Parker L A
College of Nursing, University of Florida, Gainesville 32610, USA.
Neonatal Netw. 1997 Dec;16(8):17-21.
Severe congenital neutropenia, or Kostmann syndrome, describes a rare autosomal recessive disorder in which the neutrophils fail to reach a mature and functional state. The infant with SCN often presents with severe infections beginning in the first month of life. Recently, treatment with recombinant granulocyte colony-stimulating factor has been found to be effective in reducing the mortality and morbidity of this often fatal disorder.
严重先天性中性粒细胞减少症,即科斯特曼综合征,是一种罕见的常染色体隐性疾病,其中中性粒细胞无法达到成熟和功能状态。患有严重先天性中性粒细胞减少症的婴儿通常在出生后第一个月就开始出现严重感染。最近发现,使用重组粒细胞集落刺激因子进行治疗可有效降低这种往往致命疾病的死亡率和发病率。