Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations.

Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations. Research Abstract Details 

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Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations. Abstract Text:

O A Minai,J K Stoller,

alpha(1)-Antitrypsin (A1AT) deficiency is inherited as an autosomal codominant disorder characterised by reduced levels of A1AT in the serum. Low levels of A1AT in blood perfusing the lung cause low levels in the lung interstitium, making it susceptible to proteolytic damage from resident neutrophil elastase. A 'protective threshold' serum A1AT level of 11 micromol/L has been identified by epidemiological studies as a minimum value below which there is an increased risk of emphysema. Intravenous augmentation therapy for patients with severe deficiency of A1AT has been shown to have biochemical efficacy. Although the clinical efficacy of intravenous augmentation therapy has not been demonstrated in a randomised clinical trial, available studies suggest that augmentation therapy is associated with a slowed rate of decline of lung function and enhanced survival. The criteria for patient selection include: age >18 years, serum A1AT level

Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations. Publishing Authors By Initials

OA Minai,JK Stoller,

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Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: BioDrugs : clinical immunotherapeutics, biopharmac

VOLUME: 13

Page Numbers: 135-47

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ISSN: 1173-8804

DAY: 23

MONTH: Feb

YEAR: 2000

Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations. Information

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LANGUAGE: eng

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Grant and Affiliation Information for Therapy for alpha(1)-Antitrypsin Deficiency: Pharmacology and Clinical Recommendations.

AFFILIATION: Department of Pulmonary and Critical Care Medicine, The Cleveland Clinic Foundation, Cleveland, Ohio, USA.

Country: New Zealand

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MEDLINETA: BioDrugs

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