Survival in the Swedish cohort with alpha-1-antitrypsin deficiency, up to the age of 43-45 years
(2019) In International Journal of COPD 14. p.525-530- Abstract
Alpha-1-antitrypsin deficiency (AATD) is a hereditary disorder. AATD is a known risk factor for the development of emphysema and liver disease. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening in 1972-1974 and has been followed up since birth. Our aim was to study survival in this cohort up to 43-45 years of age in comparison with the general Swedish population. Methods: Data from 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull subjects, who were identified by the neonatal screening in 1972-1974, were included in the study. To compare death rates in the PiZZ and PiSZ individuals with the general Swedish population, a standardized mortality ratio (SMR)... (More)
Alpha-1-antitrypsin deficiency (AATD) is a hereditary disorder. AATD is a known risk factor for the development of emphysema and liver disease. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening in 1972-1974 and has been followed up since birth. Our aim was to study survival in this cohort up to 43-45 years of age in comparison with the general Swedish population. Methods: Data from 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull subjects, who were identified by the neonatal screening in 1972-1974, were included in the study. To compare death rates in the PiZZ and PiSZ individuals with the general Swedish population, a standardized mortality ratio (SMR) was calculated as the ratio of observed to expected deaths. Results: Seven PiZZ subjects died during the follow-up, to be compared with an expected 3.66 deaths for the general population, giving an SMR of 1.91 (95% CI 0.77-3.94). Four PiSZ subjects died compared to an expected 1.53 deaths, giving an SMR of 2.61 (95% CI 0.71-6.71). The cumulative probability of survival up to the age of 45 years was 94% (95% CI 90%-98%) for the study population. Six deaths occurred before the age of 8 years. Conclusion: Up to 43-45 years of age, there was no difference in survival between PiZZ and PiSZ individuals in comparison with the Swedish general population. The majority of deaths occurred during childhood.
(Less)
- author
- Mostafavi, Behrouz LU ; Piitulainen, Eeva LU and Tanash, Hanan A. LU
- organization
- publishing date
- 2019
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Alpha-1-antitrypsin deficiency, Causes of death, Screening, Survival
- in
- International Journal of COPD
- volume
- 14
- pages
- 6 pages
- publisher
- Dove Medical Press Ltd.
- external identifiers
-
- pmid:30880942
- scopus:85062979933
- ISSN
- 1176-9106
- DOI
- 10.2147/COPD.S183205
- language
- English
- LU publication?
- yes
- id
- a7d03e26-18a6-40a1-bf6b-41926a84f2b7
- date added to LUP
- 2019-03-28 13:59:01
- date last changed
- 2024-09-18 15:45:35
@article{a7d03e26-18a6-40a1-bf6b-41926a84f2b7, abstract = {{<p>Alpha-1-antitrypsin deficiency (AATD) is a hereditary disorder. AATD is a known risk factor for the development of emphysema and liver disease. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening in 1972-1974 and has been followed up since birth. Our aim was to study survival in this cohort up to 43-45 years of age in comparison with the general Swedish population. Methods: Data from 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull subjects, who were identified by the neonatal screening in 1972-1974, were included in the study. To compare death rates in the PiZZ and PiSZ individuals with the general Swedish population, a standardized mortality ratio (SMR) was calculated as the ratio of observed to expected deaths. Results: Seven PiZZ subjects died during the follow-up, to be compared with an expected 3.66 deaths for the general population, giving an SMR of 1.91 (95% CI 0.77-3.94). Four PiSZ subjects died compared to an expected 1.53 deaths, giving an SMR of 2.61 (95% CI 0.71-6.71). The cumulative probability of survival up to the age of 45 years was 94% (95% CI 90%-98%) for the study population. Six deaths occurred before the age of 8 years. Conclusion: Up to 43-45 years of age, there was no difference in survival between PiZZ and PiSZ individuals in comparison with the Swedish general population. The majority of deaths occurred during childhood.</p>}}, author = {{Mostafavi, Behrouz and Piitulainen, Eeva and Tanash, Hanan A.}}, issn = {{1176-9106}}, keywords = {{Alpha-1-antitrypsin deficiency; Causes of death; Screening; Survival}}, language = {{eng}}, pages = {{525--530}}, publisher = {{Dove Medical Press Ltd.}}, series = {{International Journal of COPD}}, title = {{Survival in the Swedish cohort with alpha-1-antitrypsin deficiency, up to the age of 43-45 years}}, url = {{http://dx.doi.org/10.2147/COPD.S183205}}, doi = {{10.2147/COPD.S183205}}, volume = {{14}}, year = {{2019}}, }