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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Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients. It develops due to excessive accumulation of glycogen in the hepatocytes.

It is typically reversible with good glycemic control and rarely progresses to mild fibrosis, but not cirrhosis. Hepatomegaly with or without elevated transaminases levels is a common condition in patients with uncontrolled type 1 diabetes mellitus DM.

Glycogenic hepatopathy GH is a rare disease that develops due to excessive accumulation of glycogen in the hepatocytes, leading to hepatomegaly and elevated transaminases levels in patients with uncontrolled type 1 DM. Although GH is well-described, it is still underdiagnosed. In this report, we describe the clinical, biochemical, and histopathological features in a Saudi girl with GH.

Her past history was unremarkable, with normal developmental history.

The family history was unremarkable for liver disease. She had hepatomegaly liver span 14 cmbut no stigmata of chronic liver disease. Examination of other systems was unremarkable. The initial test results were as follows: Liver synthetic function glycogenci, albumin, and coagulation profile and lipid profile were normal.

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

Thyroid stimulating hormone TSHceliac screen, ammonia, lactate, and metabolic screenings were all normal. Ultrasound of the glycogenjc showed hepatomegaly Biopsy showed preserved liver glycogeenic, but the hepatocytes exhibited marked cytoplasmic swelling and prominent plasma membranes. No inflammation or fibrosis was found. Genetic panel testing for glycogen storage diseases known mutations was negative.

Ultimately, a diagnosis of GH was made. Histopathological section of the liver showing: Hepatomegaly with or without raised transaminase levels is common in patients with DM.

Glycogen-induced hepatomegaly in type 1 DM was described first by Mauriac inas a component of a syndrome characterized by hepatomegaly, growth retardation, cushingoid appearance, and delayed puberty. Mauriac syndrome has become rare with the advent of better insulin regimens and improved multidisciplinary care.

The exact incidence and prevalence of the condition are not well known, as the condition is hepayopathy. The clinical symptoms and signs of GH are nonspecific, and include abdominal pain, hepatomegaly, and marked transaminases elevation resembling acute and relapsing hepatitis.


Liver synthetic hfpatopathy are typically preserved. The hepatic glycogen level is maintained by the balance between glycogenesis and glycogenolysis in the liver.

In diabetic patients with poor glycemic control, blood glucose and insulin levels often demonstrate significant fluctuations, with hypo- and hyperglycemic hepatopxthy. In the presence of hyperglycemia, glucose passively diffuses into hepatocytes insulin-independent mechanismand is subsequently converted to glucosephosphate, which is then converted to glycogen under the influence of insulin; these changes are exaggerated in the presence of hyperglycemia and over-insulinization, which are believed to glycoenic the primary mechanism in the development of GH.

Glycogenic hepatopathy: A narrative review

So far, liver biopsy is the only diagnostic tool that can differentiate between the 2 conditions. The clinical presentation of markedly elevated liver enzymes in the background of poorly controlled type 1 DM is highly suggestive of GH. Genetic testing, as performed in our case, can help to rule out GSD.

Liver biopsy is the gold standard test for GH diagnosis. The main hepatopatthy features of GH are marked glycogen accumulation, leading to pale, swollen hepatocytes, with typically minimal inflammation, steatosis, and fibrosis. There is still a need for larger-scale and long-term studies to explore the consequences of fibrosis over time. Some case reports described the utility of radiological imaging studies such as computed tomography CT scan and magnetic resonance imaging MRI in establishing the diagnosis of GH depending primarily on the interval changes in the liver density and its correlation with the clinical picture; however, further larger-scale studies are needed to confirm the findings from these reports, and to hepxtopathy their sensitivity and specificity for this condition.

Glycogenic hepatopathy is a benign condition that is potentially reversible within 2 to 14 weeks, both clinically and biochemically, with good glycemic control. In conclusion, Although it is a well-known complication of poorly controlled type 1 DM, GH is still an underdiagnosed cause of elevated transaminase levels and hepatomegaly in these patients.

Physicians should be aware of this condition and should consider it in the differential diagnosis of hepatomegaly with elevated liver enzymes during the workup of type 1 DM patients. Early diagnosis and subsequent hepatopatjy of good glycemic control will ultimately lead to an excellent prognosis.

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. The authors gratefully thank Dr. All figures or photographs should be submitted in a high resolution minimum DPI electronic version saved in jpeg or tiff format.


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Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

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National Center for Biotechnology InformationU. Journal List Saudi Med J v. Author information Article notes Copyright glycogwnic License information Disclaimer. Address correspondence and reprint request to: Received Jul 24; Accepted Oct This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. This article has been cited by other articles in PMC. Abstract Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients.

Open in a separate window. Glyycogenic Hepatomegaly with or without glycoenic transaminase levels is common in patients with DM.

Acknowledgment The authors gratefully thank Dr. Am J Surg Pathol. Grosventre, hepatomegalie, troble de la croissance chez les enfants diabetiques: Gaz Hebl Sci Med Bordeaux. Four cases of type 1 diabetes mellitus showing sharp serum transaminase increases and hepatomegaly due to glycogenic hepatopathy. 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.

Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus. World Hepatoathy of diabetes. Thinking Outside the Box.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

Hepatopathy of Mauriac syndrome: Sweetser S, Kraichely RE. The bright liver of glycogenic hepatopathy.

A case of glycogenic hepatopathy developed in a patient with new-onset fulminant type 1 diabetes: Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus. Support Center Support Center. Glycgoenic review our privacy policy.