Eisenstein B, Davidovitz M, Garty B Z, Shmueli D, Ussim A, Stark H
Paediatric Nephrology Unit, Beilinson Medical Centre, Petah Tiqva, Sackler School of Medicine Tel Aviv University, Israel.
Pediatr Nephrol. 1992 Jan;6(1):57-9. doi: 10.1007/BF00856835.
A 9.5-year-old girl, whose early symptoms were polyuria and growth retardation, is described. During the progression of her disease, hyperkalaemia developed out of proportion to the degree of renal insufficiency. Her fractional excretion of sodium increased from 3.3% to 35%, and her fractional excretion of potassium decreased from 55% to 22%. The plasma aldosterone level and plasma renin activity (PRA) were very high--290 ng/ml and 100 ng/dl per hour, respectively (normal range for this age 2.6-20.8 ng/ml and 1.2-2.7 ng/ml per hour, respectively). In an attempt to reduce these hormone levels, an acute and sustained saline load, captopril and peritoneal dialysis were used. Only the sustained saline load normalized the PRA, and only peritoneal dialysis sufficiently suppressed the plasma aldosterone level. Successful renal transplantation normalized both plasma aldosterone and PRA. This girl presents the unusual occurrence of pseudohypoaldosteronism type I, during the course of familial juvenile nephronophthisis.
本文描述了一名9.5岁女孩，其早期症状为多尿和生长发育迟缓。在疾病进展过程中，高钾血症的发生与肾功能不全程度不成比例。她的钠排泄分数从3.3%增加到35%，钾排泄分数从55%下降到22%。血浆醛固酮水平和血浆肾素活性（PRA）非常高，分别为290 ng/ml和每小时100 ng/dl（该年龄正常范围分别为每小时2.6 - 20.8 ng/ml和1.2 - 2.7 ng/ml）。为降低这些激素水平，采用了急性和持续性生理盐水负荷、卡托普利及腹膜透析。只有持续性生理盐水负荷使PRA恢复正常，只有腹膜透析充分抑制了血浆醛固酮水平。成功的肾移植使血浆醛固酮和PRA均恢复正常。该女孩在家族性青少年肾单位肾痨病程中出现了罕见的Ⅰ型假性醛固酮增多症。
