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儿科贝塞特病:单一三级中心的经验

Ceyhun Açarı¹, Rana İşgüder¹, Rüya Torun¹

¹Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, Izmir, Türkiye.

Archives of rheumatology

|September 8, 2023

概括

儿科护理儿童护理

科学领域:

类风湿病学类风湿病学
儿科儿科儿科
免疫学免疫学免疫学

背景情况:

儿科贝切特病 (PEDBD) 是一种罕见的多系统性炎症性疾病.
早期诊断和适当的治疗对于改善患者的治疗结果至关重要.

研究的目的:

分析PEDBD的临床和表型特征.
在儿科队列中评估诊断标准的满足率.

主要方法:

在2010-2019年间诊断的34名PEDBD患者的回顾性评估.
使用1990年国际研究小组 (ISG),2014年贝塞特病国际标准 (ICBD) 和PEDBD标准对患者进行重新分类.

主要成果:

最常见的症状包括口腔口腔 (97.1%),生殖器 (47.0%) 和眼部卷入 (44.1%).
诊断标准的满足情况各不相同:ISG (52.9%),ICBD (82.4%) 和PEDBD (50.0%).
皮肤病检测阳性率为37.8%,人类白细胞抗原 (HLA) -B51阳性率为78.5%.

结论:

与ISG标准相比,贝塞特病国际标准 (ICBD) 确定了较高比例的患者.
当标准不满足时,Pathergy测试和HLA-B51可以支持诊断.
专家临床意见对于确定PEDBD诊断至关重要.

关键词:

诊断标准诊断标准诊断标准这就是HLA-B51的原因.儿科贝赫塞特病.儿童贝赫塞特病.帕特尔基 (Pathergy) 是一种父亲的态度.

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