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Table 9 Case reports of patients with atypical hemolytic uremic syndrome on their native kidneys treated with eculizumab

From: Atypical hemolytic uremic syndrome

Reference Mutation Age at onset of HUS, evolution and response to plasmatherapy Age at eculizumab initiation Response to plasmatherapy of HUS episode at eculizumab initiation Serum creatinine level at eculizumab initiation Delay of hematological and renal improvement after initiation of eculizumab Delay until complete remission of HUS after initiation of eculizumab Protocol Evolution of HUS
Last Screat (follow-up under eculizumab)
Gruppo et al
2009 [155]
R. Gruppo PC
NI < 8 days
3 relapses over 11 m,
PI sensitive
19 m PE resistant 265 μmol/L 2 days 10 days Complete protocol Remission
35 μmol/L
(2 y 4 m)
Fremont et al
2009 [156]
CFH 4 y 4 y PE partially sensitive 80 μmol/L ND 2 weeks Complete protocol Remission
26 μmol/L
(10 weeks)
Mache et al
2009 [157]
NI 17.8 y
PE sensitive
3 relapses at PE tapering
17.11 y PE resistant 690 μmol/L ~ 3 days
(platelets increase)
5 days (hematologic remission) Single dose Relapse at 2w
ESRF
Kose et al
2010 [158]
NI 18 y 18 y PE resistant ~310 μmol/L 1 day ~ 7 days Single dose Relapse at 2m
ESRF
Lapeyraque et al 2010 [159]
AL. Lapeyraque PC
CFH
S1191L
V1197A
7 m
11 relapses over 5.4 y, PE/PI sensitive
6 y PI resistant 108 μmol/L A few days 1 week Complete protocol Remission
44 μmol/L
(1 y 3 m)
Prescott et al
2010 [160]
HC. Prescott PC
CFI
p.A258T
47 y
PE sensitive
Relapse 2 w after PE cessation
47 y PE resistant 610 μmol/L - 7 days (Screat decrease)
- 49 days
(platelets normalization)
~1.5 m Complete protocol Remission
230 μmol/L
(7 m)
Ohanian et al 2011 [161] ND 50 y 50 y No plasmatherapy 600 μmol/L - 4 days (LDH decrease)
- 10 days (Screat decrease)
~ 1.5 m Complete protocol Remission
198 μmol/L
(2.5 m)
  1. PC: personal communication, with permission; Screat: serum creatinine; CFH: factor H; CFI: factor I; NI: none identified; ND: not documented; ESRF: end stage renal failure; PE: plasma exchange; PI: plasma infusion