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[血管性水肿患者治疗中的年龄特异性方面]

[Age-specific aspects in the treatment of angioedema patients].

作者信息

Staubach P

机构信息

Spezialsprechstunde Angioödeme, Hautklinik und Poliklinik, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.

出版信息

Hautarzt. 2019 Feb;70(2):101-106. doi: 10.1007/s00105-018-4346-8.

Abstract

Manifestation of angioedema can occur at any age. We distinguish between two main subtypes: mast cell mediator-associated angioedema (often with hives) and the non-mast cell mediator-associated angioedema. The patient's history is very important due to the fact that one subtype can be hereditary, but we also have to consider new mutations and even not yet diagnosed patients. Acquired non-mast cell mediator-associated angioedema is rare in children, but very common in adults due to the intake of angiotensin-converting enzyme inhibitors. From a detailed anamneses, classification is very often possible. But especially in childhood, symptoms are often projected onto the gastrointestinal tract, which have to be seen differently. The burden of disease defined as disease activity and quality of life may change in the course of life, but is not always predictable. Therapies are available for all angioedema subtypes in any age. If treatment is necessary, the attacks should be treated early to positively influence duration and severity of the attacks. The management of the patients includes besides information, education of the patient and family members regarding therapy options and prevention of triggers and the repetition of education, especially in case of any change of life circumstances.

摘要

血管性水肿可在任何年龄出现。我们区分两种主要亚型:肥大细胞介质相关的血管性水肿(常伴有荨麻疹)和非肥大细胞介质相关的血管性水肿。患者的病史非常重要,因为一种亚型可能是遗传性的,但我们也必须考虑新的突变,甚至是尚未确诊的患者。获得性非肥大细胞介质相关的血管性水肿在儿童中罕见,但在成人中由于服用血管紧张素转换酶抑制剂而非常常见。通过详细的问诊,通常可以进行分类。但特别是在儿童期,症状常表现在胃肠道,对此必须有不同的认识。定义为疾病活动度和生活质量的疾病负担在人生过程中可能会改变,但并非总是可预测的。任何年龄的所有血管性水肿亚型都有相应治疗方法。如果需要治疗,应尽早治疗发作,以对发作的持续时间和严重程度产生积极影响。对患者的管理除了提供信息外,还包括对患者及其家庭成员进行有关治疗选择、预防触发因素的教育,以及重复教育,特别是在生活环境发生任何变化的情况下。

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