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Table 2 Clinical and laboratory characteristics of primary complement deficiency patients

From: Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies

Pt

Agea (yrs)/Sex

Comp

Def

Laboratory results

Infections history (SBI, n)

1

3 / M

C2

Undetectable CH50, ↓ C2 level and function; C1q, C1r, C1s levels normal

Pneumococcal pneumonia, E. coli bacteremia, otitis media; recurrent acute otitis media (SBI = 2)

2

12 / M

C6

Family history of C6 deficiency, undetectable CH50, ↓ C6 level; (C5, C7-C9 levels normal).

Neisseria meningitidis meningitis and bacteremia (SBI = 1)

3

4 / M

C2

Undetectable CH50, undetectable C2 level; (C1, C3- C9 levels normal)

Two episodes of Streptococcal bacteremia, one episode of streptococcal meningitis, recurrent sinusitis (SBI = 3)

4

15 / M

C6

Undetectable CH50, ↓ C6 level; (C3, C5, C7-C9 levels normal)

Neisseria meningitidis meningitis and bacteremia (SBI = 1)

5

11 / M

C1q

Undetectable CH50, ↓ C1 level and function, ↓ C1q, (C1r, C1s, C2-C9 levels and function normal)

Streptococcal pneumoniae bacteremia and meningitis (SBI = 1)

6

10 / F

C2

Undetectable CH50, undetectable C2 level; (C3-C9 levels normal)

Neisseria meningitidis meningitis, recurrent otitis media (SBI = 1)

7

21 / M

C8

↓ CH50, undetectable AH50, ↓ C8 level, (C2-C7, C9 levels, properdin level normal)

Two episodes of Neisseria meningitidis meningitis, recurrent otitis media (SBI = 2)

8

6 / M

C1q

Undetectable CH50, ↓ C1q level; (AH50, C1r, C1s, C2, C4 levels normal)

Streptococcal bacteremia

(SBI = 1)

9b

5 / M

C2

Family history of C2 deficiency, undetectable CH50, ↓ C2 level

None (Sjogren’s syndrome)

(SBI = 0)

10b

4 / F

C1q

Family history of C1 deficiency, undetectable CH50, low C1q level

None (Discoid lupus)

(SBI = 0)

11

3 / M

C2

Undetectable CH50, ↓ C2 level and function

Group B streptococcal meningitis and bacteremia; Streptococcal pneumoniae bacteremia, otitis media (SBI = 2)

12

18 / F

C6

Family history of C6 deficiency, undetectable CH50 and AH50, undetectable C6 function

None (erythromelalgia)

(SBI = 0)

13

20 / F

C2

Undetectable CH50, previous physician diagnosis.

Recurrent otitis media and sinusitis; recurrent UTI, pyelonephritis (SBI = 2)

14

18 / M

C2

Undetectable CH50, undetectable C2 level and function; (AH50, C1, C4 levels normal)

Recurrent otitis media and sinusitis; multifocal bacterial pneumonia, (SBI = 2)

15

17 / M

C2

Undetectable CH50, undetectable C2 level and function; (C1, C3-C9 levels normal)

Streptococcus pneumoniae meningitis (SBI = 2)

16

13 / M

C2

Undetectable CH50, undetectable C2 level and function; (C1, C3-C9 levels normal)

Streptococcal bacteremia, Hemophilus influenza pneumonia (SBI = 2)

17b

18 / F

C2

Undetectable CH50, undetectable C2 level and function; (C3, C4 levels normal)

Neisseria meningitidis septic arthritis, spontaneous bacterial peritonitis (SBI = 2)

18

5 / M

C2

Undetectable CH50, undetectable C2 level and function; (C1, C3, C4 levels normal)

Recurrent episodes of Streptococcus pneumoniae bacteremia (SBI = 6)

  1. ↓, low; NL, normal
  2. a Age is calculated at the time of last clinical evaluation
  3. b Patients on hydroxychloroquine