低丙球血症的临床分析及病因探讨

目的：通过对２５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,以期挽求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必血清ＩｇＧ、Ａ、Ｍ水平。利用ＣＤ３－ＦＴＴＣ、ＣＤ４－Ｅ,ＣＤ８－ＦＴＴＣ和ＣＤ１９－ＰＥ,通过流式细胞两 淋巴细胞亚群,临床患儿临床表现及ＩＶＩＧ替补治疗效果。结果：１．全部患儿均有各以感染史,以呼吸道感染

Clinical analysis and study on pathogenesis in children with hypogammaglobulinemia

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