Korean J Nephrol.
1999 Mar;18(2):329-333.
A Case of Alport's Syndrome in an Adult
- Affiliations
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- 1Department of Internal Medicine, National Police Hospital, Seoul, Korea.
- 2Department of Otolaryngology, National Police Hospital, Seoul, Korea.
- 3Department of Pathology, Gachon Medical College, Gil Medical Center, Inchon, Korea.
Abstract
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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.