. 2019 Nov 6;14(12):1719–1732. doi: 10.2215/CJN.05830519

Table 1.

Clinical parameters and complement activation markers in incident patients with atypical hemolytic uremic syndrome

Patient	Genetic Variants	Disease Phase (Trigger)	Creatinine (0.3–1.3 mg/dl)	Hemoglobin (12–17 g/dl)	Platelets (130–400 10⁹/L)	Lactate Dehydrogenase (<234 U/L)	Haptoglobin (0.3–1.8 g/L)	Serum C3 (0.8–1.9 g/L)	Serum C4 (0.1–0.5 g/L)	Serum 50% Hemolytic Complement (28–60 U/ml)	Serum C5b-9 Deposits (Mean±SEM)	Activated-Plasma C5b-9 Deposits (Mean±SEM)
Patient 1	MCP: (c.478G>T) p.Val160Phe	Acute (none)	4.9	8.7	117	317	N.D.	1.0	N.D.	55	3.2±1.5^a	N.A.
Patient 2	C3: c.3125G>A MCP: c.1148C>T	Acute (respiratory infection)	3.9	9.4	7	2430	N.D.	0.8	0.3	72	2.5±1.2^a	N.A.
Patient 2	C3: c.3125G>A MCP: c.1148C>T	Remission^b	0.8	12.1	259	146	1.1	0.8	N.D.	N.D.	1.4±0.9^a ^c	N.A.
Patient 3	No	Acute (none)	5	8.5	196	415	N.D.	0.7	0.1	28	1.9±1^a	N.A.
Patient 3	No	Remission^d	4.4	9.2	492	194	N.D.	0.9	0.2	7	0.5±0.5^c	N.A.
Patient 4	MCP: exon 6, heterozygosis (c.800_820Del) p.Thr267Asn273del CFH: exon 6, heterozygosis (c.292C>T) p.Leu98Phe	Acute (urinary infection)	8.6	10.9	73	1353	N.D.	0.6	0.2	24	1.7±0.8^a	5.6±0.2^a
Patient 4		Remission^e	6	12.7	158	516	1.29	0.8	0.3	9	0.6±0.5^c	0.3±0.1^c
Patient 5	CFH: exon 19, heterozygosis (c.2850G>T) p.Gln950	Acute (postpartum)	2.4	5.8	61	1678	N.D.	N.D.	N.D.	N.D.	2±0.8^a	4.9±0.1^a
Patient 5	CFH: exon 19, heterozygosis (c.2850G>T) p.Gln950	Remission^e	0.8	11.7	305	247	0.5	0.9	0.2	N.D.	0.1±0.1^c	0.1±0.18^c
Patient 6	No	Acute (postpartum)	4.1	6.7	74	1781	0.2	1.4	0.5	21	1.6±0.4^a	5.9±0.4^a
Patient 6	No	Remission^e	0.8	10.9	268	179	1.12	0.8	0.2	12	0.8±0.1^c	0.3±0.1^c
Patient 7	No	Acute (postpartum)	1.6	13.0	93	266	0.3	1.1	0.3	N.D.	2.7±0.4^a	4.1±0.3^a
Patient 7	No	Partial remission^e	1.5	13.5	115	251	1.1	0.9	0.2	N.D.	2.7±0.5^a	2.4±0.1^a
Patient 8	CFI: exon 5, heterozygosis (c.739T>G) p.Cys247Gly	Acute (pancreatic cancer)	1.9	8.3	40	2407	N.D.	1.7	0.2	55	2.8±0.5^a	3.2±0.2^a
Patient 8	CFI: exon 5, heterozygosis (c.739T>G) p.Cys247Gly	Remission^e	1.3	12.3	290	399	0.3	1.0	0.2	16	1.1±0.6	0.5±0.1^c
Patient 9	CFH: heterozygosis (c.3514G>T) p.Glu172Stop	Acute^b (cocaine)	7.2	7.1	105	682	0.3	0.8	0.2	41	N.A.	13.1±0.2^a
Patient 9	CFH: heterozygosis (c.3514G>T) p.Glu172Stop	Remission^e	2.2	13.0	197	304	1.3	0.7	0.2	8	N.A.	0.78±0.1^c
Patient 10	CFH: heterozygosis (c.3514G>T) p.Glu172Stop	Acute (cocaine)	12.2	5.9	44	3214	N.D.	N.D.	N.D.	N.D.	N.A.	10.9±0.3^a
Patient 10	CFH: heterozygosis (c.3514G>T) p.Glu172Stop	Remission^e	1.3	12.2	207	349	0.8	0.8	0.2	11	N.A.	1.1±0.2^c
Patient 11	CFHR1::CFH, hybrid gene (loss of signal in exon 6 of CFHR1 and gain of signal exon 23 of CFH)	Acute (postpartum)	4.2	9.8	114	1649	0.1	0.4	0.1	29	N.A.	6.4±0.2^a
Patient 11		Remission^e	Hemodialysis	11.3	214	430	1.2	1.0	0.4	6	N.A.	0.8±0.1^c

Statistical analysis was performed with raw data using the t test for paired samples. N.D., not done; N.A., sample not available.

Values statistically different than control values. (P<0.05).

Plasma exchange treatment.

Values statistically different than acute phase values. (P<0.05).

Plasma exchange and eculizumab treatment.

Eculizumab treatment.