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Surfactant lipidomics in healthy children and childhood interstitial lung disease
Matthias Griese1, Hannah G Kirmeier1, Gerhard Liebisch2
1Department 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.
Insights
Lipidomic profiling of children
Area of Science:
- Pediatric Pulmonology
- Biochemistry
- Medical Diagnostics
Background:
- Pulmonary surfactant, primarily lipids, is vital for breathing.
- Interstitial lung diseases (ILD) in children may disrupt alveolar surfactant.
- Investigating surfactant alterations can aid in ILD categorization.
Purpose of the Study:
- To determine if childhood interstitial lung diseases (ILD) alter alveolar surfactant composition.
- To explore the potential of lipidomics for classifying pediatric ILD.
- To identify specific lipid signatures associated with different ILD types.
Main Methods:
- Lipidomics profiling of broncho-alveolar lavage fluid using electrospray ionization tandem mass spectrometry (ESI-MS/MS).
- Analysis of 115 children, comparing broad ILD cases to reference populations.
- Detailed examination of surfactant-specific phospholipids and inflammatory lipids.
Main Results:
- No significant differences in lipid composition were found in ILDs related to developmental disorders, infant tachypnoea, or cardiac/lymphoid issues.
- Specific ILDs, including alveolar surfactant region disorders and neonatal respiratory distress, showed significant alterations in surfactant phospholipids.
- ABCA3-deficiency mutations led to severe reductions in specific phosphatidylcholine and phosphatidylglycerol species.
Conclusions:
- Lipidomic profiling can identify pediatric ILDs with surfactant alterations.
- This technique characterizes molecular patterns associated with ABCA3 transporter function.
- While not universally diagnostic, lipidomics can rule out surfactant metabolism involvement in some alveolar disorders.
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.
