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Nutritional rehabilitation in cystic fibrosis: a 5 year follow-up study
A M Dalzell1, R W Shepherd, B Dean
1Cystic Fibrosis Clinic, Brisbane, Australia.
Insights
Intensive nutritional supplementation for malnourished cystic fibrosis (CF) children improves long-term growth and nutritional status. These benefits persist years after therapy cessation, suggesting sustained positive effects on CF patient health.
Area of Science:
- Pediatrics
- Nutrition Science
- Pulmonology
Background:
- Malnutrition is a common complication in cystic fibrosis (CF) patients.
- Previous studies showed short-term benefits of nutritional supplementation in CF children.
- Long-term effects of nutritional intervention in CF require further investigation.
Purpose of the Study:
- To evaluate the long-term effects of aggressive nutritional supplementation on growth, nutritional status, and pulmonary function in malnourished CF children.
- To compare the clinical progress of supplemented versus non-supplemented CF patients over a 5-year period.
Main Methods:
- A prospective study comparing a group of malnourished CF children receiving aggressive nutritional supplementation with a matched control group receiving standard therapy.
- Clinical progress, including weight, height, and pulmonary function (FEV1, FVC), was monitored over 5 years.
- Mortality rates were also compared between the groups.
Main Results:
- The previously supplemented group showed significantly greater weight and height z scores at 4 and 5 years compared to the non-supplemented group.
- Lower mortality was observed in the supplemented group (2 vs. 4), though not statistically significant.
- While pulmonary function decline was initially greater in the non-supplemented group, no significant differences in deterioration rates were observed between groups after 5 years among survivors.
Conclusions:
- Intensive nutritional support for 1 year in CF children yields both short- and long-term benefits in nutrition and growth, persisting years after therapy cessation.
- Extended supplementation periods (longer than 1 year) may enhance these gains and potentially prolong pulmonary function improvements.
- Nutritional rehabilitation is a critical component of long-term management for pediatric cystic fibrosis patients.
Abstract:
Previously, we reported catch-up weight gain, growth, and improved lung function in a group of malnourished cystic fibrosis (CF) children receiving aggressive nutritional supplementation for 1 year compared with a forced expiratory volume in 1 s (FEV1)-, height-, and sex-matched comparison group receiving standard therapy. To evaluate long-term effects, the clinical progress of both groups has been studied over a 5 year period. The supplemented group (n = 10) received supplements for a median of 1.35 years to achieve nutritional rehabilitation. Compared with the nonsupplemented group (n = 14), the previously supplemented group had lower mortality (2 vs. 4, N.S.) and significantly greater weight and height z scores at 4 and 5 years. The progression of pulmonary function abnormalities as measured by FEV1 and forced vital capacity (FVC) slopes was greater at 3 years in the nonsupplemented group (FEV1, p less than 0.05) but no significant differences in rates of deterioration of pulmonary function were seen after 5 years in the two groups of survivors. We conclude that intensive nutritional support for 1 year has both short- and long-term effects on nutrition and growth, still evident some years after the cessation of this therapeutic modality. Supplementation for periods of longer than 1 year may produce greater gains and possibly prolong the improvement in pulmonary function observed in the earlier study.