If a person had a genetic defect affecting perforin production, which cells and immune function would be affected?

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Pediatric Immune Disorder Test Questions Questions

Question 1 of 5

If a person had a genetic defect affecting perforin production, which cells and immune function would be affected?

Correct Answer: A

Rationale: Perforin is critical for cytotoxic T cells and NK cells to kill target cells.

Question 2 of 5

The MOST appropriate method to correct the immune deficiency in complete DiGeorge syndrome is

Correct Answer: D

Rationale: Complete DiGeorge syndrome lacks T-cell function due to absent thymus; thymic transplantation restores T-cell immunity, addressing the root cause.

Question 3 of 5

Severe neutropenia is defined as absolute neutrophil count (ANC) less than

Correct Answer: A

Rationale: Severe neutropenia is defined as ANC <500/μL, increasing infection risk significantly.

Question 4 of 5

Leukemoid reactions (WBC count >50,000/μL) are caused by all the following EXCEPT

Correct Answer: D

Rationale: Chronic granulomatous disease causes defective neutrophil function, not leukemoid reactions, which are triggered by severe infections like shigellosis.

Question 5 of 5

All the following matching about genetic deficiencies of plasma complement components and associated clinical findings are true EXCEPT

Correct Answer: A

Rationale: C4 deficiency is more strongly linked to systemic lupus erythematosus than discoid lupus, making this pairing less accurate.

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