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网状系统发育不全是极罕见的严重复合免疫缺陷。本病以先天性粒细胞缺乏症、淋巴细胞缺乏、淋巴样组织和胸腺发育不全以及细胞和体液免疫功能缺乏为特征。患儿多在出生后最初几天内死于致命性的感染。仅有1例幼儿在无菌环境下存活17周的报道。作者用同种异体骨髓移植治疗1例获得成功。患儿父母亲无亲缘关系,借助产钳分娩。生后一直母乳哺育。3周内体重迅速增加。后因患上呼吸道感染,用氨节青霉素治疗,发生口腔念珠菌感染,制霉菌素治疗效果不佳。7周时呼吸道感染加重,胸部及会阴部有斑驳状的斑丘疹。皮疹用氢化可的松乳剂、
Reticulated hypoplasia is an extremely rare and severe complex immunodeficiency. The disease is characterized by congenital agranulocytosis, lymphocytic deficiency, lymphoid tissue and thymus hypoplasia, and lack of cellular and humoral immune function. Most children die of fatal infections in the first few days after birth. Only 1 case of young children surviving in a sterile environment for 17 weeks of coverage. The authors used allogeneic bone marrow transplantation in 1 case was successful. Parents with children unrelated, with forceps delivery. After birth has been breast-feeding. Body weight rapidly increased in 3 weeks. After suffering from respiratory tract infection, with ammonia penicillin treatment, oral candidiasis, nystatin treatment ineffective. Severe respiratory infections at week 7, with mottled rash on the chest and perineum. Rash with hydrocortisone emulsion,