Faculdade de Medicina da Universidade de São Paul, Department of Dermatology, São Paulo/SP, Brazil.
Clinics (Sao Paulo). 2013 Jan;68(1):81-3. doi: 10.6061/clinics/2013(01)oa13.
Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the quality of life in Brazilian hereditary angioedema patients.
Patients older than 15 years with any severity of hereditary angioedema and laboratory confirmation of C1 inhibitor deficiency were included. Two questionnaires were used: a clinical questionnaire and the SF-36 (a generic questionnaire). This protocol was approved by the Ethics Committee of Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo.
The SF-36 showed that 90.4% (mean) of all the patients had a score below 70 and 9.6% had scores equal to or higher than 70. The scores of the eight dimensions ranged from 51.03 to 75.95; vitality and social aspects were more affected than other arenas. The internal consistency of the evaluation was demonstrated by a Cronbach's alpha value above 0.7 in seven of the eight domains.
In this study, Brazilian patients demonstrated an impaired quality of life, as measured by the SF-36. The most affected domains were those related to vitality and social characteristics. The generic SF-36 questionnaire was relevant to the evaluation of quality of life; however, there is a need for more specific instruments for better evaluation.
遗传性血管性水肿是一种严重的医学病症，由罕见的常染色体显性遗传疾病引起，与 C1 酯酶抑制剂的产生不足或功能障碍有关。在大多数情况下，受影响的患者会经历意想不到的、反复的皮下、胃肠道和喉头水肿危机。疾病的不可预测性、强度和其他因素会影响遗传性血管性水肿患者的生活质量。我们评估了巴西遗传性血管性水肿患者的生活质量。
纳入年龄大于 15 岁、任何严重程度的遗传性血管性水肿且实验室确认 C1 抑制剂缺乏的患者。使用了两种问卷：临床问卷和 SF-36（一般问卷）。本方案获得了圣保罗大学附属医院临床医院伦理委员会的批准。
SF-36 显示，所有患者中有 90.4%（平均值）的得分低于 70，9.6%的得分等于或高于 70。八个维度的得分范围为 51.03 至 75.95；活力和社会方面比其他领域受影响更大。评估的内部一致性通过八个领域中的七个领域的 Cronbach's alpha 值大于 0.7 来证明。
在这项研究中，巴西患者的生活质量受损，这是通过 SF-36 测量得出的。受影响最大的领域是与活力和社会特征相关的领域。通用的 SF-36 问卷与生活质量评估相关；然而，需要更具体的工具来进行更好的评估。
