儿童的异常性性综合征
Damien G Noone1, Kazumoto Iijima2, Rulan Parekh3
1Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
Lancet (London, England)
|June 19, 2018
概括
儿童的异常性综合征 (NS) 的发病率和原因不尽相同,通常涉及免疫问题或遗传因素. 虽然对类固醇反应的NS有很好的预后,但类固醇耐药性表明病的风险更高.
科学领域:
- 儿童脏病学
- 免疫学
- 遗传学
背景情况:
- 每10万名儿童中有1.15-16.9名患有异常性瘤综合征 (NS),发病率因种族和地区而异.
- 发病过程很复杂,可能涉及免疫失调,循环因子或细胞异常.
- 基因因素对耐固醇性NS的影响更大.
研究的目的:
- 总结当前对儿童异常性综合征的了解.
- 突出其发病,治疗,并发症和预后的关键方面.
主要方法:
- 对形性综合征的现有文献进行审查.
- 对流行病学数据,病原机制,治疗方法和预后因素的分析.
主要成果:
- 主要治疗类固醇反应的病例是普得尼松.
- 频繁复发可能需要替代免疫抑制剂.
- 主要并发症包括感染,静脉血栓塞和急性损伤.
结论:
- 对类固醇反应的NS通常具有很好的长期结局.
- 类固醇耐药性是未来慢性或末期病风险的关键决定因素.
- 对类固醇耐药病例进行进一步的研究和治疗是有必要的.
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