Hepatic glycogen storage disorders: what have we learned in recent years?

• Published in: Current opinion in clinical nutrition and metabolic care Vol. 18; no. 4; p. 415
• Main Authors: Burda, Patricie, Hochuli, Michel
• Format: Journal Article
• Language: English
• Published: England 01.07.2015
• Subjects: Animals; Cardiomyopathies - complications; Cardiomyopathies - diet therapy; Cardiomyopathies - physiopathology; Diet, Carbohydrate-Restricted; Diet, High-Fat; Dietary Carbohydrates - administration & dosage; Dietary Fats - administration & dosage; Dietary Proteins - administration & dosage; Disease Models, Animal; Glycogen - metabolism; Glycogen Storage Disease Type I - complications; Glycogen Storage Disease Type I - diagnosis; Glycogen Storage Disease Type I - diet therapy; Glycogen Storage Disease Type I - physiopathology; Glycogen Storage Disease Type III - complications; Glycogen Storage Disease Type III - diagnosis; Glycogen Storage Disease Type III - diet therapy; Glycogen Storage Disease Type III - physiopathology; Glycogen Storage Disease Type VI - complications; Glycogen Storage Disease Type VI - diagnosis; Glycogen Storage Disease Type VI - diet therapy; Glycogen Storage Disease Type VI - physiopathology; Humans; Liver - physiopathology; Liver Cirrhosis - complications; Liver Cirrhosis - diet therapy; Liver Cirrhosis - physiopathology

Glycogen storage disorders (GSDs) are inborn errors of metabolism with abnormal storage or utilization of glycogen. The present review focuses on recent advances in hepatic GSD types I, III and VI/IX, with emphasis on clinical aspects and treatment. Evidence accumulates that poor metabolic control i...