W8 Lec [Sub] - Deficiencies of Complement Components Flashcards

1
Q

C1 (q, r, or s)

A

Lupuslike syndrome; recurrent infections

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2
Q

C2

A

Lupuslike syndrome; recurrent infections; atherosclerosis

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3
Q

C3

A

Severe recurrent infections; glomerulonephritis

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4
Q

C4

A

Lupuslike syndrome

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5
Q

C5-C8

A

systemic Neisseria infections

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6
Q

C9

A

No known disease association

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7
Q

C1INH

A

Hereditary angioedema

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8
Q

DAF/decay accelerating factor

A

Paroxysmal nocturnal hemoglobinuria

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9
Q

MIRL/membrane inhibitor of reactive lysis

A

Paroxysmal nocturnal hemoglobinuria

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10
Q

Factor H or Factor I

A

Recurrent pyogenic infections

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11
Q

MBL/mannose-binding lectin

A

Pneumococcal diseases, sepsis, Neisseria infections, and meningococcal diseases

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12
Q

Properdin

A

Neisseria infections

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13
Q

MASP-2/mannose-associated serine protease 2

A

Pneumococcal diseases

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14
Q

Associated with Lupuslike syndrome

A
  • C1 (q, r, or s)
  • C2
  • C4
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15
Q

Associated with recurrent infections

A
  • C1 (q, r, or s)
  • C2
  • C3 (severe)
  • Factor H or I (pyogenic)
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16
Q

atherosclerosis

A

C2

17
Q

glomerulonephritis

A

C3

18
Q

Associated with Neisseria infections

A
  • C5-C8
  • MBL
  • Properdin
19
Q

hereditary angioedema

A

C1INH

20
Q

Associated with Paroxysmal nocturnal hemoglobinuria

A
  • DAF
  • MIRL
21
Q

Associated with pneumococcal diseases

A
  • MBL
  • MASP-2
22
Q

sepsis

A

MBL

23
Q

Most common complement deficiency?

A

C2, found in 1/20,000 individuals

24
Q

C2-deficient individuals are prone to recurrence of?

A

staphylococcal and streptococcal infections

25
Q

C2-deficient individuals have low levels of?

A

Factor B because their gene location or alleles are close to each other

26
Q

Most serious form of complement deficiency?

A

C3, because they are prone to severe recurrent life-threatening infections with encapsulated bacteria such as Streptococcus pneumoniae.