Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.

ABSTRACT:

Background

Current world experience regarding living donor liver transplantation (LDLT) in the treatment of propionic acidemia (PA) is limited, especially in terms of using obligate heterozygous carriers as donors. This study aimed to evaluate the clinical outcomes of LDLT in children with PA.

Methods

From November 2017 to January 2020, 7 of the 192 children who underwent LDLT at our institution had been diagnosed with PA (median age, 2.1 years; range, 1.1-5.8 years). The primary indication for transplantation was frequent metabolic decompensations in 6 patients and preventative treatment in 1 patient. Of the seven parental living donors, six were genetically proven obligate heterozygous carriers.

Results

During a median follow-up of 23.9 months (range, 13.9-40.2 mont

SUBMITTER: Zeng ZG 

PROVIDER: S-EPMC8862340 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature