J Korean Soc Pediatr Nephrol > Volume 1(1); 1997 > Article
J Korean Soc Pediatr Nephrol 1997;1(1): 91-96.
상염색체 열성 다낭신 4례
최웅규, 이승철, 박용원, 이종국
1인제대학교 서울백병원 소아과학교실
2인제대학교 서울백병원 소아과학교실
3인제대학교 서울백병원 소아과학교실
4인제대학교 서울백병원 소아과학교실
Four Cases of Autosomal Recessive Polycystic Kidney Disease
Woong-Kyu Choi, Seung-Cheol Lee, Yong-Won Park, Chong-Guk Lee
1Department of Pediatrics, Seoul Paik Hospital, Inje University, College of Medicine
2Department of Pediatrics, Seoul Paik Hospital, Inje University, College of Medicine
3Department of Pediatrics, Seoul Paik Hospital, Inje University, College of Medicine
4Department of Pediatrics, Seoul Paik Hospital, Inje University, College of Medicine
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ABSTRACT
Polycystic kidney disease is defined as a heritable disorder with diffuse involvement of both kidneys without dysplasia other than cysts. The major clinical entities of autosomal recessive polycystic kidney disease and autosomal dominant polycystic kidney disease have a considerable overlap in clinical presentations and radiographic features in the pediatric population. We experienced three cases of autosomal recessive polycystic kidney disease of neonate who expired within 24 hours due to respiratory difficulty and the other case was detected by gross hematuria. So we report four cases with brief review of literatures.
Key words: Polycystic kidney disease | Autosomal recessive
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