Table 2.

IMD cases with immune dysfunction

Patient, age, sex, region	Cause of immune dysfunction	Infecting serogroup	Clinical presentation/complications	Outcome
17-mo, female, Spain [123]	Complement C5 deficiency	E	Meningococcal sepsis	Not reported
17-mo, female, Italy [124]	Complement C8 deficiency	B	Sepsis	Recovery
23-mo, female, USA [125]	Complement deficiency	Non-groupable	Septic shock	Recovery
34-mo, male, Spain [123]	Complement C5 deficiency	Y	IMD	Recovery
4-yo, female, Italy [124]	Complement C8 deficiency	Not reported	Sepsis	Recovery
5-yo, female, Australia [38]	Complement C7 deficiency	E	Meningitis	Not reported
6-yo, female, Portugal [87]	Complement C5 deficiency	B	Septic shock	Recovery
6-yo, female,a Netherlands [114]	Complement C8 deficiency	Z, C, then non-typable	Recurrent meningococcemia (3 episodes)	Recovery
7-yo, female, Spain [123]	Complement C5 deficiency	Non-groupable	Recurrent IMD (2 episodes)	Not reported
9-yo, female,b Spain [28]	Eculizumab treatment	B	IMD, meningitis	Recovery
12-yo, female, France [76]	Complement C6 deficiency	W	Purpura fulminans	Recovery
15-yo, male, Italy [124]	Complement C8 deficiency	Non-groupable	Sepsis	Recovery
16-yo, female, USA [92]	Eculizumab treatment	Non-groupable	Waterhouse–Friderichsen syndrome	Death
18-yo, male, Belgium [121]	Complement C6 deficiency	Y	Recurrent meningitis (2 episodes), shock, purpura fulminans	Limb amputation
19-yo, male, Brazil [64]	HIV, detectable viral load	C	Chronic meningococcemia, sepsis	Recovery

HIV human immunodeficiency virus, IMD invasive meningococcal disease, mo month-old, yo year-old

^aThe patient had complement C8 deficiency and experienced three episodes of meningococcemia over ≥ 2.5 years. She had discontinued amoxicillin prophylaxis 6 months before the third episode

^bThe patient was receiving eculizumab, prednisone, and mycophenolate mofetil for hemolytic uremic syndrome