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    Energy metabolism in infants with cystic fibrosis

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    26
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    Author
    Bines, JE; Truby, HD; Armstrong, DS; Phelan, PD; Grimwood, K
    Date
    2002-05-01
    Source Title
    JOURNAL OF PEDIATRICS
    Publisher
    MOSBY-ELSEVIER
    University of Melbourne Author/s
    Bines, Julie; Grimwood, Keith
    Affiliation
    Paediatrics Royal Children'S Hospital
    Metadata
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    Document Type
    Journal Article
    Citations
    Bines, J. E., Truby, H. D., Armstrong, D. S., Phelan, P. D. & Grimwood, K. (2002). Energy metabolism in infants with cystic fibrosis. JOURNAL OF PEDIATRICS, 140 (5), pp.527-533. https://doi.org/10.1067/mpd.2002.123284.
    Access Status
    This item is currently not available from this repository
    URI
    http://hdl.handle.net/11343/26174
    DOI
    10.1067/mpd.2002.123284
    Description

    C1 - Journal Articles Refereed

    Abstract
    OBJECTIVE: To determine whether a defect in energy metabolism exists in infants with cystic fibrosis (CF). DESIGN: Unselected, newly-diagnosed subjects with CF (n = 46) and 24 healthy infants aged <20 weeks had measurements of resting energy expenditure (REE), total energy expenditure (TEE) (n = 25), and body composition. Metabolizable energy intake (MEI) was calculated. Genotype, energy intake, and pancreatic status was determined in all subjects with CF, and 24 underwent bronchial lavage. RESULTS: At diagnosis, infants with CF detected by newborn screening had significant anthropometric deficits (mean [SD] z-weight = 0.5 [1.0], z-length = 0.7 [1.3]) associated with pancreatic insufficiency. Their REE, TEE, or MEI (absolute measurements, per unit body weight or fat-free mass) were not increased. No relationship between REE, TEE, or MEI and Delta F(508) genotype, and no proportional differences in individual components of MEI between subjects with CF and controls, or between subjects with CF who were homozygotes or compound heterozygotes for Delta F(508) were observed. REE and TEE were not correlated with bronchial infection or inflammation. CONCLUSION: Growth impairment during the first weeks of life in infants with CF is associated with pancreatic insufficiency. However, there is no evidence for a defect of energy metabolism related to Delta F(508), and in infants with CF, minimal lung disease is unaccompanied by increased energy expenditure.
    Keywords
    Paediatrics; Nutrition

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