Hemorrhagic Shock and Encephalopathy-Like Syndrome: Report of a Case with Emphasis on the Pathological Findings

出血性休克腦病變樣症候群：一病例報告

王瀛洲(Yng-Jou Wang) ； 麥淑珍(Suk-Chun Mak) ； 遲景上(Ching-Shiang Chi)

《Acta Paediatrica Sinica》 34卷5期 (1993/10) Pp. 398-404

摘要

本病例乃一五個月大男孩，其臨床表現符合出血性休克腦病變症候群的診斷，然其肝臟病理變化卻與先前文獻報告者有所不同。光學顯微鏡下可見顯著的脂肪變性合併散在出血點，電鏡呈現（氵彌）漫性粒線體和過氧小體內毛絮狀緻密沈積物，此不同之病理發現，可能代表著不同之致病機轉。因此，我們特別提出一個新的名詞“出血性休克腦病變樣症候群”，用來泛指一群與先前文獻描述者類似，但卻並不完全相同之疾病。同時，希望藉此病例提起大家的興趣，從而對這個來勢洶洶的疾病有更進一步的研究與認識。

並列摘要

The case of a 5-month-old boy with sudden onset of shock, encephalopathy, watery diarrhea, bleeding tendency, and impaired hepatic and renal functions was reported. The different pathological findings as compared with the previous reports made the term ”hemorrhagic shock encephalopathy-like syndrome” (HSE-like syndrome) more suitable for this case. Marked fatty metamorphosis with hemorrhage was found with a light microscope. Electron microscopy revealed flocculent densities in the mitochondria which might be a poor prognostic indicator in this syndrome. Diffuse electron-dense deposits in the peroxisomes were also seen and the significance of this finding was discussed. The patient died on the third day after admission due to multi-organ failures in spite of our aggressive management.

並列關鍵字

hemorrhagic shock encephalopathy-like syndrome ； fatty metamorphosis ； flocculent densities