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【简答题】
11 岁,因手足水肿入院,病史由其母亲。 患者于 5 年前无明显诱因出现手、脚和脸的肿胀,伴声音变粗、呼吸困难,偶尔有腹痛。该症状反复发作 6-7 次 / 年。每次发作会持续 2-3 天。入院前 4 天,患者无明显诱因再次出现上述症状,自行用药后病情无缓解。无发热,皮肤无瘙痒、发红或 湿疹 。无传染病接触食物、药物过敏史。家庭病史显示母亲自孩童时期起也有相似的发作史,患者哥哥也有相似的发作史,并在 8 岁一次相似的发作时死于呼吸窘迫。其父亲体健。 体格检查: T37°C , P110 次 / 分, R28 次 / 分, BP100/60mmHg ,发育正常,营养良好,神清,精神可。皮肤黏膜无黄疸、发紺或苍白。浅表淋巴结未触及肿大。患者眼睑、嘴唇和手背有轻度的非凹陷性水肿,伴轻度的声音加重。间接的喉镜检查显示喉部水肿,累及杓状会厌襞,真假声带。双肺未见异常。腹软肝脾未及。 实验室检查:血细胞计数、尿液分析、肝肾功能正常。血浆 C4 0.68 mmol/l (正常值 0.7-2.43 mmol/l )。 C1 酯酶抑制因子为 29% (正常值 70%-130% )。临床诊断为:遗传性血管神经质水肿。给病人以口服一日两次 1mg 治疗六,目前的病情发作得到缓解。当病情缓解情况持续时,剂量减为一日一次 1mg ,为期 6 个月。治疗期间定期检测其肝功,未见异常。询问父母该疾病的本质,虽然母亲有非常少的很轻的发作,但不需要任何积极的干预,她的血清 C4 水平减至 0.06 g /L , C1 酯酶抑制因子减至 29% 。 该患者为反复出现水肿及呼吸困难,该病要如何治疗?
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