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World J Diabetes. Mar 15, 2015; 6(2): 321-325
Published online Mar 15, 2015. doi: 10.4239/wjd.v6.i2.321
Hepatic glycogenosis: An underdiagnosed complication of diabetes mellitus?
María Teresa Julián, Núria Alonso, Isabel Ojanguren, Eduarda Pizarro, Enric Ballestar, Manel Puig-Domingo
María Teresa Julián, Núria Alonso, Manel Puig-Domingo, Department of Medicine, Universitat Autònoma de Barcelona, 08035 Barcelona, Spain
María Teresa Julián, Eduarda Pizarro, Enric Ballestar, Department of Endocrinology, Hospital de Mataró, 08034 Mataró, Barcelona, Spain
Núria Alonso, Manel Puig-Domingo, Department of Endocrinology, Hospital Germans Trias i Pujol, 08916 Badalona, Barcelona, Spain
Isabel Ojanguren, Department of Pathology, Hospital Germans Trias i Pujol, 08916 Badalona, Barcelona, Spain
Author contributions: Julián MT conceived the idea for the review, designed and wrote the manuscript; Alonso N and Puig-Domingo M provided expert opinions and reviewed the paper; all the authors contributed to the final preparation of the manuscript.
Conflict-of-interest: The authors declare that the present review was written in the absence of any comercial or financial relationships that could be construed as a potential conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: María Teresa Julián, MD, Department of Endocrinology, Hospital de Mataró, Carrer Prolongació Cirera s/n, 08034 Mataró, Barcelona, Spain. mjulian@csdm.cat
Telephone: +34-93-7417700 Fax: +34-93-741733
Received: August 18, 2014
Peer-review started: August 19, 2014
First decision: September 16, 2014
Revised: October 15, 2014
Accepted: December 18, 2014
Article in press: December 19, 2014
Published online: March 15, 2015
Abstract

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 T1D. The aim of this article is to review the epidemiology, clinical characteristics, pathogenesis and histology of HG.

Keywords: Hepatic complications, Diabetes mellitus, Type 1 diabetes, Hepatic glycogenosis, Non-alcoholic fatty liver disease

Core tip: Hepatic glycogenosis (HG) is a complication of diabetes mellitus that is often underdiagnosed. It is defined as pathological glycogen storage in hepatocytes with hepatomegaly and elevated liver enzymes and mainly occurs in patients with longstanding poorly controlled type 1 diabetes. HG cannot easily be distinguished from non-alcoholic fatty liver disease (NAFLD) by history, physical examination or ultrasound; only liver biopsy can provide a definitive diagnosis. The hallmark of this condition is its reversibility with improved glycemic control; in contrast, NAFLD can progress to fibrosis.