Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations

Rafiq, Meena, Flanagan, Sarah E., Patch, Ann-Marie, Shields, Beverley M., Ellard, Sian, Hattersley, Andrew T. and Neonatal Diabetes International Collaborative Group (2008) Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations. Diabetes Care, 31 2: 204-209. doi:10.2337/dc07-1785


Author Rafiq, Meena
Flanagan, Sarah E.
Patch, Ann-Marie
Shields, Beverley M.
Ellard, Sian
Hattersley, Andrew T.
Neonatal Diabetes International Collaborative Group
Title Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations
Journal name Diabetes Care   Check publisher's open access policy
ISSN 0149-5992
1935-5548
Publication date 2008-02
Sub-type Article (original research)
DOI 10.2337/dc07-1785
Volume 31
Issue 2
Start page 204
End page 209
Total pages 6
Place of publication Alexandria, VA, United States
Publisher American Diabetes Association
Language eng
Formatted abstract
OBJECTIVE—Neonatal diabetes can result from mutations in the Kir6.2 or sulfonylurea receptor 1 (SUR1) subunits of the ATP-sensitive K+ channel. Transfer from insulin to oral sulfonylureas in patients with neonatal diabetes due to Kir6.2 mutations is well described, but less is known about changing therapy in patients with SUR1 mutations. We aimed to describe the response to sulfonylurea therapy in patients with SUR1 mutations and to compare it with Kir6.2 mutations.
RESEARCH DESIGN AND METHODS—We followed 27 patients with SUR1 mutations for at least 2 months after attempted transfer to sulfonylureas. Information was collected on clinical features, treatment before and after transfer, and the transfer protocol used. We compared successful and unsuccessful transfer patients, glycemic control before and after transfer, and treatment requirements in patients with SUR1 and Kir6.2 mutations.
RESULTS—Twenty-three patients (85%) successfully transferred onto sulfonylureas without significant side effects or increased hypoglycemia and did not need insulin injections. In these patients, median A1C fell from 7.2% (interquartile range 6.6–8.2%) on insulin to 5.5% (5.3–6.2%) on sulfonylureas (P = 0.01). When compared with Kir6.2 patients, SUR1 patients needed lower doses of both insulin before transfer (0.4 vs. 0.7 units · kg−1 · day−1; P = 0.002) and sulfonylureas after transfer (0.26 vs. 0.45 mg · kg−1 · day−1; P = 0.005).
CONCLUSIONS—Oral sulfonylurea therapy is safe and effective in the short term in most patients with diabetes due to SUR1 mutations and may successfully replace treatment with insulin injections. A different treatment protocol needs to be developed for this group because they require lower doses of sulfonylureas than required by Kir6.2 patients.
Keyword SUR1
Sulfonylurea receptor 1
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
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