Four Cases of Autosomal Recessive Polycystic Kidney Disease

Choi, Woong-Kyu; Lee, Seung-Cheol; Park, Yong-Won; Lee, Chong-Guk

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

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