Hepatocyte nuclear factor-1 beta mutations cause neonatal diabetes and intrauterine growth retardation: The proportion of cases caused by a de novo pathogenic variant is unknown for the majority of MODY-related genes. Eur J Hum Genet. Nat Clin Pract Endocrinol Metab. When compared to patients with type 2 diabetes, MODY patients are often more sensitive to sulphonylureas, such that a lower dose should be used to initiate treatment to avoid hypoglycaemia. For the individual, it informs optimal treatment options in some instances eg, the efficacy of sulphonylureas in HNF1A and HNF4A mutations and obviates the need for treatment in others in the case of mild hyperglycemia in GCK mutations.
Describe the clinical characteristics of MODY.
Maturity-onset diabetes of the young (MODY): an update.