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Questions and Answers
Which type of collagen is present in granulation tissue, embryonic tissue, uterus, and keloids?
What is the primary cause of dehiscence, or rupture of a wound?
Which factor is a cofactor for collagenase, necessary for replacing type III collagen with type I collagen in wound healing?
Which type of healing leads to minimal scar formation by bringing wound edges together?
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Which primary immunodeficiency disorder is characterized by T-cell deficiency, hypocalcemia, and abnormalities of heart, great vessels, and face?
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Which enzyme deficiency leads to the buildup of toxic products in lymphocytes, causing susceptibility to fungal, viral, bacterial, and protozoal infections in severe combined immunodeficiency (SCID)?
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Which condition presents after 6 months of life with recurrent bacterial, enterovirus, and Giardia Lamblia infections, and is due to a complete lack of immunoglobulin due to disordered B-cell maturation?
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Which autoimmune disorder is characterized by autoimmune destruction of lacrimal and salivary glands?
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Which immunodeficiency disorder is characterized by thrombocytopenia, eczema, and recurrent infections?
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Which complement deficiency increases the risk for Neisseria infections?
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Which process is characteristic of acute inflammation?
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What is the role of Toll-like receptors (TLRs) in acute inflammation?
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Which enzyme is responsible for the release of arachidonic acid (AA) from the cell membrane?
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Which cells predominate after neutrophils and peak 2-3 days after inflammation begins?
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Which cells mediate fibrosis via fibrogenic growth factors and cytokines in the case of abscess formation?
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Which cell type secretes cytokines that promote chronic inflammation by presenting antigens to activate CD4+ helper T cells?
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Which type of killing is less effective than O2-dependent killing and occurs via enzymes present in leukocyte secondary granules?
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Which proinflammatory mediator is produced by Hageman factor when activated?
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What is the primary cause of redness and warmth in inflammation?
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What is the main defect in neutrophil adhesion deficiency?
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Study Notes
Immunodeficiency and Autoimmune Disorders: Key Points
- Common Variable Immunodeficiency (CVID) is characterized by low immunoglobulin due to B-cell or helper T-cell defects, with increased risk for infections and autoimmune diseases.
- IgA deficiency results in low serum and mucosal IgA, with increased risk for mucosal infections, especially viral.
- Hyper-IgM syndrome is characterized by elevated IgM due to mutated CD40L or CD40 receptor, resulting in recurrent pyogenic infections.
- Wiskott-Aldrich syndrome is characterized by thrombocytopenia, eczema, and recurrent infections due to a mutation in the WASP gene.
- Complement deficiencies, such as C5-C9 deficiencies, increase the risk for Neisseria infections, while Cl inhibitor deficiency results in hereditary angioedema.
- Autoimmune disorders are characterized by immune-mediated damage of tissues, with a 1% prevalence in the US, more common in women, and likely triggered by environmental factors in genetically susceptible individuals.
- Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease more common in women, with clinical features including fever, malar rash, arthritis, and renal damage.
- SLE is characterized by antinuclear antibody (ANA) and anti-dsDNA antibodies, and is associated with antiphospholipid antibody syndrome and drug-induced SLE.
- Sjogren syndrome is characterized by autoimmune destruction of lacrimal and salivary glands, presenting as dry eyes and mouth, and is associated with other autoimmune diseases and an increased risk for lymphoma.
- Scleroderma is an autoimmune disease characterized by tissue damage, activation of fibroblasts, and deposition of collagen, with diffuse and localized types.
- Mixed Connective Tissue Disease is an autoimmune disease with mixed features of SLE, systemic sclerosis, and polymyositis, characterized by serum antibodies against Ul ribonucleoprotein.
- Wound healing occurs through a combination of regeneration and repair, with labile tissues possessing stem cells that continuously cycle to regenerate the tissue.
Inflammatory Mediators and Neutrophil Function
- 5-lipoxygenase produces leukotrienes (LT), which have diverse effects on inflammation.
- LTB4 attracts and activates neutrophils, while LTC4, LTD4, and LTE4 mediate vasoconstriction, bronchospasm, and increased vascular permeability.
- Mast cells, widely distributed throughout connective tissue, are activated by various stimuli, resulting in immediate and delayed inflammatory responses.
- Complement proteins, circulating as inactive precursors, are activated through three pathways, resulting in the production of various proinflammatory mediators.
- Hageman factor (Factor XII) is an inactive proinflammatory protein produced in the liver, which, when activated, affects coagulation, complement, and kinin systems.
- Cardinal signs of inflammation include redness and warmth due to vasodilation, swelling due to fluid leakage, and pain due to sensitization of sensory nerve endings.
- Inflammation is associated with fever, caused by the release of IL-1 and TNF, resulting in increased cyclooxygenase activity.
- Neutrophil arrival and function involve a series of steps, including margination, rolling, adhesion, transmigration, chemotaxis, phagocytosis, and destruction of phagocytosed material.
- Neutrophil rolling and adhesion involve the upregulation of selectins and adhesion molecules on endothelial cells and leukocytes.
- Neutrophil adhesion deficiency, often due to integrin defects, can lead to clinical manifestations such as delayed umbilical cord separation and recurrent bacterial infections without pus formation.
- Phagocytosis is enhanced by opsonins, and defects in phagolysosome formation can lead to conditions such as Chediak-Higashi syndrome.
- Neutrophil destruction of phagocytosed material involves the oxidative burst, which can be defective in conditions like chronic granulomatous disease (CGD) and myeloperoxidase (MPO) deficiency.
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Description
Test your knowledge of immunodeficiency and autoimmune disorders with this quiz. Explore key points about conditions such as Common Variable Immunodeficiency, IgA deficiency, Wiskott-Aldrich syndrome, Systemic Lupus Erythematosus, Sjogren syndrome, and more.
