Glassberg Jeffrey
Mount Sinai School of Medicine, New York, NY, USA.
Emerg Med Pract. 2011 Aug;13(8):1-20; quiz 20.
Sickle cell disease (SCD) is the most common genetic disease in the US, affecting approximately 100,000 individuals. In SCD, genetically mutated hemoglobin (HbS) forms rigid polymers when deoxygenated, giving red blood cells a characteristic sickled shape. Increased blood viscosity and cell adhesion produce intermittent vaso-occlusion. The vaso-occlusive phenotype of SCD, which is marked by higher hemoglobin, manifests with frequent painful crises and is associated with a higher risk for developing acute chest syndrome. The hemolytic phenotype is characterized by lower baseline levels of hemoglobin and elevated markers of hemolysis. There are no reliable markers of vaso-occlusive crisis (VOC), ie, vital signs and laboratory tests are normal. After intravenous (IV) opiate titration, patient-controlled anesthesia (PCA) pumps are encouraged. Excess IV fluids have been associated with development of atelectasis, a risk factor for acute chest syndrome. Acute chest syndrome has clinical symptoms similar to pneumonia; these patients will develop progressive hypoxemia, acute respiratory distress syndrome, and death if exchange transfusion is not initiated.
镰状细胞病（SCD）是美国最常见的遗传疾病，影响约10万人。在SCD中，基因突变的血红蛋白（HbS）在脱氧时形成刚性聚合物，使红细胞呈现特征性的镰状。血液粘度增加和细胞粘附导致间歇性血管阻塞。SCD的血管阻塞表型以较高的血红蛋白为特征，表现为频繁的疼痛性危机，并与发生急性胸综合征的较高风险相关。溶血表型的特征是血红蛋白基线水平较低和溶血标志物升高。目前尚无血管阻塞性危机（VOC）的可靠标志物，即生命体征和实验室检查均正常。静脉注射（IV）阿片类药物滴定后，鼓励使用患者自控镇痛（PCA）泵。过量的静脉输液与肺不张的发生有关，肺不张是急性胸综合征的一个危险因素。急性胸综合征的临床症状与肺炎相似；如果不进行换血治疗，这些患者将出现进行性低氧血症、急性呼吸窘迫综合征并死亡。
