A 6-week-old infant presents with generalized edema, massive proteinuria (urine protein:creatinine ratio 30), and hypoalbuminemia (1.5 g/dL). There is no hematuria. Complement levels are normal. The condition is steroid-resistant. What is the most likely diagnosis?
- A Congenital nephrotic syndrome (Finnish type) ✓
- B Minimal change disease
- C Focal segmental glomerulosclerosis
- D Membranoproliferative glomerulonephritis
Correct answer: A. Congenital nephrotic syndrome (Finnish type)
Explanation
Nephrotic syndrome presenting before 3 months of age is congenital nephrotic syndrome. The Finnish type (congenital nephrotic syndrome of the Finnish type, NPHS1 mutation) is the most common, presenting with massive proteinuria, edema, and hypoalbuminemia. It is steroid-resistant and carries a poor prognosis without nephrectomy and transplantation. Minimal change disease presents after 1 year and is steroid-sensitive. FSGS and MPGN are causes of steroid-resistant nephrotic syndrome in older children.
Reference: Nelson Textbook of Pediatrics, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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