No Deterioration in Glycemic Control in HNF-1α Maturity-Onset Diabetes of the Young Following Transfer From Long-Term Insulin to Sulphonylureas
- Maggie Shepherd, PHD1,
- Ewan R. Pearson, MB BCHIR1,
- Jane Houghton, MSC2,
- Gill Salt, BSC3,
- Sian Ellard, PHD1 and
- Andrew T. Hattersley, DM1
- 1Peninsula Medical School, Exeter, U.K
- 2Royal Preston Hospital, Preston, U.K
- 3New Cross Hospital, Wolverhampton, U.K
- Address correspondence to Professor Andrew T. Hattersley, Diabetes and Vascular Medicine, Peninsula Medical School, Barrack Road, Exeter, Devon, EX2 5DW, U.K. E-mail: a.t.hattersley{at}exeter.ac.uk
Maturity-onset diabetes of the young (MODY) accounts for ∼1% of diabetes in the U.K. It is characterized by autosomal dominant inheritance of young-onset diabetes that is not insulin dependent. MODY is frequently misdiagnosed as type 1 diabetes and treated immediately with insulin due to its presentation with marked hyperglycemia in slim adolescents/young adults (1–4). With diagnostic molecular genetic testing now well established, it is possible to make a definitive diagnosis of specific genetic subtypes of MODY (5).
HNF-1α mutations account for ∼65% of U.K. MODY (MODY 3) cases. Patients with a mutation in HNF-1α are sensitive to the hypoglycemic action of sulfonylureas (6). This suggests that patients with HNF-1α MODY started on insulin from diagnosis could transfer to a sulfonylurea, as described in isolated …
