"We could have our 10-year-old daughter tested, as the ideal stem cell donor is a human leukocyte antigen-identical sibling."Treatment options for SCID include stem cell and bone marrow transplantation. Hematopoietic stem cell transplantation is the definitive therapy for SCID; the best outcome is achieved if the disease is recognized and treated early in life. The ideal donor is a human leukocyte antigen-identical sibling.
  • A nurse is reviewing treatment options with parents of an infant born with severe combined immunodeficiency disease (SCID). The nurse recognizes that the parents understand the teaching based on which statement?
  • Antiretroviral medications should be offered to clients with T-cell counts less than
  • A patient had unprotected sex with an HIV-infected person and arrives in the clinic requesting HIV testing. Results determine a negative HIV antibody test and an increased viral load. What stage does the nurse determine the patient is in?
  • The nurse's base knowledge of primary immunodeficiencies includes which of the following statements?
6 weeksViral load tests are measured immediately before initiating antiretroviral therapy and then again in 2 to 8 weeks. In most clients, adherence to a regimen of potent antiretroviral agents should result in a large decrease in the viral load by 2 to 8 weeks. Therefore, a return visit at 6 weeks would be in this time frame. By 16 to 20 weeks, the viral load should continue to decline, dropping below detectable levels.
  • A client is prescribed didanosine (Videx) as part of his highly active antiretroviral therapy (HAART). Which instruction would the nurse emphasize with this client?
  • A client is presenting an anaphylactic response to unknowingly ingesting nuts at a family celebration. What type of hypersensitivity did this client exhibit?
  • A client is beginning highly active antiretroviral therapy (HAART). The client demonstrates an understanding of the need for follow up when he schedules a return visit for viral load testing at which time?
  • A client is diagnosed with severe combined immunodficiency (SCID). Which of the following would the nurse expect to integrate into the client's plan of care?
Tickle in the throatContinually assess the client for adverse reactions; be especially aware of complaints of a tickle or lump in the throat, which could be the precursor to laryngospasm that precedes bronchoconstriction.
  • A client is presenting an anaphylactic response to unknowingly ingesting nuts at a family celebration. What type of hypersensitivity did this client exhibit?
  • A client is scheduled to receive an intravenous immunoglobulin (IVIG) infusion. The client asks the nurse about the infusion's administration and its adverse effects. Which condition should the nurse instruct this client to report immediately?
  • A client with suspected exposure to HIV has been tested with the enzyme-linked immunosorbent assay (ELISA) with positive results twice. The next step for the nurse to explain to the client for confirmation of the diagnosis is to perform a:
  • A client that is HIV+ has been diagnosed with Pneumocystis pneumonia caused by P. jiroveci. What medication does the nurse expect that the client will take for the treatment of this infection?
HeadacheEarly manifestations of HIV encephalopathy include headache, memory deficits, difficulty concentrating, progressive confusion, psychomotor slowing, apathy, and ataxia. Later stages include hyperreflexia, a vacant stare, and hallucinations.
  • Which condition is an early manifestation of HIV encephalopathy?
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • Which of the following indicates that a client with HIV has developed AIDS?
  • More than 50% of individuals with this disease develop pernicious anemia:
Do not overuse the medication as rebound congestion can occur.Overusing oxymetazoline nasal spray can cause rebound congestion. The medication does not cause fungal infection. Corticosteroids should be tapered, but it is not necessary to taper oxymetazoline. Oxymetazoline does not cause sleepiness so the client can operate machinery or drive.
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
  • A client calls the clinic and asks the nurse if using oxymetazoline nasal spray would be alright to relieve the nasal congestion he is experiencing due to seasonal allergies. What instructions should the nurse provide to the client to avoid complications?
  • A client is scheduled to receive an intravenous immunoglobulin (IVIG) infusion. The client asks the nurse about the infusion's administration and its adverse effects. Which condition should the nurse instruct this client to report immediately?
  • A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. Which of the following would the nurse expect to be started?
Disturbed body image related to loss of fat in the face and armsThe client is experiencing lipoatrophy, which results in a localized loss of subcutaneous fat in the face (manifested as sinking of the cheeks, eyes, and temples), arms, legs, and buttocks. These changes as well as his statement about the side effects of the drug being worse than the disease indicate that he is concerned about how he appears to others. Therefore, the nursing diagnosis of disturbed body image would be the priority. Deficient knowledge, risk for infection, and risk for impaired liver function may be applicable; however, they are not concerns at this time.
  • The nurse's base knowledge of primary immunodeficiencies includes which of the following statements?
  • A nurse is reviewing treatment options with parents of an infant born with severe combined immunodeficiency disease (SCID). The nurse recognizes that the parents understand the teaching based on which statement?
  • A client who is HIV positive is receiving highly active antiretroviral therapy (HAART) that includes a protease inhibitor (PI). The client comes to the clinic for a follow-up visit. Assessment reveals lipoatrophy of the face and arms. The client states, "I'm thinking the side effects of the drug are worse than the disease. Look what's happening to me." The nurse would most likely identify which nursing diagnosis as the priority?
  • Antiretroviral medications should be offered to clients with T-cell counts less than
For their immunosuppressant effectsDrug therapy using anti-inflammatory and immunosuppressive agents is the mainstay for alleviating symptoms. Some antineoplastic (cancer) drugs also are used for their immunosuppressant effects. Antineoplastic drugs do not decrease the body's risk of infection; an autoimmune disease is not a neoplastic disease. Drugs are not ordered just so the client has strong drug therapy.
  • A client with lupus has had antineoplastic drugs prescribed. Why would the physician prescribe antineoplastic drugs for an autoimmune disorder?
  • A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. Which of the following would the nurse expect to be started?
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
  • A client is diagnosed with severe combined immunodficiency (SCID). Which of the following would the nurse expect to integrate into the client's plan of care?
Stool specimen for ova and parasitesA stool specimen for ova and parasites will give a definitive diagnosis. The organism is spread by the fecal-oral route from contaminated water, food, or human or animal waste. Those infected can lose from 10 to 20 L of fluid per day. Losing this magnitude of fluid quickly leads to dehydration and electrolyte imbalances.
  • A client with lupus has had antineoplastic drugs prescribed. Why would the physician prescribe antineoplastic drugs for an autoimmune disorder?
  • A client with suspected exposure to HIV has been tested with the enzyme-linked immunosorbent assay (ELISA) with positive results twice. The next step for the nurse to explain to the client for confirmation of the diagnosis is to perform a:
  • A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. Which of the following would the nurse expect to be started?
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
-Acetaminophen-Diphenhydramine-CorticosteroidBefore administering IVIG, the nurse would premedicate the client with acetaminophen and diphenhydramein as orderded 30 minutes before the start of the infusion. Corticosteroids also may be used to prevent possible severe reactions in clients perceived to be at risk. Odansetron is used to control nausea and vomiting associated with chemotherapy. Ranitidine is used to reduce gastric acid secretion.
  • The nurse is working with a parent whose child has just been diagnosed with selective immunoglobulin A deficiency. The parent asks the nurse, "Does this mean that my child is going to die?" How should the nurse respond?
  • A nurse is preparing to adminster intravenous immunoglobulin (IVIG) to a client. Which of the following would the nurse expect to administer as ordered before initiating the infusion? Select all that apply.
  • The nurse identifies a nursing diagnosis of ineffective airway clearance related to pneumocystis pneumonia and increased bronchial secretions for a client with AIDS. Which of the following would be appropriate for the nurse to include in the client's plan of care?
  • A nurse is working in a support group for clients with acquired immunodeficiency syndrome (AIDS). Which point is most important for the nurse to stress?
Primary immunodeficiencies develop early in life after protection from maternal antibodies decreases.These disorders may involve one or more components of the immune system. Primary immunodeficiencies are seen primarily in infants and young children. Primary immunodeficiencies are rare disorders with genetic origins. Without treatment, infants and children with these disorders seldom survive to adulthood.
  • A client who is HIV positive is receiving highly active antiretroviral therapy (HAART) that includes a protease inhibitor (PI). The client comes to the clinic for a follow-up visit. Assessment reveals lipoatrophy of the face and arms. The client states, "I'm thinking the side effects of the drug are worse than the disease. Look what's happening to me." The nurse would most likely identify which nursing diagnosis as the priority?
  • The nurse's base knowledge of primary immunodeficiencies includes which of the following statements?
  • Antiretroviral medications should be offered to clients with T-cell counts less than
  • A patient had unprotected sex with an HIV-infected person and arrives in the clinic requesting HIV testing. Results determine a negative HIV antibody test and an increased viral load. What stage does the nurse determine the patient is in?
Herpes simplex ulcer persisting for 2 monthsA diagnosis of AIDS cannot be made until the person with HIV meets case criteria established by the Centers for Disease Control and Prevention. The immune system becomes compromised. The CD4 T-cell count drops below 200 cells and develops one of the opportunistic diseases, such as Pneumocystis carinii pneumonia, candidiasis, cytomegalovirus, or herpes simplex.
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • Which of the following indicates that a client with HIV has developed AIDS?
  • The term used to define the amount of virus in the body after the initial immune response subsides is
  • Which condition is an early manifestation of HIV encephalopathy?
Common variable immunodeficiency (CVID)More than 50% of clients with CVID develop pernicious anemia. Pernicious anemia is not associated with the other conditions.
  • More than 50% of individuals with this disease develop pernicious anemia:
  • A client that is HIV+ has been diagnosed with Pneumocystis pneumonia caused by P. jiroveci. What medication does the nurse expect that the client will take for the treatment of this infection?
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • Which of the following indicates that a client with HIV has developed AIDS?
biopsy.KS is diagnosed through biopsy of the suspected lesions. Visual assessment will not confirm a diagnosis. A computed tomography scan will not assist in determining skin cell changes. Skin scraping is a procedure to collect cells, not to evaluate cells.
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • More than 50% of individuals with this disease develop pernicious anemia:
  • Kaposi sarcoma (KS) is diagnosed through
  • During assessment of a patient with Kaposi's sarcoma, the nurse knows to look for the initial sign of:
Deep purple cutaneous lesions.Localized cutaneous lesions may be the first manifestation of this HIV-related malignancy, which appears in 90% of patients as immune function deteriorates. Other symptoms develop over time as the lesions increase in size and spread to other locations.
  • A client receiving antiretroviral therapy reports "not urinating enough." What is the nurse's best action?
  • During assessment of a patient with Kaposi's sarcoma, the nurse knows to look for the initial sign of:
  • A client is prescribed didanosine (Videx) as part of his highly active antiretroviral therapy (HAART). Which instruction would the nurse emphasize with this client?
  • Which of the following indicates that a client with HIV has developed AIDS?
viral set point.The amount of virus in the body after the initial immune response subsides is referred to as the viral set point, which results in an equilibrium between HIV levels and the immune response that may be elicited. During the primary infection period, the window period occurs because a person is infected with HIV but negative on the HIV antibody blood test. The period from infection with HIV to the development of antibodies to HIV is known as the primary infection stage. The amount of virus in circulation and the number of infected cells equals the rate of viral clearance.
  • The term used to define the amount of virus in the body after the initial immune response subsides is
  • Which of the following indicates that a client with HIV has developed AIDS?
  • A client receiving antiretroviral therapy reports "not urinating enough." What is the nurse's best action?
  • When learning about HIV/AIDS, the student should be able to differentiate the two subtypes of virus by which characteristic?
CD4+ countsThe most important consideration in decisions to initiate antiretroviral therapy is CD4+ counts.
  • The term used to define the amount of virus in the body after the initial immune response subsides is
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • During assessment of a patient with Kaposi's sarcoma, the nurse knows to look for the initial sign of:
  • Which is usually the most important consideration in the decision to initiate antiretroviral therapy?
Following safer-sex practicesIt's essential for clients with AIDS to follow safer-sex practices to prevent transmission of the human immunodeficiency virus. Although it's helpful if clients with AIDS avoid using recreational drugs and alcohol, it's more important that I.V. drug users use clean needles and dispose of used needles for purposes of avoiding transmission. Whether the client with AIDS chooses to tell anyone about the diagnosis is his decision; there is no legal obligation to do so.
  • A nurse is working in a support group for clients with acquired immunodeficiency syndrome (AIDS). Which point is most important for the nurse to stress?
  • The nurse is working with a parent whose child has just been diagnosed with selective immunoglobulin A deficiency. The parent asks the nurse, "Does this mean that my child is going to die?" How should the nurse respond?
  • The nurse identifies a nursing diagnosis of ineffective airway clearance related to pneumocystis pneumonia and increased bronchial secretions for a client with AIDS. Which of the following would be appropriate for the nurse to include in the client's plan of care?
  • A nurse is preparing to adminster intravenous immunoglobulin (IVIG) to a client. Which of the following would the nurse expect to administer as ordered before initiating the infusion? Select all that apply.
Immunosuppressive agentsFor a client undergoing a hematopoietic stem cell transplant, immunosuppression is started to ensure engraftment of depleted bone marrow. Antibiotic therapy may or may not be indicated. Chest physiotherapy would be appropriate for clients with ataxia-telangiectasis who have chronic lung disase. Anticoagulation would not be used.
  • A client is prescribed didanosine (Videx) as part of his highly active antiretroviral therapy (HAART). Which instruction would the nurse emphasize with this client?
  • A client with lupus has had antineoplastic drugs prescribed. Why would the physician prescribe antineoplastic drugs for an autoimmune disorder?
  • A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. Which of the following would the nurse expect to be started?
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
-Mature B-cells-Plasma cellsTwo types of inherited B-cell deficiencies exist. The first type results from lack of differentiation of B-cell precursors into mature B cells. As a result, plasma cells are absent, and the germinal centers from all lymphatic tissues disappear, leading to a complete absence of antibody production against invading bacteria, viruses, and other pathogens.
  • The two types of inherited B-cell deficiencies result from lack of differentiation of B cells. These types result from which two of the following deficiencies? Choose the two that apply.
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
  • A client is beginning highly active antiretroviral therapy (HAART). The client demonstrates an understanding of the need for follow up when he schedules a return visit for viral load testing at which time?
  • A client is prescribed didanosine (Videx) as part of his highly active antiretroviral therapy (HAART). Which instruction would the nurse emphasize with this client?
ThrombocytopeniaMajor symptoms of Wiskott-Aldrich syndrome include thrombocytopenia, infections, and malignancies. Ataxia occurs with ataxia-telangiectasia. Episodes of edema in various body parts occur with angioneurotic edema. Bacterial infection occurs with hyperimmunoglobulinemia E syndrome.
  • Which condition is an early manifestation of HIV encephalopathy?
  • Which is a major manifestation of Wiskott-Aldrich syndrome?
  • Which of the following indicates that a client with HIV has developed AIDS?
  • Which is usually the most important consideration in the decision to initiate antiretroviral therapy?
"Be sure to take this drug about 1/2 hour before or 2 hours after you eat."Didanosine should be taken 30 to 60 minutes before or 2 hours after meals. Other antiretroviral agents, such as abacavir, emtricitabine, or lamivudine can be taken without regard to meals. High-fat meals should be avoided when taking amprenavir. Atazanavir should be taken with food and not with antacids.
  • A client is prescribed didanosine (Videx) as part of his highly active antiretroviral therapy (HAART). Which instruction would the nurse emphasize with this client?
  • A client is presenting an anaphylactic response to unknowingly ingesting nuts at a family celebration. What type of hypersensitivity did this client exhibit?
  • A client is beginning highly active antiretroviral therapy (HAART). The client demonstrates an understanding of the need for follow up when he schedules a return visit for viral load testing at which time?
  • A client is diagnosed with severe combined immunodficiency (SCID). Which of the following would the nurse expect to integrate into the client's plan of care?
Western blot test for confirmation of diagnosis.The enzyme-linked immunosorbent assay (ELISA) test, an initial HIV screening test, is positive when there are sufficient HIV antibodies; it also is positive when there are antibodies from other infectious diseases. The test is repeated if results are positive. If results of a second ELISA test are positive, the Western blot is performed. The p24 antigen test and the polymerase chain reaction test determine the viral load, and the T4-cell count is not used for diagnostic confirmation of the presence of HIV in the blood.
  • A client with AIDS is admitted to the hospital with severe diarrhea and dehydration. The physician suspects an infection with Cryptosporidium. What type of specimen should be collected to confirm this diagnosis?
  • A client with lupus has had antineoplastic drugs prescribed. Why would the physician prescribe antineoplastic drugs for an autoimmune disorder?
  • A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. Which of the following would the nurse expect to be started?
  • A client with suspected exposure to HIV has been tested with the enzyme-linked immunosorbent assay (ELISA) with positive results twice. The next step for the nurse to explain to the client for confirmation of the diagnosis is to perform a:
Assist with chest physiotherapy every 2 to 4 hours.The nurse should include interventions such as assisting with and/or performing chest physiotherapy every 2 to 4 hours to prevent stasis of secretions, assist the client to attain the semi- or high Fowler's position to facilitate breathing and airway clearance, allow for frequent rest periods to prevent excessive fatigue, and maintain a fluid intake of at least 3 liters per day unless contraindicated.
  • The nurse identifies a nursing diagnosis of ineffective airway clearance related to pneumocystis pneumonia and increased bronchial secretions for a client with AIDS. Which of the following would be appropriate for the nurse to include in the client's plan of care?
  • The nurse is admitting a client to the unit with a diagnosis of ataxia-telangiectasia. A clinical manifestation is telangiectasia. The nurse would recognize that the client is exhibiting telangiectasia when assessing the presence of what?
  • A nurse is preparing to adminster intravenous immunoglobulin (IVIG) to a client. Which of the following would the nurse expect to administer as ordered before initiating the infusion? Select all that apply.
  • The nurse is working with a parent whose child has just been diagnosed with selective immunoglobulin A deficiency. The parent asks the nurse, "Does this mean that my child is going to die?" How should the nurse respond?
"Your child has a mild genetic immune deficiency caused by a lack of immunoglobulin A, a type of antibody that protects against infections of the lining the mouth and digestive tract."Selective immunoglobulin A deficiency is congenital and characterized by a lack of immunoglobulin A, which predisposes clients to recurrent infections, adverse reactions to blood transfusions or immunoglobulin, autoimmune diseases, and hypothyroidism. There is no current treatment.
  • The nurse is working with a parent whose child has just been diagnosed with selective immunoglobulin A deficiency. The parent asks the nurse, "Does this mean that my child is going to die?" How should the nurse respond?
  • A nurse is preparing to adminster intravenous immunoglobulin (IVIG) to a client. Which of the following would the nurse expect to administer as ordered before initiating the infusion? Select all that apply.
  • A nurse is working in a support group for clients with acquired immunodeficiency syndrome (AIDS). Which point is most important for the nurse to stress?
  • The nurse identifies a nursing diagnosis of ineffective airway clearance related to pneumocystis pneumonia and increased bronchial secretions for a client with AIDS. Which of the following would be appropriate for the nurse to include in the client's plan of care?
HypocalcemiaThe most frequent presenting sign in clients with thymic hypoplasia (DiGeorge syndrome) is hypocalcemia that is resistant to standard therapy. It usually occurs within the first 24 hours of life.
  • A client that is HIV+ has been diagnosed with Pneumocystis pneumonia caused by P. jiroveci. What medication does the nurse expect that the client will take for the treatment of this infection?
  • A client is scheduled to receive an intravenous immunoglobulin (IVIG) infusion. The client asks the nurse about the infusion's administration and its adverse effects. Which condition should the nurse instruct this client to report immediately?
  • A client is presenting an anaphylactic response to unknowingly ingesting nuts at a family celebration. What type of hypersensitivity did this client exhibit?
  • During a third-trimester transabdominal ultrasound, cardiac anomaly and facial abnormalities are noted in the fetus. Further testing reveals that the thymus gland has failed to develop normally, and the fetus is diagnosed with thymic hypoplasia. Based on this diagnosis, the nurse anticipates careful monitoring for which common manifestation during the first 24 hours of life?
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