Introduction and Objective: Maturity-onset diabetes of the young (MODY) is a rare monogenic form of diabetes affecting approximately 1-6% of individuals with diabetes and characterized by phenotypic heterogeneity. Data on racial and ethnic differences in youth with MODY are limited. We aimed to characterize racial and ethnic differences in youth with MODY. Methods: We conducted a retrospective chart review of youth diagnosed with MODY at a tertiary care center between January 2010 and April 2025. Of 141 identified cases, 98 youth with genetic confirmation were included. Race and ethnicity were determined from medical record documentation. Clinical characteristics at diagnosis and last follow-up were compared across race/ethnicity groups using descriptive statistics and multivariable regression analyses. Results: The cohort comprised 55% (n=54) female, with a median age at diagnosis of 12 years (Q1-Q3: 8-14); age at last visit 17 years (14-18). The most common MODY subtypes were GCK (43%), HNF1A (24.2%), HNF1B (15.6%), and HNF4A (7%). Race/ethnicity data were available for 88 youth: 42% non-Hispanic White (NHW), 35% Hispanic, 16% non-Hispanic Black (NHB), and 7% non-Hispanic Other (NHO). At diagnosis, NHB youth had higher glucose levels (p=0.03), and NHO youth had the highest prevalence of islet autoimmunity (p0.001). Age, sex, HbA1c, C-peptide, BMI z-score, and family history of diabetes were similar across groups. At the last visit, NHW youth had the lowest HbA1c (p=0.006), while NHB youth had the highest prevalence of prior diabetic ketoacidosis. Use of insulin and sulfonylureas did not differ by race/ethnicity. After adjustment for confounders, Hispanic youth had higher HbA1c levels at the last visit compared with NHW youth (p=0.047). Conclusion: Racial and ethnic differences in clinical presentation and glycemic outcomes are present among youth with MODY. Further studies are needed to identify contributors to these differences and improve outcomes across populations. Disclosure S. Hardeep Singh: None. A.D. Nguyen: None. S. Aras: None. M.J. Redondo: Advisory Panel; Current; Sanofi. Other - Data Safety Monitoring committee; Current; Lilly. M. Tosur: None.
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