摘要
Hepatic glycogenosis(HG) is characterized by excessive glycogen accumulation in hepatocytes and represents a hepatic complication of diabetes that particularly occurs in patients with longstanding poorly controlled type 1 diabetes(T1D). HG has been reported to be a very rare disease, although it is believed to be extremely underdiagnosed because it is not possible to distinguish it from non-alcoholic fatty liver disease(NAFLD) unless a liver biopsy is performed. In contrast to HG, NAFLD is characterized by liver fat accumulation and is the more likely diagnosis for patients with type 2 diabetes and metabolic syndrome. The pathogenesis of HG involves the concomitant presence of insulin and excess glucose, which increases glycogen storage in the liver. HG is characterized by a transient elevation in liver transaminases and hepatomegaly. Differentiating between these two conditions is of the utmost importance because HG is a benign disease that is potentially reversible by improving glycemic control, whereas NAFLD can progress to cirrhosis. Therefore, HG should be suspected when liver dysfunction occurs in patients with poorly controlled T1 D. The aim of this article is to review the epidemiology, clinical characteristics, pathogenesis and histology of HG.
Hepatic glycogenosis （HG） is characterized by excessiveglycogen accumulation in hepatocytes and representsa hepatic complication of diabetes that particularlyoccurs in patients with longstanding poorly controlledtype 1 diabetes （T1D）. HG has been reported tobe a very rare disease, although it is believed to beextremely underdiagnosed because it is not possibleto distinguish it from non-alcoholic fatty liver disease（NAFLD） unless a liver biopsy is performed. In contrastto HG, NAFLD is characterized by liver fat accumulationand is the more likely diagnosis for patients with type2 diabetes and metabolic syndrome. The pathogenesisof HG involves the concomitant presence of insulin andexcess glucose, which increases glycogen storage inthe liver. HG is characterized by a transient elevation inliver transaminases and hepatomegaly. Differentiatingbetween these two conditions is of the utmost importancebecause HG is a benign disease that is potentiallyreversible by improving glycemic control, whereas NAFLDcan progress to cirrhosis. Therefore, HG should besuspected when liver dysfunction occurs in patients withpoorly controlled T1D. The aim of this article is to reviewthe epidemiology, clinical characteristics, pathogenesisand histology of HG.
