Successful Liver Transplantation for Adolescent Patient With Pyruvate Kinase Deficiency-induced Cirrhosis.
J Pediatr Hematol Oncol
; 43(4): e605-e607, 2021 05 01.
Article
em En
| MEDLINE
| ID: mdl-32590423
ABSTRACT
We present the case of a successful liver transplant in a young adult patient with cholestasis and cirrhosis secondary to severe pyruvate kinase (PK) deficiency. Liver transplant resulted in resolution of liver dysfunction, decreased need for blood transfusions and eligibility for bone marrow transplantation. This case represents the third reported patient in the literature with severe PK deficiency who successfully underwent liver transplant as a result of profound cholestasis and liver failure. Explant pathology demonstrated a lack of significant iron deposition indicating that PK deficiency predisposes the liver to injury independent of transfusion-related iron overload.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Piruvato Quinase
/
Erros Inatos do Metabolismo dos Piruvatos
/
Transplante de Fígado
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Anemia Hemolítica Congênita não Esferocítica
/
Cirrose Hepática
Tipo de estudo:
Etiology_studies
Limite:
Adolescent
/
Female
/
Humans
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Ano de publicação:
2021
Tipo de documento:
Article