22q11.2删除综合征对免疫系统的影响
Nicolai S C van Oers1, Kathleen E Sullivan2
1Departments of Immunology, Microbiology and Pediatrics, The University of Texas Southwestern Medical Center, Dallas, TX, USA.
概括
22q11.2删除综合征 (22q11.2DS) 导致免疫系统问题,影响T,B和巨细胞. 本综述详细介绍了它对胸膜功能的影响以及感染和自身免疫疾病的风险增加.
科学领域:
- 遗传学 是一个遗传学.
- 免疫学 免疫学 免疫学
- 发展生物学 发展生物学
背景情况:
- 22q11.2删除综合征 (22q11.2DS) 是一种常见的遗传疾病,大约影响每2,150个个体中的1个.
- 临床表现是多样化的,包括先天性异常,如胸腺缺血,甲状腺缺血,心脏缺陷和面部形.
- 晚期发作的神经和行为问题,包括自闭症谱系障碍和精神分裂症,也与22q11.2DS有关.
研究的目的:
- 审查22q11.2DS对胸膜功能的原因和后果.
- 为了阐明22q11.2DS对免疫系统的更广泛影响.
- 突出患者对感染,自身免疫性疾病和过敏症的易感性.
主要方法:
- 这是一篇综述性文章,综合了现有的研究和临床数据.
- 从已发表的关于22q11.2DS,胸腺功能和免疫系统异常的文献中收集了信息.
- 分析的重点是22q11.2删除与其对免疫细胞发育和功能的影响之间的关系.
主要成果:
- 在22q11.2DS患者中,胸膜缺血症发生在60-70%的患者中,导致T细胞淋巴衰竭.
- 需要进行胸膜移植的先天性无血症是一种罕见但严重的后果.
- 免疫细胞包括T,B细胞和巨细胞受到影响,增加感染性,自身免疫性和过敏性疾病的风险.
结论:
- 22q11.2DS显著损害了胸膜功能和整体免疫系统的完整性.
- 系统性影响,包括血管透性和血脑屏障的破坏,有助于疾病的复杂性.
- 了解这些影响对于管理22q11.2DS患者多方面的健康挑战至关重要.
相关概念视频
What is the Immune System?
127.7K
Overview
127.7K
Humoral Immune Responses
83.8K
Overview
83.8K
Second Order systems II
406
In an underdamped second-order system, where the damping ratio ζ is between 0 and 1, a unit-step input results in a transfer function that, when transformed using the inverse Laplace method, reveals the output response. The output exhibits a damped sinusoidal oscillation, and the difference between the input and output is termed the error signal. This error signal also demonstrates damped oscillatory behavior. Eventually, as the system reaches a steady state, the error diminishes to zero.
406
First Order Systems
426
First-order systems, such as RC circuits, are foundational in understanding dynamic systems due to their straightforward input-output relationship. Analyzing their responses to different input functions under zero initial conditions reveals significant insights into system behavior.
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
426
Second Order systems I
592
A servo system exemplifies a second-order system, featuring a proportional controller and load elements that ensure the output position aligns with the input position. The relationship between these components is described by a second-order differential equation. Applying the Laplace transform under zero initial conditions yields the transfer function, showing how inputs are converted to outputs in the system.
By reinterpreting the system, one can derive the closed-loop transfer function, which...
By reinterpreting the system, one can derive the closed-loop transfer function, which...
592
Nephrotic Syndrome I : Introduction
623
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
623


