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. 2014 Jul 18;124(11):1715–1726. doi: 10.1182/blood-2014-02-558296

Table 1.

Circulating complement profile in aHUS patients

Complement parameters Disease phase Overall Mutations or anti-CFH Ab No mutations
Reduced C3 serum levels (83-180 mg/dL)* Acute 10 (18) 5 (9) 5 (9)
Remission 15 (32) 11 (25) 4 (7)
Increased C5a plasma levels (1.9-13.1 ng/mL)* Acute 9 (19) 3 (10) 6 (9)
Remission 21 (36) 15 (27) 6 (9)
Increased SC5b-9 plasma levels (127-400 ng/mL)* Acute 10 (19) 4 (10) 6 (9)
Remission 23 (36) 20 (27) 3 (9)

Ab, antibody.

*

Limits of normal ranges (as defined in supplemental “Methods”). In parentheses are the numbers of patients for whom data were available, and outside the parentheses are the numbers of patients with reduced C3 or increased C5a or SC5b-9 levels.

One patient was receiving eculizumab at the time of the test.

Eight patients were receiving eculizumab at the time of the tests.