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Table 1 Characteristics of patients with complement deficiency who developed invasive meningococcal disease in England

From: Invasive meningococcal disease in patients with complement deficiencies: a case series (2008–2017)

Case Month + year of IMD Age (years) Detection method Capsular group PorA Pen MIC Pen CtrA cycle number ACWY Dose 1 ACWY Dose 2 4CMenB Dose 1 4CMenB Dose 2 Eculizumab Underlying Condition Antibiotic Prophylaxis
1 Jun 2011 9 Culture & PCR Y P1.5–1,10–1 0.125 S 20      No Factor D Penicillin after IMD
2a May 2002 15 Culture & PCR B N 0.04 S 26 2011     No C6 deficiency No, rescue Amoxycillin
2b Mar 2011 24 Culture & PCR Y P1.5–1,10–1 0.047 S 31
3a Oct 1989 20 Culture only Y NTa 0.04 S   2008     No C8 deficiency Amoxycillin
3b Nov 2011 42 Culture only B P1.5–2,10–1 0.047 S  
4 Jun 2017 15 PCR only NGb No product NA NA 34      No   No
5a Oct 2005 4 PCR only B ND NA NA 31 2016     No C6 deficiency No
5b Mar 2018 16 Culture & PCR Y P1.5–1,10–1 0.064 S 36
6 Mar 2017 12 PCR Y P1.5–2,10–2 NA NA 30      No C7 deficiency No
7 Mar 2013 17 Culture only Y P1.5–1,10–1 0.012 S       No Hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis) on immunosuppression therapy (diagnosed in 2010) No
8 Apr 2012 15 PCR only Y P1.5–1,10–1 NA NA 28 2013   Oct 2014 Nov 2014 No focal glomerulonephritis with persistently low complement C3/C4 (diagnosed in 2012) Penicillin
9 Oct 2008 20 Culture & PCR B P1.17,16–3 0.047 S 34 Jul 2008 Dec 2011 Jul 2014 Aug 2014 Yes PNH Penicillin
10 Sep 2011 20 Culture only B P1.22,14 0.25 S   Aug 2011   Nov 2014 Jan 2015 Yes PNH Ciprofloxacin
11 Jan 2017 25 Culture only Y P1.18–1,3 0.094 S   May 2016     Yes PNH Penicillin
12 Oct 2009 40 Culture only B P1.22,14 0.064 S       Yes PNH No
13 Mar 2017 23 PCR only W P1.5,2 NA NA 26 Dec 2016     Yes PNH Penicillin
14 Sep 2015 22 Culture only NG (B) c P1.22,14 0.50 R   Mar 2015   Apr 2015 May 2015 Yes (Mar 2015) Renal Penicillin
15 May 2016 20 Culture only E P1.5,2 0.094 S   Aug 2011   Feb 2015 Apr 2015 Yes (Oct 2013) Renal Not known
16a Dec 2014 20 PCR only NG (B)c P1.7–1,1 NA NA CtrA not detected (but siaD B CT 30) Apr 2014   Apr 2014 May 2014 Yes (Apr 2014) Renal Not known
16b Mar 2017 22 PCR only NG (B)c P1.7–1,1 NA NA CtrA not detected (but siaD B CT 31)
  1. a NT. PorA phenotypically not typeable (before PorA sequencing was available); b NG. CtrA PCR positive, but negative for capsular groups A, B, C, W, & Y; c NG (B). Non-groupable because of interruption of ctrA gene but genotypically capsular group B;
  2. NA Not available, a culture is needed to determine the Penicillin MICs.; ND Not done