在患有严重综合免疫缺陷 (SCID) 患者的造血细胞移植后的神经发育结果:一项PIDTC研究
Virdette L Brumm1, Sharon A Kidd1, Brent R Logan2,3
1Division of Pediatric Allergy, Immunology, and Blood and Marrow Transplantation, Department of Pediatrics, University of California San Francisco School of Medicine and UCSF Benioff Children's Hospital, San Francisco, CA, USA.
概括
针对严重综合免疫缺陷 (SCID) 的血造细胞移植 (HCT) 显示了平均的神经发育结果. 然而,较低的家庭收入 (<5万美元) 与SCID移植后儿童智商下降有关.
科学领域:
- 免疫学 免疫学 免疫学
- 神经科学是一个神经科学.
- 儿科 儿科 儿科
背景情况:
- 造血细胞移植 (HCT) 为严重联合免疫缺陷 (SCID) 提供了潜在的治疗方法.
- 新生儿查 (NBS) 改善了SCID患者的生存率.
- 对于SCID的长期神经发育效应,HCT对SCID的长期神经发育效应仍未得到充分研究.
研究的目的:
- 评估NBS,HCT调节方案和基因型对SCID患者神经发育结果的影响.
- 评估SCID幸存者的认知,行为和情绪功能.
- 为了确定影响神经发育轨迹的因素,在SCID的HCT后.
主要方法:
- 一项涉及69名SCID受试者 (6-16岁,不包括ADA缺乏症) 的横截面研究,来自17个北美中心.
- 标准化神经发育测试用于评估认知,行为和情绪功能.
- 使用多变量分析来检查NBS,条件,基因型和社会经济地位的影响.
主要成果:
- 在HCT后的SCID患者的表现与标准人群相比处于平均范围内.
- 没有发现NBS,化疗条件或特定基因型对神经发育结果的显著影响.
- 智商显著下降与较低的家庭收入 (每年<5万美元) 有关.
结论:
- 对于SCID的HCT通常会导致平均的神经发育结果.
- 社会经济地位,特别是较低的家庭收入,成为影响SCID幸存者的智商的重要因素.
- 建议对HCT治疗的SCID患者进行定期神经发育监测,以检测潜在的长期缺陷.
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