Metabolic Control and "Ideal" Outcomes in Liver Transplantation for Maple Syrup Urine Disease.

ABSTRACT:

Objectives

To assess outcomes following liver transplantation for maple syrup urine disease by determining attainment and sustainability of metabolic control and apply an "ideal" outcome composite in long-term survivors.

Study design

A single center, retrospective review collected clinical data including branched-chain amino acid (leucine, isoleucine, and valine) levels following liver transplant and determined achievement of an ideal long-term outcome profile of a first allograft stable on immunosuppression monotherapy, normal growth, and absence of common transplant-related sequelae.

Results

Of 77 patients meeting inclusion criteria identified, 23 were long-term (≥10-year) survivors and were additionally assessed for ideal outcome attainment. Patient and graft survi

SUBMITTER: Ewing CB 

PROVIDER: S-EPMC9795541 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature