细胞因子风暴综合征与全身性青少年异常性关节炎有关
Sebastiaan J Vastert1, Susan P Canny2, Scott W Canna3
1Department of Paediatric Rheumatology & Immunology and Center for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Advances in experimental medicine and biology
|August 8, 2024
概括
系统性青少年异常性关节炎 (SJIA) 可能导致细胞因子风暴综合征 (CSS). 这种综合症通常被称为巨细胞激活综合症 (MAS),需要特定的术语来准确的研究沟通.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 儿科 儿科 儿科
背景情况:
- 系统性青少年异常性关节炎 (SJIA) 是儿童的一种严重的自身免疫性疾病.
- 细胞因子风暴综合征 (CSS) 是一种危及生命的高炎症状况.
- 巨细胞激活综合征 (MAS) 经常被用来描述SJIA中的CSS.
研究的目的:
- 为了澄清细胞因子风暴综合征 (CSS) 在全身性青少年异常性关节炎 (sJIA) 的术语.
- 建立关于sJIA相关的CSS的研究和临床沟通的一致语言.
- 在sJIA的背景下,要区分一般术语CSS和具体术语MAS.
主要方法:
- 文献审查和术语分析.
- 对科学出版物中使用的术语进行比较分析.
- 为与sJIA相关的CSS建立一个标准化命名体系.
主要成果:
- 术语细胞因子风暴综合征 (CSS) 适用于与sJIA相关的条件.
- 巨细胞激活综合征 (MAS) 是一个常用的,但可能不准确的术语,用于sJIA-CSS.
- 为了清晰度,建议一致使用"sJIA相关CSS" (sJIA-CSS).
结论:
- 标准化术语对于准确的儿科风湿病学研究和临床实践至关重要.
- 拟议的术语"sJIA-关联的CSS" (sJIA-CSS) 提高了清晰度并减少了模两可.
- 清晰的沟通有助于理解和管理这种严重的SJIA并发症.
相关概念视频
01:20The JAK-STAT Signaling Pathway
8.7K
Several cytokine receptors have tightly bound Janus kinase or JAK proteins attached at their cytosolic tail. Small signaling molecules such as cytokines, growth hormones, or prolactins bind to the cytokine receptors and initiate their dimerization. The dimerization brings the cytosolic JAKs together that trans-phosphorylate and activates each other. The activated JAKs now phosphorylate cytosolic tails of the cytokine receptors, which serve as binding sites for adaptor proteins such as SH2...
8.7K
01:29Autoimmune Disorders
403
Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune...
Concept and Mechanism of Autoimmune Diseases
The immune...
403
01:28Inflammatory Response
1.9K
An inflammatory response is a localized, nonspecific immune reaction that occurs when a tissue is injured. It is characterized by redness, swelling, heat, and pain, which are commonly called the cardinal signs and symptoms of inflammation. Inflammation can sometimes result in a loss of function.
Inflammation can be triggered by various stimuli, such as impact, abrasion, chemical irritation, infections, and extreme hot or cold temperatures. These can damage cells and connective tissue fibers,...
Inflammation can be triggered by various stimuli, such as impact, abrasion, chemical irritation, infections, and extreme hot or cold temperatures. These can damage cells and connective tissue fibers,...
1.9K
01:27Disorders of Leukocytes
923
Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
923
01:23Cystic Fibrosis: Pathogenesis
201
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,...
201
01:30Inflammatory Bowel Disease II: Crohn's Disease
206
Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
206