User:Alteripse/AAT

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Review of current therapies. [1]

Genetics. [2]

Prospects for gene therapy. [3]

Molecular pathophysiology. [4]

Possible advantages of AAT. [5]

Epidemiology. [6]

Abnormal AAT accumulation attracts neutrophils. [7]

Clinical manifestations and natural history. [8] xxx

Liver aspects of AAT. [9]

AAT in 26 year olds. [10]

New treatments. [11]

Risk modification. [12]

CT scanning for monitoring. [13]

Gene therapy for AAT; recent attempts. [14]

Perspectives on treatments as of 2000. [15]

New perspective on pathophysiology: damage results from overload as well as deficiency. [16]

Monitoring with CT. [17]

Brief review and intro to thorax review series. [18]

Current iv therapies for AAT. [19]

Lancet review already cited. [20] xxx

Mortality is earlier mainly due to lung and liver disease. [21]

Delayed diagnosis. [22]

Alpha-1 foundation. [23]

More than a PI imbalance? [24]

AAT in 86 yr old, cirrhosis, no emphysema, normal life expectancy. [25]


[edit] References

  1. ^ Abusrilwill H, Stockley RA. "Alpha-1-antitrypsin deficiency replacement therapy: current status.". Curr Opinion Pulm Med 12: 125-31. PMID 16456382. 
  2. ^ DeMeo DL, Silverman EK (2004). "α1-antitrypsin deficiency 2: Genetic aspects of α1-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk.". Thorax 59: 259-64. PMID 14985567. 
  3. ^ Driskell RA, Engelhardt JF (2003). "Current status of gene therapy for inherited lung diseases.". Ann Rev Physiol 65: 585-612. PMID 12524461. 
  4. ^ Lomas DA, Parfrey H (2004). "α1-antitrypsin deficiency 4: Molecular pathophysiology.". Thorax 365: 529-35. PMID 15170041. 
  5. ^ Lomas DA (2006). "The selective advantage of α1-antitrypsin deficiency.". Am J Resp Crit Care Med 173: 1072-7. PMID 16439713. 
  6. ^ Luisetti M, Seersholm N (2004). "α1-antitrypsin deficiency 1: Epidemiology of alpha1-antitrypsin deficiency.". Thorax 59: 164-9. PMID 14760160. 
  7. ^ Mulgrew AT, Taggart CC, Lawless MW, Greene CM, Brantly ML, O'Neill SJ, McElvaney NG (2004). "Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant.". Chest 125 (5): 1607-9. PMID 15136365. 
  8. ^ Needham M, Stockley RA (2004). "α1-antitrypsin deficiency 3: Clinical manifestations and natural history.". Thorax 59: 441-5. PMID 15115878. 
  9. ^ Perlmutter DH (2004). "Alpha1-antitrypsin deficiency: diagnosis and treatment.". Clin Liver Dis 8: 839-59. PMID 15464658. 
  10. ^ Piitulainen E, Carlson J, Ohlsson K, Sveger T (2005). "α1-antitrypsin deficiency in 26-year-old subjects: lung, liver, and protease/protease inhibitor studies.". Chest 128: 2076-81. PMID 16236857. 
  11. ^ Sandhaus RA (2004). "α1-antitrypsin deficiency 6: New and emerging treatments for α1-antitrypsin deficiency.". Thorax 365: 904-9. PMID 15454659. 
  12. ^ Senn O, Russi EW, Imboden M, Probst-Hensch NM (2005). "α1-Antitrypsin deficiency and lung disease: risk modification by occupational and environmental inhalants.". Eur Resp J 26 (5): 909-17. PMID 16264055. 
  13. ^ Shaker SB, Stavngaard T, Stolk J, Stoel B, Dirksen A (2004). "α1-antitrypsin deficiency 7: Computed tomographic imaging in α1-antitrypsin deficiency.". Thorax 59: 986-91. PMID 15516477. 
  14. ^ Stecenko AA, Brigham KL (2003). "Gene therapy progress and prospects: α1-antitrypsin.". Gene therapy 10 (2): 95-9. PMID 12571637. 
  15. ^ Stockley RA (2000). "Alpha1-antitrypsin deficiency: what next.". Thorax 55: 614-8. PMID xxxx. 
  16. ^ Stockley RA (2004). "Alpha1-antitrypsin: more than just deficiency.". Thorax 59 (5): 363-4. PMID 15115856. 
  17. ^ Stolk J, Ng WH, Bakker ME, Reiber JHC, Rabe KF, Putter H, Stoel BC (2003). "Correlation between annual change in health status and computer tomography derived lung density in subjects with α1-antitrypsin deficiency.". Thorax 58: 1027-30. PMID xxxx. 
  18. ^ Stoller JK (2004). "α1-antitrypsin deficiency.". Thorax 59 (2): 92-3. PMID 14760142. 
  19. ^ Stoller JK, Aboussouan LS (2004). "α1-antitrypsin deficiency 5: Intravenous augmentation therapies: current understanding.". Thorax 59: 708-12. PMID 15282394. 
  20. ^ Stoller J, Aboussouan L. "Alpha1-antitrypsin deficiency.". Lancet 365 (9478): 2225-36. PMID 15978931. 
  21. ^ Stoller JK, Tomashefski J, Crystal RG, Arroliga A, Strange C, Killian DN, Schluchter MD, Wiedemann HP (2005). "Mortality in individuals with severe deficiency of α1-antitrypsin: findings from the National Heart, Lung, and Blood Institute Registry.". Chest 127: 1196-1204. PMID 15821195. 
  22. ^ Stoller JK, Sandhaus RA, Turino G, Dickson R, Rodgers K, Strange C (2005). "Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem.". Chest 128 (4): 1989-94. PMID 16236846. 
  23. ^ Walsh JW, Snider GL, Stoller JK (2006). "A review of the alpha-1 foundation: its formation, impact, and critical success factors.". Resp Care 51 (5): 526-31. PMID 16638163. 
  24. ^ Wewers MD (2004). "α1-Antitrypsin deficiency: more than a protease imbalance?". Chest 125: 1607-9. PMID 15136365. 
  25. ^ Willson AB, Seow C, Zimmerman M. "Severe alpha-1 antitrypsin deficiency diagnosed n an 86-year-old man.". Internal Med J 34: 653. PMID 15546465.