Clinical analysis and study on pathogenesis in children with hypogammaglobulinemia
- VernacularTitle:低丙球血症的临床分析及病因探讨
- Author:
Hai HUANG
;
Lei SHEN
;
Huaiqi XU
;
Al ET
- Publication Type:Journal Article
- Keywords:
Hypogammaglobulinemia in children IgG,IgA,IgM level T,B lymphocyte IVIG replacement
- From:
Chinese Journal of Immunology
2000;0(08):-
- CountryChina
- Language:Chinese
-
Abstract:
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
