Plasma Protease Inhibitors in [alpha]1-Antitrypsin- Deficient Children.
19(8):834-835, August 1985.
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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 [alpha]2-macroglobulin (p<0.001) and antithrombin III (p<0.001) were found and significantly lower concentrations of [alpha]2-antiplasmin (p<0.001), C1-inactivator (p<0.01) and plasminogen (PiZ p< 0.02, PiSZ p<0.01). The inter-[alpha]-trypsin inhibitor concentrations differed in PiSZ children only (p<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.
(C) International Pediatrics Research Foundation, Inc. 1985. All Rights Reserved.