Paediatric cholestatic liver disease:Diagnosis,assessment of disease progression and mechanisms of fibrogenesis  

作　　者：Tamara N Pereira Meagan J Walsh Peter J Lewindon Grant A Ramm 

机构地区：[1]The Hepatic Fibrosis Group,The Queensland Institute of Medical Research [2]Department of Gastroenterology,Royal Children's Hospital

出　　处：《World Journal of Gastrointestinal Pathophysiology》2010年第2期69-84,共16页世界胃肠病理生理学杂志（英文版）（电子版）

基　　金：Supported by a Project Grant from the National Health and Medical Research Council of Australia(NHMRC#496602 to GAR and PJL);Dr.Tamara Pereira is Supported by the Phillip Bushell Foundation Post-Doctoral Research Fellowship from the Gastroenterological Society of Australia;Associate Professor Grant A Ramm is Supported by a NHMRC Senior Research Fellowship(NHMRC#552409)

摘　　要：Cholestatic liver disease causes significant morbidity and mortality in children.The diagnosis and management of these diseases can be complicated by an inability to detect early stages of fibrosis and a lack of adequate interventional therapy.There is no single gold standard test that accurately reflects the presence of liver disease,or that can be used to monitor fibrosis progression,particularly in conditions such as cystic fibrosis.This has lead to controversy over how suspected liver disease in children is detected and diagnosed.This review discusses the challenges in using commonly available methods to diagnose hepatic fibrosis and monitor disease progression in children with cholestatic liver disease.In addition,the review examines the mechanisms hypothesised to be involved in the development of hepatic fibrogenesis in paediatric cholestatic liver injury which may ultimately aid in identifying new modalities to assist in both disease detection and therapeutic intervention.Cholestatic liver disease causes significant morbidity and mortality in children. The diagnosis and management of these diseases can be complicated by an inability to detect early stages of fibrosis and a lack of adequate interventional therapy. There is no single gold standard test that accurately reflects the presence of liver disease, or that can be used to monitor fibrosis progression, particularly in conditions such as cystic fibrosis. This has lead to controversy over how suspected liver disease in children is detected and diagnosed. This review discusses the challenges in using commonly available methods to diagnose hepatic fibrosis and monitor disease progression in children with cholestatic liver disease. In addition, the review examines the mechanisms hypothesised to be involved in the development of hepatic fibrogenesis in paediatric cholestatic liver injury which may ultimately aid in identifying new modalities to assist in both disease detection and therapeutic intervention.

关 键 词：CYSTIC FIBROSIS Biliary ATRESIA Liver biopsy Ultrasound HEPATIC FIBROSIS Cirrhosis HEPATIC stellatecell Bile acid CHEMOTAXIS Monocyte CHEMOTAXIS protein-1 

分 类 号：R725.7[医药卫生—儿科]