Liver transplantation for inherited metabolic disorders: a work in progress
Sreekantam, V Kurkal, K Santra, S Suresh, V Raiman, J Johansen, L Mckiernan, P J Hutchin, T Sharif, K Kelgeri, C
Sreekantam, V
Kurkal, K
Santra, S
Suresh, V
Raiman, J
Johansen, L
Mckiernan, P J
Hutchin, T
Sharif, K
Kelgeri, C
Abstract
AIM: To describe the trend of indications and outcomes of Liver transplant (LT) for Inherited Metabolic Disorders (IMD) in a single centre over 30 years.
METHODS: A retrospective review of LT undertaken for IMD between 1989 to 2021 wasperformed. Demographic and clinical data, including LT indications, complications and outcomes, were collected. Categorical data were described as proportions; Kaplan-Meier analysis was used to determine patient/graft outcomes.
RESULTS: 55 patients (29 females) underwent 62 transplants between 1989 to 2021. Postoperative complications were rejection (17/62; 27.4%), bile leak (5/62; 8%), primary non-function (4/62; 6.5%), hepatic artery thrombosis (6/62; 9.7%) and portal vein thrombosis (1/62; 1.6%). Mortality rate was highest in organic acidaemia (7/11 or 63.6%) in the 1st 2 decades of this period. Mortality was higher in the first decade of our LT program, which improved in subsequent decades. There was 53.3% mortality from 1989 to 2000; 18.8% from 2001 to 2010; and 8.3% from 2011 to 2021. Cumulative 1-, 5-, and 10-year patient survival rates since 1989 were 78.1%,76.3%, 74.5%; graft survival rates were 69.3%,67.7% and 66.1%, respectively.
CONCLUSIONS: Although our overall patient and graft survival rates are lower than global reports, outcomes have improved in the last decade with careful patient and donor selection and changes in LT protocol.
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Date
2026-03-29
Type
Article
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Citation
Sreekantam V, Kurkal K, Santra S, Suresh V, Raiman J, Johansen L, Mckiernan PJ, Hutchin T, Sharif K, Kelgeri C, Gupte GL. Liver Transplantation for Inherited Metabolic Disorders: A Work in Progress. Acta Paediatr. 2026 Jul;115(7):1467-1473. doi: 10.1111/apa.70512. Epub 2026 Mar 29.