A Case of Alport's Syndrome. |
Jung Bae Lee1, Jong Kyun Lee1, Pyung Kil Kim1, Hyeon Joo Jeong1, In Jun Choi1 |
1Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea 2Department of Pathology, Yonsei University, College of Medicine, Seoul, Korea |
Alport 증후군 1례 |
이정배1, 이종균1, 김병길1, 정 현 주1, 최 인 준1 |
1연세대학교 의과대학 소아과학교실 2연세대학교 의과대학 병리학 교실 |
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Abstract |
We have experienced a case of Alporfs syndrome. The patient was suspicious to make the dignosis
of Alporfs syndrome at first examination when he was five years and seven months old. At that time
diagnostic work-up were included audiogram and kidney biopsy.
His family history was more impressive. His elder brother has died with undetermined cause of
nephrotic syndrome one month ago and his maternal uncle also deceased several years ago with an
uncertain glomerulonephritis.
He was seen at pediatric department for six years and three months since first admission for trial
treatment of nephrotic syndrome with steroid and several cytotoxic drugs without any improvement.
Repeated examinations including three audiograms and two more subsequent kidney biopsies were
possible the final diagnosis of Alport syndrome at age 12 years.
At present his kidney function is normal except asymptomatic microhematuria and proteinuria.
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Key Words:
Alporfs syndrome
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