ESPE Abstracts

Introduction: Glycogenic hepatopathy (GH) is a rare diabetic complication that induces hepatomegaly and elevated transaminases, which can often appear in type 1 diabetes mellitus (T1DM) with poor glycemic control. GH appears when excess glycogen accumulates in the liver through continued hyperglycemia with intensive insulin therapy. We show a pediatric case of recurrent GH due to poor glucose controlled with complicated family environment and mildly intellectual disability.

Case report: The patient was diagnosed with T1DM due to blood glucose of 516 mg/dl, HbA1c 8.6%, and an anti-insulinoma associated protein-2 antibody 0.9 U/l at six years of age, then intensive insulin therapy was started. Blood glucose was well controlled in the first one year, however, blood glucose was worsened due to complicated family environment. HbA1c worsened from 6.7 to 12.0 % at nine years of age after the end of the honeymoon period. At 10 years of age, AST/ALT deteriorated and elevated to 448/259 U/l in a few months. There were no abnormal coagulation and fibrinolytic system, viral hepatitis, autoimmune hepatitis, or biliary tract diseases. We performed abdominal ultrasonography, CT and MRI, then fatty liver and GH were suspected. Liver biopsy was not performed due to the same treatment approach for fatty liver and GH consulted by hepatologists. AST/ALT improved rapidly with strict glycemic control during hospitalization at 12 days. We recommended continuous subcutaneous insulin infusion (CSII) but declined. He was diagnosed with GH. At 10 years of age, poor glycemic control was observed (HbA1c; 10.1 to 12.4%), and again AST/ALT deteriorated and elevated to 298/146 U/L. GH had repeated and he was re-treated in hospital. The patient and family were persuaded to change the treatment modality from intensive insulin therapy to CSII. Blood glucose was well controlled (HbA1c 8.4%), which was used by CSII, and AST/ALT also improved to 28/22 U/L. HbA1c remained at 8.5-10.0%, and GH recurrence did not occur. WISC-IV was performed at 10 years of age and FIQ was 60 (mildly intellectual disability), which would have made intensive insulin therapy difficult.

Conclusion: There was recurrent GH due to familial environment and mildly intellectual disability, but CSII allowed improved glycemic control.