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25 Cards in this Set
- Front
- Back
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1. What is immunosuppression suggested by in a child?
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a. Failure to thrive
b. Atypical or difficult-to-eradicate infections (recurring otitis refractory to multiple antimicrobials). |
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2. What is Primary (Syndromic) immunodeficiency due to?
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a. A genetic defect, either inherited or related to a gene mutation.
b. Most are humoral in origin or characterised by both humoral and cellular dysfunction (severe combined immunodeficiency) |
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3. What is Chronic granulomatous disease due to?
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a. Defective macrophages.
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4. Secondary immunodeficiencies?
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a. Pts w/2° immunodeficiency have normal immune function at birth, but subsequently develop an illness or metabolic abnormality that disrupts immune cell production or function.
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5. Conditions adversely affecting a pt’s immune status?
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1. HIV
2. DM 3. Malnutrition 4. Hepatic disease 5. Autoimmune disease (scleroderma) 6. Aging 7. Stress |
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6. Rx for pregnant mothers?
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a. Zidovudine should be started by the mother during the second Trimester and given to the baby through age 6 weeks.
b. This reduces the rate of transmission from 25% to <10%. |
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7. Dx of HIV infection in pts older than 18 months?
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a. ELISA and Western Blot.
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8. Dx of HIV infection in pts <18 months?
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a. HIV DNA PCR bc of placental transfer of maternal antibodies.
b. 2 Assays are performed on separate occasions to confirm the dx. c. Neonates born to affected mothers are tested at birth and at selected intervals through approximately 6 months of age. |
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9. Tx of exposed neonate?
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a. Traditionally, the exposed neonate receives 6 weeks of antiretroviral therapy in the form of ZDV starting in the first few hours of life.
b. PCP prophylaxis in the form of TMP-SMX commences at approximately 6 weeks of age for HIV-positive infants. |
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10. 3 nucleoside reverse transcriptase inhibitors?
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1. Didanosine
2. Stavudine 3. Zidovudine |
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11. 2 non-nucleoside reverse transcriptase inhibitors?
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1. Efavirenz
2. Nevirapine |
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12. 2 protease inhibitors?
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1. Indinavir
2. Nelfinavir |
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13. Common adverse SE for all antiretroviral drugs?
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1. HA
2. Abdominal pain 3. Diarrhea b. Osteopenia and drug rash can also be seen. |
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14. Possible other abnormalities w/antiretroviral drugs?
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a. Anaemia
b. Neutropenia c. Elevated transaminases d. Hyperglycemia e. Hyperlipidemia |
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15. What vaccines are often excluded in HIV?
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a. The live vaccines:
1. MMR 2. Varicella |
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16. How is Hyperglycemia linked to 2° immunodeficiency?
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a. In diabetes, hyperglycemia promotes neutrophil dysfunction, and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infection.
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17. Presentation Leukocyte Adhesion Deficiency (LAD)?
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a. LAD is an inheritable disorder of leukocyte chemotaxis and adherence characterized by recurring sinopulmonary, oropharyngeal, and cutaneous infections w/delayed wound healing.
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18. Labs with Leukocyte Adhesion Deficiency (LAD)?
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a. Neutrophilia is common w/WBC counts of typically >50,000.
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19. What bacteria are people w/ Leukocyte Adhesion Deficiency (LAD) susceptible to?
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1. Staph species
2. Enterobacteriaceae 3. Candida b. Good skin and oral hygiene are important c. Broad-spectrum abx and surgical debridement are early considerations w/infection. |
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20. 2 types of inheritance of SCID?
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1. AR
2. X-linked. |
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21. Labs for SCID
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a. Effects both humoral and cellular immunity.
b. Serum Ig and T cells are often markedly diminished or absent. c. Thymic dysgenesis is also seen. |
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22. Presentation for SCID?
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a. Recurring cutaneous, GI, or pulmonary infections occur w/opportunistic organisms such as CMV and PCP.
b. Death typically occurs in first 12-24 months of life unless bone marrow transplantation is performed. |
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23. What mutation causes DiGeorge syndrome?
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a. 22q11 microdeletion.
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24. Pathophys of Digeorge?
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a. Decreased T-cell production with resultant recurring infections.
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25. Findings in Digeorge syndrome?
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a. Characteristic facies
b. Velocardiofacial defects such as VSD and tetralogy of Fallot. c. Thymic and parathyroid dysgenesis can also occur accompanied by hypocalcemia and seizures. d. Developmental and speech delay are common in older pts. |
