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巴特综合征和吉特林综合征。

Bartter Syndrome and Gitelman Syndrome.

作者信息

Fulchiero Rosanna, Seo-Mayer Patricia

机构信息

Department of Pediatrics, Inova Children's Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA.

Department of Pediatrics, Inova Children's Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA; Division of Nephrology and Hypertension, Pediatric Specialists of Virginia, 3023 Hamaker Court, Suite 600, Fairfax, VA 22031, USA; Virginia Commonwealth School of Medicine, Richmond, VA, USA.

出版信息

Pediatr Clin North Am. 2019 Feb;66(1):121-134. doi: 10.1016/j.pcl.2018.08.010.

Abstract

Bartter and Gitelman syndromes are conditions characterized by renal salt-wasting. Clinical presentations range from severe antenatal disease to asymptomatic with incidental diagnosis. Hypokalemic hypochloremic metabolic alkalosis is the common feature. Bartter variants may be associated with polyuria and weakness. Gitelman syndrome is often subtle, and typically diagnosed later life with incidental hypokalemia and hypomagnesemia. Treatment may involve fluid and electrolyte replenishment, prostaglandin inhibition, and renin-angiotensin-aldosterone system axis disruption. Investigators have identified causative mutations but genotypic-phenotypic correlations are still being characterized. Collaborative registries will allow improved classification schema and development of effective treatments.

摘要

巴特综合征和吉特林综合征是以肾性失盐为特征的病症。临床表现从严重的产前疾病到偶然诊断出的无症状情况不等。低钾性低氯性代谢性碱中毒是其共同特征。巴特综合征的变异型可能与多尿和肌无力有关。吉特林综合征通常较为隐匿,典型情况是在晚年因偶然发现低钾血症和低镁血症而被诊断。治疗可能包括补充液体和电解质、抑制前列腺素以及破坏肾素 - 血管紧张素 - 醛固酮系统轴。研究人员已经确定了致病突变,但基因型与表型的相关性仍在研究之中。合作登记处将有助于改进分类方案并开发有效的治疗方法。

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