Case Report

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)C31 (g/L) (0.70-2.00)4C41 (g/L) (0.10-0.50)CP2 (%) (>40)AP2 (%) (>10)sC5b-9 (ng/mL)3 (<300)

2011, June
2011, June
2011, August0.470.11<1<1196
2015, May0.540.163<1189
2016, May0.470.162<1134
2017, January 5th70.440.12<1<1106
2017, January 10th8
2017, January 10th8

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.