A mother brought her 14-year-old boy to the emergency with the complaint of cough with blood in sputum and red coloured urine. On examination his BP was 140/90 mmHg. Investigations showed his hemoglobin is 10 mg/dl and hematocrit is 31%.His urinalysis is suggestive of hematuria and proteinuria. Which of the following is the probable diagnosis?
Correct Answer: Goodpasture syndrome
Description: Goodpasture syndrome: Autoimmune disorder characterized by pulmonary alveolar haemorrhages and glomerulonephritis. Pathogenesis: The anti-glomerular basement membrane (GBM) antibody binds to the basement membrane of both the alveolus and the glomerulus. GBM antibodies attach to type IV collagen contained in the vascular endothelium. C/F: - Related to lung disease: cough, dyspnoea, pallor, ronchi, edema and hepatomegaly Related to kidney disease: hematuria, proteinuria and hypeension Diagnosis: By kidney biopsy and finding antibodies to the glomerular basement membrane Other options in question: Hemolytic-uremic syndrome presents with fever, bloody diarrhoea, and progression toward renal failure, but respiratory symptoms are not present. Nephrotic syndrome presents with edema,ascites and proteinuria and respiratory symptoms related to congestive hea failure can be seen. Poststreptococcal glomerulonephritis result in hematuria, but pulmonary hemorrhage is not seen.
Category:
Pediatrics
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