A worldwide perspective on clinical characteristics and treatment of youth with monogenic diabetes in the SWEET registry

Abstract Aims To characterize youth with monogenic diabetes worldwide in the SWEET database and define trends in clinical care and outcomes. Methods Youth with monogenic diabetes between the ages of 0 and 21 years from 44 worldwide centers in the SWEET registry divided into global regions were studied in this retrospective analysis. This included 690 youth with data at diabetes diagnosis and/or follow-up at 1 year and 214 patients with data at both time points. Demographics, comorbidities, and treatments were evaluated. Results Globally, mean age and hemoglobin A1c (HbA1c) at diagnosis were 10.1 years (SD 4.57 years) and 6.9% (52 mmol/mol) (SD 1.7%, 18 mmol/mol), respectively. At 1-year follow-up, mean HbA1c decreased by 0.4%. Average body mass index (BMI) at diabetes diagnosis was in the normal range (World Health Organization BMI SD score 0.31). At diabetes diagnosis, 3.6% presented in diabetic ketoacidosis (DKA). Seven percent were treated with oral antidiabetes medications or glucagon-like peptide-1 receptor agonists at diabetes diagnosis, increasing to 15.3% at follow-up. Overall, treatment with insulin increased by 10% at follow-up. Conclusion Worldwide, patients with monogenic diabetes typically present during late childhood/early adolescence with mild elevation in HbA1c, normal BMI, and lack of DKA. Regional differences in demographics and treatment modalities highlight heterogeneity in presentation and management of monogenic diabetes, impacting clinical care.