Heller P, Best W R, Nelson R B, Becktel J
N Engl J Med. 1979 May 3;300(18):1001-5. doi: 10.1056/NEJM197905033001801.
To determine whether sickle-cell trait and glucose-6-phosphate dehydrogenase deficiency influence the course and fatality rates of certain diseases requiring hospitalization, especially those associated with thrombotic phenomena, we conducted a co-operative study of 65,154 consecutively admitted, black male patients in 13 Veterans Administration hospitals. The overall frequency of sickle-cell trait was 7.8 per cent and of glucose-6-phosphate dehydrogenase dificiency 11.2 per cent. Both conditions were present in 0.9 per cent of those examined. There were regional, but no age-dependent, differences in the frequency of sickle-cell trait. Sickle-cell trait had no effect on average age at hospitalization or death, overall mortality, length of hospitalization on medical and surgical wards and frequency of any diagnosis, except essential hematuria and pulmonary embolism. Although statistically significant (P less than 0.001), the differences for the latter were small (1.5 per cent of all patients with normal hemoglobin and 2.2 per cent of patients with sickle-cell trait). Glucose-6-phosphate dehydrogenase deficiency had no adverse effect.
为了确定镰状细胞性状和葡萄糖-6-磷酸脱氢酶缺乏症是否会影响某些需要住院治疗的疾病的病程及死亡率，尤其是那些与血栓形成现象相关的疾病，我们在13家退伍军人管理局医院对65154名连续入院的黑人男性患者进行了一项合作研究。镰状细胞性状的总体发生率为7.8%，葡萄糖-6-磷酸脱氢酶缺乏症的发生率为11.2%。在接受检查的患者中，两种情况同时存在的比例为0.9%。镰状细胞性状的发生率存在地区差异，但不存在年龄差异。镰状细胞性状对住院或死亡的平均年龄、总死亡率、在内科和外科病房的住院时间以及任何诊断的频率均无影响，但原发性血尿和肺栓塞除外。尽管后者的差异具有统计学意义（P小于0.001），但差异很小（在所有血红蛋白正常的患者中占1.5%，在镰状细胞性状患者中占2.2%）。葡萄糖-6-磷酸脱氢酶缺乏症没有不良影响。
