Complement System Related Disorders Flashcards
1
Q
Hereditary AngioEdema
A
- Defect in C1 esterase inhibitor
- Increased bradykinin—> acute intermittent attacks of skin and mucosal edema (deep dermal swelling)
- screen: consume C4, but not C3!
- can also be caused by ACE inhibitors
- No increased risk of infection
2
Q
C3 deficiency
A
-greater risk of infections
3
Q
Deficiencies in C5-C9
A
-recurrent Neisserial infections
4
Q
Paroxysmal Nocturnal Hemoglobinuria
A
- Defect in glycosylphosphatidylinositol linkages (GPI-linkages)
- decreased DAF (CD55) and protectin (CD59)
- Increased RBC lysis by MAC
- Red urine in morning
5
Q
Complement Related Disorders (name 4)
A
1) Hereditary Angioedema
2) C3 deficiency
3) Deficiency in C5-C9
4) PNH