Glycogen hepatopathy in children with poorly controlled type 1 diabetes

Introduction: Glycogen hepatopathy has been described in children with poorly controlled type 1 diabetes mellitus (T1DM). Diabetic patients with hepatomegaly and abnormal liver enzymes have often been assumed to have nonalcoholic steatohepatitis rather than glycogen hepatopathy. It is important to recognize and distinguish glycogen hepatopathy from nonalcoholic steatohepatitis as it impacts the management and long-term prognosis. Case Report: We describe four cases of children with poorly controlled type 1 diabetes mellitus who developed glycogen hepatopathy, and review the available literature. Conclusion: Pediatricians should raise awareness of this potentially reversible liver complication in children with poorly controlled T1DM. It has been suggested that diabetic patients with hepatomegaly and elevated liver enzymes be given a trial of improved glycemic control prior to more invasive investigation.