Recurrent IgAN Post-Transplant — ESENeph MCQ

Correct answer: A — UMOD-related autosomal dominant tubulointerstitial kidney disease

The best answer is “UMOD-related autosomal dominant tubulointerstitial kidney disease”. ADTKD-UMOD is a dominant, kidney-limited disorder with slowly progressive tubulointerstitial CKD, bland urinary sediment, early hyperuricaemia and frequent gout. Normal or small kidneys without a diagnostic cyst pattern help distinguish it from ADPKD. “Autosomal dominant polycystic kidney disease” is less appropriate because the absence of characteristic cystic enlargement and the bland urine with early gout favour ADTKD-UMOD “Alport syndrome” is less appropriate because Alport syndrome usually features persistent haematuria and may include hearing or ocular disease “Primary membranous nephropathy” is less appropriate because membranous nephropathy produces substantial proteinuria rather than a bland inherited tubulointerstitial pattern “Autosomal recessive polycystic kidney disease” is less appropriate because the multigenerational dominant inheritance and adult presentation do not fit this diagnosis

Reference: GeneReviews: ADTKD-UMOD: https://www.ncbi.nlm.nih.gov/books/NBK1356/