Clinical and Complement Long-Term Follow-Up of a Pediatric Patient with C3 Mutation-Related Atypical Hemolytic Uremic Syndrome

Table 1

Complement analyses of the aHUS patient presented in this study.

Time of blood sampling (reference value)	C3^1 (g/L) (0.70-2.00)^4	C4^1 (g/L) (0.10-0.50)	CP^2 (%) (>40)	AP^2 (%) (>10)	sC5b-9 (ng/mL)^3 (<300)
2011, June; Pre-treatment^5	0.51	0.17	96	78	n.d.^6
2011, June; Post-eculizumab	0.48	0.13	<1	<1	140
2011, August	0.47	0.11	<1	<1	196
2015, May	0.54	0.16	3	<1	189
2016, May	0.47	0.16	2	<1	134
2017, January 5th^7	0.44	0.12	<1	<1	106
2017, January 10th^8; Pre-eculizumab	0.43	0.11	1	<1	n.d
2017, January 10th^8; Post-eculizumab	0.46	0.12	<1	<1	n.d

and C4 were quantified in the routine hospital laboratory using nephelometry
(classical pathway) and AP (alternative pathway) were quantified using the Wieslab® Complement screen kit (Euro Diagnostica AB, Malmö, Sweden). The reference ranges are given in % referred to 100% activity in normal human serum. These are based on screening for complement deficiencies. For detection of complete C5 blockade the values should be close to zero (< 3-5%).
soluble terminal C5b-9 complex was quantified using the sC5b-9 ELISA kit from Quidel, CA.
ranges are given in parenthesis.
the first infusion of eculizumab.
= not determined (sample not available).
obtained 7 days after and 15 days before eculizumab infusion.
Samples obtained 1 hr before and 1 hr after eculizumab infusion.