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.

Advertisement
Advertisement

Popular Now

  1. Antibiotics and the Gut Microbiome
  2. Sex Differences in Pain Pathway
  3. The Sum of Our Parts
    Features The Sum of Our Parts

    Putting the microbiome front and center in health care, in preventive strategies, and in health-risk assessments could stem the epidemic of noncommunicable diseases.

  4. Anti-Vax Doctor Found Dead
    The Nutshell Anti-Vax Doctor Found Dead

    Police are calling the death of James Bradstreet, a physician who claimed vaccines cause autism and offered autism cures to patients, an apparent suicide.

Advertisement
Shimadzu Scientific
Shimadzu Scientific
Advertisement
The Scientist