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[突尼斯儿童的肝硬化及致肝硬化疾病。65例多中心研究]

[Cirrhosis and cirrhogenic diseases in Tunisian children. Multicenter study of 65 cases].

作者信息

Amri F, Pousse H, Gueddiche M N, Radhouane M, Sfar M T, Kharrat H, Essoussi A S, Harbi A

机构信息

Service de pédiatrie, CHU, Tunisie.

出版信息

Pediatrie. 1992;47(6):473-5.

PMID:1331967
Abstract

Over a period of 10 years, 65 cases of hepatic cirrhosis and cirrhogenic disease have been observed in five Pediatric Centers in mid Tunisia. The age of the patients ranged from 30 days to 14 years. The main etiology was biliary cirrhosis (24 cases) followed by post-hepatic cirrhosis (15 cases). Eight cases had a metabolic origin, which was Wilson's diseases in five cases. Three children had cirrhosis of a pre-hepatic origin. In 15 children, the liver biopsy showed the presence of cirrhosis but the etiology could not be found. Preventive measures are needed in order to reduce the frequency of cirrhosis among Tunisian children: 1) early recognition of biliary atresia, 2) vaccination against hepatitis B virus of at risk neonates and children, 3) Genetic counselling and search for familial cases when cirrhosis of metabolic origin is identified, particularly Wilson's disease.

摘要

在突尼斯中部的五个儿科中心,经过10年的观察,共发现65例肝硬化和致肝硬化疾病病例。患者年龄从30天至14岁不等。主要病因是胆汁性肝硬化(24例),其次是肝后性肝硬化(15例)。8例有代谢性病因,其中5例为威尔逊病。3例儿童患有肝前性肝硬化。15例儿童的肝活检显示存在肝硬化,但病因不明。为降低突尼斯儿童肝硬化的发病率,需要采取预防措施:1)早期识别胆道闭锁;2)对高危新生儿和儿童接种乙肝疫苗;3)当确定为代谢性病因的肝硬化,尤其是威尔逊病时,进行遗传咨询并查找家族病例。

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