<prompt> A 28 years old man has anterior lenticonus and ESRD now. His maternal uncle also died of similar illness. Diagnosis isARPKDADPKDOxalosisAlpos syndrome  <response>: Ans. d. Alpos syndrome The given clinical picture is typical of Alpos syndrome, which is characterized by triad of hereditary nephritis ESRD, sensorineural deafness and ocular abnormalities anterior lenticonus. Alpos Syndrome Characterized by triad of Hereditary Nephritis Hematuria earliest featureQ 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 Alpos Syndrome Primary treatment is control of systemic hypeension and use of ACE inhibitors to slow renal disease progression 