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[妊娠期吉特林综合征——一种伴有良好围产期预后的严重低钾血症]

[Gitelman syndrome in pregnancy--a severe hypokalemia with favorable perinatal prognosis].

作者信息

Rušavý Z, Hudec A, Karbanová J, Korečko V, Janů R, Kališ V

机构信息

Gynekologicko-porodnická klinika LF a FN, Plzen, prednosta doc. MUDr. Z. Novotný, CSc.

出版信息

Ceska Gynekol. 2012 Oct;77(5):421-3.

Abstract

Gitelman syndrom is a rare congenital tubulopathy characterized by hypokalemia, hypomagnesemia, metabolic alkalosis and hypocalciuria. We report a case of a 32-year-old patient admitted for asymptomatic hypokalemia and hypomagnesemia in the 30th week of gestation. A diagnosis of Gitelman syndrom was made and intravenous administration of potassium chloride in high doses combined with spironolactone was started. Despite intensive potassium supplementation (8 g/day), the serum potassium levels remained at the lower limit of normality throughout the pregnancy. The patient delivered a healthy female 2670 g/48 cm after labor induction in the 39th week of gestation. A summary of 22 so far published cases of Gitelman syndrome in pregnancy is presented. The analysis of published case studies suggests a need for ion supplementation, reduction of urinary potassium wasting, monitoring of fetal well-being and amniotic fluid levels. Pregnancy has a very favorable perinatal prognosis despite critical serum levels of potassium and magnesium throughout the pregnancy.

摘要

吉特曼综合征是一种罕见的先天性肾小管疾病,其特征为低钾血症、低镁血症、代谢性碱中毒和低钙尿症。我们报告一例32岁患者,在妊娠第30周因无症状低钾血症和低镁血症入院。诊断为吉特曼综合征,并开始静脉高剂量输注氯化钾联合螺内酯治疗。尽管大量补钾(8克/天),但整个孕期血清钾水平一直维持在正常下限。患者在妊娠第39周引产,分娩出一名健康女婴,体重2670克,身长48厘米。本文总结了迄今为止已发表的22例妊娠合并吉特曼综合征病例。对已发表病例研究的分析表明,需要补充离子、减少尿钾流失、监测胎儿健康和羊水水平。尽管整个孕期血清钾和镁水平危急,但妊娠的围产期预后非常良好。

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