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吉特林综合征:癫痫发作障碍的罕见病因及系统评价

Gitelman Syndrome: A Rare Cause of Seizure Disorder and a Systematic Review.

作者信息

Shahzad Muhammad Asim, Mukhtar Maryam, Ahmed Asrar, Ullah Waqas, Saeed Rehan, Hamid Mohsin

机构信息

Resident Physician, Louis Weiss Memorial Hospital, Chicago, IL, USA.

Independent Research Scholar, Fauji Foundation Hospital, Rawalpindi, Pakistan.

出版信息

Case Rep Med. 2019 Feb 5;2019:4204907. doi: 10.1155/2019/4204907. eCollection 2019.

Abstract

Gitelman syndrome is one of the few inherited causes of metabolic alkalosis due to salt losing tubulopathy. It is caused by tubular defects at the level of distal convoluted tubules, mimicking a thiazide-like tumor. It usually presents in late childhood or in teenage as nonspecific weakness, fatigability, polyuria, and polydipsia but very rarely with seizures. It is classically associated with hypokalemia, hypomagnesemia, hypocalciuria, hyperreninemia, and hyperaldosteronism. However, less frequently, it can present with normal magnesium levels. It is even rarer to find normomagnesemic patients of GS who develop seizures as the main complication since hypomagnesemia is considered the principal etiology of abnormal foci of seizure-related brain activity in GS cases. Interestingly, patients with GS are oftentimes diagnosed during pregnancy when the classic electrolyte pattern consistent with GS is noticed. Our case presents GS with normal serum magnesium in a patient, with seizures being the main clinical presentation. We also did a comprehensive literature review of 122 reported cases to show the prevalence of normal magnesium in GS cases and an overview of clinical and biochemical variability in GS. We suggest that further studies and in-depth analysis are required to understand the pathophysiology of seizures in GS patients with both normal and low magnesium levels.

摘要

吉特曼综合征是因失盐性肾小管病导致代谢性碱中毒的少数遗传性病因之一。它由远曲小管水平的肾小管缺陷引起,类似噻嗪样病变。通常在儿童晚期或青少年期出现,表现为非特异性的虚弱、易疲劳、多尿和烦渴,但很少伴有癫痫发作。典型表现为低钾血症、低镁血症、低钙尿症、高肾素血症和高醛固酮血症。然而,较少见的是,其镁水平可正常。更罕见的是,吉特曼综合征患者以癫痫发作为主要并发症,而其血镁水平正常,因为低镁血症被认为是吉特曼综合征病例中癫痫相关脑活动异常灶的主要病因。有趣的是,吉特曼综合征患者常在孕期被诊断出来,此时会发现与吉特曼综合征相符的典型电解质模式。我们的病例报告了一名血清镁正常的吉特曼综合征患者,以癫痫发作为主要临床表现。我们还对122例报告病例进行了全面的文献综述,以显示吉特曼综合征病例中血镁正常的患病率以及吉特曼综合征临床和生化变异性的概述。我们建议需要进一步研究和深入分析,以了解血镁正常和低镁的吉特曼综合征患者癫痫发作的病理生理学。

相似文献

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[Expert consensus for the diagnosis and treatment of patients with Gitelman syndrome].[吉特林综合征患者诊断与治疗专家共识]
Zhonghua Nei Ke Za Zhi. 2017 Sep 1;56(9):712-716. doi: 10.3760/cma.j.issn.0578-1426.2017.09.021.
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Gitelman Syndrome and Hypertension: A Case Report.吉特曼综合征与高血压:一例报告
Cureus. 2023 Sep 2;15(9):e44590. doi: 10.7759/cureus.44590. eCollection 2023 Sep.
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Two Japanese patients with gitelman syndrome.两名患有吉特曼综合征的日本患者。
Clin Pediatr Endocrinol. 2006;15(4):137-42. doi: 10.1297/cpe.15.137. Epub 2006 Nov 3.

本文引用的文献

4
Gitelman Syndrome.吉特曼综合征
J Coll Physicians Surg Pak. 2017 Mar;27(3):S30-S32.
7
Cryptic exon activation in SLC12A3 in Gitelman syndrome.吉特曼综合征中SLC12A3基因的隐匿外显子激活
J Hum Genet. 2017 Feb;62(2):335-337. doi: 10.1038/jhg.2016.129. Epub 2016 Oct 27.

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