Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis

doi:10.1067/mpd.2003.152

The Journal of Pediatrics

Volume 142, Issue 6, June 2003, Pages 624-630

https://doi.org/10.1067/mpd.2003.152 Get rights and content

Abstract

Objective To determine the relation of growth and nutritional status to pulmonary function in young children with cystic fibrosis (CF). Study design The relation of weight-for-age (WFA), height-for-age (HFA), percent ideal body weight (%IBW), and signs of lung disease at age 3 years with pulmonary function at age 6 years was assessed in 931 patients with CF. Associations of changes in WFA from age 3 to 6 on pulmonary function were also assessed. Results WFA, HFA, and %IBW were poorly associated with lung disease at age 3 years, but all were strongly associated with pulmonary function at age 6 years. Those with WFA below the 5th percentile at age 3 had lower pulmonary function at age 6 compared with those above the 75th percentile (FEV₁: 86 ± 20 [SD] versus 102 ± 18 % predicted, respectively). Pulmonary function was highest in those whose WFA remained >10th percentile from age 3 to 6 (FEV₁: 100 ± 19 % predicted) and lowest in those who remained <10th percentile (84 ± 21 % predicted). Patients with signs and symptoms of lung disease at age 3 years had lower pulmonary function at age 6 years. Conclusions Aggressive intervention early in life aimed at growth and nutrition and/or lung disease may affect pulmonary function. (J Pediatr 2003;142:624-30)

Section snippets

Methods

Patients in this analysis were drawn from the Epidemiologic Study of Cystic Fibrosis (ESCF), a prospective encounter-based study designed to characterize the natural history of pulmonary disease and growth in a large population of patients with CF (n = 24,863) in the United States and Canada.¹² The sample was based on 1481 patients from 139 study sites who were enrolled in ESCF and who had reached the age of 3 years between 1994 and 1996. Of these 1481 patients, 931 were followed until at least

Results

At the time of this analysis, 1481 patients from 139 study sites were enrolled in ESCF by age 3.5 years. The number of patients per site ranged from 1 to 37 with an average of 6.7 patients, with 31 sites having 10 or more patients and 8 sites having 22 or more patients. Of these 1481 patients, 1108 had complete follow-up data for growth and signs and symptoms of lung disease to age 6 years. Of these, 931 (470 boys, 461 girls) had at least one pulmonary function test recorded between age 5.5 and

Discussion

This study demonstrates that in children with CF, lower indexes of growth and nutrition at age 3 years are highly associated with lower pulmonary function at age 6 years—when it is first possible to measure pulmonary function in a reliable manner in most individuals. Signs and symptoms of lung disease at age 3 are also associated with lower pulmonary function at age 6 years. Nevertheless, indexes of growth and nutrition at age 3 years are strong predictors of pulmonary function at age 6 years

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The present study used a qualitative approach to explore the perceptions and experiences of caregivers of cwCF with G-tube introductions and recommendations, as well as factors influencing G-tube decision-making.
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In cystic fibrosis (CF), pathophysiologic changes in the gastrointestinal tract lead to malnutrition and altered gut microbiome. Microbiome alterations have been linked to linear growth, gut inflammation and respiratory manifestations. Elucidating these gut microbiome alterations may provide insight into future nutritional management in CF.
Infants were followed for 12-months at four sites in the United States (US-CF) and Australia (AUS-CF). 16S rRNA gene sequencing was performed on longitudinal stool samples. Associations between microbial abundance and age, antibiotic prophylaxis, malnutrition, and breast feeding were evaluated using generalized linear mixed models. Taxonomic and predictive functional features were compared between groups.
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Weight status, dietary intake, pulmonary function, and lipid profile differed significantly in children with CF by pancreatic status. Nevertheless, the percentage of overweight and obesity was higher in children with PS than in those with PI. To avoid obesity, dietary recommendations for a high-calorie, high-fat diet should be reconsidered in patients with CF regarding their pancreatic status.
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^☆: Genentech, Inc, funds the Epidemiologic Study of Cystic Fibrosis.

^☆☆: Drs Konstan, Wohl, Wagener, and Morgan are members of the scientific advisory board for the Epidemiologic Study of Cystic Fibrosis and have received grants and other support from Genentech, Inc.

^★: Reprint requests: Michael W. Konstan, MD, Rainbow Babies and Children's Hospital, 11100 Euclid Ave, Cleveland, OH 44106. E-mail: [email protected] .

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Original ArticlesGrowth and nutritional indexes in early life predict pulmonary function in cystic fibrosis☆,☆☆,★

Abstract

Section snippets

Methods

Results

Discussion

J Pediatr

J Pediatr

J Pediatr

J Pediatr

Am J Clin Nutr

J Clin Epidemiol

J Pediatr

Cystic fibrosis: state of the art

Am J Respir Crit Care Med

Nutritional growth retardation is associated with defective lung growth in cystic fibrosis: a preventable determinant of progressive pulmonary dysfunction

Nutrition

Prospective evaluation of resting energy expenditure, nutritional status, pulmonary function, and genotype in children with cystic fibrosis

Pediatr Res

Nutritional state and lung disease in cystic fibrosis

Neth J Med

The interrelationship of nutrition and pulmonary function in patients with cystic fibrosis

Curr Opin Pulm Med

Original Articles
Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis☆,☆☆,★