Patho Ch 19 Quiz

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  • 1/22 Questions

    Infants are born with a passive immunity that occurs when immunoglobulin antibodies cross the placenta from the maternal circulation prior to birth. Which immunoglobulin is capable of crossing the placenta?

    • IgM
    • IgD
    • IgG
    • IgE
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About This Quiz

Patho Ch 19 quiz assesses understanding of various immunodeficiency disorders including DiGeorge Syndrome, Secondary Immunodeficiency, and more. It evaluates key concepts crucial for students and professionals in medicine, focusing on both congenital and acquired immunodeficiencies.

Patho Ch 19 Quiz - Quiz

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  • 2. 

    IgE-mediated disorders

    • Type I hypersensitivity reaction

    • Type II hypersensitivity reaction

    • Type III hypersensitivity reaction

    • Type IV hypersensitivity reaction

    Correct Answer
    A. Type I hypersensitivity reaction
    Explanation
    Type I hypersensitivity reactions, also known as immediate hypersensitivity reactions, are caused by the release of histamine and other mediators from mast cells and basophils. These reactions occur within minutes of exposure to an allergen and are characterized by symptoms like hives, itching, sneezing, and wheezing. IgE antibodies play a crucial role in type I hypersensitivity reactions by binding to mast cells and basophils, leading to their activation and subsequent release of inflammatory mediators. This type of reaction is commonly associated with allergies, such as hay fever, asthma, and anaphylaxis.

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  • 3. 

    Some people are so sensitive to certain antigens that they react within minutes by developing itching, hives, and skin erythema, followed shortly thereafter by bronchospasm and respiratory distress. What is this commonly known as?

    • Antigen reaction

    • Anaphylactic reaction

    • Hyposensitive reaction

    • Arthus reaction

    Correct Answer
    A. Anaphylactic reaction
    Explanation
    Anaphylactic reaction refers to a severe and immediate immune response triggered by certain antigens. This reaction is characterized by symptoms such as itching, hives, skin erythema (redness), bronchospasm (constriction of the airways), and respiratory distress. It occurs within minutes of exposure to the antigen and can be life-threatening if not treated promptly.

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  • 4. 

    Partial or complete failure of development of the thymus and parathyroid glands

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. DiGeorge Syndrome
    Explanation
    DiGeorge Syndrome is a genetic disorder that results in the partial or complete failure of development of the thymus and parathyroid glands. This leads to a weakened immune system and can cause various health problems, including immunodeficiency. Therefore, DiGeorge Syndrome is the correct answer for the given question.

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  • 5. 

    In levels of serum and secretory IgA

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Selective IgA deficiency
    Explanation
    Selective IgA deficiency is a condition characterized by low levels or absence of immunoglobulin A (IgA) in the blood. IgA is an antibody that plays a crucial role in the immune system's defense against infections in the mucous membranes, such as those in the respiratory and gastrointestinal tracts. Selective IgA deficiency can make individuals more susceptible to recurrent infections, particularly in these areas. It is the most common primary immunodeficiency disorder and is usually asymptomatic, but some individuals may experience symptoms such as respiratory or gastrointestinal infections, allergies, or autoimmune disorders.

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  • 6. 

    T-cell mediated disorders

    • Type I hypersensitivity reaction

    • Type II hypersensitivity reaction

    • Type III hypersensitivity reaction

    • Type IV hypersensitivity reaction

    Correct Answer
    A. Type IV hypersensitivity reaction
    Explanation
    Type IV hypersensitivity reaction is a delayed hypersensitivity reaction mediated by T-cells. It is characterized by the activation of T-cells upon exposure to an antigen, leading to the recruitment and activation of inflammatory cells such as macrophages and cytotoxic T-cells. This type of reaction is involved in various immune-mediated disorders such as contact dermatitis, tuberculin skin test reactions, and graft rejection. Unlike other types of hypersensitivity reactions, type IV reactions do not involve antibodies but rather the direct involvement of T-cells in the immune response.

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  • 7. 

    Low IgG and IgA levels, high IgM concentrations 

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Hyper-IgM syndrome
    Explanation
    Hyper-IgM syndrome is characterized by low levels of IgG and IgA, and high concentrations of IgM. This condition is caused by a genetic mutation that affects the ability of B cells to switch from producing IgM to other types of antibodies, such as IgG and IgA. As a result, individuals with Hyper-IgM syndrome have a weakened immune system and are more susceptible to infections.

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  • 8. 

    Combined immunodeficiency syndrome (CIDS) is distinguished by low, not absent, T-cell function. These diseases are usually associated with other disorders and arise from diverse genetic causes. Which of the following diseases is considered a CIDS?

    • Pierre-Robin syndrome

    • Angelman syndrome

    • Ataxia-telangiectasia

    • Adair-Dighton syndrome

    Correct Answer
    A. Ataxia-telangiectasia
    Explanation
    Ataxia-telangiectasia is considered a CIDS because it is characterized by low T-cell function. This syndrome is associated with other disorders and is caused by diverse genetic factors. Pierre-Robin syndrome, Angelman syndrome, and Adair-Dighton syndrome are not classified as CIDS because they do not exhibit low T-cell function.

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  • 9. 

    The immune system typically responds to invaders of all types in our bodies. However, it can also cause tissue injury and disease. What is this effect called?

    • Hypersensitivity reaction

    • Antigen reaction

    • Mediator response action

    • Allergen stimulating reaction

    Correct Answer
    A. Hypersensitivity reaction
    Explanation
    The immune system typically responds to invaders in our bodies, but sometimes it can overreact and cause tissue injury and disease. This overreaction is known as a hypersensitivity reaction.

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  • 10. 

    Antibody-mediated disorders

    • Type I hypersensitivity reaction

    • Type II hypersensitivity reaction

    • Type III hypersensitivity reaction

    • Type IV hypersensitivity reaction

    Correct Answer
    A. Type II hypersensitivity reaction
    Explanation
    Type II hypersensitivity reaction is characterized by the binding of antibodies (IgG or IgM) to antigens on the surface of cells or tissues. This leads to activation of the complement system and recruitment of immune cells, causing damage to the affected cells or tissues. Examples of type II hypersensitivity reactions include autoimmune hemolytic anemia, transfusion reactions, and some drug-induced immune reactions.

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  • 11. 

    Complement-mediated immune disorders

    • Type I hypersensitivity reaction

    • Type II hypersensitivity reaction

    • Type III hypersensitivity reaction

    • Type IV hypersensitivity reaction

    Correct Answer
    A. Type III hypersensitivity reaction
    Explanation
    Type III hypersensitivity reaction is characterized by the formation of immune complexes that deposit in tissues, leading to inflammation and tissue damage. This reaction occurs when there is an excess of soluble antigens and antibodies in the bloodstream. The immune complexes can activate complement proteins, leading to an inflammatory response. This type of reaction is associated with autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis.

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  • 12. 

    Acquired later in life

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Secondary Immunodeficiency
    Explanation
    Secondary immunodeficiency refers to a weakened immune system that is acquired later in life, as opposed to being inherited. This can be caused by various factors such as infections, certain medications, chemotherapy, malnutrition, or chronic diseases like HIV/AIDS. Unlike primary immunodeficiency disorders, which are genetic and present from birth, secondary immunodeficiency can develop at any age. It is characterized by a decrease in immune function, making individuals more susceptible to infections and other health complications.

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  • 13. 

    Cause of severe combined immunodeficiency disease

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Adenosine deaminase deficiencies and T-cell cytokine receptor mutations
    Explanation
    Adenosine deaminase deficiencies and T-cell cytokine receptor mutations can cause severe combined immunodeficiency disease. Severe combined immunodeficiency disease (SCID) is a group of rare genetic disorders characterized by a severely compromised immune system. Adenosine deaminase (ADA) deficiencies result in the accumulation of toxic metabolites that damage lymphocytes, leading to a lack of functional immune cells. T-cell cytokine receptor mutations can also impair the development and function of T-cells, which are crucial for immune responses. Both of these genetic abnormalities can result in SCID and its associated immunodeficiency.

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  • 14. 

    Decreases in one or more IgG subgroups

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Immunoglobulin G subclass deficiency
    Explanation
    Immunoglobulin G subclass deficiency refers to a condition where there is a decrease in one or more IgG subgroups. This means that the body is unable to produce or properly function with certain types of IgG antibodies, which are important for immune system function. This deficiency can lead to an increased susceptibility to infections, as the body is unable to effectively fight off pathogens.

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  • 15. 

    Primary cell-mediated disorders of the immune system cause severe problems with infections. Children with these disorders rarely survive beyond childhood without a bone marrow transplant. Which of the following is a disease that involves primary cell-mediated disorders of the immune system?

    • DiGeorge syndrome

    • Y-Linked hyper-IgM syndrome

    • X-Linked agammaglobulinemia

    • Y-linked agammaglobulinemia

    Correct Answer
    A. DiGeorge syndrome
    Explanation
    DiGeorge syndrome is a disease that involves primary cell-mediated disorders of the immune system. It is characterized by the absence or underdevelopment of the thymus, which is responsible for the maturation of T-cells, a type of white blood cell involved in cell-mediated immunity. Without a functional thymus, individuals with DiGeorge syndrome have impaired cell-mediated immune responses, making them more susceptible to severe infections. Therefore, this disease fits the description of a primary cell-mediated disorder of the immune system.

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  • 16. 

    Essentially undetectable levels of all serum immunoglobulins

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. X-linked agammaglobulinemia
    Explanation
    X-linked agammaglobulinemia is a primary immunodeficiency disorder characterized by the absence or very low levels of immunoglobulins, particularly immunoglobulin G (IgG), in the serum. This condition is caused by a mutation in the Bruton's tyrosine kinase (BTK) gene, which is essential for B-cell development and maturation. As a result, individuals with X-linked agammaglobulinemia have impaired B-cell function and are unable to produce normal levels of immunoglobulins. This leads to a significant deficiency in antibody-mediated immune responses, making them susceptible to recurrent infections, particularly of the respiratory tract.

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  • 17. 

    Drug-induced secondary hypogammaglobulinemia are reversible. Which of the following is not a drug that produces hypogammaglobulinemia?

    • Phenytoin

    • Corticosteroids

    • Carbamazepine

    • Disease modifying antirheumatic drugs

    • Interferon-beta 1a drugs

    Correct Answer
    A. Interferon-beta 1a drugs
    Explanation
    Interferon-beta 1a drugs are not known to produce hypogammaglobulinemia. The other options listed, such as phenytoin, corticosteroids, carbamazepine, and disease modifying antirheumatic drugs, have been associated with drug-induced secondary hypogammaglobulinemia.

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  • 18. 

    Combined immunodeficiency syndrome is a disorder in which both B and T lymphocytes are affected. This results in defects in both humoral and cell-mediated immunity. What could be the cause of this disorder?

    • Multiple misplaced genes that influence lymphocyte development and response

    • A single mutation in any gene that influences major histocompatibility antigens

    • A single misplaced gene that influences major histocompatibility

    • Multiple mutations in genes that influence lymphocyte development and response

    Correct Answer
    A. A single mutation in any gene that influences major histocompatibility antigens
    Explanation
    A single mutation in any gene that influences major histocompatibility antigens can cause combined immunodeficiency syndrome. Major histocompatibility antigens play a crucial role in the immune system by presenting antigens to T cells and triggering an immune response. If there is a mutation in any gene that influences these antigens, it can lead to defects in both B and T lymphocytes, resulting in the impairment of both humoral and cell-mediated immunity.

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  • 19. 

    Terminal differentiation of mature B cells to plasma cells is blocked

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Common variable immunodeficiency
    Explanation
    Common variable immunodeficiency is characterized by a failure of B cells to differentiate into plasma cells, resulting in decreased production of immunoglobulins. This leads to a deficiency in antibodies, making individuals more susceptible to infections. Other immunodeficiencies listed in the options may have different mechanisms or manifestations, but only common variable immunodeficiency specifically involves a block in terminal differentiation of B cells to plasma cells.

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  • 20. 

    Susceptible to infections caused by encapsulated microorganisms

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Wiskott-Aldrich syndrome
    Explanation
    Wiskott-Aldrich syndrome is a primary immunodeficiency disorder characterized by a triad of symptoms including eczema, thrombocytopenia (low platelet count), and recurrent infections. It is caused by a mutation in the WAS gene, which leads to defective immune cell function. Individuals with Wiskott-Aldrich syndrome are susceptible to infections caused by encapsulated microorganisms, such as Streptococcus pneumoniae and Haemophilus influenzae, due to impaired antibody production and defective immune response. This susceptibility to infections caused by encapsulated microorganisms is a characteristic feature of Wiskott-Aldrich syndrome.

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  • 21. 

    Repeated bouts of upper respiratory and middle ear infections

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Transient hypogammaglobulinemia of infancy
    Explanation
    Transient hypogammaglobulinemia of infancy is a condition characterized by a temporary decrease in the levels of certain antibodies (immunoglobulins) in the blood. This can lead to repeated bouts of upper respiratory and middle ear infections, as the immune system is not able to effectively fight off infections. However, this condition typically resolves on its own as the child grows older and their immune system matures. Therefore, it is the most appropriate explanation for the given symptoms of repeated infections.

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  • 22. 

    Lymphopenia and a decrease in the ratio of CD4+ helper T cells to CD8+ suppressor T cells

    • DiGeorge Syndrome

    • Secondary Immunodeficiency

    • Hyper-IgM syndrome

    • X-linked agammaglobulinemia

    • Selective IgA deficiency

    • Adenosine deaminase deficiencies and T-cell cytokine receptor mutations

    • Transient hypogammaglobulinemia of infancy

    • Ataxia-telangiectasia

    • Common variable immunodeficiency

    • Immunoglobulin G subclass deficiency

    • Wiskott-Aldrich syndrome

    Correct Answer
    A. Ataxia-telangiectasia
    Explanation
    Ataxia-telangiectasia is a genetic disorder that affects multiple systems in the body, including the immune system. It is characterized by a decrease in the ratio of CD4+ helper T cells to CD8+ suppressor T cells, as well as lymphopenia. These abnormalities in T cell subsets can lead to immune dysfunction and increased susceptibility to infections. Therefore, Ataxia-telangiectasia is the most likely explanation for the given presentation.

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    Quiz Edited by
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