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幼年特发性关节炎:诊断与管理。

Juvenile Dermatomyositis: Diagnosis and Management.

机构信息

Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.

Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia;

出版信息

Skinmed. 2024 Sep 17;22(4):272-275. eCollection 2024.

Abstract

Juvenile dermatomyositis (JDM) is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Seven patients with JDM found in the records from 1998-2019 of the Department of Dermatology Farhat Hached Hospital, Sousse, Tunisia. Our study concerned a total of six girls and one boy with a median age at disease onset of 8,16 years. The average time before diagnosis was 8,8 months. The onset of the disease was acute in 2 patients. All patients displayed skin manifestations at diagnosis, with proximal muscular weakness in 4 cases. Four patients had elevated muscle enzymes and all of them showed myopathic findings on electromyography. Oral corticosteroids were prescribed in 6 patients, in association with other systemic therapies. Three patients achieved a good outcome while two others relapsed. The two other patients showed corticosteroids resistance with a fatal outcome in one case. This study highlights the diagnostic features and management of juvenile dermatomyositis.

摘要

幼年型皮肌炎(JDM)是儿童自身免疫性特发性慢性炎性肌病的主要原因。在突尼斯苏塞 Farhat Hached 医院皮肤科的 1998-2019 年的记录中发现了 7 名 JDM 患者。我们的研究共涉及 6 名女孩和 1 名男孩,疾病发病的中位年龄为 8.16 岁。平均诊断前时间为 8.8 个月。2 名患者的疾病发病呈急性。所有患者在诊断时均有皮肤表现,4 例有近端肌肉无力。4 例患者肌肉酶升高,所有患者肌电图均显示肌病表现。6 例患者口服皮质类固醇,同时联合其他全身治疗。3 例患者获得良好结局,2 例患者复发。另外 2 例患者对皮质类固醇耐药,其中 1 例结局为死亡。本研究强调了幼年型皮肌炎的诊断特征和治疗方法。

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