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Cognitive function of children with cystic fibrosis: deleterious effect of early malnutrition

Rebecca L Koscik1, Philip M Farrell, Michael R Kosorok

  • 1Department of Biostatistics/Medical Informatics, University of Wisconsin, Madison 53706-1532, USA.

Pediatrics
|June 3, 2004
PubMed

Insights

Early diagnosis of cystic fibrosis (CF) through neonatal screening and prompt nutritional therapy, especially addressing vitamin E deficiency, improves cognitive function in children. This helps prevent long-term malnutrition and supports better cognitive outcomes.

Area of Science:

  • Pediatric Neurology
  • Nutritional Neuroscience
  • Genetic Disorders

Background:

  • Cystic Fibrosis (CF) diagnosis via traditional methods often leads to delayed nutritional intervention.
  • Early malnutrition in children with CF can negatively impact neurodevelopment.
  • Neonatal screening offers an opportunity for early detection and intervention.

Purpose of the Study:

  • To evaluate cognitive function in children with CF.
  • To assess the influence of early diagnosis through neonatal screening on cognitive outcomes.
  • To investigate the role of early malnutrition, particularly vitamin E deficiency, in cognitive development.

Main Methods:

  • Cognitive assessment using the Test of Cognitive Skills, Second Edition (CSI) was performed on 89 children with CF (aged 7.3-17 years).
  • Participants were categorized into a screened group (N=42) and a traditional diagnosis control group (N=47) from the Wisconsin CF Neonatal Screening Project.
  • Cognitive factor scores (Verbal, Nonverbal, Memory) and overall CSI were analyzed in relation to diagnostic group, nutritional status (plasma alpha-tocopherol), and family factors.

Main Results:

  • Overall cognitive scores were comparable to normative data (CSI: 102.5 ± 16.6).
  • Lower cognitive scores correlated with malnutrition indicators, low plasma alpha-tocopherol (vitamin E) levels at diagnosis, and adverse family factors.
  • Children with CF and vitamin E deficiency at diagnosis in the control group exhibited significantly lower CSI scores compared to other subgroups.

Conclusions:

  • Early diagnosis and nutritional management, particularly preventing prolonged vitamin E deficiency, are linked to enhanced cognitive function in children with CF.
  • Neonatal screening for CF facilitates timely intervention, mitigating nutritional insults and supporting cognitive development.
  • Optimizing nutritional status, including vitamin E levels, is crucial for cognitive well-being in pediatric CF patients.
Abstract

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