最初无肝脏受累的脑病合并内脏脂肪变性综合征

来源 :国外医学参考资料(儿科学分册) | 被引量 : 0次 | 上传用户:fallleaf
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本病的诊断依靠病史、体检和化验的综合分析。病儿常有流感样前驱症状,随之而来是严重呕吐,定向力障碍,进而迅速转入昏迷状态,伴有或不伴有局部或全身性痉挛。过去的报告强调脑炎性表现合并肝功能障碍(如凝血酶元时间延长,谷草转氨酶、乳酸脱氢酶等升高)。肝活检能证实诊断,表现为全小叶所有肝细胞均有脂肪沉着而无炎症反应,故本病的脑病常认为是肝功能障碍的结果。本文报告1例经活检和尸检证实之病例,其神经系统的表现明显地发生在肝脏疾患早期征象之前。此例为一11岁男孩,起病前1周有轻微上呼吸道感染, The diagnosis of the disease rely on medical history, physical examination and laboratory analysis. Sick children often have flu-like prodromal symptoms, followed by severe vomiting, disorientation, and then quickly into a coma, with or without local or systemic spasms. Past reports emphasize the manifestation of encephalitogenic liver dysfunction (such as prolonged thrombin time, aspartate aminotransferase, lactate dehydrogenase, etc.). Liver biopsy can confirm the diagnosis, manifested as whole leaflets are all fat cells without inflammatory reaction, so encephalopathy of the disease often considered as a result of liver dysfunction. This article reports a case of biopsy and autopsy confirmed the case, the performance of the nervous system obviously occurred in the early signs of liver disease before. This case is an 11-year-old boy with a slight upper respiratory infection 1 week prior to onset,
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