Abstract
ObjectiveTo review the literature addressing the relationship of growth and nutritional parameters with pulmonary function in pediatric patients with cystic fibrosis. Data sourceA collection of articles published in the last 15 years in English, Portuguese and Spanish was made by research in electronic databases – PubMed, Cochrane, Medline, Lilacs and Scielo – using the keywords cystic fibrosis, growth, nutrition, pulmonary function in varied combinations. Articles that addressed the long term association of growth and nutritional parameters, with an emphasis on growth, with pulmonary disease in cystic fibrosis, were included, and we excluded those that addressing only the relationship between nutritional parameters and cystic fibrosis and those in which the aim was to describe the disease. Data synthesisSeven studies were included, with a total of 12,455 patients. Six studies reported relationship between growth parameters and lung function, including one study addressing the association of growth parameters, solely, with lung function, and all the seven studies reported relationship between nutritional parameters and lung function. ConclusionsThe review suggests that the severity of the lung disease, determined by spirometry, is associated with body growth and nutritional status in cystic fibrosis. Thus, the intervention in these parameters can lead to the better prognosis and life expectancy for cystic fibrosis patients.
Highlights
Cystic Fibrosis (CF) is the most common lethal genetic disease in Caucasian populations
This study showed a progressive loss of FEV1 in patients with mild and severe lung disease
Height and height velocity at all the four growth milestones were considered as a whole, the differences between patients with mild and severe disease were highly significant
Summary
Cystic Fibrosis (CF) is the most common lethal genetic disease in Caucasian populations. By evaluating titles and abstracts, the following recuperation criteria for complete articles were: studies of cohort, longitudinal, cross-sectional, descriptive and prospective, which results evaluated the relationship among pulmonary function and growth parameters in CF patients, excluding those which, despite of appearing in the search results, did not address the subject under this point of view. In this stage, 27 papers were screened. The review was concluded with the reading of the complete articles, and, in the final manuscript, seven articles were included,12---18 all of them in English (Fig. 1; Tables 1 and 2)
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