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Journal of the Korean Society of Pediatric Nephrology 1997;1(2): 183-188.
Familial Juvenile Hyperuricemic Nephropathy 2례
박진호, 최보화, 이소영, 유은실, 박영서
1울산의대 서울중앙병원 소아과
2울산의대 서울중앙병원 소아과
3울산의대 서울중앙병원 소아과
4울산의대 서울중앙병원 진단병리과
5울산의대 서울중앙병원 소아과
Two cases of Familial Juvenile Hyperuricemic Nephropathy
Jin-Ho Park, Bo-Hwa Choi, So-Young Lee, Eun-Sil Yoo, Young-Seo Park
1Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
2Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
3Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
4Department of Diagnostic Pathology, Asan Medical Center, University of Ulsan, College of Medicine
5Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
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ABSTRACT
Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.
Key words: Familial juvenile hyperuricemic nephropathy
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