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AL Diabetes Insipidus and Langerhans Cell Stiocytosis-A Case Report and Review of Literature

中樞性尿崩症與蘭格罕細胞組織球增生症-病例報告與文獻回顧

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摘要


中樞性尿崩症是因作用在抗利尿激素分泌位置的病變而引起多尿、劇渴和高血鈉。蘭格罕細胞組織球增生症是其中的一個原因。蘭格罕細胞組織球增生症是一罕見的病變,在身體的多個地方會有肉芽腫狀的沉澱,能侵犯下視丘腦垂體軸而中樞性尿崩症為其普遍的表現。我們報告一個五十二歲的婦人以全身無力、多尿、劇渴為呈現,她有高血鈉(血清鈉173mEq/L)且在水份限制測試後診斷為中樞性尿崩症。腦部核磁共振造影在下視丘有腫瘤。在外科手術後,病理報告為蘭格罕細胞組織球增生症。在文章中我們討論蘭格罕細胞組織球增生症及臨床病程。

並列摘要


Central diabetes insipidus (DI), resulting from disorders that act on the site involved in the secretion of antidiuretic hormone (ADH), leads to polyuria, polydipsia, and hypernatremia. Langerhans cell histiocytosis (LCH) is one of the causes. LCH, a rare disorder in which granulomatous deposits occur at multiple sites within the body, could involve the hypothalamo-pituitary axis and DI would be a common manifestation. We report the case of a 52-year-old woman, with initial presentation of general weakness, polyuria, and polydipsia, who had hypernatremia (serum sodium level: 173 mEq/L) and central DI was diagnosed after a water deprivation test. Brain magnetic resonance imaging (MRI) revealed a mass lesion in the hypothalamus. The pathological report after surgical intervention revealed LCH. Here, LCH and her clinical course are briefly discussed.

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