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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.

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