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遗传性皮肤疾病:多学科诊所的价值。

Genetic skin disorders: The value of a multidisciplinary clinic.

机构信息

University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

Section of Dermatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

出版信息

Am J Med Genet A. 2021 Apr;185(4):1159-1167. doi: 10.1002/ajmg.a.62095. Epub 2021 Jan 27.

Abstract

Genodermatoses are inherited disorders with skin manifestations and can present with multisystem involvement, resulting in challenges in diagnosis and treatment. To address this, the expertise of dermatology and clinical genetics through a multidisciplinary clinic (Genodermatoses Clinic) were combined. A retrospective cohort study of 45 children seen between March 2018 and February 2019 in the Genodermatoses Clinic at The Children's Hospital of Philadelphia was performed. Patient demographics, referral information, genetic testing modality, diagnoses, and patient satisfaction scores were evaluated to assess the clinic's impact. The majority of patients (42.2%) were referred from Dermatology and 86.7% were referred for diagnosis. Two-thirds of the patients were recommended genetic testing, and subsequently 73.3% completed testing. Nearly three-quarters, 26 out of 36 patients (72.2%), of our undiagnosed patients received a clinical and/or molecular diagnosis, which is imperative in managing their care. Twenty-two individuals pursued genetic testing. In eight individuals (36%), molecular testing was diagnostic. However, in two individuals the molecular diagnosis did not completely explain the phenotype. However, there are still obstacles to genetic testing, such as cost of testing and insurance barriers. Almost all (91.4%) rated the Genodermatoses Clinic as "Very Good," the top Press Ganey score. High patient satisfaction scores suggest a positive impact of the Genodermatoses clinic, emphasizing the importance to increase support for the clinical and administrative time needed for patients with genodermatoses.

摘要

遗传性皮肤病是具有皮肤表现的遗传性疾病,可出现多系统受累,导致诊断和治疗困难。为了解决这个问题,通过多学科诊所(遗传性皮肤病诊所)将皮肤科和临床遗传学的专业知识结合起来。对 2018 年 3 月至 2019 年 2 月期间在费城儿童医院遗传性皮肤病诊所就诊的 45 名儿童进行了回顾性队列研究。评估了患者人口统计学资料、转诊信息、基因检测方式、诊断和患者满意度评分,以评估该诊所的影响。大多数患者(42.2%)由皮肤科转诊,86.7%是为了诊断而转诊。三分之二的患者建议进行基因检测,随后 73.3%的患者完成了检测。将近四分之三(26/36)未确诊的患者获得了临床和/或分子诊断,这对他们的治疗至关重要。22 名患者进行了基因检测。在 8 名患者(36%)中,分子检测具有诊断意义。然而,在 2 名患者中,分子诊断并未完全解释表型。然而,基因检测仍然存在障碍,例如检测费用和保险障碍。几乎所有人(91.4%)都对遗传性皮肤病诊所的评价为“非常好”,这是最高的盖恩斯调查分数。高的患者满意度评分表明遗传性皮肤病诊所具有积极的影响,强调需要增加对遗传性皮肤病患者所需的临床和行政时间的支持。

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本文引用的文献

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Practice and Educational Gaps in Genodermatoses.遗传性皮肤病的实践与教育差距
Dermatol Clin. 2016 Jul;34(3):303-10. doi: 10.1016/j.det.2016.02.007.

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