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Mayla Sgrulletti1,2, Giorgio Costagliola3, Giuliana Giardino4
1Pediatric Immunopathology and Allergology Unit, Policlinico Tor Vergata, University of Tor Vergata, 00133 Rome, Italy.
未分类的原发性抗体缺乏症 (unPAD) 可以演变成持续性原发性免疫缺乏症 (PID). 低IgA和较差的肺炎球菌抗体反应预测持久性,需要长期监测.
科学领域:
- 免疫学 免疫学 免疫学
- 临床医学 临床医学
- 遗传学 遗传学 是一个
背景情况:
- 未分类的初级抗体缺乏症 (unPAD) 是一种新出现的免疫的先天性错误 (IEI).
- 对于非PAD患者,长期随访 (FU) 数据有限.
- 了解unPAD的演变对于诊断持续性原发性免疫缺陷 (PID) 至关重要.
研究的目的:
- 研究从童年到成年期的非PAD患者的临床和免疫变化.
- 在非PAD病例中确定与PID持久性相关的生物标志物.
- 提供对 unPAD.的长期结果的见解.
主要方法:
- 一组23名儿科非PAD患者的队列在临床和免疫学上进行了跟踪.
- 平均随访时间为14年 (范围为3-32年).
- 统计分析确定了与IEI持久性相关的因素.
主要成果:
- 随着时间的推移,UnPAD的诊断发生了变化;44%的病例被解决为婴儿期的过渡性低血糖球蛋白血症.
- 56%的患者患有持续的PID,包括已确认的PAD (30%) 和其他特定的PID.
- 低IgA,肺炎球菌抗体反应受损和复发性呼吸道感染预测了持续的PID.
结论:
- 长期监测非PAD患者是必不可少的.
- 监测有助于确定患者的结果和潜在的发展到特定的IEI诊断.
- 识别生物标志物有助于预测PID持久性.
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