An unusual etiology of thrombotic microangiopathy in an adolescent male: Questions
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CLINICAL QUIZ
An unusual etiology of thrombotic microangiopathy in an adolescent male: Questions Charushree Prasad 1 & Deborah M. Levy 2,3
&
Diane Hebert 1,3 & Rose Chami 4,5 & Chia Wei Teoh 1,3
Received: 12 February 2020 / Accepted: 20 February 2020 # IPNA 2020
Clinical presentation A previously healthy 14-year-old adolescent male of South Asian ancestry presented to the emergency room (ER) with significant fatigue and a 3-month history of abdominal and lower back pain. Two months prior to presentation, he was seen for his abdominal symptoms and diagnosed with constipation by his primary care physician. At the time, he had normal investigations: a normal hemoglobin 129 g/L [normal 125–170 g/L], platelet count 186 × 109/L [150–400 × 109/L], urea 4.9 mmol/L [2.6–7.6 mmol/L], creatinine 56 μmol/L [41–74 μmol/L], and normal findings on abdominal ultrasound. In the ER, he reported no history of bleeding, though there was a note of darker colored stools (one stool per day) for a few days prior to admission. He was noted to be pale by his family and had significant fatigue and decreased oral intake, but no change in urine output, no gross hematuria, swelling, or edema. He had no history of fevers, night sweats, or weight loss. There was a history of cough and congestion a few days prior to presentation. Review of systems was otherwise
The answers to these questions can be found at https://doi.org/10.1007/ s00467-020-04515-5. * Chia Wei Teoh [email protected] 1
Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
2
Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada
3
Department of Paediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
4
Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada
5
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
unremarkable, except for some non-specific arthralgias reported in his back, knees, and neck. Family history was unremarkable from a renal and hematological standpoint, and the parents were not consanguineous. Physical examination revealed a pale adolescent who was afebrile, with a normal heart rate (80 beats/min) and with mildly elevated blood pressure of 129/77 mmHg. Full examination was unremarkable except for a systolic ejection murmur grade II/VI and mild tenderness along the lumbar spine. There was no rash or ecchymoses noted. Musculoskeletal examination did not reveal any joint swelling or erythema. He had normal range of movement of all joints. Investigations revealed a normocytic anemia with hemoglobin 42 g/L [normal 110–145 g/L] and MCV 81.2 fL [76.7– 89.2 fL], white blood cell count 4.53 × 109/L [3.84–9.84 × 109/L], and thrombocytopenia with platelets 19 × 109/L [175–332 × 10 9 /L]. His blood smear showed moderate polychromasia, marked anisocytosis, and severe schistocytes. Coombs/Direct antiglobulin testing was negative. Lactate dehydroge
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