[Clinical aspects, diagnostic lung function and biochemical parameters in children with homozygous alpha 1-antitrypsin deficiency].

Both the clinical course of the homozygous alpha 1-PI deficiency and also the pulmonary function and measured clinical parameters in these children revealed very marked inter-individual fluctuations. In one child, the lung function revealed certain signs of incipient pulmonary emphysema. The biochemical parameters of two children revealed TIC/alpha 1-PI- and PEIC/alpha 1-PI ratios greater than 1. This indicates that, in addition to alpha 1-PI, further inhibitors may develop a compensatory effect.