Abstract：
OBJECTIVE: This exploratory study evaluates rating scale usage by experts from the European Reference Network for Rare Neurological Diseases (ERN-RND) for paediatric MD, considering factors like diagnosis, intellectual disability, age, and transition to adult care. The aim is to propose a preliminary framework for consistent application.
METHODS: A multicentre survey among 25 ERN-RND experts from 10 European countries examined rating scale usage in paediatric MD, categorizing MD into acute, non-progressive, and neurodegenerative types. Factors influencing scale choice and the transition to adult care practices were analysed. A comprehensive literature search was conducted to identify the earliest age of application of these scales in paediatric patients.
RESULTS: The study identifies various rating scales and establishes their usage frequencies for different MDs. Experts highlighted the need for standardized scales and proposed preliminary evaluation strategies based on clinical contexts. Challenges in applying scales to young, non-cooperative patients were acknowledged.
CONCLUSIONS: The study recommends developing standardized rating scales for paediatric MDs to improve evaluations and data collection. It suggests potential scales for specific clinical scenarios to better evaluate disease progression. Comprehensive, patient-centred care remains crucial during the transition to adult care, despite the identified challenges. This exploratory approach aims to enhance patient outcomes and care.
METHODS: A multicentre survey among 25 ERN-RND experts from 10 European countries examined rating scale usage in paediatric MD, categorizing MD into acute, non-progressive, and neurodegenerative types. Factors influencing scale choice and the transition to adult care practices were analysed. A comprehensive literature search was conducted to identify the earliest age of application of these scales in paediatric patients.
RESULTS: The study identifies various rating scales and establishes their usage frequencies for different MDs. Experts highlighted the need for standardized scales and proposed preliminary evaluation strategies based on clinical contexts. Challenges in applying scales to young, non-cooperative patients were acknowledged.
CONCLUSIONS: The study recommends developing standardized rating scales for paediatric MDs to improve evaluations and data collection. It suggests potential scales for specific clinical scenarios to better evaluate disease progression. Comprehensive, patient-centred care remains crucial during the transition to adult care, despite the identified challenges. This exploratory approach aims to enhance patient outcomes and care.
摘要：
目的：这项探索性研究评估了欧洲罕见神经系统疾病参考网络（ERN-RND）专家对儿科MD的评定量表使用情况，考虑到诊断等因素，智力残疾,年龄,过渡到成人护理。目的是为一致应用提出一个初步框架。
方法：对来自10个欧洲国家的25名ERN-RND专家进行了一项多中心调查，检查了儿科MD的使用情况，将MD分类为急性，非进步,和神经退行性类型。分析了影响量表选择和向成人护理实践过渡的因素。进行了全面的文献检索，以确定在儿科患者中使用这些量表的最早年龄。
结果：该研究确定了各种评分量表，并确定了不同MD的使用频率。专家们强调了标准化量表的必要性，并提出了基于临床背景的初步评估策略。将量表应用于年轻人的挑战，不合作的患者被确认。
结论：该研究建议开发儿科MD的标准化评定量表，以改善评估和数据收集。它提出了特定临床情景的潜在量表，以更好地评估疾病进展。全面,在向成人护理过渡期间，以患者为中心的护理仍然至关重要，尽管已确定的挑战。这种探索性方法旨在提高患者的预后和护理。
方法：对来自10个欧洲国家的25名ERN-RND专家进行了一项多中心调查，检查了儿科MD的使用情况，将MD分类为急性，非进步,和神经退行性类型。分析了影响量表选择和向成人护理实践过渡的因素。进行了全面的文献检索，以确定在儿科患者中使用这些量表的最早年龄。
结果：该研究确定了各种评分量表，并确定了不同MD的使用频率。专家们强调了标准化量表的必要性，并提出了基于临床背景的初步评估策略。将量表应用于年轻人的挑战，不合作的患者被确认。
结论：该研究建议开发儿科MD的标准化评定量表，以改善评估和数据收集。它提出了特定临床情景的潜在量表，以更好地评估疾病进展。全面,在向成人护理过渡期间，以患者为中心的护理仍然至关重要，尽管已确定的挑战。这种探索性方法旨在提高患者的预后和护理。
