Table 6

Clinical and complement data in patients with aHUS with anti-fH autoantibodies

PatientHUS onset
First serum sample analyzed
Current age and situation
Age, yPlasma therapyRenal functionTime after onsetAutoAb titer, AU/mL*C3, mg/dL*C4, mg/dL*CFH, mg/dL*
H87 PE Full recovery Onset 600 55.7 8.85 25 11 y; unknown 
H108 PE Full recovery 3 mo 1600 86.2 28.4 22 8 y; ESRD 
H119 None ESRD 13 y 600 117 16.6 28 20 y; RT since 1995 
H151 PE + PI Full recovery 7 mo 6000 66.7 16.7 31 8 y; stable with corticoids 
H154 PE Full recovery Onset 18 000 58.5 19.6 26 10 y; stable with azathioprine 
H171 None ESRD 5 mo 3600 81.3 40.6 25 4 y; RT since March 2009 
H177 PF + 5% albumin Death (myocarditis) Onset 16 000 58.5 19.6 22 Died during first episode 
PatientHUS onset
First serum sample analyzed
Current age and situation
Age, yPlasma therapyRenal functionTime after onsetAutoAb titer, AU/mL*C3, mg/dL*C4, mg/dL*CFH, mg/dL*
H87 PE Full recovery Onset 600 55.7 8.85 25 11 y; unknown 
H108 PE Full recovery 3 mo 1600 86.2 28.4 22 8 y; ESRD 
H119 None ESRD 13 y 600 117 16.6 28 20 y; RT since 1995 
H151 PE + PI Full recovery 7 mo 6000 66.7 16.7 31 8 y; stable with corticoids 
H154 PE Full recovery Onset 18 000 58.5 19.6 26 10 y; stable with azathioprine 
H171 None ESRD 5 mo 3600 81.3 40.6 25 4 y; RT since March 2009 
H177 PF + 5% albumin Death (myocarditis) Onset 16 000 58.5 19.6 22 Died during first episode 

PE indicates plasma exchange; PF, plasmapheresis; PI, plasma infusion; ESRD, end-stage renal disease; and RT, renal transplant.

*

Normal values: AutoAb titer, < 150 AU/mL; C3, 77-135 mg/dL; C4, 14-47 mg/dL; CFH, 12-56 mg/dL.

Value out of normal range.

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