具有正常C1抑制剂的遗传性血管炎:四分之一世纪的进步和持续的障碍
Sandra C Christiansen1, Aleena Banerji2, Jonathan A Bernstein3
1Division of Allergy, Department of Medicine, University of California San Diego, La Jolla, Calif.
概括
具有正常C1抑制剂 (HAE-nl-C1INH) 的遗传性血管仍然具有诊断和治疗的挑战性. 本综述总结了25年来的进展,并提出了一个分类方案,以指导医生在治疗复发性血管患者时.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
- 临床医学 临床医学
背景情况:
- 具有正常C1抑制剂 (HAE-nl-C1INH) 的遗传性血管炎首次描述于25年前.
- 尽管取得了进展,但诊断,分类和治疗HAE-nl-C1INH存在重大临床挑战.
- 了解HAE-nl-C1INH的病理机制对于有效管理至关重要.
研究的目的:
- 总结关于HAE-nl-C1INH的当前科学共识.
- 为照顾HAE-nl-C1INH患者的医生提供见解.
- 提出一个分类方案,以帮助评估复发性血管.
主要方法:
- 过去25年来科学文献的综述.
- 综合当前数据和专家意见.
- 关于HAE-nl-C1INH的拟议分类方案的制定.
主要成果:
- 在了解HAE-nl-C1INH方面已经发生了重大科学发展.
- 拟议的分类方案旨在促进临床决策.
- 准确的诊断和病理机制的理解是有针对性的治疗的关键.
结论:
- 拟议的分类方案可以指导医生在管理HAE-nl-C1INH.
- 可以根据准确的诊断和病理机制设计有针对性的治疗策略.
- 预计了解HAE-nl-C1INH的进一步进展将改善患者的治疗结果.
相关概念视频
Antihypertensive Drugs: Angiotensin-Converting Enzyme Inhibitors
440
Angiotensin-converting enzyme (ACE), a vital component of the renin-angiotensin-aldosterone system, is abundant in lung endothelial cells. ACE converts the inactive decapeptide, angiotensin I, into the active octapeptide, angiotensin II. This potent vasoconstrictor narrows blood vessels, increasing resistance to blood flow and elevating blood pressure. Angiotensin II also stimulates aldosterone production, encouraging kidney cells to reabsorb more sodium and water from urine, thereby increasing...
440
Antihypertensive Drugs: Direct Renin Inhibitors
468
The renin-angiotensin-aldosterone system (RAAS) is an intricate physiological pathway involving numerous enzymes and hormones, including renin, angiotensin-converting enzyme (ACE), angiotensin I and II, and aldosterone. Imbalances within this system increase the production of angiotensin II and aldosterone. Increased angiotensin II levels promote vasoconstriction and blood pressure elevation. Concurrently, higher aldosterone levels stimulate sodium and water reabsorption in the kidneys,...
468
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
126
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
126
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System
360
The activation of the sympathetic nervous system and the renin-angiotensin-aldosterone system (RAAS) contributes to cardiac remodeling, and inhibiting the RAAS is a pharmacological target in heart failure management. As a result, neurohumoral modulation is a crucial treatment principle for managing heart failure. This approach involves using medications like ACE inhibitors (ACEIs), angiotensin receptor blockers (ARBs), β-blockers, mineralocorticoid receptor antagonists (MRAs), and neutral...
360
Angle Closure Glaucoma: Treatment
425
Angle-closure glaucoma, or closed-angle glaucoma, is an eye condition where the iris bulges out and blocks the iridocorneal angle, resulting in a buildup of aqueous humor and increased intraocular pressure. Immediate medical attention is necessary due to the sudden onset of symptoms. The treatment for angle-closure glaucoma includes short-term and long-term approaches. Short-term treatment involves using eye drops like pilocarpine to lower intraocular pressure by increasing aqueous humor...
425
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
122
Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
122


