Knight J, Murphy T M, Browning I
Division of Pediatric Pulmonary Diseases, Duke University Medical Center, Durham, North Carolina 27710, USA.
Pediatr Pulmonol. 1999 Sep;28(3):205-16. doi: 10.1002/(sici)1099-0496(199909)28:3<205::aid-ppul8>3.0.co;2-z.
Sickle cell disease is the most common inherited disorder in African-Americans. Although the primary defect is hematological, the changes in the erythrocytes lead to a vasculopathy with multiorgan injury. The pulmonary complications, i.e., acute chest syndrome and chronic sickle cell lung disease, are significant causes of morbidity and mortality. The pulmonary manifestations result from a unique constellation of factors which come into play in sickle cell disease. Based on the growing understanding of the molecular and cellular biology of sickle cell disease, new therapies are being developed that are likely to ameliorate the natural history of this disease and its complications.
镰状细胞病是非洲裔美国人中最常见的遗传性疾病。虽然主要缺陷是血液学方面的，但红细胞的变化会导致血管病变并伴有多器官损伤。肺部并发症，即急性胸综合征和慢性镰状细胞性肺病，是发病和死亡的重要原因。肺部表现是由镰状细胞病中起作用的一系列独特因素导致的。基于对镰状细胞病分子和细胞生物学的日益了解，正在开发新的疗法，这些疗法可能会改善这种疾病及其并发症的自然病程。
