Hansen Klavs Würgler, Mosekilde Leif
Medicinsk Endokrinologisk Afdeling C, Arhus Amtssygehus, Arhus Universitetshospital, DK-8000 Arhus C.
Ugeskr Laeger. 2003 Mar 10;165(11):1123-7.
Gitelmans syndrome represents the clinical manifestations of inactivating mutations in the gene encoding for the thiazide sensitive sodium chloride cotransporter in the distal convoluted tubule. Thus, the biochemical characteristics resemble those seen with thiazide diuretics: hypokalemia, hypomagnesemia, hypocalcuria, metabolic alkalosis and blood pressure in the low normal range. Until the genetic background was clarified in 1996, Gitelman's syndrome was often mistaken for Bartter's syndrome, which is now attributed to defects in the ion transportation system in the thick ascending limb of Henle's loop. In Bartter's syndrome, hypomagnesemia is not a constant finding and urinary calcium excretion is normal or high. Bartter,s syndrome is often diagnosed neonatally and followed by growth retardation and nefrocalcinosis. Gitelman's syndrome is typically diagnosed accidentally (hypokalemia) in adolescents or adults and the course is benign. If present, the most prominent symptoms are muscular fatigue or occasional tetany. Treatment includes magnesium and potassium supplements and potassium saving diuretics.
吉特林综合征表现为编码远曲小管中噻嗪类敏感型氯化钠协同转运蛋白的基因发生失活突变的临床表现。因此,其生化特征与噻嗪类利尿剂所致者相似:低钾血症、低镁血症、低尿钙、代谢性碱中毒以及血压处于正常低限范围。在1996年其遗传背景得以阐明之前,吉特林综合征常被误诊为巴特综合征,后者现被认为是由于髓袢升支粗段离子转运系统缺陷所致。在巴特综合征中,低镁血症并非持续存在的表现,尿钙排泄正常或增加。巴特综合征常于新生儿期被诊断出来,随后出现生长发育迟缓及肾钙质沉着症。吉特林综合征通常在青少年或成人中偶然(因低钾血症)被诊断出来,病程呈良性。若出现症状,最突出的症状是肌肉疲劳或偶尔发生的手足搐搦。治疗包括补充镁和钾以及保钾利尿剂。