W8 Lec [Sub] - Deficiencies of Complement Components Flashcards
C1 (q, r, or s)
Lupuslike syndrome; recurrent infections
C2
Lupuslike syndrome; recurrent infections; atherosclerosis
C3
Severe recurrent infections; glomerulonephritis
C4
Lupuslike syndrome
C5-C8
systemic Neisseria infections
C9
No known disease association
C1INH
Hereditary angioedema
DAF/decay accelerating factor
Paroxysmal nocturnal hemoglobinuria
MIRL/membrane inhibitor of reactive lysis
Paroxysmal nocturnal hemoglobinuria
Factor H or Factor I
Recurrent pyogenic infections
MBL/mannose-binding lectin
Pneumococcal diseases, sepsis, Neisseria infections, and meningococcal diseases
Properdin
Neisseria infections
MASP-2/mannose-associated serine protease 2
Pneumococcal diseases
Associated with Lupuslike syndrome
- C1 (q, r, or s)
- C2
- C4
Associated with recurrent infections
- C1 (q, r, or s)
- C2
- C3 (severe)
- Factor H or I (pyogenic)
atherosclerosis
C2
glomerulonephritis
C3
Associated with Neisseria infections
- C5-C8
- MBL
- Properdin
hereditary angioedema
C1INH
Associated with Paroxysmal nocturnal hemoglobinuria
- DAF
- MIRL
Associated with pneumococcal diseases
- MBL
- MASP-2
sepsis
MBL
Most common complement deficiency?
C2, found in 1/20,000 individuals
C2-deficient individuals are prone to recurrence of?
staphylococcal and streptococcal infections
C2-deficient individuals have low levels of?
Factor B because their gene location or alleles are close to each other
Most serious form of complement deficiency?
C3, because they are prone to severe recurrent life-threatening infections with encapsulated bacteria such as Streptococcus pneumoniae.
