WBR0283

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Author [[PageAuthor::Ayokunle Olubaniyi, M.B,B.S [1] (Edited by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Renal
Prompt [[Prompt::A 5-year-old boy is brought to the emergency room by his mother because she notices that his eyes have appeared swollen when he wakes up in the morning for the past 4 days. The eye swelling tends to resolve over the course of the day. The mother tells you that he was stung by a bee two weeks ago, and that there was also an outbreak of influenza in his school some few weeks back. He is generally very healthy and there is no family history of any chronic disease. Upon examination, the temperature is 36.7°C, the blood pressure 140/90 mm Hg, the pulse 92 beats per minute, and the respiratory rate 17 breaths per minute. Physical examination is normal. Urinalysis shows oval fat bodies and profound proteinuria. Which of the following microscopy findings is required in order to establish a diagnosis?]]
Answer A AnswerA::Segmental sclerosis and hyalinosis
Answer A Explanation [[AnswerAExp::These are characteristics dislayed in focal segmental glomerulosclerosis. This is the most common glomerular disease amoung patients with HIV orsickle cell disease. It is also the most common cause of nephrotic syndrome in adults.]]
Answer B AnswerB::Diffuse capillary and glomerular basement membrane thickening
Answer B Explanation [[AnswerBExp::This is a finding observed in membranous glomerulonephritis (diffuse membranous glomerulonephropathy). This is a slowly progressive kidney disease prevalent amoung Caucasians between 30 and 50 years of age.]]
Answer C AnswerC::Normal glomeruli
Answer C Explanation [[AnswerCExp::The glomeruli in minimal change disease are normal or near normal when examined using a light microscope. Electron microscopy demonstrates flattening and fusion of the podocyte foot processes in the glomeruli.]]
Answer D AnswerD::Enlarged, hypercellular glomeruli with proliferation of mesangial and endothelial cells
Answer D Explanation [[AnswerDExp::Enlarged, hypercellular glomeruli with proliferation of mesangial and endothelial cells using a light microscope are indicative of acute post-streptococcal glomerulonephritis. Electron microscopy demonstrates sub-epithelial immune complexes and a "lumpy-bumpy" appearance. It may present with dark urine, hypertension, and periorbital edema with or without oliguria. Acute glomerulonephritis is characterized by the onset of hematuria, proteinuria, and red blood cell casts in the urine.]]
Answer E AnswerE::“Wire looping” of capillaries
Answer E Explanation [[AnswerEExp::“Wire looping” of capillaries is a feature indicative of diffuse proliferative glomerulonephrits, frequently observed amoung patients with systemic lupus erythematosus (SLE).]]
Right Answer RightAnswer::C
Explanation [[Explanation::The boy in this scenario has nephrotic syndrome, characterized by proteinuria, hypoalbuminemia, and edema. Minimal change disease, also referred to as lipoid nephrosis, is the most common cause of nephrotic syndrome in very young children. It usually starts with facial edema and can be triggered by a bee sting, previous upper respiratory tract infections, drugs, or malignancies. Treatment is very effective with corticosteroids.

Educational Objective: Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia and edema. Minimal change disease is the commonest cause of NS in children, and it can be effectively treated with steroids.
References: First Aid 2014 page 536]]

Approved Approved::Yes
Keyword WBRKeyword::Nephrotic syndrome, WBRKeyword::Minimal change disease, WBRKeyword::Proteinuria, WBRKeyword::Renal, WBRKeyword::Kidney, WBRKeyword::Lipoid nephrosis
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