All issues > Volume 30(9); 1987

Case Report

J Korean Pediatr Soc. 1987;30(9):1040-1048. Published online September 30, 1987.

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

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.

Keywords :Alporfs syndrome