A NEW VARIANT OF ALPHA-1-ANTITRYPSIN DEFICIENCY (SIIYAMA) ASSOCIATED WITH PULMONARY-EMPHYSEMA

被引：7
作者：
TAKABE, K
SEYAMA, K
SHINADA, H
NOUCHI, T
MIYAHARA, Y
NUKIWA, T
MIYAKE, K
TSUKIMOTO, K
ICHIOKA, M
MARUMO, F
机构：
来源：
INTERNAL MEDICINE | 1992年 / 31卷 / 05期
关键词：
TAMOXIFEN; AUGMENTATION THERAPY; HUMAN ALPHA-1-ANTITRYPSIN (AAT);
D O I：
10.2169/internalmedicine.31.702
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
A 38-year-old male with pulmonary emphysema due 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.
引用
收藏
页码：702 / 707
页数：6
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