HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Fung, E. B. Articles by Stallings, V. A. Search for Related Content PubMed PubMed Citation Articles by Fung, E. B. Articles by Stallings, V. A.

A Six-Month Study of Growth and Energy Expenditure in Children with Cystic Fibrosis Taking a Pulmonary Inhalation Medication (rhDNase)

Division of Gastroenterology and Nutrition (E.B.F., E.M.B., D.W., B.S.Z., V.A.S.), The Children’s Hospital of Philadelphia, Pennsylvania, State University of New York, Stony Brook, New York
The Cystic Fibrosis Center (B.T.H., T.F.S.), The Children’s Hospital of Philadelphia, Pennsylvania, State University of New York, Stony Brook, New York
Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, State University of New York (D.W.), Stony Brook, New York

Address reprint requests to: Ellen B. Fung, PhD, RD, School of Nursing, Nursing Education Building, Room 137, 420 Guardian Drive, University of Pennsylvania, Philadelphia, PA 19104-6096.

Objective: To characterize the effects of recombinant human deoxyribonuclease (rhDNase) on growth velocity, body composition, resting energy expenditure (REE) and food intake in children with cystic fibrosis (CF).

Methods: A prospective, six-month pilot study was conducted in twenty-one subjects with CF (twelve male, nine female, ages 11.5±3.1 years) measured at baseline, two and six months post-baseline. Repeated measures ANOVA was used to examine the change in variables across time.

Results: The majority (75%) of subjects had minimal lung disease at baseline (FEV₁: 80%–119% predicted). As expected for growing children, weight and height gains (1.6 kg and 2.5 cm) were observed between baseline and six months (p=0.0001). No change was observed in weight z-scores from six months prior to initiation of rhDNase therapy to six months post, though a significant decline (p=0.049) in Ht z-score was observed over this twelve-month period. Triceps skinfolds and mid-arm muscle circumference increased from baseline to six months (p<0.01); respective z-scores remained stable. Energy intake remained constant during the period it was studied from baseline to two months of therapy: 120%±27% RDA. REE, though slightly elevated compared to healthy children (baseline 106%±8% predicted), remained stable throughout the study and at a level which may be expected for children with minimal lung disease. A trend (p=0.057) towards a decrease in the number of subjects requiring hospitalization for pulmonary exacerbations during the trial period was observed.

Conclusions: In summary, these pilot data from younger children with milder CF-related lung disease do not confirm anecdotal reports of improved rate of weight gain, caloric intake or decreases in the elevated REE. Future research might focus on documentation of the possible nutritional effects of rhDNase in clinical trials of children with more severe lung disease.

Key words: cystic fibrosis, dietary intake, growth, Pulmozyme®, pulmonary function, rhDNase, resting energy expenditure

Abbreviations: CF=cystic fibrosis • FEV₁=forced expiratory volume in 1 second • FEF_25%–75%=forced expiratory flow at 25%–75% of vital capacity • FFM=fat-free mass • FVC=forced vital capacity • GCRC=General Clinical Research Center • rhDNase=recombinant human deosyribonuclease commercially known as Pulmozyme® • RDA=Recommended Dietary Allowance • REE=resting energy expenditure • WHO=World Health Organization