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Clinical characteristics and long-term management of HNF1A-MODY in a dedicated MODY clinic
Patrick McCluskey , Mairead T Crowley , Nick Ng , Conor Larney , Katherine Mc Donald , Genesis A Gabo , Marie Burke , Siobhan Bacon & Maria M Byrne
Mater Misericordiae University Hospital, Dublin, Ireland
HNF1A-MODY is sensitive to sulphonylureas (SU). Efficacy can decline over time with weight gain and diabetes duration. GLP-1RAs improve glycaemic control and reduce weight in HNF1A-MODY. This study aimed to establish long-term treatment response. 79 HNF1A-MODY subjects participated in a retrospective observational study. Subjects were phenotyped with annual follow-up. Non-parametric data provided as median and interquartile range with Wilcoxon signed-rank test performed in SPSS. Median age was 48 (37-63), BMI 24.4 kg/m2 (22.0-27.5), diabetes duration 20 (15-31) years and follow-up 8 (3-14.5) years. Prior to genetic diagnosis, 20 (25.3%) were on insulin alone, 14 (17.7%) SU alone, 11 (13.9%) SU combination, 17 (21.5%) diet and 17 (21.5%) non-SU and/or insulin. Following genetic diagnosis 65 (82%) switched to SU-containing therapy with 27 (41.5%) on SU monotherapy at follow-up. 5 maintained pre-diagnosis SU monotherapy. Follow-up SU monotherapy dose (modified release) and HbA1c was 30 mg (15-60) and HbA1c 53 mmol/mol (45-58) respectively. HbA1c was non-inferior after SU conversion (54 mmol/mol [48-63] vs 53 mmol/mol [45-58], P = 0.210). 16 switched insulin to SU therapy with 7 remaining on SU monotherapy (HbA1c 52 mmol/mol [45-63] vs 54 mmol/mol [46-56], P = 1.00). 19 (24%) were on a GLP-1RAs at follow-up (4 [3-4.25] years) with improvement in HbA1c (63 mmol/mol [58-65] to 55 mmol/mol [44-60], P = 0.01) and weight (88.6 kg [80.1-104.2] to 82 kg [74.1-87.9], P = <0.001). Retinopathy occurred in 45.6% (11.4% significant). There was low burden of cardiovascular disease, nephropathy and neuropathy. Diagnosis of HNF1A-MODY facilitates SU switch with adequate long-term glycaemic control in 41.5%. GLP-1RAs can be utilised for glycaemic and weight control.
Volume 115
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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