A Case of Alport's Syndrome in an Adult / 대한신장학회잡지

ABSTRACT

Alport's syndrome is a hereditary disorder whose incidence is 1 in 10,000 in general population, and, majority are developed in childhood, so it is rare to develop in adults. This syndrome consists of hematuria, sensorineural hearing loss, and lenticonus, in classic form. However, there are many atypical forms which have not all clinical manifestations. Although most asymptomatic hematuria in adults results from IgA nephropathy(Berger's disease) or thin basement disease(benign hematuria), Alport's syndrome should be considered as a cause of hematuria. The authors experienced a case of Alport's syndrome in a 21-year-old man who showed only hematuria and proteinuria, and who was diagnosed by history, family history, audiogram, and kidney needle biopsy. So, we report this case with a review of literature.