Popow C, Pollak A, Herkner K, Schober E, Waldhäusl W
Padiatr Padol. 1982;17(2):503-12.
We report about a girl, 22 months old, with pseudohypoaldosteronism. The clinical course was characterized mainly by renal salt loosing syndrome, complicated by suddenly occurring hyperkalemic phases and a complete inability of renal electrolyte and acid-base regulation. Congenital adrenal hyperplasia was excluded and the diagnosis based on renal salt wasting despite hyperaldosteronaemia and increased plasma renin activity. Diagnosis and therapy of pseudo-hypoaldosteronism are discussed as well as the problems of long-term oral sodium chloride substitution and long-term therapy with sodium-polystyrol-sulfonate, a cation-exchange resin.
我们报告了一名22个月大患有假性醛固酮减少症的女童。临床病程主要表现为肾性失盐综合征,并发突然出现的高钾血症阶段以及肾脏对电解质和酸碱调节的完全无能。排除了先天性肾上腺增生,诊断依据是尽管醛固酮增多症和血浆肾素活性增加,但仍存在肾性失盐。讨论了假性醛固酮减少症的诊断和治疗,以及长期口服氯化钠替代治疗和使用阳离子交换树脂聚苯乙烯磺酸钠进行长期治疗的问题。