Paradoxical role of proteinase inhibitors in cystic fibrosis

-antichymotrypsin is associated with less severe pulmonary disease in patients with cystic fibrosis.

By | December 28, 2000

While free proteinases destroy lung tissue in cystic fibrosis, logic predicts that proteinase inhibitors should offer protection. However, for some time doctors have observed that a mild genetic deficiency in an inhibitor is actually associated with a better, rather than worse, outcome. In January's issue of Thorax, Dr Mahadeva and colleagues from the University of Cambridge support this observation with their new findings (Thorax 2001 56:53-58).

They screened 157 patients with cystic fibrosis and identified 10 with a plasma deficiency of α1-antichymotrypsin (plasma concentration <0.2 g/l). In a multivariate analysis these individuals had significantly less severe lung disease than those who had normal or raised levels of α1-antichymotrypsin. They concluded, paradoxical though it may be, that a deficiency of the inhibitor α1-antichymotrypsin is associated with less severe pulmonary disease in patients with cystic fibrosis.

Popular Now

  1. Top 10 Innovations 2016
    Features Top 10 Innovations 2016

    This year’s list of winners celebrates both large leaps and small (but important) steps in life science technology.

  2. Gut Microbes Linked to Neurodegenerative Disease
  3. Pubic Hair Grooming Linked to STI Risk
    The Nutshell Pubic Hair Grooming Linked to STI Risk

    Observational study suggests pubic hair grooming correlates with heightened risk of acquiring sexually transmitted infections, although causation remains unclear.

  4. Naive T Cells Find Homes in Lymphoid Tissue
Rockland