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Bubble Boy Disease

Bubble Boy Disease. Severe combined immunodeficiency (SCID). It is a genetic disorder in which the adaptive immune system is nonfunctional.

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Bubble Boy Disease

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  1. Bubble Boy Disease Severe combined immunodeficiency (SCID) It is a genetic disorderin which the adaptive immune system is nonfunctional. SCID is a severe form of heritable immunodeficiency. It is also known as the “bubble boy” disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, become famous for living in a sterile environment.

  2. AIDS and immunity The human immunodeficiency virus (HIV) infects and destroys a particular type of white blood cells. HIV white blood cell

  3. AIDS and immunity The human immunodeficiency virus (HIV) infects and destroys a particular type of white blood cells. HIV white blood cell

  4. AIDS and immunity Over time, the immunity of the infected person may be seriously weakened, resulting in AIDS. HIV white blood cell

  5. AIDS and immunity Patients usually die from infections that are harmless in healthy people. HIV white blood cell

  6. 1 What is immunity

  7. 26.1 Non-specific defence mechanisms immune system (免疫系統) pathogens

  8. Immune system • provides body defence specific defence mechanisms (特異性防禦機制) non-specific defence mechanisms (非特異性防禦機制)

  9. Non-specific defence mechanisms • provide general protection against pathogens • prevent the entry of all types of foreign substances  non-specific

  10. Non-specific defence mechanisms • include physical and chemicalbarriers first line of defence (第一道防線)

  11. Non-specific defence mechanisms • include physical and chemicalbarriers blood clotting phagocytosis inflammatory responses

  12. Physical barriers • physical structures that prevent pathogens from entering the body

  13. Physical barriers 1 Skin • covers the whole body layer of dead cells - constantly worn away and replaced by new cells

  14. Physical barriers 2 Ciliated epithelium of the respiratory tract ciliated epithelial cell physical barrier closely packed cells

  15. Physical barriers 2 Ciliated epithelium of the respiratory tract mucus-secreting cell produces sticky mucus traps dust and pathogens

  16. 26.1 Non-specific defence mechanisms Physical barriers 2 Ciliated epithelium of the respiratory tract cilia beating action movesdust and pathogens in mucus up fromthe bronchi to the pharynx

  17. Physical barriers 2 Ciliated epithelium of the respiratory tract cilia beating action dust and pathogens are swallowed or coughed out

  18. Chemical barriers • chemical secretions that may kill or stop thegrowth of pathogens

  19. Chemical barriers 1 Sebum sebum • secreted by sebaceous glands • a naturalantiseptic(消毒劑) • kills pathogens on the skin

  20. Chemical barriers 2 Gastric juice gastric juice • secreted by gastric glands • contains hydrochloric acid • kills pathogens in the food in the stomach

  21. Chemical barriers 3 Tears and saliva • secreted by tear glands tears

  22. Chemical barriers 3 Tears and saliva • secreted by salivary glands

  23. Chemical barriers 3 Tears and saliva • contain lysozyme (溶菌酶) breaksdown the cell walls of certain bacteria on the conjunctiva and in the mouth cavity

  24. Chemical barriers 4 Vaginal secretion • acidic inhibits the growth of pathogens in the vagina

  25. Mucus is a chemical barrier that kills pathogens. mucus

  26. In fact, mucus is a physical barrier that only traps pathogens without killing them. mucus

  27. 26.1 Identifying features of mammalian skin that are related to body defence 1 Examine a prepared slide of mammalian skin under a microscope using low power magnification or a skin model. 2 Identify the structures that are related to body defence.

  28. Blood clotting • blood clot(血凝塊) seals thewound and stops breeding prevents pathogens from entering through the wound

  29. Blood clotting • formation of a blood clot: - a blood vessel damaged - blood platelets attracted to the wound

  30. Blood clotting • formation of a blood clot: - blood platelets release chemicals soluble protein fibrinogen (纖維蛋白原) insoluble fibrin (纖維蛋白)

  31. Blood clotting • formation of a blood clot: - blood cells trapped in the net of fibrin blood platelet white blood cell red blood cell

  32. Blood clotting • formation of a blood clot: - blood clot dries to form a scab (痂) covering the wound

  33. Phagocytosis • carried out byphagocytes (吞噬細胞) phagocyte bacterium

  34. Phagocytosis phagocyte pathogen enzymes nucleus

  35. Phagocytosis 1 A phagocyte engulfs a pathogen.

  36. Phagocytosis 2 The pathogen is digested by enzymes.

  37. Phagocytosis 3 The digested pathogen is released.

  38. Inflammatory responses Animation wound is infected pathogens

  39. Inflammatory responses 1 Capillary increases its permeability. capillary

  40. Inflammatory responses 2 More phagocytes come out of the capillary. phagocyte

  41. Inflammatory responses 3 Phagocytes engulf and digest the pathogens in the tissue. carrying out phago-cytosis

  42. Inflammatory responses • increased blood flow and accumulation of tissue fluid  infected area becomes red, hot, swollenandpainful inflammation(炎症)

  43. Inflammatory responses • pus (膿) may form inside the wound - consists of the remains of killed pathogens and dead phagocytes

  44. Skin surface Swelling Pin Phagocytes Bacteria Phagocytes andfluid moveinto area Chemicalsignals Whiteblood cell 1 Tissue injury; release ofchemical signals such ashistamine 2 3 Dilation and increased leakinessof local blood vessels; migrationof phagocytes to the area Phagocytes (macrophages andneutrophils) consume bacteriaand cell debris; tissue heals The inflammatory response mobilizes nonspecific defense forces • Tissue damage triggers the inflammatory response The inflammation can disinfect tissues and limit further infection

  45. Lymphatic system Lymphatic system = lymphatic ducts and lymph nodes • The lymphatic system becomes a crucial battleground during infection • It returns tissue fluid to the circulatory system • It fights infections

  46. LYMPHATICVESSEL Tonsil VALVE Right lymphaticduct, enteringvein Tissue cells Lymph nodes Interstitialfluid Bloodcapillary Thoracic duct,entering vein Thoracicduct Thymus LYMPHATICCAPILLARY Appendix Spleen Masses oflymphocytes and Macrophages Bonemarrow Lymphaticvessels Primarylymph organs : Thymus and bone marrow Secondary lymph organs : lymph nodes

  47. It will return it as lymph to the blood • Lymph contains less oxygen and fewer nutrients than interstitial fluid • This lymphatic vessel is taking up fluid from tissue spaces in the skin LYMPHATICVESSEL VALVE Tissue cells Interstitialfluid Bloodcapillary LYMPHATICCAPILLARY

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