Age at the time of sulfonylurea initiation influences treatment outcomes in KCNJ11-related neonatal diabetes

Aims/hypothesis Individuals with heterozygous activating mutations of the KCNJ11 gene encoding a subunit of the ATP-sensitive potassium channel (KATP) can usually be treated with oral sulfonylurea (SU) pills in lieu of insulin injections. The aim of this study was to test our hypothesis that younger...

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Bibliographic Details
Published in:Diabetologia 2015-07, Vol.58 (7), p.1430-1435
Main Authors: Thurber, Brian W., Carmody, David, Tadie, Elizabeth C., Pastore, Ashley N., Dickens, Jazzmyne T., Wroblewski, Kristen E., Naylor, Rochelle N., Philipson, Louis H., Greeley, Siri Atma W.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Age Factors
Age of Onset
Child
Child, Preschool
Clinical outcomes
Diabetes
Diabetes Mellitus - congenital
Diabetes Mellitus - drug therapy
Dose-Response Relationship, Drug
Drug dosages
Female
Genetic testing
Glyburide - therapeutic use
Glycated Hemoglobin A - analysis
Human Physiology
Humans
Hypoglycemia
Hypoglycemic Agents - therapeutic use
Hypotheses
Infant
Infant, Newborn
Influence
Insulin
Internal Medicine
Male
Medical records
Medicine
Medicine & Public Health
Metabolic Diseases
Mutation
Mutation - genetics
Potassium
Potassium Channels, Inwardly Rectifying - genetics
Retrospective Studies
Sulfonylurea Compounds - therapeutic use
Treatment Outcome
Working groups
Young Adult
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Summary:Aims/hypothesis Individuals with heterozygous activating mutations of the KCNJ11 gene encoding a subunit of the ATP-sensitive potassium channel (KATP) can usually be treated with oral sulfonylurea (SU) pills in lieu of insulin injections. The aim of this study was to test our hypothesis that younger age at the time of initiation of SU therapy is correlated with lower required doses of SU therapy, shorter transition time and decreased likelihood of requiring additional diabetes medications. Methods We performed a retrospective cohort study using data on 58 individuals with neonatal diabetes due to KCNJ11 mutations identified through the University of Chicago Monogenic Diabetes Registry ( http://monogenicdiabetes.uchicago.edu/registry ). We assessed the influence of age at initiation of SU therapy on treatment outcomes. Results HbA 1c fell from an average of 8.5% (69 mmol/mol) before transition to 6.2% (44 mmol/mol) after SU therapy ( p  
ISSN:0012-186X
1432-0428
DOI:10.1007/s00125-015-3593-9