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长期预防治疗下血管性水肿患者前驱症状分析及短期预防需求

Analysis of prodromal symptoms and need for short-term prophylaxis in angioedema patients under long-term prophylaxis.

作者信息

Lochbaum Robin, Hoffmann Thomas K, Greve Jens, Hahn Janina

机构信息

Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ulm University Medical Center, Frauensteige 12, Ulm, 89075, Germany.

出版信息

Orphanet J Rare Dis. 2025 Feb 1;20(1):47. doi: 10.1186/s13023-025-03562-1.

Abstract

BACKGROUND

Patients with hereditary angioedema (HAE) experience recurrent, unpredictable episodes of edema. These swellings are often preceded by prodromal symptoms. HAE management includes acute treatment, long-term prophylaxis (LTP), and short-term prophylaxis (STP) before procedures with a risk of swelling. The effects of LTP on prodromal symptoms and the necessity for STP in patients on LTP remain unclear.

METHODS

A questionnaire-based study involving HAE and AAE patients receiving LTP was conducted. Changes in prodromal symptoms and the incidence of procedures with an increased risk of swelling, including surgeries, dental procedures, and endoscopies were assessed.

RESULTS

A total of 26 patients were included in the study. Among them, 18 experienced zero to three attacks since starting LTP. Abdominal attacks constituted 60% of all attacks, followed by swellings of the extremities and head and neck. The most frequently reported trigger factors were stress and mechanical stimuli, followed by infections. 9 patients reported surgical procedures, with 8 using STP. Of these, 4 experienced breakthrough attacks, including one laryngeal attack. 105 dental procedures were reported, with STP used for only one. Only one angioedema attack occurred after an intervention without STP. For endoscopies, 7 procedures were reported, 3 of which were performed under STP. Two abdominal attacks were reported by the same patient, both without prior STP. Prodromal symptoms remained consistent in type but varied in intensity and frequency under LTP.

CONCLUSIONS

For dental procedures, the mandatory use of STP in HAE patients on effective LTP should be reconsidered, provided acute treatment is available and other trigger factors are absent.

摘要

背景

遗传性血管性水肿(HAE)患者会经历反复发作且不可预测的水肿发作。这些肿胀通常先有前驱症状。HAE的管理包括急性治疗、长期预防(LTP)以及在有肿胀风险的手术前进行短期预防(STP)。LTP对前驱症状的影响以及LTP患者进行STP的必要性尚不清楚。

方法

对接受LTP的HAE和获得性血管性水肿(AAE)患者进行了一项基于问卷的研究。评估前驱症状的变化以及包括手术、牙科手术和内镜检查在内的肿胀风险增加的手术发生率。

结果

共有26名患者纳入研究。其中,18名患者自开始LTP以来发作零至三次。腹部发作占所有发作的60%,其次是四肢、头颈部肿胀。最常报告的触发因素是压力和机械刺激,其次是感染。9名患者报告了外科手术,其中8名使用了STP。其中,4名经历了突破性发作,包括1次喉部发作。报告了105次牙科手术,仅1次使用了STP。在未进行STP的干预后仅发生1次血管性水肿发作。对于内镜检查,报告了7次手术,其中3次在STP下进行。同一名患者报告了2次腹部发作,均未事先进行STP。在LTP下,前驱症状类型保持一致,但强度和频率有所不同。

结论

对于牙科手术,在有有效LTP的HAE患者中,如果有急性治疗且不存在其他触发因素,应重新考虑强制使用STP。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2f9/11786469/72ad2600ef33/13023_2025_3562_Fig1_HTML.jpg

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