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Mapping Pulmonary and Systemic Inflammation in Preschool Aged Children With Cystic Fibrosis
Shivanthan Shanthikumar1,2,3, Sarath C Ranganathan1,2,3, Richard Saffery1,2
1Infection and Immunity Theme, Murdoch Children's Research Institute, Parkville, VIC, Australia.
Insights
Children with cystic fibrosis (CF) exhibit distinct pulmonary immune cell profiles and elevated inflammatory markers in their airways. This study reveals key differences in the immune landscape of pediatric CF lungs compared to healthy controls.
Area of Science:
- Pediatric Immunology
- Respiratory Medicine
- Inflammation Research
Background:
- The immune system in children's respiratory tracts is not well understood.
- Mechanisms behind common childhood respiratory diseases like cystic fibrosis (CF) are unclear.
Purpose of the Study:
- To characterize the local and systemic immune response in preschool children with CF.
- To compare immune profiles in CF patients versus age-matched healthy children.
Main Methods:
- Utilized high-parameter flow cytometry and inflammatory cytokine profiling.
- Analyzed bronchoalveolar lavage (BAL) fluid and whole blood samples.
- Employed advanced analytical pipelines for immune profiling.
Main Results:
- Children with CF showed increased pulmonary granulocytes (CD66b+) and elevated BAL cytokines (MIP-1α, MIG, MCP-1, IL-8, IL-6).
- Systemic neutrophils correlated positively with age in CF, while T and B cells correlated inversely.
- Inflammatory cells in BAL exhibited higher activation/migration markers than systemic cells in both groups.
Conclusions:
- Multiplex immune profiling effectively maps childhood lung disease mechanisms.
- Identified specific immune signatures associated with pediatric cystic fibrosis.
- Highlighted differences in local versus systemic immune cell activation.
Abstract:
The immune landscape of the paediatric respiratory system remains largely uncharacterised and as a result, the mechanisms of globally important childhood respiratory diseases remain poorly understood. In this work, we used high parameter flow cytometry and inflammatory cytokine profiling to map the local [bronchoalveolar lavage (BAL)] and systemic (whole blood) immune response in preschool aged children with cystic fibrosis (CF) and aged-matched healthy controls. We demonstrate that children with CF show pulmonary infiltration of CD66b+ granulocytes and increased levels of MIP-1α, MIG, MCP-1, IL-8, and IL-6 in BAL relative to healthy control children. Proportions of systemic neutrophils positively correlated with age in children with CF, whilst systemic CD4 T cells and B cells were inversely associated with age. Inflammatory cells in the BAL from both CF and healthy children expressed higher levels of activation and migration markers relative to their systemic counterparts. This work highlights the utility of multiplex immune profiling and advanced analytical pipelines to understand mechanisms of lung disease in childhood.
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