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Glycemic control and motor development in a patient with intermediate DEND
Hideharu Oka1, Shigeru Suzuki, Akiko Furuya
1Department of Pediatrics, Asahikawa Medical University, Asahikawa, Japan.
Summary
Glibenclamide therapy improved glycemic control and motor development in a child with intermediate DEND (iDEND), a KCNJ11 gene mutation disorder. This sulfonylurea treatment offers a promising therapeutic option for iDEND patients.
Area of Science:
- Genetics and Molecular Biology
- Pediatrics
- Endocrinology
Background:
- Neonatal diabetes mellitus is often caused by KCNJ11 gene mutations.
- Mutations can lead to DEND syndrome (developmental delay, epilepsy, neonatal diabetes) or milder iDEND.
- Effective management of iDEND requires addressing both glycemic control and developmental aspects.
Observation:
- A case of intermediate DEND (iDEND) in a 17-month-old child with poor glycemic control and delayed motor development is presented.
- The patient was transitioned from insulin to glibenclamide therapy.
- Continuous glucose monitoring and developmental quotient (DQ) assessments were utilized.
Findings:
- Glibenclamide therapy significantly improved glycemic control, with HbA1c decreasing from 10.2% to 5.6% within three months.
- Blood glucose fluctuations were notably suppressed under glibenclamide treatment.
- Motor development showed marked improvement, with the motor DQ increasing from 60 to 91, while language and adoptive abilities remained stable.
Implications:
- Glibenclamide is an effective treatment for improving glycemic control in iDEND patients.
- Sulfonylurea therapy can positively impact motor development in children with KCNJ11-related iDEND.
- This case highlights the potential of glibenclamide as a therapeutic strategy for iDEND, offering benefits beyond glycemic control.
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