title: Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease.
abstract: BACKGROUND
  Abnormalities of the intracellular metabolism of the hydrophobic surfactant proteins SP-B and SP-C and their precursors may be causally linked to chronic childhood diffuse lung diseases. The profile of these proteins in the alveolar space is unknown in such subjects.
  
  
  METHODS
  We analyzed bronchoalveolar lavage fluid by Western blotting for SP-B, SP-C and their proforms in children with pulmonary alveolar proteinosis (PAP, n = 15), children with no SP-B (n = 6), children with chronic respiratory distress of unknown cause (cRD, n = 7), in comparison to children without lung disease (n = 15) or chronic obstructive bronchitis (n = 19).
  
  
  RESULTS
  Pro-SP-B of 25-26 kD was commonly abundant in all groups of subjects, suggesting that their presence is not of diagnostic value for processing defects. In contrast, pro-SP-B peptides cleaved off during intracellular processing of SP-B and smaller than 19-21 kD, were exclusively found in PAP and cRD. In 4 of 6 children with no SP-B, mutations of SFTPB or SPTPC genes were found. Pro-SP-C forms were identified at very low frequency. Their presence was clearly, but not exclusively associated with mutations of the SFTPB and SPTPC genes, impeding their usage as candidates for diagnostic screening.
  
  
  CONCLUSIONS
  Immuno-analysis of the hydrophobic surfactant proteins and their precursor forms in bronchoalveolar lavage is minimally invasive and can give valuable clues for the involvement of processing abnormalities in pediatric pulmonary disorders.
authors.affiliation: Kinderklinik and Poliklinik, Dr. von Haunersches Kinderspital, Ludwig-Maximilians University, Munich, Germany. matthias.griese@med.uni-muenchen.de
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
authors.forename: Silja
authors.initials: S
authors.lastname: Schumacher
authors.forename: Mohammed
authors.initials: M
authors.lastname: Tredano
authors.forename: Manuela
authors.initials: M
authors.lastname: Steinecker
authors.forename: Annika
authors.initials: A
authors.lastname: Braun
authors.forename: Susan
authors.initials: S
authors.lastname: Guttentag
authors.forename: Michael F
authors.initials: MF
authors.lastname: Beers
authors.forename: Michel
authors.initials: M
authors.lastname: Bahuau
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000032:analysis; D029481:Bronchitis, Chronic / Q000175:diagnosis* / Q000378:metabolism*; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D005260:Female; D020869:Gene Expression Profiling; D006801:Humans; D057927:Hydrophobic and Hydrophilic Interactions; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D037701:Pulmonary Surfactant-Associated Protein B / Q000032:analysis*; D037721:Pulmonary Surfactant-Associated Protein C / Q000032:analysis*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
chemical_list: D015415:Biomarkers; D037701:Pulmonary Surfactant-Associated Protein B; D037721:Pulmonary Surfactant-Associated Protein C