Alpha1-antitrypsin Deficiency Registry

NCT00005292 | Completed

• 1 other identifier: 2014
• Study Type: observational
• Target: N/A
• Locations: 0 countries
• Sites: N/A

Brief Summary

To collect data from the 37 participating clinical centers on patients with alpha1-antitrypsin deficiency, including those who received replacement therapy with an intravenous preparation of alpha1-proteinase inhibitor (A1Pi) concentrate.

Conditions

Lung Diseases; Emphysema; Alpha-1 Antitrypsin Deficiency; Chronic Obstructive Pulmonary Disease

Eligibility Criteria

• Age: Up to 100 Years
• Sex: male
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

No eligibility criteria

Contact the study team to discuss eligibility requirements. They can help determine if this study is right for you.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Related Publications (9)

• Stoller JK, Williams GW, Crystal RG. Alpha 1-antitrypsin deficiency registry. Chest. 1990 May;97(5):1278. doi: 10.1378/chest.97.5.1278b. No abstract available.

  PMID: 2331946 BACKGROUND

• Schluchter MD. Methods for the analysis of informatively censored longitudinal data. Stat Med. 1992 Oct-Nov;11(14-15):1861-70. doi: 10.1002/sim.4780111408.

  PMID: 1480878 BACKGROUND

• A registry of patients with severe deficiency of alpha 1-antitrypsin. Design and methods. The Alpha 1-Antitrypsin Deficiency Registry Study Group. Chest. 1994 Oct;106(4):1223-32.

  PMID: 7924498 BACKGROUND

• Stoller JK, Buist AS, Burrows B, Crystal RG, Fallat RJ, McCarthy K, Schluchter MD, Soskel NT, Zhang R. Quality control of spirometry testing in the registry for patients with severe alpha1-antitrypsin deficiency. alpha1-Antitrypsin Deficiency Registry Study Group. Chest. 1997 Apr;111(4):899-909. doi: 10.1378/chest.111.4.899.

  PMID: 9106567 BACKGROUND

• McElvaney NG, Stoller JK, Buist AS, Prakash UB, Brantly ML, Schluchter MD, Crystal RD. Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Alpha 1-Antitrypsin Deficiency Registry Study Group. Chest. 1997 Feb;111(2):394-403. doi: 10.1378/chest.111.2.394.

  PMID: 9041988 BACKGROUND

• Turino GM, Barker AF, Brantly ML, Cohen AB, Connelly RP, Crystal RG, Eden E, Schluchter MD, Stoller JK. Clinical features of individuals with PI*SZ phenotype of alpha 1-antitrypsin deficiency. alpha 1-Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med. 1996 Dec;154(6 Pt 1):1718-25. doi: 10.1164/ajrccm.154.6.8970361.

  PMID: 8970361 BACKGROUND

• Schluchter MD, Stoller JK, Barker AF, Buist AS, Crystal RG, Donohue JF, Fallat RJ, Turino GM, Vreim CE, Wu MC. Feasibility of a clinical trial of augmentation therapy for alpha(1)-antitrypsin deficiency. The Alpha 1-Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med. 2000 Mar;161(3 Pt 1):796-801. doi: 10.1164/ajrccm.161.3.9906011.

  PMID: 10712324 BACKGROUND

• Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med. 1998 Jul;158(1):49-59. doi: 10.1164/ajrccm.158.1.9712017.

  PMID: 9655706 BACKGROUND

• Eden E, Hammel J, Rouhani FN, Brantly ML, Barker AF, Buist AS, Fallat RJ, Stoller JK, Crystal RG, Turino GM. Asthma features in severe alpha1-antitrypsin deficiency: experience of the National Heart, Lung, and Blood Institute Registry. Chest. 2003 Mar;123(3):765-71. doi: 10.1378/chest.123.3.765.

  PMID: 12628876 BACKGROUND

MeSH Terms

Conditions

Lung Diseases; Emphysema; alpha 1-Antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive

Condition Hierarchy (Ancestors)

Respiratory Tract Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Liver Diseases; Digestive System Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Subcutaneous Emphysema; Lung Diseases, Obstructive; Chronic Disease; Disease Attributes

Study Officials

• Mark Schluchter

  The Cleveland Clinic

Study Design

• Study Type: observational
• Sponsor Type: NIH

Study Record Dates

• First Submitted: May 25, 2000
• First Posted: May 26, 2000
• Study Start: September 1, 1988
• Study Completion: November 1, 1999
• Last Updated: March 25, 2016

Record last verified: 2004-08