Recurrent Benign Acute Childhood Myositis (BACM) and Incidental Generalised Joint Hypermobility (GJH); A Case Study and Discussion

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Abstract

Benign Acute Childhood Myositis (BACM) is rare clinical entity with an incidence of 2.69 cases per 100,000 that is caused by a range of viral, bacterial and rarely fungal pathogens. Recurrent BACM is even more rare with unknown incidence. Despite BACM being an unusual condition, it is widely reported in the literature, however a lack of knowledge results in missed, or delayed diagnosis. This case report discusses a rare presentation of recurrent BACM in an 11 year old female who presented to the authors’ rural emergency department with her mother. She had an 3 day history of difficulty walking following an upper respiratory tract infection that began 5 days prior to onset of symptoms. Examination revealed bilateral tender calves, sacroiliac joints and reluctance to weightbear with an abnormal gait. An incidental finding of generalised joint hypermobility (GJH) was noted. CK level was 2324 U/L. The patient’s mother disclosed a complex rheumatologic history on her side of the family and 2 identical episodes of BACM occurring within a 12 month timeframe. A discussion on current understanding of the pathophysiology of BACM and gaps in current literature is provided. BACM carries a small risk of rhabdomyolysis and renal failure and patients should be referred for assessment in the Emergency Department. No literature has explored the relationship between GJH and BACM and the relationship between recurrent BACM and other rheumatic diseases such as autoimmune juvenile myositis, or systemic myopathies is not known.
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复发性良性急性儿童肌炎(BACM)和偶发性全身关节活动过多症(GJH);病例研究与讨论
儿童良性急性肌炎(BACM)是一种罕见的临床病症,发病率为每十万人中 2.69 例,由一系列病毒、细菌和极少数真菌病原体引起。复发性 BACM 更为罕见,发病率未知。尽管 BACM 是一种不常见的疾病,但在文献中却被广泛报道,然而由于缺乏相关知识,导致漏诊或延误诊断。本病例报告讨论的是一名 11 岁女性复发性 BACM 的罕见病例,她在母亲的陪同下到作者所在的乡镇急诊科就诊。她在发病前 5 天开始上呼吸道感染,随后 3 天出现行走困难。检查发现她双侧小腿和骶髂关节发软,不愿负重,步态异常。偶然发现全身关节活动过度(GJH)。CK 水平为 2324 U/L。患者的母亲透露,她的家族有复杂的风湿病史,在12个月内曾发生过两次相同的BACM。本文讨论了目前对 BACM 病理生理学的理解以及现有文献的不足之处。BACM 有发生横纹肌溶解症和肾衰竭的小风险,患者应转诊至急诊科进行评估。没有文献探讨过 GJH 与 BACM 之间的关系,也不知道复发性 BACM 与其他风湿性疾病(如自身免疫性幼年肌炎或全身性肌病)之间的关系。
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