Limits...
Surfactant lipidomics in healthy children and childhood interstitial lung disease.

Griese M, Kirmeier HG, Liebisch G, Rauch D, Stückler F, Schmitz G, Zarbock R, ILD-BAL working group of the Kids-Lung-Regist - PLoS ONE (2015)

Bottom Line: Two reference populations were compared to a broad range of children with ILD.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.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.

View Article: PubMed Central - PubMed

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.

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.

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Related in: MedlinePlus

Calculation and expression of the results of the lipid analysis.All analyzed lipids are the sum of all analyzed phospholipid classes, cholesteryl ester and free cholesterol (all expressed as nmol/ml). Phospholipid classes are expressed as % of all analyzed phospholipids and the species as percentage of that phospholipid class. In the body of the manuscript mainly the results of all lipid classes and the composition of one of its classes, i.e. phosphatidylcholine, are indicated. Composition of the other classes are presented in the supplemental materials. For overview see also panel A in S1 Fig.
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pone.0117985.g001: Calculation and expression of the results of the lipid analysis.All analyzed lipids are the sum of all analyzed phospholipid classes, cholesteryl ester and free cholesterol (all expressed as nmol/ml). Phospholipid classes are expressed as % of all analyzed phospholipids and the species as percentage of that phospholipid class. In the body of the manuscript mainly the results of all lipid classes and the composition of one of its classes, i.e. phosphatidylcholine, are indicated. Composition of the other classes are presented in the supplemental materials. For overview see also panel A in S1 Fig.

Mentions: Results are presented as total lipids, total phospholipids, cholesteryl ester and free cholesterol (all expressed as nmol/ml) (Fig. 1, panel A in S1 Fig.). Species were only taken into consideration for presentation if the species had an abundance of ≥ 0.5%. To guarantee quality of analysis and to eliminate a sample factor, diluted bronchoalveolar lavages with a total lipid concentration < 15 μmol/l or a phospholipid concentration < 10 μmol/l were excluded from calculations, as most lipid species are below or close to the limit of detection. In total 28 of the 143 subjects were excluded from the final analysis, as detailed in Table 1.


Surfactant lipidomics in healthy children and childhood interstitial lung disease.

Griese M, Kirmeier HG, Liebisch G, Rauch D, Stückler F, Schmitz G, Zarbock R, ILD-BAL working group of the Kids-Lung-Regist - PLoS ONE (2015)

Calculation and expression of the results of the lipid analysis.All analyzed lipids are the sum of all analyzed phospholipid classes, cholesteryl ester and free cholesterol (all expressed as nmol/ml). Phospholipid classes are expressed as % of all analyzed phospholipids and the species as percentage of that phospholipid class. In the body of the manuscript mainly the results of all lipid classes and the composition of one of its classes, i.e. phosphatidylcholine, are indicated. Composition of the other classes are presented in the supplemental materials. For overview see also panel A in S1 Fig.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4333572&req=5

pone.0117985.g001: Calculation and expression of the results of the lipid analysis.All analyzed lipids are the sum of all analyzed phospholipid classes, cholesteryl ester and free cholesterol (all expressed as nmol/ml). Phospholipid classes are expressed as % of all analyzed phospholipids and the species as percentage of that phospholipid class. In the body of the manuscript mainly the results of all lipid classes and the composition of one of its classes, i.e. phosphatidylcholine, are indicated. Composition of the other classes are presented in the supplemental materials. For overview see also panel A in S1 Fig.
Mentions: Results are presented as total lipids, total phospholipids, cholesteryl ester and free cholesterol (all expressed as nmol/ml) (Fig. 1, panel A in S1 Fig.). Species were only taken into consideration for presentation if the species had an abundance of ≥ 0.5%. To guarantee quality of analysis and to eliminate a sample factor, diluted bronchoalveolar lavages with a total lipid concentration < 15 μmol/l or a phospholipid concentration < 10 μmol/l were excluded from calculations, as most lipid species are below or close to the limit of detection. In total 28 of the 143 subjects were excluded from the final analysis, as detailed in Table 1.

Bottom Line: Two reference populations were compared to a broad range of children with ILD.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.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.

View Article: PubMed Central - PubMed

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.

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.

Show MeSH
Related in: MedlinePlus