Two cases of hereditary angioedema treated with plasma-derived C1-inhibitor

  • SAWADA Yusuke
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • AOKI Makoto
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • KANEKO Minoru
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • MURATA Masato
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • KANBE Masahiko
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • HAGIWARA Shuichi
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • NAKAMURA Takuro
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital
  • OHYAMA Yoshio
    Emergency and General Medical Center, Gunma University Hospital
  • TAMURA Jun'ichi
    Emergency and General Medical Center, Gunma University Hospital
  • OSHIMA Kiyohiro
    Department of Emergency Medicine, Gunma University Graduate School of Medicine Emergency and General Medical Center, Gunma University Hospital

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Other Title
  • 迅速なC1インヒビター製剤投与が奏功した遺伝性血管性浮腫の2例
  • 症例報告 迅速なC1インヒビター製剤投与が奏功した遺伝性血管性浮腫の2例
  • ショウレイ ホウコク ジンソク ナ C1 インヒビター セイザイ トウヨ ガ ソウコウ シタ イデンセイ ケッカンセイ フシュ ノ 2レイ

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症例1:30代女性。朝から突然の呼吸困難を自覚し当院を受診。歯科治療時に同様の既往があり,家族歴で祖母の窒息死と叔父の全身性浮腫があった。頸部聴診で狭窄音,両足背に浮腫を認め,喉頭内視鏡検査では著明な喉頭浮腫を認めた。遺伝性血管性浮腫(Hereditary angioedema; HAE)を疑い,C1インヒビター(C1-INH)製剤を投与したところ喉頭浮腫は改善した。血液検査でC4濃度及びC1-INH活性が低下しており,HAEと診断した。症例2:20代男性。HAE治療のため他院通院中。腹痛と嘔気,嘔吐が出現し当院を受診。HAE急性発作と考えC1-INH製剤の投与で消化器症状は改善した。HAEの疾患認知度はまだ十分とは言えないが,救急領域では気道緊急や急性腹症を引き起こす疾患として重要である。HAE急性発作に対しては迅速な判断と即座にC1-INH製剤を使用できる体制構築が必要である。

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