Complement deficiencies Flashcards
1
Q
Actions of complement system
A
chemotaxis, cell lysis and opsonisation
2
Q
C1 inhibitor (C1-INH) protein deficiency
A
causes hereditary angioedema
C1-INH is a multifunctional serine protease inhibitor
probable mechanism is uncontrolled release of bradykinin resulting in oedema of tissues
3
Q
C1q, C1rs, C2, C4 deficiency (classical pathway components)
A
predisposes to immune complex disease
e.g. SLE, Henoch-Schonlein Purpura
4
Q
C3 deficiency
A
causes recurrent bacterial infections
5
Q
C5 deficiency
A
predisposes to Leiner disease
recurrent diarrhoea, wasting and seborrhoeic dermatitis
6
Q
C5-9 deficiency
A
encodes the membrane attack complex (MAC)
particularly prone to Neisseria meningitidis infection
7
Q
A