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Worldwide Distribution and Extracutaneous Manifestations of Henoch-Schönlein Purpura in Adults: Narrative Review. 成人白癜风的全球分布和皮肤外表现:叙述性综述。
Q3 Medicine Pub Date : 2024-01-25 DOI: 10.2196/49746
Blair W Harris, Luke Maxfield, Abigail Hunter, Mandy Alhajj, Byung Ban, Kayd J Pulsipher

Background: Henoch-Schönlein purpura (HSP), a leukocytoclastic small vessel vasculitis, exhibits both cutaneous and systemic manifestations. While predominantly observed in childhood, it may manifest in adults with more pronounced systemic involvement. Furthermore, HSP is a global phenomenon showcasing epidemiological and systemic variances.

Objective: This study aims to scrutinize extracutaneous manifestations in adults with HSP, discerning distinctions according to geographical regions on a worldwide scale.

Methods: A comprehensive search encompassing PubMed, Embase, Cochrane Library, and Web of Science was executed, covering papers published from January 1, 1970, to December 1, 2019. Keywords used included "Henoch-Schönlein purpura," "henoch schonlein purpura+adult," "IgA vasculitis+adult," "HSP+adult," and "IgAV." A total of 995 publications were identified, from which 42 studies encompassing 4064 patients were selected, with a predominant focus on cases reported in Asia, Europe, and the Americas.

Results: Among adults afflicted with HSP, European patients exhibited a higher propensity for male predominance (P<.001), gastrointestinal involvement (P<.001), and musculoskeletal complications (P<.001). Conversely, patients from the Americas were least likely to experience genitourinary involvement (P<.001).

Conclusions: HSP demonstrates a variance in distribution and extracutaneous manifestations within distinct geographical boundaries. In the adult population, European patients exhibited a higher prevalence of male gender and gastrointestinal and musculoskeletal involvement. Asian patients were more predisposed to genitourinary involvement when compared to their American counterparts. The establishment of prospective studies using standardized reporting measures is imperative to validate the relationships unveiled in this investigation.

背景:白癜风(Henoch-Schönlein purpura,HSP)是一种白细胞破损性小血管炎,有皮肤和全身两种表现。虽然主要见于儿童,但成人也可能出现更明显的全身受累。此外,HSP 是一种全球性现象,显示出流行病学和系统性差异:本研究旨在仔细观察成人 HSP 患者的皮外表现,并在全球范围内根据地理区域进行区分:方法:对PubMed、Embase、Cochrane Library和Web of Science进行全面检索,涵盖1970年1月1日至2019年12月1日发表的论文。使用的关键词包括 "Henoch-Schönlein purpura"、"henoch schonlein purpura+成人"、"IgA 血管炎+成人"、"HSP+成人 "和 "IgAV"。共鉴定了 995 篇出版物,从中选出了 42 项研究,涵盖 4064 名患者,主要集中在亚洲、欧洲和美洲的病例报告中:结果:在患有 HSP 的成人中,欧洲患者以男性为主的倾向较高(PC结论:HSP 的发病率和分布存在差异:HSP在不同地域的分布和皮外表现存在差异。在成年人群中,欧洲患者中男性比例较高,胃肠道和肌肉骨骼受累的比例也较高。与美国患者相比,亚洲患者更易受累于泌尿生殖系统。要验证这项调查所揭示的关系,必须使用标准化的报告措施开展前瞻性研究。
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引用次数: 0
An Unusual Case of Anderson-Fabry Disease: Case Report. 一例罕见的安德森-法布里病:病例报告。
Q3 Medicine Pub Date : 2024-01-16 DOI: 10.2196/49573
Alpana Mohta, Achala Mohta, Pramila Kumari

Angiokeratoma is a group of capillary malformations characterized by the formation of variably sized dark red hyperkeratotic papules. Initially, it was believed that angiokeratoma corporis diffusum was a telltale sign of Anderson-Fabry disease; however, current consensus states that it is also seen in various other lysosomal enzymatic deficiencies. In this report, we present the case of a 12-year-old boy who developed angiokeratoma corporis diffusum with sensorineural deafness, acroparesthesias, and renal involvement.

血管角化瘤是一组毛细血管畸形,其特征是形成大小不一的暗红色角化过度丘疹。起初,人们认为弥漫性血管角化瘤是安德森-法布里病的征兆;但目前的共识是,它也可见于其他各种溶酶体酶缺乏症。在本报告中,我们介绍了一个 12 岁男孩的病例,他患有血管角化瘤,伴有感音神经性耳聋、尖锐湿疣和肾脏受累。
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引用次数: 0
REDCap as a Platform for Cutaneous Disease Management in Street Medicine: Descriptive Study. REDCap 作为街头医疗的皮肤病管理平台:描述性研究。
Q3 Medicine Pub Date : 2024-01-09 DOI: 10.2196/48940
Emily Eachus, Kayla Schwartz, Taha Rasul, Daniel Bergholz, Jonette Keri, Armen Henderson
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引用次数: 0
Reflecting on Decades of Data: The Global Burden of Disease-Cochrane Project. 反思几十年的数据:全球疾病负担--Cochrane 项目。
Q3 Medicine Pub Date : 2024-01-05 DOI: 10.2196/41323
Madeline Adelman, Isaac Weber
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引用次数: 0
Correction: Brachioradial Pruritus Due to Cervical Spine Pathology. 更正:颈椎病变引起的肱动脉瘙痒症。
Q3 Medicine Pub Date : 2023-12-29 DOI: 10.2196/55896
Maria Grabnar, Maneesh Tiwari, Jayesh Vallabh

[This corrects the article DOI: 10.2196/39863.].

[此处更正了文章 DOI:10.2196/39863]。
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引用次数: 0
Crowdsourcing Skin Demarcations of Chronic Graft-Versus-Host Disease in Patient Photographs: Training Versus Performance Study. 众包患者照片中慢性移植物抗宿主病的皮肤分界:培训与性能研究
Q3 Medicine Pub Date : 2023-12-26 DOI: 10.2196/48589
Andrew J McNeil, Kelsey Parks, Xiaoqi Liu, Bohan Jiang, Joseph Coco, Kira McCool, Daniel Fabbri, Erik P Duhaime, Benoit M Dawant, Eric R Tkaczyk

Background: Chronic graft-versus-host disease (cGVHD) is a significant cause of long-term morbidity and mortality in patients after allogeneic hematopoietic cell transplantation. Skin is the most commonly affected organ, and visual assessment of cGVHD can have low reliability. Crowdsourcing data from nonexpert participants has been used for numerous medical applications, including image labeling and segmentation tasks.

Objective: This study aimed to assess the ability of crowds of nonexpert raters-individuals without any prior training for identifying or marking cGHVD-to demarcate photos of cGVHD-affected skin. We also studied the effect of training and feedback on crowd performance.

Methods: Using a Canfield Vectra H1 3D camera, 360 photographs of the skin of 36 patients with cGVHD were taken. Ground truth demarcations were provided in 3D by a trained expert and reviewed by a board-certified dermatologist. In total, 3000 2D images (projections from various angles) were created for crowd demarcation through the DiagnosUs mobile app. Raters were split into high and low feedback groups. The performances of 4 different crowds of nonexperts were analyzed, including 17 raters per image for the low and high feedback groups, 32-35 raters per image for the low feedback group, and the top 5 performers for each image from the low feedback group.

Results: Across 8 demarcation competitions, 130 raters were recruited to the high feedback group and 161 to the low feedback group. This resulted in a total of 54,887 individual demarcations from the high feedback group and 78,967 from the low feedback group. The nonexpert crowds achieved good overall performance for segmenting cGVHD-affected skin with minimal training, achieving a median surface area error of less than 12% of skin pixels for all crowds in both the high and low feedback groups. The low feedback crowds performed slightly poorer than the high feedback crowd, even when a larger crowd was used. Tracking the 5 most reliable raters from the low feedback group for each image recovered a performance similar to that of the high feedback crowd. Higher variability between raters for a given image was not found to correlate with lower performance of the crowd consensus demarcation and cannot therefore be used as a measure of reliability. No significant learning was observed during the task as more photos and feedback were seen.

Conclusions: Crowds of nonexpert raters can demarcate cGVHD images with good overall performance. Tracking the top 5 most reliable raters provided optimal results, obtaining the best performance with the lowest number of expert demarcations required for adequate training. However, the agreement amongst individual nonexperts does not help predict whether the crowd has provided an accurate result. Future work should explore the performance of crowdsourcing in standard clinical p

背景:慢性移植物抗宿主疾病(cGVHD)是异基因造血细胞移植患者长期发病和死亡的重要原因。皮肤是最常受影响的器官,而对 cGVHD 的视觉评估可靠性较低。来自非专业参与者的众包数据已被用于许多医疗应用,包括图像标记和分割任务:本研究旨在评估非专业评定者人群(事先未接受过任何识别或标记 cGHVD 培训的人)对受 cGVHD 影响的皮肤照片进行分界的能力。我们还研究了培训和反馈对人群表现的影响:使用 Canfield Vectra H1 3D 相机拍摄了 36 名 cGVHD 患者的 360 张皮肤照片。由一名经过培训的专家提供三维真实分界,并由一名经过认证的皮肤科医生进行审核。通过 DiagnosUs 移动应用程序共创建了 3000 张 2D 图像(不同角度的投影),用于人群分界。评分者被分为高反馈组和低反馈组。对 4 个不同的非专业人群的表现进行了分析,包括低反馈组和高反馈组每张图像 17 名评分者,低反馈组每张图像 32-35 名评分者,以及低反馈组每张图像前 5 名评分者:在 8 次分界比赛中,高反馈组招募了 130 名评分员,低反馈组招募了 161 名评分员。结果,高反馈组共进行了 54,887 次单独分界,低反馈组共进行了 78,967 次单独分界。非专家人群在分割受 cGVHD 影响的皮肤方面取得了良好的整体性能,只需少量训练,高反馈组和低反馈组所有人群的皮肤像素表面积误差中位数均小于 12%。低反馈人群的表现略逊于高反馈人群,即使使用了更大的人群也是如此。对每张图像跟踪低反馈组中最可靠的 5 个评分者,其结果与高反馈组的结果相似。对于特定图像,评分者之间较高的变异性与较低的人群共识分界性能之间没有关联,因此不能用作可靠性的衡量标准。随着照片和反馈的增多,在任务过程中没有观察到明显的学习现象:结论:由非专业人员组成的群众评定员可以对 cGVHD 图像进行分界,且整体表现良好。跟踪前 5 位最可靠的评定者可获得最佳结果,在充分训练所需的最低专家分界数量下获得最佳性能。不过,非专家个人之间的一致意见无助于预测人群是否提供了准确的结果。未来的工作应探索众包在标准临床照片中的表现,并进一步探索估算共识分界可靠性的方法。
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引用次数: 0
From the Cochrane Library: Interventions for Pemphigus Vulgaris and Pemphigus Foliaceus. 来自Cochrane图书馆:寻常性天疱疮和对叶天疱疮的干预措施(预印本)
Q3 Medicine Pub Date : 2023-12-15 DOI: 10.2196/46812
Ramiro Rodriguez, Torunn E Sivesind, Dedee Murrell, Robert P Dellavalle
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引用次数: 0
The Accuracy and Appropriateness of ChatGPT Responses on Nonmelanoma Skin Cancer Information Using Zero-Shot Chain of Thought Prompting. 使用 "零镜头思维链 "提示对非黑色素瘤皮肤癌信息的聊天 GPT 反应的准确性和适当性。
Q3 Medicine Pub Date : 2023-12-14 DOI: 10.2196/49889
Ross O'Hagan, Dina Poplausky, Jade N Young, Nicholas Gulati, Melissa Levoska, Benjamin Ungar, Jonathan Ungar
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引用次数: 0
A Mobile Health App for Facilitating Disease Management in Children With Atopic Dermatitis: Feasibility and Impact Study. 促进特应性皮炎患儿疾病管理的移动健康应用程序:可行性和影响研究
Q3 Medicine Pub Date : 2023-12-13 DOI: 10.2196/49278
Alex Zvulunov, Stepan Lenevich, Natalia Migacheva

Background: Inadequate control of atopic dermatitis (AD) increases the frequency of exacerbations and reduces the quality of life. Mobile health apps provide information and communication technology and may increase treatment adherence and facilitate disease management at home. The mobile health app, Atopic App, designed for patients and their caregivers, and the associated web-based patient education program, Atopic School, provide an opportunity for improving patients' and caregivers' engagement and adherence to the management of AD.

Objective: This noninterventional, observational study aimed to explore the feasibility and potential impact on the management of AD in children by caregivers using the Atopic App mobile health app.

Methods: The patient-oriented eczema measure (POEM) and numerical rating scale for the grading of pruritus were used as severity scores (scale range: 0-28). The artificial intelligence model of the app was used to assess the severity of AD based on the eczema area and severity index approach. The deidentified data enabled the analysis of the severity of AD, treatment plan history, potential triggers of flare-ups, usage of available features of the app, and the impact of patient education.

Results: During a 12-month period, of the 1223 users who installed the app, 910 (74.4%) registered users were caregivers of children with AD. The web-based Atopic School course was accessed by 266 (29.2%) caregivers of children with AD, 134 (50.4%) of whom completed the course. Usage of the app was significantly more frequent among those who completed the Atopic School program than among those who did not access or did not complete the course (P<.001). Users who completed a second POEM 21 to 27 days apart exhibited a significant improvement of AD severity based on the POEM score (P<.001), with an average improvement of 3.86 (SD 6.85) points. The artificial intelligence severity score and itching score were highly correlated with the POEM score (r=0.35 and r=0.52, respectively).

Conclusions: The Atopic App provides valuable real-world data on the epidemiology, severity dynamics, treatment patterns, and exacerbation-trigger correlations in patients with AD. The significant reduction in the POEM score among users of the Atopic App indicates a potential impact of this tool on health care engagement by caregivers of children with AD.

背景:特应性皮炎(AD)控制不力会增加病情恶化的频率并降低生活质量。移动医疗应用程序提供了信息和通信技术,可提高治疗依从性并促进在家进行疾病管理。专为患者及其护理人员设计的移动健康应用 Atopic App 和相关的网络患者教育项目 Atopic School 为提高患者和护理人员对 AD 治疗的参与度和依从性提供了机会:这项非干预性观察研究旨在探讨护理人员使用 Atopic App 移动医疗应用程序管理儿童特应性湿疹的可行性和潜在影响:方法:使用以患者为导向的湿疹测量法(POEM)和瘙痒分级数字评分表作为严重程度评分(评分范围:0-28)。该应用程序的人工智能模型根据湿疹面积和严重程度指数法评估 AD 的严重程度。去身份化数据可用于分析 AD 的严重程度、治疗方案历史、潜在的复发诱因、应用程序可用功能的使用情况以及患者教育的影响:在为期 12 个月的时间里,在 1223 名安装了该应用程序的用户中,有 910 名(74.4%)注册用户是 AD 儿童的护理者。有 266 名(29.2%)注意力缺失症儿童的看护者访问了特应性学校的网络课程,其中 134 名(50.4%)完成了课程。完成特应性学校课程的用户使用该应用程序的频率明显高于未访问或未完成课程的用户(PConclusions:特应性应用程序提供了有关注意力缺失症患者的流行病学、严重程度动态、治疗模式以及恶化与触发相关性的宝贵真实数据。特应性应用程序用户的 POEM 分数明显降低,这表明该工具对 AD 儿童护理者参与医疗保健具有潜在的影响。
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引用次数: 0
The Effect of Remote Digital Services on Health Care Inequalities Among People Under Long-Term Dermatology Follow-Up: Cross-Sectional Questionnaire Study 远程数字服务对长期皮肤科随访人群中医疗不平等现象的影响:横断面问卷调查研究
Q3 Medicine Pub Date : 2023-12-08 DOI: 10.2196/48981
S. Ramjee, Hanen Mohamedthani, Aditya Umeshkumar Patel, Rebeca Goiriz, Catherine A Harwood, Richard H Osborne, Christina Cheng, Z. Hasan
Given the expansion of remote digital dermatology services from the National Health Service, particularly during the COVID-19 pandemic, there is a need for methods that identify patients at risk of digital exclusion to guide equitable representation in service co-design processes and tailor remote services to the needs of their patient population. This quality improvement project aims to inform the redesign of remote services to optimally support the ongoing needs of patients with chronic skin diseases, ensuring that the services are tailored to patients’ digital health literacy requirements. We profiled the digital health literacy of 123 people with chronic skin conditions who require long-term surveillance in 2 specialist clinics (London, United Kingdom) using the Multidimensional Readiness and Enablement Index for Health Technology (READHY) questionnaire alongside the Optimizing Health Literacy and Access (Ophelia) process for hierarchical cluster analysis. The cluster analysis of READHY dimensions in responding participants (n=116) revealed 7 groups with distinct digital and health literacy characteristics. High READHY scores in groups 1 (n=22, 19%) and 2 (n=20, 17.2%) represent those who are confident with managing their health and using technology, whereas the lower-scoring groups, 6 (n=4, 3.4%) and 7 (n=12, 10.3%), depended on traditional services. Groups 3 (n=27, 23.3%), 4 (n=23, 19.8%), and 5 (n=8, 6.9%) had varying digital skills, access, and engagement, highlighting a population that may benefit from a co-designed dermatology service. By identifying patient groups with distinguishable patterns of digital access and health literacy, our method demonstrates that 63.8% (n=74) of people attending specialist clinics in our center require support in order to optimize remote follow-up or need an alternative approach. Future efforts should streamline the READHY question profile to improve its practicality and use focus groups to elicit strategies for engaging patients with digital services.
鉴于国家卫生服务体系扩大了远程数字皮肤科服务,特别是在2019冠状病毒病大流行期间,需要制定方法来识别面临数字排斥风险的患者,以指导服务协同设计过程中的公平代表性,并根据患者群体的需求定制远程服务。这一质量改进项目旨在为重新设计远程服务提供信息,以最佳方式支持慢性皮肤病患者的持续需求,确保这些服务符合患者的数字健康素养要求。我们使用健康技术多维准备和实现指数(READHY)问卷以及优化健康素养和获取(Ophelia)过程进行分层聚类分析,对2家专科诊所(英国伦敦)需要长期监测的123名慢性皮肤病患者的数字健康素养进行了分析。对应答参与者(n=116)的READHY维度进行聚类分析,发现7组具有明显的数字和健康素养特征。READHY得分较高的第1组(n=22, 19%)和第2组(n=20, 17.2%)代表那些对管理自己的健康和使用技术有信心的人,而得分较低的第6组(n=4, 3.4%)和第7组(n=12, 10.3%)依赖传统服务。第3组(n= 27,23.3%),第4组(n= 23,19.8%)和第5组(n= 8,6.9%)具有不同的数字技能,访问和参与度,突出了可能从共同设计的皮肤科服务中受益的人群。通过识别具有可区分的数字访问模式和健康素养的患者群体,我们的方法表明,63.8% (n=74)在我们中心的专科诊所就诊的人需要支持以优化远程随访或需要替代方法。未来的工作应该精简READHY问题配置文件,以提高其实用性,并利用焦点小组来引出吸引患者使用数字服务的策略。
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引用次数: 0
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JMIR dermatology
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