title: [Alpha 1-antitrypsin phenotypes and their role in the development of early and late cirrhosis of the liver].
abstract: On the basis of cooperation with the Children's Health Centre the role was analysed of certain phenotypes of alpha 1-antitrypsin (alpha 1-AT), mainly Pi Z and Pi MZ phenotypes in the development of infantile cirrhosis. A significant participation was demonstrated of the latter phenotype in patients developing infantile cirrhosis. The preliminary analysis of the incidence of pathological alpha 1-AT phenotypes in the group of patients with cirrhosis manifested at the adult age seems to indicate the higher incidence of the Pi MZ phenotype as compared with the general population. The genetically determined low serum alpha 1-AT activity is probably one out of many factors determining the development of this disease. Further studies will be aimed at explaining whether the combination of action of two factors: alpha 1-AT deficiency and exposure to hepatotoxic agents is not accelerating the appearance of the disease and is not potentiating its clinical intensity.
authors: Instytutu Medycyny Pracy im. prof. J. Nofera w Lodzi.
authors: B
authors: B
authors: Piłacik
authors: J
authors: J
authors: Rujner
authors: J
authors: J
authors: Socha
authors: K
authors: K
authors: Madaliński
authors: J
authors: J
authors: Cianciara
authors: N
authors: N
authors: Mian
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000201:enzymology / Q000209:etiology* / Q000235:genetics; D010641:Phenotype; D013997:Time Factors; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D000515:alpha 1-Antitrypsin
vernacular_title: Fenotypy alpha 1-antytrypsyny i ich rola w rozwoju wczesnej i późnej marskości watroby.