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木糖醇诱发的肾脑草酸沉积症

Reno-cerebral oxalosis induced by xylitol.

作者信息

Ludwig B, Schindler E, Bohl J, Pfeiffer J, Kremer G

出版信息

Neuroradiology. 1984;26(6):517-21. doi: 10.1007/BF00342692.

Abstract

A 20-year-old man suffering from Crohn's disease developed coma and generalized seizures following ileocecal resection. During postoperative parenteral feeding he received xylitol in an unusually high concentration. CT examinations a few days before death showed intense hypodensity and swelling of brainstem and basal ganglia and increasing triventricular dilatation. Autopsy revealed, mainly in the brainstem and cerebellum, a destruction of intracerebral, intracerebellar and leptomeningeal vessel walls by birefringent crystals (probably calcium oxalate), an early inflammatory reaction and severe brain edema with final tonsillar herniation. The same crystalloid deposits were found in the kidneys.

摘要

一名患有克罗恩病的20岁男性在回盲部切除术后出现昏迷和全身性癫痫发作。术后肠外营养期间,他接受了浓度异常高的木糖醇。死亡前几天的CT检查显示脑干和基底神经节有明显的低密度影和肿胀,以及三脑室扩张加重。尸检发现,主要在脑干和小脑,双折射晶体(可能是草酸钙)破坏了脑内、脑桥小脑和软脑膜血管壁,出现早期炎症反应和严重脑水肿,最终导致小脑扁桃体疝。在肾脏中也发现了同样的晶体沉积物。

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