Publications from Registry Research

Foil K, Blanton M, Sanders C, Kim J, Al Ashry H, Kumbhare S, Strange C. Sequencing Alpha-1 MZ Individuals Shows Frequent Biallelic Mutations. Pulm Med. 2018 Sep 5;2018:2836389. doi: 10.1155/2018/2836389. PMID: 30254761

Strange, C. Anti-Proteases and Alpha-1 Antitrypsin Augmentation Therapy. Respir Care. 2018 Jun; 63(6):690-698. doi: 10.1186/s12931-018-0792-1 PMID: 29794204

Stolk J, Stoel B, Parr D, Ficker JH, Strange C. Transitioning clinical data into patient care: recent real-life experiences with Alpha-1. EMJ Respir 2017 5(1):32-40.

Kelbel T, Morris D, Walker D, Craig T. The Allergist’s Role in Detection of Severe Alpha-1 Antitrypsin Deficiency. Journal of Allergy and Clinical Immunology: In Practice. 2017 Mar 8. pii: S2213-2198(17)30016-8. doi: 10.1016/j.japi.2017.01.008 PMID: 2824783

Kumbhare S, Hunt K, Nietert P, Schwarz L, Strange C. Hypertension prevalence in the United States varies with differences in alpha-1 antitrypsin genotype: a cross sectional study. British Journal of Medicine and Medical Research 2015. DOI:10.9734-BJMMR-2015-13447.

Wanner A, Groft SC, Teagarden R, Krischer J, Davis BR, Coffey CS, Hickam DH, Teckman J, Nelson DR, McCaleb ML, Loomba R, Strange C, Sandhaus RA, Brantly Edelman JM, Farrugia A. Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases J COPD Foundation. 2015,2(2):177-190. PMID: 28848840

Strange C, Monk R, Schwarz L, Walker D, Kumbhare S, Beiko T. The United States Alpha-1 Foundation Research Registry: Genesis, Impact and Future. COPD 2015, 12(S1):42-5. PMID: 25938291

Parrott RL, Smith RA, Hong SJ, Worthington A. Congruence-Incongruence Patterns in Alpha-1 Antitrypsin Deficiency Couples' Genetic Determinist Beliefs and Perceived Control over Genes: Implications for Clinical and Public Health Genomic Communication. J Genet Couns. 2015 Jun; 24(3):532-40. doi: 10.1007/s10897-014-9786-4. Epub 2014 Nov 21 PMID: 25413221

Olfert IM, Malek MH, Eagan TM, 'Wagner' H, Wagner PD. Inflammatory cytokine response to exercise in alpha-1-antitrypsin deficient COPD patients 'on' or 'off' augmentation therapy. BMC Pulm Med. 2014 Jun 30;14:106. PMID: 24975928

Hoth KF, Wamboldt FS, Ford DW, Sandhaus RA, Strange C, Bekelman DB, Holm KE. The social environment and illness uncertainty in chronic obstructive pulmonary disease. International Journal of Behavioral Medicine 2015 Apr;22(2):223-32. doi: 10.1007/s12529-014-9423-5. PMID: 25008041

Williams PH, Shore L, Sineath M, Quill J, Warner B, Keith J, Walker D, Wienke S, Flavin S, Strange C. Genetics' influence on patient experiences with a rare chronic disorder: a photovoice study of living with alpha-1 antitrypsin deficiency. Nurs Clin North Am. 2013 Dec;48(4):627-36. PMID 24295190.

Smith, R. A., Wienke, S. E., & Coffman, D. L. Alpha-1 couples: Interpersonal and intrapersonal predictors of spousal communication and stress. J Genet Couns. 2013 Aug 11. PMID: 23934327

Smith, R. A., Wienke, S. E., & Baker, M. K. Classifying married adults diagnosed with alpha-1 antitrypsin deficiency based on spousal communication patterns using latent class analysis: Insights for Intervention. 2013 Nov Journal of Genetic Counseling. doi: 10.1007/s10897-013-9661-8. PMID: 24177906

Williams PH, Strange C. Assessing Community Engagement Factors to Support Individuals Impacted by a Rare Disease: Alpha-1 Antitrypsin Deficiency. Internet J Advanced Nursing Practice 2013, 12(1).

Holm KE, Wamboldt FS, Ford DW, Sandhaus RA, Strand M, Strange C, Hoth KF. Prospective Association of Perceived Criticism with Dyspnea in Chronic Lung Disease. J Psychosom Res. 2013 May; 74: 450-3. doi: 10.1016/j.jpsychores.2013.02.001. Epub 2013 Feb 28. PMID: 23597335
 
Holm KE, Borson S, Sandhaus RA, Ford DW, Strange C, Bowler RP, Make BJ, Wamboldt FS. Differences in Adjustment between Individuals with Alpha-1 Antitrypsin Deficiency (AATD) Associated COPD and Non-AATD COPD. J COPD, April 2013, Vol. 10, No. 2 Pages 226-234. doi: 10.3109/15412555.2012.719049. PMID: 23547634  

Hoth KF, Holm KE, Ford DW, Wamboldt FS, Strange C, Strand S, Sandhaus RA. Prospective Impact of Illness Uncertainty on Outcomes in Chronic Lung Disease. Health Psychology 2013; 32(11):1170-4. PMID 23772888.

Hoth KF, Ford DW, Sandhaus RA, Strange C, Wamboldt FS, Kristen KE. Alcohol Use Predicts ER Visits in Individuals with Alpha-1 Antitrypsin Deficiency (AATD) Associated COPD. J Chronic Obstuctive Pulm Dis 2012, 9:1-9. PMID 22651849.

Carpenter MJ, Strange C, Jones Y, Dickson MR, Carter C, Moseley MA, Gilbert GE. Does genetic testing result in behavioral health change? Changes in smoking behavior following testing for Alpha-1 Antitrypsin Deficiency. Ann Behav Med, 2007, 33(1):22-8. PMID: 17291167

Eden E, Holliday B, Xie L, Strange C. Asthma and allergy in alpha-1 antitrypsin deficiency. Respiratory Medicine, 2006; 100(8):1384-91. Epub 2006 Jan 5. PMID: 16403617

Strange C, Moseley MA, Jones Y, Schwarz L, Xie L, Brantly ML. Genetic testing of minors for alpha-1 antitrypsin deficiency, Arch Pediatr Adolesc Med 2006, 160:531-4. PMID: 16651497
 
Bowlus C, Strange C, Willner I, Reuben A, Xie L, Woolson R, Holladay B. Factors associated with advanced liver disease in adults with alpha1-antitrypsin deficiency. Clinical Gastroenterology and Hepatology 2005, 3(4):390-6. PMID: 15822045
 
Fanos JH, Strange C. 'The lion, the witch, and the wardrobe': impact of siblings of individuals with AAT deficiency. American J Medical Genetics 2004, 130(3):251-7. PMID: 15378547

Strange C, Dickson M, Carter C, Carpenter MJ, Holladay, Lundquist R, Brantly ML. Genetic testing for alpha1-antitrypsin deficiency. Genet Med 2004, 6: 204-10. PMID: 15266208

Mullins CD, Wang J, Stoller JK. Major components of the direct medical costs of alpha1-antitrypsin deficiency. Chest 2003, 124(3):826-31. PMID: 12970004
 
Strange C, Wilner I. Alpha-1 Foundation Research Registry: from the past to the future. J Ped Gastro and Nutrition 2002, 34:1. PMID: 11791523  

Mayer AS, Stoller JK, Bucher Bartelson B, James Ruttenber A, Sandhaus RA, Newman LS. Occupational exposure risks in individuals with PI*Z alpha (1)-antitrypsin deficiency. Am J Respir Crit Care Med 2000, 162(2 Pt 1):553-8. PMID: 10934086

Publications from Registry Recruitment

Strange C, Senior RM, Sciurba F, O'Neal S, Morris A, Wisniewski SR, Bowler R, Hocheiser HS, Michael Becich M, Joseph K. Leader JK, Kaminski N, Sandhaus RA for the GRADS Alpha-1 Study Group. Rationale and design of the genomic research in alpha-1 antitrypsin deficiency and sarcoidosis (GRADS) study: alpha-1 protocol, Ann Am Thorac Soc, 2015,12(10):1551-60. PMID: 26153726

Campos MA, Kueppers F, Stocks JM, Strange C, Chen J, Griffin R, Wang-Smith L, Brantly ML. Safety and pharmacokinetics of 120 mg/kg versus 60 mg/kg weekly intravenous infusions of alpha-1 protease inhibitor in alpha-1 antitrypsin deficiency: a multicenter, randomized, double-blind , crossover study (SPARK). COPD 2013. 10(6):687-95. PMID 23862647.

Mueller C, Flotte TR. Gene-bases therapy for alpha-1 antitrypsin deficiency. COPD 2013 Mar; 10 Suppl 1:44-9 PMID: 23527792

Tan L, Dickens J, DeMeo DL, Miranda E, Perez J, Rashid ST, Day J, Ordoñez A, Marciniak SL, Haq I, Barker AF, Campbell EJ, Eden E, McElvaney NG, Rennard SI, Sandhaus RA, Stocks JM, Stoller JK, Strange C, Turino G, Rouhani F, Brantly M, Lomas DA. Circulating polymers in α1-antitrypsin deficiency. Eur Resp J 2014 May;43(5):1501-4. doi: 10.1183/09031936.00111213. Epub 2014 Mar 6. PMID: 24603821

Siedlinski M, Klanderman B, Sandhaus RA, Barker AF, Brantly ML, Eden E, McElvaney G, Rennard SI, , Stocks JM, Stoller JK, Strange C, Turino G, Campbell EJ, DeMeo DL. Association of cigarette smoking and CRP levels with DNA methylation in Alpha-1 Antitrypsin Deficiency. Epigenetics 2012, 7(7):720-8. PMID 22617718.

Flotte TR, Trapnell BC, Humphries M, Carey B, Calcedo R, Rouhani F, Campbell-Thompson M, Yachnis AT, Sandhaus RA, McElvaney NG, Mueller C, Messina LM, Wilson JM, Brantly M, Knop DR, Ye GJ, Chulay JD. Phase 2 clinical trial of a recombinant adeno-associated viral vector expressing α1-antitrypsin: interim results. Hum Gene Ther. 2011 Oct;22(10):1239-47. doi: 10.1089/hum.2011.053. Epub 2011 Aug 24. PMID: 21609134
 
Holm KE, Bowler RP, Make BJ, Wamboldt FS. Family relationship quality is associated with psychological distress, dyspnea, and quality of life in COPD. COPD, 2009 6(5):359-68. PMID: 19863365

DeMeo DL, Campbell EJ, Barker AF, Brantly ML, Edward Eden E, McElvaney NG, Rennard S, Sandhaus RA, Stocks JM, Stoller JK, Strange C, Turino GM, , Silverman EK. IL10 polymorphisms are associated with airflow obstruction in severe alpha 1-antitrypsin deficiency. Am J Respir Cell Mol Biol; 2008, 38:114-20. PMID: 17690329

DeMeo DL, Sandhaus RA, Barker AF, Brantly ML, Edward Eden E, McElvaney NG, Rennard S, Burchard E, Stocks JM, Stoller JK, Strange C, Turino GM, Campbell EJ, Silverman EK. Determinants of airflow obstruction in severe alpha 1-antitrypsin deficiency. Thorax 2007, 62(9): 806-13. PMID: 17389752
 
Stocks JM, Brantly M, Barker A, Kueppers F, Strange C, Donohue JF, Sandhaus R. Multi-center study: the biochemical efficacy, safety and tolerability of a new alpha-1-proteinase inhibitor, Zemaira. Journal of COPD, 2006,3:17-23. PMID: 17175661