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- W2324281311 abstract "血管浮腫は,即時型過敏反応,血清病のような全身性免疫反応,そして血清中の補体第1成分阻害因子(C1 inhibitor; C1-INH)の活性低下などにより引き起こされる.われわれは,頻回に腹痛を訴え,その腹痛発作時に補体値の低下を認めた15歳の女性の症例を経験した.低補体値より,血管浮腫を疑いC1-INHを検索したところ, C1-INHの活性とC1-INH蛋白の低下が認められた.家族内の検索において,他に同様の臨床症状および低補体値, C1-INH活性の低下を示す者は認められなかった.しかし, C1qが正常範囲でありリンパ球増殖性疾患やC1-INHに対する自己抗体が認められないことから突然変異による遺伝性血管神経性浮腫(hereditary angioneurotic edema; HANE)と診断した.また,本症例はシアログラフィー,口唇唾液腺の組織学的検索よりSjogren症候群と診断されており, Sjogren症候群に合併したHANEの1例と考えられる." @default.
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- W2324281311 date "1992-01-01" @default.
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- W2324281311 title "Hereditary angioneurotic edema associated with Sjoegren's syndrome." @default.
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- W2324281311 doi "https://doi.org/10.2177/jsci.15.81" @default.
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