Evolving Trends in Liver Transplant for Metabolic Liver Disease in the United States

Liver Transplantation - Tập 25 Số 6 - Trang 911-921 - 2019
Patrick McKiernan1,2, Armando Ganoza3, James E. Squires1,2, Robert H. Squires1,2, Jerry Vockley4, George Mazariegos3, Kyle Soltys3, Qing Sun3, Rakesh Sindhi3
1Division of Pediatric Gastroenterology, Hepatology and Nutrition,University of Pittsburgh,Pittsburgh,PA
2Pittsburgh Liver Research Center,University of Pittsburgh,Pittsburgh,PA
3Hillman Center for Pediatric Transplantation,University of Pittsburgh,Pittsburgh,PA
4Division of Medical Genetics,Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, University of Pittsburgh,Pittsburgh,PA

Tóm tắt

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 the following: temporal changes in indications, longterm outcomes, and factors predicting survival. Patients were grouped by the presence of structural liver disease (SLD) and whether the defect was confined to the liver. There were 5996 patients who underwent LT for metabolic disease, 2354 (39.3%) being children. LT for metabolic disease increased in children but not in adults. Children experienced a 6‐fold increase in LT for metabolic disease without SLD. Indications for LT remained stable in adults. Living donor liver transplantation increased between era 1 and era 3 from 5.6% to 7.6% in children and 0% to 4.5% in adults. Patient and graft survival improved with time. The latest 5‐year patient survival rates 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), whereas SLD did not affect outcomes. 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 outcomes in all ages with male sex predicting survival in childhood only. Children without structural disease were less likely to die awaiting LT and had improved post‐LT survival compared with children with chronic liver disease. In conclusion, LT for metabolic disease is increasingly used for phenotypic correction in children; extrahepatic manifestations significantly impact survival at all ages; where indicated, transplantation should not be unnecessarily delayed; and the development of new allocation models may be required.

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Tài liệu tham khảo

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Liver Transplantation

Tập 25 Số 6

911-921

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