Successful maintenance on sulphonylurea therapy and low diabetes complication rates in a HNF1A-MODY cohort.

Publisher: Blackwell Science Country of Publication: England NLM ID: 8500858 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1464-5491 (Electronic) Linking ISSN: 07423071 NLM ISO Abbreviation: Diabet Med Subsets: MEDLINE

Publication: Oxford : Blackwell Science
Original Publication: Chichester [Sussex, England] ; New York : Wiley, [c1984-

Diabetes Complications/*epidemiology ; Diabetes Mellitus, Type 1/*drug therapy ; Diabetes Mellitus, Type 2/*drug therapy ; Hepatocyte Nuclear Factor 1-alpha/*genetics ; Hypoglycemic Agents/*therapeutic use ; Insulin/*therapeutic use ; Sulfonylurea Compounds/*therapeutic use; Adult ; Albuminuria/epidemiology ; Albuminuria/etiology ; Cardiovascular Diseases/epidemiology ; Cardiovascular Diseases/etiology ; Case-Control Studies ; Coronary Disease/epidemiology ; Coronary Disease/etiology ; Diabetes Complications/etiology ; Diabetes Mellitus, Type 1/complications ; Diabetes Mellitus, Type 2/complications ; Diabetes Mellitus, Type 2/genetics ; Diabetes Mellitus, Type 2/metabolism ; Diabetic Nephropathies/epidemiology ; Diabetic Nephropathies/etiology ; Diabetic Retinopathy/epidemiology ; Diabetic Retinopathy/etiology ; Disease Progression ; Female ; Glycated Hemoglobin/metabolism ; Humans ; Maintenance Chemotherapy/methods ; Male ; Middle Aged ; Treatment Outcome ; United Kingdom ; Young Adult

Aims: HNF1A gene mutations are the most common cause of maturity-onset diabetes of the young (MODY) in the UK. Persons with HNF1A-MODY display sensitivity to sulphonylurea therapy; however, the long-term efficacy is not established. There is limited literature as to the prevalence of micro- and macrovascular complications in this unique cohort. The aim of this study was to determine the natural progression and clinical management of HNF1A-MODY diabetes in a dedicated MODY clinic.
Methods: Sixty patients with HNF1A-MODY and a cohort of 60 BMI-, age-, ethnicity- and diabetes duration-matched patients with Type 1 diabetes mellitus participated in the study. All patients were phenotyped in detail. Clinical follow-up of the HNF1A-MODY cohort occurred on a bi-annual basis.
Results: Following a genetic diagnosis of MODY, the majority of the cohort treated with sulphonylurea therapy remained insulin independent at 84-month follow-up (80%). The HbA1c in the HNF1A-MODY group treated with sulphonylurea therapy alone improved significantly over the study period [from 49 (44-63) mmol/mol, 6.6 (6.2-7.9)% to 41 (31-50) mmol/mol, 5.9 (5-6.7)%; P = 0.003]. The rate of retinopathy was significantly lower than that noted in the Type 1 diabetes mellitus group (13.6 vs. 50%; P = 0.0001).There was also a lower rate of microalbuminuria and cardiovascular disease in the HNF1A-MODY group compared with the Type 1 diabetes mellitus group.
Conclusions: This study demonstrates that the majority of patients with HNF1A-MODY can be maintained successfully on sulphonylurea therapy with good glycaemic control. We note a significantly lower rate of micro- and macrovascular complications than reported previously. The use of appropriate therapy at early stages of the disorder may decrease the incidence of complications.
(© 2015 Diabetes UK.)

098395 United Kingdom Wellcome Trust

0 (Glycated Hemoglobin A)
0 (HNF1A protein, human)
0 (Hepatocyte Nuclear Factor 1-alpha)
0 (Hypoglycemic Agents)
0 (Insulin)
0 (Sulfonylurea Compounds)
0 (hemoglobin A1c protein, human)

Maturity-Onset Diabetes of the Young, Type 3

Date Created: 20151020 Date Completed: 20180109 Latest Revision: 20221207

20221213

10.1111/dme.12992

26479152