Abnormal neurodevelopmental outcomes are common in children with transient congenital hyperinsulinism

Research output: Contribution to journalArticle

  • External authors:
  • Hima Bindu Avatapalle
  • Indraneel Banerjee
  • Sajni Shah
  • Megan Pryce
  • Jacqueline Nicholson
  • Lindsey Rigby
  • Louise Caine
  • Mohammed Didi
  • Mars Skae
  • Sarah Ehtisham
  • Leena Patel
  • Raja Padidela

Abstract

Introduction: Neuroglycopenia is recognized to be associated with abnormal neurodevelopmental outcomes in 26-44% of children with persistent congenital hyperinsulinism (P-CHI). The prevalence of abnormal neurodevelopment in transient CHI (T-CHI) is not known. We have aimed to investigate abnormal neurodevelopment and associated factors in T-CHI and P-CHI. Materials and Methods: A cohort of children with CHI (n = 67, age 2.5-5 years) was assessed at follow-up review and noted to have normal or abnormal (mild or severe) neurodevelopmental outcomes for the domains of speech and language, motor, and vision. Children were classified as P-CHI (n = 33), if they had undergone surgery or remained on medical therapy, or T-CHI (n = 34), if medical treatment for hypoglycemia was stopped. Results: Overall, abnormal neurodevelopment was present in 26 (39%) children with CHI, of whom 18 (69%) were severe. Importantly, the incidence of abnormal neurodevelopment in T-CHI was similar to that in P-CHI (30 vs. 47% respectively, p = 0.16). The prevalence of severe abnormal neurodevelopment in speech, motor, and vision domains was similar in both T-CHI and P-CHI children. For this cohort, we found that the severity of disease [based upon maximal diazoxide dose (odds ratio 95% confidence intervals) 1.3 (1.1; 1.5), p = 0.03], and early presentation of CHI

Bibliographical metadata

Original languageEnglish
Article numberArticle 60
JournalFrontiers in Endocrinology
Volume4
Issue numberMAY
DOIs
Publication statusPublished - 2013