Plasma protease inhibitors in alpha 1-antitrypsin-deficient children

Pediatr Res. 1985 Aug;19(8):834-5. doi: 10.1203/00006450-198508000-00011.


Seven plasma protease inhibitors were analyzed in 8-yr-old children-75 PiZ (severely deficient), one PiZ-(with a null gene), 32 PiSZ (moderately deficient), and 35 normals. In PiZ and PiSZ alpha 1-antitrypsin-deficient children, significantly higher concentrations of alph 2-macroglobulin (p less than 0.001) and antithrombin III (p less than 0.001) were found and significantly lower concentrations of alpha 2-antiplasmin (p less than 0.001), C1-inactivator (p less than 0.01) and plasminogen (PiZ p less than 0.02, PiSZ p less than 0.01). The inter-alpha-trypsin inhibitor concentrations differed in PiSZ children only (p less than 0.01). At this age the high alpha 2-macroglobulin levels may be of importance to protect the lung tissue against free proteolytic activity, while importance of the other differences are unknown or reflect changes of purely theoretical interest.

MeSH terms

  • Child
  • Humans
  • Prospective Studies
  • Protease Inhibitors / blood*
  • alpha 1-Antitrypsin Deficiency*


  • Protease Inhibitors