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Related Experiment Videos

Complement in cystic fibrosis.

M Götz, G Lubec

    European Journal of Pediatrics
    |January 17, 1978
    PubMed
    Summary
    This summary is machine-generated.

    Cystic fibrosis patients show decreased complement components C3 and C4, unlike previous findings. Pseudomonas aeruginosa infection is linked to these complement defects, suggesting pulmonary infection as the likely cause.

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    Area of Science:

    • Immunology
    • Pulmonology
    • Biochemistry

    Background:

    • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including the lungs.
    • The role of complement system activation in CF pathogenesis is not fully understood.
    • Previous studies have yielded conflicting results regarding complement levels in CF patients.

    Purpose of the Study:

    • To investigate complement component levels (C3, C4, C3A) in cystic fibrosis patients.
    • To explore the relationship between complement levels, clinical scores, sputum microbiology, and patient sex.
    • To determine the potential involvement of complement activation pathways in CF.

    Main Methods:

    • Quantification of complement components C3, C4, and C3A in 30 CF patients (ages 1-21) and 40 healthy controls.

    Related Experiment Videos

  • Correlation analysis with Shwachman clinical scores, sputum microbiology (including Pseudomonas aeruginosa), and patient sex.
  • Assessment of alternative pathway activation.

    • Main Results:

    • Significant reductions in C3 (P < 0.001) and C4 (P < 0.02) were observed in CF patients compared to controls.
    • C3A levels remained unaltered; no complement component increases were detected.
    • Pseudomonas aeruginosa in sputum was associated with complement defects (14/18 patients).
    • Alternative pathway activation was detected in 32% of patients.
    • Clinical scores, disease exacerbations, and patient sex did not influence complement levels.

    Conclusions:

    • The study demonstrates decreased C3 and C4 levels in CF patients, contrary to some prior reports.
    • Pulmonary infection, particularly with Pseudomonas aeruginosa, is strongly implicated in complement activation and subsequent defects in CF.
    • Observed complement defects are likely secondary to chronic pulmonary inflammation and infection.