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Immunodeficiency Disorders PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Immunodeficiency disorders".

This medical PowerPoint presentation talks about immunodeficiency disorders. Immunodeficiency disorders are conditions that weaken the immune system, making people more vulnerable to infections and other diseases. There are two main types of immunodeficiency disorders: primary and secondary.

Primary immunodeficiency disorders are caused by genetic defects that affect the development or function of the immune system. These disorders are usually diagnosed in childhood, but they can also appear in adulthood.

Secondary immunodeficiency disorders, on the other hand, are acquired conditions that result from other medical conditions or treatments.

Tags : Immunodeficiency disorders, Primary immunodeficiency disorders, Secondary immunodeficiency disorders

Published on : Mar 16, 2023 | Views : 2944 | Downloads : 7

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Slide 1-

Immunodeficiency disorders Immunology Unit Department of Pathology College of Medicine KSU
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Reference Kuby Immunology 7th Edition 2013 Chapter 18 Pages 593-624
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Objectives Identify that Immunodeficiency is due to a defect in the immune function. Describe the classification of Immunodeficiency. Explain the presentations of different types of Immuno-deficiencies (e.g. recurrent infections). Understand the varieties of immune system deficiencies involving defects in : - T cells, B cells, phagocytes and complement. Know the laboratory investigations for immunodeficiency disorders
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Definition A state in which the ability of the immune system to fight infectious disease is compromised or entirely absent A person who has an immunodeficiency is said to be immuno-compromised
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A boy with congenital ID lived in a bubble for 12 years before he died
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Distribution of Primary immunodeficiencies
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Pattern of infections and symptoms associated with primary immunodeficiencies
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T-cell defects
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Absence or underdevelopment of the Thymus gland (hypoplasia) Hypoparathyroidism - Facial abnormalities - Cardiovascular abnormalities A congenital defect that is marked by: DiGeorge Syndrome(Congenital Thymic Aplasia )
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Children may present with tetany In the complete form: - Extreme susceptibility to viral protozoal, and fungal infections - Profound depression of T-cell numbers - Absence of T-cell responses Features of DiGeorge syndrome
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Fetal thymus tissue graft (14 weeks old) Management of DiGeorge syndrome
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B-cell defects (Gammaglobulinaemias)
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Patients with B-cell defects are subject to: Recurrent bacterial infections but Display normal immunity to most viral and fungal infections Why ???
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Diverse spectrum ranging from: - Complete absence of B-cells - Complete absence of plasma cells Low or absent immunoglobulins Selective absence of certain immunoglobulins Genetic Transmission - Autosomal recessive -X-linked disease: Females : carriers (normal) Males : manifest the disease
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The most common type, 80 to 90 percent Defect in Bruton Tyrosine Kinase (BTK) The defect involves a block in maturation of pre- B- cells to mature B-cells in bone marrow X-linked agammaglobulinaemia (XLA) or Bruton’s hypogammaglobulinaemia (Congenital disease)
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- Reduced B-cell counts to 0.1 percent (normally 5-15 percent) - Absence of Immunoglobulins - Affected children suffer from recurrent pyogenic bacterial infections Features of XLA
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IgA deficiency (1:700) Most are asymptomatic: but may have increased incidence of respiratory tract infections (R.T.I) Some have recurrent R.T.I and gastrointestinal tract symptoms Selective immunoglobulin deficiency(Congenital disease)
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Characterized by: - Low IgG, IgA & IgE - Variable IgM levels most frequently high X- linked hyper-IgM Syndrome (Congenital disease)
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Management of immunoglobulin deficiencies: *Periodic intravenous immunoglobulin (IVIG) reduces infectious complications
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Severe Combined Immunodeficiency (SCID) (Congenital disease) Causes of SCID: Enzyme deficiencies: 1. ADA (adenosine deaminase ) deficiency 2. PNP (purine phosphorylase) deficiency Toxic metabolites accumulate in T and B cells
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Features of SCID - Increased susceptibility to :viral, fungal, bacterial protozoal infections (starting at 3 months of age)
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Management of SCID 1. Infusion of purified enzymes 2. Gene therapy
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Leukocyte defects Quantitative Qualitative
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Congenital agranulocytosis: Defect in the gene inducing G-CSF (granulocyte colony stimulating factor) Features: Pneumonia, otitis media, abscesses Quantitative Defects
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A. Defect in chemotaxis Leukocyte adhesion deficiency (LAD) Defect: in the adhesion molecules responsible of leukocyte trafficking and migration to sites of infection B. Defect in intracellular Killing Chronic granulomatous disease: Defect: in the oxidative complex responsible for producing superoxide radicals Qualitative Defects (Congenital disease)
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Chronic granulomatous disease (CGD)(Congenital disease) Neutrophils lack the "respiratory burst" upon phagocytosis - Characterized by recurrent life- threatening bacterial and fungal infections and granuloma formation
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Complement Deficiency
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Deficiency of all complement components have been described C1-C9
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Laboratory diagnosis of ID Complete blood count : total & differential Evaluation of antibody levels and response to antigens T and B cells counts (Flowcytometry) Measurement of complement proteins and function (CH50) Assessment of phagocytosis and respiratory burst (oxygen radicals)
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Take Home Message Immunodeficiency may be congenital or acquired It can involve any component of the immune system such as cells, antibodies, complement etc. Most common presentation of immunodeficiency is recurrent infections that may be fatal due to delay in diagnosis and lack of appropriate therapy