Survival in the Swedish cohort with alpha-1-antitrypsin deficiency, up to the age of 43-45 years

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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) 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.

Detaljer

Författare
Enheter & grupper
Externa organisationer
  • Skåne University Hospital
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Lungmedicin och allergi
  • Folkhälsovetenskap, global hälsa, socialmedicin och epidemiologi

Nyckelord

Originalspråkengelska
Sidor (från-till)525-530
Antal sidor6
TidskriftInternational Journal of COPD
Volym14
StatusPublished - 2019
PublikationskategoriForskning
Peer review utfördJa