All are TRUE about alpo syndrome, EXCEPT:

Correct Answer: Renal failure occurs in 50% of affected males
Description: Alpo syndrome in its classic form is a hereditary nephropathy associated with 1. Sensorineural hearing loss and 2. Ocular manifestations. The characteristic renal features is persistent microscopic hematuria appearing in early childhood. Proteinuria is often absent in the early stages of the disease, but increases progressively with age and a nephrotic syndrome may develop. Renal failure occurs in all affected males, and in most cases during the third or fouh decade. Juvenile AS is defined as mean age at onset of ESRD <=30 years, and adult AS as mean age at onset of ESRD >30 years. Ref: Dan Med Bull 2009; 56:105-52.
Category: Medicine
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