WBR0283

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Author [[PageAuthor::Ayokunle Olubaniyi, M.B,B.S [1] (Reviewed by Yazan Daaboul, M.D. and Alison Leibowitz [2])]]
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 physician's office because his mother noticed that his eyes are unusually swollen every morning for the past 3 days. The mother explains that the swelling self-resolves during the day. The boy does not report any obvious exacerbating of alleviating factor. The mother recalls that the patient was stung by a bee two weeks prior to presentation. Upon further questioning, the mother also explains that a few weeks back, there was a viral outbreak at the boy's school and several students had respiratory symptoms. The patient has no past medical history with no family history for chronic diseases. His temperature is 36.7 °C (98 °F), his blood pressure 142/94 mm Hg, and his heart rate is 82/min. Physical examination in the clinic is unremarkable. Urinalysis demonstrates oval fat bodies and profound proteinuria. The physician suspects the patient's condition is caused by a renal disease. Which of the following findings on light microscopy is most likely consistent with this patient's condition?]]
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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