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[基层医疗中罕见病的遗传诊疗方案:以沃夫勒姆综合征为例]

[Genetic protocol in primary care for rare diseases: Wolfram syndrome as a prototype].

作者信息

Esteban-Bueno Gema, Díaz-Anadón Lucas Ramón, Rodríguez González Antonio, Navarro Cabrero Miguel, Berenguel Hernández Aída María

机构信息

Medicina familiar y comunitaria, Unidad de Gestión clínica Almería Periferia, Distrito Sanitario Almería, Servicio Andaluz de Salud, Asociación Española para la Investigación y Ayuda al Síndrome de Wolfram, Miembro del Grupo de Trabajo SEMFYC sobre Genética Clínica y Enfermedades Raras, SAMFYC Genética Clínica y Enfermedades Raras, Almería, España.

Pediatría y sus Áreas Específicas, Hospital Público Carmen y Severo Ochoa, Cangas del Narcea, Asturias.

出版信息

Aten Primaria. 2022 May;54(5):102285. doi: 10.1016/j.aprim.2022.102285. Epub 2022 Mar 16.

Abstract

Rare diseases, despite their individual low frequency, affect 7% of the population all combined. Consequently, every primary care practitioner (PCP) will have several of these patients under his care. 80% of rare diseases are genetically determined, which makes genetic counseling fundamental in these cases. The follow-up of patients with Wolfram syndrome (WS) can be used to design a protocol to support these patients, with the participation of researchers and healthcare professionals specialized in WS, the patients themselves and their familial environment. This protocol can be suitable for the diagnosis and management of other diseases as well. The main steps of every genetic clinical procedure are developed in this article, emphasizing the role of PCP in supporting patients and their families and in transmitting genetic information in a comprehensible manner.

摘要

罕见病尽管各自发病率低,但合计影响7%的人口。因此,每位初级保健医生都会照料数位此类患者。80%的罕见病由基因决定,这使得遗传咨询在这些病例中至关重要。对沃夫勒姆综合征(WS)患者的随访可用于设计一个方案来支持这些患者,该方案有专门研究WS的研究人员、医疗保健专业人员、患者本人及其家庭环境的参与。此方案也可能适用于其他疾病的诊断和管理。本文阐述了每项基因临床程序的主要步骤,强调了初级保健医生在支持患者及其家庭以及以易懂的方式传递遗传信息方面的作用。

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