Table 5.

Frequency of patients with mutations and ESRD in aHUS

IDENTIFIED MUTATIONS		Frequency in patients with aHUS (%)3-5,23,47,88–96	ESRD or death within 3–10 years of diagnosis (%)3,5,23,96,97
	CFH mutations	20–52	66–80
	CFH autoantibodies and/or CFHR1-3 deletions	5–10	30–63
	CFI mutations	4–10	50–72
	THBD mutations	3-10	54–60
	C3 mutations	2–10	56–67
	CFB mutations	1–4	70
	Isolated MCP mutations	5–15	6–38
	Combined MCP mutations	2	47
DGKE mutations	~27	46
NO IDENTIFIED MUTATIONS		30–50	32–50

Note: Data from references.3–5,47,88–96

Abbreviations: CFB, complement factor B; CFD, complement factor D; CFI, complement factor I; CFH, complement factor H; MCP, membrane cofactor protein; THBD, thrombomodulin gene; MAC, membrane attack complex; CFHR, complement factor receptor; DGKE, diacylglycerol kinase ε; aHUS, atypical hemolytic uremic syndrome.