[High sensitivity to sulphonylurea treatment in 2 patients with maturity-onset diabetes of the young type 3]

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Title:	[High sensitivity to sulphonylurea treatment in 2 patients with maturity-onset diabetes of the young type 3]
Author(s):	Bosselaar, M. ; Hattersley, A.T.; Tack, C.J.J.
Publication year:	2002
Source:	Nederlands Tijdschrift voor Geneeskunde, vol. 146, iss. 15, (2002), pp. 726-729
ISSN:	0028-2162
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : http://hdl.handle.net/2066/165279
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Subject:	Hypertension and Circulation Hypertensie en circulatie
Organization:	Internal Medicine
Journal title:	Nederlands Tijdschrift voor Geneeskunde
Volume:	vol. 146
Issue:	iss. 15
Page start:	p. 726
Page end:	p. 729
Classification:	Clinical MedicinePharmacology Medical sciences Bescherming en bevordering van de menselijke gezondheid Geneeskunde: algemeen
Abstract:	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-1 alpha 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.