A New Variant of Alpha-1-Antitrypsin Deficiency (Siiyama) Associated with Pulmonary Emphysema.
スポンサーリンク
概要
- 論文の詳細を見る
A 38-year-old male with pulmonary emphysemadue to severely reduced serum alpha-1-antitrypsin (AAT) level (14.5 mg/dl) was found to have an inherited new AAT deficient variant Siiyama. Chest roentogenogram and CT scanning revealed advanced emphysema, and severe obstructive ventilatory impairment was observed. During the 4-year follow-up period, the annual rate of decline of FEV 1.0 showed approximately 10-fold greater than the normal decline in FEV 1.0 (-380 ml/yr). Treatment with tamoxifen in order to raise the serum AAT level only resulted in an insufficient increase. Augmentation therapy of human AAT should be considered in the future.(Internal Medicine 31 : 702-707, 1992)
- 社団法人 日本内科学会の論文
著者
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Marumo Fumiaki
The Second Department Of Internal Medicine Tokyo Medical And Dental University
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Ichioka Masahiko
The Second Department Of Internal Medicine Tokyo Medical And Dental University
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SEYAMA Kuniaki
the Department of Respiratory Medicine, Juntendo University Medical School
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NUKIWA Toshihiro
the Department of Respiratory Medicine, Juntendo University School of Medicine
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TAKABE Kazuhiko
the Department of Internal Medicine, Hokushin General Hospital
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SHINADA Hideho
the Department of Internal Medicine, Hokushin General Hospital
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NOUCHI Toshihiko
the Department of Internal Medicine, Hokushin General Hospital
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MIYAHARA Yasuhiro
the Department of Internal Medicine, Hokushin General Hospital
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MIYAKE Kazuhiko
the First Department of Internal Medicine, Teikyo University School of Medicine
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TSUKIMOTO Koichi
the Second Department of Internal Medicine, Tokyo Medical and Dental University School of Medicine
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ICHIOKA Masahiko
the Second Department of Internal Medicine, Tokyo Medical and Dental University School of Medicine
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