Hepatic glycogen storage diseases

NaUn T, et al. In vitro digestion of starches in a dynamic gastrointestinal model an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases. J Inherit Metab Dis. 2014. doi 10.107/s10545-014-9763-y. 

Amalfitano, A., Yie-Wylie, A. J., Hu, H., Dawson, T. L., Raben, N., Plotz, P. and Chen, Y. T. (1999). Systemic correction of the muscle disorder glycogen storage disease type II after hepatic targeting of a modified adenovirus vector encoding human acid-alpha-glucosidase. Proc. Natl. Acad. Sci. USA 96, 8861-8866. 

Lee, R, Mather, S., Owens, X, Leonard, X, Dicksmireaux, C. Hepatic ultrasound findings in the glycogen storage diseases. Brit. X Radiol. 1994 67 1062-1066... 

Hepatic glycogenoses Glycogen storage disease type I Glucose-6-phosphatase G6PC, SLC37A4 Von Gierke disease... 

Glycogen storage disease type VI Hepatic phosphoiylase PYGL ... 

The main long-term complications of glycogen storage disease type I are renal and hepatic complications. The kidney has elevated renal... 

Bao Y, et al. Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene. J Clin Invest. 1996 97(4) 941-8. 

McConMe-RoseU A, et al. Clinical and laboratory findings in four patients with the non-progressive hepatic form of type IV glycogen storage disease. J Inherit Metab Dis. 1996 19(1) 51-8. 

Attempts to Influence Hepatic Glucose-O-phosphatase in Glycogen Storage Disease. 121... 

Bianchi L (1993) Glycogen storage disease I and hepatocellular tumors. Eur J Pediatr 152 (Suppl 1) S63-S70 Bluteau O, Jeannot E, Bioulac-Sage P, et al (2002) Bi-allelic inactivation of TCFl in hepatic adenomas. Nat Genet 32 312-315... 

Weghuber, D., M. Mandl, M. Krssak, M. Roden, P. Nowotny, A. Brehm, M. Krebs, K. Widhalm and M.G. Bischof, 2007 Characterization of hepatic and brain metabolism in young adults with glycogen storage disease type 1 a magnetic resonance spectroscopy study.Am J Physiol Endocrinol Metab. 293, El378-384. 

Because the liver is a major processor of dietary and endogenous carbohydrates, liver disease affects carbohydrate metabolism in a variety of ways (see Chapter 25). However, none of the conventional modes of evaluating carbohydrate metabolism have value in the diagnosis of liver disease. Because the liver is the major site of both glycogen storage and gluconeogenesis, hypoglycemia is a common complication in certain liver diseases, particularly Reye s syndrome, fulminant hepatic failure, advanced cirrhosis, and hepatocellular carcinoma. 

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