Demographics and Clinical Profile of COPD Patients With Alpha-1 Antitrypsin Deficiency PiMZ Genotype Started on Augmentation Therapy

CHEST Journal ◽  
2014 ◽  
Vol 146 (4) ◽  
pp. 42A
Author(s):  
Navid Zaidi ◽  
Debra Greek ◽  
Crystal Piepenbrink
Keyword(s):  
Clinical Profile ◽  
Augmentation Therapy ◽  
Copd Patients ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

CLINICAL PROFILE AND OUTCOMES OF ALPHA-1 ANTITRYPSIN DEFICIENCY CARRIERS RECEIVING AUGMENTATION THERAPY IN THE HOME

CHEST Journal ◽  
2018 ◽  
Vol 154 (4) ◽  
pp. 739A
Author(s):  
ALLYSON CHECKLEY ◽  
SAMANTHA KILE ◽  
LORETTA KRISTOFEK ◽  
BILL BOLGAR ◽  
DONALD SCHMECHEL
Keyword(s):  
Clinical Profile ◽  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Medical Costs of Alpha-1 Antitrypsin Deficiency-Associated COPD in the United States

2019 ◽  
Author(s):  
Jan Sieluk ◽  
Julia F Slejko ◽  
Henry Silverman ◽  
Eleanor Perfetto ◽  
C. Daniel Mullins
Keyword(s):  
Index Date ◽  
Healthcare Resource ◽  
The United States ◽  
Chronic Obstructive ◽  
Augmentation Therapy ◽  
Copd Patients ◽  
Before And After ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Abstract Background There are limited data on economic aspects of the genetic variant of chronic obstructive pulmonary disease (COPD) in the context of the more prevalent form of COPD. The objective of this study was to isolate the healthcare resource utilization and economic burden attributable to the presence of a genetic factor among COPD patients with and without Alpha-1 Antitrypsin Deficiency (AATD), twelve months before and after their initial COPD diagnosis.Results The study population consisted of 8,881 patients (953 cases matched with 7,928 controls). The AATD-associated COPD cohort had higher expenditures and use of OV and OTH services, as well as OV, OP, ER, RX, and OTH before and after the index date, respectively. Adjusted total cost ratios for AATD-associated COPD patients as compared to controls were 2.04 [95% CI: 1.60 – 2.59] and 1.98 [95% CI: 1.55 – 2.52] while the incremental cost difference totaled $6,861 [95% CI: $3,025 - $10,698] and $5,772 [95% CI: $1,940 - $9,604] per patient before and after the index date, respectively.Conclusions Twelve months before and after their initial COPD diagnosis, patients with AATD incur higher healthcare utilization costs that are double the cost of similar COPD patients without AATD. This study also suggests that increased costs of AATD-associated COPD are not solely attributable to augmentation therapy use. Future studies should further explore the relationship between augmentation therapy, healthcare resource use, and other AATD-associated COPD expenditures.

scholarly journals Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know

2021 ◽  
Vol 12_suppl ◽  
pp. 204062232110101
Author(s):  
Igor Barjaktarevic ◽  
Michael Campos
Keyword(s):  
Lung Disease ◽  
Treatment Guidelines ◽  
Management Program ◽  
Lifestyle Changes ◽  
Chronic Obstructive ◽  
Augmentation Therapy ◽  
Lung Function Decline ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Management of lung disease in patients with alpha-1 antitrypsin deficiency (AATD) includes both non-pharmacological and pharmacological approaches. Lifestyle changes with avoidance of environmental pollutants, including tobacco smoke, improving exercise levels and nutritional status, all encompassed under a disease management program, are crucial pillars of AATD management. Non-pharmacological therapies follow conventional treatment guidelines for chronic obstructive pulmonary disease. Specific pharmacological treatment consists of administering exogenous alpha-1 antitrypsin (AAT) protein intravenously (augmentation therapy). This intervention raises AAT levels in serum and lung epithelial lining fluid, increases anti-elastase capacity, and decreases several inflammatory mediators in the lung. Radiologically, augmentation therapy reduces lung density loss over time, thus delaying disease progression. The effect of augmentation therapy on other lung-related outcomes, such as exacerbation frequency/length, quality of life, lung function decline, and mortality, are less clear and questions regarding dose optimization or route of administration are still debatable. This review discusses the rationale and available evidence for these interventions in AATD.

Differences in training response between patients with alpha-1 antitrypsin deficiency and COPD patients

2015 ◽  
Author(s):  
Inga Heinzelmann ◽  
Sebastian Gehlert ◽  
Tobias Welte ◽  
Wilhelm Bloch ◽  
Sabina Janciauskiene ◽  
...  
Keyword(s):  
Copd Patients ◽  
Training Response ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Natural Progression of Alpha-1 Antitrypsin Deficiency Among Hospitalized COPD Patients in the US

CHEST Journal ◽  
2015 ◽  
Vol 148 (4) ◽  
pp. 497A
Author(s):  
Christopher Blanchette ◽  
Joshua Noone ◽  
Debosree Roy ◽  
Bryce Van Doren ◽  
Emily Zacherle ◽  
...  
Keyword(s):  
Copd Patients ◽  
Natural Progression ◽  
The Us ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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