Perrine R P, Pembrey M E, John P, Perrine S, Shoup F
Ann Intern Med. 1978 Jan;88(1):1-6. doi: 10.7326/0003-4819-88-1-1.
We studied 270 Saudi Arabs with homozygous sickle cell anemia, using chart review, a register (since 1969), and home visiting in 42 cases. Average follow-up for the total group was 10 years. Seventy-four percent of those diagnosed by age 3 years presented on screening or with merely anemia; 26% presented with illness, abnormal physical findings, or pain. Compared with American or Jamaican blacks, serious complications occurred only 6% to 25% as frequently; leg ulcers did not occur at all; the mortality under age 15 years was 10% as great; mean levels of blood hemoglobin were higher (10 g/dl), reticulocyte count was lower (5% to 6%), and mean fetal hemoglobin (HbF), which was inversely correlated with reticulocytes, was higher (22% to 26.8%). The high HbF is believed to account for the very mild clinical manifestations.
我们对270名患有纯合子镰状细胞贫血的沙特阿拉伯人进行了研究，采用病历审查、一份自1969年起的登记册，并对42例患者进行了家访。整个组的平均随访时间为10年。3岁前确诊的患者中，74%是在筛查时或仅因贫血就诊；26%是因疾病、异常体征或疼痛就诊。与美国或牙买加黑人相比，严重并发症的发生频率仅为其6%至25%；腿部溃疡根本未出现；15岁以下的死亡率仅为其10%；平均血红蛋白水平更高（10 g/dl），网织红细胞计数更低（5%至6%），且与网织红细胞呈负相关的平均胎儿血红蛋白（HbF）更高（22%至26.8%）。高HbF被认为是临床表现非常轻微的原因。
