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Ebook Harrison''s principles of internal medicine (20/E): Part 2

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Ebook Harrison''s principles of internal medicine (20/E): Part 2

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2o life and an important tool in the fight for survival against pathogenic microorganisms. The human immune system can be divided into two major compon

ents: the innate immune system and the adaptive immune system (Chap. 342). The innate immune system provides the rapid triggering of inflammatory resp Ebook Harrison''s principles of internal medicine (20/E): Part 2

onses based on the recognition (at the cell surface or within cells) of either molecules expressed by microorganisms or molecules that serve as danger

Ebook Harrison''s principles of internal medicine (20/E): Part 2

signals" released by cells under attack. These receptor/ligand interactions trigger signaling events that ultimately 'ead to inflammation. Virtually

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2ajor role because of their phagocytic capacity. The adaptive immi ne system operates by clonal recognition of antigens followed by a dramatic expansio

n of antigen reactive cells and execution of an immune effector program. Most of the effector cells die off rapidly, whereas memory cells persist. Alt Ebook Harrison''s principles of internal medicine (20/E): Part 2

hough noth T and B lymphocytes recognize distinct chemical moieties and execute distinct adaptive immune responses, the latter IS largely dependent on

Ebook Harrison''s principles of internal medicine (20/E): Part 2

the former in generating long-lived humoral immunity. Adaptive responses utilize components of th^ nnate immune system; for example, the antigenprese

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2 of regulatory mechanisms.Hundreds of gene products have been characterized as effec ors or mediators of the immune system (Chap. 342). Whenever the e

xpression or function of one of these products is genetically impaired (provided the function is nonredundant), a primary immunodeficiency (PID) occur Ebook Harrison''s principles of internal medicine (20/E): Part 2

s.PIDs are genetic diseases with primarily Mendellan inheritance. More than 350 conditions have now been described, and deleterious mutations in -346

Ebook Harrison''s principles of internal medicine (20/E): Part 2

genes have b 'en identified. The overall prevalence of PIDs has been estimated in various countries at 5-10 per 100,000 individuals; however, given th

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2responses, from innate through adaptive, cell differentiation, and effecto function and regulation. For the sake of clarity, PIDs should be classified

according to (1) the arm of the immune system that s defective and (2) the mechanism of the defect (when known). Table 344-1 classifies the most prev Ebook Harrison''s principles of internal medicine (20/E): Part 2

alent PIDs according to this manner of classification; however, one should bear in mind that the classification of PIDs sometimes involves arbitrary d

Ebook Harrison''s principles of internal medicine (20/E): Part 2

ecisions because of overlap and, in some cases, lack of data.httPL//etoQKsmed.iQLne,net449473/14/201»TABLE 344-1Classification of Primary Immune Defic

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2hesion: leukocyte adhesion deficiency (LAD)-Impaired killing: chronic granulomatous disease (CGD)•Innate immunity receptors and signal transduction:-D

efects in Toll-like receptor signaling-Mendelian susceptibility to mycobacterial diseaseComplement deficiencies:■ Classical, alternative, and lectin p Ebook Harrison''s principles of internal medicine (20/E): Part 2

athways-Lytic phaseDeficiencies of the Adaptive Immune System•T lymphocytes:-Impaired developmentSevere combined immune deficiencies (SCIDs)-Impaired

Ebook Harrison''s principles of internal medicine (20/E): Part 2

survival, migration, functionDiGeorge’s syndromeCombined immunodeficienciesHyper-IgE syndrome (autosomal dominant)DOCKS deficiencyCD40 Igand deficienc

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

Ebook Harrison''s principles of internal medicine (20/E): Part 2ired functionHyper-IgM syndrome

43173Harrison’s Principles of Internal Medicine, 20e >Chapter 344: Primary Immune Deficiency DiseasesAlain FischerINTRODUCTIONImmunity is intrinsic to

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