遗传性血管性水肿，呼叫中心对发作的紧急管理。

Hereditary angioedema, emergency management of attacks by a call center.

机构信息

AP-HP, Urgences, Centre de référence sur les angiœdèmes à kinines, Hôpital Louis Mourier, Université Paris 7, 92700 Colombes, France; AP-HP, Urgences - Samu 93, Hôpital Avicenne, Université Paris 13, Inserm U942, 93000 Bobigny, France.

AP-HP, Urgences, Centre de référence sur les angiœdèmes à kinines, Hôpital Louis Mourier, Université Paris 7, 92700 Colombes, France.

出版信息

Eur J Intern Med. 2019 Sep;67:42-46. doi: 10.1016/j.ejim.2019.05.007. Epub 2019 May 18.

DOI:10.1016/j.ejim.2019.05.007

PMID:31109849

Abstract

OBJECTIVE

Hereditary angiœdema (HAE) is a rare autosomal dominant disease characterized by recurrent, unpredictable, potentially life-threatening swelling. Objective is to assess the management of the acute HAE attacks in the real life setting through a call center in France.

METHODS

A pre-specified ancillary study of SOS-HAE, a cluster-randomized prospective multicenter trial, was conducted. HAE patients were recruited from 8 participating reference centers. The outcome of interest was the rate of hospitalization.

RESULTS

onerhundred patients were included. The median (quartile) age was 38 (29-53) years, and 66 (66%) were female. Eighty (80%) patients had HAE type I, 8 (8%) had HAE type II and 12 (12%) patients had FXII-HAE. Fifty-one (51%) patients had experienced at least one time the call center during the follow-up. Nine over 166 (5%) attacks for 9 different patients resulted in hospital admission to the hospital (in the short-stay unit, ie, <24 h) during the follow-up period. During 2 years, there were 166 calls to call center for 166 attacks. All attacks were treated at home after call center contact.

CONCLUSIONS

Use of emergency departments and hospitalizations are reduced by the use of a coordinated national call center in HAE after therapeutic education program that promoted self-administration of specific treatment and use of call to call center.

TRIAL REGISTRATION

clinicalTrials.gov identifier: NCT01679912.

摘要

目的

遗传性血管性水肿（HAE）是一种罕见的常染色体显性遗传疾病，其特征为反复发作、不可预测、可能危及生命的肿胀。目的是通过法国的一个呼叫中心，评估真实环境中急性 HAE 发作的管理情况。

方法

这是 SOS-HAE 研究的一项预先指定的辅助研究，这是一项多中心、前瞻性、集群随机对照试验。从 8 个参与的参考中心招募 HAE 患者。主要观察终点为住院率。

结果

共纳入 100 例患者。中位（四分位数）年龄为 38（29-53）岁，66 例（66%）为女性。80 例（80%）患者为 HAE Ⅰ型，8 例（8%）为 HAE Ⅱ型，12 例（12%）患者为 FXII-HAE。在随访期间，51 例（51%）患者至少有一次拨打过呼叫中心。9 例（5%）不同患者的 166 次发作中有 9 次导致住院（在短期住院病房，即 <24 小时）。在 2 年的随访期间，共接到 166 次呼叫中心的 166 次发作电话。所有发作均在呼叫中心联系后在家中治疗。