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Med."],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Therapy with sulfonylurea is preferable to insulin in the majority of individuals with <jats:italic><jats:styled-content style=\"fixed-case\">KCNJ<\/jats:styled-content>11<\/jats:italic> mutations, but not all of these people achieve target levels of HbA<jats:sub>1c<\/jats:sub> in long\u2010term follow\u2010up. We aimed to compare sulfonylurea therapy with insulin treatment in two sulfonylurea\u2010sensitive individuals with a <jats:italic><jats:styled-content style=\"fixed-case\">KCNJ<\/jats:styled-content>11<\/jats:italic> mutation who had poorly controlled permanent neonatal diabetes mellitus.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case report<\/jats:title><jats:p>We report on two individuals with a <jats:italic><jats:styled-content style=\"fixed-case\">KCNJ<\/jats:styled-content>11<\/jats:italic> mutation (p.R201H) who are currently aged 35 (<jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>1) and 21 years (<jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>2). These individuals were switched from insulin to sulfonylurea in 2005. Data from the first 4 (<jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>2) and 8 years (<jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>1) of the follow\u2010up showed improved diabetes control and increased quality of life for both individuals. During the following years, however, both individuals failed to retain good diabetes control (HbA<jats:sub>1c<\/jats:sub> \u2264 53 mmol\/mol; 7.0%). We therefore changed the therapy to a combination of insulin and sulfonylurea in both individuals, or to insulin monotherapy in <jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>1, and compared the effects on HbA<jats:sub>1c<\/jats:sub> with those of sulfonylurea monotherapy. HbA<jats:sub>1c<\/jats:sub> levels in both individuals worsened after adding insulin to sulfonylurea [67 mmol\/mol (8.3%) vs 77 mmol\/mol (9.2%) in <jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>1 and 106 mmol\/mol (11.8%) vs 110\u00b119 mmol\/mol (12.2\u00b11.7%) in <jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>2], and after switching to only insulin therapy in <jats:styled-content style=\"fixed-case\">SVK<\/jats:styled-content>1 [57 mmol\/mol (7.4%) vs 62 mmol\/mol (7.8%)] when compared with sulfonylurea monotherapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Our data show that sulfonylurea monotherapy might be preferable to insulin in people with permanent neonatal diabetes mellitus sensitive to sulfonylurea even when HbA<jats:sub>1c<\/jats:sub> is above target.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dme.13575","type":"journal-article","created":{"date-parts":[[2017,12,26]],"date-time":"2017-12-26T13:56:59Z","timestamp":1514296619000},"page":"386-391","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Sulfonylurea vs insulin therapy in individuals with sulfonylurea\u2010sensitive permanent neonatal diabetes mellitus, attributable to a <i><scp>KCNJ<\/scp>11<\/i> mutation, and poor glycaemic control"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0538-1274","authenticated-orcid":false,"given":"J.","family":"Stanik","sequence":"first","affiliation":[{"name":"DIABGENE Laboratory Institute of Experimental Endocrinology Biomedical Research Center of Slovak Academy of Sciences Bratislava Slovakia"},{"name":"Children Diabetes Centre at the Department of Paediatrics Medical Faculty of Comenius University and Children Faculty Hospital Bratislava Slovakia"},{"name":"Centre for Paediatric Research Leipzig University Hospital for Children and Adolescents University of Leipzig Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Dankovcikova","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Children Faculty Hospital Kosice Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Barak","sequence":"additional","affiliation":[{"name":"Children Diabetes Centre at the Department of Paediatrics Medical Faculty of Comenius University and Children Faculty Hospital Bratislava Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Skopkova","sequence":"additional","affiliation":[{"name":"DIABGENE Laboratory Institute of Experimental Endocrinology Biomedical Research Center of Slovak Academy of Sciences Bratislava Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Palko","sequence":"additional","affiliation":[{"name":"First Department of Internal Medicine Medical Faculty of Safarik University Kosice Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Divinec","sequence":"additional","affiliation":[{"name":"Outpatient Diabetes Clinic DIAHELP Pardubice Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Klimes","sequence":"additional","affiliation":[{"name":"DIABGENE Laboratory Institute of Experimental Endocrinology Biomedical Research Center of Slovak Academy of Sciences Bratislava Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Gasperikova","sequence":"additional","affiliation":[{"name":"DIABGENE Laboratory Institute of Experimental Endocrinology Biomedical Research Center of Slovak Academy of Sciences Bratislava Slovakia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,2,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/pedi.12192"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa061759"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00125-016-3921-8"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.4274\/Jcrpe.524"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1464-5491.2009.02690.x"},{"issue":"2","key":"e_1_2_7_7_1","doi-asserted-by":"crossref","first-page":"S260","DOI":"10.2337\/dc09-S319","article-title":"Initiating insulin therapy in type 2 diabetic patients failing on oral hypoglycemic agents: basal or prandial insulin? 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