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1. A patient with a primary immunodeficiency (PID) affecting adaptive immunity most likely has a deficiency in which immune system component?

A. Complement B. Neutrophils C. Macrophages D. B cells E. Unsure

2. What is a typical starting dose of intravenous immune globulin (IVIG) for most patients with PID?

A. 100 to 150 mg every 4 weeks B. 100 to 150 mg/kg every week C. 400 to 600 mg every 8 weeks D. 400 to 600 mg/kg every 4 weeks E. Unsure

3. What is an acceptable initial target IgG trough level for a patient with common variable immune deficiency (CVID) and a baseline trough of 372 mg/dL?

A. <300 mg/dL B. 400 to 500 mg/dL C. >800 mg/dL D. >1500 mg/dL E. Unsure

4. GJ is a 12-month-old male recently experiencing bruising, thrombocytopenia, and several infections. He was initially diagnosed with thrombocytopenic purpura. What PID should be considered in this patient?

A. Agammaglobulinemia B. Selective IgA deficiency (SIGAD) C. Wiskott-Aldrich syndrome (WAS) D. Severe combined immunodeficiency (SCID) E. Unsure

5. DG is a 7-year-old boy with SIGAD and no other pertinent medical history. He is to begin IVIG therapy after recurrent infection despite prophylactic antibiotics. Among products with the following characteristics, which is most appropriate for DG?

A. Lyophilized IVIG; osmolality 630 mOsm/L; IgA content <1 mcg/mL B. Lyophilized IVIG; osmolality 1074 mOsm/L; IgA content 720 mcg/mL C. 5% liquid IVIG; osmolality 380 mOsm/L; IgA content <100 mcg/mL D. 10% liquid IVIG; osmolality 380 mOsm/L; IgA content <100 mcg/mL E. Unsure

6. Which patient currently treated with IVIG is the most appropriate candidate for a switch to subcutaneous immune globulin (SCIG)?

A. A patient with kidney disease and wear-off symptoms B. A patient who experiences headache with IVIG administration C. A patient with WAS D. A patient with elevated body mass index (BMI) E. Unsure

7. A patient with CVID is transferring care to your clinic in the fall. This individual has received IVIG infusions every 4 weeks and have maintained a target trough IgG level. What is the most appropriate action to mitigate risk of seasonal influenza?

A. Increase IgG target trough level until spring B. Convert to SCIG therapy until spring C. Ensure the patient receives inactivated influenza vaccine D. No action; the patient is adequately protected from antibodies received from IVIG E. Unsure

8. What is an appropriate option for antimicrobial prophylaxis in a patient with chronic granulomatous disease (CGD)?

A. Interferon gamma-1b monotherapy B. Trimethoprim/sulfamethoxazole (TMP/SMX) monotherapy C. TMP/SMX + itraconazole combination D. Acyclovir + interferon gamma-1b combination E. Unsure

9. A patient newly diagnosed with WAS asks for your recommendations regarding immunization. Which vaccine should this patient be counseled to avoid?

A. Quadrivalent inactivated influenza vaccine B. Measles, mumps, and rubella (MMR) vaccine C. Hepatitis A vaccine D. Diphtheria, tetanus, and acellular pertussis vaccine E. Unsure

10. Which counseling point is most appropriate for a patient newly initiating SCIG therapy?

A. Premedicate with acetaminophen prior to infusion of SCIG B. Inactivated vaccines should be avoided during SCIG therapy C. Seek emergency care if infusion site reactions occur D. Rotate infusion sites between infusions E. Unsure

Maintenance is scheduled for 05/16/2024 5:00 PM EST - 05/17/2024 5:00 AM EST.

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1. A patient with a primary immunodeficiency (PID) affecting adaptive immunity most likely has a deficiency in which immune system component?

2. What is a typical starting dose of intravenous immune globulin (IVIG) for most patients with PID?

3. What is an acceptable initial target IgG trough level for a patient with common variable immune deficiency (CVID) and a baseline trough of 372 mg/dL?

4. GJ is a 12-month-old male recently experiencing bruising, thrombocytopenia, and several infections. He was initially diagnosed with thrombocytopenic purpura. What PID should be considered in this patient?

5. DG is a 7-year-old boy with SIGAD and no other pertinent medical history. He is to begin IVIG therapy after recurrent infection despite prophylactic antibiotics. Among products with the following characteristics, which is most appropriate for DG?

6. Which patient currently treated with IVIG is the most appropriate candidate for a switch to subcutaneous immune globulin (SCIG)?

7. A patient with CVID is transferring care to your clinic in the fall. This individual has received IVIG infusions every 4 weeks and have maintained a target trough IgG level. What is the most appropriate action to mitigate risk of seasonal influenza?

8. What is an appropriate option for antimicrobial prophylaxis in a patient with chronic granulomatous disease (CGD)?

9. A patient newly diagnosed with WAS asks for your recommendations regarding immunization. Which vaccine should this patient be counseled to avoid?

10. Which counseling point is most appropriate for a patient newly initiating SCIG therapy?

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Maintenance is scheduled for 05/16/2024 5:00 PM EST - 05/17/2024 5:00 AM EST.

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1. A patient with a primary immunodeficiency (PID) affecting adaptive immunity most likely has a deficiency in which immune system component?

2. What is a typical starting dose of intravenous immune globulin (IVIG) for most patients with PID?

3. What is an acceptable initial target IgG trough level for a patient with common variable immune deficiency (CVID) and a baseline trough of 372 mg/dL?

4. GJ is a 12-month-old male recently experiencing bruising, thrombocytopenia, and several infections. He was initially diagnosed with thrombocytopenic purpura. What PID should be considered in this patient?

5. DG is a 7-year-old boy with SIGAD and no other pertinent medical history. He is to begin IVIG therapy after recurrent infection despite prophylactic antibiotics. Among products with the following characteristics, which is most appropriate for DG?

6. Which patient currently treated with IVIG is the most appropriate candidate for a switch to subcutaneous immune globulin (SCIG)?

7. A patient with CVID is transferring care to your clinic in the fall. This individual has received IVIG infusions every 4 weeks and have maintained a target trough IgG level. What is the most appropriate action to mitigate risk of seasonal influenza?

8. What is an appropriate option for antimicrobial prophylaxis in a patient with chronic granulomatous disease (CGD)?

9. A patient newly diagnosed with WAS asks for your recommendations regarding immunization. Which vaccine should this patient be counseled to avoid?

10. Which counseling point is most appropriate for a patient newly initiating SCIG therapy?

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