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Circulating markers to assess nutritional therapy in cystic fibrosis.

Abstract

Cystic fibrosis (CF) is the most commonly occurring lethal autosomal recessive disorder. The gene defect causes defective sodium and chloride transport across epithelial cells of the respiratory, hepatobiliary, gastrointestinal and reproductive tracts, resulting in thick mucus secretions. In the respiratory tract, mucus traps bacteria, causing repeated lung infections, progressive bronchiectasis and eventual death due to respiratory failure. In the gastrointestinal tract, mucus prevents pancreatic enzymes reaching the gut, leading to nutrient malabsorption. Careful nutritional management has a dramatic effect on growth and survival rates in CF. Appropriate nutritional support includes pancreatic enzyme replacement therapy, a high-fat/high-energy diet and essential nutrient supplementation, specifically fat-soluble vitamins and essential fatty acids (EFA). Long-term studies are required to examine the effects of nutritional interventions on key clinical outcomes in CF, such as the rate of decline of lung function. The use of circulating markers to assess the influence of nutritional therapy allows short-term intervention studies to predict the potential for clinical improvements. This article provides an overview of the biomarkers useful in the prediction of the efficacy of nutritional therapy on improvements in quality and quantity of life in CF.

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  • Authors+Show Affiliations

    ,

    Department of Respiratory and Sleep Medicine, Hunter Medical Research Institute, John Hunter Hospital, Newcastle, 2310, NSW, Australia.

    ,

    Source

    MeSH

    Cystic Fibrosis
    Humans
    Nutritional Status

    Pub Type(s)

    Journal Article
    Research Support, Non-U.S. Gov't
    Review

    Language

    eng

    PubMed ID

    15698587

    Citation

    Wood, Lisa G., et al. "Circulating Markers to Assess Nutritional Therapy in Cystic Fibrosis." Clinica Chimica Acta; International Journal of Clinical Chemistry, vol. 353, no. 1-2, 2005, pp. 13-29.
    Wood LG, Gibson PG, Garg ML. Circulating markers to assess nutritional therapy in cystic fibrosis. Clin Chim Acta. 2005;353(1-2):13-29.
    Wood, L. G., Gibson, P. G., & Garg, M. L. (2005). Circulating markers to assess nutritional therapy in cystic fibrosis. Clinica Chimica Acta; International Journal of Clinical Chemistry, 353(1-2), pp. 13-29.
    Wood LG, Gibson PG, Garg ML. Circulating Markers to Assess Nutritional Therapy in Cystic Fibrosis. Clin Chim Acta. 2005;353(1-2):13-29. PubMed PMID: 15698587.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Circulating markers to assess nutritional therapy in cystic fibrosis. AU - Wood,Lisa G, AU - Gibson,Peter G, AU - Garg,Manohar L, PY - 2004/09/15/received PY - 2004/11/03/accepted PY - 2005/2/9/pubmed PY - 2005/4/23/medline PY - 2005/2/9/entrez SP - 13 EP - 29 JF - Clinica chimica acta; international journal of clinical chemistry JO - Clin. Chim. Acta VL - 353 IS - 1-2 N2 - Cystic fibrosis (CF) is the most commonly occurring lethal autosomal recessive disorder. The gene defect causes defective sodium and chloride transport across epithelial cells of the respiratory, hepatobiliary, gastrointestinal and reproductive tracts, resulting in thick mucus secretions. In the respiratory tract, mucus traps bacteria, causing repeated lung infections, progressive bronchiectasis and eventual death due to respiratory failure. In the gastrointestinal tract, mucus prevents pancreatic enzymes reaching the gut, leading to nutrient malabsorption. Careful nutritional management has a dramatic effect on growth and survival rates in CF. Appropriate nutritional support includes pancreatic enzyme replacement therapy, a high-fat/high-energy diet and essential nutrient supplementation, specifically fat-soluble vitamins and essential fatty acids (EFA). Long-term studies are required to examine the effects of nutritional interventions on key clinical outcomes in CF, such as the rate of decline of lung function. The use of circulating markers to assess the influence of nutritional therapy allows short-term intervention studies to predict the potential for clinical improvements. This article provides an overview of the biomarkers useful in the prediction of the efficacy of nutritional therapy on improvements in quality and quantity of life in CF. SN - 0009-8981 UR - https://www.unboundmedicine.com/medline/citation/15698587/full_citation L2 - https://linkinghub.elsevier.com/retrieve/pii/S0009-8981(04)00532-7 DB - PRIME DP - Unbound Medicine ER -