Natural History and Life Expectancy in Severe Alpha1‐Antitrypsin Deficiency, Pi Z
C Larsson - Acta Medica Scandinavica, 1978 - Wiley Online Library
C Larsson
Acta Medica Scandinavica, 1978•Wiley Online LibraryClinical data from 246 adult Swedish individuals with severe α1‐antitrypsin deficiency, Pi Z,
diagnosed in 1963‐77, were analyzed. Primary emphysema was present in 109 cases. Of
75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7
showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40
years, compared to 53 in non‐smokers (p< 0.001). Of the Pi Z individuals over the age of 50,
19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 …
diagnosed in 1963‐77, were analyzed. Primary emphysema was present in 109 cases. Of
75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7
showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40
years, compared to 53 in non‐smokers (p< 0.001). Of the Pi Z individuals over the age of 50,
19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 …
Abstract
Clinical data from 246 adult Swedish individuals with severe α1‐antitrypsin deficiency, Pi Z, diagnosed in 1963‐77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40 years, compared to 53 in non‐smokers (p<0.001). Of the Pi Z individuals over the age of 50, 19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 deceased patients, 56 died from COPD and 12 from liver disease. A greatly reduced survival was demonstrated in Pi Z individuals, regardless of sex. Smoking Pi 2 individuals had a significantly lower life expectancy than Pi Z non‐smokers (p<0.01).
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