Liver involvement in children with ciliopathies

被引：34

作者：

Rock, N. ^{[1
]}

McLin, V. ^{[1
]}

机构：

[1] Univ Hosp Geneva, Dept Pediat, Swiss Ctr Liver Dis Children, CH-1205 Geneva, Switzerland

来源：

CLINICS AND RESEARCH IN HEPATOLOGY AND GASTROENTEROLOGY | 2014年 / 38卷 / 04期

关键词：

CONGENITAL HEPATIC-FIBROSIS; POLYCYSTIC KIDNEY-DISEASE; SINGLE-CENTER; CILIA; MECHANISMS; TRANSPLANTATION; OLIGOPHRENIA; EXPERIENCE; ATAXIA;

D O I：

10.1016/j.clinre.2014.04.001

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Abnormalities in primary cilia lead to diseases called ciliopathies. Multiple organ involvement is the norm since primary cilia are present in most cells. When cholangiocyte cilia are abnormal, ductal plate malformation ensues leading to such conditions as congenital hepatic fibrosis, Caroli disease or syndrome, or other fibrocystic disease. (C) 2014 Elsevier Masson SAS. All rights reserved.

引用

页码：407 / 414

页数：8

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