Glycemic control and motor development in a patient with intermediate DEND

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 p...

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Published inPediatrics international Vol. 56; no. 3; pp. 432 - 435
Main Authors Oka, Hideharu, Suzuki, Shigeru, Furuya, Akiko, Matsuo, Kumihiro, Amamiya, Satoshi, Oshima, Miho, Oka, Toshiaki, Mukai, Tokuo, Okayama, Akie, Araki, Akiko, Azuma, Hiroshi, Tanahashi, Yusuke
Format Journal Article
LanguageEnglish
Published Australia Blackwell Publishing Ltd 01.06.2014
Subjects
Babies
Child Development - physiology
DEND
Diabetes
Diabetes Mellitus - drug therapy
Diabetes Mellitus - physiopathology
Epilepsy - drug therapy
Epilepsy - physiopathology
Glucose - metabolism
Glyburide - therapeutic use
Humans
Infant
Infant, Newborn, Diseases - drug therapy
Infant, Newborn, Diseases - physiopathology
KCNJ11
Male
Medical treatment
Motor Activity - physiology
neonatal diabetes
Pediatrics
Psychomotor Disorders - drug therapy
Psychomotor Disorders - physiopathology
sulfonylurea
DEND
sulfonylurea
neonatal diabetes
KCNJ11
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Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
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ISSN:1328-8067
1442-200X
DOI:10.1111/ped.12294