Analysis Of 20 Cases With Wiskott-Aldrich Syndrome In China

Background and Objective:Wiskott-Aldrich syndrome is a group of rare inherited immunodeficiency disease and a common pediatric hemorrhagic disease. It is X-linked recessive inheritance. The main clinical manifestations associated with blood platelet disorders, eczema, Immunodeficiency disease.Wiskott-Aldrich syndrome children patients will occur in autoimmune disease and malignant tumors with their survival time increasing. The pathogenesis of WAS syndrome is the WAS gene mutation, leading to its encoded protein mutations or expression missing. In this paper, by analyzing the characteristics of the clinical manifestations of WAS syndrome, immunology and genetics, we can raise awareness, diagnosis and treatment of this disease level of medical workers.Method:Analysis from 2012 to 2015 in the Children’s Hospital of Chongqing Medical University, kidney Immunology first hospitalization for treatment of clinical and immunological data WAS syndrome children.Results:20 cases WAS syndrome were onset within 6 months af-ter birth, the onset of which 17 cases within a month after birth. The main clinical manifestations associated with blood platelet disorders, eczema,Immunodeficiency disease. Immunodeficiency disease can be manifested as recurrent infections(mostly respiratory infections and gastrointestinal infections),autoimmune diseases and malignancy.20 cas-es had thrombocytopenia with bleeding primary performance.Conclusion:Within six months of age, especially infants less than a month if there is unexplained bleeding, eczema or recurrent infections, With or without a family history of childhood mortality, WAS syndrome should be alert to possible, it is best to improve the WAS protein and genetic testing.