婴儿期暂时性低丙种球蛋白血症的研究进展

摘要

婴儿期暂时性低丙种球蛋白血症（ THI）属原发性抗体缺陷病一种。其病因及发病机制尚不完全清楚。 THI患儿易并发感染性疾病和过敏性疾病。 THI患儿的血清免疫球蛋白（ Ig） G水平低于相同年龄组水平的2个标准差，可伴IgA和IgM浓度降低；循环中B细胞计数多为正常；对疫苗接种能产生正常的特异性抗体反应。 THI诊断仍基于临床标准和排除其他疾病。处置 THI 以治疗和预防感染为主；当发生严重感染时，可考虑使用静脉注射用人免疫球蛋白替代治疗。 THI 预后大多良好，至3周岁时，其血清Ig水平可自行恢复正常。%Transient hypogammaglobulinemia of infancy(THI) is a primary antibody deficiency.The eti-ology and pathogenesis of THI remain unclear.THI may be associated with recurrent infections and anaphy-lactic diseases.IgG levels of THI patients were 2 standard deviations below normal,and may be accompanied with low levels of IgA and IgM as well.The circulating B-cell values are mostly normal.The patients have nor-mal specific antibody response to immunization .THI diagnosis is still based on clinical criteria and exclusion of other diseases.The major management of THI is to treat and prevent infections,and replacement therapy of human immunoglobulin for intravenous injection(IVIG) in case of severe infections.The prognosis of THI is mostly good and the immunoglobulin levels usually return to normal spontaneously by 3 years of age.