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. 2018 Sep 10;8(5):122–141. doi: 10.5500/wjt.v8.i5.122

Table 3.

Complement studies for atypical hemolytic uremic syndrome (aHUS)

Complement test aHUS
Complement protein levels C3, C4, FB1, C51
Complement regulatory protein levels FH, FI, Properdin1, CD462
Complement split products C3c1, C3d1, Bb1, sC5b-91
Complement functional assays CH50, AH50, hemolytic assays, FH assays1
Autoantibodies Anti-FH
Genetic screening CFH, CFI, C3, CD46, CFB Genomic rearrangements across the FH-FHR locus (e.g., by MLPA) Sequencing of coding regions and assessment of CNV Non-complement genetic screening includes THBD and DGKE
1

Currently available only at specific laboratories; they are research and not clinically validated assays;

2

CD46 is also known as MCP. Adapted from: Goodship et al[58]. AH50: Alternative pathway hemolytic assay; C3: Complement component 3; C4: Complement component 4; C5: Complement component 5; CFB: Complement factor B gene; CFH: Complement factor H gene; CFHR: Complement factor H related genes; CFI: Complement factor I gene; CH50: Classical pathway hemolytic assay; CNV: Copy number variation; DGKE gene: Diacylglycerol kinase epsilon gene; FB: Complement factor B; FH: Complement factor H; FI: Complement factor I; MLPA: Multiplex ligation-dependent probe amplification; sC5b-9: Soluble C5b-9; THBD: Thrombomodulin; aHUS: Atypical hemolytic uremic syndrome.