接受新生儿和儿科肺高血压的挑战
Dunbar Ivy1, Erika B Rosenzweig2, Steven H Abman3
1Pediatric Cardiology, University of Colorado School of Medicine, and Children's Hospital Colorado, Aurora, CO, USA.
The European respiratory journal
|August 29, 2024
概括
儿童肺动脉高血压 (PAH) 由于相关疾病需要独特的方法. 本综述更新了儿科PH的诊断,风险分层和治疗,突出了临床试验中的知识差距和挑战.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 肺部病理学 肺部病理学
- 关键护理医学 关键护理医学
背景情况:
- 儿科肺动脉高血压 (PAH) 与成人PAH具有共同的特征,但具有独特的挑战.
- 相关疾病和特定的患者群体需要不同的管理策略.
研究的目的:
- 审查最近的进展,正在进行的挑战和儿童肺高血压 (PH) 的独特方法.
- 提供关于儿童PH的诊断,分类,风险分层和治疗的最新信息.
- 确定关键的知识差距,并为儿科PH管理提出解决方案.
主要方法:
- 第七届肺高血压世界研讨会儿科工作组的共识审查.
- 包括更新的风险分层工具和处理算法.
- 讨论与先天性心脏病和新生儿PH相关的PAH的新策略.
主要成果:
- 一个更新的儿童PH风险分层工具和治疗算法,包括相关心肺疾病的策略.
- 突出新生儿PH诊断的进展,使用心声回声学,并强调团队方法.
- 讨论儿科药物批准和临床试验设计中的挑战.
结论:
- 儿科PH管理需要量身定制的方法,针对特定的患者群体和相关疾病.
- 尽管取得了进展,但由于缺乏强大的临床试验和药物开发方面的挑战,治疗仍然受到阻碍.
- 基于多模式和团队的方法对于优化PH婴儿和儿童的结果至关重要.
相关概念视频
Pulmonary Hypertension: Classification and Pathogenesis
158
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
158
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
187
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
187
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
142
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...
142
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
160
Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
160
Pulmonary Cycle: Exhalation
1.5K
In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.5K
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
147
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...
147


