신우요관이행부폐색과 동반된 일과성 가성저알도스테론혈증 1례 = A Case of Transient Pseudohypoaldosteronism Secondary to Ureteropelvic Junction Obstruction

국문 초록 (Abstract)

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.

다국어 초록 (Multilingual Abstract)

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.

참고문헌 (Reference)

1 Schoen EJ, "Transient pseudohypoaldosteronism with hyponatremia-hyperkalemia in infant urinary tract infection" 167 : 680-682, 2002

2 Gabriele B, "Transient pseudohypoaldosteronism secondary to posterior urethral valves - A case report and review of the literature" 11 : 277-279, 2001

3 Rodriguez-Soriano J, "Transient pseudohypoaldosteronism secondary to obstructive uropathy in infancy" 103 : 375-80, 1983

4 Kuhnle U, "Transient pseudohypoaldosteronism in obstructive renal disease with transient reduction of lymphocytic aldosterone receptors" 39 : 152-5, 1993

5 Klahr S, "The pathophysiology of obstructive nephropathy:the role of vasoactive compounds in the hemodynamic and structural abnormalities of the obstructed kidney" 23 : 219-23, 1994

6 Levin TL, "Salt-losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux-value of ultrasonography in diagnosis" 21 : 413-5, 1991

7 Maruyama K, "Reversible secondary pseudohypoaldosteronism due to pyelonephritis" 17 : 1069-1070, 2003

8 Watanabe T, "Reversible secondary pseudohypoaldosteronism" 18 : 486-, 2003

9 Warshaw B, "Progression to end-stage renal disease in children with obstructive uropathy" 100 : 183-7, 1982

10 Avner ED, "Pediatric Nephrology" Lippincott Williams & Wilkins 554-560, 2004

11 Husted RF, "Induction of resistance to mineralocorticoid hormone in cultured inner medullary collecting duct cell by TGF-b1" 267 : 767-75, 1994

12 Bonny O, "Disturbances of Na/K Balance:Pseudohypoaldosteronism Revisited" 13 : 2399-2414, 2002

13 Melzi M, "Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations" 14 : 56-59, 1995