Risk factors associated with mortality in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and clinical implications.

ABSTRACT:

Background

Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) has high prevalence in East Asia, and has been reported in other parts of the world. NICCD is also the most common form of genetic cholestasis among East Asians. There has been reports of mortalities or liver transplants associated with NICCD, but risk factors associated with poor outcome were unknown. Our objective is to report NICCD mortalities in a tertiary pediatric hepatology center, and to explore associated risk factors along with implications to clinical practice.

Method

This is a retrospective analysis of NICCD cases collected from June 2003 until January 2017 in the Children's Hospital of Fudan University. Clinical, biochemical, and genetic data were compared between deceased cases and

SUBMITTER: Abuduxikuer K 

PROVIDER: S-EPMC6330752 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature