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Transition from insulin to sulfonylurea in a child with diabetes due to a mutation in KCNJ11 encoding Kir6.2—initial and long-term response to sulfonylurea therapy

European Journal of Pediatrics volume 168, pages 359–361 (2009)Cite this article

Abstract

Background

Mutations in the KCNJ11 gene encoding the adenosine triphosphate (ATP)-sensitive potassium channel (KATP) subunit Kir6.2 are the most frequent cause of diabetes in infancy. Sulfonylurea (SU) treatment restores insulin secretion in patients with KCNJ11 mutations.

Materials and methods

We report a 9-year-old boy who presented at the age of three months with diabetic ketoacidosis. Results Sequencing of the KCNJ11 gene revealed an R201H mutation. Therefore, he was transferred from insulin to oral SU therapy. He required a high-threshold dose before insulin could be discontinued. After transition, a subsequent dose reduction was necessary to avoid hypoglycemia. Improved sustained metabolic control without complications was achieved on a low SU maintenance dose twice daily over 36 months.

Conclusion

SU therapy is safe for patients with diabetes due to KCNJ11 mutations. The mechanism of a threshold dose and the twice-daily requirement needs further attention.

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Fig. 1

Abbreviations

SU:

Sulfonylurea

ATP:

Adenosine triphosphate

SUR:

Sulfonylurea receptor subunit

HbA1c :

Hemoglobin A1c

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Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Endocrinology and Diabetology, University of Lübeck, Lübeck, Germany

    Verena M. Wagner, Britta Kremke & Olaf Hiort

  2. Institute of Biomedical and Clinical Sciences, Peninsula Medical School, Exeter, UK

    Sarah E. Flanagan

  3. Division of Medicine and Therapeutics, University of Dundee, Dundee, Scotland, UK

    Ewan R. Pearson

  4. Klinik für Kinder- und Jugendmedizin, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Germany

    Verena M. Wagner

Authors
  1. Verena M. Wagner
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  2. Britta Kremke
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  3. Olaf Hiort
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  4. Sarah E. Flanagan
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  5. Ewan R. Pearson
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Correspondence to Verena M. Wagner.

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Wagner, V.M., Kremke, B., Hiort, O. et al. Transition from insulin to sulfonylurea in a child with diabetes due to a mutation in KCNJ11 encoding Kir6.2—initial and long-term response to sulfonylurea therapy. Eur J Pediatr 168, 359–361 (2009). https://doi.org/10.1007/s00431-008-0757-3

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  • DOI: https://doi.org/10.1007/s00431-008-0757-3

Keywords

  • Children
  • Diabetes mellitus
  • Drug therapy
  • Kir6.2 channel
  • Sulfonylurea receptor