Jindal Nina Lakhani, Harniman Elaine, Prior Nieves, Perez-Fernandez Elia, Caballero Teresa, Betschel Stephen
Division of Clinical Immunology and Allergy, St. Michael's Hospital, Bond Street, 4 CC Specialty Clinics, Toronto, ON M5B 1W8 Canada.
Musculoskeletal Health and Outcomes Research, Li Ka Shing Knowledge Institute, St Michael's Hospital, Toronto, ON Canada.
Allergy Asthma Clin Immunol. 2017 Jan 19;13:4. doi: 10.1186/s13223-016-0176-3. eCollection 2017.
Hereditary angioedema (HAE) is a rare but serious condition characterized by recurrent spontaneous attacks of angioedema affecting superficial tissues of upper respiratory and gastrointestinal tracts. The potentially fatal and disfiguring nature of HAE impacts the health-related quality of life (HRQoL) of patients with this condition.
To assess the health-related quality of life of Canadian patients with HAE using the 36-item Short-Form Health Survey (SF-36v2).
Twenty-one patients living in Canada over age 18 with known diagnosis of hereditary angioedema due to C1-INH deficiency (HAE), completed the SF-36v2 (generic HRQoL questionnaire). Results were compared to Canadian normative data by converting the SF-36 scores into z scores.
The SF-36v2 showed a significant reduction in general health (p = 0.0063) in patients with HAE when compared with healthy Canadians. Percentage of patients with z scores below 0.8 (large effect) was 47.6% for general health subscale, 33.3% for bodily pain and vitality subscales and 28.6% for physical component scores. Mean scores of eight dimensions ranged from 57.7 to 88.9. Mean Physical and mental component scores were 49.1 and 50.4. Internal consistency of evaluation was demonstrated by Cronbach's alpha value above 0.7 for all scales. General perception of health was significantly different in these patients, compared to Canadian normative data.
This study of Canadian patients with HAE shows that General Health is most frequently affected followed by Bodily Pain and Vitality, as measured by SF-36v2. The SF-36v2 offers valuable insight to assess quality of life in patients with HAE, however a larger number of Canadian patients and specific tools for assessment are needed for better evaluation.
遗传性血管性水肿（HAE）是一种罕见但严重的疾病，其特征为血管性水肿反复发作，累及上呼吸道和胃肠道的浅表组织。HAE潜在的致命性和毁容性影响了该病患者的健康相关生活质量（HRQoL）。
使用36项简明健康调查问卷（SF-36v2）评估加拿大HAE患者的健康相关生活质量。
21名居住在加拿大、年龄超过18岁、已知因C1-INH缺乏而诊断为遗传性血管性水肿（HAE）的患者完成了SF-36v2（通用HRQoL问卷）。通过将SF-36得分转换为z得分，将结果与加拿大的标准数据进行比较。
与健康的加拿大人相比，SF-36v2显示HAE患者的总体健康状况显著下降（p = 0.0063）。z得分低于0.8（影响较大）的患者在总体健康子量表中占47.6%，在身体疼痛和活力子量表中占33.3%，在身体成分得分中占28.6%。八个维度的平均得分在57.7至88.9之间。身体和精神成分的平均得分分别为49.1和50.4。所有量表的Cronbach's α值均高于0.7，证明了评估的内部一致性。与加拿大的标准数据相比，这些患者对健康的总体认知存在显著差异。
这项对加拿大HAE患者的研究表明，按照SF-36v2的测量结果，总体健康最常受到影响，其次是身体疼痛和活力。SF-36v2为评估HAE患者的生活质量提供了有价值的见解，然而，需要更多的加拿大患者和特定的评估工具来进行更好的评估。
