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肝静脉闭塞性疾病:一例报告。

Veno-occlusive liver disease: a case report.

作者信息

Schneider Jakob, Tsegaye Yohannes, W/Tensae Mihret, G/Selassie Solomon, Haile Tewodros, Bane Abate, Ali Ahmed, Mesfin Gebrekidan, Seboxa Teshale

机构信息

Addis Ababa University, Faculty of Medicine, Department of Pathology, Addis Ababa, Ethiopia.

出版信息

Ethiop Med J. 2012 Apr;50 Suppl 2:47-51.

Abstract

A case of veno-occlusive liver disease (VOLD) in a 12-years old Ethiopian boy is described The salient clinical features and gross and microscopic examination of biopsy material are reviewed. Veno-occlusive disease which occurs in the West Indies, East and West Africa, and India is an acute, subacute or chronic condition that affects the central and sublobular hepatic veins. In the West Indies (1) it is related to the consumption of bush tea made from plants that contain toxic pyrrolizidine alkaloids, such as Crotalaria and Senecio (2). Hepatotoxic compounds in Crotalaria, Senecio, Heliotropium and other composite plants can also enter the diet through the contamination of cereals with weed seeds. For example 28 of 67 patients died with veno-occlusive disease in central India after consuming a local cereal, gondli contaminated with the seeds of Crotalaria (3). Heliotropium Popovii has been implicated in outbreaks in villages in northwestern Afghanistan, with high mortality (4). The primary pathological change of hepatic veno-occlusive disease is sub-endothelial edema followed by intimal growth of connective tissue, with narrowing and occlusion of the central and sub-lobular hepatic veins. Atrophy or necrosis of liver cells, with consequent fibrosis leads to gross changes similar to those seen in cardiac cirrhosis, portal hypertension results. The present report, the first of it kind in Ethiopia describes a case of veno-occlusive liver disease in a 12-year old Ethiopian boy.

摘要

本文描述了一名12岁埃塞俄比亚男孩患肝静脉闭塞性疾病(VOLD)的病例。回顾了活检材料的显著临床特征以及大体和显微镜检查结果。肝静脉闭塞性疾病发生在西印度群岛、东非和西非以及印度,是一种影响肝中央静脉和小叶下静脉的急性、亚急性或慢性疾病。在西印度群岛,(1)它与饮用由含有有毒吡咯里西啶生物碱的植物制成的灌木茶有关,如猪屎豆属和千里光属植物(2)。猪屎豆属、千里光属、天芥菜属和其他菊科植物中的肝毒性化合物也可通过杂草种子污染谷物进入饮食。例如,印度中部67名患有肝静脉闭塞性疾病的患者中有28人在食用了被猪屎豆属种子污染的当地谷物贡德利后死亡(3)。阿富汗西北部村庄的疫情爆发与波波夫天芥菜有关,死亡率很高(4)。肝静脉闭塞性疾病的主要病理变化是内皮下水肿,随后结缔组织内膜增生,导致肝中央静脉和小叶下静脉狭窄和闭塞。肝细胞萎缩或坏死,继而纤维化,导致大体变化类似于心源性肝硬化所见,引发门静脉高压。本报告是埃塞俄比亚首例此类报告,描述了一名12岁埃塞俄比亚男孩患肝静脉闭塞性疾病的病例。

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