ATI Hematologic System Related

Review ATI Hematologic System related questions and content

A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?

  • A. Interferon gamma receptor-associated kinase 4
  • B. Inducible RAS activating kinase 4
  • C. Interleukin-1 receptor-associated kinase 4
  • D. Immune response activating kinase 4
Correct Answer: C

Rationale: Step 1: Identify the function of IRAK4.
IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation.

Step 2: Break down the acronym IRAK4.
IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4.

Step 3: Link the information in the question to the correct answer.
Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer.

Summary:
A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4.
B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4.
C: Correct - Matches the function of IRAK4