Preiss D J
St. John's Mercy Medical Center, St. Louis, USA.
Adv Nurse Pract. 1998 Jun;6(6):32-9.
Approximately 2,000 infants with sickle cell disease are born each year in the United States. Sickle cell disease is an inherited disorder of red blood cell hemoglobin. Sickle cells increase adhesion and cause blockage in the small blood vessels, resulting in tissue damage. The cells' production of hemoglobin S results in two major pathophysiologic features of sickle cell disorders: chronic hemolytic anemia and vaso-occlusion. These disorders cause ischemic tissue damage and acute and chronic organ failure. Potential complications for children with sickle cell disease include vaso-occlusive events, splenic sequestration, bacterial septicemia from splenic hypofunction, aplastic crisis, pulmonary compromise including acute chest syndrome, renal tubular dysfunction and renal failure, priapism, aseptic necrosis, gallstones, delayed growth and development, leg ulcers, stroke and premature death. Three major sickle cell complications during the first years of life are dactylitis, splenic hypofunction and splenic sequestration. The risk for pneumococcal meningitis is 36 times greater in children with sickle cell anemia than for black children without the disease, and 314 times greater than for white children.
在美国，每年约有2000名患有镰状细胞病的婴儿出生。镰状细胞病是一种红细胞血红蛋白的遗传性疾病。镰状细胞会增加黏附性，并导致小血管堵塞，从而造成组织损伤。细胞产生的血红蛋白S导致了镰状细胞病的两个主要病理生理特征：慢性溶血性贫血和血管阻塞。这些病症会引起缺血性组织损伤以及急慢性器官衰竭。镰状细胞病患儿的潜在并发症包括血管阻塞事件、脾滞留、脾功能减退引起的细菌性败血症、再生障碍性危象、肺部并发症（包括急性胸部综合征）、肾小管功能障碍和肾衰竭、阴茎异常勃起、无菌性坏死、胆结石、生长发育迟缓、腿部溃疡、中风和过早死亡。生命最初几年的三大镰状细胞并发症是指炎、脾功能减退和脾滞留。镰状细胞贫血患儿患肺炎球菌脑膜炎的风险比无此病的黑人儿童高36倍，比白人儿童高314倍。
