Successful Liver Transplantation for Adolescent Patient With Pyruvate Kinase Deficiency-induced Cirrhosis.
J Pediatr Hematol Oncol
; 43(4): e605-e607, 2021 05 01.
Article
en 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
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Piruvato Quinasa
/
Errores Innatos del Metabolismo del Piruvato
/
Trasplante de Hígado
/
Anemia Hemolítica Congénita no Esferocítica
/
Cirrosis Hepática
Tipo de estudio:
Etiology_studies
Límite:
Adolescent
/
Female
/
Humans
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2021
Tipo del documento:
Article