Lane P A, Rogers Z R, Woods G M, Wang W C, Wilimas J A, Miller S T, Khakoo Y, Buchanan G R
Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver.
J Pediatr. 1994 Jun;124(6):859-62. doi: 10.1016/s0022-3476(05)83171-3.
We retrospectively examined the medical and autopsy records of seven previously unpublished cases of fatal pneumococcal septicemia in children with hemoglobin SC disease. The earliest death occurred in a 1-year-old child who had congenital heart disease with cyanosis; the other children were aged 3 1/2 to 15 years. Only one child had received pneumococcal vaccine or prophylactic penicillin therapy. All seven children had an acute febrile illness and rapid clinical deterioration despite parenterally administered antibiotic therapy and intensive medical support. Erythrocyte pit counts in two patients were 40.3% and 41.7%, respectively (normal, < or = 3.6%). Autopsy data from five cases showed marked splenic congestion without infarction in five, splenomegaly in four, and bilateral adrenal hemorrhage in three. These cases illustrate that functional asplenia predisposes some children with hemoglobin SC disease to the development of fatal septicemia after the age of 3 years. We conclude that pneumococcal vaccine should be administered to all children with hemoglobin SC disease and that acute febrile illnesses should be investigated promptly for the possibility of septicemia. The routine use of prophylactic penicillin therapy in infants and children with hemoglobin SC disease remains controversial.
我们回顾性研究了7例先前未发表的血红蛋白SC病患儿致命性肺炎球菌败血症病例的医疗和尸检记录。最早死亡的是一名患有先天性心脏病并伴有紫绀的1岁儿童；其他儿童年龄在3.5岁至15岁之间。只有一名儿童接种过肺炎球菌疫苗或接受过预防性青霉素治疗。尽管接受了静脉注射抗生素治疗和强化医疗支持，所有7名儿童均出现急性发热性疾病且临床迅速恶化。两名患者的红细胞凹坑计数分别为40.3%和41.7%（正常范围，≤3.6%）。5例尸检数据显示，5例有明显的脾充血但无梗死，4例脾肿大，3例双侧肾上腺出血。这些病例表明，功能性无脾使一些血红蛋白SC病患儿在3岁后易发生致命性败血症。我们得出结论，应给所有血红蛋白SC病患儿接种肺炎球菌疫苗，对于急性发热性疾病应及时调查是否有败血症的可能。对于血红蛋白SC病婴幼儿常规使用预防性青霉素治疗仍存在争议。
