Table 9.

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

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)

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