Mortality in alpha-1-antitrypsin deficiency in the United Kingdom

Paul Dawkins; Alice Turner; Peter Nightingale; Robert Stockley

doi:10.1016/j.rmed.2009.04.004

Mortality in alpha-1-antitrypsin deficiency in the United Kingdom

Paul Dawkins
, Alice Turner
, Peter Nightingale
, Robert Stockley

Research output: Contribution to journal › Article

20 Citations (Scopus)

Abstract

BACKGROUND: Four hundred and eighty-eight PiZ alpha-1-antitrypsin deficient patients, who had joined the UK registry over a 9-year period, were followed in an observational study to determine mortality. None had received A1AT augmentation therapy. METHODS: Cause of death was confirmed from death certification and medical records. Patients were censored according to length of time on the program or until they withdrew from the program. RESULTS: There were 56 deaths of which 30 were attributed to respiratory causes. Of the remaining 26 deaths, 4 were due to complications from lung transplant, 6 due to liver disease (including 2 post-liver transplant) and the other 16 due to a variety of causes. Kaplan-Meier plots indicated a cumulative hazard for mortality of 18.1% in 9 years, correcting for time of follow up. When categorised for FEV1 percent-predicted, the group with severe impairment had increased mortality (p =

Original language	English
Pages (from-to)	1540-1547
Number of pages	8
Journal	Respiratory Medicine
Volume	103
Issue number	10
DOIs	https://doi.org/10.1016/j.rmed.2009.04.004
Publication status	Published - 1 Oct 2009

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10.1016/j.rmed.2009.04.004

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title = "Mortality in alpha-1-antitrypsin deficiency in the United Kingdom",

abstract = "BACKGROUND: Four hundred and eighty-eight PiZ alpha-1-antitrypsin deficient patients, who had joined the UK registry over a 9-year period, were followed in an observational study to determine mortality. None had received A1AT augmentation therapy. METHODS: Cause of death was confirmed from death certification and medical records. Patients were censored according to length of time on the program or until they withdrew from the program. RESULTS: There were 56 deaths of which 30 were attributed to respiratory causes. Of the remaining 26 deaths, 4 were due to complications from lung transplant, 6 due to liver disease (including 2 post-liver transplant) and the other 16 due to a variety of causes. Kaplan-Meier plots indicated a cumulative hazard for mortality of 18.1\% in 9 years, correcting for time of follow up. When categorised for FEV1 percent-predicted, the group with severe impairment had increased mortality (p = ",

author = "Paul Dawkins and Alice Turner and Peter Nightingale and Robert Stockley",

year = "2009",

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doi = "10.1016/j.rmed.2009.04.004",

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T1 - Mortality in alpha-1-antitrypsin deficiency in the United Kingdom

AU - Dawkins, Paul

AU - Turner, Alice

AU - Nightingale, Peter

AU - Stockley, Robert

PY - 2009/10/1

Y1 - 2009/10/1

N2 - BACKGROUND: Four hundred and eighty-eight PiZ alpha-1-antitrypsin deficient patients, who had joined the UK registry over a 9-year period, were followed in an observational study to determine mortality. None had received A1AT augmentation therapy. METHODS: Cause of death was confirmed from death certification and medical records. Patients were censored according to length of time on the program or until they withdrew from the program. RESULTS: There were 56 deaths of which 30 were attributed to respiratory causes. Of the remaining 26 deaths, 4 were due to complications from lung transplant, 6 due to liver disease (including 2 post-liver transplant) and the other 16 due to a variety of causes. Kaplan-Meier plots indicated a cumulative hazard for mortality of 18.1% in 9 years, correcting for time of follow up. When categorised for FEV1 percent-predicted, the group with severe impairment had increased mortality (p =

AB - BACKGROUND: Four hundred and eighty-eight PiZ alpha-1-antitrypsin deficient patients, who had joined the UK registry over a 9-year period, were followed in an observational study to determine mortality. None had received A1AT augmentation therapy. METHODS: Cause of death was confirmed from death certification and medical records. Patients were censored according to length of time on the program or until they withdrew from the program. RESULTS: There were 56 deaths of which 30 were attributed to respiratory causes. Of the remaining 26 deaths, 4 were due to complications from lung transplant, 6 due to liver disease (including 2 post-liver transplant) and the other 16 due to a variety of causes. Kaplan-Meier plots indicated a cumulative hazard for mortality of 18.1% in 9 years, correcting for time of follow up. When categorised for FEV1 percent-predicted, the group with severe impairment had increased mortality (p =

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VL - 103

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JO - Respiratory Medicine

JF - Respiratory Medicine

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Mortality in alpha-1-antitrypsin deficiency in the United Kingdom

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