Sulfonylrea Treatment in Permanent Neonatal Diabetes Due to G53D Mutation in the KCNJ11 Gene

Improvement in glycemic control and neurological function

  1. Lucimary C. Gurgel, MD1,
  2. Felipe Crispim, BSC1,
  3. Maria Helena S. Noffs, MSC2,
  4. Erich Belzunces, BSC2,
  5. Marcio A. Rahal, MD2 and
  6. Regina S. Moisés, MD, PHD1
  1. 1Department of Endocrinology, Federal University of São Paulo, São Paulo, Brazil
  2. 2Department of Neurology, Federal University of São Paulo, São Paulo, Brazil
  1. Address correspondence to Regina S. Moisés, MD, PhD, Universidade Federal de São Paulo, Rua Botucatu, 740-2o, Andar, 04034-970 São Paulo, Brazil. E-mail: rmoises{at}

Previous studies have reported the successful switch from insulin to sulfonylrea therapy in some patients who have neonatal diabetes due to KCNJ11 mutations (1); however, data on adults are limited (2,3). Also, it has not yet been determined whether neurological symptoms can be improved by the action of sulfonylrea therapy.

Here, we report the glycemic and neurological responses in an adult patient with the G53D mutation in the KCNJ11 gene who was transferred from insulin to sulfonylurea.

A 26-year-old male patient was diagnosed with diabetes in the third month of …

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