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Table of Contents
Vol. 73, No. 2, 2006
Issue release date: March 2006
Section title: Clinical Investigations
Respiration 2006;73:185–190
(DOI:10.1159/000088061)

Results of a Survey of Patients with Alpha-1 Antitrypsin Deficiency

Strange C. · Stoller J.K. · Sandhaus R.A. · Dickson R. · Turino G.
aDivision of Pulmonary and Critical Care Medicine, Department of Medicine, Medical University of South Carolina, Charleston, S.C.; bDivision of Pulmonary, Allergy, and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, Ohio; cDivision of Pulmonary and Critical Care Medicine, National Jewish Medical Center, Denver, Colo., and dDivision of Pulmonary Medicine, St. Luke’s/Roosevelt Hospital, New York, N.Y., USA

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Article / Publication Details

First-Page Preview
Abstract of Clinical Investigations

Published online: 3/22/2006

Number of Print Pages: 6
Number of Figures: 4
Number of Tables: 3

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: http://www.karger.com/RES

Abstract

Background: α1-Antitrypsin deficiency (AATD) is an uncommon genetic disease which occurs in 1–2.5% of Americans with chronic obstructive pulmonary disease (COPD). Little is known about current demographics of AATD. Objectives: This survey study reviews the clinical characteristics of diseased individuals in North America. Methods: A survey of members from the mailing lists of US AATD patient support organizations was commissioned with duplicate persons omitted. The survey was mailed to 5,222 unique individuals with AATD. Questionnaires were returned by 1,953 individuals, including 1,810 with severe deficiency, 93 with the carrier state and 41 who were caregivers of others. Results: The majority (81%) of participants reported COPD with symptoms of asthma, chronic bronchitis, and emphysema, usually in combination. The mean age of respondents [53.1 ± 13.2 (SD) years] is older than the general US population. Lung or liver transplantation was reported by 9% of all respondents (n = 175), including 66 single lung transplants, 68 double lung transplants, and 47 liver transplants. Another 6.6% (n = 128) reported that they were currently on a transplant list. Twenty-one percent of lung transplants report continuing augmentation therapy use. Augmentation use is reported by 75% of those with obstructive lung disease. The majority of patients with liver disease also have COPD. Conclusions: AATD remains a devastating illness for many of those affected as reflected in a high incidence of transplantation for liver and lung disease.


  

Author Contacts

Charlie Strange, MD, FCCP
Division of Pulmonary and Critical Care Medicine
96 Jonathan Lucas Street, 812 CSB, Medical University of South Carolina
Charleston, SC 29425 (USA)
Tel. +1 843 792 2153, Fax +1 843 792 0732, E-Mail strangec@musc.edu

  

Article Information

Received: November 15, 2004
Accepted after revision: March 3, 2005
Published online: August 31, 2005
Number of Print Pages : 6
Number of Figures : 4, Number of Tables : 3, Number of References : 12

  

Publication Details

Respiration (International Journal of Thoracic Medicine)

Vol. 73, No. 2, Year 2006 (Cover Date: March 2006)

Journal Editor: Bolliger, C.T. (Cape Town)
ISSN: 0025–7931 (print), 1423–0356 (Online)

For additional information: http://www.karger.com/RES


Article / Publication Details

First-Page Preview
Abstract of Clinical Investigations

Published online: 3/22/2006

Number of Print Pages: 6
Number of Figures: 4
Number of Tables: 3

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: http://www.karger.com/RES


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