A 28 years old man has anterior lenticonus and ESRD now. His maternal uncle also died of similar illness. Diagnosis is

Ans. d. Alpo's syndrome The given clinical picture is typical of Alpo's syndrome, which is characterized by triad of hereditary nephritis (ESRD), sensorineural deafness and ocular abnormalities (anterior lenticonus). Alpo's Syndrome (Characterized by triad of) Hereditary Nephritis Hematuria (earliest feature)Q Mild ProteinuriaQ Chronic glomerulosclerosis Progressive renal insufficiency Nephrotic syndrome (rare) Sensorineural Deafness MC extra-renal abnormalityQ AsymmetricQ Ocular Abnormalities Anterior lenticonusQ Dot and Fleck retinopathy Recurrent corneal erosionsQ and opacitiesQ CataractQ Retinal detachment Nystagmus Leiomyomatosis Treatment of Alpo's Syndrome Primary treatment is control of systemic hypeension and use of ACE inhibitors to slow renal disease progression