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Transplant Proc ; 45(10): 3668-9, 2013.
Article in English | 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.


Subject(s)
Adenoma, Liver Cell/surgery , Glycogen Storage Disease Type I/surgery , Liver Neoplasms/surgery , Liver Transplantation , Adenoma, Liver Cell/diagnosis , Adenoma, Liver Cell/etiology , Adult , Disease Progression , Glycogen Storage Disease Type I/complications , Glycogen Storage Disease Type I/diagnosis , Humans , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/surgery , Kidney Transplantation , Liver Neoplasms/diagnosis , Liver Neoplasms/etiology , Male , Quality of Life , Time Factors , Treatment Outcome
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