https://www.ncbi.nlm.nih.gov/pubmed/30753750 Liver transplant

Liver Transpl. 2019 Feb 12. doi: 10.1002/lt.25433. [Epub ahead of print]
Evolving trends in liver transplant for metabolic liver disease in the United States.
McKiernan PJ1,2, Ganoza A3, Squires JE1,2, Squires RH1,2, Vockley J4, Mazariegos G3, Soltys K3, Sun Q3, Sindhi R3.
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Abstract

RATIONALE:
Indications for liver transplantation (LT) in metabolic disease are evolving. We reviewed the US experience with primary LT for metabolic disease in the Scientific Registry for Transplant Recipients (October 1987 to June 2017) to determine: 1. Temporal changes in indications, 2. long-term outcomes, and 3. factors predicting survival.

METHOD:
Subjects were grouped by the presence of structural liver disease and whether the defect was confined to the liver.

RESULTS:
5996 underwent LT metabolic disease, 2354 [39.3%] being children. LT for metabolic disease increased in children but not in adults. Children experienced a six-fold increase in LT for metabolic disease without structural liverdisease. Indications for LT remained stable in adults. Living donor LT increased with eras from 5.6 to 7.6% in children, and 0 to 4.5% in adults. Patient and graft survival improved with time. Latest 5-year patient survival were 94.5 and 81.5% in children and adults respectively. Outcomes were worse in adults and in those with extrahepatic disease, (p<0.01) while structural liver disease did not affect outcome. Survival improved with younger age at LT until age <2 years. On multivariate analysis; diagnostic category, inpatient status, age at LT and transplant era significantly predicted outcome in all ages, with male gender predicting survival in childhood only. Children without structural disease were less likely to die awaiting LT and had improved post LT survival compared to children with chronic liverdisease.

CONCLUSIONS:
Liver transplantation for metabolic disease: 1. is increasingly utilized for phenotypic correction in children. 2. Extrahepatic manifestations significantly impact survival at all ages. 3. Where indicated should not be unnecessarily delayed. 4. May require the development of new allocation models.

Published on: 
Feb-2019

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