PDF(Pubmed)
Abstract：
UNASSIGNED: Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder, predisposing development of benign and malignant tumours. Given the oncogenic potential, long-term surveillance is important in patients with NF1. Proposals for NF1 care and its specific manifestations have been developed, but lack integration within routine care. This guideline aims to assimilate available information on NF1 associated tumours (based on evidence and/or expert opinion) to assist healthcare professionals in undertaking tumour surveillance of NF1 individuals.
UNASSIGNED: By comprehensive literature review, performed March 18th 2020, guidelines were developed by a NF1 expert group and patient representatives, conversant with clinical care of the wide NF1 disease spectrum. We used a modified Delphi procedure to overcome issues of variability in recommendations for specific (national) health care settings, and to deal with recommendations based on indirect (scarce) evidence.
UNASSIGNED: We defined proposals for personalised and targeted tumour management in NF1, ensuring appropriate care for those in need, whilst reducing unnecessary intervention. We also incorporated the tumour-related psychosocial and quality of life impact of NF1.
UNASSIGNED: The guideline reflects the current care for NF1 in Europe. They are not meant to be prescriptive and may be adjusted to local available resources at the treating centre, both within and outside EU countries.
UNASSIGNED: This guideline has been supported by the European Reference Network on Genetic Tumour Risk Syndromes (ERN GENTURIS). ERN GENTURIS is funded by the European Union. DGE is supported by the Manchester NIHRBiomedical Research Centre (IS-BRC-1215-20007).
UNASSIGNED: By comprehensive literature review, performed March 18th 2020, guidelines were developed by a NF1 expert group and patient representatives, conversant with clinical care of the wide NF1 disease spectrum. We used a modified Delphi procedure to overcome issues of variability in recommendations for specific (national) health care settings, and to deal with recommendations based on indirect (scarce) evidence.
UNASSIGNED: We defined proposals for personalised and targeted tumour management in NF1, ensuring appropriate care for those in need, whilst reducing unnecessary intervention. We also incorporated the tumour-related psychosocial and quality of life impact of NF1.
UNASSIGNED: The guideline reflects the current care for NF1 in Europe. They are not meant to be prescriptive and may be adjusted to local available resources at the treating centre, both within and outside EU countries.
UNASSIGNED: This guideline has been supported by the European Reference Network on Genetic Tumour Risk Syndromes (ERN GENTURIS). ERN GENTURIS is funded by the European Union. DGE is supported by the Manchester NIHRBiomedical Research Centre (IS-BRC-1215-20007).
摘要：
未经证实：1型神经纤维瘤病（NF1）是一种多系统遗传性疾病，良性和恶性肿瘤的易感发展。鉴于致癌潜力，NF1患者的长期监测很重要.已经制定了NF1护理及其具体表现的建议，但在常规护理中缺乏整合。本指南旨在吸收NF1相关肿瘤的可用信息（基于证据和/或专家意见），以协助医疗保健专业人员对NF1个体进行肿瘤监测。
未经评估：通过全面的文献综述，2020年3月18日进行，指南由NF1专家组和患者代表制定，熟悉广泛的NF1疾病谱的临床护理。我们使用改进的Delphi程序来克服针对特定（国家）医疗保健环境的建议中的可变性问题，并处理基于间接(稀缺)证据的建议。
UNASSIGNED：我们为NF1中的个性化和有针对性的肿瘤管理制定了建议，以确保为有需要的人提供适当的护理。减少不必要的干预。我们还纳入了NF1对肿瘤相关的社会心理和生活质量的影响。
UNASSIGNED：该指南反映了欧洲目前对NF1的护理。它们并非是规定性的，可以根据治疗中心的当地可用资源进行调整，在欧盟国家内外。
UASSIGNED：本指南已得到欧洲遗传性肿瘤风险综合征参考网络（ERNGENTURIS）的支持。ERNGENTURIS由欧盟资助。DGE由曼彻斯特NIHR生物医学研究中心（IS-BRC-1215-20007）支持。
未经评估：通过全面的文献综述，2020年3月18日进行，指南由NF1专家组和患者代表制定，熟悉广泛的NF1疾病谱的临床护理。我们使用改进的Delphi程序来克服针对特定（国家）医疗保健环境的建议中的可变性问题，并处理基于间接(稀缺)证据的建议。
UNASSIGNED：我们为NF1中的个性化和有针对性的肿瘤管理制定了建议，以确保为有需要的人提供适当的护理。减少不必要的干预。我们还纳入了NF1对肿瘤相关的社会心理和生活质量的影响。
UNASSIGNED：该指南反映了欧洲目前对NF1的护理。它们并非是规定性的，可以根据治疗中心的当地可用资源进行调整，在欧盟国家内外。
UASSIGNED：本指南已得到欧洲遗传性肿瘤风险综合征参考网络（ERNGENTURIS）的支持。ERNGENTURIS由欧盟资助。DGE由曼彻斯特NIHR生物医学研究中心（IS-BRC-1215-20007）支持。
