首页> 外文期刊>American Journal of Kidney Diseases: The official journal of the National Kidney Foundation >Acquired Gitelman syndrome in a patient with primary Sjogren syndrome.
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Acquired Gitelman syndrome in a patient with primary Sjogren syndrome.

机译:原发性干燥综合征患者的后天吉特曼综合征。

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Acquired Gitelman syndrome (GS) associated with Sjogren syndrome (SS) is rare, and the test to determine the pathophysiological state of acquired GS in patients with primary SS has not been reported previously. A 47-year-old woman with sicca complex presented to our clinic with intermittent muscle cramping and weakness involving both lower extremities over several months. Laboratory findings showed hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria, which met the criteria for GS. Diagnostic evaluation identified primary SS as the cause of the acquired GS. Light microscopic examination of renal tissue from the patient showed mild tubulointerstitial nephritis. Immunohistochemical staining of renal tissue showed the absence of the sodium-chloride cotransporter (NCCT) in the distal convoluted tubules. Incubation of the patient's serum with normal mouse kidney tissue showed a pattern of NCCT in the distal convoluted tubules similar to that of incubation of normal mouse kidney with the rabbitpolyclonal anti-NCCT antibody. This is a rare case of acquired GS associated with primary SS, and our findings suggest the presence of circulating autoantibodies to NCCT.
机译:与干燥综合征(SS)相关的后天性吉特曼综合征(GS)很少见,以前没有关于确定原发性SS患者后天性GS病理生理状态的测试的报道。一位47岁的患有干燥综合征的妇女因几个月来双下肢肌肉痉挛和无力而出现在我们诊所。实验室检查结果显示低钾代谢性碱中毒,低镁血症和低钙尿症符合GS标准。诊断评估确定主要的SS是获得性GS的原因。病人的肾组织的光学显微镜检查显示轻度肾小管间质性肾炎。肾脏组织的免疫组织化学染色显示,在远端的曲折小管中不存在氯化钠共转运蛋白(NCCT)。患者血清与正常小鼠肾脏组织的温育显示出在远端螺旋小管中的NCCT模式,类似于使用兔多克隆抗NCCT抗体温育正常小鼠肾脏的模式。这是与原发性SS相关的获得性GS的罕见病例,我们的发现表明存在针对NCCT的循环自身抗体。

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