测量神经纤维瘤病 1 型皮肤神经纤维瘤对生活质量的影响。
Measuring the Effect of Cutaneous Neurofibromas on Quality of Life in Neurofibromatosis Type 1.
机构信息
From the Department of Dermatology (S.M., M.D.), Biostatistics Core, Masonic Cancer Center (N.R.), and Department of Pediatrics (C.M.), University of Minnesota, Minneapolis; Department of Clinical Genetics (Y.B., C.W.), Royal North Shore Hospital; Faculty of Medicine (Y.B.), University of Sydney, Australia; and Department of Neurology and Cancer Center (S.R.P.), Massachusetts General Hospital, Boston.
出版信息
Neurology. 2021 Aug 17;97(7 Suppl 1):S25-S31. doi: 10.1212/WNL.0000000000012427. Epub 2021 Jul 6.
OBJECTIVE
In order to explore the use of Skindex scoring in patients with neurofibromatosis type 1 (NF1) across multiple clinical sites and inform design of additional quality of life measures, we analyzed correlations between Skindex, site, and clinical measures for 79 patients with NF1 from specialized clinics in Sydney, Australia (Royal North Shore Hospital [RNS]) and Minneapolis, Minnesota (University of Minnesota [UMN]).
METHODS
The relationship between clinical factors and Skindex scores were explored by clinic site and overall.
RESULTS
A total of 40 participants were recruited from RNS and 39 from UMN. Female sex, total number of cutaneous neurofibroma (cNF), and whether cNF were present on the face correlated highly with Skindex and not Riccardi scores. The UMN site had lower average scores, but these differences were almost entirely removed after adjusting for age, sex, facial cNF, and total cNF number.
CONCLUSIONS
The development of cNF in adolescence and adulthood in NF1 often leads to progressive disfigurement and discomfort and is among one of the most common reasons for patients to seek medical treatment. Skindex has been used to assess skin-related quality of life in NF1 previously but is not specific to NF1. These findings highlight the need for a low threshold for referral to dermatologists for all patients with NF1 regardless of the severity of disease. The finding that facial cNF and higher total number of cNF correlates with poorer skin-related quality of life may benefit design of more specific NF1 skin-related quality of life measures.
目的
为了探索 Skindex 评分在 1 型神经纤维瘤病（NF1）患者中的应用，在多个临床场所进行研究，并为其他生活质量测量方法的设计提供信息，我们分析了来自澳大利亚悉尼皇家北岸医院（RNS）和明尼苏达州明尼阿波利斯大学（UMN）专门诊所的 79 例 NF1 患者的 Skindex、临床和临床指标之间的相关性。
方法
通过临床场所和整体来探索临床因素与 Skindex 评分之间的关系。
结果
共招募了来自 RNS 的 40 名参与者和来自 UMN 的 39 名参与者。女性性别、皮肤神经纤维瘤（cNF）总数以及 cNF 是否存在于面部与 Skindex 和 Riccardi 评分高度相关。UMN 场所的平均评分较低，但在调整年龄、性别、面部 cNF 和总 cNF 数量后，这些差异几乎完全消除。
结论
NF1 患者在青春期和成年期 cNF 的生长通常会导致容貌受损和不适，这是患者寻求治疗的最常见原因之一。Skindex 之前曾用于评估 NF1 患者的皮肤相关生活质量，但并不专门针对 NF1。这些发现强调了所有 NF1 患者都应及时转诊给皮肤科医生的必要性，无论疾病严重程度如何。面部 cNF 和更高的 cNF 总数与较差的皮肤相关生活质量相关的发现可能有助于设计更具体的 NF1 皮肤相关生活质量测量方法。
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