Premium
Glycemic control and motor development in a patient with intermediate DEND
Author(s) -
Oka Hideharu,
Suzuki Shigeru,
Furuya Akiko,
Matsuo Kumihiro,
Amamiya Satoshi,
Oshima Miho,
Oka Toshiaki,
Mukai Tokuo,
Okayama Akie,
Araki Akiko,
Azuma Hiroshi,
Tanahashi Yusuke
Publication year - 2014
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12294
Subject(s) - medicine , glibenclamide , sulfonylurea , glycemic , epilepsy , diabetes mellitus , insulin , endocrinology , pediatrics , psychiatry
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.