Biochemical reaction of alpha 1 antitrypsin during the substitution therapy of patients with homozygote PI-ZZ deficit.
The homozygote deficit of alpha 1 antitrypsin (alpha 1 PI-ZZ) in patients frequently results in a premature development of emphysema in the lung due to incomplete protection against proteases. An active inhibitor substitution appears to be useful. The presented study proves the biological effect of alpha 1 antitrypsin infused into 8 patients. The results were an activity increase of leukocyte elastase and trypsin inhibition in serum as well as doubling of alpha 1 antitrypsin in sputum. This therapeutical conception (with a dose of 60 mg/kg body weight/week) results in an efficient protection. Inhibitors specific for mucosa are not influenced. An improvement of lung function during 6 weeks of intravenous therapy was not achieved. The progressive destruction of lung parenchyma can be probably prevented, however.
Gillissen
A
A
Berufsgenossenschaftliche Krankenanstalten Bergmannsheil Bochum Universitätsklinik Medizinische Klinik und Poliklinik Bochum.
Schmidt
E W
EW
Rasche
B
B
Ulmer
W T
WT
D000328:Adult; D000368:Aged; D001798:Blood Proteins / Q000008:administration & dosage* / Q000378:metabolism; D004334:Drug Administration Schedule; D005260:Female; D006720:Homozygote; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D010196:Pancreatic Elastase / Q000097:blood; D010641:Phenotype; D011656:Pulmonary Emphysema / Q000235:genetics* / Q000628:therapy; D012463:Saliva / Q000201:enzymology; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
D001798:Blood Proteins; D000515:alpha 1-Antitrypsin; D010196:Pancreatic Elastase