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Cellular proteolysis and systemic inflammation during exacerbation in cystic fibrosis

Alina A Ionescu1, Lisette S Nixon, Dennis J Shale

  • 1Section of Respiratory and Communicable Diseases, Department of Medicine, University of Wales College of Medicine, UK.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|February 9, 2005
PubMed

Insights

In cystic fibrosis (CF), low fat-free mass (FFM) and inflammation are linked to increased cellular protein breakdown, contributing to poor weight preservation during exacerbations.

Area of Science:

  • Biochemistry
  • Clinical Medicine
  • Nutrition Science

Background:

  • Weight loss is a poor prognostic indicator in cystic fibrosis (CF).
  • Systemic inflammation and fat-free mass (FFM) depletion may contribute to increased cellular proteolysis during CF exacerbations.

Purpose of the Study:

  • To investigate the association between FFM, systemic inflammation, and cellular proteolysis in CF patients during respiratory exacerbations.

Main Methods:

  • Prospective study of 26 CF patients at the start, end, and 2 weeks post-antibiotic treatment.
  • Assessed urinary Pseudouridine (PSU) excretion as a marker of cellular protein breakdown.
  • Measured body composition using dual-energy X-ray absorptiometry (DXA) and inflammatory mediators.

Main Results:

  • CF patients exhibited elevated PSU levels at all assessment points.
  • Lower FFM correlated with higher PSU levels.
  • PSU levels were associated with C-reactive protein (CRP) and TNF-alpha soluble receptors, indicating increased inflammation.

Conclusions:

  • Increased cellular protein breakdown is linked to reduced FFM and heightened systemic inflammation in CF.
  • These factors may contribute to weight loss and poor outcomes in CF patients.
Abstract

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