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The clinical and pathological analysis of 24 children with vanishing bile duct syndrome

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Author(s): Shu-hong LIU | Jing-min ZHAO | Guang-de ZHOU | Yu-lai ZHAO | Wen-shu LI | Xiao-dong GUO | Li LIANG | Lu XIONG | Li-xin WEI

Journal: Medical Journal of Chinese People's Liberation Army
ISSN 0577-7402

Volume: 36;
Issue: 1;
Start page: 80;
Date: 2011;
Original page

Keywords: vanishing bile duct syndrome; child; pathology; clinical

ABSTRACT
Objective To investigate the clinical and pathological characteristics of vanishing bile duct syndrome(VBDS),and provide references for clinical diagnosis.Methods Twenty four children with VBDS diagnosed by liver biopsy were collected in 302 Hospital of PLA from 2007 to 2009.Liver tissues were stained with haematoxylin eosin staining.The clinical and pathological characteristics were analyzed.Results The mean age of all patients(mean SD) was 4.08±3.11 years and the sex ratio(male to female) was 2.4:1.The most common symptoms were jaundice and pruritus in 24 children with VBDS.The mean levels of ALT,AST,ALP,GGT and TBil were 203.45 239.42U/L,238.54 224.11U/L,524.04 300.96U/L,242.17 220.86U/L and 242.17 220.86 μmol/L,respectively.Nineteen of 24 cases were positive for anti-CMV IgM,anti-CMV IgG or CMV PP65.Five cases were undergone the operation of biliary atresia.The main pathological characteristic of VBDS was atresia or disappearance of interlobular bile ducts to various degrees.Moreover,histological changes varied with respect to different causes.Obvious inflammatory infiltration in the portal tracts and hepatocytes damage were more common in VBDS caused by cytomegalovirus(CMV) infection.Conclusions The incidence of VBDS is higher in boys than in girls.CMV infection plays an important role in the progress of VBDS.Different pathological characteristics may exist in VBDS caused by different etiological factors.

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