免疫缺陷【儿科】.ppt

IMMUNODEFICIENCY;亚太免疫缺陷学会（APSID）;精品资料; 你怎么称呼老师？ 如果老师最后没有总结一节课的重点的难点，你是否会认为老师的教学方法需要改进？ 你所经历的课堂，是讲座式还是讨论式？ 教师的教鞭 “不怕太阳晒，也不怕那风雨狂，只怕先生骂我笨，没有学问无颜见爹娘 ……” “太阳当空照，花儿对我笑，小鸟说早早早……”; ; Recognize self Reject non-self ; -- Anti-infection Clear infectious agents -- Immune homeostasis Clear aged, damaged and dead cells, maintaining stability -- Immune surveillance  Identify and remove mutated cells and prevent tumor ;Immune functions;; 1st phase: Antigen presenting 2nd phase: proliferation of lymphocytes 3rd phase: Effecter function 4th phase: Apoptosis of activated lymphocytes;; ; ﹡ Phagocytosis  -- macrophages , dentritic cells (APC) ↓ -- neutraphil↓   ﹡ T lymphocyte: -- CD40L ↓, CD28 expression ↓  -- TH1/TH2 ↓  ﹡ B lymphocyte: -- maternal IgG cross placenta  -- IgG2 late development (after 2yo)  ﹡ complements; ;;summary; ;A diverse group of illnesses that, as a result of one or more abnormalities of the immune system, increased susceptibility to infection.;  --Gene mutation associated:   primary immunodeficiency, PID)  --Environment-associated:  Secondary immunodeficiency ，SID   or immunocompromise --HIV infection: Acquired immunodeficiency syndrome，AIDS; ;  Nomenclature;Milestones of PID;;Newest classification of PID;Different features of infections in PID;X-linked agammaglobulinemia (XLA); ; ;;Hyper IgM symdrome;IgA;Severe combined immunodeficiency (SCID);γc γ γ ;Wiskott Aldrich syndrome;WAS例5，男，10月，家族史阳性，2004年4月5日入院;WASp expression on PBMC;Summary of WASp gene mutation;Long term outcome of WAS patients;;; ;  -- 反复感染  - 反复性呼吸道感染  - 严重细菌性感染( 肺炎, 败血症, 脑