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1、Immunodeficiency diseases“bubby boy” David Vetter1. DefinitionCaused by inherited gene defects resulting in congenital deficiency or by acquired damageInclude the deficiency of immune organs, immune cells, immune molecules and signal transductionDivided into the primary immunodeficiency and acquired
2、 immunodeficiencyI . Introductionfailures of host defense 2. Classfication （1）Primary immunodeficiency diseases（PIDD） B cell deficiency disease T cell deficiency disease Combined immunodeficiency Complement deficiency disease Phagocyte deficiency disease（2）Acquired immunodeficiency diseases （AIDD）3.
3、 Features of immunodeficiency diseases recurrent and often overwhelming infections allergy, abnormal proliferation of lymphocytes, autoimmunity susceptible to certain types of tumor PIDD related to genetic factorsII. Primary immunodeficiency diseases（PIDD） congenital deficiencyTongxin ChenProfessor,
4、 MD, PhDDepartment of Immunology,Shanghai Children s Medical Center,Shanghai Jiao Tong University School of Medicine,III. Acquired immunodeficiency disease（AIDD）Cause : infection, malnutrition therapeutic agents: X - rays, cytotoxic drugs, corticosteroids surgical operation , trauma iatrogenic immun
5、odeficiency disease radiographic exposure application of immunosuppressive agents and antibiotics, anti tumor chemotherapy IV. Acquired immunodeficiency syndrome (AIDS) 1981.6.5 USA CDC predisposition to opportunistic infection: Kaposi s sarcoma or B - cell lymphoma concurrent depletion of CD4 + T c
6、ells DNA toxicityHIV I9.2kbhigh USA, EuropeHIV II9.26kblow Africa1983, HIV-1, HIV-2 USA NIH and France Paris Harald zur Hausen (centre), with fellow Medicine Laureates Luc Montagnier (left) and Franoise Barr-Sinoussi (right) during their interview with Nobelprize.org in Stockholm, 6 December 2008. T
7、wo French scientists who discovered the AIDS virus and a German who bucked conventional wisdom to find a virus that causes cervical cancer were awarded the 2008 Nobel prize for medicine.Robert Charles Gallo (born March 23, 1937) is an American biomedical researcher. He is best known for his role in
8、the discovery of the human immunodeficiency virus (HIV), the infectious agent responsible for acquired immune deficiency syndrome (AIDS), and he has been a major contributor to subsequent HIV research.医学免疫学上海第二医科大学免疫教研室CUBY p445 f19-8医学免疫学上海第二医科大学免疫教研室AIDS distribution in the world1.Route of transmi
9、ssionexposure to bodily fluids from an infected individual sexual intercourse blood, or blood products (infusion, contaminated needles for intravenous drug delivery)Maternal-fetal transmission during birth or by breastfeeding2. The pathogenic mechanism of HIV CD4T Mo-M DC neurogliocyteExpress CD4+co
10、-receptor of HIV The process of HIV invasion target cellsAbul K. abbas et al. Cellular and Molecular Immunology, 7th edition, Elsevier SaundersRANTES, MIP-1, MIP-1CCR5 (1) CD4+ T a. HIV kill CD4Th directly HIV gp120CD4 CCR5, CXCR4b. HIV kill CD4+T indirectly induction of cytotoxic cytokines product
11、ion Tc activation kill Th； ADCC kill Th； HIV（superantigen）, kill Thc. HIV induced apoptosis gp120 cross link with CD4, Ca2+ , induce apoptosis gp120 cross link with CD4, induction Fas expression of Th（2）B cell polyclonal activation High Ig production of self-Abdeclined help function of Th to B cell（
12、3）M、DC Virus refuge ，diffusion（4）NKADCC function production of TNF- , IFN- , IL-2 and IL-12Injury of peripheral lymphoid tissueCentral nervous system lesions Digestive system TumorKaposis sarcoma, Hodgkins disease, cervical and anal cancer3. Mechanism of immune evasion by HIVEpitope sequence variat
13、ionDC： complete package of viral particles, prevent HIV from inactivation and swallowedLatent infection ：non expression of HIV protein on infected cells, decreased expression of CD4+ and MHC molecules 4. Immune response to HIVHumoral immunity (limited) Neutralizing antibody: but the antibody titer i
14、s low, virus mutates quickly anti P24：the effect is unknown anti gp120 and anti gp41：ADCC kill infected cells Cellular immunity CD8+T：kill infected cells CD4+T：produce cytokines5. The clinical course of AIDSAcute infection Asymptomatic period Syptomatic TypicalFlu-like symptom period period6. Diagno
15、sis of AIDSVirus antigen：p24Acute infection period and late period：AbDetection of viral nucleic acidIsolation and identification of virusCD4+T cell count7. Treatment of AIDS Nucleoside and non-nucleoside reverse transcriptase inhibitors Protease inhibitorDavid Ho, HIV/AIDS researcher He championed t
16、he combination anti-retroviral therapy. Hos research team is working on developing vaccines for AIDS.On January 8, 2001, he was presented with the Presidential Citizens Medal by President Clinton. Ho was Time magazines 1996 Man of the Year. IV. Diagnosis and therapy principle of IDDPrevention of infectionBone marrow or stem cell transplantationGene therapyInfusion of Ig or immune cellsKey points1.Definition and classification of IDD2.Pathogenic mechanism of HIV3.Immune evasion by HIV4.Immune system fights for HIV
