TY - JOUR
T1 - The prevalence of alpha-1 antitrypsin deficiency among patients found to have airflow obstruction
AU - Rahaghi, Franck F.
AU - Sandhaus, Robert A.
AU - Strange, Charlie
AU - Hogarth, Douglas Kyle
AU - Eden, Edward
AU - Stocks, James M.
AU - Krowka, Michael J.
AU - Stoller, James K.
N1 - Funding Information:
This work was supported by a grant of the Alpha-1 Foundation. Dr. Stoller has served as a Consultant to Talecris, Baxter, Shire, Boehringer Ingelheim, Asth-matx. He received grants from NIH, Alpha-1 Foundation, Astra Zeneca. He has received speaker’s fees from Talecris Biotherapeutics, Baxter Healthcare, Grifols, Inc., and CSL-Behring, Inc. Dr. Rahaghi has served as a consultant for and has received speaker’s fees from Baxter Healthcare CSL-Behring, Inc. and Talecris Biotherapeutics (now Grifols). Dr. Sandhaus, receiving grant support from Kamada, Talecris Biotherapeutics, and CSL Behring, and honoraria for consulting or lectures from Kamada, Dey, Talecris Biotherapeutics, and CSL Behring, with all fees and honoraria donated to AlphaNet. Dr. Strange has served as a consultant for Astra Zeneca, Baxter Healthcare, CSL-Behring, Inc., Forest Laboratories, Medimmune, Talecris Biotherapeutics, and Uptake Medical on the topic of COPD. He has grants with the Alpha-1 Association, Alpha-1 Foundation, Astra Zeneca, Centocor, Forest, NIH, Novartis, Pfizer, and Talecris. In the past 3 years he has been on the speaker’s bureau for Actelion, Astra Zeneca, France Foundation for Intermune, Gilead, Glaxo Smith Kline, Grifols, and Pfizer. Dr. Hogarth is on the speakers’ bureau for CSL Behring, Talecris (Grifols), and Baxter; has received research support from CSL Behring and Baxter. Dr. Eden has no conflicts to declare. Dr. Stocks has received grant support from Talecris Biotherapeutics (Grifols), Baxter, CSL Behring, and Kamada and has served as a paid consultant/ speaker for Baxter and Talecris. Dr. Krowka has no conflicts to declare. All authors have contributed to the composition, critical analysis, and the review of the manuscript.
PY - 2012/8
Y1 - 2012/8
N2 - Introduction: Alpha-1 antitrypsin deficiency (AATD) is a genetic disease that may be manifested by chronic obstructive pulmonary disease. Despite professional society guidelines that recommend broad testing of at-risk individuals, fewer than 10 of affected individuals have been identified. The goals of this study were to estimate the frequency of abnormal AAT genotypes among patients found to have fixed airflow obstruction and to assess the feasibility of having Pulmonary Function Laboratory personnel administer the study. Methods: Nineteen medical centers in the United States participated in the study. Eligible patients (> GOLD II, FEV1/FVC ratio < 0.7, with post-bronchodilator FEV1<80% predicted) were offered testing for AATD by the Pulmonary Function Laboratory personnel at the time of pulmonary function testing. Results: A total of 3,457 patients were tested, of whom 3152 were eligible. Deficient patients (ZZ, SZ) constituted 0.63% of subjects, while 10.88% were carriers (MS, MZ). Neither demographic (except African-American race) nor post-bronchodilator pulmonary function variables (FEV1, FVC, FEV1/FVC ratio, TLC, and FEV1/FVC) allowed us to predict AAT heterozygote or deficiency status. Conclusions: The prevalence of AATD among patients undergoing pulmonary function tests with fixed airflow obstruction was 0.63%. Pulmonary Function Laboratory personnel effectively conducted the study.
AB - Introduction: Alpha-1 antitrypsin deficiency (AATD) is a genetic disease that may be manifested by chronic obstructive pulmonary disease. Despite professional society guidelines that recommend broad testing of at-risk individuals, fewer than 10 of affected individuals have been identified. The goals of this study were to estimate the frequency of abnormal AAT genotypes among patients found to have fixed airflow obstruction and to assess the feasibility of having Pulmonary Function Laboratory personnel administer the study. Methods: Nineteen medical centers in the United States participated in the study. Eligible patients (> GOLD II, FEV1/FVC ratio < 0.7, with post-bronchodilator FEV1<80% predicted) were offered testing for AATD by the Pulmonary Function Laboratory personnel at the time of pulmonary function testing. Results: A total of 3,457 patients were tested, of whom 3152 were eligible. Deficient patients (ZZ, SZ) constituted 0.63% of subjects, while 10.88% were carriers (MS, MZ). Neither demographic (except African-American race) nor post-bronchodilator pulmonary function variables (FEV1, FVC, FEV1/FVC ratio, TLC, and FEV1/FVC) allowed us to predict AAT heterozygote or deficiency status. Conclusions: The prevalence of AATD among patients undergoing pulmonary function tests with fixed airflow obstruction was 0.63%. Pulmonary Function Laboratory personnel effectively conducted the study.
KW - Alpha 1-Antitrypsin Deficiency
KW - Chronic Obstructive Pulmonary Disease
KW - Pulmonary Function Test
KW - Respiratory Care
KW - Spirometry
UR - http://www.scopus.com/inward/record.url?scp=84865194902&partnerID=8YFLogxK
U2 - 10.3109/15412555.2012.669433
DO - 10.3109/15412555.2012.669433
M3 - Article
C2 - 22506682
AN - SCOPUS:84865194902
SN - 1541-2555
VL - 9
SP - 352
EP - 358
JO - COPD: Journal of Chronic Obstructive Pulmonary Disease
JF - COPD: Journal of Chronic Obstructive Pulmonary Disease
IS - 4
ER -