Literature DB >> 1087749

Lung function studies in asymptomatic individuals with moderately (Pi SZ) and severely (Pi Z) reduced levels of alpha1-antitrypsin.

C Larsson, H Dirksen, G Sundström, S Eriksson.   

Abstract

Extensive physiological evaluation of lung function were conducted in two groups of asymptomatic individuals with deficiencies of alpha1-antitrypsin: seven subjects with phenotype Pi SZ and six subjects with phenotype Pi Z. Four symptomatic individuals of Pi Z type were also investigated. The asymptomatic individuals of both SZ and Z phenotypes revealed reduction of elastic recoil, hypoventilation and hypoperfusion of the lung bases with increased closing and nitogen wash-out volumes. No signs of bronchial obstruction were observed. The asymptomatic Pi SZ subjects revealed as many signs of subclinical disease as did the asymptomatic Pi Z subjects. These data suggest that Pi SZ subjects are at increased risk of emphysema similar to Pi Z subjects.

Entities:  

Mesh:

Year:  1976        PMID: 1087749

Source DB:  PubMed          Journal:  Scand J Respir Dis        ISSN: 0036-5572


  16 in total

Review 1.  alpha-1-Antitrypsin and the pathogenesis of emphysema.

Authors:  R A Stockley
Journal:  Lung       Date:  1987       Impact factor: 2.584

2.  PI SZ phenotype in chronic obstructive pulmonary disease.

Authors:  L Alvarez-Granda; M J Cabero-Perez; A Bustamante-Ruiz; D Gonzalez-Lamuño; M Delgado-Rodriguez; M García-Fuentes
Journal:  Thorax       Date:  1997-07       Impact factor: 9.139

3.  Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency.

Authors:  D H Perlmutter; J Travis; P I Punsal
Journal:  J Clin Invest       Date:  1988-06       Impact factor: 14.808

4.  Alpha 1-antitrypsin screening of 18-year-old men.

Authors:  T Sveger; P Mazodier
Journal:  Thorax       Date:  1979-06       Impact factor: 9.139

Review 5.  Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis.

Authors:  C P Hersh; M Dahl; N P Ly; C S Berkey; B G Nordestgaard; E K Silverman
Journal:  Thorax       Date:  2004-10       Impact factor: 9.139

6.  Clarifying the Risk of Lung Disease in SZ Alpha-1 Antitrypsin Deficiency.

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

7.  Danazol-induced augmentation of serum alpha 1-antitrypsin levels in individuals with marked deficiency of this antiprotease.

Authors:  J E Gadek; J D Fulmer; J A Gelfand; M M Frank; T L Petty; R G Crystal
Journal:  J Clin Invest       Date:  1980-07       Impact factor: 14.808

8.  Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase.

Authors:  F Ogushi; G A Fells; R C Hubbard; S D Straus; R G Crystal
Journal:  J Clin Invest       Date:  1987-11       Impact factor: 14.808

9.  Evaluation of "at risk" alpha 1-antitrypsin genotype SZ with synthetic oligonucleotide gene probes.

Authors:  T Nukiwa; M Brantly; R Garver; L Paul; M Courtney; J P LeCocq; R G Crystal
Journal:  J Clin Invest       Date:  1986-02       Impact factor: 14.808

10.  Functional assessment of genetic variants of alpha 1-antitrypsin.

Authors:  G D Billingsley; D W Cox
Journal:  Hum Genet       Date:  1982       Impact factor: 4.132

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