Paediatric Department, Royal Darwin Hospital, Darwin, Northern Territory, Australia.
Global Health Division, Menzies School of Health Research, John Mathews Building Royal Darwin Hospital Campus, Darwin, Northern Territory, Australia.
J Paediatr Child Health. 2023 Nov;59(11):1210-1216. doi: 10.1111/jpc.16481. Epub 2023 Aug 17.
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.
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.
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.
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.
风湿性舞蹈病是一种免疫介导的神经精神疾病，也是急性风湿热（ARF）诊断的主要标准。澳大利亚北部偏远地区的儿童患 ARF 的比例过高，但针对该人群的风湿性舞蹈病的流行病学、临床表现和治疗方法的研究有限。
我们对 2002 年 1 月至 2022 年 4 月期间在皇家达尔文医院因风湿性舞蹈病住院的所有≤18 岁的儿科患者进行了回顾性病例系列研究。病例是通过医院的临床编码系统（ICD10）确定的。我们查阅了病历，并提取了人口统计学、临床表现、检查结果、治疗和结局的数据，对其进行了去识别化分析。
2002 年至 2022 年间共发生 110 例风湿性舞蹈病，其中 109 例（99%）为原住民儿童，其中 85%居住在非常偏远的地方。最常见的是全身性运动障碍（影响四肢），占 49%。33 例（30%）报告有神经精神症状，86 例（78%）在就诊时超声心动图显示有风湿性心脏病。所有患者均接受了苄星青霉素治疗，但舞蹈病的治疗方法存在很大差异，包括支持性治疗、抗惊厥药对症治疗、免疫调节药物（包括皮质类固醇）。
本病例系列研究强调了澳大利亚北部原住民儿童患风湿性舞蹈病的巨大负担，并表明治疗方法存在广泛差异。需要进行高质量的临床试验来确定这种致残疾病的最佳治疗方法。
