Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal.
Patan Academy of Health Sciences, Lalitpur, Nepal.
JNMA J Nepal Med Assoc. 2021 Nov 15;59(243):1166-1169. doi: 10.31729/jnma.7019.
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.
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。
