儿科学经典教学课件-Immunodeficiency （七年制）2014.pptVIP

IMMUNODEFICIENCY Review of basic Immunology Physiological features of immune function in childhood Definition of immunodeficiency disease Classification of PIDs Common clinical presentations Screening laboratory tests Principles of PID management Secondary immunodeficiency 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 Review of basic Immunology Physiological features of immune function in childhood Definition of immunodeficiency disease Classification of PID Common clinical presentations Screening laboratory tests Principles of PID management Secondary immunodeficiency ﹡ 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 Nearly all branches of immune function are maturely developed at birth Transient dysfunction or immunocomprimise condition is mainly due to insufficient exposure to antigens Review of basic Immunology Physiological features of immune function in childhood Definition of immunodeficiency disease Classification of PID Common clinical presentations Screening laboratory tests Principles of PID management Secondary immunodeficiency A diverse group of illnesses that, as a result of one or more abnormalities of the immune system, increased susceptibility to infection.  --Gene mutation