Wong W Y, Powars D R, Chan L, Hiti A, Johnson C, Overturf G
Department of Pediatrics, Division of Hematology-Oncology, University of Southern California, School of Medicine, Los Angeles.
Am J Hematol. 1992 Mar;39(3):176-82. doi: 10.1002/ajh.2830390305.
Annual age-specific incidence rates of Streptococcus pneumoniae or Haemophilus influenzae bacterial septicemia in sickle cell anemia (SS) were determined for the years of 1957 through 1989. Forty-nine patients had 64 episodes of septicemia among a population of 786 SS patients observed for 8,138 person-years. Peak frequency of infection occurred between 1968-1971 and 1975-1981 with a conspicuous absence of episodes in 1972, 1973, 1982-1984, and 1986-1987, thus demonstrating cycles of high and low attack rates. The annual age-specific incidence rate of septicemia varied from 64.5 (1965) to 421.1 (1980) per 1,000 person-years for those under 2 years of age and never exceeded 10.2 per 1,000 in those over 4 years of age. Following the introduction of pneumococcal polyvalent vaccine in 1978, incidence of infection decreased in SS children greater than 2 years of age. No modification of the risk of infection was observed in immunized children less than 2 years of age. During these three decades, there has been a ten-fold increase in the number of SS adults over 20 years of age. The relative risk of chronic sickle complications comparing the survivors of septicemia to the non-infected patients was: subsequent death 1.76, retinopathy 4.06, avascular necrosis 1.95, symptomatic cholelithiasis 1.33, stroke 1.30, and priapism 1.26. These data suggest that prognosis for lifetime severe SS is initially manifested as an increased risk of septicemia during childhood.
测定了1957年至1989年镰状细胞贫血（SS）患者中肺炎链球菌或流感嗜血杆菌细菌性败血症的年度年龄别发病率。在786名SS患者组成的群体中观察了8138人年，49名患者发生了64次败血症发作。感染的高峰频率出现在1968 - 1971年和1975 - 1981年之间，1972年、1973年、1982 - 1984年以及1986 - 1987年明显没有发作，从而显示出发病率的高低循环。2岁以下儿童败血症的年度年龄别发病率每1000人年从64.5（1965年）到421.1（1980年）不等，4岁以上儿童从未超过每1000人年10.2。1978年引入肺炎球菌多价疫苗后，2岁以上SS儿童的感染发病率下降。在2岁以下免疫儿童中未观察到感染风险的改变。在这三十年中，20岁以上的SS成年人数量增加了十倍。将败血症幸存者与未感染患者相比，慢性镰状细胞并发症的相对风险为：随后死亡1.76，视网膜病变4.06，缺血性坏死1.95，有症状的胆石症1.33，中风1.30，以及阴茎异常勃起1.26。这些数据表明，终生重度SS的预后最初表现为儿童期败血症风险增加。
