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

An important study that was derived from Registry participants was published this spring in the American Review of Respiratory and Critical Care Medicine. This study examined whether occupational exposures to mineral dust, fumes, or smoke contribute to cough or low lung function in Alpha-1. The findings are strong evidence that a clean air environment is important to lung health for individuals with Alpha-1. For full abstract at the National Library of Medicine.

2. Stoller J, Brantley M, Fleming LE, Bean JA, Walsh J. Formation and current results of a patient-organized registry for alpha(1)-antitrypsin deficiency. Chest 2000; 118(3):843-8.

The design and initial description of Registry participants was just published in the journal, Chest. This description is interesting reading for Registry participants since it shows how diverse a population the Alpha-1 community is and compares the current registry to the National Institute of Health Registry that ended in 1996. For full abstract at the National Library of Medicine.

3.Mullins C.D., Ph.D.; H. Xingue, M.S.; S. Merchant, M.S., MBA; and J.K. Stoller, M.D., FCCP. The Direct Medical Cost of Alpha1-Antitrypsin Deficiency Chest 2001; 119(3): 745-50 
The cost of illness study conducted by Dr. Dan Mullins at the University of Maryland has been completed and published in the March 2001 edition of the journal, Chest. The study surveyed 292 Alpha One Research Registry members to assess annual expenditures by affected individuals. Data regarding healthcare costs, alpha-1 status and demographic information was collected from a research questionnaire between the years 1997 and 1999. Self-reported cost information collected from 292 PI*ZZ-phenotype individuals revealed an annual average healthcare expenditure of $30,948. In comparison the annual average healthcare costs reported by 53 non-PI*ZZ-phenotype individuals included in the study was $20,673. The annual average costs reported by 185 PI*ZZ individuals receiving AAT replacement therapy was estimated to be even higher at $40,123/yr. Self-reported cost estimates were then compared to cost estimates based on Medicare reimbursement rates for AAT replacement therapy. Results showed the mean annual cost of AAT replacement therapy alone to be >$50,000 for men and > $30,000 for women. Therefore, comparison of the anticipated yearly cost of AAT replacement therapy with the average total annual expenditures reported by AAT replacement recipients suggests subjects to be underestimating their total yearly costs. The study concludes that the healthcare costs incurred to persons diagnosed with Alpha1-Antitrypsin Deficiency are very high with or without replacement therapy as compared to healthcare cost incurred by individuals not affected by Alpha-1.


















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Sifers, Richard N., Finegold M.J., and Woo S.L.C. Alpha1-Antitrypsin Deficiency: Accumulation or Degradation of Mutant Variants within Hepatic Endoplasmic Reticulum. American J. Respir. Cell Mol. Biol. 1989; 1: 341-345

Dongfeng, Qu, Teckman J.H. and Perlmutter D.H. Review: Alpha1-Antitrypsin Deficiency associated liver disease. Journal of Gastroenterology and Hepatology 1997; 12: 404-416

The Alpha1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 Decline in Individuals with Severe Deficiency of Alpha1-Antitrypsin. American Journal of Respiratory Critical Care Medicine 1998; 158: 49-59

Ferkol T., Mularo F., Hilliard J., Lodish S., Perales J.C., Ziady A., and Konstan M. Transfer of the Human Alpha1-Antitrypsin Gene into Pulmonary Macrophages In Vivo. Am J. of Respir. Cell Mol. Biol. 1998; 18: 591-601

Wencker M., Banik N., Buhl R., Seidel R. and Konietzko N. Long-term Treatment of Alpha1-Antitrypsin Deficiency-Related Pulmonary Emphysema with Human Alpha1-Antitrypsin. Eur. Respir. Journal 1998; 11: 428-433

Eckman E.A., Mallender W.D., Szegletes T., Silski C.L. and Schreiber J.R. In Vitro Transport of Active Alpha1-Antitrypsin to the Apical Surface of Epithelia by Targeting the Polymeric Immunoglobulin Receptor. Am J of Respir. Cell Mol. Biol. 1999; 21: 246-252

Coakley R.J., Taggart C., O'Neil S. and McElvaney N.G. Alpha1-Antitrypsin Deficiency: Biological Answers to Clinical Questions. Am J Med Sci 2001; 321(1): 33-41 Review