European Journal of Pediatrics

, Volume 168, Issue 3, pp 359–361

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

  • Verena M. Wagner
  • Britta Kremke
  • Olaf Hiort
  • Sarah E. Flanagan
  • Ewan R. Pearson
Short Report



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.


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.


Children Diabetes mellitus Drug therapy Kir6.2 channel Sulfonylurea receptor 





Adenosine triphosphate


Sulfonylurea receptor subunit


Hemoglobin A1c

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Verena M. Wagner
    • 1
    • 4
  • Britta Kremke
    • 1
  • Olaf Hiort
    • 1
  • Sarah E. Flanagan
    • 2
  • Ewan R. Pearson
    • 3
  1. 1.Department of Pediatrics, Division of Pediatric Endocrinology and DiabetologyUniversity of LübeckLübeckGermany
  2. 2.Institute of Biomedical and Clinical SciencesPeninsula Medical SchoolExeterUK
  3. 3.Division of Medicine and TherapeuticsUniversity of DundeeDundeeScotland, UK
  4. 4.Klinik für Kinder- und JugendmedizinUniversität zu LübeckLübeckGermany

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