Table 2.

Clinical and genetic data available for patients in remission phase of atypical haemolytic uraemic syndrome (aHUS)

Patient no.	aHUS pathogenic change	Gender	Age at first onset/time of study	Treatment	Outcome	Transplantation history
P1r*	CFH: p.Arg1206Cys 8	Male	22 years/27 years	Plasma therapy, haemodialysis	Remission	None
P2r*†	CFH: p.Arg1206Cys	Female	21 years/48 years	Plasma therapy, haemodialysis	ESRD	aHUS in graft
P3r	CFI: p.Arg474Stop 9	Female	49 years/57 years	Plasma therapy, haemodialysis	ESRD	No recurrence
P4r*	C3: p.Arg161Trp 15,16	Male	52 years/59 years	Plasma therapy, haemodialysis	ESRD	No recurrence
P5r*	C3: p.Arg161Trp	Female	23 years/35 years	Plasma therapy	ESRD	No recurrence
P6r	THBD: p.Ala43Thr 18	Female	2 months/5 years	Antibiotics, CVVH	Remission	None
P7r	None	Male	24 years/29 years	Haemodialysis	ESRD	None, on the waiting list for transplantation
P8r	None	Female	3 years/23 years	Haemodialysis	ESRD	aHUS in graft
P9r	None	Male	1 years/13 years	Peritoneal dialysis	ESRD	aHUS in graft
P10r	None	Female	15 years/24 years	Haemodialysis	ESRD	No recurrence
P11r§	None	Male	8 years/17 years	Blood transfusions	ESRD	No recurrence

^*Familial aHUS.

^†Mother of P1r.

^§Familial steroid-resistant nephrotic syndrome. Full spectrum aHUS developed during cyclosporin treatment. CFH = complement factor H; CFI = complement factor I; CVVH = continuous veno-venous haemofiltration; ESRD = end stage renal disease; THBD = thrombomodulin.