<prompt> A 28 years old man has anterior lenticonus and ESRD now. His maternal uncle also died of similar illness. Diagnosis is:ARPKDADPKDOxalosisAlports syndrome  <response>: Ans. d. Alports syndrome Ref: Harrison 19e p2513, 18e p2351The given clinical picture is typical of Alports syndrome, which is characterized by triad of hereditary nephritis ESRD, sensorineural deafness and ocular abnormalities anterior lenticonus.Alports Syndrome Characterized by triad ofHereditary NephritisSensorineural DeafnessOcular Abnormalities Hematuria earliest featureQ Mild ProteinuriaQ Chronic glomerulosclerosis Progressive renal insufficiency Nephrotic syndrome rare MC extra-renal abnormalityQ AsymmetricQ Anterior lenticonusQ Dot and Fleck retinopathy Recurrent corneal erosionsQ and opacitiesQ CataractQ Retinal detachment Nystagmus LeiomyomatosisTreatment of Alports SyndromePrimary treatment is control of systemic hypertension and use of ACE inhibitors to slow renal disease progression 