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Title: | Effective treatment with oral sulfonylureas in patients with diabetes due to sulfonylurea receptor 1 (SUR1) mutations. | Authors: | Flanagan S.E.;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 R.-M.;Van Der Meulen J.;Patch A.-M.;Rafiq M.;Carson D.;Bruining J.;Batra C.;Hattersley A.T.;Ellard S.;Shields B.M. | Institution: | (Rafiq, Flanagan, Patch, Shields, Ellard, Hattersley) Institute of Biomedical and Clinical Science, Peninsula Medical School, Exeter, United Kingdom (Rafiq) St. George's University of London, London, United Kingdom (Hattersley) Peninsula Medical School, Barrack Road, Exeter, EX2 5DW, United Kingdom (Batra) Batra Hospital, New Delhi, India (Bruining) Sophia Children's Hospital, Rotterdam, Netherlands (Carson) Royal Belfast Hospital for Sick Children, Belfast, United Kingdom (Codner) University of Chile, Santiago, Chile (Cummings) IWK Health Centre, NS, Canada (Curran) University of Western Australia, Perth, WA, Australia (Davis) Princess Margaret Hospital for Children, Perth, WA, Australia (Deiss, Herr) Charitie Campus Virchow Klinikum, Berlin, Germany (Hussain) Great Ormond Street Hospital for Children, London, United Kingdom (Legault) Montreal Children's Hospital, Montreal, QC, Canada (Malecki) Jagiellonian University, Krakow, Poland (Paskova) Children's University Hospital, Kosice, Slovakia (Pearson) University of Dundee, Dundee, United Kingdom (Rodda) Monash Medical Centre, VIC, Australia (Rothenbuhler) Hopital Saint Vincent de Paul, Paris, France (Sanchez) Rilex Hospital for Children, Indianapolis, IN, United States (Shield) Bristol Children's Hospital, Bristol, United Kingdom (Stanca) Pediatrics Hospital of Sibiu, Sibiu, Romania (Van Der Meulen) McMaster Children's Hospital, ON, Canada | Issue Date: | 28-Feb-2008 | Copyright year: | 2008 | Publisher: | American Diabetes Association Inc. | Place of publication: | Romania | Publication information: | Diabetes Care. 31 (2) (pp 204-209), 2008. Date of Publication: February 2008. | Journal: | Diabetes Care | 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. © 2008 by the American Diabetes Association. | DOI: | http://monash.idm.oclc.org/login?url=http://dx.doi.org/10.2337/dc07-1785 | PubMed URL: | 18025408 [http://www.ncbi.nlm.nih.gov/pubmed/?term=18025408] | ISSN: | 0149-5992 | URI: | https://repository.monashhealth.org/monashhealthjspui/handle/1/31537 | Type: | Article |
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