Glycogenic hepatopathy

Glycogenic hepatopathy is a rare and underdiagnosed complication of Type 1 diabetes, characterized by the excessive accumulation of glycogen in the hepatocytes. It was first described in 1930 by Mauriac as a component of Mauriac syndrome, a rare pediatric syndrome associated with poorly controlled type 1 diabetes.

Etiology

The exact cause of glycogenic hepatopathy is not well understood. It is believed to be related to poor glycemic control in patients with type 1 diabetes. The condition is thought to result from the rapid fluctuation of blood glucose levels, leading to excessive glycogen storage in the hepatocytes.

Clinical Presentation

Patients with glycogenic hepatopathy typically present with hepatomegaly, elevated liver enzymes, and poorly controlled diabetes. The condition is often mistaken for nonalcoholic fatty liver disease (NAFLD), as the clinical presentation and laboratory findings can be similar. However, unlike NAFLD, glycogenic hepatopathy is reversible with improved glycemic control.

Diagnosis

The diagnosis of glycogenic hepatopathy is typically made through a combination of clinical history, laboratory findings, and liver biopsy. The liver biopsy will show swollen hepatocytes filled with glycogen, without the inflammation or fibrosis typically seen in NAFLD.

Treatment

The primary treatment for glycogenic hepatopathy is improved glycemic control. This can be achieved through a combination of diet, exercise, and medication. In severe cases, insulin therapy may be required. With improved glycemic control, the hepatomegaly and liver enzyme elevations typically resolve within weeks to months.

Prognosis

The prognosis for glycogenic hepatopathy is generally good, with most patients experiencing complete resolution of symptoms with improved glycemic control. However, if left untreated, the condition can lead to severe liver damage and potentially liver failure.

See Also

• Type 1 diabetes
• Mauriac syndrome
• Nonalcoholic fatty liver disease

Glycogenic hepatopathy Resources
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