Transforming growth factor-beta(1) in bronchoalveolar lavage fluid from children with cystic fibrosis.

Academic Article


  • RATIONALE: Transforming factor beta(1) (TGF-beta(1)) genetic polymorphisms have been identified as a modifier of cystic fibrosis (CF) lung disease severity. However, few data link TGF-beta(1) protein levels and clinical markers of CF lung disease severity. OBJECTIVES: To determine the association between protein levels of TGF-beta(1) in pediatric CF bronchoalveolar lavage fluid (BALF) and clinical parameters of CF lung disease severity. METHODS: Total TGF-beta(1) was measured in BALF from 30 pediatric CF patients and 12 non-CF disease controls undergoing clinically indicated flexible bronchoscopy, and compared to four indicators of clinical disease: infection, inflammation, pulmonary function, and recent/recurrent hospitalization. RESULTS: TGF-beta(1) was elevated in CF BALF compared to non-CF controls (135 +/- 15 pg/ml vs. 57 +/- 10 pg/ml, P < 0.01). In CF BALF, increased TGF-beta(1) was associated with elevated BALF PMN % (r = 0.67, P < 0.01). BALF TGF-beta(1) was increased in CF subjects whose FEV(1) after the completion of antibiotic therapy remained below CF age-normative median values (205.9 +/- 20.5 pg/ml vs. 106.4 +/- 24.0, P = 0.01). BALF TGF-beta(1) was increased in CF children hospitalized in the previous year compared to those not recently hospitalized (169.9 +/- 21.6 pg/ml vs. 107.5 +/- 17.5 pg/ml, P = 0.04). Neither the presence of a bacterial pathogen nor bacterial quantity was associated with BALF TGF-beta(1). CONCLUSIONS: In CF, BALF TGF-beta(1) is elevated compared to non-CF controls. Increased BALF TGF-beta(1) is associated with neutrophilic inflammation, diminished lung function and recent hospitalization. Further investigation is needed to address mechanisms behind these associations.
  • Published In


  • Airway Remodeling, Biomarkers, Bronchoalveolar Lavage Fluid, Case-Control Studies, Child, Cross-Sectional Studies, Cystic Fibrosis, Disease Progression, Female, Humans, Inflammation, Male, Neutrophils, Prospective Studies, Respiratory Function Tests, Severity of Illness Index, Transforming Growth Factor beta1
  • Digital Object Identifier (doi)

    Author List

  • Harris WT; Muhlebach MS; Oster RA; Knowles MR; Noah TL
  • Start Page

  • 1057
  • End Page

  • 1064
  • Volume

  • 44
  • Issue

  • 11