Pulmonary hypertension - An increasingly recognized complication of hereditary hemolytic anemias and HIV infection
Pulmonary hypertension - An increasingly recognized complication of hereditary hemolytic anemias and HIV infection
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DOI：
10.1001/jama.299.3.324
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发表时间：
2008-01-23
影响因子：
120.7
通讯作者：
Machado, Roberto F.
中科院分区：
文献类型：
--
作者：
Barnett, Christopher F.;Hsue, Priscilla Y.;Machado, Roberto F.
现代医疗保健大大延长了先天性溶血性贫血（如镰状细胞病和地中海贫血）和人类免疫缺陷病毒（HIV）感染患者的寿命。据估计，10% 的血红蛋白病患者和 0.5% 的 HIV 感染患者会出现中度至重度肺动脉高压。肺动脉高压是一种不断进展的疾病，导致右心衰竭和死亡。全球估计有 3000 万镰状细胞病或地中海贫血患者，以及 4000 万艾滋病毒患者。考虑到这些人群中肺血管疾病的患病率，镰状细胞病和艾滋病毒疾病可能是全世界肺动脉高压的最常见原因。在这篇综述中，总结了这些疾病的流行病学、血流动力学、机制和治疗策略的现有数据。由于治疗可能会降低发病率并延长生存期，因此筛查、诊断和治疗这些患者的努力代表了全球健康机会。
Modern health care has greatly increased longevity for patients with congenital hemolytic anemias ( such as sickle cell disease and thalassemia) and human immunodeficiency virus ( HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for these diseases are summarized. Because therapy is likely to reduce morbidity and prolong survival, efforts to screen, diagnose, and treat these patients represent a global health opportunity.