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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >Manifestations of Tuberous Sclerosis Complex: The Experience of a Provincial Clinic
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Manifestations of Tuberous Sclerosis Complex: The Experience of a Provincial Clinic

机译：结核硬化复合体的表现：省级诊所的经验

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Background: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. Methods: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children's Hospital. Patients were identified through epilepsy and clinical neurophysiology databases. Results: The study population comprised 81 patients, born between 1987 and 2014, who were a median of 10 years (range, 0.2-23.2) at most recent follow-up. Epilepsy occurred in 91% of patients, including 32% with a history of infantile spasms. Nineteen patients underwent epilepsy surgery, nine (47%) of whom were seizure-free at most recent follow-up. Overall, 61% of epilepsy patients had been seizure-free for at least 1 year at the time of last follow-up. Neuropsychiatric disorders were diagnosed in 49% of children, with autism (25%), attention deficit hyperactivity order (19%) and anxiety (16%) being the most common. Cardiac rhabdomyomata occurred in 35% of children and renal angiomyolipomas were seen in 43%. A total of 91% had skin manifestations. Conclusions: This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.
机译：背景：肺结核硬化复合体（TSC）是一种神经皮肤病，具有广泛的表现形式。最近的共识建议强调了TSC的儿童多学科管理的重要性。本研究的目的是检查大型推荐中心的TSC的表现，以确定该人群的护理需求。方法：对不列颠哥伦比亚省儿童医院进行TSC管理的儿童进行了回顾性的系统图表审查。通过癫痫和临床神经生理数据库鉴定患者。结果：该研究人口包括81名患者，1987年至2014年间出生，他们是最近的10年（范围，0.2-23.2）的中位数。癫痫发生在91％的患者中，其中包括32％的婴儿痉挛史。 19名患者接受了癫痫手术，其中九（47％）在最近的后续行动中被扣押。总的来说，61％的癫痫患者在最后一次随访时已经扣押至少1年。神经精神障碍被诊断为49％的儿童，自闭症（25％），注意力缺陷多动顺序（19％）和焦虑（16％）是最常见的。 35％的儿童和肾血管病瘤发生的心脏横纹肌瘤发生在43％。共有91％的皮肤表现。结论：本研究概述了TSC的多系统表现，通过大型儿科推荐中心观察到。癫痫和神经精神病学疾病是该年龄组中发病率的主要原因，为治疗临床医生提供了许多挑战。因为研究人群的子集仍然很年轻，所以神经精神障碍的患病率可能低估了。

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