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首页> 外文期刊>Pediatrics: Official Publication of the American Academy of Pediatrics >Successful Transplantation of the Thymus in Nezelof's Syndrome
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Successful Transplantation of the Thymus in Nezelof's Syndrome

机译:Nezelof综合征的胸腺成功移植

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A 6-month-old girl with congenital thymic dysplasia or Nezelof's syndrome (lack of T cell function and normal levels of immunoglobulins) was given a transplant of a human thymus gland from a 14-week-old fetus and is surviving 36 months after transplant. Her clinical condition is the principal index of the success of the transplant since most in vitro and in vivo data indicate a full immunologic restoration has not been achieved. However, the number of E-rosettes in the peripheral blood has increased, the number of surface immunoglobulin-bearing cells has decreased, but there is little improvement in mitogen responsitivity. Immunoglobulin levels have increased with age, but there is no demonstrable specific antibody activity. Nevertheless, she remains free of infection, living in an unprotected environment.
机译:一名患有先天性胸腺发育异常或Nezelof综合征(缺乏T细胞功能和正常免疫球蛋白水平)的6个月大女孩接受了从14周龄胎儿的人胸腺移植手术,并且在移植后存活了36个月。她的临床状况是移植成功的主要指标,因为大多数体外和体内数据表明尚未实现完全的免疫修复。但是,外周血中的E-玫瑰红的数量增加了,表面免疫球蛋白的细胞数减少了,但是丝裂原反应性几乎没有改善。免疫球蛋白水平随着年龄的增长而增加,但是没有可证明的特异性抗体活性。尽管如此,她仍然没有受到感染,生活在不受保护的环境中。

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