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Article in Japanese
Abstract
Special Reports
Practical guide for the diagnosis and treatment of α1-antitrypsin deficiency in Japan
Toyohiro Hiraia Kuniaki Seyamab Koichiro Tatsumic the Respiratory Failure Research Group of the Japanese Ministry of Health, Labour and Welfare
aDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto University
bDivision of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine
cDepartment of Respirology, Graduate School of Medicine, Chiba University
ABSTRACT
α1-Antitrypsin (AAT) is a major plasma serine protease inhibitor. A deficiency of AAT results in a protease/protease inhibitor imbalance in the lung and increases risk for the development of early-onset emphysema and chronic obstructive impairment. AAT deficiency (AATD) is extremely rare in Japanese compared with Europeans and Americans. According to the research project supported by the Ministry of Health, Labour and Welfare in 2015, AATD has been specified as one of the intractable diseases for which their medical expenses can be subsidized by the government. AAT augmentation therapy as a specific treatment for AATD is expected to be available in Japan.
KEYWORDS
Emphysema COPD Airflow limitation Antiprotease Intractable disease
Received 18 Mar 2016 ／ Accepted 28 Mar 2016
AJRS, 5(4): 159-165, 2016
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