Liver transplant for inherited metabolic disease among siblings.
clinical decision-making
genetics
liver disease
metabolic
recipient selection
Journal
Clinical transplantation
ISSN: 1399-0012
Titre abrégé: Clin Transplant
Pays: Denmark
ID NLM: 8710240
Informations de publication
Date de publication:
11 2020
11 2020
Historique:
received:
01
06
2020
revised:
26
08
2020
accepted:
30
08
2020
pubmed:
22
9
2020
medline:
24
6
2021
entrez:
21
9
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32955727
doi: 10.1111/ctr.14090
pmc: PMC10064292
mid: NIHMS1880294
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e14090Subventions
Organisme : NIDDK NIH HHS
ID : P30 DK120531
Pays : United States
Informations de copyright
© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Références
Exp Clin Transplant. 2015 Nov;13 Suppl 3:101-6
pubmed: 26640926
Am J Transplant. 2007 Aug;7(8):1974-83
pubmed: 17617862
Transpl Int. 2008 Feb;21(2):189-91
pubmed: 17949404
Pediatr Transplant. 2014 Dec;18(8):851-9
pubmed: 25307019
World J Hepatol. 2019 May 27;11(5):450-463
pubmed: 31183005
Transplantation. 2005 Sep 15;80(5):623-8
pubmed: 16177636
Pediatr Transplant. 2011 May;15(3):281-4
pubmed: 21235710
N Engl J Med. 1991 Jan 3;324(1):39-42
pubmed: 1984162
Pediatr Transplant. 2010 Sep 1;14(6):796-805
pubmed: 20557477
Transpl Int. 2000;13 Suppl 1:S171-3
pubmed: 11111990
J Pediatr Gastroenterol Nutr. 2006 Jul;43(1):136-8
pubmed: 16819392
Transplantation. 2002 Jan 15;73(1):67-9
pubmed: 11792980
Transplantation. 2014 Sep 27;98(6):e61-3
pubmed: 25221904
Liver Transpl. 2019 Jun;25(6):911-921
pubmed: 30753750