Glycogenic hepatopathy (GH) is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. Periodic acid Schiff stain for glycogen was positive in these hepatocytes. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. We describe a presentation of glycogenic hepatopathy in a poorly controlled type I diabetic patient. As patients with glycogenic hepatopathy.

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Case Reports in Hepatology

ALP, Total bilirubin may be elevated. Control liver biopsy was not performed in this case after laboratory and radiological improvements; therefore, histologic remission was not valuated.

Glucosephosphate is converted to glycogen by the enzyme glycogen synthase, which exists in an active dephosphorylated form and in an inactive phosphorylated form. Patients with GH present with nonspecific complaints, which often delays diagnosis.

Lifestyle modification and pharmacologic therapies. Examination of other systems was unremarkable. Rapid onset of glycogen storage hepatomegaly in a type-2 diabetic patient after a massive dose of long-acting insulin and large doses of glucose. Kransdorf et al[ glycogeenic ], This term has been used universally since then to describe this unique pathologic feature[ 44 ]. The author recommended DILI as a possibility whenever GH is suspected with concomitant use of hepatotoxic medications.

Medicine Baltimore ; Case Rep Crit Care.

Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

Cuthbertson et al[ 6 ], Am J Surg Pathol. Adequate glycemic control can result in complete remission of clinical, laboratory and histological abnormalities. Microscopically, the biopsy demonstrated pale hepatocytes with diffuse hepatocyte ballooning. This high concentration of free fatty acids inhibits glucose oxidation in muscles, and they may have similar effects on liver promoting excess storage of glycogen[ 2 ].

Case A year-old male patient was admitted to the emergency department due to loss of appetite and nausea complaints that continued for two days. Therefore, lactic acidosis could be secondary to reduced gluconeogenesis and a lack of conversion of pyruvate to glucose[ 35 ].


Unfortunately, our patient never returned for additional follow-up. Isr Med Assoc J. Dantuluri et al[ 16 ], These patients may already start to present with hypoglycemia and hepatomegaly during infancy[ 60 ]. Elevations in liver enzymes were thought to be due to enzyme hepwtopathy from hepatocyte membrane injury, not cell death.

However, the best modality to accurately diagnose a GSD is next-generation genetic sequencing. Excessive insulin levels associated with exogenous insulin administration augments the glycogen deposition. One of the inherent functions of insulin is to stimulate glycogenesis. The hepatocytes in hepatopathh conditions are markedly swollen and filled with glycogen, while the subtle difference can be the presence of higher cytoplasmic clumping of glycogen in GSD[ 44 ].

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

Although it was first observed in the pediatric population, since hepaotpathy, it has been reported hepatlpathy adolescents and adults with or without ketoacidosis. The prevalence of elevated liver enzymes has increased among diabetic patients. Glycogenic hepatopathy is a benign condition that is potentially reversible within 2 to 14 weeks, both clinically and biochemically, with good glycemic control. This article has been cited by other articles in PMC.

Tomihira and colleagues investigated the gene structure of phosphorylase enzyme to define possible mutations; however, they could not determine a probable gene defect that might cause hepatic glycogenosis[ 53 ].

Because there is an association between T1DM and autoimmune hepatitis, testing for autoimmune antibodies, such as ANA, anti-smooth muscle antibody and antimitochondrial antibody is essential. Patients with GH could have elevated levels of plasma lactate, with or without the presence of a DKA episode, although the mechanism is still poorly understood[ 3545 hepatopatjy.

Here we briefly reviewed the English literature on pathogenesis involved, recent progress in the evaluation, differential diagnosis, and management. A total of fifty-one studies involving adolescents or adults were uepatopathy for this review. Hepaotpathy transaminases mild to moderate and ALP may be markedly subnormal US, CT, may show signs of cirrhosis and normal caudate lobe which is contrary to other types cirrhosis Vary largely from fatty changes to cirrhosis and occasionally fulminant hepatic necrosis.


Alkaline phosphatase; T bili: High glucose levels cause an influx of glucose into the hepatocytes via facilitated diffusion through the glucose transporter 2 GLUT2independent of insulin[ 43 ]. Published online Feb In a retrospective review by Fitzpatrick of 31 patients with Mauriac syndrome, almost half the patients had elevated lactic acid level despite no signs of DKA[ 45 ]. Though management is aimed at better glycemic control, the degree of improvement needed is unclear. He became hypoglycemic and was later administered gycogenic large dose of intravenous glucose to counteract the persistent hypoglycemia.

Biochemical features Most patients with GH present glycigenic hepatocellular abnormality with a predominant elevation in aspartate transaminase AST and alanine transaminase ALT levels, although a mixed or predominantly cholestatic pattern can rarely occur[ 311203044 ].

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Future progress is required in understanding the biochemical defects underlying GH and development of fibrosis. Biochemical tests including genetic testing, radiologic, and liver biopsy. Original hard copies of all figures may be requested when necessary.

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. However, prior to biopsy, viral, autoimmune, and underlying metabolic liver disease should be excluded via corresponding laboratory investigations.

The clinical presentation of markedly elevated liver enzymes in the background of poorly controlled type 1 DM is highly suggestive of GH. Liver glyckgenic may show extensive, dense, perisinusoidal fibrosis, and immunostaining reveals basement membrane components in a perisinusoidally distribution[ 62 ].

Awareness of this entity glyycogenic the medical community including specialists is low. The cardiovascular and respiratory system examination results were normal.

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