Bartter综合征的诊断与治疗——附5例报告

Diagnosis and treatment of Bartter Syndrome

目的探讨Bartter综合征在儿童中的临床特点及诊断与治疗。方法分析本院5例患儿的临床资料。结果Bartter综合征儿童发病,男性多见,其特点具有①水、电解质及酸碱失衡;②血清肾素、血管紧张素、醛固酮明显增高,而血压正常,③应用氯化钾、消炎痛、安体舒通等综合治疗,效果明显。结论Bartter综合征儿童中首发症状大多为多饮多尿或反复呕吐、低钾血症、低氯性代谢性碱中毒、肾素-血管紧张素Ⅱ-醛固酮明显升高。血压正常者有助于诊断与鉴别诊断。

ObjectiveTo explore the clinical features, diagnosis and treatment of Bartter syndrome in children. Methods5cases of Bartter syndrome were reported and reviewed with literature. The occurrence , clinical manifestation, laboratory investigationand treatment were analyzed. ResultsMost patients had been confirmed diagnosis in childhood or in early adolescence. Thesepatients shared myriad clinical symptoms and laboratory abnormalities:①hypokalemia, hypochloremic metabolic alkalosis; ②plasma renin activity, serum concentration of angiotersin Ⅱ and aldosterone were markly elevated, the blood pressure was normal; ③potassium choloride supplementation was the main step of therapy. ConclusionsThe first symptom in children is polydipsia,polyuria, vomiting or diarrhea. The disease is characterized by hypokalemia, hypochloremic metabolic ackalosis, increased renin-angiotension Ⅱ-aldosterone levels and normal blood pressure.