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继发于全身幼年特发性关节炎的巨噬细胞活化综合征:病例报告。

Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report.

机构信息

Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal.

Patan Academy of Health Sciences, Lalitpur, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2021 Nov 15;59(243):1166-1169. doi: 10.31729/jnma.7019.

Abstract

Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.

摘要

巨噬细胞活化综合征是一种罕见但危及生命的疾病,常与全身型幼年特发性关节炎相关。其临床表现包括发热、肝脾肿大、高甘油三酯血症、低纤维蛋白原血症、高铁蛋白血症和肝酶异常。这些症状令人担忧但缺乏特异性,往往导致诊断延迟。一名 12 岁男性因间歇性发热就诊,曾接受抗生素治疗,但在住院几天后仍未见效。经过一系列排除多种诊断的检查后,他被诊断为全身型幼年特发性关节炎继发的巨噬细胞活化综合征,并接受了类固醇治疗。

相似文献

本文引用的文献

1
Macrophage activation syndrome: early diagnosis is key.巨噬细胞活化综合征:早期诊断是关键。
Open Access Rheumatol. 2018 Aug 31;10:117-128. doi: 10.2147/OARRR.S151013. eCollection 2018.
2
Macrophage Activation Syndrome.巨噬细胞活化综合征
Clin Pediatr (Phila). 2016 Oct;55(11):1001-4. doi: 10.1177/0009922816664498. Epub 2016 Aug 24.

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