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European Journal of Pediatrics

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01-03-2009 | Short Report

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

Authors: Verena M. Wagner, Britta Kremke, Olaf Hiort, Sarah E. Flanagan, Ewan R. Pearson

Published in: European Journal of Pediatrics | Issue 3/2009

Abstract

Background

Mutations in the KCNJ11 gene encoding the adenosine triphosphate (ATP)-sensitive potassium channel (K_ATP) 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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10.

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11.

Tonini G, Bizzarri C, Bonfanti R, Vanelli M, Cerutti F, Faleschini E, Meschi F, Prisco F, Ciacco E, Cappa M, Torelli C, Cauvin V, Tumini S, Iafusco D, Barbetti F; Early-Onset Diabetes Study Group of the Italian Society of Paediatric Endocrinology and Diabetology (2006) Sulfonylurea treatment outweighs insulin therapy in short-term metabolic control of patients with permanent neonatal diabetes mellitus due to activating mutations of the KCNJ11 (Kir6.2) gene. Diabetologia 49:2210–2213. DOI 10.1007/s00125-006-0329-x PubMedCrossRef

Title: 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
Authors: Verena M. Wagner
Britta Kremke
Olaf Hiort
Sarah E. Flanagan
Ewan R. Pearson
Publication date: 01-03-2009
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 3/2009
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-008-0757-3

At a glance: The STEP trials

Springer Medicine

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

Abstract

Background

Materials and methods

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Materials and methods

Conclusion

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