Buchanan G R, Smith S J, Holtkamp C A, Fuseler J P
Pediatrics. 1983 Jul;72(1):93-8.
Although the epidemiology and pathophysiology of serious bacterial infection in homozygous sickle cell anemia (SS disease) have become increasingly well understood, information about infection risk and splenic reticuloendothelial function in hemoglobin SC disease is quite limited. Therefore, the type and frequency of invasive bacterial disease were examined in 51 children with SC disease followed for 370 person-years and splenic function was assessed in 31 patients by quantitation of pitted erythrocytes. Seven serious bacterial infections occurred in four of the patients, five due to Streptococcus pneumoniae and two to Haemophilus influenzae. A primary focus of infection was present in all episodes, none of which proved fatal. Although 30 episodes of pneumonia or chest syndrome occurred in 20 of the patients, a bacterial etiology was proven in only three instances. Splenic function was usually impaired, with a mean pit count of 7.1% +/- 8.2% (range 0% to 22.9%). This is significantly greater than normal, but less than pit counts in patients with SS disease or asplenic subjects. Children with SC disease may have a greater risk of bacterial infection than normal children, but their infection rate is not nearly as high as that in patients with SS disease.
尽管纯合子镰状细胞贫血（SS病）中严重细菌感染的流行病学和病理生理学已越来越为人所知，但关于血红蛋白SC病的感染风险和脾网状内皮功能的信息却十分有限。因此，我们对51例随访370人年的SC病患儿的侵袭性细菌性疾病的类型和发生率进行了研究，并通过对有核红细胞计数对31例患者的脾功能进行了评估。4例患者发生了7次严重细菌感染，其中5次由肺炎链球菌引起，2次由流感嗜血杆菌引起。所有感染均有原发感染灶，无一例死亡。尽管20例患者发生了30次肺炎或胸部综合征，但仅3例证实有细菌病因。脾功能通常受损，平均有核红细胞计数为7.1%±8.2%（范围0%至22.9%）。这明显高于正常水平，但低于SS病患者或无脾者的有核红细胞计数。SC病患儿可能比正常儿童有更高的细菌感染风险，但其感染率远低于SS病患者。
