Development and performance of a hereditary hemorrhagic telangiectasia-specific quality-of-life instrument.
Development and performance of a hereditary hemorrhagic telangiectasia-specific quality-of-life instrument.
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DOI：
10.1182/bloodadvances.2022007748
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发表时间：
2022-07-26
期刊：
影响因子：
7.5
通讯作者：
McCrae, Keith R.
中科院分区：
文献类型：
--
作者：
Kasthuri, Raj S.;Chaturvedi, Shruti;Thomas, Sonia;Vandergrift, Nathan;Bann, Carla;Schaefer, Nicole;Clancy, Marianne S.;Pyeritz, Reed;McCrae, Keith R.
HHT特定QoL工具简短、易于管理且可靠。HHT-QoL与患者报告的HHT严重程度和PROMIS QoL相关性良好。这将是未来临床研究的重要组成部分。遗传性出血性毛细血管扩张症（HHT）以动静脉畸形和毛细血管扩张为特征，主要临床表现为鼻出血和消化道出血以及由此引起的贫血。HHT对健康相关生活质量（HR-QoL）产生负面影响;然而，现有的HR-QoL测量工具并不具有HHT特异性。我们的目标是开发一种HHT特异性HR-QoL（HHT-QoL）工具，并在HHT患者的横断面调查中评估其性能。开发了四个HHT特定问题，以评估HHT对生产力以及社会和个人互动的影响。通过Cure HHT进行了一项匿名电子邮件调查。参与者还指出了他们感知的HHT严重程度，并完成了3份患者报告结局测量信息系统（PROMIS）问卷：自由裁量的社会活动，社会角色和情绪困扰。完整的数据可用于290名参与者，他们自认为他们的HHT严重程度为轻度（29%），中度（46%）或重度（25%）。HHT-QoL量表具有可靠性（Cronbach's-α，0.83）。主成分分析表明，该仪器是一维的。参与者的QoL水平较低，他们能够参与自主社交活动（PROMIS平均值为36.4 [标准差14.3]）和履行社会角色（41.5 [17.2]），并且存在高水平的情绪困扰（64.8 [24.2]）。HHT-QoL评分与PROMIS自主社交活动（r =-0.65）和社会角色（r =-0.68）呈负相关，与PROMIS情绪困扰（r = 0.51）呈正相关。总之，4项HHT-QoL工具提供了有价值的见解，并可能是一个有用的除了未来的临床研究HHT。
The HHT-specific QoL instrument is short, easy to administer, and reliable. The HHT-QoL correlates well with patient-reported HHT severity and PROMIS QoL. It will be a valuable component of future clinical research. Hereditary hemorrhagic telangiectasia (HHT) is characterized by arteriovenous malformations and telangiectasia, with primary clinical manifestations of epistaxis and gastrointestinal bleeding and resultant anemia. HHT negatively affects health-related quality of life (HR-QoL); however, existing tools to measure HR-QoL are not HHT specific. Our objective was to develop an HHT-specific HR-QoL (HHT-QoL) instrument and evaluate its performance in a cross-sectional survey of individuals with HHT. Four HHT-specific questions were developed to evaluate the impact of HHT on productivity and social and personal interactions. An anonymous e-mail survey was conducted through Cure HHT. Participants also indicated their perceived HHT severity and completed 3 Patient-Reported Outcomes Measurement Information System (PROMIS) questionnaires: Discretionary Social Activities, Social Roles, and Emotional Distress. Complete data were available for 290 participants who self-identified their HHT severity as mild (29%), moderate (46%), or severe (25%). The HHT-QoL scale was reliable (Cronbach’s-α, 0.83). Principal components analysis indicated the instrument was unidimensional. Participants had low levels of QoL with their ability to participate in discretionary social activities (PROMIS mean 36.4 [standard deviation 14.3]) and perform in social roles (41.5 [17.2]), and the presence of a high level of emotional distress (64.8 [24.2]). The HHT-QoL score correlated negatively with PROMIS Discretionary Social Activities (r = −0.65) and Social Roles (r = −0.68) and positively correlated with PROMIS Emotional Distress (r = 0.51). In conclusion, the 4-item HHT-QoL instrument provides valuable insight and may be a useful addition to future clinical research in HHT.
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影响因子：
7.5
作者：
Chaturvedi, Shruti;Clancy, Marianne;McCrae, Keith R.
通讯作者：
McCrae, Keith R.
影响因子：
6.4
作者：
Merlo, Christian A.;Yin, Linda X.;Reh, Douglas D.
通讯作者：
Reh, Douglas D.
影响因子：
2.6
作者：
Hoag, Jeffrey B.;Terry, Peter;Merlo, Christian A.
通讯作者：
Merlo, Christian A.
DOI：
10.1177/194589240501900112
发表时间：
2005-01-01
期刊：
AMERICAN JOURNAL OF RHINOLOGY
影响因子：
--
作者：
Lennox, PA;Hitchings, AE;Howard, DJ
通讯作者：
Howard, DJ
影响因子：
4.3
作者：
Bevans, Margaret;Ross, Alyson;Cella, David
通讯作者：
Cella, David