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6 岁儿童幼年系统性红斑狼疮的一种新表现。

A novel presentation of childhood systemic lupus erythematosus in a 6-year-old child.

机构信息

Medical Student, 51374Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA.

Department of Biomedical Sciences, 51374Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA.

出版信息

Lupus. 2022 Sep;31(10):1276-1279. doi: 10.1177/09612033221111955. Epub 2022 Jul 8.

Abstract

Childhood systemic lupus erythematosus (cSLE) is an autoimmune disorder, characterized by the formation of autoantibodies, that most often presents in children around the age of 12. Antiphospholipid antibody syndrome (APS) is a condition in which antiphospholipid antibodies produced by the patient cause the formation of thromboses. While reports estimate that 30%-40% of cSLE patients have antiphospholipid antibodies, only 4% develop symptomatic APS, with venous thromboses being more common than arterial. Overall, thrombosis has an incidence of about 0.1/10,000 in the pediatric population and usually only occurs in the setting of underlying disease or in relation to medical procedures. Here, we report a case of a 6-year-old female who presented with abdominal pain, arthralgia, fever, and right popliteal artery thrombus. Further testing revealed an abundance of autoantibodies including antinuclear, lupus anticoagulant, and anticardiolipin which resulted in a diagnosis of cSLE with associated APS. This presentation is novel due to both the young age of the patient at disease onset and the presence of concurrent cSLE and APS at initial diagnosis. Childhood systemic lupus erythematosus can be difficult for providers to recognize as it can present with varied nonspecific symptoms but being aware of the potential for onset in children significantly younger than the average will allow for early detection and potentially better outcomes.

摘要

儿童系统性红斑狼疮(cSLE)是一种自身免疫性疾病,其特征是形成自身抗体,通常在 12 岁左右的儿童中出现。抗磷脂抗体综合征(APS)是一种患者产生的抗磷脂抗体导致血栓形成的病症。虽然有报道估计 30%-40%的 cSLE 患者有抗磷脂抗体,但只有 4%发展为有症状的 APS,静脉血栓比动脉更常见。总的来说,在儿科人群中,血栓的发病率约为 0.1/10000,通常仅在基础疾病存在或与医疗程序相关的情况下发生。在这里,我们报告了一例 6 岁女性,她出现腹痛、关节痛、发热和右腘动脉血栓。进一步的检查显示存在大量自身抗体,包括抗核抗体、狼疮抗凝物和抗心磷脂抗体,导致 cSLE 合并相关 APS 的诊断。这种表现很新颖,因为患者在疾病发作时年龄很小,且在初始诊断时同时存在 cSLE 和 APS。儿童系统性红斑狼疮可能很难被医生识别,因为它可能表现出不同的非特异性症状,但意识到儿童发病的潜在可能性比平均年龄小得多,将有助于早期发现并可能获得更好的结果。

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