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为了战胜中性白细胞减少和与之相关的反复性严重细菌感染,作者对2例糖原累积病 Ib 型(GSD-Ib)患儿用集落刺激因子进行了治疗。这种遗传代谢性疾病是由于6-磷酸葡萄糖转移系统缺陷所致。除因糖原不能转换成葡萄糖而引起症状(如肝大、空腹血糖过低与乳酸酸中毒)外,这些病人易患反复性细菌感染。2例患儿(14岁与19个月)患中耳炎,反复患口腔与粘膜溃疡、牙龈炎与牙周炎。这些感染是由于骨髓成熟紊乱和巨噬细胞功能障碍所引起的严重中性白细胞减少所致。皮下应用重组粒细胞集落刺激因
In order to overcome neutropenia and associated recurrent severe bacterial infections, the authors treated two patients with glycogen storage disease type Ib (GSD-Ib) with colony stimulating factor. This inherited metabolic disease is due to a defect in the glucose-6-phosphate transfer system. Except for glycogen which can not be converted to glucose and causes symptoms such as hepatomegaly, impaired fasting glucose and lactic acidosis, these patients are susceptible to recurrent bacterial infections. Two children (14 and 19 months) had otitis media, repeated oral and mucosal ulcers, gingivitis and periodontitis. These infections are due to severe neutropenia caused by disturbed bone marrow maturation and macrophage dysfunction. Subcutaneous application of recombinant granulocyte colony stimulating factor