中华皮肤科杂志 ›› 1988, Vol. 21 ›› Issue (5): 296-298.

• 论著 • 上一篇    下一篇

一个遗传性血管性水肿家系的调查研究

张宏誉1, 张茸1, 何海娟1, 张进友2   

  1. 1. 北京协和医院变态反应科;
    2. 河北省沧州地区人民医院
  • 收稿日期:1987-07-04 修回日期:1987-09-30 出版日期:1988-10-15 发布日期:1988-10-15

INVESTIGATION INTO HEREDITARY ANGIOEDEMA IN A FAMILY

ZHANG Hong-Yu1, ZHANG Rong1   

  1. Peking Union Medical College Hospital, Beijing
  • Received:1987-07-04 Revised:1987-09-30 Online:1988-10-15 Published:1988-10-15

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摘要： 本文报告了一个5代均有患病的遗传性血管性水肿家系.共发现10例病人,其中4人数年前已死亡,对尚存活的6例进行一年的随访.我们用RID法进行C1INH及C4测定,C1INH正常值(X±S)为247.3±34.5mg/L,C4正常值为796.4±167.2mg/L.本家系所有具有典型症状的病人其C1INH及C4的水平皆明显低于正常.另有5例C1INH及C4均低,但迄今无症状出现.因先证者发病频繁,且有喉水肿史,故给予丹那哇治疗,疗效显著.

Abstract: A family with hereditary angioedema (HAE) in 5 generation is reported. Ten paitents were found and 4 of them died many years ago. The remaining 6 cases were followed up for 1 year.