Like-Triple Diabetes as First Manifestation of MODY2 in an Overweight Teenager With Transient Multiple Antibodies

Patients with heterozygous inactivating mutations in the gene encoding glucokinase, which causes type 2 maturity-onset diabetes of the young (MODY2), have mild fasting hyperglycemia that usually remains considerably stable during life and requires only diabetic diet as treatment. Atypical course of disease is uncommon.An 11-year-old, prepubertal, Caucasian overweight boy (BMI 22.5 kg/m2) with gynecomastia/steatomastia and diabetes was diagnosed on the basis of an oral glucose tolerance test without any typical symptoms (2-h blood glucose 215 mg/dL). Insulin levels were quite high (fasting 17.1 IU/mL, 2-h 114 IU/mL). Since 6 years of age he had been treated for Asperger syndrome, currently with risperidone. His family history was positive for diabetes in the previous three generations. At the time of diabetes diagnosis, dyslipidemia with elevated total cholesterol (212 mg/dL), triglycerides (150 mg/dL), and LDL cholesterol (133.1 mg/dL) was observed. Diabetes autoantibodies were significantly positive: islet antigen 2 antibodies …