Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
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DOI：
10.1186/s12955-017-0586-z
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发表时间：
2017-01-23
影响因子：
3.6
通讯作者：
Antonio Parra, Jose
中科院分区：
文献类型：
--
作者：
Zarrabeitia, Roberto;Farinas-Alvarez, Concepcion;Antonio Parra, Jose
背景：遗传性出血性毛细血管扩张症是一种以鼻出血为主要症状的罕见疾病，其一般生活质量参数、健康状况描述标准以及与一般人群数据的比较研究很少。目的：评估西班牙出血性毛细血管扩张症患者的生活质量，并将其与普通人群进行比较。设计和方法：2005年1月1日至2013年12月31日，187名确诊为出血性毛细血管扩张症的成年患者首次就诊时住进西拉拉纳州医院HHT病房，EUROQOL 5D-3L(五维度三水平)生活质量描述性测验和视觉模拟量表(VAS)。结果：HHT患者在EuroQol 5D-3L的5个维度上存在较大的问题，尤其是疼痛/不适和焦虑/抑郁。在VAS和社会指数值上，HHT患者的得分也低于一般人群，尤其是老年患者、男性患者和HHT2患者。他们的价值观也与慢性病患者的价值观相似。HHT患者对鼻出血严重程度的主观知觉与VAS和社会指数值密切相关。结论：EUROQOL 5D-3L量表对HHT患者的生活质量有全方位的影响。这些评分与其他慢性病患者的评分相似。老年患者、男性和ACVRL1突变携带者在这些量表上的得分通常较低。VAS和社会指数值与主要与鼻出血相关的临床症状的严重程度有很好的相关性。
Background: There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is a cardinal symptom.Purpose: To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population.Design and methods: Between January 1st 2005 and December 31st 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L (five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS). The numerical social index value was also determined and the subjective effect of the nasal epistaxis on their quality of life was estimated classified as mild, moderate or severe.Results: Patients with HHT had greater problems than the general population in the five dimensions of the EuroQol 5D-3L, particularly considering pain/discomfort and anxiety/depression. In the VAS and the social index value, patients with HHT also scored lower than the general population, particularly older patients, males, and patients with HHT2. They also had values similar to those of populations with chronic illnesses. The subjective perception of the severity of epistaxis correlated strongly with the VAS and social index values.Conclusions: The quality of life of patients with HHT, estimated using the EuroQol 5D-3L scale, is affected across all dimensions. The scores are similar to those seen in cases of other chronic diseases. Older patients, males and the carriers of the ACVRL1 mutation generally have worse scores on these scales. The VAS and the social index value are index that correlate well with the severity of the clinical symptoms associated mainly with epistaxis.