Fifteen years of follow-up of a liver transplant recipient with glycogen storage disease type Ia (Von Gierke disease).
Transplant Proc
; 45(10): 3668-9, 2013.
Article
em En
| MEDLINE
| ID: mdl-24314991
ABSTRACT
Von Gierke's disease or glycogen storage disease type Ia (GSD-Ia) is an infrequent metabolic disease caused by an atypical accumulation of glycogen. The principal cause of this pathology is deficiency of the glucose-6-phosphatase enzyme. Herein we have reported a case of a young man with a history of Von Gierke's disease (GSD-Ia) since childhood who developed hepatocellular adenomatosis brought to light by ultrasounds and TACs. The patient began to develop early chronic renal failure, necessitating simultaneous liver and kidney transplantation. Years later continuous reviews at the nephrology and hepatobiliopancreatic surgery services show he has a good quality of life and a normal hepatorenal profile.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença de Depósito de Glicogênio Tipo I
/
Transplante de Fígado
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Adenoma de Células Hepáticas
/
Neoplasias Hepáticas
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article