Alpha 1-antitrypsin deficiency medical therapy: Difference between revisions

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**[[Oxygen|Supplemental oxygen]] if needed
**[[Oxygen|Supplemental oxygen]] if needed
**[[Lung transplantation]]
**[[Lung transplantation]]
**[[Treatments|Treatment]] of [[Chronic obstructive pulmonary disease|COPD exacerbation]] in all [[patients]] of AATD should include AAT repletion.
*[[Treatments|Treatment]] of [[Chronic obstructive pulmonary disease|COPD exacerbation]] in all [[patients]] of AATD should include AAT repletion.
* '''1 Alpha 1-antitrypsin deficiency (A1AD)'''  
* '''1 Alpha 1-antitrypsin deficiency (A1AD)'''  
** 1.1 A'''ssociated lung disease'''
** 1.1 A'''ssociated lung disease'''
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*Following infusion AAT levels remain above the protective threshold for most of the dosing interval.
*Following infusion AAT levels remain above the protective threshold for most of the dosing interval.
*The infused AAT has the ability to neutralize [[neutrophil elastase]] activity.
*The infused AAT has the ability to neutralize [[neutrophil elastase]] activity.
*Augmentation therapy recipients demonstrate a slower rate of FEV1 decline than nonrecipients
*Augmentation therapy recipients demonstrate a slower rate of FEV1 decline than nonrecipients.
*Wencker and colleagues conducted a before-after study and found that the greatest effect of augmentation therapy in changing FEV1 slope was observed in individuals with a rapid FEV1 decline before augmentation therapy was initiated (ie, FEV1 decline 256 mL/y before therapy vs 53 mL/y during therapy).
*Wencker and colleagues conducted a before-after study and found that the greatest effect of augmentation therapy in changing FEV1 slope was observed in individuals with a rapid FEV1 decline before augmentation therapy was initiated (ie, FEV1 decline 256 mL/y before therapy vs 53 mL/y during therapy).


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**Follow-up with these [[patients]].
**Follow-up with these [[patients]].


Improving [[lung]] [[Function (biology)|function]]
* Improving [[lung]] [[Function (biology)|function]]:
 
**Efforts should be made to improve [[lung]] [[Function (biology)|function]] in [[patients]] with AATD associated [[emphysema]].
*Efforts should be made to improve [[lung]] [[Function (biology)|function]] in [[patients]] with AATD associated [[emphysema]].
**Administration of short-acting [[beta-adrenergic]] agents and [[ipratropium bromide]] [[bronchodilator]] can help maximize [[lung]] [[Function (biology)|function]]. The preferred route of administration is metered-dose [[inhalers]] because they have a lower [[incidence]] of [[adverse effects]] than other [[Routes of administration|routes]]. These [[drugs]] have no long-term effect on [[disease]] progression.
*Administration of short-acting [[beta-adrenergic]] agents and [[ipratropium bromide]] [[bronchodilator]] can help maximize [[lung]] [[Function (biology)|function]]. The preferred route of administration is metered-dose [[inhalers]] because they have a lower [[incidence]] of [[adverse effects]] than other [[Routes of administration|routes]]. These [[drugs]] have no long-term effect on [[disease]] progression.
**[[Corticosteroids|Inhaled corticosteroids]] provides symptomatic relief in [[patients]] with frequent exacerbations. [[Adverse effect]] includes [[infection]].
*[[Corticosteroids|Inhaled corticosteroids]] provides symptomatic relief in [[patients]] with frequent exacerbations. [[Adverse effect]] includes [[infection]].
**[[Beta-adrenergic-blocking agents|Long-acting inhaled beta-adrenergic drugs]] and [[anticholinergics]] improve [[bronchodilation]] in the [[patient]] [[population]] with AATD.
*[[Beta-adrenergic-blocking agents|Long-acting inhaled beta-adrenergic drugs]] and [[anticholinergics]] improve [[bronchodilation]] in the [[patient]] [[population]] with AATD.
**Reserve [[Corticosteroids|oral corticosteroids]] for acute exacerbations with increased [[cough]] and [[sputum]].  
*Reserve [[Corticosteroids|oral corticosteroids]] for acute exacerbations with increased [[cough]] and [[sputum]].  
**Long-term [[Corticosteroids|corticosteroid]] use is associated with many detrimental adverse effects. Limit [[oral]] [[steroid]] use to brief courses of 1-2 weeks. Start [[therapy]] to prevent [[osteoporosis]] when long courses are administered.
*Long-term [[Corticosteroids|corticosteroid]] use is associated with many detrimental adverse effects. Limit [[oral]] [[steroid]] use to brief courses of 1-2 weeks. Start [[therapy]] to prevent [[osteoporosis]] when long courses are administered.
**A therapeutic trial of [[theophylline]] may be indicated for selected [[patients]]. [[Theophylline]] administration requires frequent monitoring of serum levels as it has a narrow therapeutic range and its [[metabolism]] is frequently altered by other [[drugs]] or [[Illnesses|illness]], which can lead to episodes of [[drug toxicity]]. [[Theophylline]] is [[metabolized]] by the [[liver]]. [[Smoking]] increases the [[metabolism]] of [[theophylline]].
*A therapeutic trial of [[theophylline]] may be indicated for selected [[patients]]. [[Theophylline]] administration requires frequent monitoring of serum levels as it has a narrow therapeutic range and its [[metabolism]] is frequently altered by other [[drugs]] or [[Illnesses|illness]], which can lead to episodes of [[drug toxicity]]. [[Theophylline]] is [[metabolized]] by the [[liver]]. [[Smoking]] increases the [[metabolism]] of [[theophylline]].


==References==
==References==

Latest revision as of 18:02, 22 January 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]

Overview

Treatment guidelines for AATD include alpha 1 antitrypsin enzyme repletion, smoking cessation, long-acting inhaled bronchodilators, preventive vaccinations against influenza and pneumococcus, pulmonary rehabilitation for patients with functional impairment, supplemental oxygen if needed lung transplantation, treatment of COPD exacerbation in all patients of AATD should include AAT repletion.

Medical Therapy

References

  1. Petrache I, Hajjar J, Campos M (2009). "Safety and efficacy of alpha-1-antitrypsin augmentation therapy in the treatment of patients with alpha-1-antitrypsin deficiency". Biologics. 3: 193–204. PMC 2726081. PMID 19707408.
  2. Edgar RG, Patel M, Bayliss S, Crossley D, Sapey E, Turner AM (2017). "Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review". Int J Chron Obstruct Pulmon Dis. 12: 1295–1308. doi:10.2147/COPD.S130440. PMC 5422329. PMID 28496314.


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