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J Cyst Fibros. 2019 Apr 24. pii: S1569-1993(18)30860-9. doi: 10.1016/j.jcf.2019.04.012. [Epub ahead of print]

Lavage lipidomics signatures in children with cystic fibrosis and protracted bacterial bronchitis.

Author information

Hauner Children's Hospital, Ludwig Maximilians University, German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.
Institute of Clinical Chemistry and Laboratory Medicine, Regensburg University Hospital, Germany.
Department of Pediatric Pulmonology, University Rostock, Germany.
Praxis Prof. Karl Paul, Schöneberger Ufer 69, 10785 Berlin, Germany.
Hospital for Sick Children, University of Toronto, Canada.
CF-Centre, Faculty of Medicine, University of Cologne, Germany.
Hauner Children's Hospital, Ludwig Maximilians University, German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany. Electronic address:



Balanced composition of a well-functioning pulmonary surfactant is crucial and essential for normal breathing. Here, we explored whether the composition of lipids recovered by broncho-alveolar lavage (BAL) in children with cystic fibrosis (CF) differ from children with protracted bacterial bronchitis (PBB) and controls. We wanted to differentiate, if alterations are primarily caused by the disease process or secondary due to an increased amount of cell-membrane lipids derived from inflammatory cells.


Comprehensive lipidomics profiles of BAL fluid from children diagnosed with CF, PBB and controls were generated by electrospray ionization tandem mass spectrometry analysis. BAL cell differential and numbers were examined.


55 children (37 patients with CF, 8 children with PBB and 10 controls) were included in this study. Results showed comparable total quantities of lipids in all groups. Phospholipids were the major lipid fraction and similar in all groups, whereas the fractions of cholesteryl esters were less and of free cholesterol were increased in children with CF. Among the phospholipids, patients with CF had higher proportion of the non-surfactant membrane-lipids in the classes phosphatidylethanolamine based plasmalogens (PE P), phosphatidylethanolmine (PE) and phosphatidylserine (PS), but a lower proportion of phosphatidylcholine (PC) compared to healthy controls. No such changes were identified in the BAL fluid of children diagnosed with PBB. No differences were observed for the surfactant lipids dipalmitoyl-phosphatidylcholin (PC 32:0) and phosphatidylglycerol (PG).


In CF patients with neutrophilic airway inflammation the lipid composition for surfactant phospholipid components were unchanged, whereas alteration in lipid profile were characteristic for those found in membranes of inflammatory cells. We suspect that the changes in CF were caused by the prolonged inflammation in contrast to a relatively short standing process in PBB.


Bronchoalveolar lavage; Children; Cystic fibrosis; Infants; Lipids


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