Table 3.

Transient desialylation of FH/FHRs in two Spanish and four Hungarian SP-HUS patients.

Patient code	Gender	Age at onset (mo)	Previous vaccination	Time after onset	Clinical status	Complement profile	FHR-5 Levelsa (μg/mL)	FH/FHRs desialylation	Neuraminidase Activity (U/L)b	Genetic findingsc
H837	Male	21	No info available	1 days	Onset	Low C4, FH, FI	3.61	YES	8.03	No pathogenic variants
				3 mo	Remission	Normal	0.03	NO	0
H946	Male	21	Prevenar	4 days	Onset	Low C3, C4	1.97	YES	0.45	Non-available
				2 mo	Remission	Normal	0.80	NO	0
HUN156d	Female	18	Pneumovax	10 days	Onset	Low C3,C4,FB,FI	1.51	YES	3.94	CFI (c.148C>G, p.Pro50Ala); MCPggaac
				11 mo	Remission	Low FI	0.48	NO	0
HUN816	Male	36	No info available	9 days	Onset	Low C3, C4, FH	2.81	YES	0.54	Non available
				2 mo	Remission	High C3, FI, FB	0.86	NO	0.47
HUN1869	Female	30	No info available	5 days	Onset	Low C3, C4, FI	1.19	YES	2.50	DelCFHR3-CFHR1 (HOM)
				2 mo	Remission	Normal	0.42	NO	0.17
HUN2638	Male	32	Prevenar	23 days	Onset	Low C3, C4, FH	1.08	YES	0.42	C3 (c.2852G>A, p.Arg951His); C3 (c.304C>G;R102G); C3 P314L; CFH (c.184G>A; V62I); CFH(H3); MCPggaac
				42 days	Remission	Normal	0.86	NO	0.44

Complement profile and neuraminidase activity in the two plasma samples drawn during disease onset and at remission. Anti-FH autoantibodies were negative in all samples.

^aMean levels in controls: 1.98 ± 1.02 μg/mL.

^bBackground level in controls: 0.50 U/L.

^cAll genetic variants in patients HUN156 and HUN2638 are in heterozygosis.

^dDescribed in (11).