How are we in Brazil with the treatment of alpha-1 antitrypsin deficiency?

Castellano, M.; Feitosa, Paulo Henrique Ramos

doi:10.36416/1806-3756/e20210445

Cited by 1 publication

(1 citation statement)

References 9 publications

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“…4 AAT treatment is provided through regulatory approvals in North America (Canada and USA), Europe (Austria, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland) and Asia (Japan). 26 However, there is still much work to be carried out in developing countries. For instance, in Brazil, treatment with AAT is typically obtained through legal action.…”

Section: General Therapiesmentioning

confidence: 99%

Diagnosis and augmentation therapy for alpha-1 antitrypsin deficiency: current knowledge and future potential

Feitosa¹

2023

DIC

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The underdiagnosis of alpha-1 antitrypsin (AAT) deficiency (AATD) has been recognized for many years, yet little progress has been made in treatment of the disease. In this review, we summarize the AATD disease process as well as its diagnosis and treatment by AAT augmentation therapy. AATD is a rare autosomal disease that primarily affects the lungs and liver. AATD is associated with an increased susceptibility to developing pulmonary emphysema. The specific pharmacological treatment for AATD is intravenous administration of exogenous AAT. Augmentation therapy with AAT increases serum and pulmonary epithelial AAT levels, restores anti-elastase capacity, and decreases inflammatory mediators in the lung. Augmentation therapy reduces the loss of lung density over time, thus slowing progression of the disease. The effects of augmentation therapy on outcomes, such as frequency/duration of flare-ups, quality of life, lung function decline and mortality, are assessed. Wider testing for AATD, potentially through primary care physicians, could result in earlier treatment and better outcomes for individuals with AATD-induced lung respiratory disease.

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Section: General Therapiesmentioning

confidence: 99%

Diagnosis and augmentation therapy for alpha-1 antitrypsin deficiency: current knowledge and future potential

Feitosa¹

2023

DIC

View full text Add to dashboard Cite

show abstract

How are we in Brazil with the treatment of alpha-1 antitrypsin deficiency?

Cited by 1 publication

References 9 publications

Diagnosis and augmentation therapy for alpha-1 antitrypsin deficiency: current knowledge and future potential

Diagnosis and augmentation therapy for alpha-1 antitrypsin deficiency: current knowledge and future potential

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