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首页> 外文期刊>Allergy >Standard care impact on angioedema because of hereditary C1 inhibitor deficiency: a 21-month prospective study in a cohort of 103 patients.
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Standard care impact on angioedema because of hereditary C1 inhibitor deficiency: a 21-month prospective study in a cohort of 103 patients.

机译：由于遗传性C1抑制剂缺乏，标准治疗对血管性水肿的影响：一项针对103名患者的为期21个月的前瞻性研究。

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BACKGROUND: Hereditary angioedema (HAE) due to the deficiency of C1 inhibitor (C1-INH) causes chronically recurrent cutaneous, abdominal and laryngeal angioedema that are disabling and potentially life-threatening. OBJECTIVE: We designed a prospective study to quantify the residual disease in patients with HAE treated according to the existing consensus documents. METHODS: Data were collected from diaries recording occurrence, duration, location and treatment of acute angioedema attacks. A total of 386 semesters properly completed were analyzed. Forty-seven of 103 patients were on prophylactic treatment, 41 with attenuated androgens and six with tranexamic acid. A total of 1532 angioedema attacks (one every 45.3 days) were registered. RESULTS: Peripheral attacks were the most frequent (698), followed by abdominal (503) and combined locations (232), laryngeal edema was less common (99). Patients on prophylaxis with attenuated androgens had 7.7 attacks/year lasting 1.47 days, those on tranexamic acid had 8.1 attacks/year lasting 1.59 days, and those without prophylaxis had 8.9 attacks/year lasting 1.68. Plasma-derived C1-INH was used by 44 patients to treat a total of 376 acute attacks that resolved faster (1.1 day) than those not treated (1.85 day) or treated with tranexamic acid (1.79 day). No adverse events related to C1-INH infusion were reported. CONCLUSION: Our data demonstrate that tranexamic acid is not effective in the treatment of acute attacks and indicate that under the current therapeutic approach, the HAE related disability is effectively but partially reduced. Incomplete success does not appear to depend on limited efficacy of the drugs but on their limited use that can be overcome by implementing specific treatment strategies.
机译：背景：由于缺乏C1抑制剂（C1-INH）而导致的遗传性血管性水肿（HAE）导致慢性复发性皮肤，腹部和喉部血管性水肿，致使生命危险，甚至可能危及生命。目的：我们根据现有共识文件设计了一项前瞻性研究，以量化治疗的HAE患者的残留疾病。方法：从日记中收集数据，记录急性血管性水肿发作的发生，持续时间，位置和治疗。总共386个学期正确完成了分析。 103例患者中有47例接受了预防性治疗，其中41例使用减毒雄激素，6例使用氨甲环酸。总共记录了1532次血管性水肿发作（每45.3天1次）。结果：周围性发作最频繁（698），其次是腹部（503）和合并部位（232），喉部水肿较少见（99）。预防性减毒雄激素的患者每年发作7.7次，持续1.47天，氨甲环酸治疗的患者每年发作8.1次，持续1.59天，而无预防性的患者每年发作8.9次，持续1.68天。血浆源性C1-INH被44例患者用于治疗共376例急性发作，这些发作的缓解时间（1.1天）比未治疗（1.85天）或氨甲环酸治疗（1.79天）更快。没有报道与C1-INH输注有关的不良事件。结论：我们的数据表明氨甲环酸不能有效治疗急性发作，并表明在目前的治疗方法下，与HAE相关的残疾得到有效缓解，但部分缓解。不完全成功似乎并不取决于药物的有限疗效，而是取决于可以通过实施特定的治疗策略来克服其有限的用途。

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