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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Liver synthetic functions are typically preserved. Control liver biopsy was not performed in this case after laboratory and radiological improvements; therefore, histologic remission was not valuated.

Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

Hormones like adrenaline, cortisol, or growth hormones released due to hypoglycemia, could synergistically act and release large quantities of non-esterified fatty acids from adipose tissue.

If arrows are used please ensure they appear in a different color to the background color, preferably black with a white border, or hepagopathy with a black border. Her physical exam and symptoms of sharp abdominal pain prompted further evaluation.

Inborn errors of glucose and glycogen metabolism results in abnormal deposition of glycogen.

Unusual glycogenic hepatopathy causing abnormal liver enzymes in a morbidly obese adolescent with well-controlled type 2 diabetes: In a study by Iancu et al[ 58 ] patients helatopathy received steroid therapy: Thirty-eight percent of reported cases are male patients, with most cases occurring in adolescence.

Larger-scale and long-term studies are still needed to clarify the incidence and prevalence of the condition, and to tests the utility of noninvasive diagnostic modalities such as MRI in the diagnosis of this condition. However, the best modality to accurately diagnose a GSD is next-generation genetic sequencing.


Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acid-Schiff, and periodic acid-Schiff with diastase. Ultrastructural analysis was performed in 2 cases. Ultimately, a diagnosis of GH was made.

This term has been used universally since then to glycogwnic this unique pathologic feature[ 44 ]. Thereupon, a liver biopsy was performed.

Resnick et al[ 56 ] reported a case of a toddler fed via gastrostomy tube, who developed GH secondary to Dumping syndrome after Nissen fundoplication. ALP, Total bilirubin may be elevated.

Hepatic glycogen storage disorders: Future progress is required in understanding the biochemical defects underlying GH and development of fibrosis. The collection of glycogen seen on a liver biopsy is critical for the diagnosis.

Several authors have also demonstrated elevated hepatic glycogen concentration in subjects with glycogen storage disease using glycgenic MRS Magnetic Resonance Spectroscopywhich has the advantage of assessing the entire liver while avoiding the risks of liver biopsy[ 61 ].

Case Reports in Hepatology

Glycogenic hepatopathy in type 1 diabetes: Her symptoms resolved with minimal alteration to her insulin regimen and only slightly improved glucose control. Histopathological section of the liver showing: The pathogenesis of growth retardation in those initial cases reported in children was thought to be multifactorial[ 52 ]. Future studies warranted to further assess for development of fibrosis in GH as it can progress to cirrhosis. The pathophysiology of Dumping syndrome can resemble that of GH seen in people with diabetes, eventually causing the hepatic accumulation of glycogen.


Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

As such, exogenous insulin therapy is necessary to maintain their serum glucose levels. GH develops due to excessive and irreversible accumulation of glycogen in the hepatocytes and causes liver function disorders and hepatomegaly [ 34 ]. Both her parents were immunized for hepatitis and there was no family history of liver, gastrointestinal, or autoimmune diseases.

Work up for infectious causes hepatitis B virus, hepatitis C virus, cytomegalovirus, and Epstein-Barr virus were negative.

Summary of the major studies in English pub med indexed on Glycogenic hepatopathy in type 1 diabetes mellitus.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

This case gllycogenic GH in a type I diabetic diagnosed via the characteristic pathology and treated successfully despite only modest improvements in glucose control. In some patients with glycogenix controlled diabetes and mild abnormalities in liver function tests, it is reasonable to defer biopsy until following a trial of improved glycemic control. It is typically reversible with good glycemic control and rarely progresses to mild fibrosis, but not cirrhosis.

Mild steatosis, mostly macrovesicular, was present. Several laboratory tests may be done initially to rule out other causes of hepatomegaly and chronic hepatitis.