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A Multidisciplinary Consensus for Clinical Care and Research Needs for Sturge-Weber Syndrome

机译：用于刺激和孢子综合征的临床护理和研究需求的多学科共识

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BackgroundSturge-Weber syndrome is a neurocutaneous disorder associated with port-wine birthmark, leptomeningeal capillary malformations, and glaucoma. It is associated with an unpredictable clinical course. Because of its rarity and complexity, many physicians are unaware of the disease and its complications. A major focus moving ahead will be to turn knowledge gaps and unmet needs into new research directions. MethodsOn October 1-3, 2017, the Sturge-Weber Foundation assembled clinicians from the Clinical Care Network with patients from the Patient Engagement Network of the Sturge-Weber Foundation to identify our current state of knowledge, knowledge gaps, and unmet needs. ResultsOne clear unmet need is a need for consensus guidelines on care and surveillance. It was strongly recommended that patients be followed by multidisciplinary clinical teams with life-long follow-up for children and adults to monitor disease progression in the skin, eye, and brain. Standardized neuroimaging modalities at specified time points are needed together with a stronger clinicopathologic understanding. Uniform tissue banking and clinical data acquisition strategies are needed with cross-center, longitudinal studies that will set the stage for new clinical trials. A better understanding of the pathogenic roles of cerebral calcifications and stroke-like symptoms is a clear unmet need with potentially devastating consequences. ConclusionsBiomarkers capable of predicting disease progression will be needed to advance new therapeutic strategies. Importantly, how to deal with the emotional and psychological effects of Sturge-Weber syndrome and its impact on quality of life is a clear unmet need.
机译：BackgroundSturge-Weber综合征是与葡萄酒胎记，Leptomeningeal毛细血管畸形和青光眼相关的神经皮肤病。它与一个不可预测的临床课程有关。由于其稀有性和复杂性，许多医生都没有意识到这种疾病及其并发症。前进的主要焦点将成为新的研究方向的知识差距和未满足的需求。 Methodson 2017年10月1日至3日，Sturge-Weber基金会从临床护理网络组装了临床护理患者，患者从鲟鱼队的患者参与网络中识别我们目前的知识状态，知识差距和未满足的需求。 Luestone Mone明确的未满足需求是需要进行护理和监测的共识指导。强烈建议患者随后是多学科临床团队，为儿童和成人进行终身随访，以监测皮肤，眼睛和大脑的疾病进展。在特定时间点处具有标准化的神经影像模式，需要与更强的临床病理学理解一起。需要统一的组织银行和临床数据采集策略，跨中心，纵向研究将设定新的临床试验的阶段。更好地了解脑钙化和中风样症状的致病作用是一种明显的未满足需求，具有潜在的破坏性后果。结论，需要预测疾病进展的结论，以推进新的治疗策略。重要的是，如何应对鲟鱼综合征的情绪和心理作用及其对生活质量的影响是明确的未满足需求。

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