ACAAI Review Chapter 6 Exam with Questions and Answers

ACAAI Review Chapter 6 Exam with Questions and Answers Which complement component is decreased in acquired, but not hereditary, angioedema? ANSWER C1q Which medication approved for treatment of acute attacks of HAE carries a black box warning for anaphylaxis? ANSWER Ecallantide (Kalbitor) What is the most common primary immunodeficiency disorder? ANSWER Selective IgA deficiency What is the lymphocyte phenotype (T/B/NK) with SCID secondary to a defect secondary to Artemis? ANSWER T-B-NK+ What gene defect can lead to chronic mucocutaneous candidiasis? Other associated clinical features? ANSWER AIRE gene resulting in autoimmune type symptoms, including endocrinopathies, hypoparathyroidism, hypoadrenalism What is the mutation that causes X-linked agammaglobulinemia? Clinically describe the lymphoid tissue present? ANSWER BTK; there is absent or limited lymphoid tissue secondary to no germinal centers What is the molecular defect in WHIM syndrome? ANSWER CXCR4, which is needed in bone marrow trafficking of progenitor cells What are two prophylactic antibiotic options for IRAK4/MyD88 deficiency? ANSWER TMP-SMX and penicillin V