低丙球血症的临床分析及病因探讨  

Clinical analysis and study on pathogenesis in children with hypogammaglobulinemia

作　　者：黄海[1] 沈蕾[2] 许怀祺 朱亚忠[2] 周昕[2] 林梓[2] 王耀平[1] 应大明[1] 

机构地区：[1]上海第二医科大学附属新华医院儿内科,上海200092 [2]上海市儿科医学研究所,上海200092

出　　处：《中国免疫学杂志》2000年第8期440-444,共5页Chinese Journal of Immunology

摘　　要：目的 :通过对 2 5例低丙球血症患儿的临床及实验室指标的检查 ,探查其病因 ,提出早期诊断及治疗的方法 ,以期挽救病儿生命并提高患儿的生命质量。方法 :利用IgG、IgA、IgM单抗 ,通过放射比浊法测定患儿血清IgG、A、M水平。利用CD3 FITC、CD4 E、CD8 FITC和CD19 PE ,通过流式细胞仪 (FACS)检测病人淋巴细胞亚群 ,临床观察患儿临床表现及IVIG替补治疗效果。结果 :1.全部患儿均有各系统反复感染史 ,以呼吸道感染最多见 ,其次为腹泻等。 2 .全部病例均有血清IgG水平的显著下降 ,特别是IgG1,2 ,3亚型的降低 ,10例B细胞降低 ,仅 1例T细胞降低。故根据上述病史及上述指标检测即可确诊本病。 3 经基因及其产物蛋白检测 ,明确诊断 :CVID 14例 ,高IgM血症 1例 ,THG 4例 ,XLA 6例。结论 :1 低丙球血症是反复感染的原因之一。 2 需要应用多种高技术的检测方法来明确其病因。 3 低丙球血症可应用长期静脉替补丙球进行治疗。Objective:To study the clinical presentation and laboratory examination data in children with hypogammaglobulinemia. It may help to study the pathogenesis early diagnosis and find the best way of treatment to save these children's life and improve their quality of life .Methods:With monoclonal antibodies detect IgG, A, M and IgG subset level in these children .With CD3 FITC ,CD4 PE, CD8 FITC ,CD19 PE as reagents, FACS was used to detect the lymphocyte subset distribution pattern ,combined with clinical observation and IVIG replacement therapy.Results:1.All these children had clinical presentation of recurrent infection ,often happened in respiratory tract and gastrointestinal tract.2.Serum IgG level was significantly reduced especially in IgG1,2,3 subsets ,10 children had B lymphocyte decrement (66.67%), only 1 case had T lymphocyte decrement,3.After screening test on the Btk gene and its protein product,these cases were dignosed:CVID 14 cases, HIM 1 case,THG 4 cases,XLA 6 cases. Conclusion:1.Hypogammaglobulinemia was one of the causes in recurrent infection, 2.It's necessary to use many high technique assays to determine the real diagnosis of hypogammaglobulinemia.3.IVIG replacement is useful for these patients,but always require such treatment in whole life span

关 键 词：低丙球血症 B细胞亚群 T细胞亚群 病因 

分 类 号：R553.02[医药卫生—血液循环系统疾病]