Literature DB >> 26321218

Inhaled alpha1-proteinase inhibitor therapy in patients with cystic fibrosis.

Amit Gaggar1, Junliang Chen2, James F Chmiel3, Henry L Dorkin4, Patrick A Flume5, Rhonda Griffin6, David Nichols7, Scott H Donaldson8.   

Abstract

BACKGROUND: Inhaled alpha1-proteinase inhibitor (PI) is known to reduce neutrophil elastase burden in some patients with CF. This phase 2a study was designed to test inhaled Alpha-1 HC, a new aerosolized alpha1-PI formulation, in CF patients.
METHODS: We performed a randomized, double-blind, placebo-controlled study and evaluated the safety of 100 or 200mg of inhaled Alpha-1 HC once daily for 3 weeks in subjects with CF. Thirty adult subjects were randomized in a 2:1 ratio to receive Alpha-1 HC or placebo.
RESULTS: Drug delivery was confirmed by a dose-dependent increase in the sputum alpha1-PI. Seven (20.0%) of the 35 adverse events in the 100-mg dose group, 3 (13.0%) of 23 in the 200-mg dose group, and 4 (14.3%) of 28 in the placebo group were drug-related in these subjects. One serious adverse event occurred in 1 subject within each group.
CONCLUSIONS: Alpha-1 HC inhalation was safe and well tolerated.
Copyright © 2015. Published by Elsevier B.V.

Entities:  

Keywords:  Alpha-1 HC; Alpha-1 antitrypsin; Cystic fibrosis; Prolastin

Mesh:

Substances:

Year:  2015        PMID: 26321218      PMCID: PMC4993024          DOI: 10.1016/j.jcf.2015.07.009

Source DB:  PubMed          Journal:  J Cyst Fibros        ISSN: 1569-1993            Impact factor:   5.482


  22 in total

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4.  Aerosol alpha 1-antitrypsin treatment for cystic fibrosis.

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8.  Prolastin aerosol therapy and sputum taurine in cystic fibrosis.

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  19 in total

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Review 4.  The Serpin Superfamily and Their Role in the Regulation and Dysfunction of Serine Protease Activity in COPD and Other Chronic Lung Diseases.

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Review 9.  Glycosaminoglycans as Multifunctional Anti-Elastase and Anti-Inflammatory Drugs in Cystic Fibrosis Lung Disease.

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