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

Meena Rafiq, Sarah E. Flanagan, Ann Marie Patch, Beverley M. Shields, Sian Ellard, Andrew T. Hattersley, Chandar Batra, Jan Bruining, Dennis Carson, Ethel Codner, Elizabeth Cummings, Jacqueline Curran, Elizabeth Davis, Dorothee Deiss, Mathias Herr, Khalid Hussain, Laurent Legault, Maciek Malecki, Magdalena Paskova, Ewan PearsonChristine Rodda, Anya Rothenbuhler, Juan Sanchez, Julian Shield, Ràcealà Motoc Stanca, John Van Der Meulen

Research output: Contribution to journalArticle

154 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)204-209
Number of pages6
JournalDiabetes Care
Volume31
Issue number2
DOIs
StatePublished - Feb 2008

Fingerprint

Sulfonylurea Receptors
Mutation
Insulin
Therapeutics
Patient Transfer
Injections
Clinical Protocols
Hypoglycemia

ASJC Scopus subject areas

  • Internal Medicine
  • Endocrinology, Diabetes and Metabolism

Cite this

Rafiq, M., Flanagan, S. E., Patch, A. M., Shields, B. M., Ellard, S., Hattersley, A. T., ... Van Der Meulen, J. (2008). Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations. Diabetes Care, 31(2), 204-209. https://doi.org/10.2337/dc07-1785

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.; Batra, Chandar; Bruining, Jan; Carson, Dennis; Codner, Ethel; Cummings, Elizabeth; Curran, Jacqueline; Davis, Elizabeth; Deiss, Dorothee; Herr, Mathias; Hussain, Khalid; Legault, Laurent; Malecki, Maciek; Paskova, Magdalena; Pearson, Ewan; Rodda, Christine; Rothenbuhler, Anya; Sanchez, Juan; Shield, Julian; Stanca, Ràcealà Motoc; Van Der Meulen, John.

In: Diabetes Care, Vol. 31, No. 2, 02.2008, p. 204-209.

Research output: Contribution to journalArticle

Rafiq, M, Flanagan, SE, Patch, AM, Shields, BM, Ellard, S, Hattersley, AT, Batra, C, Bruining, J, Carson, D, Codner, E, Cummings, E, Curran, J, Davis, E, Deiss, D, Herr, M, Hussain, K, Legault, L, Malecki, M, Paskova, M, Pearson, E, Rodda, C, Rothenbuhler, A, Sanchez, J, Shield, J, Stanca, RM & Van Der Meulen, J 2008, 'Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations', Diabetes Care, vol. 31, no. 2, pp. 204-209. https://doi.org/10.2337/dc07-1785
Rafiq, Meena ; Flanagan, Sarah E. ; Patch, Ann Marie ; Shields, Beverley M. ; Ellard, Sian ; Hattersley, Andrew T. ; Batra, Chandar ; Bruining, Jan ; Carson, Dennis ; Codner, Ethel ; Cummings, Elizabeth ; Curran, Jacqueline ; Davis, Elizabeth ; Deiss, Dorothee ; Herr, Mathias ; Hussain, Khalid ; Legault, Laurent ; Malecki, Maciek ; Paskova, Magdalena ; Pearson, Ewan ; Rodda, Christine ; Rothenbuhler, Anya ; Sanchez, Juan ; Shield, Julian ; Stanca, Ràcealà Motoc ; Van Der Meulen, John. / Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations. In: Diabetes Care. 2008 ; Vol. 31, No. 2. pp. 204-209.
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T1 - Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations

AU - Rafiq, Meena

AU - Flanagan, Sarah E.

AU - Patch, Ann Marie

AU - Shields, Beverley M.

AU - Ellard, Sian

AU - Hattersley, Andrew T.

AU - Batra, Chandar

AU - Bruining, Jan

AU - Carson, Dennis

AU - Codner, Ethel

AU - Cummings, Elizabeth

AU - Curran, Jacqueline

AU - Davis, Elizabeth

AU - Deiss, Dorothee

AU - Herr, Mathias

AU - Hussain, Khalid

AU - Legault, Laurent

AU - Malecki, Maciek

AU - Paskova, Magdalena

AU - Pearson, Ewan

AU - Rodda, Christine

AU - Rothenbuhler, Anya

AU - Sanchez, Juan

AU - Shield, Julian

AU - Stanca, Ràcealà Motoc

AU - Van Der Meulen, John

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N2 - 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.

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