Hepatobiliary and Pancreatic: Glycogenic hepatopathy: A reversible condition

Glycogenic hepatopathy: A reversible conditionj gh_6178 646Glycogenic hepatopathy is an under recognised condition, described as a pathological overloading of hepatocytes with glycogen in patients with poorly controlled type 1 diabetes mellitus.Clinical presentations can include abdominal pain, tender hepatomegaly, nausea and elevated transaminases.We report a case of a 33 year old woman, with poorly controlled type 1 diabetes mellitus (HbA1c 13.7%) who was referred for evaluation of diarrhoea and abnormal liver enzymes, to highlight the diagnostic challenges of glycogenic hepatopathy.Physical examination revealed a diffusely tender abdomen.Liver enzymes were significantly elevated at ALP 205 U/L, GGT 88 U/L, AST 428 U/L and ALT 404 U/L.Bilirubin and liver synthetic function were normal, and screening for other causes of liver disease was negative.Ultrasound examination suggested fatty infiltration of the liver.The degree of liver enzyme elevation led to a liver biopsy.The biopsy showed enlarged, swollen hepatocytes with no evidence of steatosis, inflammation, fibrosis or necrosis (Figure 1).Mallory hyaline bodies were not seen.The enlarged hepatocytes showed intense cytoplasmic staining with Periodic Acid-Schiff stain, and negative staining with Periodic Acid-Schiff Diastase.This is suggestive of glycogen accumulation (Figure 2), and consistent with glycogenic hepatopathy.At 12 month follow-up, the patient had achieved significant improvement in glycaemic control (HbA1c 9.3%), with normalisation of liver enzymes.Fibroscan (non-invasive method of measuring liver elastography), was performed on our patient.A mean reading of 5.3 Kpa was found, suggesting early fibrosis.