Immune

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    What are immunodeficiency diseases?

    Conditions where host defenses are impaired, leading to infections and autoimmune diseases.

    What defines primary immunodeficiency (PID)?

    Congenital disorders that impair the ability to produce normal immune responses.

    How does X-linked agammaglobulinaemia differ from other immunodeficiencies?

    It specifically involves a lack of gamma globulin and affects B cell function.

    What is the consequence of immunoglobulin deficiency syndrome?

    Severe recurrent infections due to lack of immunoglobulin production.

    What happens during transient hypogammaglobulinaemia in infancy?

    Maternally derived IgG levels decrease as the infant's immune system develops.

    What characterizes Hyper IgM syndrome?

    Defective CD40 signaling leads to low IgA and IgG levels with increased IgM.

    What is the implication of isolated IgA deficiency?

    Increased risk of respiratory infections and autoimmune diseases.

    What causes Di George syndrome?

    Defective development of the thymus and parathyroid glands.

    How does purine nucleoside phosphorylase deficiency affect T cell function?

    It leads to severe and selective deficiency of T lymphocyte function.

    What is Wiskott-Aldrich Syndrome?

    An X-linked recessive disorder characterized by eczema, thrombocytopenia, and recurrent infections.

    What defines ataxia telangiectasia?

    An autosomal recessive disorder with cerebellar ataxia and combined immunodeficiency.

    What is the effect of MHC class II deficiency?

    Impaired development of CD4+ T cells leading to recurrent infections.

    What happens in severe combined immunodeficiency (SCID)?

    Combined deficiency of humoral and cell-mediated immunity leads to early recurrent infections.

    How do complement deficiencies affect immune response?

    They can lead to increased susceptibility to infections and immune complex diseases.

    What is chronic granulomatous disease?

    An autosomal recessive disorder affecting neutrophil function, leading to persistent infections.

    What characterizes Chediak-Higashi syndrome?

    Decreased pigmentation and impaired neutrophil function due to large lysosomal granules.

    What is the consequence of G6PD deficiency in leukocytes?

    Inability to destroy engulfed bacteria, leading to increased infection risk.

    What causes secondary immunodeficiencies?

    Underlying diseases or therapeutic measures that depress the immune system.

    How does AIDS affect the immune system?

    It destroys CD4 T cells, leading to increased vulnerability to opportunistic infections.