Glycogenic Hepatopathy in Type 1 Diabetes: An Underrecognized Condition

The combination of poorly controlled diabetes, acute liver injury with marked elevation in serum aminotransferases, and the characteristic histological changes on liver biopsy are diagnostic of glycogenic hepatopathy. A similar condition was described by Mauriac in 1930, characterized by growth retardation, hepatomegaly, Cushingoid features, and delayed puberty (1). A 19-year-old type 1 diabetic female with poor glycemic control, complicated by recurrent diabetic ketoacidosis (DKA) was admitted in August 2011 with symptoms of feeling generally unwell, abdominal pain, vomiting, and breathlessness. Her glycemic control had been suboptimal for several years (HbA1c [NGSP] 14.6%, [International Federation of Clinical Chemistry and Laboratory Medicine] 136 mmol/mol in July 2010). She was treated for DKA and had tender hepatomegaly. Investigations revealed abnormal liver function tests including γ-glutamyl transferase 317 (normal range [NR] 1–42 units/L), alanine aminotransferase 199 (NR …