Endocrine Abstracts

JOINT319

Type 1 diabetes mellitus (T1DM) in children is commonly associated with other autoimmune diseases, especially coeliac disease and autoimmune thyroiditis. Autoimmune hepatitis (AIH) is rare among patients with type 1 diabetes with a reported prevalence of 3.8%. However, none has been reported to present with acute liver failure with hyperglycaemia prior to diagnosis. We describe a case of 9-year-old girl developed icterus with acute liver failure and incidental finding of asymptomatic hyperglycaemia (lab glucose 20 mmol/l and ketone of 0.1 mmol/l) at presentation. Her initial laboratory investigations revealed hyperbilirubinaemia (179μmol/l) which consisted mainly of conjugated bilirubin (156μmol/l) with raised liver enzymes (aspartate aminotransferase (212IU/l), alanine aminotransaminase (248IU/l) and gamma-glutamyl transferase (132IU/l)). Furthermore, coagulation profile was found to be deranged with a prothrombin time of 34.2s and INR of 2.71, fulfilling the Paediatric Acute Liver Failure (PALF) study group definition of acute liver failure. Concurrently, her HbA1C was raised at 6.8% with positive T1DM autoantibodies for Anti Glutamic Acid Decarboxylase (>280,000IU/ml), Anti Islet Cell (75.31 IU/ml) and Anti Insulinoma Associated Antigen 2 (5.801IU/ml) confirming a diagnosis of T1DM. Interestingly, she has a normal random C-peptide (703pmol/l) and insulin (301pmol/l) at presentation prior to the initiation of insulin therapy. However, a repeated c-peptide showing a rapid drop to a low level after 6 weeks. Her subsequent investigations fulfilling the scoring list for autoimmune hepatitis issued by the International Autoimmune Hepatitis Group (IAIHG), leading to a definite diagnosis of AIH. She responded well to prednisolone (2mg/kg/day) and azathioprine, leading to a normalisation of her co-agulation profile. However, she required a daily insulin dose up to 2.8unit/kg/day to achieve a good glycemic control during her treatment period. This leads to an improvement of her HbA1C down to 5.8% after 6 weeks. Screening of other associated autoimmune diseases were negative (Normal thyroid function tests with negative thyroid peroxidase antibodies, anti-endomysium and anti-tissue transglutaminase antibodies) In summary, we have presented the case of a 9-year-old girl developed T1DM and AIH at the same time. Although patients with T1DM commonly present with polyosmolar symptoms or diabetic ketoacidosis at diagnosis, our case illustrate that severe AIH can occurs at presentation of T1DM in children. Therefore, there should be a high index of suspicion for other rare forms of autoimmunity in children with T1DM.