Table 6.

Response to metabolic and complement-targeted therapy

Response to metabolic therapy
Clinical recovery	54 %, n = 17
Improvement of GFR	81 %, n = 13
Stable GFR	19 %, n = 3
Pretreatment GFR (ml/min/1.73 m2)	49.5 n = 10
Posttreatment GFR (ml/min/1.73 m2)	89, n = 10
Mortality
Overall mortality	44 % (n = 16/36)
Infantile onset	56 %, (n = 9/16)
Noninfantile onset	35 %, (n = 7/20)
With neurological disease	50 %, (n = 8/16)
With cardiopulmonary disease	79 %, (n = 11/14)
Untreated	100 %, (n = 4/4)
Complement-targeted therapy (response)
Plasma exchange (patients 22, 24, 27, 33)	1* out of 4
Eculizumab (patients 33, 34)	No response

GFR glomerular filtration rate