title: Transforming growth factor-beta(1) in bronchoalveolar lavage fluid from children with cystic fibrosis.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Division of Pulmonology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama, USA. tharris@peds.uab.edu
authors.forename: William T
authors.initials: WT
authors.lastname: Harris
authors.forename: Marianne S
authors.initials: MS
authors.lastname: Muhlebach
authors.forename: Robert A
authors.initials: RA
authors.lastname: Oster
authors.forename: Michael R
authors.initials: MR
authors.lastname: Knowles
authors.forename: Terry L
authors.initials: TL
authors.lastname: Noah
mesh_terms: D056151:Airway Remodeling / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry*; D016022:Case-Control Studies; D002648:Child; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000276:immunology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007249:Inflammation / Q000276:immunology*; D008297:Male; D009504:Neutrophils; D011446:Prospective Studies; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D053773:Transforming Growth Factor beta1 / Q000032:analysis*
chemical_list: D015415:Biomarkers; D053773:Transforming Growth Factor beta1