Case Report
Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis
Table 1
Summary of demographic, clinical, and complement levels in the index patient and relatives (ND: not determined).
| | II.1 (index) | I.2 | II.2 | II.3 |
| Age | 19 years | 38 years | 18 years | 11 years | Infections | Meningococcal sepsis | — | — | — | Classical complement pathway activity (%, 66–113) | <12.5 | 98.1 | <12.5 | <12.5 | Alternative complement pathway activity (%, 20–60) | 0 | 24 | 0 | 0 | C3 (7.9–15.2 mg/dl) | 18.1 | ND | 10.7 | 14.8 | C4 (1.6–3.8 mg/dl) | 3.8 | ND | 1.3 | 2.2 | C5 (1.1–25 mg/dl) | 34.5 | ND | ND | ND | C6 (>4.5 mg/dl) | <0.5 | ND | <0.5 | <0.5 | C7 (>5.5 mg/dl) | 8.9 | ND | ND | ND | C8 (>11.2 mg/dl) | 19.4 | ND | ND | ND | C9 (>12.5 mg/dl) | 17.5 | ND | ND | ND |
|
|