复旦大学：《医学遗传学 Medical Genetics》课程教学资源（PPT课件讲稿）15 免疫缺陷 immunodeficiency

Medical Genetics 15免疫缺陷 immunodeficiency

Medical Genetics 15 免疫缺陷 immunodeficiency

Medical Genetics The immune system is a network of interacting cellular and soluble components. Its function is to distinguish entities within the body as self or nonself and to eliminate those that are nonself

Medical Genetics The immune system is a network of interacting cellular and soluble components. Its function is to distinguish entities within the body as "self" or "nonself" and to eliminate those that are nonself

Medical Genetics Microorganisms are the principa nonself entities, but neoplasms transplants, and certain foreign substances(eg, some toxins) are also important. To accomplish its tasks, the immune system has evolved two mechanisms: nonspecific immunity and specific immunity which are linked to and influence each other

Medical Genetics Microorganisms are the principal nonself entities, but neoplasms, transplants, and certain foreign substances (eg, some toxins) are also important. To accomplish its tasks, the immune system has evolved two mechanisms: nonspecific immunity and specific immunity, which are linked to and influence each other

Medical Genetics Nonspecific (Innate) Immunity This type of immunity is older phylogenetically, is present at birth, does not require a previous encounter with the offending substance, and does not develop memory Innate immunity includes barriers, such as the skin and chemical protection, such as gastric acid

Medical Genetics Nonspecific (Innate) Immunity This type of immunity is older phylogenetically, is present at birth, does not require a previous encounter with the offending substance, and does not develop memory. Innate immunity includes barriers, such as the skin, and chemical protection, such as gastric acid

Medical Genetics There are two cellular components: (1)the phagocytic system, whose function is to ingest and digest invading microorganisms, and (2) natural killer(Nk) cells, whose function is to kill some tumors, microorganisms, and virally infected cells. The soluble components consist of complement proteins, acute phase reactants, and cytokines

Medical Genetics There are two cellular components: (1)the phagocytic system, whose function is to ingest and digest invading microorganisms, and (2) natural killer (NK) cells, whose function is to kill some tumors, microorganisms, and virally infected cells. The soluble components consist of complement proteins, acute phase reactants, and cytokines

Medical Genetics Specific(Adaptive) Immunity Specific immunity has the hallmarks of learning adaptability and memory. The cellular component is the lymphocyte and immunoglobulins(igs)are the soluble component

Medical Genetics Specific (Adaptive) Immunity Specific immunity has the hallmarks of learning, adaptability, and memory. The cellular component is the lymphocyte, and immunoglobulins (Igs) are the soluble component

Medical Genetics Immunodeficiency diseases: A group of diverse conditions caused by one or more immune system defects and characterized clinically by increased susceptibility to infections with consequent severe, acute, recurrent or chronic disease

Medical Genetics Immunodeficiency diseases: A group of diverse conditions caused by one or more immune system defects and characterized clinically by increased susceptibility to infections with consequent severe, acute, recurrent, or chronic disease

Medical Genetics An immunodeficiency disorder should be considered in anyone with infections that are unusually frequent severe, and resistant without a symptom-free interval; from an unusual organism; or with unexpected or severe complications

Medical Genetics An immunodeficiency disorder should be considered in anyone with infections that are unusually frequent, severe, and resistant; without a symptom-free interval; from an unusual organism; or with unexpected or severe complications

Medical Genetics Since immunodeficiency disorders are relatively uncommon, other disorders leading to recurrent infection should be considered first If these disorders can be excluded a defect in host defense should be suspected

Medical Genetics Since immunodeficiency disorders are relatively uncommon, other disorders leading to recurrent infection should be considered first. If these disorders can be excluded, a defect in host defense should be suspected

Medical Genetics DISORDERS WITH INCREASED SUSCEPTIBILITY TO UNUSUAL INFECTIONS Type of Disorder Conditions Disorder Conditions Circulatory Sickle cell disease, diabetes Primary X-linked gammaglobulin and sys mellitus, nephrosis, vari- immuno emia, DiGeorge anomaly temic cose veins, congenital deficien chronic granulomatous disorders cardiac defects cie disease, C3 deficiency Obstructive Ureteral or urethral stenosis, Unusual Antibiotic overgrowth. disorders bronchial asthma. bron micro- chronic infections with chiectasis, allergic rhinitis, biologi resistant organism, contin blocked eustachian tubes factors uous reinfection(contami- cystic fibrosis nated water supply Intest Eczema. burns, skull frac infectious contact. con- mentary tures. midline sinus tracts laminated inhalation ther. defects ciliary abnormalities apy equipment Secondary Malnutrition, prematurity, Foreign Ventricular shunts, central immuno lymphoma, splenectomy, bodies venous catheter, artificial deficien- uremia, immunosuppress heart valves, urinary cath cies sive therapy, protein- eter, aspirated foreign losing enteropathy bodies chronic viral diseases

Medical Genetics DISORDERS WITH INCREASED SUSCEPTIBILITY TO UNUSUAL INFECTIONS