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Abnormal Neurodevelopmental Outcomes are Common in Children with Transient Congenital Hyperinsulinism

Hima Bindu Avatapalle1, Indraneel Banerjee, Sajni Shah

  • 1Department of Paediatric Endocrinology, Royal Manchester Children's Hospital Manchester, UK.

Frontiers in Endocrinology
|June 5, 2013
PubMed

Insights

Abnormal neurodevelopment affects one-third of children with congenital hyperinsulinism (CHI), regardless of disease type. Early presentation and disease severity are key risk factors for neurodevelopmental issues in these children.

Area of Science:

  • Pediatric Endocrinology
  • Neurodevelopmental Pediatrics
  • Metabolic Disorders

Background:

  • Congenital hyperinsulinism (CHI) is linked to abnormal neurodevelopment in persistent (P-CHI) cases.
  • The neurodevelopmental impact of transient CHI (T-CHI) is not well-established.
  • This study investigates neurodevelopmental outcomes in both T-CHI and P-CHI.

Purpose of the Study:

  • To compare neurodevelopmental outcomes in children with T-CHI and P-CHI.
  • To identify factors associated with abnormal neurodevelopment in CHI.
  • To inform early recognition and intervention strategies.

Main Methods:

  • A cohort of 67 children (2.5-5 years) with CHI was assessed for neurodevelopmental outcomes (speech, motor, vision).
  • Children were classified as P-CHI (n=33) or T-CHI (n=34) based on treatment duration.
  • Statistical analysis identified factors associated with neurodevelopmental outcomes.

Main Results:

  • Abnormal neurodevelopment was observed in 39% of all CHI cases, with similar rates in T-CHI (30%) and P-CHI (47%).
  • Severe neurodevelopmental abnormalities in speech, motor, and vision were comparable between T-CHI and P-CHI.
  • Disease severity (maximal diazoxide dose) and early presentation (<7 days) were significant risk factors for abnormal neurodevelopment.

Conclusions:

  • Abnormal neurodevelopment affects approximately one-third of children with both T-CHI and P-CHI.
  • Early presentation and disease severity are significant risk factors for neurodevelopmental impairment in CHI.
  • Prompt recognition and management of hypoglycemia are crucial to prevent adverse neurodevelopmental outcomes.
Abstract

Keywords:
congenital hyperinsulinismdevelopmental delayhypoglycemianeurodevelopmentneurological outcomeseizurestransient congenital hyperinsulinism

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