title: Surfactant lipidomics in healthy children and childhood interstitial lung disease.
abstract: BACKGROUND
  Lipids account for the majority of pulmonary surfactant, which is essential for normal breathing. We asked if interstitial lung diseases (ILD) in children may disrupt alveolar surfactant and give clues for disease categorization.
  
  
  METHODS
  Comprehensive lipidomics profiles of broncho-alveolar lavage fluid were generated in 115 children by electrospray ionization tandem mass spectrometry (ESI-MS/MS). Two reference populations were compared to a broad range of children with ILD.
  
  
  RESULTS
  Class and species composition in healthy children did not differ from that in children with ILD related to diffuse developmental disorders, chronic tachypnoe of infancy, ILD related to lung vessels and the heart, and ILD related to reactive lymphoid lesions. As groups, ILDs related to the alveolar surfactant region, ILD related to unclear respiratory distress syndrome in the mature neonate, or in part ILD related to growth abnormalities reflecting deficient alveolarisation, had significant alterations of some surfactant specific phospholipids. Additionally, lipids derived from inflammatory processes were identified and differentiated. In children with ABCA3-deficiency from two ILD causing mutations saturated and monounsaturated phosphatidylcholine species with 30 and 32 carbons and almost all phosphatidylglycerol species were severely reduced. In other alveolar disorders lipidomic profiles may be of less diagnostic value, but nevertheless may substantiate lack of significant involvement of mechanisms related to surfactant lipid metabolism.
  
  
  CONCLUSIONS
  Lipidomic profiling may identify specific forms of ILD in children with surfactant alterations and characterized the molecular species pattern likely to be transported by ABCA3 in vivo.
authors.affiliation: Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
authors.affiliation: Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.
authors.forename: Hannah G
authors.initials: HG
authors.lastname: Kirmeier
authors.affiliation: Institute of Computational Biology, Helmholtz Center Munich, Neuherberg, Germany.
authors.forename: Gerhard
authors.initials: G
authors.lastname: Liebisch
authors.affiliation: Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.
authors.forename: Daniela
authors.initials: D
authors.lastname: Rauch
authors.affiliation: Institute of Computational Biology, Helmholtz Center Munich, Neuherberg, Germany.
authors.forename: Ferdinand
authors.initials: F
authors.lastname: Stückler
authors.affiliation: Institute of Clinical Chemistry and Laboratory Medicine, University of Regensburg, Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany.
authors.forename: Gerd
authors.initials: G
authors.lastname: Schmitz
authors.affiliation: Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Zarbock
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics / Q000378:metabolism; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008055:Lipids / Q000032:analysis*; D017563:Lung Diseases, Interstitial / Q000378:metabolism*; D008297:Male; D011663:Pulmonary Surfactants / Q000378:metabolism*; D021241:Spectrometry, Mass, Electrospray Ionization; D053719:Tandem Mass Spectrometry
chemical_list: C441524:ABCA3 protein, human; D018528:ATP-Binding Cassette Transporters; D008055:Lipids; D011663:Pulmonary Surfactants