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全身型幼年特发性关节炎或斯蒂尔病的非典型临床表现:两例报告

Atypical clinical presentation of systemic juvenile idiopathic arthritis or Still's disease: a report of two cases.

作者信息

Tiogouo Emeline, Eloundou Paul, Moukodi Hermine, Fozeu Leo, Wafeu Guy Sadeu

机构信息

Pediatric Unit, Efoulan District Hospital, Yaounde, Cameroon.

Department of Internal Medicine and Specialties, Faculty of Medicine and Biomedical Sciences, Yaounde, Cameroon.

出版信息

Pan Afr Med J. 2020 May 12;36:12. doi: 10.11604/pamj.2020.36.12.21932. eCollection 2020.

DOI:10.11604/pamj.2020.36.12.21932
PMID:32774589
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7388600/
Abstract

Juvenile idiopathic arthritis (JIA) constitutes a group of arthritis of unknown origin that begins before the age of 16 years. Still´s disease is the systemic form of this condition. Its clinical presentation is marked by fever, rash and sometimes joint pain, in the absence of evidence of another aetiology of the fever. We present the cases of two boys aged 4 and 10 years admitted for fever, with a cerebral origin for the first and no infectious site for the second. Fever persisted after antimalarial treatment and adequate antibiotics. Ferritinaemia, elevated sedimentation rate, lactate dehydrogenase (LDH), triglycerides, and increased serum transaminases, all in the absence of evidence of other inflammatory or malignant diseases were suggestive of Still's disease. Both children received a corticosteroid therapy with progressive dose reduction associated to methotrexate during treatment. Fever disappeared within a few hours after initiation of corticosteroid therapy, with considerable improvement in clinical state. To the best of our knowledge, these cases are among the rare cases of childhood Still disease reported in sub-Saharan Africa. These cases highlight the importance of investigating non-infectious causes of persistent fever in children, in a context of infectious disease endemicity.

摘要

青少年特发性关节炎(JIA)是一组病因不明的关节炎,起病于16岁之前。斯蒂尔病是这种疾病的全身型。其临床表现以发热、皮疹,有时伴有关节疼痛为特征,且不存在其他引起发热的病因证据。我们报告了两名分别为4岁和10岁男孩因发热入院的病例,第一名男孩发热源于脑部,第二名男孩无感染部位。抗疟治疗和足量抗生素治疗后发热仍持续。铁蛋白血症、血沉升高、乳酸脱氢酶(LDH)、甘油三酯以及血清转氨酶升高,且均无其他炎症或恶性疾病的证据,提示为斯蒂尔病。两名儿童在治疗期间均接受了糖皮质激素治疗,并逐步减量,同时联合甲氨蝶呤。糖皮质激素治疗开始后数小时内发热消失,临床状态有显著改善。据我们所知,这些病例是撒哈拉以南非洲地区报告的儿童斯蒂尔病罕见病例。这些病例凸显了在传染病流行的背景下,调查儿童持续性发热的非感染性病因的重要性。

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Atypical clinical presentation of systemic juvenile idiopathic arthritis or Still's disease: a report of two cases.全身型幼年特发性关节炎或斯蒂尔病的非典型临床表现:两例报告
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本文引用的文献

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Juvenile Idiopathic Arthritis: Diagnosis and Treatment.青少年特发性关节炎:诊断与治疗
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