title: Glycemic control and motor development in a patient with intermediate DEND.
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.
authors.affiliation: Department of Pediatrics, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Hideharu
authors.initials: H
authors.lastname: Oka
authors.forename: Shigeru
authors.initials: S
authors.lastname: Suzuki
authors.forename: Akiko
authors.initials: A
authors.lastname: Furuya
authors.forename: Kumihiro
authors.initials: K
authors.lastname: Matsuo
authors.forename: Satoshi
authors.initials: S
authors.lastname: Amamiya
authors.forename: Miho
authors.initials: M
authors.lastname: Oshima
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Oka
authors.forename: Tokuo
authors.initials: T
authors.lastname: Mukai
authors.forename: Akie
authors.initials: A
authors.lastname: Okayama
authors.forename: Akiko
authors.initials: A
authors.lastname: Araki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Azuma
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Tanahashi
mesh_terms: D002657:Child Development / Q000502:physiology*; D003920:Diabetes Mellitus / Q000188:drug therapy / Q000503:physiopathology*; D004827:Epilepsy / Q000188:drug therapy / Q000503:physiopathology*; D005947:Glucose / Q000378:metabolism*; D005905:Glyburide / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007232:Infant, Newborn, Diseases / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D009043:Motor Activity / Q000502:physiology; D011596:Psychomotor Disorders / Q000188:drug therapy / Q000503:physiopathology*
chemical_list: D005947:Glucose; D005905:Glyburide
keywords: DEND; KCNJ11; neonatal diabetes; sulfonylurea