Published in Chest on October 01, 2005
Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency. Clin Gastroenterol Hepatol (2011) 0.94
The prevalence of alpha-1 antitrypsin deficiency in Ireland. Respir Res (2011) 0.93
Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis. Drug Des Devel Ther (2011) 0.92
Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach. Orphanet J Rare Dis (2013) 0.85
Alpha-1-antitrypsin deficiency in Serbian adults with lung diseases. Genet Test Mol Biomarkers (2012) 0.85
Alpha-1 couples: interpersonal and intrapersonal predictors of spousal communication and stress. J Genet Couns (2013) 0.84
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The role of omalizumab in the treatment of severe allergic asthma. Can Respir J (2006) 0.81
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Classifying married adults diagnosed with alpha-1 antitrypsin deficiency based on spousal communication patterns using latent class analysis: insights for intervention. J Genet Couns (2013) 0.80
Support Seeking or Familial Obligation: An Investigation of Motives for Disclosing Genetic Test Results. Health Commun (2015) 0.78
Results from a large targeted screening program for alpha-1-antitrypsin deficiency: 2003 - 2015. Orphanet J Rare Dis (2016) 0.78
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Validation and development of an immunonephelometric assay for the determination of alpha-1 antitrypsin levels in dried blood spots from patients with COPD. J Bras Pneumol (2013) 0.77
Alpha1-antitrypsin deficiency: a clinical-genetic overview. Appl Clin Genet (2011) 0.77
Characteristics of Alpha-1 Antitrypsin-Deficient Individuals in the Long-term Oxygen Treatment Trial and Comparison with Other Subjects with Chronic Obstructive Pulmonary Disease. Ann Am Thorac Soc (2015) 0.77
Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol. Ann Am Thorac Soc (2015) 0.77
Keeping Secrets or Educating Others: A Dyadic Analysis of Group Entitativity's Influence on Spouses' Label Management Connected to AATD. Health Commun (2015) 0.75
Are differences in travel time or distance to healthcare for adults in global north countries associated with an impact on health outcomes? A systematic review. BMJ Open (2016) 0.75
Selected metabolic aspects of elastin and collagen fiber proteolysis in diseases of the respiratory system - the significance of α1 antitrypsin deficiency. Kardiochir Torakochirurgia Pol (2016) 0.75
Alpha-1 antitrypsin Pi*Z gene frequency and Pi*ZZ genotype numbers worldwide: an update. Int J Chron Obstruct Pulmon Dis (2017) 0.75
Development and results of the Spanish registry of patients with alpha-1-antitrypsin deficiency. Int J Chron Obstruct Pulmon Dis (2007) 0.75
Application of a diagnostic algorithm for the rare deficient variant Mmalton of alpha-1-antitrypsin deficiency: a new approach. Int J Chron Obstruct Pulmon Dis (2016) 0.75
Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med (2006) 9.17
Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med (2011) 6.74
A randomized study of endobronchial valves for advanced emphysema. N Engl J Med (2010) 6.23
An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med (2012) 5.37
Clinical practice. Alpha1-antitrypsin deficiency. N Engl J Med (2009) 3.49
Long-term effects of inhaled corticosteroids on FEV1 in patients with chronic obstructive pulmonary disease. A meta-analysis. Ann Intern Med (2003) 2.94
Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med (2007) 2.86
Phase 2 clinical trial of a recombinant adeno-associated viral vector expressing α1-antitrypsin: interim results. Hum Gene Ther (2011) 2.75
Evaluation of an intensive insulin protocol for septic patients in a medical intensive care unit. Crit Care Med (2006) 2.22
Biologic lung volume reduction in advanced upper lobe emphysema: phase 2 results. Am J Respir Crit Care Med (2009) 1.78
Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol (2009) 1.78
Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med (2013) 1.69
alpha1-Antitrypsin augmentation therapy for PI*MZ heterozygotes: a cautionary note. Chest (2008) 1.60
Treatment of pulmonary arterial hypertension: a preliminary decision analysis. Chest (2003) 1.54
Neurocognitive impairment in obstructive sleep apnea. Chest (2012) 1.50
Clinical characteristics of subjects with symptoms of alpha1-antitrypsin deficiency older than 60 years. Chest (2008) 1.47
Validation of claims-based diagnostic and procedure codes for cardiovascular and gastrointestinal serious adverse events in a commercially-insured population. Pharmacoepidemiol Drug Saf (2010) 1.43
COPD and lung cancer: are they both airways diseases? Chest (2010) 1.41
Obese man treated with drotrecogin alfa (activated). Ann Pharmacother (2003) 1.38
Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease: results from the scleroderma lung study. Arthritis Rheum (2007) 1.35
Does genetic testing result in behavioral health change? Changes in smoking behavior following testing for alpha-1 antitrypsin deficiency. Ann Behav Med (2007) 1.27
Pneumothorax in cystic fibrosis. Chest (2005) 1.25
Human Treg responses allow sustained recombinant adeno-associated virus-mediated transgene expression. J Clin Invest (2013) 1.20
Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency. Thorax (2007) 1.12
Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med (2010) 1.09
IL10 polymorphisms are associated with airflow obstruction in severe alpha1-antitrypsin deficiency. Am J Respir Cell Mol Biol (2007) 1.08
Trends in the diagnosis of symptomatic patients with alpha1-antitrypsin deficiency between 1968 and 2003. Chest (2005) 1.06
Factors associated with advanced liver disease in adults with alpha1-antitrypsin deficiency. Clin Gastroenterol Hepatol (2005) 1.04
Neutrophil elastase-mediated lung disease. COPD (2013) 1.02
Emphysema lung lobe volume reduction: effects on the ipsilateral and contralateral lobes. Eur Radiol (2012) 0.94
Mortality in individuals with severe deficiency of alpha1-antitrypsin: findings from the National Heart, Lung, and Blood Institute Registry. Chest (2005) 0.91
Baseline regional perfusion impacts exercise response to endobronchial valve therapy in advanced pulmonary emphysema. Chest (2013) 0.90
Impact of race and baseline PSA on longitudinal PSA. Int J Cancer (2006) 0.90
Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival. Chest (2008) 0.90
Detection of alpha-1 antitrypsin deficiency by respiratory therapists: experience with an educational program. Respir Care (2013) 0.88
Association of IREB2 and CHRNA3 polymorphisms with airflow obstruction in severe alpha-1 antitrypsin deficiency. Respir Res (2012) 0.88
A 56-year-old woman with arm pain, dyspnea, and an elevated diaphragm. Chest (2008) 0.87
Risk factors for symptom onset in PI*Z alpha-1 antitrypsin deficiency. Int J Chron Obstruct Pulmon Dis (2006) 0.86
Chest ultrasound for "Dummies". Chest (2003) 0.86
Circulating polymers in α1-antitrypsin deficiency. Eur Respir J (2014) 0.85
Empiric auto-titrating CPAP in people with suspected obstructive sleep apnea. J Clin Sleep Med (2010) 0.85
Primary care diagnosis of alpha-1 antitrypsin deficiency: issues and opportunities. Cleve Clin J Med (2007) 0.85
Effects of a disease management program in individuals with alpha-1 antitrypsin deficiency. COPD (2009) 0.85
Genetic testing for alpha1-antitrypsin deficiency. Genet Med (2004) 0.85
Scleroderma lung disease: "if you don't know where you are going, any road will take you there". Am J Respir Crit Care Med (2008) 0.84
Expanded carrier screening panels-does bigger mean better? J Community Genet (2013) 0.83
Barriers to genetic testing among persons at risk for alpha-1 antitrypsin deficiency. Genet Test (2008) 0.83
Quantitative airway assessment on computed tomography in patients with alpha1-antitrypsin deficiency. COPD (2009) 0.82
Asthma and allergy in alpha-1 antitrypsin deficiency. Respir Med (2006) 0.82
The prevalence of alpha-1 antitrypsin deficiency among patients found to have airflow obstruction. COPD (2012) 0.82
Results of a survey of patients with alpha-1 antitrypsin deficiency. Respiration (2005) 0.81
Association of cigarette smoking and CRP levels with DNA methylation in α-1 antitrypsin deficiency. Epigenetics (2012) 0.81
The impact of age on outcomes in chronic obstructive pulmonary disease differs by relationship status. J Behav Med (2013) 0.81
Adverse events during the Scleroderma Lung Study. Am J Med (2011) 0.81
Impact of asthma control on sleep, attendance at work, normal activities, and disease burden. Ann Allergy Asthma Immunol (2010) 0.81
Perspectives for improving the evaluation and access of therapies for rare lung diseases in Europe. Respir Med (2012) 0.80
Segmental approach to lung volume reduction therapy for emphysema patients. Respiration (2014) 0.80
Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapy. Respir Med (2009) 0.80
Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med (2016) 0.80
Radiation therapy for palliation of Eisenmenger's syndrome-associated painful splenomegaly. Radiat Med (2008) 0.79
Treatment for secondary pulmonary hypertension. Chest (2005) 0.79
Multi-center study: the biochemical efficacy, safety and tolerability of a new alpha1-proteinase inhibitor, Zemaira. COPD (2006) 0.78
"The lion, the witch and the wardrobe": impact on sibs of individuals with AAT deficiency. Am J Med Genet A (2004) 0.78
Is PiSS Alpha-1 Antitrypsin Deficiency Associated with Disease? Pulm Med (2010) 0.78
Pharmacokinetic comparability of Prolastin®-C to Prolastin® in alpha₁-antitrypsin deficiency: a randomized study. BMC Clin Pharmacol (2010) 0.78
Effect of obstructive sleep apnea treatment on mail-in cognitive function screening instrument. Am J Med Sci (2014) 0.78
Prospective impact of illness uncertainty on outcomes in chronic lung disease. Health Psychol (2013) 0.78
Aclidinium bromide plus formoterol for the treatment of chronic obstructive pulmonary disease. Expert Opin Pharmacother (2015) 0.77
Alpha-1 Foundation research registry: from the past to the future. J Pediatr Gastroenterol Nutr (2002) 0.77
The prospective association of perceived criticism with dyspnea in chronic lung disease. J Psychosom Res (2013) 0.77
Alcohol use predicts ER visits in individuals with alpha-1 antitrypsin deficiency (AATD) associated COPD. COPD (2012) 0.77
The Ratio of Free to Bound Desmosine and Isodesmosine May Reflect Emphysematous Changes in COPD. Lung (2015) 0.77
Is systemic sclerosis interstitial lung disease slowly progressive? J Bras Pneumol (2011) 0.76
Patterns of Emphysema Heterogeneity. Respiration (2015) 0.76
Augmentation therapy in alpha-1 antitrypsin deficiency. COPD (2009) 0.75
Unilateral pulmonary artery aplasia in a pregnant patient. Case Rep Med (2011) 0.75
The social environment and illness uncertainty in chronic obstructive pulmonary disease. Int J Behav Med (2015) 0.75
Differences in adjustment between individuals with alpha-1 antitrypsin deficiency (AATD)-associated COPD and non-AATD COPD. COPD (2013) 0.75