Opmerkelijke gevoeligheid voor sulfonylureumderivaten bij 2 patiënten met 'maturity-onset diabetes of the young' type 3

- In 2 patients, a 26-year-old woman and a 47-year-old man, diabetes mellitus was diagnosed during their teens, although they had (almost) no symptoms at the time. Since then, the disease was well controlled in both patients with the use of tolbutamide at a low dose. Diabetes occurred in at least 3 generations of both patients' families, inherited as an autosomal dominant trait. Genetic screening in both individuals revealed two separate mutations in the HNF-1α gene, confirming maturity-onset diabetes of the young type 3 (MODY-3). MODY-3 patients are unusually sensitive to the hypoglycaemic effects of sulphonylureas. These agents remain effective in these patients for years, even at low doses.