Introduction: Development of type 1 diabetes is well known in cases of type 2 autoimmune polyglandular syndrome (APS). We describe a case of APS2 who developed a hybrid form of diabetes that responded to metformin therapy.
Case: The patient is a 17.5 year old male patient who has been diagnosed with APS2 at the age of 13 years with primary adrenal insufficiency and autoimmune hypothyroidism .he has been on treatment with prednisolone 5 mg once daily, fludrocortisone 0.1mg once daily and levothyroxine 125 mcg daily .
At the age of 17 years, he was noticed to have excessive weight gain (BMI = 28.5 kg/m^2 ) and acanthosis nigricans. He had no history of polyuria or polydipsia. investigation showed: HbA1c = 7.2%, fasting RBS of 7 mmol/l and the 2 hour OGTT =14.6mmol/l, c-peptide of 2 ng/ml(0.9 to 7),fastinginsulin level was 40 pmol/l (20-570),HOMA-IR 1.8, autoantibodies screen was positive for 3 antibodies (anti GAD65, anti-islet 2 ab and ant ZnT8 ab ).
A continuous glucose monitoring (CGM) was done with IPRO for 7 days showed postprandial hyperglycemic episodes .
Due to the presence of signs of insulin resistance, high HbA1c, absence of hypo-insulinemia, and self-correcting hyperglycemia, we elected to start metformin 1000 mg BID and closely observe the glycemic control. One month later, the CGM showed marked improvement in the glycemic control with a drop in the HbA1c to 6.2%. The patient continued to be asymptomatic and his BMI decreased to 25.5kg/m^2, and he reported decrease in appetite .
1. Discussion: Patients with APS-2 are characterized by at least two of the following three endocrinopathies: type 1 diabetes, autoimmune thyroid disease, and Addison's disease. The DR3-DQ2/DRB1*04:04-DQ8 genotype has been associated with type 1 diabetes in patients with APS 2. This is the first report that describes the occurrence of hybrid diabetes in a case of APS 2 that responded well to metformin therapy.
Conclusion: We reported a Hybrid form of diabetes that responded well to metformin therapy in an overweight adolecent with APS2
19 - 21 Sep 2019
European Society for Paediatric Endocrinology