Glycemic control and motor development in a patient with intermediate DEND.
Pediatr Int
; 56(3): 432-5, 2014 Jun.
Article
en En
| MEDLINE
| ID: mdl-24894933
ABSTRACT
The most common cause of neonatal diabetes, KCNJ11 gene mutation, can manifest as a neurological disorder. The most severe form consists of a constellation of developmental delay, epilepsy, and neonatal diabetes (DEND). Intermediate DEND (iDEND) refers to a milder presentation without epilepsy. We present a child with iDEND, for whom insulin injections were replaced with glibenclamide therapy at 17 months of age because of poor glycemic control and delayed motor development. Three months after initiation of glibenclamide, HbA1c decreased from 10.2% to 5.6%. Continuous glucose monitoring indicated that blood glucose fluctuations were suppressed while on glibenclamide. Furthermore, after initiating glibenclamide therapy, the developmental quotient (DQ) for motor ability markedly improved from 60 to 91, whereas the DQ for language and adoptive ability remained as they had been before the sulfonylurea treatment. Sulfonylurea treatment improved glycemic control and motor development in the present patient.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Trastornos Psicomotores
/
Desarrollo Infantil
/
Diabetes Mellitus
/
Epilepsia
/
Glucosa
/
Enfermedades del Recién Nacido
Límite:
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Pediatr Int
Asunto de la revista:
PEDIATRIA
Año:
2014
Tipo del documento:
Article
País de afiliación:
Japón