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神经结节病相关的中枢性尿崩症被肾上腺功能不全所掩盖。

Neurosarcoidosis-associated central diabetes insipidus masked by adrenal insufficiency.

作者信息

Non Lemuel, Brito Daniel, Anastasopoulou Catherine

机构信息

Department of Medicine, Albert Einstein Medical Center, Philadelphia, Pennsylvania, USA.

Department of Endocrinology, Albert Einstein Medical Center, Elkins Park, Pennsylvania, USA.

出版信息

BMJ Case Rep. 2015 Jan 22;2015:bcr2014206390. doi: 10.1136/bcr-2014-206390.

Abstract

Central diabetes insipidus (CDI) is an infrequent complication of neurosarcoidosis (NS). Its presentation may be masked by adrenal insufficiency (AI) and uncovered by subsequent steroid replacement. A 45-year-old woman with a history of NS presented 2 weeks after abrupt cessation of prednisone with nausea, vomiting, decreased oral intake and confusion. She was diagnosed with secondary AI and intravenous hydrocortisone was promptly begun. Over the next few days, however, the patient developed severe thirst and polyuria exceeding 6 L of urine per day, accompanied by hypernatraemia and hypo-osmolar urine. She was presumed to have CDI due to NS, and intranasal desmopressin was administered. This eventually normalised her urine output and serum sodium. The patient was discharged improved on intranasal desmopressin and oral prednisone. AI may mask the manifestation of CDI because low serum cortisol impairs renal-free water clearance. Steroid replacement reverses this process and unmasks an underlying CDI.

摘要

中枢性尿崩症(CDI)是神经结节病(NS)的一种罕见并发症。其表现可能被肾上腺功能不全(AI)掩盖,并在随后进行类固醇替代治疗时显现出来。一名有NS病史的45岁女性在泼尼松突然停药2周后出现恶心、呕吐、口服摄入量减少和意识模糊。她被诊断为继发性AI,并立即开始静脉注射氢化可的松。然而,在接下来的几天里,患者出现严重口渴和多尿,每天尿量超过6升,伴有高钠血症和低渗尿。她被推测因NS患有CDI,并给予了鼻内去氨加压素治疗。这最终使她的尿量和血清钠恢复正常。患者出院时,鼻内去氨加压素和口服泼尼松治疗后病情好转。AI可能掩盖CDI的表现,因为低血清皮质醇会损害肾脏的自由水清除能力。类固醇替代治疗可逆转这一过程,并使潜在的CDI显现出来。

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