MMWR Morb Mortal Wkly Rep. 1998 Mar 13;47(9):169-72.
Sickle cell disease (SCD) is an autosomal recessive disorder characterized by production of abnormal (sickle) hemoglobin, resulting in anemia, susceptibility to pneumococcal and other infections, pain, stroke, and multiple organ dysfunctions. The most common types include hemoglobin SS (homozygous) disease, sickle cell-hemoglobin C disease, and the sickle beta-thalassemia syndromes. A randomized controlled trial published in 1986 indicated that daily oral penicillin prophylaxis reduced the incidence of serious infection in young children with SCD and led to widespread adoption of newborn screening programs for SCD. To study the effectiveness and utilization of prevention programs among large populations of infants with SCD, several newborn screening programs in the United States are now attempting to determine rates of complications and actual use of early medical interventions (e.g., penicillin prophylaxis and pneumococcal vaccination). This report focuses on recent mortality in California, Illinois, and New York. In California and Illinois, mortality from all causes among black children born during 1990-1994 with SCD was slightly less than overall mortality for all black children born in the same time period.
镰状细胞病（SCD）是一种常染色体隐性疾病，其特征是产生异常（镰刀状）血红蛋白，导致贫血、易患肺炎球菌及其他感染、疼痛、中风和多器官功能障碍。最常见的类型包括血红蛋白SS（纯合子）病、镰状细胞-血红蛋白C病以及镰状β地中海贫血综合征。1986年发表的一项随机对照试验表明，每日口服青霉素预防可降低SCD幼儿严重感染的发生率，并促使SCD新生儿筛查项目广泛开展。为研究SCD婴儿大群体中预防项目的有效性和利用率，美国几个新生儿筛查项目目前正试图确定并发症发生率以及早期医疗干预措施（如青霉素预防和肺炎球菌疫苗接种）的实际使用情况。本报告重点关注加利福尼亚州、伊利诺伊州和纽约州近期的死亡率。在加利福尼亚州和伊利诺伊州，1990 - 1994年出生的患有SCD的黑人儿童的全因死亡率略低于同期出生的所有黑人儿童的总体死亡率。
