GLP-1 RA and dual GIP/GLP-1 RA treatment in MODY: a descriptive case series

Apr 24, 2025BMJ open diabetes research & care

Treatment with GLP-1 and combined GIP/GLP-1 medications in MODY: a case series

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Abstract

GLP-1 receptor agonists reduced HbA1c by 1.3% in patients with HNF1A and HNF4A types of maturity-onset diabetes of the young (MODY).

In patients with HNF1A-MODY and HNF4A-MODY, GLP-1 receptor agonists also decreased body mass index (BMI) by 2.90 kg/m and reduced sulfonylurea dose by 66.6%.
Dual GIP/GLP-1 receptor agonist treatment resulted in a non-statistically significant HbA1c decrease of 1.8% and a significant BMI reduction of 8.73 kg/m.
All patients receiving dual GIP/GLP-1 receptor agonists who were on sulfonylurea discontinued its use, with one patient also stopping insulin.
For ABCC8-MODY and HNF1B-MODY patients, GLP-1 receptor agonists lowered HbA1c by 1.2% and 1.8%, and BMI by 1.1 kg/m and 1.2 kg/m, respectively.
Patients with ABCC8-MODY and HNF1B-MODY no longer required sulfonylurea and insulin treatments, respectively.

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INTRODUCTION: Glucagon-like peptide-1 receptor agonists (GLP-1 RA) and dual glucose insulinotropic polypeptide (GIP)/GLP-1 RA are widely prescribed, but their effectiveness in different subtypes of maturity-onset diabetes of the young (MODY) is unknown.

RESEARCH DESIGN AND METHODS: We present a descriptive case series of individuals from two MODY cohorts who used GLP-1 RA or dual GIP/GLP-1 RA. Pairedtests were used to compare HbA1c, body mass index (BMI), and sulfonylurea (SU) dose before and after GLP-1 RA or dual GIP/GLP-1 RA therapy. t

RESULTS: 10 individuals (4 hepatocyte nuclear factor-1α (HNF1A)-MODY, 4 hepatocyte nuclear factor-4α (HNF4A)-MODY, 1 ATP-binding cassette transporter subfamily C member 8 (ABCC8)-MODY, 1 hepatocyte nuclear factor-1β (HNF1B)-MODY) were identified who used GLP-1 RA or dual GIP/GLP-1 RA. In patients with HNF1A-MODY and HNF4A-MODY, GLP-1 RA reduced HbA1c by 1.3% (p=0.010), BMI by 2.90 kg/m(p=0.008), and total daily dose of SU by 66.6% (p=0.005). Dual GIP/GLP-1 RA treatment led to a non-statistically significant decrease in HbA1c of 1.8% (p=0.104), a statistically significant reduction in BMI of 8.73 kg/m(p=0.010), and all patients discontinued SU (n=2) and one discontinued insulin. In patients with ABCC8-MODY and HNF1B-MODY, GLP-1 RA reduced HbA1c by 1.2% and 1.8%, BMI by 1.1 kg/mand 1.2 kg/m, and the patients no longer required treatment with SU or insulin, respectively. 2 2 2 2

CONCLUSIONS: GLP-1 RA and dual GIP/GLP-1 RA lowered HbA1c, BMI, and SU dose in patients with MODY.

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GLP-1 RA and dual GIP/GLP-1 RA treatment in MODY: a descriptive case series

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