表现为失盐危象的婴幼儿暂时性假性醛固酮减少症1例临床分析  

Clinical analysis of an infant case with transient pseudohypoaldosteronism—salt-losing crisis

作　　者：朱艳[1] 匡新宇[1] 康郁林[1] 朱光华[1] 黄文彦[1] 吴滢[1] ZHU Yan;KUANG Xinyu;KANG Yulin;ZHU Guanghua;HUANG Wenyan;WU Ying(Department of Nephrology and Rheumatology,Shanghai Children's Hospital Affiliated to Shanghai Jiao Tong University,Shanghai 200062,China)

机构地区：[1]上海市儿童医院上海交通大学附属儿童医院肾脏风湿科

出　　处：《临床儿科杂志》2019年第8期601-604,共4页Journal of Clinical Pediatrics

基　　金：上海市卫生和计划生育委员会科研课题(No.201740014)

摘　　要：目的探讨暂时性假性醛固酮减少症的诊断和治疗。方法回顾分析1例暂时性假性醛固酮减少症患儿的临床资料,并复习相关文献。结果女性患儿,3月龄初次出现电解质紊乱(低钠血症、高钾血症)和代谢性酸中毒,考虑为先天性肾上腺皮质增生症可能,予以激素治疗后未再随访。6月龄时再次出现电解质紊乱、代谢性酸中毒。实验室检查均提示17-羟孕酮无异常,血醛固酮、肾素水平升高。多项影像学检查提示泌尿道畸形(左侧重复肾畸形、左侧输尿管囊肿、左侧膀胱输尿管反流(III-IV级)。经积极控制感染、纠正水电解质紊乱症状很快缓解,血醛固酮、肾素水平迅速恢复正常。考虑为泌尿道畸形和/或泌尿道感染引起的暂时性假性醛固酮减少症故停用激素,积极抗感染治疗后外科手术治疗泌尿道畸形,随访中未再发生电解质紊乱及代谢性酸中毒。结论新生儿期后的婴幼儿失盐危象,须排查是否存在泌尿道感染和泌尿道畸形,并观察抗生素治疗后48小时内是否能迅速纠正水电解质紊乱,以避免过度检查和治疗。Objective To explore the diagnosis and treatment of transient pseudohypoaldosteronism.Methods Clinical data including clinical manifestations,laboratory findings,diagnosis and treatments of a case with transient pseudohypoaldosteronism were retrospectively analyzed,and related literatures were reviewed.Results A female infant,3 months of age,was hospitalized due to electrolyte disturbances.Laboratory findings showed hyponatremia,hyperkalemia,metabolic acidosis,elevated plasma aldosterone and renin,normal 17 hydroxyprogesterone,and ultrasonography showed urinary tract abnormalities.The child was diagnosed as congenital adrenal hyperplasia and then given the hormone therapy.When she was 6 months old,she was hospitalized due to electrolyte disturbances again,subsequent laboratory findings showed hyponatremia,hyperkalemia,metabolic acidosis,elevated plasma aldosterone and renin,normal 17 hydroxyprogesterone,and urine culture confirmed pyelonephritis caused by Klebsiella pneumonia>105 CFU/mL and urinary tract abnormalities.After prompt correction of electrolyte disturbances and anti-infection,the electrolyte,plasma aldosterone and renin levels quickly returned to normal.Related literatures review was performed using PubMed,and there has no case reported in China but some foreign literatures,we considered the child as a transient pseudohypoaldosteronism caused by urinary tract abnormalities or/and urinary tract infections.Therefore,we stopped the hormone therapy and then gave anti-infection treatment followed by surgical procedures for the treatment of urinary tract anomalies.Conclusions Diagnosis of congenital adrenal disorders is essential in a baby who develops a salt-losing crisis in the first few weeks of life.However,urinary tract abnormalities and urinary tract infection should be considered and rapidly excluded in any infant presenting with a salt-losing crisis with hyponatremia and hyperkalemia in particular in the late neonatal period.

关 键 词：失盐危象 假性醛固酮减少症 泌尿道感染 泌尿道畸形 

分 类 号：R725.8[医药卫生—儿科]