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P Bowman, Å Sulen, F Barbetti, J Beltrand, P Svalastoga, E Codner, EH Tessmann, PB Juliusson, T Skrivarhaug, ER Pearson, SE Flanagan, T Babiker, NJ Thomas, MH Shepherd, S Ellard, I Klimes, M Szopa, M Polak, D Iafusco, AT Hattersley and PR Njølstad
Abstract
KCNJ11 mutations cause permanent neonatal diabetes through pancreatic ATP-sensitive potassium channel activation. 90% of patients successfully transfer from insulin to oral sulfonylureas with excellent initial glycaemic control; however, whether this control is maintained in the long term is unclear. Sulfonylurea failure is seen in about 44% of people with type 2 diabetes after 5 years of treatment. Therefore, we did a 10-year multicentre follow-up study of a large international cohort of patients with KCNJ11 permanent neonatal diabetes to address the key questions relating to long-term efficacy and safety of sulfonylureas in these patients.
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