Cognitive and developmental outcome of conservatively treated children with congenital hyperinsulinism

Yael Levy-Shraga*, Orit Pinhas-Hamiel, Efrat Kraus-Houminer, Heddy Landau, Kineret Mazor-Aronovitch, Dalit Modan-Moses, David Gillis, Ilana Koren, Daphna Dollberg, Lidia V. Gabis

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants. Its management can be extremely complicated, and may involve medical therapy and surgery. The mainstay of the treatment is to maintain normoglycemia, since hypoglycemia during infancy can have severe neurological consequences. Objective: To assess the cognitive and developmental levels and the adaptive skills achieved by children with CHI who were treated medically over the past decade. Subjects and methods: Fourteen children with CHI, under the age of 10 years, who received medical treatment only, underwent a physical and neurological examination and standardized assessments that included the Bayley Scale of Infant and Toddler Development, 3rd Edition, or Kaufman Assessment Battery for Children, the Vineland Adaptive Behavior Scales and the Achenbach Child Behavior Checklist (CBCL) parent questionnaire form. Results: Twelve children (86 % ) achieved normal range scores in the cognitive and development assessments (Bayley Scale of Infant and Toddler Development or Kaufman Assessment Battery for Children). Only two showed cognitive achievements below the normal range. The Vineland questionnaire, which was based on parental report, showed below normal adaptive skills in eight patients (57 % ). Conclusions: In contrast to previous studies showing a high prevalence of neuro developmental difficulties in children with congenital hyperinsulinism, our study showed normal cognitive achievements in most children. This may be attributed to the earlier recognition and better management of the disease in the past decade.

Original languageEnglish
Pages (from-to)301-308
Number of pages8
JournalJournal of Pediatric Endocrinology and Metabolism
Volume26
Issue number3-4
DOIs
StatePublished - Apr 2013

Keywords

  • Cognitive outcome
  • Congenital hyperinsulinism

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