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Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis

  • Ankitha Madde
    Affiliations
    Pediatric Pulmonary Medicine, UPMC, Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA 15224, USA

    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
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  • William Okoniewski
    Affiliations
    Pediatric Pulmonary Medicine, UPMC, Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA 15224, USA

    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
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  • Don B. Sanders
    Affiliations
    Division of Pediatric Pulmonology, Allergy, and Sleep Medicine, Riley Hospital for Children, Indianapolis, IN, USA

    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
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  • Clement L. Ren
    Affiliations
    Division of Pediatric Pulmonology, Allergy, and Sleep Medicine, Riley Hospital for Children, Indianapolis, IN, USA

    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
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  • Daniel J. Weiner
    Affiliations
    Pediatric Pulmonary Medicine, UPMC, Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA 15224, USA

    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
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  • Erick Forno
    Correspondence
    Corresponding author at: Pediatric Pulmonary Medicine, UPMC, Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA 15224, USA.
    Affiliations
    Pediatric Pulmonary Medicine, UPMC, Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA 15224, USA

    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    Search for articles by this author
Published:December 28, 2021DOI:https://doi.org/10.1016/j.jcf.2021.12.014

      Highlights

      • The association between BMI and FEV1 in CF differs significantly by pancreatic status (PS vs PI).
      • Higher BMI was associated with a smaller slope of FEV1 increase in PS-CF.
      • In PS-CF, BMI about the 85th percentile may be associated with decreasing FEV1.
      • The decline in FEV1 with age is minimal in PS-CF and does not differ by BMI.

      Abstract

      Background

      There is a strong association between nutrition and long-term FEV1 in cystic fibrosis (CF), but studies have been driven by data from subjects with pancreatic insufficiency (PI-CF). We thus evaluated the association between body mass index (BMI) and FEV1 percent-predicted (FEV1pp) in children with pancreatic sufficiency (PS-CF) and contrasted it with the association in PI-CF.

      Methods

      We utilized data from the CF Foundation Patient Registry. The cohort included children born 1995–2010, diagnosed <2 years of age, and who had annualized data on BMI percentile and FEV1pp at ages 6–16 years. Pancreatic status was defined based on pancreatic enzyme replacement therapy. The association between BMI and FEV1 was evaluated using linear and mixed-effects longitudinal regression.

      Results

      There were 424 children with PS-CF and 7,849 with PI-CF. The association between BMI and FEV1 differed significantly by pancreatic status: each 10-pct higher BMI was associated with 2% [95%CI = 1.9–2.1] higher FEV1pp in PI-CF, compared to just 0.9% [0.5–1.3] in PS-CF (PINTERACTION < 0.001). Within the at-risk nutritional category (BMI <25pct), each 10-pct higher BMI was associated with 5% higher FEV1pp in PI-CF, but no significant increase in PS-CF. Moreover, in PS-CF, overweight/obesity (BMI ≥85pct) was associated with decreasing FEV1pp. In addition, FEV1pp decline through age 20 years in youth with PS-CF was modest (-0.6% per year) and independent of BMI (BMI*age PINTERACTION = 0.37).

      Conclusions

      In children with PS-CF, BMI remains an important determinant of lung function. However, it may be less critical to attain a BMI >50th percentile; and BMI ≥85th percentile may be detrimental.

      Keywords

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