BACKGROUND: A study was undertaken to clarify whether the PI SZ phenotype of the protease inhibitor system predisposes to chronic obstructive pulmonary disease (COPD). METHODS: The prevalence of PI Z and PI SZ deficient phenotypes was investigated in a population of 702 patients with COPD followed up at the Chest Unit of a tertiary hospital and in 15400 newborn infants from the same geographical area. Individuals with deficiency were detected by screening of dried blood spots on filter paper using a comparative electro-immunodiffusion technique for alpha 1-antitrypsin and transferrin. The serum phenotype was confirmed by means of isoelectrofocusing on polyacrylamide gel. RESULTS: Of the 702 blood samples from patients with COPD, six PI Z subjects (0.85%) and one PI SZ (0.14%) were detected. Of the 15400 samples from neonates, the number of PI Z subjects was eight (0.052%) and that of PI SZ was 24 (0.156%). The difference between the two groups was significant for PI Z but not for PI SZ. CONCLUSIONS: The data do not indicate an increased risk for development of COPD associated with the PI SZ phenotype but confirm the predisposition of PI Z individuals for the development of COPD.
BACKGROUND: A study was undertaken to clarify whether the PI SZ phenotype of the protease inhibitor system predisposes to chronic obstructive pulmonary disease (COPD). METHODS: The prevalence of PI Z and PI SZ deficient phenotypes was investigated in a population of 702 patients with COPD followed up at the Chest Unit of a tertiary hospital and in 15400 newborn infants from the same geographical area. Individuals with deficiency were detected by screening of dried blood spots on filter paper using a comparative electro-immunodiffusion technique for alpha 1-antitrypsin and transferrin. The serum phenotype was confirmed by means of isoelectrofocusing on polyacrylamide gel. RESULTS: Of the 702 blood samples from patients with COPD, six PI Z subjects (0.85%) and one PI SZ (0.14%) were detected. Of the 15400 samples from neonates, the number of PI Z subjects was eight (0.052%) and that of PI SZ was 24 (0.156%). The difference between the two groups was significant for PI Z but not for PI SZ. CONCLUSIONS: The data do not indicate an increased risk for development of COPD associated with the PI SZ phenotype but confirm the predisposition of PI Z individuals for the development of COPD.
Authors: Alessandro N Franciosi; Brian D Hobbs; Oliver J McElvaney; Kevin Molloy; Craig Hersh; Louise Clarke; Cedric Gunaratnam; Edwin K Silverman; Tomás P Carroll; Noel G McElvaney Journal: Am J Respir Crit Care Med Date: 2020-07-01 Impact factor: 21.405
Authors: Gerard N McElvaney; Robert A Sandhaus; Marc Miravitlles; Gerard M Turino; Niels Seersholm; Marion Wencker; Robert A Stockley Journal: Eur Respir J Date: 2020-06-18 Impact factor: 16.671
Authors: María Torres-Durán; José Luis López-Campos; Juan Luis Rodríguez-Hermosa; Cristina Esquinas; Cristina Martínez-González; José María Hernández-Pérez; Carlota Rodríguez; Ana Bustamante; Francisco Casas-Maldonado; Miriam Barrecheguren; Cruz González; Marc Miravitlles Journal: ERJ Open Res Date: 2022-09-26