Liver transplantation in children with inborn errors of metabolism: 30 years experience in NSW, Australia.

ABSTRACT:

Background

Inborn errors of metabolism (IEM) are a diverse group of genetic disorders that can result in significant morbidity and sometimes death. Metabolic management can be challenging and burdensome for families. Liver transplantation (LT) is increasingly being considered a treatment option for some IEMs. IEMs are now considered the second most common reason for pediatric LT.

Aim

To review the data of all children with an IEM who had LT at The Children's Hospital at Westmead (CHW), NSW, Australia between January 1986 and January 2019.

Methods

Retrospective data collected from the medical records and genetic files included patient demographics, family history, parental consanguinity, method of diagnosis of IEM, hospital and intensive care unit admissions, age at LT

SUBMITTER: Elserafy N 

PROVIDER: S-EPMC8260479 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature