Primary Immunodeficiency Disease, PID) Wang Xiaochuan Childrens Hospital
Wang Xiaochuan Children’s Hospital (Primary Immunodeficiency Disease, )
Primary Immunodeficiency Disease A group of disorders characterized by an impaired ability to produce normal immune response. Most of these disorders are cased by mutations in genes involved in the development and function of immune organs, cells, and molecules Clinical features: Recurrent infection, high risk of autoimmune diseases, allergy and malignancy Wang Xiaochuan Childrens Hospital
Wang Xiaochuan Children’s Hospital Primary Immunodeficiency Disease • A group of disorders characterized by an impaired ability to produce normal immune response. Most of these disorders are cased by mutations in genes involved in the development and function of immune organs, cells, and molecules. • • Clinical features：Recurrent infection, high risk of autoimmune diseases, allergy and malignancy
Complement Phagocyte 2% 18% 10% Cell mediated Antibod 50% 20% Combined Wang Xiaochuan Childrens Hospital
Wang Xiaochuan Children’s Hospital 50% 20% 10% 18% 2% Antibody Complement Phagocyte Cell mediated Combined
Predictive patients number of PiD in Shanghai X-Linked Agammaglobulinemia(XLA) 100 1/100.000 Selective IgA Deficiency 1/10000 500 Severe Combined Immunodeficiency (SCID)1/1,000,000 Chronic Granulomatous Disease(CGD) 10 1/1,000,000 Wang Xiaochuan Childrens Hospital
Wang Xiaochuan Children’s Hospital Predictive patients number of PID in Shanghai • X-Linked Agammaglobulinemia (XLA) 1/ 100,000 • Selective IgA Deficiency 1/10000 • Severe Combined Immunodeficiency (SCID) 1/1,000,000 • Chronic Granulomatous Disease (CGD) 1/1,000,000 100 500 10 10
Classification (old) Antibody (B cell) deficiency T cell deficiency Combined immunodeficiency Phagocyte deficiency Complement deficiency Wang Xiaochuan Childrens Hospital
Wang Xiaochuan Children’s Hospital Classification (old) • Antibody(B cell) deficiency • T cell deficiency • Combined immunodeficiency • Phagocyte deficiency • Complement deficiency