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Liver transplant for inherited metabolic disease among siblings
Author(s) -
McKiernan Patrick J.,
Squires James E.,
Squires Robert H.,
Vockley Jerry,
Mazariegos George V.,
Soltys Kyle,
Ganoza Armando,
Strauss Kevin,
Khanna Ajai,
Sindhi Rakesh
Publication year - 2020
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/ctr.14090
Subject(s) - medicine , disease , liver transplantation , transplantation
Liver transplantation is a successful option for inherited metabolic disease yet little is published on the outcome among siblings. We report outcomes of siblings who have undergone liver transplantation for metabolic disease in a single program. Seventy‐one siblings (35 males) from 33 individual families underwent liver transplantation since 1982. Outcomes were compared over three consecutive eras. Twenty‐eight families had two siblings, four had three siblings, and one had four siblings. In half of families where dates of listing were known, siblings were listed simultaneously. Mean (SD) age at listing for the oldest and second sibling was 13.2 (7.1) and 9.8 (5.7) years, respectively ( p < .01). In 18/33 families, the oldest sibling underwent transplantation first. Mean (SD) age at transplant fell from the oldest to second sibling from 12.9 (7.2) to 9.5 (6.3) years, respectively ( p < .001). Ten‐year patient survival was 83.5% which improved over the eras: era 1 (1982‐1994) 65.0%, era 2 (1995‐2007) 87.5%, and era 3 (2008–2019) 93.8%: p < .03. Sex, age at transplant, order of transplant, and presence of structural liver disease did not significantly impact survival. When siblings undergo liver transplant for inherited metabolic disease, later siblings are listed and transplanted at a significantly younger age.