在患有原发性免疫缺陷的患者中神经系统的参与
Hulya Kose1, Zuhal Karali1, Muhittin Bodur2
1Department of Pediatric Immunology and Rheumatology, Bursa Uludag University, Turkey.
Allergologia et immunopathologia
|January 8, 2024
概括
神经症状在原发性免疫缺陷疾病 (PID) 中很常见,影响了本研究中99%的患者. 早期诊断和治疗对于管理PID患者的神经并发症至关重要.
科学领域:
- 免疫学 免疫学 免疫学
- 神经学 神经学
- 遗传学 遗传学 是一个
背景情况:
- 初级免疫缺陷疾病 (PID) 的特点是经常性感染,过敏,自身免疫和恶性瘤.
- 神经系统症状是某些PID综合征的显著表现,包括阿塔克西亚-太阳系外症 (AT),尼日梅根断裂综合征 (NBS) 和纯核酸酸酶 (PNP) 缺乏.
- 病理机制,DNA修复障碍,代谢异常和自身免疫与PID的神经条件有关.
研究的目的:
- 研究被诊断患有原发性免疫缺陷疾病 (PID) 的患者的神经系统参与的频谱和患病率.
- 识别常见的神经症状和相关的PID类型.
- 突出早期诊断和干预PID神经并发症的重要性.
主要方法:
- 对108名PID患者进行了回顾性分析,这些患者从一个较大的6000名患者队列中进行了选择.
- 收集和分析了神经症状,诊断和神经成像发现的数据.
主要成果:
- 在108名PID患者中检测到神经问题,中位数发病年龄为7岁.
- 迪乔治综合征 (30%) 和常见可变免疫缺陷 (CVID) (27%) 是该队列中最常见的PID类型.
- 认知延迟 (58%), (23%), (18%) 是最常见的神经结果. 在99%的患者中观察到中枢神经系统的参与,小脑缩 (34%) 和白质病变 (28.4%) 是常见的MRI发现.
结论:
- 神经症状是患有原发性免疫缺陷疾病的患者的重要和频繁的并发症.
- 神经学表现可能是某些PID的初始标志,强调需要全面评估.
- 及时诊断和管理神经问题对于减轻长期损害和改善患者的结果至关重要.
关键词:
视频 视频 视频 在线Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs Dgs神经系统 神经系统神经系统异常 神经系统异常原发性免疫缺陷是什么意思更多相关视频
相关概念视频
Immunodeficiency Diseases
941
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...
There are three main causes of immunodeficiency...
941
Immune Response Against Viral Pathogens
789
The immune system's response to viral infections is a complex and coordinated process involving natural killer (NK) cells, T cell-mediated responses, and antibody-mediated responses.
NK Cells
NK cells are a crucial part of our innate immune system, acting as the first line of defense against viral infections. These cells can recognize and kill infected cells without prior exposure to the virus, effectively slowing down the spread of infection. Additionally, NK cells produce proinflammatory...
NK Cells
NK cells are a crucial part of our innate immune system, acting as the first line of defense against viral infections. These cells can recognize and kill infected cells without prior exposure to the virus, effectively slowing down the spread of infection. Additionally, NK cells produce proinflammatory...
789
Humoral Immune Responses
72.7K
Overview
72.7K
T Cell Types and Functions
1.0K
When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
1.0K
Cell-mediated Immune Responses
68.3K
Overview
68.3K


