目的:Sydenham舞蹈症是一种免疫介导的神经精神疾病,和诊断急性风湿热(ARF)的主要标准。澳大利亚北部偏远地区的儿童患ARF的比例过高,然而,对流行病学的研究,Sydenham舞蹈病的临床表现和治疗在该人群中受到限制.
方法:我们从2002年1月至2022年4月进行了回顾性病例系列研究,研究了皇家达尔文医院收治的Sydenham舞蹈病患者年龄≤18岁的所有儿科患者。使用医院的临床编码系统(ICD10)识别病例。对医疗记录和人口统计数据进行了审查,临床表现,调查结果,提取治疗和结果,去识别和分析。
结果:在2002年至2022年之间发生了一百一十次Sydenham舞蹈表演,其中109(99%)是原住民儿童,85%居住在非常偏远的地方。最常见的是,舞蹈病表现为影响所有四肢的全身运动障碍(49%)。33例(30%)出现神经精神症状,在就诊时,86例(78%)的超声心动图显示有风湿性心脏病的证据。所有患者都接受了苄星青霉素,但是舞蹈病的管理存在很大差异,从支持性管理,抗惊厥药的对症管理,免疫调节药物,包括皮质类固醇。
结论:本病例系列强调了生活在澳大利亚北部的第一民族儿童中Sydenham舞蹈病的重大负担,并显示了治疗方法的差异。需要高质量的临床试验来确定这种残疾状况的最佳治疗方法。
OBJECTIVE: Sydenham chorea is an immune-mediated neuropsychiatric condition, and a major criterion for diagnosis of acute rheumatic fever (ARF). Children in remote Northern Australia experience disproportionately high rates of ARF, yet studies looking at the epidemiology, clinical presentation and management of Sydenham chorea are limited in this population.
METHODS: We conducted a retrospective
case series from January 2002 to April 2022 of all paediatric patients aged ≤18 years admitted to Royal Darwin Hospital with Sydenham chorea. Cases were identified using the hospital\'s clinical coding system (ICD10). Medical records were reviewed and data on demographics, clinical presentation, investigation results, treatment and outcome were extracted, deidentified and analysed.
RESULTS: One hundred ten presentations of Sydenham chorea occurred between 2002 and 2022, 109 (99%) of these were in First Nations children, with 85% residing in very remote locations. Most commonly, chorea presented as a generalised movement disorder affecting all four limbs (49%). Neuropsychiatric symptoms were reported in 33 (30%), and there was evidence of rheumatic heart disease on echocardiogram in 86 (78%) at presentation. All patients received benzathine penicillin, but there was significant variation in management of chorea, ranging from supportive management, to symptomatic management with anticonvulsants, to immunomodulatory medications including corticosteroids.
CONCLUSIONS: This
case series highlights the significant burden of Sydenham chorea among First Nations children living in Northern Australia and demonstrates wide variation in treatment approaches. High-quality clinical trials are required to determine the best treatment for this disabling condition.