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Abstract

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Article in Japanese

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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