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Pathology Complement system e467e81f

A 25-year-old woman presents with a history of recurrent shoness of breath and severe wheezing. Laboratory studies demonstrate that she has a deficiency of C1 inhibitor, an esterase inhibitor that regulates the activation of the classical complement pathway. What is the diagnosis?

A
Chronic granulomatous disease
B
Hereditary angioedema
C
Myeloperoxidase deficiency
D
Selective IgA deficiency
High-Yield Explanation
=Deficiency of C1 inhibitor, with excessive cleavage of C4 and C2 by C1s, is associated with the syndrome of hereditary angioedema. Feature:- Episodic Painless Non-pitting edema of soft tissues. =Chronic granulomatous disease (choice A) is due to a hereditary deficiency of NADPH oxidase. =Myeloperoxidase deficiency (choice C) increases susceptibility to infections with Candida. =Selective IgA deficiency (choice D) and Wiskott-Aldrich syndrome are congenital immunodeficiency disorders associated with defects in lymphocyte function

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