title: Fifteen years of follow-up of a liver transplant recipient with glycogen storage disease type Ia (Von Gierke disease).
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.
authors: General Surgery and Digestive, Hepatobiliopancreatic Surgery, Virgen del Rocio University Hospitals, Seville, Spain. Electronic address: antoniocarlosmaya@hotmail.com.
authors: A C
authors: AC
authors: Maya Aparicio
authors: C
authors: C
authors: Bernal Bellido
authors: J
authors: J
authors: Tinoco González
authors: S
authors: S
authors: Garcia Ruíz
authors: L
authors: L
authors: Aguilar Romero
authors: L M
authors: LM
authors: Marín Gómez
authors: G
authors: G
authors: Suárez Artacho
authors: J M
authors: JM
authors: Alamo Martínez
authors: J
authors: J
authors: Serrano Díez-Canedo
authors: F J
authors: FJ
authors: Padillo Ruíz
authors: M A
authors: MA
authors: Gomez Bravo
mesh_terms: D018248:Adenoma, Liver Cell / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D000328:Adult; D018450:Disease Progression; D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D008113:Liver Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D011788:Quality of Life; D013997:Time Factors; D016896:Treatment Outcome