C3型血球瘤病:密集沉积病和C3型血球炎
Claudio Ponticelli1, Marta Calatroni2,3, Gabriella Moroni2,3
1Independent Researcher, Milan, Italy.
Frontiers in medicine
|December 11, 2023
概括
密集沉积体疾病和C3球体膜炎是C3球体瘤病,由替代补充路径过度激活引起. 这些脏疾病往往导致功能衰竭和移植复发.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學.
- 免疫学 免疫学 免疫学
- 补充系统 补充系统
背景情况:
- 密集沉积性疾病 (DDD) 和C3型血小板炎 (C3GN) 是C3型血小板病.
- 它的特点是C3沉积,球变化和替代补充路径失调.
- 可能是由获得的因素引起的,如C3性因子 (C3NeFs) 或遗传变化.
研究的目的:
- 总结DDD和C3GN的关键特征,原因和结果.
- 突出补充替代途径失调的作用.
- 提供有关临床表现,诊断和预后的信息.
主要方法:
- 审查关于DDD和C3GN的现有文献.
- 分析病理特征和病因因素.
- 临床表现和患者结局的总结.
主要成果:
- DDD和C3GN涉及细胞内细胞的增加,质细胞壁的加厚和C3沉积.
- 替代补充通路过度激活,通常是由于C3NeFs,是主要原因.
- 低血清C3与正常的C4水平是常见的诊断发现.
- 这两种情况都可以发展为末期病 (ESRD),移植复发率高.
结论:
- DDD和C3GN是截然不同的,但相关的C3球体病变,源于补体调节失调.
- 早期诊断和了解潜在的补体异常至关重要.
- 管理策略应解决补充路径失调,以改善患者的治疗结果并减少移植复发.
相关概念视频
Renal Corpuscle
2.3K
The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
2.3K
Nephrons
2.5K
The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
2.5K
Glaucoma: Overview
584
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
584
Complement System
2.4K
The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
2.4K
Cystic Fibrosis: Pathogenesis
252
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
252
Vesicular Tubular Clusters
2.5K
After budding out from the ER membrane, some COPII vesicles lose their coat and fuse with one another to form larger vesicles and interconnected tubules called vesicular tubular clusters or VTCs. These clusters constitute a compartment at the ER-Golgi interface known as ERGIC (Endoplasmic Reticulum Golgi Intermediate Compartment). The ERGIC is a mobile membrane-bound cargo transport system that sorts proteins secreted from ER and delivers them to the Golgi.
With the help of motor proteins such...
With the help of motor proteins such...
2.5K


