儿童时期的慢性中性质:三级中心的经验
Letícia Matushita1, Elvis Terci Valera2, Davi Casale Aragon1
1Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Pediatria, Ribeirão Preto, SP, Brazil.
Jornal de pediatria
|January 5, 2024
概括
自身免疫性中性 (AIN) 影响2%的儿科患者,通常需要多学科护理. 专家的早期评估对于治疗儿童这种慢性疾病至关重要.
科学领域:
- 儿科血液学 儿科血液学
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
背景情况:
- 慢性中性衰竭 (CN) 是一种影响中性粒细胞数量的疾病.
- 自身免疫性中性 (AIN) 是一种特定类型的CN,具有独特的诊断标准.
研究的目的:
- 为了确定18岁以下儿童中CN和AIN的患病率.
- 描述患有CN和AIN的儿科患者的临床特征和结果.
主要方法:
- 对1,039名儿科患者进行了回顾性观察研究.
- 定义CN为绝对中性粒细胞数 (ANC) <1.5 × 10^9/L超过三个月.
- 根据临床标准和对葡萄糖皮质类药物的反应来定义AIN;与其他疾病相关的二次AIN.
主要成果:
- 20%的患者患有CN,2%患有AIN (57%为主发性).
- AIN患者的平均感染率为4.2;二次AIN的感染率更高.
- 在38%的AIN患者中,中性缺陷在19.6个月内消失;8名二次AIN患者有先天性免疫错误.
结论:
- 在这个儿科队列中,AIN的患病率为2%.
- 大多数AIN病例最初是由免疫学家或风湿病学家看的,而不是血液学家.
- 建议使用涉及儿科免疫学,风湿学和血液学的多学科方法来管理AIN.
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