在童年发病的狼神经炎中发生血管病变
Kyle Ying-Kit Lin1, Eugene Yu-Hin Chan2,3, Yuen-Fun Mak4
1Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR.
Pediatric nephrology (Berlin, Germany)
|September 9, 2024
概括
血管病变 (RVLs) 影响了近四分之一的儿童狼性炎 (cLN) 患者,表明严重的呈现. 非炎症性死性血管病变 (NNV) 特别预测这些儿童的长期脏结果会更差.
科学领域:
- 儿科病学 儿科病学
- 类风湿病学 类风湿病学
- 病理学 病理学 病理学
背景情况:
- 儿童发病性狼性炎 (cLN) 是一种严重的自身免疫性疾病,影响脏.
- 血管病变 (RVL) 是cLN的潜在并发症,但它们的临床意义需要进一步阐明.
研究的目的:
- 调查患有狼性炎的儿科患者RVL的患病率和临床意义.
- 确定与RVLs相关的因素及其对cLN中长期脏结果的影响.
主要方法:
- 追溯审查107个脏活检从84名中国患者的活检证明的cLN (2004-2020).
- 评估RVL患病率,相关的临床因素和复合结局 (晚期CKD,衰竭,死亡).
主要成果:
- 在22.6%的cLN患者中发现了RVL,包括各种类型,包括非炎症性瘤性血管病变 (NNV).
- 存在RVL与较低的EGFR,血红蛋白和血小板计数相关.
- NNV与较差的存率显著相关 (日志等级测试,p=0.0018) 并被确定为不良结局的预测因素 (HR 7.08).
结论:
- 在很大一部分cLN病例中,RVL是值得注意的发现,通常表明更严重的疾病呈现.
- 非炎症性死性血管病变 (NNV) 是一种关键的RVL亚型,与较差的长期病预后有关.
- 建议在未来的cLN分类标准中对RVL进行常规评估和潜在的纳入.
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