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