Literature DB >> 19017884

Clinical characteristics of subjects with symptoms of alpha1-antitrypsin deficiency older than 60 years.

Michael A Campos1, Saleh Alazemi2, Guoyan Zhang3, Matthias Salathe2, Adam Wanner2, Robert A Sandhaus4, Horst Baier2.   

Abstract

BACKGROUND: The clinical characteristics of elderly subjects with alpha(1)-antitrypsin deficiency (AATD)-associated COPD have not been described.
METHODS: The clinical, demographic, health-related quality of life (HRQoL) characteristics and 1-year exacerbation rates of 275 subjects with AATD and COPD receiving augmentation therapy aged > 59 years (mean [+/- SD] age, 66.3 +/- 5.7 years) were compared to those of 354 subjects aged 50 to 59 years (mean age, 54.3 +/- 2.8 years) and 293 subjects < 50 years (mean age, 43.9 +/- 3.8 years).
RESULTS: Older subjects received diagnoses later in life (mean age at diagnosis, 55.0 +/- 8.5 years) and had a longer diagnostic delay (mean age at diagnosis, 12.9 +/- 14.3 years) than subjects in the other two age groups. Although the proportion of lifetime nonsmokers was higher in the older group, the majority (64%) had significant tobacco exposure but with a longer interval of tobacco abstinence. The mean FEV(1) values (n = 641) were similar between the three age groups, suggesting a slower disease progression in the oldest group. Subjects in the older group were less symptomatic, had less concomitant asthma, and had significantly better scores in most domains of two HRQoL instruments. During follow-up, older subjects had fewer acute exacerbations.
CONCLUSIONS: Subjects with AATD-associated COPD who reach an older age exhibit a more indolent clinical course than younger affected individuals, possibly related in part to differences in tobacco exposure. This finding supports current guidelines that recommend screening of all patients with COPD for AATD, regardless of their age and prior smoking history.

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Year:  2008        PMID: 19017884     DOI: 10.1378/chest.08-1129

Source DB:  PubMed          Journal:  Chest        ISSN: 0012-3692            Impact factor:   9.410


  7 in total

1.  Differences in adjustment between individuals with alpha-1 antitrypsin deficiency (AATD)-associated COPD and non-AATD COPD.

Authors:  Kristen E Holm; Soo Borson; Robert A Sandhaus; Dee W Ford; Charlie Strange; Russell P Bowler; Barry J Make; Frederick S Wamboldt
Journal:  COPD       Date:  2013-04       Impact factor: 2.409

2.  Characteristics of Chronic Obstructive Pulmonary Disease (COPD) Patients Reporting Alpha-1 Antitrypsin Deficiency in the WebMD Lung Health Check Database.

Authors:  Tyler McGrady; David M Mannino; Elisha Malanga; Byron M Thomashow; John Walsh; Robert A Sandhaus; James K Stoller
Journal:  Chronic Obstr Pulm Dis       Date:  2015-04-15

3.  The impact of age on outcomes in chronic obstructive pulmonary disease differs by relationship status.

Authors:  Kristen E Holm; Melissa R Plaufcan; Dee W Ford; Robert A Sandhaus; Matthew Strand; Charlie Strange; Frederick S Wamboldt
Journal:  J Behav Med       Date:  2013-05-04

4.  A long diagnostic delay in patients with Hereditary Haemorrhagic Telangiectasia: a questionnaire-based retrospective study.

Authors:  Paola Pierucci; Gennaro M Lenato; Patrizia Suppressa; Patrizia Lastella; Vincenzo Triggiani; Raffaella Valerio; Mario Comelli; Daniela Salvante; Alessandro Stella; Nicoletta Resta; Giancarlo Logroscino; Francesco Resta; Carlo Sabbà
Journal:  Orphanet J Rare Dis       Date:  2012-06-07       Impact factor: 4.123

Review 5.  Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review.

Authors:  Ross G Edgar; Mitesh Patel; Susan Bayliss; Diana Crossley; Elizabeth Sapey; Alice M Turner
Journal:  Int J Chron Obstruct Pulmon Dis       Date:  2017-05-02

6.  Safety and efficacy of alpha-1-antitrypsin augmentation therapy in the treatment of patients with alpha-1-antitrypsin deficiency.

Authors:  Irina Petrache; Joud Hajjar; Michael Campos
Journal:  Biologics       Date:  2009-07-13

Review 7.  Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach.

Authors:  Robert A Stockley; Marc Miravitlles; Claus Vogelmeier
Journal:  Orphanet J Rare Dis       Date:  2013-09-24       Impact factor: 4.123

  7 in total

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