No Deterioration in Glycemic Control in HNF-1α Maturity-Onset Diabetes of the Young Following Transfer From Long-Term Insulin to Sulphonylureas

Abstract
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 …