神经纤维瘤病1的皮肤表现显着降低与健康相关的生活质量。然而,缺乏现有调查在捕获神经纤维瘤病1皮肤治疗结局方面的效用数据.这项定量研究检查了临床医生评估的严重程度和能见度以及患者评估的瘙痒和生活质量(QoL)之间的关系,以(1)建立与皮肤和条件相关的QoL的基线水平,痒,抑郁和焦虑;(2)确定患者的关注点,以告知皮肤干预措施的发展和评估;(3)比较不同QoL措施的敏感性。验证的量表包括Skindex-29、皮肤病生活质量指数(DLQI)、神经纤维瘤病1-成人生活质量(NF1-AdQOL)问卷,和医院焦虑和抑郁量表(HADS)。我们招募了100名参与者(应答率:95%)。其中,42%报告瘙痒,23%有可能的临床焦虑。与对照组相比,我们的队列具有更高的焦虑水平和总HADS评分。使用多元回归分析,使用Skindex-29,NF1-AdQOL,增加可见性显著预测较差的QoL,和DLQI(p<0.05);在Skindex-29和NF1-AdQOL中,瘙痒显着预测QoL恶化(p<0.05)。Skindex-29和NF1-AdQOL中平均得分最高的问题担心皮肤病恶化和尴尬。DLQI中得分最高的问题是关于痒,疼痛,和尴尬。特别询问皮肤神经纤维瘤(cNF)的项目得分高于可比较的皮肤特异性问题(t检验p值<0.05)。总之,这项研究提供了对导致QoL受损的因素的见解,焦虑,NF1皮肤神经纤维瘤患者的情绪。确定用于cNF测量的关键因素包括可见性,痒,焦虑,尴尬,对皮肤病恶化的恐惧,和cNF特定的问题。
The skin manifestations of
neurofibromatosis 1 significantly reduce health-related quality-of-life. However, data on the utility of existing surveys in capturing
neurofibromatosis 1 skin treatment outcomes are lacking. This quantitative study examined the relationship between clinician-rated severity and visibility and patient-rated itch and quality-of-life (QoL) to (1) establish baseline levels of skin- and condition-specific-related QoL, itch, depression and anxiety; (2) identify patient concerns to inform the development and evaluation of skin interventions; and (3) compare the sensitivity of different QoL measures. Validated scales included Skindex-29, Dermatology Life Quality Index (DLQI),
Neurofibromatosis 1-adult quality-of-life (NF1-AdQOL) questionnaire, and the Hospital Anxiety and Depression Scale (HADS). We recruited 100 participants (response rate: 95%). Of these, 42% reported itch and 23% had probable clinical anxiety. Our cohort had higher levels of anxiety and total HADS scores compared to a control population. Using multivariate regression analysis, increasing visibility significantly predicted poorer QoL using the Skindex-29, NF1-AdQOL, and DLQI (p < 0.05); and itch significantly predicted worse QoL in Skindex-29 and NF1-AdQOL (p < 0.05). The highest mean scoring questions in Skindex-29 and NF1-AdQOL concerned worry about worsening skin disease and embarrassment. The highest mean scoring questions in DLQI were regarding itch, pain, and embarrassment. Items asking specifically about cutaneous neurofibromas (cNF) scored higher than comparable skin-specific questions (t-test p value <0.05). In summary, this study provides insights into the factors contributing to impaired QoL, anxiety, and mood in NF1 patients with cutaneous neurofibromas. Key factors identified for use in cNF measures include visibility, itch, anxiety, embarrassment, fears of worsening skin disease, and cNF-specific questions.