Type 2 & other diabetes

Maturity-Onset Diabetes of the Young (MODY) in a Racially/Ethnically Diverse Pediatric Population

J Clin Endocrinol MetabPublished Retrospective case review

Authors' conclusion

The current guidelines to consider a diagnosis of MODY may not apply to a racially/ethnically diverse US population of children with diabetes.

Study context

Population
Racially/ethnically diverse youth with genetically confirmed MODY, large academic pediatric hospital, Southwestern USgenetically confirmed MODY
Participants
50 participants
Exposure
GCK-MODY vs HNF1A-MODY vs HNF1B-MODY vs HNF4A-MODY vs Dual molecular diagnosis
  • GCK-MODYn = 30
  • HNF1A-MODYn = 8
  • HNF1B-MODYn = 9
  • HNF4A-MODYn = 2
  • Dual molecular diagnosisn = 1
Outcome
Features of MODY cases vs guideline criteria
Methodological detailssingle-centre, genetically confirmed cases
  • Single-centre
  • Genetically confirmed cases

Main finding

As reported in the abstract

Race/ethnicity other than non-Hispanic White
52% (n = 26)
Lack of parental diabetes history
24% (n = 12)
Obesity/overweight at diagnosis
22.2% (n = 11)
Dyslipidemia and/or hypertension
30% (n = 15)
Acanthosis nigricans on physical examination
12% (n = 6)
Islet antibody positivity in GCK-MODY
8% (n = 4)

Observational data: associations, not effects.

Further findings

Mean HbA1c at diabetes diagnosis — HNF4A-MODY, HNF1A-MODY, HNF1B-MODY vs GCK-MODY · higher in HNF4A-, HNF1A- and HNF1B-MODY

P = 0.005

Original publication

Maturity-Onset Diabetes of the Young (MODY) in a Racially/Ethnically Diverse Pediatric Population. J Clin Endocrinol Metab. Published 24 June 2025. doi:10.1210/clinem/dgaf360

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