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
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2
Q

C3 deficiency

A

-greater risk of infections

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3
Q

Deficiencies in C5-C9

A

-recurrent Neisserial infections

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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
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5
Q

Complement Related Disorders (name 4)

A

1) Hereditary Angioedema
2) C3 deficiency
3) Deficiency in C5-C9
4) PNH

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