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Nutritional assessment of infants with cystic fibrosis diagnosed through screening
E H Mischler1, M S Marcus, S A Sondel
1Department of Pediatrics, University of Wisconsin Children's Hospital, Madison.
Insights
Early nutritional intervention in infants with cystic fibrosis (CF) can normalize nutrition. However, males with CF require closer monitoring for nutritional issues and potential fat intake reduction during their first year.
Area of Science:
- Pediatrics
- Biochemistry
- Genetics
Background:
- Neonatal screening identifies infants with cystic fibrosis (CF) presymptomatically.
- Presymptomatic infants with CF may exhibit biochemical signs of malnutrition.
- Early diagnosis allows for timely nutritional and medical interventions.
Purpose of the Study:
- To evaluate the effectiveness of early dietary management and pancreatic enzyme treatment in presymptomatic infants with CF.
- To identify potential sex-based differences in nutritional status and growth during the first year of life in infants with CF.
Main Methods:
- Biochemical assessment of nutritional indices in infants diagnosed with CF via neonatal screening.
- Implementation of aggressive dietary management and pancreatic enzyme replacement therapy.
- Comparative analysis of growth and nutritional markers between male and female infants with CF.
Main Results:
- Aggressive nutritional management normalized biochemical indices in most infants by 12 months.
- Male infants with CF showed a higher risk for abnormal growth and nutritional markers compared to females.
- A decrease in fat intake was observed in males with CF relative to females.
Conclusions:
- Early nutritional intervention is effective in improving nutritional status in infants with CF.
- Male infants with CF warrant closer monitoring for nutritional abnormalities, particularly concerning fat intake.
- Further research, including ongoing randomized controlled trials, is needed to determine long-term outcomes.
Abstract:
Presymptomatic infants diagnosed through neonatal screening for cystic fibrosis can have biochemical evidence of malnutrition. With aggressive dietary management and treatment with pancreatic enzymes, normal biochemical indices of nutrition can be achieved at 12 months of life in most cases. Males with cystic fibrosis appear to be more at risk than females for abnormal growth and biochemical indices of nutrition in the first year of life. This may be related to the observed decrease in fat intake when compared to females. Males, especially, should be carefully observed for development of nutritional abnormalities based on this data. Careful attention should be paid to vitamin E and essential fatty acid status in all CF infants. The numbers in this study are small and the long-term consequences of early nutritional intervention await the conclusion of the randomized, controlled study on-going in Wisconsin.