신우요관이행부폐색과 동반된 일과성 가성저알도스테론혈증 1례

A Case of Transient Pseudohypoaldosteronism Secondary to Ureteropelvic Junction Obstruction

  • 최준기 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 한혜원 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 유한욱 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 박영서 (울산대학교 의과대학 서울아산병원 소아과)
  • Choi Jun-Gi (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Hahn Hye-Won (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center) ;
  • You Han-Wook (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Park Young-Seo (Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center)
  • 발행 : 2004.04.01

초록

저자들은 신우요관이행부폐색에 의한 심한 수신증이 있는 한 환아에서 2M NaCl 경구투여만으로 증상이 호전되고 폐색성 요로 병증의 교정 이후 재발되지 않는 일과성 가성저알도스테론혈증 1례를 경험하였기에 보고하는 바이다.

We report a 2-month-old boy who presented with severe hyponatremia and hyperkalemia secondary to ureteropelvic junction(VPJ) obstruction. By prenatal ultrasonography at 19 weeks of gestation, severe hydronephrosis was found which was confirmed postnatally Pyeloplasty was done on the 45th day of life, and fifteen days after pyeloplasty, non-bilious vomiting, decreased activity and dehydration developed. Severe hyponatremia and hyperkalemia were observed, as a result of elevated serum aldosterone and plasma renin activity. The anterior posterior pelvic diameter(APPD) and Society for Fetal Urology(SFU) grade measured showed no interval change before and after pyeloplasty. Pseudohypoaldosteronism was diagnosed, and 2M NaCl was administrated orally for 7 days. The electrolyte imbalance was corrected, and 8 weeks later, the elevated levels of aldosterone and plasma renin activity were normalized. The left hydronephrosis was improved at 5 months of age. We hereby report a transient pseudohypoaldosteronism secondary to UPJ obstruction with a review of the literature.

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