<prompt> A 25-yr old man comes with features of chronic renal failure. He also gives a ho an uncle who died of renal failure 10 years ago. Ocular examination by slit-lamp reveals keratoconus. What is the most probable diagnosis?ARPKDADPKDDenys Drash syndromeAlpos syndrome  <response>: Classical features of Alpos syndrome: Hematuria Thinning and splitting of the GBMs Mild proteinuria Chronic glomerulosclerosis leading to renal failure Sensorineural deafness Lenticonus of the anterior lens capsule Dot and fleck retinopathy Also know: Approximately 85 of patients with Alpos syndrome have an X-linked inheritance of mutations in the 5IV collagen chain on chromosome Xq22-24. Early onset of severe deafness, lenticonus, or proteinuria suggests a poorer prognosis. Ref: Lewis J.B., Neilson E.G. 2012. Chapter 283. Glomerular Diseases. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo Eds,Harrisons Principles of Internal Medicine, 18e. 