The effects of epistaxis on health-related quality of life in patients with hereditary hemorrhagic telangiectasia
The effects of epistaxis on health-related quality of life in patients with hereditary hemorrhagic telangiectasia
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DOI：
10.1002/alr.21374
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发表时间：
2014-11-01
影响因子：
6.4
通讯作者：
Reh, Douglas D.
中科院分区：
文献类型：
--
作者：
Merlo, Christian A.;Yin, Linda X.;Reh, Douglas D.
背景：遗传性出血性毛细血管扩张症（HHT）是一种常染色体显性遗传病，96%以上的患者以鼻出血为主要特征。最近，一个被称为HHT鼻出血严重程度评分（ESS）的有效问卷被开发出来。然而，人们对鼻出血与生活质量之间的关系知之甚少。我们假设鼻出血严重程度是预测HHT患者健康相关生活质量（HR-QoL）的主要因素。方法采用横断面研究。通过网络调查对被试进行ESS问卷和医学结果研究36项短表（SF-36）。通过库拉索标准或基因检测，所有参与者都有明确的HHT诊断。还收集了人口统计信息、遗传学和广泛的历史。描述性分析通过计算连续变量的均值和标准差（SDs）和分类变量的比例进行。然后进行线性回归来评估HR-QoL与ESS之间的关系。结果2008年4月至8月共604名受试者参与。所有患者均报告鼻出血，285例（47.2%）有毛细血管扩张，545例（90.2%）有HHT家族史；167例（27.6%）患者有轻度鼻出血（ESS）
BackgroundHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients.MethodsThis is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curacao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS.ResultsA total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS