Tsaras Geoffrey, Owusu-Ansah Amma, Boateng Freda Owusua, Amoateng-Adjepong Yaw
Combined Medicine-Pediatrics, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut 06610, USA.
Am J Med. 2009 Jun;122(6):507-12. doi: 10.1016/j.amjmed.2008.12.020. Epub 2009 Apr 24.
Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-Saharan Africa. Long considered a benign carrier state with relative protection against severe malaria, sickle cell trait occasionally can be associated with significant morbidity and mortality. Sickle cell trait is exclusively associated with rare but often fatal renal medullary cancer. Current cumulative evidence is convincing for associations with hematuria, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise-related sudden death. Sickle cell trait is probably associated with complicated hyphema, venous thromboembolic events, fetal loss, neonatal deaths, and preeclampsia, and possibly associated with acute chest syndrome, asymptomatic bacteriuria, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head, and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. Nonetheless, given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.
镰状细胞性状在全球约3亿人身上出现,在撒哈拉以南非洲地区患病率最高,约为30%至40%。长期以来,镰状细胞性状被认为是一种良性携带状态,对重症疟疾有相对的保护作用,但偶尔也可能与严重的发病和死亡相关。镰状细胞性状仅与罕见但往往致命的肾髓质癌有关。目前的累积证据令人信服地表明,它与血尿、肾乳头坏死、低渗尿、脾梗死、运动性横纹肌溶解以及运动相关的猝死有关。镰状细胞性状可能与复杂性前房积血、静脉血栓栓塞事件、胎儿丢失、新生儿死亡和先兆子痫有关,也可能与急性胸综合征、无症状菌尿和妊娠期贫血有关。没有足够的证据表明它与视网膜病变、胆石症、阴茎异常勃起、腿部溃疡、肝坏死、股骨头缺血性坏死和中风有独立关联。尽管存在这些关联,但具有镰状细胞性状的个体的平均寿命与普通人群相似。尽管如此,鉴于有大量具有镰状细胞性状的人,医生了解这些关联很重要。