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

Elias Seidl1, Hannah Kiermeier1, Gerhard Liebisch2

  • 1Hauner Children's Hospital, Ludwig Maximilians University, German Center for Lung Research (DZL), Lindwurmstr. 4a, D-80337 Munich, Germany.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|April 29, 2019
PubMed

Insights

Lipid profiles in broncho-alveolar lavage fluid differ in children with cystic fibrosis (CF) compared to protracted bacterial bronchitis (PBB) and controls. Alterations in CF are linked to inflammatory cell membranes, not surfactant function.

Area of Science:

  • Pulmonary Medicine
  • Biochemistry
  • Pediatric Respiratory Diseases

Background:

  • Pulmonary surfactant composition is vital for normal breathing.
  • Children with cystic fibrosis (CF) and protracted bacterial bronchitis (PBB) may have altered lipid profiles in broncho-alveolar lavage (BAL) fluid.
  • Distinguishing disease-specific changes from those due to inflammatory cells is crucial.

Purpose of the Study:

  • To compare lipid profiles in BAL fluid from children with CF, PBB, and healthy controls.
  • To determine if lipid alterations in CF are disease-driven or secondary to inflammation.
  • To investigate the role of inflammatory cell lipids in CF airway changes.

Main Methods:

  • Lipidomics profiling of BAL fluid using electrospray ionization tandem mass spectrometry.
  • Analysis of BAL cell differential and counts.
  • Comparison of lipid composition across pediatric groups: CF, PBB, and controls.

Main Results:

  • Total lipid quantities and major phospholipid fractions were similar across groups.
  • Children with CF showed increased free cholesterol and decreased cholesteryl esters.
  • CF patients had higher proportions of non-surfactant membrane lipids (PE P, PE, PS) and lower phosphatidylcholine (PC) compared to controls; PBB showed no such changes.
  • Key surfactant lipids (PC 32:0, PG) remained unchanged in CF patients.

Conclusions:

  • Lipid profile alterations in cystic fibrosis (CF) BAL fluid are characteristic of inflammatory cell membranes, not surfactant dysfunction.
  • These changes in CF are likely due to prolonged inflammation, unlike the shorter process in PBB.
  • The study differentiates lipid changes caused by CF disease progression versus secondary inflammatory responses.
Abstract

Keywords:
Bronchoalveolar lavageChildrenCystic fibrosisInfantsLipids

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