the hepatectomy surface, and the operation was terminated. The operation time was 2 h and 15 min and the bleeding volume was 1 g.
Macroscopic findings of the resected specimen revealed a heterogeneous and mostly yellow tumor with fibrous white and black parts, accompanied with a white surrounding capsule (Fig. ). Light microscopy showed that the tumor consisted mainly of collagen fibers, lymphocytes, and plasma cells (Fig. ). Cholesterin-containing giant cells (Fig. ) and hyalinization (Fig.) were also observed. Thus, the tumor was diagnosed as hepatic IPT. In the liver parenchyma outside the tumor area, expansion of the portal area was observed as well as crosslinked fibers (Fig. ), chronic non-pyogenic cholangitis, and epithelial cell granulation, suggesting that background liver condition was PBC. All resected specimen tissues were analyzed, but no lesions suggesting tumor existed. Immunochemical staining with anti-AFP antibody was performed, and some hepatocytes around the tumor were stained, but the inside of the tumor did not stain at all.
Postoperatively, the patient recovered uneventfully and was discharged on postoperative day 7. On postoperative day 36, a blood examination showed that both AFP (4 ng/ml) and L3 fraction (8.4%) had decreased to within normal ranges. None of the findings, including contrast CT performed on the same day, suggested tumor in the residual liver (Fig. ).
In this case, HCC was strongly suspected because AFP and L3 fraction were elevated. Imaging findings were consistent with HCC, although they were not completely typical. Thus, laparoscopic hepatectomy was performed under the preoperative diagnosis of HCC. However, the liver lesion was ultimately diagnosed as hepatic IPT by histopathology. According to a report on tumor markers in liver IPT, CA 19-9 was elevated in 7 of 29 patients (24.1%) and AFP was elevated in 2 of 58 (3.4%) []. AFP is also frequently elevated in benign liver diseases such as hepatitis and liver cirrhosis [], and L3 fraction has been used as a tumor marker for highly specific HCC []. It is also reported that a high percentage of L3 fraction significantly correlates with low survival rate after HCC treatment []. Thus, the significance of L3 fraction as a marker for biological malignancy has been drawing attention.
In this case, although the pathological diagnosis was liver IPT, the AFP and L3 fractions were elevated before resection, and both markers postoperatively decreased to the normal ranges. This prompted us to consider the possibility of spontaneous regression of HCC, so we investigated all resected specimen tissues, but no other tumor components were found. AFP L3 fraction is also elevated in some cases of acute hepatitis or chronic active hepatitis [, ], and it is further elevated in fulminant hepatic failure [, ]. Additionally, it is elevated in acute liver injury, including acute-onset autoimmune hepatitis and acute liver failure []. These data suggested AFP L3 fraction may reflect liver regeneration. With regard to tumors, it is reported that elevated AFP L3 fraction has been observed in multiple pancreatic acinar cell carcinoma patients [], but to our knowledge, there have been no reports of elevated L3 fraction being decreased by resection of liver tumors other than HCC, including cholangiocarcinoma and IPT. Considering that L3 fraction can be elevated by severe acute hepatitis, elevation of the L3 fraction in this case could be caused by local intense inflammation within the tumor. However, we cannot explain the detailed mechanism of this, and future research to improve the understanding of the pathology and other aspects of liver IPT is expected. |
pmc-6134480-1 | A 91-year-old man was referred to our dermatology clinic for a 3 cm × 2 cm ulcerative nodule at his chin (). The lesion had been rapidly growing since approximately 2 months. Biopsy showed skin metastasis originating from a gastric adenocarcinoma. The patient had no fever and did not experience night sweat or weight loss. Computed tomographic scanning of the whole body revealed a gastric tumor with blood vessel infiltration, peritoneal carcinomatosis, pulmonary metastases with pericardium infiltration, and multiple disseminated subcutaneous metastases. The patient refused therapy and died a month after diagnosis. |
pmc-6134491-1 | A 73-year-old man with history of NVAF, hypertension, bradycardia requiring pacemaker implantation, and history of upper gastrointestinal bleed while on anticoagulation was deemed a good candidate for LAA occlusion device implantation. His CHA2DS2-VASc score was 3, for diastolic heart failure, age, and history of hypertension, and HAS-BLED score was calculated to be 4, putting him at elevated risk for another serious bleed while on therapeutic anticoagulation. The procedure was performed under general anesthesia, and transseptal access was performed with transesophageal echocardiography (TEE) and fluoroscopy guidance. His maximum LAA width measured by echocardiography was 21 mm with maximum depth, 27 mm, measured to the anterior lobe. A 27-mm Watchman device was selected and prepped in the usual fashion and delivered via a dual curve sheath. The activated clotting time during deployment was 213 seconds. As the device was being delivered through the sheath at the area of greatest curvature, the sheath whipped anteriorly before the device exited. Contrast injection during fluoroscopy revealed pericardial staining. Given a presumptive diagnosis of LAA perforation, the device was deployed with sheath remaining in the same distal position given the concern for losing LAA access. After deployment, angiography through the sheath confirmed LAA laceration, with TEE visualization of the device in the transverse sinus (). Given increasing hypotension, 2 pericardial drains were placed with acute evacuation of approximately 1 L of blood. The patient was immediately transfused with packed red blood cells, and anticoagulation was reversed with protamine with the sheath remaining in the atrium. The patient was hemodynamically stabilized but required continuous pericardial drainage despite reversal of anticoagulation. Given the presence of an intact proximal portion of the LAA, the decision was made to deploy the device in the LAA to provide an impediment to blood loss. Following device deployment guided primarily by TEE visualization of the delivery sheath, there was an almost immediate cessation of fluid accumulation in the pericardial space. The patient remained hemodynamically stable and transferred to intensive care unit for further monitoring. The patient was kept sedated and intubated to assure stabilization. On postoperative day (POD) 2, a TEE revealed trace pericardial effusion with adequate device position and no peri-device leak (). The patient was subsequently extubated. A repeat echocardiogram showed only trivial pericardial effusion on POD 4. The patient was maintained on dual antiplatelet therapy (DAPT) and colchicine, and he was discharged home on POD 5. Computed tomography of the chest, 1-month postimplantation, showed adequate positioning of the device without any evidence of extravasation or device-related thrombus (). |
pmc-6134501-1 | We present a case of 2-year-old white girl with chronic crackles admitted to our Pediatric and Allergy Clinic. The pregnancy was unremarkable and after birth the child was healthy until the seventh month of life, when she developed RSV infection. From then on she had a LRTI every month treated with antibiotics, mainly macrolides for presumed bacterial pneumonia; symptoms persisted daily. She had been under the care of pulmonologists from a different department, who suspected childhood interstitial lung disease (chILD) and prescribed systemic and inhaled steroids, short-acting β2-mimetics, and antileukotriene. This treatment, however, did not lead to any clinical improvement; symptoms of crackles were present at all times. She was hospitalized eight times due to exacerbation of symptoms such as dyspnea, cough, and persistent crackles during physical examination. At the age of 11 months she had high resolution computed tomography (HRCT) which revealed lung areas of uneven aeration in the middle lobe of her right lung and small areas of densities which indicated postinflammatory changes. Due to suspected Pneumocystis jirovecii (carinii) infection, she was unsuccessfully treated with sulfamethoxazole and trimethoprim.
She was admitted to our clinic at 23 months of age with intense cough, dyspnea, and chronic crackles. A chest X-ray showed areas of density due to parenchymal and interstitial inflammatory changes. Autoimmune disease and atypical inflammatory infections (Mycoplasma pneumoniae, Chlamydia pneumoniae, and Bordetella pertussis) were excluded by use of a multiplex assay; immunodeficiency was also excluded. An echocardiogram revealed no abnormalities. Next, she was referred to the Pneumonology and Cystic Fibrosis Department in Rabka for bronchoscopy with bronchoalveolar lavage (BAL). The result showed: copious purulent secretions in her lower throat; mucosal edema of the larynx (Fig. ), trachea, and bronchial tree; and retention of the purulent mucus in bronchi (Fig. ) with normal movement of bronchial cilia (high frequency video microscopy). Microbiological testing with growth on blood/chocolate agar isolated high colony count of Moraxella catarrhalis in the BAL fluid. It was beta-lactamase producer sensitive to amoxicillin-clavulanate. The BAL also showed epithelial cells, macrophages, and neutrophils under high power field. She was administered amoxicillin-clavulanate for 14 days with good clinical improvement in respiratory rate, labored breathing, and cough and she was discharged. She was observed for 2 months after discharge from the hospital and showed no signs of recurrence. Then, she had a few more respiratory tract infections (usually every other month) treated with antibiotics (crackles were present at each time during infection); between infections she remained healthy, without any crackles or wheezing. |
pmc-6134551-1 | A 62-year-old man with a history significant for chronic hepatitis B initially presented with hematuria and urine cytology positive for malignant cells. A CT urogram revealed a large ill-defined mass of the left posterolateral aspect of the urinary bladder with extramural extension, likely involvement of the seminal vesicles and the prostate, and bilateral enlargement of the external iliac chain lymph nodes. Partial transurethral resection of the tumor was performed, and pathology confirmed an invasive high-grade urothelial carcinoma with squamous differentiation with muscularis propria and lymphovascular invasion. A CT scan of the chest revealed several pulmonary nodules concerning for metastatic disease. An MRI of the liver did not reveal presence of metastatic disease or concern for hepatocellular carcinoma (HCC). Concurrent testing of serum alpha-fetoprotein (AFP) intended for HCC screening (for chronic, but inactive hepatitis B virus infection) showed a very high value of 934.7 ng/mL (normal < 5 ng/mL). He next received neoadjuvant gemcitabine/cisplatin followed by radical cystoprostatectomy and pelvic lymphadenectomy. Pathologic examination revealed a high-grade, poorly differentiated urothelial carcinoma with squamous differentiation with involvement of the left ureter, lymphovascular invasion, extension into perivesical fat, and involvement of 8 of 9 resected lymph nodes. Immunohistochemistry (IHC) of the primary tumor revealed strong staining for AFP (Fig. A-B), confirming tumor-derived AFP production. Serum AFP levels showed a steep decline following surgery, further validating this as a tumor marker, which was subsequently followed throughout the patient’s treatment course (Fig. ). Molecular testing of the primary tumor using a targeted next-generation sequencing assay (SNaPshot V1) revealed a single nucleotide variant in TP53 (Arg282Trp). FISH was consistent with amplification of the HER2 gene, but there were no targetable alterations. On surveillance CT of the abdomen and pelvis three months following surgery, the patient experienced a significant disease relapse, initially deferred initiation of chemotherapy, however, ultimately began treatment with pemetrexed for a total of three cycles (Fig. ). He continued to demonstrate rising serum AFP levels that correlated with progressive disease, now with palpable metastatic lesions in the head and neck area and continued visceral progression. Finally, he received paclitaxel monotherapy for two cycles, but did not tolerate this therapy well and continued to experience rapidly progressing disease. Histologic examination of his primary tumor revealed strong staining for PD-L1 (Fig. ). Due to his chronic hepatitis B infection, however, he was not eligible for clinical trials of immunotherapies. We therefore initiated therapy with the anti-PD-1 checkpoint inhibitor pembrolizumab (2 mg/kg every 3 weeks). Within 6 weeks, his AFP levels dropped from a peak level of ~ 3800 ng/mL to 42 ng/mL. This coincided with a dramatic clinical response, with reduction or resolution of all palpable metastatic lesions. After 4 doses of pembrolizumab, imaging revealed significant shrinkage of all metastatic lesions in the abdomen, including peritoneal masses and mesenteric lymphadenopathy (Fig. ). Notably, no lung nodules were appreciated on these imaging studies. The patient went on to receive a total of 16 cycles of pembrolizumab and sustained complete remission also reflected by normalized AFP levels, while only experiencing minimal adverse effects. However, after 12 cycles of pembrolizumab therapy, the patient was noted to have a left lower lobe lung nodule measuring ~ 7 mm in largest dimension, which increased to 10 mm on subsequent imaging. Due to concern for a metastatic escape lesion, and in the absence of other evidence of disease, the patient underwent wedge resection of this lung nodule. Of note, this lesion occurred in the absence of AFP elevation. Histopathologic assessment of the resection specimen demonstrated a benign intraparenchymal lymph node with no malignant cells seen (Fig. ). Since the resection, the patient received no additional systemic therapy and continues to have no evidence of disease for 18 months (as of July 2018). |
pmc-6134575-1 | A 41-year-old woman presented a month ago with epigastralgia referred to our facility. Physiological and laboratory assessments were unremarkable except for elevated serum CA19-9 and elastase-1 levels (CA19-9, 207 IU/L; elastase-1, 150 IU/L). Enhanced multi-detector CT revealed a hypovascular tumor in the uncinate process of the pancreas that was in wide contact with the superior mesenteric artery (SMA, > 180°; Fig. ). Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated stenosis of the main pancreatic duct and mild distal dilatation. Pancreatic juice and biliary juice cytology were categorized as class V (adenocarcinoma), and a biliary plastic stent was placed during the initial ERCP procedure. She was subsequently diagnosed with cStage III pancreatic cancer (Ph, TS2 (35 mm), cT4, cCH1, cDU1, cS1, cRP1, cPL1, cVsm0, cAsm1, cN0, cM0) that was also categorized as an unresectable LAPC (UR-LA) according to the Japan Pancreatic Society (JPS) classification, 7th edition [, ]. Therefore, she immediately underwent 3 cycles of chemotherapy (gemcitabine (GEM) 1000 mg/m2 + nanoparticle albumin-bound paclitaxel (nab-PTX) 260 mg/m2) on days 1, 8, and 15 of a 28-day cycle. In addition, she underwent 55.2 GyE (RBE) of CIRT with concurrent GEM chemotherapy (GEM 1000 mg/m2, days 1, 8, and 15) after GEM + nab-PTX chemotherapy.
CA19-9 antigen levels fell within normal range (CA19-9, 207 → 25 IU/L) after three courses of induction GEM + nab-PTX chemotherapy and remained within the normal range (CA19-9, 22.9 IU/L) until conversion surgery. Although the primary tumor showed remarkable shrinkage after induction chemotherapy (Fig. ) and subsequent CIRT (Fig. ), clinically and radiologically apparent frequent cholangitis due to unidentified duodenal narrowing (Fig. ), along with peripheral sensory neuropathy due to the nab-PTX regimen, prevented continued chemotherapy. FDG-PET scan did not reveal any hot spots either in the primary tumor or in the entire body, and 9 months after her first visit, we decided to perform conversion surgery. As dissection around the SMA was necessary for achieving a R0 resection, we initially identified and taped the SMA and histology on rapid frozen sections of a part of the nerve plexus around the SMA confirmed absence of tumor cells. Therefore, we performed a radical subtotal stomach preserving pancreaticoduodenectomy (SSPPD). Group 1 and 2 lymph node dissections (D2) were also performed while predominantly preserving the nerve plexus around the SMA, except for the resected tissue used for rapid frozen section analysis (JPS 7th ed.). The procedure lasted 4 h and 55 min with a blood loss of 587 g. Preoperative therapy did not affect surgical factors such as difficulty of the surgical procedure, blood loss, or operation time, and the patient was discharged on the 22nd postoperative day.
Grade B postoperative pancreatic fistula (POPF) and deep incision surgical site infection (SSI) were identified as postoperative complications. Histopathological examination revealed an invasive ductal carcinoma, a tubular adenocarcinoma (tub1>tub2), Ph, TS1(20 × 8 × 7 mm), infiltrative type, ypT3, ypCH1, ypDU0, ypS0, ypRP0, ypPVX, ypAX, ypPL0, ypOO0, ypPCM0, ypBCM0, ypDPM0, intermediate type, INFβ, ly0, v1, ne1 mpd0, ypN0(0/7), ypM0, ypT3N0M0 ypStageIIA. Histologically, it was categorized as Evans grade IIB (Fig. ) and the patient underwent an almost full dose of S-1 adjuvant chemotherapy for 6 months, and no signs of recurrence have been seen for 23 months.
Pancreatic cancer is associated with an extremely poor prognosis, and prolonged survival is only achieved by R0 resection. However, LAPC accounts for 30–40% of all pancreatic cancers [–], and the two internationally used criteria for LAPC are from the National Comprehensive Cancer Network (NCCN) and from the joint consensus conference of the Americas Hepato-Pancreato-Biliary Association (AHPBA), the Society for Surgical Oncology (SSO), and the Society for Surgery of the Alimentary Tract (SSAT) (AHBPA/SSO/SSAT) [, ]. JPS has also established its own criteria, and these are followed in Japan [, ]. Importantly, it is known that a R0 resection for LAPC cannot be achieved with surgery alone and that LAPC requires powerful local or regional control, including with chemo- and/or radiotherapy. On the other hand, previous reports have shown that one third of all patients with LAPC treated with chemoradiotherapy eventually develop metastatic progression [–]. Thus, even in LAPC, it is necessary to deliver more effective systemic chemotherapy early in the course of the cancer, apart from providing locoregional treatment to improve outcomes. The advantages of combining rapid induction chemotherapy and chemoradiotherapy, including carbon-ion beams, are as follows. First, generally, chemotherapy can be introduced rapidly and without major adverse reactions compared to chemoradiotherapy, and second, it can save patients with occult metastasis from undergoing subsequent chemoradiotherapy. Several non-randomized controlled trials have demonstrated the feasibility and efficacy of combining rapid induction chemotherapy and chemoradiation [, ]. This therapeutic strategy of “chemotherapy-first” may be considered similar to adjuvant therapy for pancreatic cancer. Recently, more powerful regimens have been made available for pancreatic cancer, and it would be ideal to investigate their efficacy using randomized controlled trial (RCT).
Carbon-ion beams can provide ideal dose distributions [] and greater biological efficacy [], and its efficacy does not depend on cell-cycle stage []. These biological features are considered as anatomically suitable for locoregional control of LAPC. A few non-RCTs on CIRT for pancreatic cancer are available from Japan [, , ], and these reports show feasibility and efficacy of CIRT for pancreatic cancer. A recent multi-institutional study on CIRT for LAPC from Japan (J-CROS1403) was conducted in 72 patients, and the results indicate favorable clinical outcomes with median OS of 21.5 months and no severe toxicity-related gastrointestinal disorders, including stenosis or obstruction, except in 1 case (1%) of grade 3 duodenal ulcer due to late toxicity (19). Thus, we believe that it is reasonable to expect that a reduction in the risk of local relapse by CIRT will lead to an increase in the cure rate for LAPC.
In our case, frequent retrograde cholangitis, probably caused by duodenal stenosis, was clinically apparent. Possible explanations include (1) the presence of a huge pancreatic head tumor, due to which the boundary between the tumor and the duodenal wall often remains unclear, or (2) that the tumor has invaded the duodenum. In these scenarios, it is possible that the planned target volume of carbon-ion beams included a part of the duodenum, which then influenced her clinical course. However, the patient did not complain of nausea or bilious vomiting related to duodenal stenosis, and furthermore, pathological examination of resected specimen revealed no obvious evidences of stenosis or direct invasion of the duodenum by the tumor except for mild fibrosis in the muscular layer of the same portion. Thus, the following factors may have caused duodenal compression from outside the duodenal wall. First, while the therapeutic response of grade IIB (EVANS) is an excellent result, the resected specimens showed that most of the tumor had been replaced by fibrosis. Therefore, we cannot discount the possibility that the fibrous tissue inhibited wall ductility of the duodenum. Second, as the patient was thin, it is possible that she had developed the SMA syndrome. Thus, it is our opinion that the retrograde cholangitis was not a simple adverse event of CIRT but the result of a favorable therapeutic response of the tumor.
The duration of induction therapy in this case was 9 months. According to previous reports, the median duration of induction chemo (radio) therapy in patients who undergo conversion surgery is 7–9 months [, , ]. In our patient, histopathological findings revealed high efficacy of chemoradiotherapy, and the patient is currently in long-term, relapse-free survival. These facts suggest that a combination of rapid induction GEM + nab-PTX chemotherapy followed by CIRT can be safely used and that it might be an effective treatment option for LAPC. |
pmc-6134579-1 | Α 54 year old Caucasian woman of Greek ancestry presented to the Retina Service of our Department complaining of metamophopsia and reduced visual acuity in both eyes, gradually worsening over the past four months. Her past medical history revealed that she suffers from beta thalassemia intermedia for which she receives blood transfusion biweekly and chelation therapy for the past 14 years. Her chelation therapy consists of DFO (50 mg/kg 5 times a week by an 8-hourly subcutaneous infusion), as well as deferiprone (50 mg/kg, per os, daily). Ferritin level was within normal limits. The remainder of her medical history included hypothyroidism treated with levothyroxine. No history of color and peripheral vision changes nor hearing impairment was reported. Her past ocular history was unremarkable.
On the initial examination, best corrected visual acuity (BCVA) was 20/25 in the right eye (OD) and 20/22 in the left eye (OS). Slit lamp biomicroscopy revealed no pathology from the anterior segment and intraocular pressure (IOP) measured by applanation tonometry was 14 mmHg in both eyes (OU). Dilated fundus examination showed a yellowish roundish macular lesion surrounded by RPE changes, as well as angioid streaks emanating from the optic disc sparing the macula in OU (Fig. ). The retinal vasculature appeared normal.
Spectral-Domain Optical Coherence Tomography (SD-OCT) revealed a subfoveal hyperreflective deposit above the level of the RPE corresponding to the lesion observed fundoscopically (Fig. ) while mild stippling was noticed in the macular area in fundus autofluorescence (FAF) (Fig. ). Finally, fluoroscein angiography revealed staining of the macular lesion in the late phases of the angiogram (Fig. ). To evaluate possible DFO retinopathy in this patient, electrophysiology testing, more specifically, full-field electroretinogram (ffERG) and pattern electroretinogram (PERG), was performed. Amplitudes and implicit times for each major waveform component were included. The responses were taken according to the International Society for Clinical Electrophysiology of Vision (ISCEV) []. The ERG revealed no abnormal rode and cone responses OU (Fig. ). Visual field examination, performed by automated perimetry (Humphrey 30–2 SITA-standard, Carl Zeiss Meditec, Dublin, CA) was also normal.
Taking into consideration the normal ffERG and after consultation with her hematologist, it was decided that the patient would remain on the prescribed chelation therapy, due to the severity of her systemic condition. The patient was followed up every 3 months in our department with SD-OCT for possible deterioration. Her BCVA remained stable for the next 2 years, with no anatomic change on OCT findings (Fig. ). |
pmc-6134579-2 | A 63 year old Caucasian woman of Greek ancestry presented to our Retina Service complaining of decreased central vision OU. She had a medical history of sickle beta thalassemia for which she was receiving regular blood transfusions. She was also receiving iron-chelation treatment with DFO (50 mg/kg 5 times a week by an 8-hourly subcutaneous infusion), for the past 25 years. The rest of her medical and her ocular history were unremarkable. The patient did not report any peripheral, night or color vision problems. There was no history of hearing loss. Her BCVA was 20/50 OU. Slit lamp biomicroscopy was normal and IOP was 15 mmHg in OD and 13 mmHg in OS. Dilated fundus examination revealed the presence of a yellow-brown macular lesion OU. The lesion consisted of yellow pigment lines expanding to the perimacular area in a tri-radiating pattern surrounded by areas of granular hyper-pigmented brown material. Fundus autofluorescence revealed hyperfluorescent areas in a butterfly shaped pattern corresponding to the pigment clumping areas seen in fundoscopy OU (Fig. ). Fluoroscein angiography demonstrated a large hypofluorescent, butterfly-shaped macular lesion surrounded by areas of focal hyperfluorescence OU (Fig. ). Spectral Domain-OCT demonstrated a subfoveal hyperreflective lesion at the level and above the RPE OU (Fig. ). Electroretinogram showed no abnormal cone and rod responses. Humphrey visual field perimetry using 30–2 SITA-Standard algorithm was within normal limits.
Haematologists were consulted regarding DFO discontinuation but they decided not to make any change in chelation therapy since the risks pertaining to the patient’s underlying disease outweighed the risk of possible visual loss. Therefore, it was decided to follow-up our patient closely every three months. During a 4 years follow-up period BCVA remained stable without any signs of anatomic change as it was demonstrated with repeated OCT examinations (Fig. , ). |
pmc-6134588-1 | The 50-year-old woman was admitted for a fever persisted for 20 days. Computed tomography (CT) scanning showed pulmonary infection. Cytopenia was observed in peripheral blood. White blood cells, red blood cells, and neutrophil graneulocytes were all decreased. Hemoglobin was only 74.0 g/L. Ferritin was increased to be 3602.5 g/L. Autoimmune antibody test found no abnormity. The available laboratory data were summarized in Table .
NK cells activity was detected according to IFN-γ secretion by using whole blood as previously established in our laboratory []. Moreover, functional activity of NK cells was detected using K562 cells as target cells. Low activity of NK cells was found in two assays. In both assays, the activity of NK cells was only 30% of the low limit of healthy controls. Bone marrow aspiration confirmed hemophagocytosis (Fig. ). Laboratory tests exclude EBV or Cytomegalovirus (CMV) infection, common cause of HLH. This patient was diagnosed and treated according to HLH-2004 guidelines [, ].
To explore the genetic cause of HLH in this patient, a targeted next generation sequencing (NGS) panel was applied, including LYST, CTPS1, PIK3CD, PRF1, SRGN, CD27, LAMP1, ARF6, GZMB, RAB27A, BLOC1S6, CORO1A, UNC13D, STXBP2, GNLY, STK4, PRKCD, AP3B1, ITK, STX11, CARD11, MCM4, MAGT1, SH2D1A, XIAP, and IL2RG genes. The mean depth was 315 folds. 98.44% of target region was covered by at least 20 folds. The NGS was performed on the Ion Torrent Personal Genome Machine as previously described []. However, NGS targeting HLH associated gene found no pathogenic variant.
Coagulation tests showed the FVII:C was decreased to be 4%. The FVII:C of the patient’s brother was 5%, who suffered postoperative hemorrhage after surgical resection of renal cell carcinoma 3 years ago. Both the patient and her brother showed prolonged PT. Family tree was drawn (Fig. ). Genomic DNA was extracted from peripheral blood mononuclear cell (PBMC). Coding exons and adjacent splice junctions were amplified for the F7 gene. Sanger sequencing was performed bi-directionally on ABI 3500 Dx. NM_000131.4 was used as reference transcript of the F7 gene. Genetic analysis of the F7 gene in the patient and her family members identified recurrent compound heterozygous F7 c.64 + 5G > A and c.1224 T > G (p.His408Gln) mutations in this patient and her brother. Heterozygotes were found in other family members who showed slightly decreased FVII:C (Fig. , Table ). Heterozygotes were asymptomatic. |
pmc-6134697-1 | This case describes a 29-year-old woman who was first diagnosed with ameloblastoma as a child at 7 years old. The lesion originated in the ascending branch of the left mandible, and the first surgical procedure was performed in March 1997 followed by disease recurrence in April 1999. A second resection was performed in May 1999, and during the next 16 years, the patient underwent several surgical approaches that were consistently followed by disease recurrence. Some of the procedures were conservative surgeries, but others were radical procedures that left her with several deforming scars. She presented to our clinic in January 2015 with a new magnetic resonance imaging (MRI) that evidenced a right, triangular aspect, paracellarlesion, extending to the homolateral cavernous sinus (13 × 9 mm), which was suspected to be a residual lesion that would have achieved the cavernous sinus by contiguity growth after several surgeries. Her last surgery had been performed in April 2014 and was followed by local radiotherapy in May 2014. She was asymptomatic and not willing to undergo a new invasive procedure. She decided to be followed without further intervention.
For the next 18 months, she was clinically stable and asymptomatic, but she returned in July 2016 with intense pain on the right side of her face that required multiple hospital visits for intravenous analgesia. MRI revealed an extensive heterogeneous lesion with contrast enhancement centered on the right cavernous sinus anterior to the cavus of Meckel and exhibiting anterior extension towards the upper orbital fissure (measuring approximately 19 × 15 × 16 mm). To identify new treatment possibilities, we decided to perform a new biopsy and conduct molecular testing (Fig. ). A BRAF mutational analysis by the allele-specific protein chain reaction (PCR) certified test revealed the presence of a BRAF c.1799 T > A;p.V600E mutation corresponding to a V600E amino acid substitution. After tumor board discussion and a careful conversation with the patient, she decided to undergo BRAF inhibitor therapy.
A treatment regimen with vemurafenib 960 mg PO twice daily was started on October 4, 2016. Prior to the initiation of therapy, a new MRI performed on September 24 revealed a lesion measuring 24 × 21 × 19 mm. After 2 weeks of therapy, the patient was asymptomatic and was not using any analgesic medication. During the course of therapy, she experienced grade one anorexia, nausea and fatigue, without any severe therapy-related adverse events. MRI performed in April 2017 revealed stable disease (24 × 18 × 15 mm), and her last MRI performed in September 2017 evidenced a reduction of the lesion size (18 × 13 × 14 mm) (Fig. ). The patient currently remains asymptomatic with excellent tolerance to the medication. |
pmc-6134773-1 | A 38-year-old female presented with a red left eye for one month. Physical examination revealed an inflammatory granuloma in the lateral canthus and a fistula with purulent secretion. Lateral conjunctival congestion and chemosis were observed (Fig. ). A computerized tomography scan detected swelling in the area of the left lacrimal gland (Fig. ). Under topical and local anesthesia, a blunt needle was inserted into the fistula, and then the fistula was incised. A huge dacryolith (10 mm × 5 mm × 3 mm) was identified at the end of the dilated lacrimal gland ductule and removed surgically (Fig. ). Histopathologic examination showed a hair nucleus surrounded by lamellar structure (Fig. ). The symptoms were resolved in 2 weeks after dacryolith extraction. Further history taking revealed that the patient had raised hundreds of rabbits in an enclosed room since she was 10 years old and that she had experienced foreign body sensation for many years, suggesting that the hairy nucleus might be a rabbit fur. |
pmc-6134805-1 | The case was a Swiss man, in his late 40s, who supposedly reached FG by land in April 2018 after a long trip in South America. For several weeks he lived in a forest dwelling near the river Comté (Roura municipality). He was reported to have worked on a trail development in the same area the week preceding symptom onset. He reportedly developed mild symptoms (fever, body aches and myalgia) on 4 August. He first sought medical help the following day and was discharged with the diagnosis of acute dengue-like viral infection. He subsequently developed vomiting and deep prostration, associated with persisting high fever and visited the emergency room of the Cayenne hospital on 7 August. He was admitted to the ICU on 8 August due to renal and hepatic failure. On 9 August, he was transferred to a specialised transplant centre close by Paris in mainland France, to receive a hepatic transplant. Despite this he unfortunately died on 30 August. The patient was not vaccinated against YF.
On 10 August, both RT-PCR and serological tests were performed on a blood sample collected on 8 August by the arboviruses CNR of the IPG [] confirming the diagnosis YF ().
In this case, the transmission was clearly autochthonous and sylvatic, occurring in the north of FG, ca 40 km from Cayenne.
Active case finding was carried out in the neighbourhood where the case lived. The area is sparsely populated, and only six potentially co-exposed persons were identified, all of them were vaccinated against YF.
In the area there are, however, several touristic structures, and tourists or other people potentially co-exposed e.g. while participating in outdoor sports in the area will be targeted for passive epidemiological surveillance supported by a reporting device.
For both cases, soon after YF confirmation, vector control measures were strengthened in the locations where the patients transited while viraemic. Information campaigns encouraging vaccination have been carried out in FG, aimed at the general population and clinicians; this communication has been strengthened towards people living and working in areas attended by the cases.
Already since March 2017, we have solicited inhabitants and partner institutions that regularly work in the Amazonian forest (i.e. Amazonian park, French army or organisations dedicated to the preservation of the environment), and they have not reported any evidence of unusual mortality in the non-human primates (NHP) population; nevertheless, in FG, no structured surveillance system of sylvatic epizootics exists.
Illegal gold prospectors were an at-risk, potentially unvaccinated and hard-to-reach population. They live deep in the forest and are potentially co-exposed to the YF virus. Moreover, working in illegal sites and often targeted by police operations they are difficult to reach by health professionals. When the first case occurred, we collaborated with local health and social mediators to invite members of this community who were active near Petit Saut dam lake, to receive vaccination against YF free of charge in nearby health centres. The Kourou health centre of the French Red Cross reportedly received several garimpeiros seeking vaccination in the following weeks (exact figures not available). Garimpeiros were advised to seek medical care immediately if they developed consistent symptoms: four consulted for fever with an unknown vaccination status, and tested negative for YF PCR. |
pmc-6135040-1 | A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.
The patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.
The patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.
ISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].
The same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].
The duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].
Headache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].
The incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
pmc-6135297-1 | A 45-year-old female patient with a past medical history of hypothyroidism and hyperlipidemia presented with recurrent night episodes of facial, lip, and tongue swelling. She did not have any rash during these episodes. The patient denied any allergic reaction in the past. She had not eaten anything unusual or traveled recently. There was no family history of allergic reaction or atopy. Self-medication with diphenhydramine did not relieve her symptoms. The patient was hemodynamically stable. Laboratory findings were not significant, and the eosinophilic count was normal. We reviewed her medications; she was taking levothyroxine 125 mcg daily for the last three years, and her thyroid function tests were stable. Two months back, rosuvastatin 20 mg was added for hyperlipidemia. Since that time, she had episodes of facial, lip, and tongue swelling that woke her up almost every night. The possible trigger of these episodes of angioedema was rosuvastatin, and we discontinued it. Complement component 4 (C4), C1 esterase inhibitor, and complement component 1q (C1q) binding assays were ordered. The patient's facial, lip, and tongue swelling resolved over the next 24 hours without the use of any further corticosteroid treatment. The patient's C4, C1 esterase inhibitors, and C1q binding assay were within normal range. Therefore, the diagnosis of hereditary angioedema (HAE) was ruled out. The temporal relation between rosuvastatin and the development of angioedema and prompt resolution of symptoms after drug discontinuation suggest that rosuvastatin was the most probable culprit for the development of angioedema in our patient. She was discharged home and colesevelam was started instead of rosuvastatin for hyperlipidemia. No other events were reported on follow-up visits and the patient was stable. |
pmc-6135309-1 | A 37-year-old man from the Himalayan region of Nepal presented with swelling of the right leg for ten days and sudden onset weakness of the left half of the body for two days. The swelling of the right leg had an insidious onset and was gradually progressive. The pain disabled the patient to move his right leg. Meanwhile, the patient developed weakness of the left half of the body, predominantly in the upper left limb. The patient also developed slurring of speech and deviation of the face towards the right side. He denied any history of chest pain, diaphoresis, shortness of breath, loin pain, nausea or vomiting. He had decreased urine output and red colored urine and was a non-smoker and non-alcoholic. There was no history of hypertension, chronic kidney disease, and diabetes mellitus in this patient.
On detail inquiry, the patient gave a history of recurrent throat infection during childhood, however, he was not medically managed then. Three years back, when he visited a cardiac centre with complaints of shortness of breath and palpitations, a diagnosis of RHD with severe MS and AF was made. On reviewing the past record of the patient, a significantly elevated serum antistreptolysin O (ASO) titer was seen. He was planned for per-cutaneous trans-mitral commissurotomy (PTMC) by his physician. However, he lost the follow-up and was non-compliant to his medications.
On physical examination, his blood pressure measured 110/70 mm Hg; pulse rate was irregularly irregular at 77 beats per minute; respiratory rate was 16 breaths per minute, and body temperature measured 37.1 oC orally. The patient was alert, conscious, and cooperative. There were no appreciable pallor, icterus, clubbing, splinter hemorrhages, rashes and cyanosis. On cardiovascular systemic examination, the first heart sound (S1) was variable in intensity and second heart sound (S2) was normal. There was a low pitched rough rumbling mid-diastolic murmur at the apex heard best when the patient was lying on the left side with breath held in expiration using the bell of the stethoscope. There was a high pitched blowing pansystolic murmur at the left lower sternal border. Examination of the abdomen was unremarkable. On neurological examination, the patient had a left-sided central facial nerve palsy, muscle strengths in the left upper and lower limbs were 2/5 and 3/5 respectively, and there was an ipsilateral Babinski sign. A thorough eye examination along with fundoscopy was unremarkable. Local examination of the right leg revealed swelling below the level of the knee and blackish discoloration of toes and lower third of the leg on inspection. Vesicles could be seen over the lateral aspects of the leg and dorsum of the foot. The limb was cold to touch and tender on palpation. Dorsalis pedis and posterior tibial pulses were not palpable (Figure ).
On investigation, electrocardiogram (ECG) showed ST segment elevation of more than two millimetre (mm) in chest leads V4, V5 and V6, suggesting ST elevation myocardial infarction (STEMI) and AF with normal ventricular rate (Figure ). Transthoracic echocardiography showed thickened, calcified mitral valve leaflets with mitral valve area (MVA) of 1.01 cm2 on planometry, mitral valve pressure half-time (PHT) of 344 millisecond, and mean pressure gradient of 9 mmHg (features suggestive of rheumatic severe mitral stenosis), left atrial enlargement and a left atrial clot measuring 12.8 mm x 13.2 mm, severe tricuspid regurgitation (TR) (pressure gradient of 57.8 mm Hg), hypokinesia of antero-lateral wall of the left ventricle with left ventricle ejection fraction of 35% to 40% (Figures -).
Chest radiography revealed cardiomegaly with cardiothoracic ratio 70%, straightening of left heart border, double right heart border, cephalization (upturned moustache sign or Antler sign), and widened carinal angle (more than 90o) typical of left atrial enlargement. The cardiac border was displaced laterally and downwards implying left ventricular enlargement (Figure ).
A computed tomography (CT) scan of the head showed right-sided ischemic stroke involving more than one-third of the middle cerebral artery territory (Figure ).
Arterial doppler of the right leg showed nearly occluding echogenic content in right femoral artery with minimal triphasic flow and occluding echogenic content in right popliteal artery with no flow in color and spectral Doppler study. No flow was noted in the right tibioperoneal trunk, anterior tibial artery, posterior tibial artery, and dorsalis pedis artery. Venous Doppler of the right leg demonstrated deep vein thrombosis (DVT) involving right femoral and popliteal vein. Abdominal ultrasonography was normal.
At workup, the patient had total creatine phosphokinase (CPK) 10000 unit per liter (U/L) (normal= 10 to 120 U/L), creatine phosphokinase-MB (CPK-MB) 3000 U/L (normal= 0-25 U/L), troponin I 40.6 nanogram per mililiter (ng/ml) (positive for > 0.12 ng/ml), total white cell count 18,210 per microliter (uL) (normal= 4000-11000 per uL), hemoglobin 15.4 gram per deciliter (g/dl), creatinine 3.28 milligram per deciliter (mg/dl) (normal= 0.6 to 1.1 mg/dl), sodium 122 milliequivalent per liter (mEq/L) (normal= 135 to 145 mEq/L), potassium 6.2 mEq/L (normal= 3.5 to 4.5 mEq/L), total billirubin 1.4 mg/dl (normal is < 1 mg/dl), alanine trasaminase (ALT) 516 U/L (normal= 30 to 65 U/L), aspartate trasaminase (AST) 2030 U/L (normal= 0 to 45 U/L), alkaline phosphatase 117 U/L (normal= 40 to 140 U/L), total protein 6.8 g/dl (normal= 6.4 to 8.2 g/dl), albumin 3.2 g/dl (normal= 3.8 to 4.9 g/dl), and prothrombin time (PT) was 13.3 second with international normalized ratio (INR) 1.1 (control= 12 second). Serum ASO titer was not done as there was no active rheumatic activity.
A provisional diagnosis of RHD with severe MS and TR with AF leading to multiple systemic embolizations was formulated. Anticoagulation was started with low molecular weight heparin (enoxaparin 60 mg subcutaneous twice daily). The patient received aspirin 75 mg and digoxin 0.125 mg once daily. Hyperkalaemia was managed medically with intravenous calcium gluconate one gram over 10 minutes, 50 ml of 50% dextrose with regular insulin of 10 U and salbutamol nebulization. Subsequently, the patient underwent above knee amputation of the right lower limb. His liver and kidney function tests were monitored until they were normalized. He was discharged on warfarin 6 mg, aspirin 75 mg, spirinolactone 25 mg, enalapril 2.5 mg, digoxin 0.125 mg once daily and frusemide 20 mg twice daily. The patient was adviced for regular follow-up for monitoring his clinical status and adherence to medications. He paid two visits (one week apart) to the hospital after discharge and his clinical conditions were improving. |
pmc-6135310-1 | A 32-year-old male on methadone for chronic back pain control presented for an outpatient follow-up appointment where he received an EKG for QTc monitoring, revealing an incidental new-onset atrial bigeminy (Figure ). He was sent to the emergency department for further evaluation. Telemetry and repeat EKG showed resolution of arrhythmia. Given the patient’s complaints of slowly worsening dyspnea requiring supplemental oxygen over a few-month period and new-onset arrhythmia, a CT-angiogram (CTA) of pulmonary arteries was performed. While pulmonary embolism was ruled-out, CTA revealed a PAPVC involving the pulmonary vein in the left upper lobe and lingula, returning blood to the right atrium through the left brachiocephalic vein (Figure , Figure ). An echocardiogram ruled-out ASD but revealed a mildly dilated right ventricle (Figure ), mild tricuspid valve regurgitation and normal left ventricular function. Notably, the patient had morbid obesity, obstructive sleep apnea, and acute lymphocytic leukemia with bone marrow transplant and graft-versus-host disease causing severe restrictive lung disease (total lung capacity: 42%; FEV1: 39%; FVC: 37%; and DLCO: 41% of predicted values). By that time, the patient was on two liters of oxygen supplementation around the clock at home and he had a functional capacity of four metabolic equivalents (METS). In the setting of the patient’s progressively worsening severe dyspnea requiring further evaluation of the shunt, a right and left heart catheterization was performed. It revealed pulmonary hypertension with pulmonary artery systolic pressure of 52 mmHg, pulmonary artery diastolic pressure of 43 mmHg, mean pulmonary artery pressure of 40 mmHg pulmonary vascular resistance of 1.7 Wood Units, pulmonary capillary wedge pressure of 25 mmHg and cardiac output (Fick) of 7.47 L/min. It also revealed a shunt-index, pulmonary to systemic flow ratio (Qp/Qs), of 1.22:1. Despite his severe symptoms and echocardiogram findings, a multi-disciplinary meeting concluded that the patient would not benefit from surgical intervention. Nine months later, our patient didn’t show signs or symptoms of clinical deterioration. He had no increased requirements for oxygen supplementation and his functional capacity remained stable at four METS. |
pmc-6135561-1 | A 9-year-old boy presented to our pediatric emergency department with abdominal pain. The pain was initially located at the periumbilical area and then migrated to the right lower abdomen. Fever for 2 days was reported. The patient had not experienced nausea or vomiting. His physical examination revealed abdominal tenderness over the right lower abdomen, without peritonitis. Laboratory analysis revealed a white blood cell count of 13,100 μL (range: 3500–9100 μL; neutrophilia, 84.9%) and creatinine level of 0.71 mg/dL (range: 0.70–1.30 mg/dL). Abdominal CT revealed a fecalith at the tip of the appendix and absence of hydronephrosis in the bilateral kidney (). Because acute appendicitis was suspected, the patient underwent LA. Acute perforated appendicitis with turbid diffused ascites was noted.
The patient received triple antibiotic therapy postoperatively (ampicillin, 1000 mg four times daily; metronidazole, 260 mg three times daily; and gentamicin, 50 mg two times daily). The early postoperative course was uneventful, with adequate urinary output of 2800 mL/day and white blood cell count of 9700 μL (neutrophilia, 67.5%). During the operation, ascites culture grew Escherichia coli, Streptococcus constellatus, and Pseudomonas aeruginosa. After observing good bowel function, well-tolerated diet, and reduced pain, outpatient follow-up was scheduled, and the patient was discharged.
However, during follow-up at 10 days postoperatively, the patient complained of mild abdominal discomfort with poor appetite and reported vomiting twice. No fever was reported, and his wound was dry, clean, and healing well. Physical examination revealed knocking pain over the bilateral flank area. Follow-up with abdominal ultrasonography revealed bilateral hydronephrosis, but no intra-abdominal abscess (). Laboratory analysis revealed a white blood cell count of 13,900 μL (range: 3500–9100 μL; neutrophilia 82.5%) and creatinine levels of 12.85 mg/dL (range: 0.70–1.30 mg/dL). Oliguria was also noted. Kidney, ureter, and bladder radiograph (KUB) studies revealed increased stomach and bowel gas patterns, but no obvious ureteral stone formation (). Because bilateral ureteral obstruction with hydronephrosis was suspected, cystoscopy was conducted. Bilateral obstructing stones were noted at the right ureteral orifice and left ureter, ∼2 cm proximal to the ureterovesical junction, resulting to severe hydronephrosis (). We used flexible 4 mm ureteroscope and forcep for extracting the stones. No ureteral meatotomy or laser was used. Bilateral Double-J catheters (F 4.7 × 24 cm) were inserted. Postoperation, KUB follow-up revealed that the right Double-J catheter was appropriately placed; however, it was observed to be kinking at the ureter. Laboratory analyses conducted on postoperative day 2 revealed a white blood cell count of 12,300 μL (range: 3500–9100 μL; neutrophilia 84.9%) and creatinine levels of 0.76 mg/dL (range: 0.70–1.30 mg/dL). Kidney sonogram revealed mild left hydronephrosis. The bilateral Double-J catheter was removed 1 month after outpatient follow-up, and only a mild blood clot was noted at the right ureter. Furthermore, the kidney sonogram revealed no hydronephrosis. |
pmc-6136219-1 | A 68-year-old Japanese man, who had a history of total gastrectomy for gastric cancer and transcatheter arterial embolization for left adrenal gland aneurysm rupture, had been transported to our emergency department by ambulance several times. He had a history of repeated hypoglycemia after meals, leading to a diagnosis of late dumping syndrome. Prior to the most recent admission, he had abdominal pain followed by diarrhea after breakfast at approximately 8:30 a.m. He was found unconscious sitting on the toilet seat at approximately 9:00 p.m. and was transported to our emergency department 20 minutes later.
On arriving at our hospital, he was unable to describe his symptoms. His vital signs were as follows: Glasgow Coma Scale score, E3V4M6; respiratory rate, 30 breaths/minute; oxygen saturation, 99% under room air; blood pressure, 60/28 mmHg; heart rate, 90 beats/minute; and body temperature, 36.1 °C. Arterial blood gas analysis revealed metabolic acidosis with respiratory compensation, hyperglycemia, and hyperlactatemia (Table ). Blood biochemistry findings were within the normal limits (Table ). After rapid administration of 2000 mL of bicarbonate Ringer’s solution, his systolic blood pressure transiently increased to 100 mmHg, but this increase was not sustained. A chest radiograph and computed tomography images of his brain and whole body revealed no abnormal findings. Ultrasonography revealed normal contractility of his heart and collapse of the inferior vena cava. He was transferred to our intensive care unit (ICU) with further administration of bicarbonate Ringer’s solution.
In the ICU, his lactate was decreased to 7.4 mmol/L, while the hypotension persisted. A central venous catheter was inserted into the right internal jugular vein, and continuous infusion of noradrenaline was started and increased to 0.13 μg/kg per minute. Antibiotics were not administered as neither blood chemistry nor imaging revealed any findings of infection. As his hemodynamics gradually stabilized, after 3500 mL of fluid administration, continuous infusion of noradrenaline was stopped 4 hours after the initial infusion. He was able to eat supper on the same day and was discharged from the ICU on the following day. During admission, the plasma cortisol level was found to be normal; therefore, no steroids were administered. |
pmc-6136232-1 | A 66 year old Caucasian woman, was admitted to hospital with malaise, macroscopic haematuria and a petechial rash on both thighs. Her past medical history included seronegative rheumatoid arthritis and hypertension. Her kidney function at that point was normal, with a creatinine of 73 μmol/l (eGFR > 60 mls/min/1.73m2, MDRD formula), and there was no proteinuria. Investigations revealed negative ANA, ANCA, anti-GBM antibodies as well as hepatitis B and C serology. A kidney biopsy showed mild focal tubular and interstitial scarring, suggestive of modest chronic ischaemic damage, but no significant glomerular lesion and negative immunoperoxidase staining on formalin fixed tissue for all immunoproteins. Rapid resolution of the rash was seen following a course of high-dose prednisolone.
Four months following the discontinuation of prednisolone and after a flu like illness, the rash recurred, along with macroscopic haematuria, malaise and anorexia. On admission, she had a blood pressure of 162/90 and a purpuric rash over both thighs. Creatinine had risen to 241 μmol/L (eGFR 18 mls/min/1.73m2). Haemoglobin 10.9 g/dL, CRP 138 mg/L. Urine protein: creatinine ratio (PCR) was elevated at 150 mg/mmol; Tests revealed negative ANCA, ANA and rheumatoid factor, normal levels of immunoglobulins and complement. No anti-GBM antibody was obtained at this time. A repeat kidney biopsy demonstrated a severe, acute crescentic pauci-immune glomerulonephritis, with evidence of moderate chronic kidney damage. 13/22 glomeruli showed evidence of vasculitic lesions, 3 were globally sclerosed and 6 were normal. Immunoperoxidase and immunofluorescence were negative for IgG, IgM and IgA and C3 (Fig. ).
Treatment with intravenous methylprednisolone and cyclophosphamide was commenced. An anti-GBM antibody titre, obtained 2 weeks post-discharge, was significantly elevated at 359 IU/ml (NR 0–10, ELiA, Phadia systems, Thermo Scientific). This elevation was confirmed on repeat testing. 14 cycles of plasma exchange were performed with 6 doses of pulsed cyclophosphamide. At four months creatinine was 130 μmol/l and at 4 years 105 μmol/l (eGFR 37 and 47 mls/min respectively)while ANCA and anti-GBM have remained negative (Table ). |
pmc-6136232-2 | A 70 year old previously healthy Caucasian woman presented with lethargy, anorexia, nausea, vomiting, and a two kilogram weight loss over the course of a fortnight.
Investigations showed an elevated serum creatinine of 477 μmol/L (eGFR 9 ml/min, CKD-EPI formula), with a previous creatinine of 68 μmol/L (eGFR 91 ml/min) 3 months earlier. The blood pressure was 180/100 mmHg, other physical findings were normal. Urinalysis revealed blood and protein, while microscopy confirmed erythrocytes, leukocytes, dysmorphic red cells, but no red cell casts. Serology showed positive MPO-ANCA with a titre of 20 IU/ml (NR 0–5) and high titre anti-GBM antibody titre > 200 IU/ml (NR 0–20; Alegria ELISA, Orgentec) and 475 U/ml (using EliA Phadia assay), complement levels were normal. Kidney function deteriorated over the next few days, with creatinine reaching 809 μmol/l (eGFR 5 ml/min). Kidney biopsy showed diffuse extracapillary necrotizing glomerulonephritis, interstitial inflammation and leucocytoclastic necrotizing vasculitis. Immunohistochemistry on formalin fixed tissue showed no immune deposits along the GBM (Fig. ). She was treated with methylprednisolone pulses, followed by oral prednisolone. Haemodialysis and 7 plasma exchanges were also initiated followed by intravenous cyclophosphamide. Although urine output improved, she remained dialysis dependent and her repeat anti-GBM antibody remained strongly positive. She therefore underwent 3 sessions of immunoabsoprtion on a protein A column which led to a drop in anti-GBM titer from > 200 IU/l to 25 IU/l. Due to severe leucocytopenia with consecutive pneumonia following the first dose of cyclophosphamide, she was switched to weekly rituximab, which was also stopped after the third dose following further infections. Anti-GBM antibodies were negative at 4 months. She has remained dialysis dependent 24 months later (Table ). |
pmc-6136232-3 | A 64 year old man presented to a local community hospital with a 5 week history of dry cough and fevers which has persisted despite 2 courses of oral antibiotics. There was a 10 year history of unclassified arthralgia. Presenting creatinine was 682 μmol/L Urinalysis: 3 + blood and protein. Urine microscopy revealed granular casts with greater than 200 red cells/ cu.mm. Urinary protein: creatinine (uPCR) ratio was 133 mg/mmol. Physical examination was unremarkable, but BP 186/74 on admission. MPO-ANCA titre was 60 IU/ml (NR < 5) and a positive anti-GBM titre of 33 IU/ml (NR < 10, ELiA, Phadia systems). Complement levels normal. Renal biopsy revealed crescentic glomerulonephritis in 80% of glomeruli. However, no glomerular deposition of immunoglobulin or complement was seen on formalin fixed tissue (Additional file : Figure S2). The patient was treated with plasma exchange, pulsed methylprednisolone, followed by oral prednisolone and six pulses of intravenous cyclophosphamide. Maintenance therapy was with a reducing course of prednisolone and mycophenolate mofetil, due to azathioprine intolerance. At 21 months of follow up serum creatinine was 127 μmol/L, and both MPO-ANCA and anti-GBM were negative (Table ). |
pmc-6136232-4 | A 79 year old woman presented to a local hospital with a 2 week history of epistaxis, arthralgia, weight loss and anorexia. She gave a history of previous recurrent iritis, with the last episode occurring 2 year prior to her presentation. Physical examination was unremarkable apart from peripheral oedema. Presenting creatinine was 430 μmol/L. Urine dipstick revealed + 3 blood and + 2 protein, and uPCR 259 mmol/mg. MPO-ANCA titer was 33 IU/ml(NR < 5) and an anti-GBM titre of 28 IU/ml(NR < 10, ELiA, Phadia systems). Renal biopsy revealed a pauci-immune crescentic glomerulonephritis, with no staining for IgG, IgA or IgM on formalin fixed tissue. There was some chronic parenchymal damage (Additional file : Figure S2). Treatment was with methylprednisolone, a short course of oral prednisolone, rituximab and six pulses of intravenous cyclophosphamide. She was also intolerant of maintenance azathioprine. Her MPO-ANCA and anti-GBM titres remained < 1 IU/ml and her creatinine was 199 μmol/L after 18 months’ follow-up (Table ). |
pmc-6136464-1 | A 69-year-old woman with a history of hypertension was identified to have an incidental renal tumor on computed tomography (CT). She denied hematuria, lower urinary tract symptoms, pain, fever, fatigue, or weight loss. Her medical history was significant for hypertension and obstructive sleep apnea. Family history and social history were noncontributory. Physical exam at the time of presentation was normal.
On CT, the tumor was an exophytic, enhancing mass (3.0 × 2.0 × 3.5 cm), arising from the lower lateral pole of the left kidney with areas of low attenuation at its inferior aspect. The remainder of the urinary system was normal. No adenopathy or sign of metastasis was detected. An imaging obtained later the same year demonstrated no interval change in the size of the lesion. Biopsy was positive for an oncocytic neoplasm, which at the time was classified as an onocytoma. The patient was managed conservatively and presented 2 years later for repeat imaging.
A repeat CT was significant for an interval increase in the size of the mass from 3.5 to 5.6 cm in the greatest dimension. Tumor compressed the lower pole calyces without ureteral obstruction. There was no radiologic evidence of tumor calcification, fat, or infiltration into the adjacent tissues. Fine needle aspiration and the biopsies of the mass were again consistent with an oncocytic neoplasm.
Comparative radiologic images are provided (); the top images are radiographs with a smaller tumor from 2 years ago, while the bottom radiographs are from the patient's recent CT with a larger tumor. Given the unusual clinical features and behavior of the tumor, the patient was referred for a radical nephrectomy.
The specimen received at the pathology lab was composed of an intact kidney and perinephric adipose tissue (205g, 8.5 × 8.0 × 4.2 cm). At the midlower pole of the kidney, there was a circumscribed, cystic, and focally solid mass (6.0 × 4.7 × 4.5 cm). The mass had a tan-brown multiloculated cut surface (). There was no gross invasion of the renal vessels, ureter, or perinephric fat.
The majority of tumor was composed of monotonous cells with distinct borders, abundant eosinophilic cytoplasm, raisinoid nuclei and perinuclear halos, raising a possibility of an eosinophilic chromophobe carcinoma. As anticipated, this cellular component had cytoplasmic staining with Hale colloidal iron and membranous staining with c-kit (CD 117) and Ec-adherin. It was negative with vimentin.
A smaller subset of tumor cells had increased atypia, higher grade, hobnail morphology, and a tubulocystic architecture set within a desmoplastic stroma. This component was negative with Hale-Colloidal Iron and Racemase and had foci of intraglandular staining with mucin. CK7 and PAX-2 were positive but PAX-5 was negative in both components. Vimentin was only positive in the higher-grade tumor component, morphologically and by immunoprofile consistent with collecting duct carcinoma (see Figures and for the H&E and staining images).
Proliferative index, assessed by Ki-67 labeling, was low in the chromophobe (0–5% nuclear staining) and high in the collecting duct carcinoma component (60% of nuclei).
On EM analysis, although the morphologic preservation was somewhat compromised by formalin fixation and paraffin embedding, ultrastructural details of the two types of tumor cells were readily visible and distinctive. The tumor section represented by monotonous cells contained rounded cells with centrally located nucleus (). At higher magnification, their cytoplasm was found to be packed with mitochondria and prevalent electron dense microvesicles (). The central tubulocystic-appearing regions contained elongated epithelioid cells with large, irregularly shaped nuclei (). At higher magnification, the plasma membrane of these cells possessed abundant microvillar projections with junctional complexes joining adjacent cells (). The cytoplasm was rich with organelles including mitochondria and rough endoplasmic reticulum. |
pmc-6136467-1 | This is a case of a 68-year-old female presented to the clinic for a 2-month history of recurrent left ankle pain, associated with swelling and edema. The patient had a history of thyroid follicular adenocarcinoma which was treated surgically with total thyroidectomy and postoperative iodine treatment 25 years prior to presentation. At 12 years post thyroidectomy, she was diagnosed with a calcaneal mass of the same pathology and diagnosed with delayed distant thyroid carcinoma metastasis to the calcaneus. 10 years after calcaneal mass excision, the patient was diagnosed with a proximal tibial mass that turned out to be also delayed metastasis of the same pathology.
At the first presentation 15 years ago, she initially presented complaining of mild edema of the left ankle with intermittent pain upon daily activity. Physical exam showed full range of motion of the ankle joint with intact motor power and intact neurovascular status. There were no cutaneous lesions.
Plain radiographs of the left ankle showed a 3-centimeter oval lytic lesion in the anterior aspect of the calcaneum ().
An MRI of the left ankle showed a 3.2 cm well-defined benign-looking lytic lesion of the calcaneal neck reaching the cortex which appeared to be mildly irregular with mild degenerative disease of the posterior subtalar joint consistent with an intraosseous ganglion cyst of the calcaneus ().
Medical treatment with NSAIDs and paracetamol was initiated, along with partial weight bearing and relative rest with no improvement at follow-up at 4 weeks. Surgical intervention was decided due to the persistent pain. The cyst was resected with a margin of surrounding fibro-osseous tissue and the bone grafted. Histopathological evaluation revealed a metastatic carcinoma of the thyroid gland. Immunostaining showed that the cells expressed cytokeratin, cytokeratin 7, and thyroglobulin, all of which confirm the diagnosis ().
For further confirmation, the recently excised cyst slides were compared to the pathology slides of the thyroid excision undertaken 12 years prior to the calcaneal presentation and were found out to be of the same pathology ().
The patient had a smooth postoperative hospital stay and clinical recovery from pain before discharge; postoperative follow-up showed necrosis of the upper part of the wound which healed by secondary intention.
The patient had a complete bone metastasis workup; chest X-ray showed a right upper lobe nodule for which an FNA biopsy under CT scan was done demonstrating the same pathology as for the calcaneus. A bone scan was ordered showing no definitive sign suggesting metastasis with no specific abnormality of the manubrium-sterni joint and the right proximal metaphysis of the right tibia which was nonspecific for distant metastasis according to the nuclear radiologist. After the discussion with the oncologist, decision for radioactive iodine therapy was made.
At 3-month postoperative follow-up, while the patient was undergoing chemotherapy, she recomplained of ankle pain upon ambulation, associated with edema. The patient had 5/5 motor strength, no numbness, and no sign of infection. A left ankle X-ray and MRI were ordered () to rule out any recurrence at the surgical site. The new investigations showed oval-shaped lytic lesion of 2.8 cm, and MRI showed increase in size of the calcaneal mass without pathologic fracture.
The patient was treated symptomatically; a follow-up MRI at 8 months showed postoperative enhancement in the surgical bed suggestive of viable tissue, and at 10 months, a follow-up MRI showed that there was near total healing of the calcaneal region.
After 10 years post calcaneal mass excision, keeping in mind that the patient was symptom-free with respect to her ankle since the surgery, the patient presented to the clinic complaining of recurrent right knee pain. Investigation showed right proximal tibial metastasis with invasion of the patellar tendon. Surgical resection of the metastasis with repair of the patellar tendon was performed, and pathology was also consistent with thyroid follicular cell carcinoma (Figures and ).
The oncologist was consulted, and the decision for treatment with teroglobulin as well as adjuvant radiation therapy was made. |
pmc-6136468-1 | A 5-year-old girl who had no previous medical history was admitted to our hospital with a right groin mass for 2 months and abdominal distension for 15 days. Her symptoms were accompanied by occasional umbilical pain, vomiting, and diarrhea. Physical examination showed acute facial features, malnutrition, abdominal swelling, apparent tenderness, a mass approximately 4 × 3 × 2 cm in size in the right inguinal region, and negative Grey Turner sign and Cullen sign. Routine blood test revealed white blood cells 2.97 × 109/L, neutrophils 47.2%, red blood cells 4.88 × 1012/L, platelets 714 × 109/L, and C-reactive protein 2.06 mg/L. Abdominal enhanced computed tomography (CT) revealed the following findings: (1) the pancreatic duct was significantly dilated, and the surrounding pancreatic head space was unclear; (2) a cystic low-density shadow was observed in the head of the pancreas (); (3) massive peritoneal effusion was observed; (4) patchy lymph node enhancement and enlargement were observed in the mesentery; and (5) intestinal aggregation in the upper abdomen along with thickening and enhancement of the bowel wall was observed. Chest CT revealed increased lung markings and no signs of TB.
After admission, the patient presented with diffuse abdominal distension. Laboratory tests indicated pancreatitis (serum lipase 3167 U/L and serum amylase 720 U/L). Serum cancer antigen- (CA-) 125 was increased to 484.5 U/mL (normal is less than 35 U/mL). An abdominal puncture was performed three days after admission. Ascites was red and yellow. Qualitative protein was positive. No acid-fast bacilli were identified via ascites smear. The anti-TB antibody in serum was negative. The purified protein derivative of the tuberculin test and interferon-gamma release assay were negative. No tumor cells were identified in the ascites, and there was no bacterial growth in the ascites for 48 hours. Seven days after admission, the patient underwent laparoscopic exploration. In total, 3600 mL of bloody ascites was present in the peritoneal cavity. Pale and fish-like masses were found. A wide and dark-red flocculent area was visible. Pathological examination revealed an inflammatory exudate. The results of periodic acid-Schiff staining, methenamine silver staining, and acid-fast staining were negative. No bacteria or tubercle bacilli were found in the ascites cultures. Using polymerase chain reaction (PCR), Mycobacterium tuberculosis DNA was identified in the resected abdominal mass. Therefore, the diagnosis of pancreatic TB was made. Fasting, somatostatin, omeprazole, and total parenteral nutrition therapy were administered. Rifampicin (10 mg/kg/day), pyrazinamide (20 mg/kg/day), and isoniazid (10 mg/kg/day) were prescribed. Three weeks later, abdominal pain, abdominal distension, cough, fever, nausea, vomiting, and diarrhea had resolved. The patient was discharged.
Two weeks after discharge, pain around the umbilicus and periumbilical tenderness were reported with nausea and vomiting. Abdominal ultrasonography revealed large pancreatic cysts. The diagnosis of a pancreatic pseudocyst was made, and ultrasound-guided puncture drainage was performed. Giant cystic lesions as thick as the wall and approximately 12 × 9 × 10 cm in size were observed by intraoperative ultrasonography. Approximately 500 mL of brown cyst fluid was expelled. On day 4 after the operation, the patient's general condition was good, and her vital signs were stable. Therefore, she was discharged again. Outpatient antitubercular therapy consisted of isoniazid, rifampicin, and pyrazinamide for 4 months and subsequently isoniazid and rifampicin for 8 months.
At the one-year outpatient follow-up visit, her appetite improved, and she regained the weight she had previously lost. Abdominal CT showed a significant reduction in the cystic low-density shadow of the head and neck of the pancreas (). The effect of the antitubercular therapy was significant. |
pmc-6136481-1 | A 20-year-old medically fit and healthy male presented for an assessment in preparation for orthognathic surgery. On examination, a 1.5 cm diameter exophytic midline tongue lesion () was noted. This lesion was smooth, regular, and soft to palpation. He reported that this had been present since birth with no change since childhood. A magnetic resonance image (MRI) of his tongue was obtained ().
The MRI report described a 1.5 cm protuberant mass arising from the dorsal aspect of the tongue in the midline at the approximate junction of the oral component and base. The imaging suggested that this was in part fatty, probably arising from the submucosa, and is also seen to demonstrate very mild contrast enhancement. There appeared to be intact overlying mucosa and no apparent involvement of the intrinsic tongue muscles. The sublingual space and salivary glands appeared normal.
Thereafter, the mass was surgically excised and submitted for histological examination including haematoxylin and eosin staining and immunohistochemistry.
Histologically, a hamartoma is characterized by a combination of fibrovascular connective tissue, smooth-muscle bundles, skeletal muscle fibers, adipose tissue, salivary tissue, blood vessels, lymphoid tissue, peripheral nerves, and ganglion cells. One type of tissue is determinant in each lesion. In this specimen, microscopy revealed circumscribed nodules covered by stratified squamous epithelium, and interlacing cords of eosinophilic spindle-shaped cells consistent with the smooth muscle within the lamina propria (Figures –). These mature spindle cells with the profile of smooth-muscle cells were determinant of a leiomyomatous hamartoma. Immunostaining for α-smooth-muscle actin demonstrated large concentrations of smooth-muscle bundles; however, S-100 was found only in peripheral nerve bundles intermingled with smooth-muscle fibers (Figures and ). No nuclear atypia, cellular pleomorphism, mitosis, or necrosis was noticed, consistent with the benign and developmental nature of these lesions. Based on these features, a histological diagnosis of a leiomyomatous hamartoma was made. |
pmc-6136482-1 | A 60-year-old woman with arthralgia and back pain lasting for several months and recent metatarsophalangeal luxation of the left toe presented to the ambulatory unit of rehabilitation clinics. She was referred to the clinics as a patient with chronic pain syndrome. The patient had joint hypermobility since childhood, diagnosed as ligament laxity. In adolescence, she is remembered to be called “a clumsy freak” due to joint mobility.
At physical evaluation, marfanoid habitus with waxy, sagging skin and varicose veins in the feet was observed. Sclerae were bluish and eyelids dropping. In fingers and toes, spontaneous subluxation in all joints could be elicited. Elbows, knees, and all fingers were overextended. She had flat feet with a bilateral hallux valgus (). She had scoliosis with prominent kyphosis. Lungs auscultation was characteristic of chronic obstructive pulmonary disease (COPD); the murmur of mitral valve prolapse was heard over the chest.
On the Beighton score, she received all (9) scores: passive apposition of the thumb to forearm and passive dorsal hyperextension of the metacarpophalangeal joint >90° on both sides were done with no strain (); she was able to actively hyperextend both elbows and knees on both sides over >10° and flex her spine to the ground with palms placed on the ground without knee flexing. On Five-point Hypermobility Questionnaire, she answered “yes” to all questions. She remembered vividly contorting her body into strange shapes and being called names by other children. Genetic analysis showed a typical mutation consistent with the classical Ehlers–Danlos syndrome.
In the ambulatory unit, she received systematic, light, nonweightbearing, and proprioception exercises; she was referred to the occupational therapist for lower limb orthosis; she was taught relaxation techniques including mindfulness-based stress reduction and counselling support, though she was already familiar with cognitive behavioural therapy. She used regular anti-inflammatory drugs. She was referred to the plastic surgeon due to the wound on her foot, since sutures should be applied generously, without tension, in layers, and left in place twice as long as usual. She was already regularly followed by her cardiologist due to mitral valve prolapse. |
pmc-6136483-1 | A 17-year-old male professional motocross athlete with a history of left tibial spine avulsion fracture and resultant chronic knee flexion contracture presented to the emergency department (ED) status-post motocross injury with isolated left thigh pain. The patient had been wearing a hard-shell, hinged, knee brace measuring approximately 43 cm in length. He reported riding over a jump of approximately 10 feet when his left leg slipped off, pinning and hinging his leg over his knee brace. He was found to have a closed and neurovascularly intact transverse femoral shaft fracture without ecchymosis, skin changes, or open wounds. The deformity measured approximately 26 cm from the tibial tuberosity on clinical exam, and the fracture was 22 cm proximal to the center of the knee as measured on anterior-posterior (AP) radiograph (). Per institutional protocol, thin-slice computed tomography (CT) was obtained to rule out femoral neck fracture, and this was negative for fracture []. The patient was placed in Buck's traction and prepared for surgical intervention. Anterograde intramedullary nailing of the left femur with a femoral reconstruction nail was performed the next morning. The patient received routine perioperative antibiotic prophylaxis, unrestricted postoperative weightbearing, and one month of chemical deep vein thrombosis (DVT) prophylaxis. The patient had returned to full activity and competitive motocross at one-year follow-up. |
pmc-6136483-2 | A 17-year-old male professional motocross athlete with a history of pediatric left tibial shaft fractures (treated nonoperatively and complicated by painless varus malunion) presented to the ED after crashing his dirt bike. He had been wearing a hard-shell, hinged, knee brace measuring approximately 42 cm in length when he fell on his left side and hyperextended his left leg over the top of his knee brace. The patient complained of isolated left thigh pain. Evaluation of the patient revealed a closed, neurovascularly intact transverse femoral shaft fracture without ecchymosis, skin changes, or open wounds. The deformity was approximately 27.0 cm from the tibial tuberosity on clinical exam and measured 21.1 cm proximal to the center of the knee on AP radiograph (). His baseline tibial deformity was unchanged. Again per institutional protocol, thin-slice CT pelvis was obtained to assess for associated femoral neck fracture, and this was negative []. The patient was taken to the operating room (OR) the next morning and was treated with an anterograde femoral reconstruction nail with cephalomedullary screws. He received routine perioperative antibiotic prophylaxis, unrestricted postoperative weightbearing, and one month of chemical DVT prophylaxis. At one-year postoperative follow-up, the patient had regained full function and had returned to motocross at his preinjury level. |
pmc-6136487-1 | A 23-year-old female was referred to our medicine dental department in UHC Sahloul, Sousse, for treatment of tooth #46. She suffered from major coronal destruction and needed to have her first molar restored. The medical history was noncontributory. Radiographic and clinical examinations were performed initially, and an extensive glass ionomer cement restoration of a nonvital tooth (46) was identified (Figures and ). The tooth was treated endodontically. The patient had an acceptable oral hygiene and a favorable occlusion. After removing the restoration, an endocrown restoration was recommended because of the amount of remaining tooth structure and the thickness of the walls (). The prosthetic decision was to restore tooth (46) with an endocrown fabricated from lithium disilicate ceramic (IPS e.Max CAD). The preparation for the endocrown is different from the conventional complete crown. This monolithic, ceramic adhesive restoration requires specific preparation techniques to be suitable for especial biomechanical needs.
This is aimed at achieving achieve an overall reduction in the height of the occlusal surface of at least 2 mm in the axial direction and to get a cervical margin or “cervical sidewalk” in the form of a butt joint. The cervical margin has to be supragingival and enamel walls less than 2 mm have to be eliminated.
Differences in levels between the various parts of the cervical margin should be linked by a slope of no more than 60° to escape a staircase effect. We used a cylindrical-conical diamond bur held parallel to the occlusal plane, to reduce the occlusal surface. Then we used a diamond wheel bur to control the orientation of the reduction and to guarantee a flat surface thanks to its shape.
We used a cylindrical-conical diamond bur with a total occlusal convergence of 7° to create continuity between the coronal pulp chamber and endodontic access cavity. The bur was orientated along the long axis of the tooth; the preparation was done without too much pressure and without touching the pulpal floor.
Removing too much tissue from the pulp chamber walls will reduce their thickness and the width strip of enamel. The depth of the cavity must be at least 3 mm.
The entrance to the pulpal canal was opened. Gutta-percha was removed to a depth not exceeding 2 mm to profit from the saddle-like anatomy of the cavity floor. Nonabrasive instrument was required to maintain the integrity of the canal entrance. No drilling of dentin was carried out. The remaining tooth structure was still strong ().
We ended the preparation with lining the root canal entrances with glass ionomer cement to protect the orifice of the canal ().
After evaluating the entire cavity and the interocclusal space, the impression of the tooth was taken by double impression technique using additional silicone. After visualization and analysis of the quality of the impression, we selected the ceramic shade and sent the impression to the laboratory.
A provisional acrylic resin restoration was made by using block technic and cemented with eugenol-free temporary cement (). The endocrown was fabricated in the laboratory using CAD-CAM technology and was positioned on the master cast ().
Then we made a try-in of the endocrown and tested occlusion, internal, and proximal adjustments. Right after this, we sent it back to the laboratory for application of the colorant and glaze. In the following session, the internal surface of the endocrown was etched with hydrofluoric acid, rinsed with water, and dried with an air syringe. Next, a coat of a silane coupling agent was applied for 1 minute and dried.
Rubber dam was used to achieve proper isolation, and then phosphoric acid was applied onto the tooth surface for 15 sec on dentin and 30 sec on enamel, then abundantly washed and dried, applied with adhesive, and polymerized for 20 sec with light curing.
A thin layer of a dual polymerizing resin was applied to the prosthetic endocrown and then was inserted into the tooth and polymerized at intervals of 5 seconds, making it easy to remove cement excesses. After that, it was polymerized for 60 seconds on all surfaces. The restoration was examined for any occlusal interference using ceramic finishing instruments (). The final restoration is shown in . |
pmc-6136504-1 | A 73-year-old woman was admitted to our hospital after a gastric tumor was identified by gastroscopy following medical examination. Gastroscopy revealed a 10-mm diameter, superficial elevated lesion with a slight central depression (type 0-IIa+IIc; ) at anterior wall of lower gastric body. Biopsy findings indicated a diagnosis of a poorly differentiated carcinoma. No lymph node or distant metastases was identified via computed tomography; however, submucosal invasion was suspected and, hence, an endoscopic submucosal dissection was performed for a therapeutic diagnosis.
Macroscopically, the tumor was 10 × 8 mm in size, well-circumscribed, and accompanied by hemorrhage (). Low-magnification microscopy confirmed the invasion of the submucosal layer (), whereas at high-magnification microscopy revealed cuboidal tumor cells with round to irregular-shaped nuclei, a prominent nucleolus, and clear cytoplasm characteristic of GCED (). A predominantly solid and trabecular growth pattern with a small proportion of tubular formation was also identified (Figures and ). The degree of nuclear atypia was severe, and cells with deformed nuclei or multinucleation were scattered. Mitosis was common, and atypical mitosis was also identified. Further findings included conspicuous stromal hemorrhage, abundant cytoplasmic glycogen (according to Alcian blue and periodic acid-Schiff staining ()), absence of mucin, and lymphovascular invasion. No CA component, hepatoid carcinoma, yolk sac tumor, or other histological cell types were found in any section. The tumor was surrounded by atrophic pyloric mucosa without intestinal metaplasia, and Helicobacter pylori was absent.
Immunohistochemically, the tumor cells were positive for the enteroblastic lineage biomarkers AFP (rabbit polyclonal, 1:250; Dako, Glostrup, Denmark), GPC3 (clone 1G12, prediluted; Nichirei, Tokyo, Japan), and SALL4 (clone 6E3, 1:800; Abnova, Taipei, Taiwan) (Figures –); they were negative for synaptophysin (clone 27G12, 1:100; Novocastra, Newcastle, UK) and HER2 (clone TAB250, 1:1; Zymed, San Francisco, CA, USA).
The chosen treatment was distal gastrectomy with lymph node dissection. The resected specimen indicated no lymph node metastasis and complete resection. Serum AFP level was normal after resection, although it was not examined before endoscopic submucosal dissection was performed. |
pmc-6136509-1 | An 8-year-old boy with chronic encephalopathy secondary to hypoxic ischemic syndrome, with cerebral palsy and symptomatic epilepsy, was admitted to the emergency department of the children's hospital. He had been seizure-free for the past year with an enteric-coated delayed release formulation of VPA (375 mg every 8 hours). Thirty days prior to hospital admission, he was started on LTG 25 mg/day along with VPA, since his seizures were no longer under control with VPA. Two weeks later, the dose was increased (50 mg/day). His morning trough plasma VPA level was measured before LTG was added to the therapy yielding a concentration of 85 mg/L. On admission, he presented macular lesions on the front of the thorax that extend to the back, followed by bilateral eyelid edema and ulcerated lesions at the level of lips, jugal mucosa, and pharynx. He developed erythematosus conjunctivitis with ulcers. He presented skin rash with high fever (39°C) and respiratory failure type I. History revealed that no such lesions occurred earlier and that was the first time such rashes have occurred. Other personal and family history was not relevant. From a dermatologic point of view and based on the history and clinical presentation, a diagnosis of SJS was made. Since the presumptive cause was LTG, the drug was discontinued immediately. Soon after the patient admission, periofocal and ocular involvement worsened. Intravenous immunoglobulin was administered for 48 hours. Mouth care (oral washes with sodium borate) and eye care (tobramycin ophthalmic drops) were also indicated.
From a hemodynamic point of view, four hours after admission, his condition deteriorated and he developed septic shock with peripheral circulatory failure. The patient was admitted to the intensive care unit with intravenous fluids and antibacterial therapy due to skin infection by Staphylococcus aureus. In addition to fluid resuscitation, dopamine was administered. Despite the inotropic treatment, the patient's condition did not improve, indicating a septic shock refractory to conventional vasopressor therapy but during treatment with milrinone and norepinephrine for six days (apart from the antibiotics), the septic shock was reversed.
His clinical state steadily improved over the following days. He made an excellent recovery under control seizure and was discharged after twelve days on admission with VPA (375 mg every 8 hours) and oral L-carnitine (2 g/day).
A blood sample (2 mL) of the patient was collected by venipuncture and was refrigerated (4–8°C) until analysis. The Wizard® genomic DNA purification kit was used to isolate the genomic DNA from whole blood. Then, it was quantified by spectrophotometry (260/280 nm) on a NanoQuant-Tecan instrument. EPHX genotype was determined by a real-time polymerase chain reaction using a TaqMan Drug Metabolism Genotyping Assay for rs1051740 and rs2234922.
Two polymorphisms, Tyr113His in exon 3 (SNP rs1051740 T > C) and His139Arg in exon 4 (SNP rs2234922 A > G), have been associated with a decrease or increase in enzyme activity, respectively [, ]. 113His/113His or 113His heterozygosity (mutated allele in exon 3) combined with His139/His139 (wild-type allele in exon 4) indicates a decrease in enzyme activity. An increase in activity occurs with 139Arg/139Arg or 139Arg heterozygosity (mutated allele in exon 4) combined with Tyr113/Tyr113 (wild-type allele in exon 3).
The genetic study revealed an increase in EPHX activity (wild-type allele in homozygosity for SNP rs1051740 and heterozygosity for SNP rs2234922).
This study was conducted in accordance with the principles of good clinical practice and the Declaration of Helsinki and was approved by the Ethics Review Committee of the Faculty of Chemistry (Uruguay). Written informed consent of the mother was obtained for the purpose of reporting this case. |
pmc-6136510-1 | We report the case of a previously healthy 38-year-old man, Afro-Brazilian, with no previous medical records. He presented to our emergency department with an acute onset of abdominal pain, jaundice, fever, nausea, weakness, and malaise. His arterial blood pressure was 90/50mmHg, heart rate was 90 bpm, axillary temperature was 35°C, and he was dehydrated. The laboratory examinations revealed serum creatinine of 0.8 mg/dl, platelets of 142 x 103/mm3, serum fibrinogen of 221 mg/dl, increased international nationalized ratio (INR 1.9), total bilirubin of 3.9mg/dl, direct bilirubin of 2.3mg/dl, ALT 751U/l, AST 540U/l, ceruloplasmin of 17 mg/dl, ferritin of 3200 ng/dl, iron of 276mcg/dl, TIBC (total iron-binding capacity) of 267mcg/dl, transferrin saturation of 103%, hemoglobin (Hb) of 14 g/dl, and hematocrit (Ht) of 41,3% without acute bleeding. US (ultrasound) showed signs of inflammation and liver fibrosis as well as iron overload. A liver biopsy was indicated. First ROTEM test showed in EXTEM CT 80s, CFT 105s, alfa-angle 70°, MCF 52 mm, and ML 37%; FIBTEM MCF 13 mm and APTEM MCF 53 mm and ML 20% (/Figures , , and ). Tranexamic Acid 1 g was administered to correct hyperfibrinolysis. Second ROTEM test presented improvement in the hyperfibrinolysis but not completely, so another 1 g of Tranexamic Acid was administered with EXTEM CT 64s, CFT 105s, alfa-angle 69°, MCF 51 mm, and ML 24%; FIBTEM MCF 11 mm, APTEM MCF 51 mm, and ML 20% (/Figures , , and ). The third ROTEM test showed persistence of maximum lysis above 15% with EXTEM CT 83s, CFT 133s, alfa-angle 66°, MCF 50 mm, and ML 21%; FIBTEM MCF 10 mm and APTEM MCF 51 mm and ML 19% (/Figures , , and ). This time 1500U of factor XIII concentrate was administrated to correct factor XIII deficiency and fourth ROTEM test was normal with EXTEM CT 64s, CFT 122s, alfa-angle 68°, MCF 50 mm, and ML 15%; FIBTEM MCF 12 mm and APTEM MCF 51 mm and ML 15% (/Figures , , and ). Thromboelastometry was normal despite CCT were still altered. So, liver biopsy was performed which succeeded with no signs of bleeding and without need of further transfusion. He was discharged from the hospital 5 days after admission. |
pmc-6136512-1 | A 57-year-old Caucasian male presented at the emergency department with acute chest pain and uncontrolled hypertension of 180/100 mmHg. Past medical history included ongoing smoking, high body mass index (BMI > 30), arterial hypertension (150/95 mmHg at rest), hyperlipidemia (serum LDL levels > 230 mg/dL), and a failed ablation for atrial fibrillation eight years previously. Medications at home were clopidogrel, acenocoumarol, oral amiodarone, a b-blocker, and a calcium-channel blocker, but it was unclear if these were taken as instructed. There was no history of vasculitis or other collagen diseases. The ECG on admission did not show ischemic abnormalities, and blood biochemistry led to the diagnosis of myocardial infarction with mildly elevated troponin level (0.4 ng/mL max value 0.1 ng/mL). His chest X-ray was unremarkable. Echocardiography was performed. The anterior-basic, anterior-septal, and the anterior-lateral portions of the myocardium of the left ventricle were hypokinetic. The left ventricular ejection fraction was calculated at 45%. No abnormal structural findings or mediastinal masses were noticeable. Coronary angiography was performed, and multiple large (over one centimeter) arterial aneurysms on both left and right coronary arteries were identified. In detail, the right coronary artery (RCA) was dilated just after its origin and along the vessel's entire length to a maximum diameter up to 43 mm and presenting with a thrombosed lumen, significant postaneurysm stenosis, and retrograde flow from the left coronary artery. The left main stem (LM) artery was 11.5 mm and gradually dilated in continuation with the anterior descending branch (LAD) to a maximum of 28 mm in diameter, but there was a patent lumen of internal diameter 9.3 mm distally. The circumflex artery (LCx) was dilated from 8.5 mm up to 12 mm for most of its tortuous length. A subsequent multislice computed tomography scan with intravenous contrast was performed to exclude the presence of other extracardiac aneurysms including the brain. The 3D reconstruction images obtained from the CT angiogram revealed the very interesting morphology of these GCAAs which was clinically overlooked in echocardiography at the acute assessment (). Myocardial revascularization was indicated due to these findings, and the patient was referred for cardiac surgery in the same week.
Standard median sternotomy was performed. Upon opening the pericardium, the operating field was dominated by large-sized coronaries and especially the RCA one which appeared larger than the aortic root (). There were no actual pressure effects on the cardiac chambers or the valvular mechanism. These aneurysms resembled hard extracardiac masses. The decision to explore the aneurysms and bypass them was taken. Bilateral internal mammary arteries were harvested for use as grafts. Cardiopulmonary bypass (CPB) was established, and cold blood-based cardioplegia (4 : 1) was delivered in antegrade initially and retrograde fashion through the coronary sinus as per standard. All aneurysms were sequentially dissected open starting with the largest RCA (). Thrombus material and calcified plaques were removed (). Samples were taken for histopathology, serology, and microbiology examination. This was repeated at the aneurysms of the left coronary artery and the circumflex artery. The LAD was dissected open distally to the stem, and endarterectomy was performed to provide an area for distal anastomosis (). A large calcified cast was sent for investigation (). Interestingly, all the distal vessel lumens had postaneurysmal stenosis as shown on . The dissected portions of the coronaries were ligated proximally and distally with prolene 5-0 sutures prior to the execution of the bypass. The pedicled left internal mammary artery (LIMA) was anastomosed to the LAD, the right internal mammary artery (RIMA) as a free graft to the marginal branch of the circumflex artery. Two saphenous vein grafts (previously harvested) were anastomosed to the RCA and the intermediate branch of the (LCx) artery, respectively. Aortic cross-clamp time was 224 min and total bypass time was 252 min. Intraoperative transfusion requirements were 2 units of red blood cells and 5 units of fresh-frozen plasma. There were no intraoperative complications, and he was transferred to the ICU on inotropic support with dobutamine 5 μg/kg/min weaned after eight hours and extubated after twelve hours. The patient was discharged on the sixth postoperative day after an uneventful stay. He was prescribed a long-life medication with oral salicylic acid 100 mg daily and acenocoumarol to target INR 2.0-3.0. The histopathological results were negative for arteritis, autoimmune pathology, or infection, and only changes related to coronary artery disease, i.e., media disruption with focal calcification and hyalinization with lipid and cholesterol crystal deposits were found. Follow-up at one and six months and thereafter a year with echocardiography was satisfactory as the patient remains pain-free and in full physical activity. |
pmc-6136515-1 | We report the case of a 59-years-old diabetic patient, without particular medical history, experiencing urgency, frequency, hesitancy, intermittency, straining, and slow stream and for three weeks.
He presented a recent onset of fever, myalgia, chill, and an episode of gross hematuria for which he was seen in a local hospital. Abdominopelvic ultrasonography showed thickness of the bladder wall without associated process and a hypoechogenic and enlarged prostate measuring 106,32 ml. Postmicturition residual urine was 143 ml ().
Laboratory analysis showed no coagulation abnormalities with a platelet count within normal limits, high erythrocyte sedimentation rate of 94 mm/h, and high white cell count of 23880 cells/mm3 with 90,6% of neutrophils.
Urine analysis revealed hematuria and leukocyturia with no growth in urine culture. His renal function tests were normal.
A subsequent computed tomography urography was performed revealing a prostatic abscess measuring 50 mm in diameter enlarging the prostate with infiltration of the periprostatic fat. The bladder was distended without anomalies within its wall. Bilateral ureterohydronephrosis with normal renal parenchyma was also noted ().
Urethral catheterization was performed and he was addressed to urology department.
Physical exam at the time of presentation to urology department found a patient in good general health apyretic. His vital signs were within normal limits. Digital rectal examination was painful and found a firm and enlarged prostate without nodules. Transrectal ultrasound was not tolerated by the patient. His International Prostate Symptom Score (IPSS) was 26 consistent with severe low urinary tract symptoms (LUST). We did not perform a prostate-specific antigen (PSA) test since patient provided us with a former test done one month earlier to his admission which was at 0,54 ng/ml. Also, since he has a prostatic abscess PSA was expected to be elevated.
The patient underwent a transurethral resection of the prostate (TURP) showing an important enlargement of the left lateral lobe obstructing the prostatic urethra. Resection permitted an unroofing of the abscess and a drainage of abundant amounts of thick whitish pus and allowed collapse of pus pockets under control of view ().
His postoperative recovery was uneventful. Tissue cultures were negative. Histologic examination of resected chips concluded on suppurated xanthogranulomatous prostatitis (). |
pmc-6136544-1 | A 72-year-old male was admitted with anorexia, shaking chills, diaphoresis, hematuria, productive cough, and fever up to 103°F.
Ten months prior to his presentation, the patient had been diagnosed with high-grade papillary noninvasive urothelial carcinoma of the bladder with no invasion of the lamina propria or muscularis propria (American Joint Committee on Cancer stage TaN0M0). After the tumor was resected, he began intravesical BCG therapy. He had his final BCG instillation approximately 17 days prior to his admission. On the day of admission, he had significant gross hematuria, shaking chills, a productive cough, profuse diaphoresis, malaise, and fever up to 103°F.
The patient's past medical history included type 2 diabetes mellitus, hypertension, and coronary artery disease. Medications on admission were losartan, INH insulin, metformin, metoprolol, and rosuvastatin. The patient was originally from India and had lived in the United States for the past thirty years. He had a 15-pack year smoking history prior to quitting 25 years ago. His family history was significant for prostate cancer in his brother.
Upon admission, the patient appeared fatigued and acutely ill. His temperature was 103°F, respiratory rate 24, blood pressure 160/80, pulse 84, and pulse oximetry 97% on 2 L nasal canula. His exam was notable for bilateral crackles at the lung bases; his abdomen was benign, and there was no costovertebral angle tenderness.
Laboratory results included a white blood cell count of 3.6 × 109/L, hemoglobin of 14.4 g/dL and platelet count of 98 × 109/L. Liver function tests showed an alkaline phosphatase of 251 U/L, alanine transaminase of 71 U/L, and aspartate transaminase of 92 U/L. Urinalysis had moderate blood, 3–10 RBC/hpf, 0–5 WBC/hpf, negative leukocyte esterase, and negative nitrites. PA and lateral chest X-ray showed increased interstitial markings bilaterally, especially at the bases. Right upper quadrant ultrasound showed mild fatty infiltration of the liver.
The patient was placed on ceftriaxone for presumed community-acquired pneumonia but failed to improve. Lack of improvement on antibiotics coupled with the multiple systemic abnormalities (including (1) chest X-ray findings suggesting pneumonitis, (2) a rise in transaminases pointing towards hepatitis, and (3) thrombocytopenia indicating bone marrow involvement) lead to the possible diagnoses of disseminated mycobacteria. However, it was not clear whether this was truly disseminated mycobacteria or a hypersensitivity reaction from BCG. Therefore, the patient was initially started on methylPREDNISolone, INH, rifampin, and ethambutol.
The patient began to improve clinically, but after six days of therapy, his transaminases were markedly more elevated with worsening thrombocytopenia. At this point, concurrent medication toxicity was suspected. His antimycobacterial medications were held, and both a liver biopsy and a bone marrow biopsy were obtained in the following two days. His transaminases returned began to decrease shortly after antimycobacterial medications were held. Rifampin and ethambutol were restarted 24 hours later.
The liver biopsy showed chronic lobular and portal inflammation consisting predominantly of lymphocytes and histiocytes, focal interface hepatitis, and small noncaseating granulomas (see ). The bone marrow biopsy showed scattered noncaseating granulomas. Acid-fast stain for mycobacteria was negative on both biopsies. All blood cultures, urine cultures, and sputum cultures were ultimately negative.
The patient was discharged on rifampin, ethambutol, and prednisone eight days after the biopsy results came back. In the following week, he developed a recurrent rise in his transaminases, leading to cessation of antimycobacterial therapy due to possible rifampin hepatotoxicity. He remained stable with normal liver enzymes after completing six weeks of steroid treatment.
Based on the aforementioned findings, the diagnosis of systemic granulomatous disease from BCG therapy was established, with hypersensitivity being the most likely etiology. |
pmc-6136560-1 | A 57-year-old female without past medical history presented to the Emergency Department (ED) at the end of May with altered mental status, nausea, and vomiting. She had felt unwell for the past week with symptoms of mild cough and intermittent fevers peaking at 39.4° Celsius (C). She had been seen by her primary care physician two days prior and was diagnosed with a urinary tract infection based on a positive urine culture for Enterococcus species. She had not started the antibiotics prior to presentation to the ED. In the ED, she appeared acutely ill. She was hypotensive (blood pressure 58/41 mmHg by cuff), tachycardic (heart rate 120 beats/minute), and hypothermic (32.4°C). Physical exam revealed dry mucus membranes, clear lung fields, and cold and mottled extremities. Initial blood work demonstrated an arterial blood gas with pH of 7.0, pCO2 32mmHg, pO2 450 mmHg on supplemental oxygen, and arterial lactate 9.6 mmol/L. Chemistries and hepatic function testing showed creatinine of 1.64 mg/dL, glucose 330 mg/dL, alanine transferase 23 U/L, and total bilirubin <0.2mg/dL. Complete blood count was notable for leukocytosis 16,300/uL with 77.4% neutrophils and 16.9% lymphocytes and hemoglobin of 18.6 g/dL. CRP was normal at 2mg/L. Procalcitonin was 0.89 ng/mL. Troponin T was elevated to 0.20 ng/mL which subsequently rose to 0.97 ng/mL on repeat. Urine toxicology screen was negative. Initial chest X-ray (CXR) showed no acute cardiopulmonary disease (). Initial ECG demonstrated sinus tachycardia. Three liters of isotonic intravenous fluids were given as bolus infusion, which resulted in transient increases in blood pressure, but systolic blood pressure remained low (<70 mm Hg) despite fluid resuscitation. A left subclavian triple lumen catheter was inserted, norepinephrine was initiated to maintain mean arterial pressure >60 mmHg, and the patient received cefepime and vancomycin for presumed septic shock.
A bedside cardiac ultrasound was performed which demonstrated a large pericardial effusion with tamponade physiology (). The patient was taken to the cardiac catheterization lab for an urgent pericardiocentesis. Prior to the procedure, the patient suffered an asystolic cardiac arrest secondary to pump failure requiring 10 minutes of cardiopulmonary resuscitation. She was intubated and started on mechanical ventilation. The patient underwent pericardiocentes with immediate evacuation of 90ml of serous fluid, and a pericardial drain was subsequently placed to manage any ongoing or residual effusion. After successful pericardiocentesis, the patient also underwent coronary angiography, which revealed angiographically normal coronaries, with no evidence of plaque or obstruction.
The patient was admitted to the Medical Intensive Care Unit. Over the following 24 hours her condition deteriorated with hypotension and a marked metabolic acidosis despite IV fluids, high dose vasopressors, broad-spectrum antibiotics, stress dose steroids, and a bicarbonate drip. Arterial lactate continued to trend up to 16.2mmol/L. Repeat transthoracic echocardiogram 12 hours after the pericardiocentesis revealed only a small anterior pericardial effusion, with normal left and right ventricular ejection fractions. Total pericardial drain output was 150mL over this time. During this time, her respiratory viral panel taken by nasal swab on admission returned positive for influenza B by PCR. Repeat CXR showed bilateral infiltrates concerning for acute respiratory distress syndrome. Oxygenation deteriorated despite high Fi02 and PEEP, and the patient was paralyzed and started on low tidal volume ventilation. Despite maximal supportive care, later that day the patient experienced another asystolic cardiac arrest from persistent hypoxia, respiratory failure, and worsening acidosis for which resuscitative efforts were unsuccessful. All blood cultures remained negative. Urine culture from presentation returned positive for 50,000 colonies of Enterococcus species; however, the urinalysis was not compatible with active infection, showing only 12 white blood cells. Repeat urine culture collected later during the admission remained negative.
Autopsy was performed after consent was obtained from family members. On gross evaluation, the myocardium was grossly firm, dense, and mottled in appearance. Lung histology revealed relatively preserved lung architecture with only focal evidence of lung injury in the left lower lobe, but no widespread evidence of pneumonitis or pneumonia. All cultures from the lung were negative for infection. In contrast the heart was markedly abnormal, with multifocal cardiac cell necrosis and subendocardial septal hemorrhage consistent with myocarditis. The pericardium was normal. Immunohistochemistry revealed extensive infiltration of the myocardium with CD3 positive lymphocytes, and hemoxylin and eosin stain demonstrated hemorrhage and myocyte necrosis (). The cause of death was felt to be myocarditis secondary to influenza B infection, given the strongly positive viral PCR. |
pmc-6136583-1 | A 71-year-old man with progressive angina was found to have severe stenosis in the circumflex artery and complex CTO of the left anterior descending artery (, Supplementary ). Coronary artery bypass surgery was offered to the patient but declined. He underwent uncomplicated stenting of the circumflex artery, followed by staged CTO recanalization of the LAD. A guidewire was passed relatively easily across the occlusion into the true lumen of the distal LAD beyond the bifurcation. Antegrade wiring of the diagonal artery was difficult. An epicardial collateral from the distal right coronary artery was used to access the diagonal branch retrograde, and this wire was steered into the antegrade guide catheter and externalized (). Both the LAD and diagonal were dilated with 3.0 mm noncompliant balloons. The diagonal was stented into the proximal LAD, and the distal LAD was rewired. The distal LAD was dilated through the stent struts to allow passage of stents into the distal LAD (Culotte technique). The distal LAD was stented using four everolimus drug-eluting stents. The stents were postdilated with 3.0 mm noncompliant balloons in the diagonal and distal LAD and 4.0 mm noncompliant balloon in the proximal LAD. Within the distal-most stent in the distal LAD, the balloon had a persistent waist until an inflation pressure of 12 ATM. At that point, the balloon suddenly expanded. Angiography revealed contrast filling of the right ventricle (RV), with the appearance of one or two focal jets of contrast extravasation at the location of the rigid lesion in the distal LAD, consistent with iatrogenic LAD-RV fistula (, Supplementary ). Of note, the flow beyond the stents in the distal LAD was not seen, likely due to shunt flow and “coronary steal.” The patient remained hemodynamically stable and was asymptomatic, and it was elected to manage the fistula conservatively. Serial echocardiograms revealed only trace pericardial effusion. He was discharged in stable condition.
Approximately three weeks after the CTO procedure, the patient was presented to the emergency department with chest pain and dyspnea. He described several episodes of “tearing” sensation in the chest. Blood pressure and heart rate were 123/69 mmHg and 64 beats per minute, respectively. A 12-lead ECG revealed inferior T wave inversion. Troponin I was elevated to 0.06 ng/mL. Urgent coronary angiography was performed, which revealed patent stents in the proximal LAD and diagonal branch. LAD-RV fistula appeared relatively unchanged compared to during the CTO procedure (). The right coronary artery was normal and provided a very faint collateral to the apical LAD. Left ventriculography in the left anterior oblique projection revealed no evidence of a ventricular septal defect. The patient developed profound hypotension of unclear etiology during angiography. A right heart catheterization revealed normal filling pressures, normal cardiac output and ratio of pulmonic to systemic flow (Qp:Qs) 1.7. A transthoracic echocardiogram revealed preserved ejection fraction and normal left ventricular wall motion with turbulent flow signals at the LV and RV apex throughout the cardiac cycle (Supplementary ). There was no pericardial effusion. The patient was transferred to the cardiac intensive care unit in stable condition.
Heart team evaluation was undertaken, and it was decided to perform coil embolization of the distal LAD. A standard 6 French left coronary guide catheter was used to advance a ProGreat (Terumo Medical Corp., Somerset, New Jersey) guidewire and 2.8 French catheter into the distal LAD. This was used to deploy two Ruby (Penumbra, Inc., Alameda, California) coils at the distal end of the stent.
There was still persistent shunt flow, so two Tornado (Cook Medical, Bloomington, Indiana) coils were subsequently deployed. Angiogram of the LAD confirmed complete cessation of flow into the distal LAD and absence of shunt flow (, Supplementary ). Hemodynamics postprocedure demonstrated a 20 mmHg increase in systolic blood pressure and normalization of Qp:Qs. A follow-up echocardiogram revealed the obliteration of apical shunt flow, normal left ventricular ejection fraction, and no left ventricular wall motion abnormalities. Creatine kinase eight-hour postprocedure was normal. The patient was discharged home in stable condition. At follow-up, one month later, the patient remained asymptomatic. |
pmc-6136621-1 | We describe the case of a 20-year-old Filipino male who presented to our Australian metropolitan hospital with a history of fever and diarrhoea. He had no significant past medical history and was not on any regular medications. He was born in the Philippines and migrated to Australia in 2008, where he was working as a chef.
He developed symptoms of fevers up to 40 °C, mild headache and watery diarrhea, two weeks after returning from a three-week trip to the Philippines. During the trip he had no sick contacts and no significant exposures to animals or fresh water. He did not receive vaccination for typhoid prior to travel. He was prescribed amoxicillin along with paracetamol and metoclopramide by his local doctor without symptomatic improvement. Hence, he presented to the emergency department seven days after symptom onset.
On presentation, he was febrile at 39.5 °C, with heart rate 105/min, and was hypotensive (80/60 mmHg); however, his blood pressure improved with intravenous fluid resuscitation. His abdomen was soft with mild tenderness in the right upper quadrant. Respiratory and cardiovascular examinations were unremarkable and there were no rashes. Initial investigations revealed haemoglobin (Hb) 141 g/L, white cell count (WCC) 3.9 × 109/L and platelets 101 × 109/L; creatinine 90 μmol/L, elevated C-reactive protein of 218 mg/L, and deranged liver function tests (alanine aminotransferase [ALT] 421 U/L, aspartate aminotransferase [AST] 743 U/L, gamma-glutamyl transferase [GGT] 171 U/L, alkaline phosphatase [ALP] 175 U/L, bilirubin 14 μmol/L). Chest X-ray and urinalysis were unremarkable; serological tests revealed negative Epstein–Barr virus IgM, positive cytomegalovirus IgG but IgM negative, and negative hepatitis B surface antigen, hepatitis C antibody and hepatitis A IgM. An ultrasound of the abdomen identified mild hepatosplenomegaly with no focal lesions. He was commenced on empiric intravenous ceftriaxone 2 g/day.
Blood cultures taken on presentation grew Salmonella enterica serovar Typhi. Susceptibility testing, performed using the Phoenix automated broth microdilution system (BD Diagnostic Systems, Sparks, MD, USA), indicated susceptibility to ampicillin (minimum inhibitory concentration [MIC] <4 µg/mL), ceftriaxone (MIC <0.5 µg/mL) and cotrimoxazole (MIC <1/19 µg/mL), using Clinical and Laboratory Standards Institute (CLSI) breakpoints. Ciprofloxacin susceptibility was confirmed using nalidixic acid disc diffusion testing according to CLSI Guidelines (zone size 25 mm, using a 30 μg nalidixic acid disc). Azithromycin susceptibility testing, performed using E-test (bioMérieux, Baulkham Hills, Australia), indicated susceptibility with an MIC of <4 µg/mL. Two further blood cultures taken on days one and two of admission were also positive for S. Typhi but were subsequently negative after 24 h of therapy. A stool sample showed no leucocytes or erythrocytes and was positive for Salmonella species by polymerase chain reaction (PCR), despite not growing on culture.
Intermittent fevers were present for five days after commencing therapy; however, his overall condition improved substantially, with resolution of diarrhoea and a reduction in inflammatory markers and liver function tests. He received eight days of ceftriaxone before being discharged with a further seven-day course of oral ciprofloxacin 500 mg twice daily. He had complete resolution of his symptoms at the conclusion of this course.
Six weeks after discharge, the patient returned to the emergency department with a five-day history of fevers, lethargy and mild headache. He had no abdominal pain or diarrhoea and no respiratory or urinary symptoms. Examination of his cardiovascular system demonstrated dual heart sounds with no murmurs; respiratory and gastrointestinal system examinations were also unremarkable. There were no peripheral stigmata of infective endocarditis. Initial investigations revealed mild pancytopenia (Hb 123 g/L, WCC 2.3 × 109/L: neutrophils 1.6 × 109/L lymphocytes 0.7 × 109/L, platelets: 127 × 109/L) and creatinine 92 µmol/L, along with elevated C-reactive protein of 110 mg/L and mildly deranged liver function tests (ALT 117 U/L, AST 194 U/L, GGT 30 U/L, ALP 67 U/L, bilirubin 10 µmol/L). A single set of blood cultures taken grew S. Typhi, and the pathogen was also demonstrated on faecal culture and PCR. He was commenced on intravenous ceftriaxone 2 g daily.
Multiple investigations were undertaken to identify a possible occult infective focus. Computed tomography scans of brain, chest, abdomen and pelvis were normal, as was a nuclear medicine bone scan. An ultrasound of the abdomen was unremarkable, with a normal appearance of the gallbladder with no cholelithiasis. A transthoracic echocardiogram identified a small, mobile vegetation on the anterior leaflet of the mitral valve. The remaining valves were structurally normal; there was no evidence of abscess formation and no features to suggest myocarditis. The 4 mm mitral valve vegetation was again demonstrated on transoesophageal echocardiography and a diagnosis of S. Typhi endocarditis was thus reached.
He was treated with a six-week course of intravenous ceftriaxone 2 g/day, followed by six weeks of oral ciprofloxacin 500 mg twice daily. His blood cultures cleared rapidly within 24 h of treatment commencement, and his symptoms resolved within the first week. Monitoring of inflammatory markers showed normalisation of the C-reactive protein and full blood count parameters. A repeat transoesophageal echocardiogram at the end of treatment demonstrated a 2 mm calcified mass on the anterior leaflet of the mitral valve, consistent with a healed vegetation. Three sets of follow-up blood cultures taken one month following treatment completion remained culture-negative. He did not have further stool examination after completion of therapy. At three months of follow-up, he remained systemically well. |
pmc-6136672-1 | A 70-year-old woman presented in our vascular clinic due to an asymptomatic juxtarenal 10 cm AAA identified as an incidental finding in a recent computed tomography (CT) scan (
). Her medical history included mild hypertension under drug medication. At physical examination of the abdomen, a large pulsating mass was present with normal pulsation of femoral and tibial vessels. There were no complaints of previous intermittent claudication.
The woman was planned for open repair. She was very anxious about the result and the possible complications of the surgical procedure. The woman had intense stress, phobia for surgery, and was consulted by a psychiatrist.
To better define the aneurysm anatomy (with 1mm imaging slices), we performed a new CT angiography (CTA) 48 hours after admission, which surprisingly revealed complete thrombosis of the AAA just below both renal arteries without any signs of acute renal insufficiency, mesenteric ischemia, or limb ischemia (
). The most impressive element of the CTA was the rich collateralization between the thoracic aorta and the common femoral arteries through the superficial epigastric and other arteries of thoracic and abdominal wall. This collateralization was not evident in the first CT 2 days earlier.
The following physical examination revealed the absence of the previous pulsating mass and absence of femoral and distal leg pulses. Both legs were warm with normal skin color. The surgical procedure was postponed and the woman was discharged from the hospital with double antiplatelet therapy and weekly follow-up for the possible signs of limb ischemia.
After a month, the patient presented with severe intermittent claudication in the left lower limb. The following digital subtraction angiogram revealed a thrombosed abdominal aorta with collateral vessels between the aorta and both common femoral arteries (
). The woman underwent a left axillary–femoral bypass with polytetrafluoroethylene No. 8 graft. The patient's postoperative course was uneventful and the symptom of intermittent claudication disappeared. She was discharged on fourth postoperative day with antiplatelet (salicylic acid 100 mg, once daily) and statin (atorvastatin 20 mg, once daily) medication. |
pmc-6136673-1 | A 75-year-old man presented at our ED 2 hours after sudden onset of severe central chest pain that followed a straining effort; the pain was described as sharp, radiated between the scapulae, and was associated with palpitations and dyspnea. The only relevant past medical history was a spontaneous superficial thrombophlebitis a month prior; this was managed in the community without anticoagulation. The physical examination revealed an aortic regurgitation murmur and chest X-ray showed a moderately enlarged mediastinum. An urgent computed tomography (CT) pulmonary angiogram was organized (
); this showed (1) a large saddle pulmonary embolus extending into both left and right pulmonary arteries, (2) a dilated 7 cm ascending aorta with an apparent flap, and (3) a hemopericardium (Hounsfield Unit 30). Given the highly suspicious appearance of the ascending aorta, a CT angiogram was then performed confirming acute type A dissection. The dissection flap involved the ascending aorta and the aortic arch, sparing the supra-aortic vessels; a thrombosed false lumen extended into the descending thoracic and abdominal aorta just above the renal arteries (
). An urgent transthoracic echocardiogram confirmed the diagnosis and documented severe aortic regurgitation. At this stage, the patient was hemodynamically stable; decision was made to proceed immediately with aortic dissection repair and pulmonary embolectomy.
Femorofemoral bypass was established and at pericardiotomy a large hemopericardium was evacuated. The aorta was transected under deep hypothermic circulatory arrest (18°C) with retrograde cerebral perfusion. The entry tear was found in the anterior wall of the ascending aorta with fresh clot within the false lumen. The aorta appeared to be ruptured posteriorly with just a thin layer of clot tamponading against the main pulmonary artery bifurcation, preventing free intrapericardial rupture.
The ascending aorta was replaced with a 38 mm Dacron graft. After performing the distal anastomosis just proximal to the innominate artery, cardiopulmonary bypass was re-established and the main pulmonary artery was opened longitudinally. Embolectomy of a large saddle-shaped pulmonary thrombus (6 cm long) was then performed using Rampleys forceps. No attachment to the pulmonary artery intima was noted, confirming the acuteness of the embolic event.
Finally, the aortic root was replaced with a 29 mm Medtronic Freestyle stentless bioprosthesis (Medtronic). The patient was weaned off bypass uneventfully and transferred to the intensive care unit in stable condition.
The postoperative course was unremarkable. No deep vein thrombosis or thrombophilic traits were identified. Pathology of the pulmonary embolus showed characteristic lines of Zahn, indicating a recent event. The patient was discharged home on lifelong warfarin on postoperative day 10. |
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