Liver Transplantation for Zellweger Syndrome.
Cirrhosis
Liver transplantation
Proptosis
Zellweger syndrome
Journal
Indian journal of pediatrics
ISSN: 0973-7693
Titre abrégé: Indian J Pediatr
Pays: India
ID NLM: 0417442
Informations de publication
Date de publication:
20 Dec 2023
20 Dec 2023
Historique:
received:
04
07
2023
accepted:
06
11
2023
medline:
20
12
2023
pubmed:
20
12
2023
entrez:
20
12
2023
Statut:
aheadofprint
Résumé
Zellweger syndrome or cerebrohepatorenal syndrome is a rare, multisystem disorder occurring due to defect in metabolic pathway within the peroxisomes. Cirrhosis with portal hypertension is an important presentation of these patients. Given its progressive, multisystem nature, the role of liver transplantation (LT) in Zellweger syndrome remains undefined and controversial. An 11-y-old boy diagnosed with Zellweger syndrome presented to the authors with decompensated cirrhosis along with bilateral proptosis. After a meticulous evaluation, he was offered an ABO incompatible liver transplantation with his mother being the donor. He had an uneventful post operative period. After a follow up of 24 mo, he has normal graft function, normal cognition along with resolution of proptosis. Therefore, in a group of carefully selected patients with Zellweger syndrome, a liver transplantation can be offered successfully with an excellent prognosis.
Identifiants
pubmed: 38117438
doi: 10.1007/s12098-023-04937-7
pii: 10.1007/s12098-023-04937-7
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.
Références
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