Lung distensibility and airway function in intermediate alpha 1-antitrypsin deficiency (Pi MZ). 1979

S F Tattersall, and R P Pereira, and D Hunter, and G Blundell, and N B Pride

We examined the role of intermediate alpha 1-antitrypsin deficiency in predisposing to abnormalities of lung distensibility and airway function in 20 heterozygotes (Pi MZ) who were individually matched with a control Pi M subject of similar age, height, and smoking habits drawn from the same male, working population. There were no significant differences between the heterozygotes and their controls in the results of spirometry, maximum expiratory flow-volume curves (breathing air), single breath nitrogen test, arterialised capillary blood oxygen pressure, or single breath carbon monoxide transfer. Additional studies were made in 12 of the pairs of Pi MZ and Pi M subjects. Comparison of maximum expiratory flow-volume curves breathing air and 80% helium-20% oxygen showed no differences between the Pi MZ and Pi M subjects. Although airway function was similar in the two groups, four of 12 Pi MZ subjects showed abnormalities of the pressure-volume curve of the lung (reduction in lung recoil pressure, abnormal shape factor, increase in functional residual capacity). Abnormalities of washout of a helium-sulphur hexafluoride gas mixture, of a type previously described as characteristic of emphysema, were found in two of the men with abnormal pressure-volume curves. The results suggest that Pi MZ subjects have an increased susceptibility to alveolar abnormalities without increased abnormalities of airway function; this may explain the increased frequency of emphysema at necropsy despite many studies showing no predisposition to abnormal airway function in life. The functional changes we observed would be unlikely to cause symptoms. The risk of disablement from chronic lung disease appears to be only slightly enhanced by intermediate alpha 1-antitrypsin deficiency.

UI MeSH Term Description Entries
D008131 London The capital of the United Kingdom. It is located in England.
D008168 Lung Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood. Lungs
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D011656 Pulmonary Emphysema Enlargement of air spaces distal to the TERMINAL BRONCHIOLES where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions. Emphysema, Pulmonary,Centriacinar Emphysema,Centrilobular Emphysema,Emphysemas, Pulmonary,Focal Emphysema,Panacinar Emphysema,Panlobular Emphysema,Pulmonary Emphysemas,Centriacinar Emphysemas,Centrilobular Emphysemas,Emphysema, Centriacinar,Emphysema, Centrilobular,Emphysema, Focal,Emphysema, Panacinar,Emphysema, Panlobular,Emphysemas, Centriacinar,Emphysemas, Centrilobular,Emphysemas, Focal,Emphysemas, Panacinar,Emphysemas, Panlobular,Focal Emphysemas,Panacinar Emphysemas,Panlobular Emphysemas
D012129 Respiratory Function Tests Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc. Lung Function Tests,Pulmonary Function Tests,Function Test, Pulmonary,Function Tests, Pulmonary,Pulmonary Function Test,Test, Pulmonary Function,Tests, Pulmonary Function,Function Test, Lung,Function Test, Respiratory,Function Tests, Lung,Function Tests, Respiratory,Lung Function Test,Respiratory Function Test,Test, Lung Function,Test, Respiratory Function,Tests, Lung Function,Tests, Respiratory Function
D006371 Helium A noble gas with the atomic symbol He, atomic number 2, and atomic weight 4.003. It is a colorless, odorless, tasteless gas that is not combustible and does not support combustion. It was first detected in the sun and is now obtained from natural gas. Medically it is used as a diluent for other gases, being especially useful with oxygen in the treatment of certain cases of respiratory obstruction, and as a vehicle for general anesthetics. Helium-4,Helium 4
D006579 Heterozygote An individual having different alleles at one or more loci regarding a specific character. Carriers, Genetic,Genetic Carriers,Carrier, Genetic,Genetic Carrier,Heterozygotes
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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