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与脑腱黄瘤病共存:患者、护理人员及专家观点

Living with Cerebrotendinous Xanthomatosis: Patient, Caregiver, and Expert Perspectives.

作者信息

Steiner Robert D, DeBarber Andrea, Larson Austin, Blanchard Bobbi, Laurie Shawn, Odedara Neel, Rosengrant Hollisa, Dutta Rana

机构信息

University of Wisconsin School of Medicine and Public Health, Madison, WI, USA.

University Shared Resources, Oregon Health and Science University, Portland, OR, USA.

出版信息

Adv Ther. 2024 Feb;41(2):467-475. doi: 10.1007/s12325-023-02687-8. Epub 2023 Dec 19.

Abstract

In this article, patients with cerebrotendinous xanthomatosis (CTX) and caregivers detail their experience with lifelong symptoms, diagnosis, treatment and efficacy, and ongoing disease management. One patient and four caregivers describe the challenges associated with pursuing a correct diagnosis for years before testing confirmed a CTX diagnosis. They also detail their ongoing struggles and desire for greater access to physicians with CTX knowledge and to reliable online resources to continue their education about the disease and strategies for symptom management. The expert perspective is a direct response by three CTX researchers, including physicians who are treating patients with CTX in the United States and experts whose laboratories provide genetic and biochemical testing for CTX. They respond to many of the patient and caregiver concerns, including steps that are being taken to identify CTX earlier and provide access to confirmatory diagnostic testing sooner, and suggest the best online resources for CTX-related information and access to webinars and support groups. While the expert perspective is a direct response to the patient and caregiver authors' CTX journeys, it should be beneficial to any patient with CTX or their caregivers.

摘要

在本文中,患有脑腱黄瘤病(CTX)的患者及其护理人员详细讲述了他们在终身症状、诊断、治疗及疗效以及持续疾病管理方面的经历。一名患者和四名护理人员描述了在检测确诊为CTX之前多年来寻求正确诊断所面临的挑战。他们还详细说明了他们持续面临的困难,以及希望能更多地接触到了解CTX的医生和可靠的在线资源,以便继续了解该疾病及症状管理策略。专家观点是由三位CTX研究人员直接作出的回应,其中包括在美国治疗CTX患者的医生以及其实验室为CTX提供基因和生化检测的专家。他们回应了许多患者和护理人员关心的问题,包括正在采取哪些措施更早地识别CTX并更快地提供确诊诊断检测,还推荐了获取CTX相关信息以及参加网络研讨会和支持小组的最佳在线资源。虽然专家观点是对患者和护理人员作者CTX经历的直接回应,但它对任何CTX患者或其护理人员都应有所助益。

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