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儿科脂肪性肝病(PeFLD):并非所有都为非酒精性脂肪性肝病——病理生理学见解与管理方法。

Paediatric fatty liver disease (PeFLD): All is not NAFLD - Pathophysiological insights and approach to management.

机构信息

Paediatric Liver, GI and Nutrition Centre and Mowatlabs, King's College Hospital, London, United Kingdom.

Liver Histopathology, Institute of Liver Studies, King's College Hospital, London, United Kingdom.

出版信息

J Hepatol. 2018 Jun;68(6):1286-1299. doi: 10.1016/j.jhep.2018.02.006. Epub 2018 Feb 20.

Abstract

The recognition of a pattern of steatotic liver injury where histology mimicked alcoholic liver disease, but alcohol consumption was denied, led to the identification of non-alcoholic fatty liver disease (NAFLD). Non-alcoholic fatty liver disease has since become the most common chronic liver disease in adults owing to the global epidemic of obesity. However, in paediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders can mimic or co-exist with a diagnosis of NAFLD. The term paediatric fatty liver disease may be more appropriate. In this article, we summarise the known causes of steatosis in children according to their typical, clinical presentation: i) acute liver failure; ii) neonatal or infantile jaundice; iii) hepatomegaly, splenomegaly or hepatosplenomegaly; iv) developmental delay/psychomotor retardation and perhaps most commonly; v) the asymptomatic child with incidental discovery of abnormal liver enzymes. We offer this model as a means to provide pathophysiological insights and an approach to management of the ever more complex subject of fatty liver.

摘要

由于肥胖在全球流行,非酒精性脂肪性肝病(NAFLD)已成为成年人中最常见的慢性肝病。但是,在儿科中,“非酒精性脂肪性肝病”这个术语似乎并不合适:儿童饮酒的情况并不常见,遗传性代谢紊乱可与 NAFLD 的诊断相似或同时存在。“小儿脂肪性肝病”这个术语可能更为恰当。在本文中,我们根据儿童脂肪变性的典型临床表现总结了已知病因:i)急性肝衰竭;ii)新生儿或婴儿期黄疸;iii)肝肿大、脾肿大或肝脾肿大;iv)发育迟缓/精神运动迟缓;v)无症状儿童偶然发现肝功能异常。我们提供了这个模型,以便为日益复杂的脂肪性肝病提供病理生理学见解和管理方法。

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