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Chapter 12-a- Disorders of the Immune Response

UNC - Chapel Hill
Uploaded: 4 years ago
Contributor: dontmove
Category: Nursing
Type: Test / Midterm / Exam
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Filename:   Chapter 12-a- Disorders of the Immune Response.rtf (62.5 kB)
Page Count: 11
Credit Cost: 1
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Page ? Page ? 1. After years of going to different physicians with vague symptoms, a 55-year-old client with a history of Hodgkin disease has been diagnosed with a secondary immunodeficiency syndrome. The client asks the nurse what this means. The nurse knows from the following list of characteristics that secondary immunodeficiency disorders: Select all that apply. A) may be inherited as a sex-linked trait. B) usually develop later in life. C) may be a result of chemotherapy being used to treat a cancer. D) can result from frequent recurring Staphylococcus aureus infections. E) can occur in a chronic obstructive pulmonary disease patient taking corticosteroids daily. Ans: B, C, E Feedback: Secondary immunodeficiency disorders develop later in life as a result of other pathophysiologic states such as malnutrition, disseminated cancers, infection of the cells of the immune system, and treatment with immunosuppressive drugs, such as chemotherapeutic agents. Primary disorders may be congenital or inherited as sex-linked, autosomal dominant, or autosomal recessive traits. Humoral (B-cell) immunodeficiencies are primarily associated with recurrent infections like Staphylococcus aureus. 2. A 2-year-old girl has had repeated ear and upper respiratory infections since she was born. A pediatrician has determined a diagnosis of transient hypogammaglobulinemia of infancy. What is the physiological origin of the child's recurrent infections? A) The child's immune system is unable to synthesize adequate immunoglobulin on its own. B) The child had a congenital absence of IgG antibodies that her body is only slowly beginning to produce independently. C) The child was born with IgA and IgM antibodies, suggesting intrauterine infection. D) The child lacks the antigen-presenting cells integral to normal B-cell antibody production. Ans: A Feedback: Transient hypogammaglobulinemia of infancy is characterized by inadequate communication between B and T cells. IgG would be inherited through placental blood, and an intrauterine infection is neither causative or nor synonymous with transient hypogammaglobulinemia of infancy. The diagnosis does not include a lack of antigen-presenting cells. 3. A nurse is providing care for a 17-year-old boy who has experienced recurrent sinus and chest infections throughout his life and presently has enlarged tonsils and lymph nodes. Blood work indicated normal levels of B cells and free immunoglobulins but a lack of differentiation into normal plasma cells. The boy is currently receiving intravenous immunoglobulin (IVIG) therapy. What is the boy's most likely diagnosis? A) X-linked hypogammaglobulinemia B) Transient hypoglobulinemia C) Common variable immunodeficiency D) IgG subclass deficiency Ans: C Feedback: The lack of a terminal differentiation from B cells into plasma cells is the hallmark of common variable immunodeficiency. Recurrent infections, enlarged lymph nodes and tonsils, and IVIG therapy are also commonly associated. 4. Which of the following patients is most likely to benefit from transplantation of thymic tissue or major histocompatibility complex (MHC)-compatible bone marrow? A) A 12-year-old girl with a history of epilepsy and low IgG levels secondary to phenytoin use B) A 7-year-old boy whose blood work indicates decreased IgA and IgG with increased IgM C) A 6-year-old boy whose pre-B cells are incapable of translation to normal B cells D) A 9-year-old girl who has a diagnosis of IgA deficiency Ans: B Feedback: Decreased IgA, IgE, and IgG with increased IgM levels are characteristic of X-linked immunodeficiency with hyper-IgM, a primary cell-mediated immunodeficiency that would respond to thymic tissue transplantation and MHC-compatible bone marrow. Low IgG levels secondary to phenytoin use, X-linked hypogammaglobulinemia, and selective IgA deficiency are unlikely to be treated with the T-cell?focused treatments like thymic tissue transplantation and MHC-compatible bone marrow. 5. An 8-week-old boy has been recently diagnosed with a severe combined immunodeficiency (SCID). His parents have performed a significant amount of research on the Internet and have brought a large amount of material to discuss with their care provider. Which of the following statements best reflects an accurate understanding of their son's health situation? A) ?We read that gene therapy could cure our son; we'd like you to look into that option.? B) ?Our son likely has a deficiency of B lymphocytes and can't produce antibodies.? C) ?We feel guilty, because dietary and environmental factors have been shown to contribute to SCID? D) ?The antibodies that our son produces are mismatched to the infections that he was born with and encounters.? Ans: B Feedback: The pathophysiology of SCID involves normal B cells but a lack of antibody production because of inadequate T-cell help. Gene therapy is not yet a realistic treatment option, and the disease has a genetic basis. Antibodies are not incorrect but rather inadequate in number. 6. A 1-year-old child who has experienced low platelet counts and bacterial susceptibility has been admitted to a pediatric medical unit of a hospital for treatment of Wiskott-Aldrich syndrome. The nurse who has admitted the child to the unit would anticipate which of the following short-term and longer-term treatment plans? A) Transfusion of clotting factors XII and XIII and serum albumin; splenectomy B) Neutropenic precautions; fresh frozen plasma transfusions; treatment of gastrointestinal symptoms C) Intravenous immunoglobulin (IVIG) treatment; thyroidectomy D) Treatment of eczema; management of bleeding; bone marrow transplant Ans: D Feedback: Common interventions for Wiskott-Aldrich syndrome involve controlling eczema, managing bleeding due to low platelets, and ultimately bone marrow transplant. The other noted interventions are not associated with the treatment of Wiskott-Aldrich syndrome. 7. A nurse has just learned that her child has a life-threatening complement disorder known as hereditary angioneurotic edema (HAE). Due to deficiency in C1-INH, the nurse needs to be prepared for which possible life-threatening clinical manifestation? A) Bulging eyeballs B) Swelling of the airway C) Compressed carotid arteries D) Compression of brachial nerves Ans: B Feedback: HAE is a rare, life-threatening complement disorder that results from deficiency of C1-inhibitor (HAE-C1-INH). It is an inherited autosomal dominant trait that causes mutation in the 11th chromosome. Deficiencies in C1-INH result in uncontrolled release of vasoactive substances that promote vascular permeability. The net result is the development of swelling in the subcutaneous tissues of the extremities, face/torso, or upper airway and GI tract. Laryngeal edema is a life-threatening manifestation that can lead to complete airway obstruction and death without interventions. 8. A 4-year-old boy presents with a chronic cough and swollen lymph nodes. His records show that he has been given antibiotics several times in the past year with limited success, most recently for a liver abscess, and that he also has a recurring fungal skin condition. Which of the following is his most likely diagnosis? A) Selective IgA deficiency B) A deficiency in IgG2 subclass antibodies C) Chronic granulomatous disease D) Ataxia?telangiectasia Ans: C Feedback: Chronic granulomatous disease, because it affects phagocytic function, increases susceptibility to soft tissue infections, particularly of the skin, lungs, lymph nodes, and liver. Selective IgA deficiency and deficient IgG2 subclass antibodies can predispose people to infection, but those infections respond readily to antibiotic treatment. Ataxia?telangiectasia can cause skin and liver problems, but its primary manifestations are ataxia and telangiectasia. 9. A patient diagnosed with a primary immunodeficiency disorder has asked his siblings to be tested as possible stem cell donors. When discussing this procedure with his family, the nurse emphasizes that stem cells can be harvested from: Select all that apply. A) bone marrow. B) peripheral blood. C) skin tissue harvesting. D) mouth swabs. E) tears. Ans: A, B Feedback: Hematopoietic stem cells can be harvested from either the bone marrow or the peripheral blood and also from the umbilical cord blood. From sibling donors, the results are effective with improved survival in approximately 90% of people. 10. When explaining what is occurring when their child has an acute bronchial asthma attack, the nurse will emphasize that which mediator is primarily responsible for the bronchial constriction? A) Tree pollen B) Mold dust C) Histamine D) T-lymphocyte proliferation Ans: C Feedback: Asthma response begins within 5 to 30 minutes of exposure to an allergen. It is mediated by mast cell degranulation and the release of preformed and/or enzymatically activated mediators. These mediators include histamine, serotonin, and acetylcholine. Histamine is the most recognized mediator of type I hypersensitivity reactions and ultimately results in bronchial constriction. 11. A male elementary school student has a severe allergy to peanuts and is displaying the signs of anaphylactic shock after inadvertently eating a peanut-containing candy bar. Which of the following statements best captures the boy's current status and preferred treatment? A) He is experiencing shortness of breath caused by potent vasoconstriction that can be relieved by epinephrine injection. B) He is approaching vascular shock and developing edema due to actions of IgE antibodies, situations that can be reversed by administration of epinephrine. C) His mast cells and basophils have been sensitized, but systemic effects can be mitigated by administration of bronchodilators. D) He is likely in a primary- or initial-stage allergic response that can be relieved by antihistamine administration. Ans: B Feedback: Anaphylactic reactions are often accompanied by vascular shock and edema, and the normal treatment is epinephrine injection. Symptoms are caused by vasodilation, not vasoconstriction; bronchodilators are not the ideal treatment. 12. A 40-year-old woman who experiences severe seasonal allergies has been referred by her family physician to an allergist for weekly allergy injections. The woman is confused as to why repeated exposure to substances that set off her allergies would ultimately benefit her. Which of the following phenomena best captures the rationale for allergy desensitization therapy? A) Repeated exposure to offending allergens binds the basophils and mast cells that mediate the allergic response. B) Allergens in large, regular quantities overwhelm the IgE antibodies that mediate the allergic response. C) Repeated exposure stimulates adrenal production of epinephrine, mitigating the allergic response. D) Injections of allergens simulate production of IgG, combining with the antigens to prevent activation of IgE antibodies. Ans: D Feedback: Repeated exposure to allergens causes an increase in IgG, which binds with antigens before they can stimulate IgE. It does not bind mast cells or basophils, nor does it overwhelm the IgE antibodies to stimulate epinephrine production. 13. A 24-year-old woman has gone to the OB-GYN clinic for her first visit since she found out she was pregnant. The clinician tested her blood type along with the usual prenatal testing. On a follow-up visit, the woman was told that she is Rh negative. When asked what that means for her baby, the nurse explains that Rh-negative women lack RhD antigens on their erythrocytes but produce anti-D antibodies. As a result of this blood type, A) ?If you and your baby have mismatched blood, it can invoke anaphylaxis in the baby.? B) ?If the types are incompatible, severe antibody-mediated inflammation occurs.? C) ?If blood types do not match, the baby's liver will produce extra cells to replace RBCs needed to oxygenate organs.? D) ?If the fetus is Rh positive, maternal anti-D antibodies can coat fetal RBCs resulting in severe anemia.? Ans: D Feedback: In utero, the development of erythroblastosis fetalis (Rh incompatibility) results when Rh-negative women produce anti-D antibodies. In Rh-positive fetus, maternal anti-D antibodies will coat fetal RBCs containing RhD, allowing them to be removed from the fetal circulation by macrophage- and monocyte-mediated phagocytosis (destroying RBCs). RBCs are produced in the bone marrow, not the liver; therefore, answer C is incorrect. 14. A 67-year-old patient diagnosed with myasthenia gravis will likely display which clinical manifestations as a result of autoantibodies ultimately blocking the action of acetylcholine, resulting in destruction of the receptors? A) Weakness of the eye muscles; difficulty in swallowing and slurred speech; impaired gait B) Tremor of hands/arms; rigidity of the arms; shuffling gait C) Short-term memory lapses; problems with orientation; a lack of drive or initiative D) Facial droop; slurred speech; weakness on one side of the body Ans: A Feedback: Myasthenia gravis ultimately results in destruction of receptors in the neuromuscular junction leading to a decrease in neuromuscular function. Answer B relates to s/s of Parkinson disease; answer C relates to Huntington disease; and answer D relates to classic s/s of CVA. 15. Following a spider bite she received while camping, a 20-year-old female presented to the emergency department with rash, edema, and fever and was subsequently diagnosed with serum sickness. Which of the following statements best conveys the physiological rationale for the broad systemic effects of this event? A) The woman is experiencing diffuse tissue necrosis as a consequence of an Arthus reaction. B) Antigen?antibody complexes have been deposited in a variety of locations throughout the body. C) Antibody binding to specific target cell receptors is bringing about a change in cell function. D) Deposited antibodies are activating her complement system. Ans: B Feedback: Serum sickness is characterized by the deposition of antigen?antibody complexes in blood vessels, joints, heart, and kidney tissue. The deposited complexes activate complement, increase vascular permeability, and recruit phagocytic cells, all of which can promote focal tissue damage and edema. Serum sickness is not synonymous with an Arthus reaction. Antibody binding to specific target cell receptors causing a change in cell function is characteristic of antibody-mediated cellular dysfunction. Serum sickness is not associated with the activation of the complement system. 16. A new nursing student is taking a tuberculin (TB) skin test as part of her preparation for beginning clinical placement in the hospital. The student is unclear of the rationale or physiology involved in this test. Which of the following is the correct explanation? A) The cell-mediated hypersensitivity associated with Mycobacterium tuberculosis remains detectable for several years. B) Formation of contact dermatitis lesions confirms prior TB contact. C) Previous TB exposure forms sensitized Th1 cells that are long-lived. D) This type of delayed-type hypersensitivity (DTH) is a response to latent Mycobacterium tuberculosis bacteria. Ans: C Feedback: Sensitized Th1 cells form DTH response to introduced antigens. This is not an example of cell-mediated hypersensitivity or contact dermatitis, and it is not a response to latent Mycobacterium tuberculosis. 17. A female dental assistant has developed signs and symptoms of a latex sensitivity and is undergoing allergy testing as well as blood work. Which of the following components of the assistant's blood work would most likely be the focus of her health care provider's analysis? A) Analysis of class II MHC antigens B) Serum IgE immunoassays C) Serum B-lymphocyte levels D) Serum CD8+ levels Ans: B Feedback: Latex sensitivity can be either a type I or type IV reaction. Though Th1 levels are relevant in a type IV reaction, IgE analysis is the most common component of relevant blood work. MHC and CD8+ levels are unlikely to be considered. 18. While undergoing a kidney transplant from a nonfamily member, the patient's transplanted kidney has just had the arterial clamps removed. The OR staff notice that the organ is turning purple with no urine output. When explaining to the family why they had to remove the donor kidney, the nurse will anticipate that the surgeon would likely include which statement? A) Obviously, there has been a mismatch during the human leukocyte antigen (HLA) testing. B) The circulating B and T lymphocytes are just doing their job. C) Hyperacute rejection occurs because antibodies against HLA antigens are deposited in vessels causing necrosis. D) Previous exposure to the HLA antigens is responsible for the high titers of complement fixing antibodies that cause the rejection. Ans: C Feedback: Antibody-mediated rejection can be hyperacute, which occurs almost immediately after vascular reperfusion to graft tissue occurs. Performed antibodies against HLA antigens are deposited in the tissue endothelium and microvasculature where they activate the classic complement pathway causing tissue necrosis and graft injury. 19. After several months on a waiting list, a 44-year-old male received a liver transplant 5 days ago. In the last 36 hours, he has developed a rash beginning on his palms and soles, along with abdominal pain and nausea. It has been determined by his care team that the immune response that is causing his symptoms originates not with his own compromised immune components but with those introduced with his new organ. This man's most likely medical diagnosis is A) graft versus host disease (GVHD). B) acute transplant rejection. C) hyperacute organ rejection. D) T-cell?mediated graft rejection. Ans: A Feedback: Rash, gastrointestinal involvement, and pernicious activity by donor immune cells are the hallmarks of GVHD. The description does not suggest acute or hyperacute transplant rejection, and T-cell?mediated rejection is not a diagnosis in and of itself, but rather one of the mechanisms of transplant rejection. 20. In the context of a workshop on rheumatoid arthritis, a clinical educator is teaching a group of nurses about autoimmune diseases. Which of the following statements by an attendee would the educator most likely want to follow up with further teaching? A) ?Introduction of a foreign antigen can sometimes induce a cascade of immune response that is not self-limiting? B) ?Often the problem can be traced to antigens that sensitize T cells without the need for presentation.? C) ?In some cases, the body attacks its own cells that are chemically similar to those of infectious organisms.? D) ?Sometimes when the body's own cells are released after a long time, they are interpreted as being foreign.? Ans: A Feedback: Autoimmune responses are not considered to be uncontrolled cascades of immune response that are catalyzed by introduction of an antigen. Superantigens are able to forego the normal antigen presentation process and directly stimulate T-cell response, resulting in overactivation of T cells. Molecular mimicry involves the misidentification of somatic cells as similar foreign cells, and self-antigens that have been sequestered for long periods can invoke an immune response.

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