在儿童中抗脂综合征
Mojca Zajc Avramovic1, Tadej Avcin1
1Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Center Ljubljana, Slovenia; Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Slovenia.
Best practice & research. Clinical rheumatology
|August 13, 2024
概括
儿科抗脂综合征 (APS) 呈现严重的频繁的血栓事件和独特的非血栓特征. 本综述涵盖了儿童APS的临床表现和新兴疗法,包括新生儿病例.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
背景情况:
- 儿童的抗脂综合征 (APS) 罕见但严重,与成人相比,血栓形成和灾难性APS的风险更高.
- 非血栓性表现在儿科APS中很常见,并且可能在血栓性事件之前发生.
- 最近的APS分类标准需要对儿科患者进行评估.
研究的目的:
- 为儿童患者提供与抗脂抗体 (aPL) 相关的临床表现的全面概述.
- 分析已发表的队列和来自国际儿科APS注册表的数据.
- 为了说明由于母性aPL转移而导致婴儿的APS,重点关注围产期血栓形成和神经发育结果.
主要方法:
- 关于儿科抗脂综合征的已发表队列的审查.
- 来自国际儿科APS注册表的数据分析.
- 对新生儿APS的病例研究进行了审查,该病例来自胎盘外母性aPL转移.
主要成果:
- 小儿APS表现出更严重的表现,频繁的血栓复发,与成人APS相比,灾难性APS的发病率更高.
- 非血栓形成的症状在儿童中更为普遍,并且可能发生在血栓形成事件之前.
- 新生儿APS是由于母亲的aPL引起的,很少引起急性围产期血栓,但与长期的神经发育问题有关.
结论:
- 由于其严重程度和独特的临床过程,儿科APS需要专门的管理.
- 新型疗法,包括B细胞和补充抑制剂,显示出希望,特别是在灾难性的APS.
- 了解跨胎盘aPL转移对于管理新生儿风险和长期神经发育随访至关重要.
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