The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Natural History and Life Expectancy in Severe Alpha1‐Antitrypsin Deficiency, Pi Z
Abstract
ABSTRACT. Clinical data from 246 adult Swedish individuals with severe α 1‐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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Citations
Cites
Maximum utilization of the life table method in analyzing survival
Cutler, S. J., & Ederer, F. (1958) · Journal of Chronic Diseases · doi:10.1016/0021-9681(58)90126-7 · Cited by 2,870
Cited by
Replacement Therapy for Alpha1-Antitrypsin Deficiency Associated with Emphysema
Wewers, M. D., Casolaro, M. A., Sellers, S. E., Swayze, S. C., McPhaul, K. M., Wittes, J. T., & Crystal, R. G. (1987) · New England Journal of Medicine · doi:10.1056/nejm198704233161704 · Cited by 641
Risk of Cirrhosis and Primary Liver Cancer in Alpha1-Antitrypsin Deficiency
Eriksson, S., Carlson, J., & Velez, R. (1986) · New England Journal of Medicine · doi:10.1056/nejm198603203141202 · Cited by 580
Alpha1-Antitrypsin Deficiency — A Model for Conformational Diseases
Carrell, R. W., & Lomas, D. A. (2002) · New England Journal of Medicine · doi:10.1056/nejmra010772 · Cited by 448
Survival and FEV 1 Decline in Individuals with Severe Deficiency of α 1 -Antitrypsin
The Alpha-1-Antitrypsin Deficiency Registry Study Group (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.158.1.9712017 · Cited by 435
Matrix metalloproteinases in destructive lung disease
McGarry Houghton, A. (2015) · Matrix Biology · doi:10.1016/j.matbio.2015.02.002 · Cited by 172
Metadata
- Authors
- Larsson, C.
- Venue
- Acta Medica Scandinavica
- Publisher
- Wiley
- Published
- 1978-01-12
- Volume
- 204
- Issue
- 1-6
- Pages
- 345-351
- ISSN
- 0001-6101 (ISSN-L)
- OpenAlex
- W2086241735
- Cited by
- 484
- References
- 1
- FWCI
- 8.74
- Type
- journal article
- Language
- en
- Open access
- closed
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- Larsson, C. (1978). Natural History and Life Expectancy in Severe Alpha1‐Antitrypsin Deficiency, Pi Z. Acta Medica Scandinavica. https://doi.org/10.1111/j.0954-6820.1978.tb08452.x
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.