Treatment and outcome of the first episode of non-Stx–HUS
Treatment or outcome . | No mutation, no. . | CFH mutation, no. . | MCP mutation, no. . | IF mutation, no. . | P1 . | P2 . | P3 . |
---|---|---|---|---|---|---|---|
Treatment of the first episode | |||||||
No treatment | 11 (74) | 2 (34) | 4 (14) | 2 (6) | .18 | .21 | .03 |
Plasma alone or in combination with drugs | 63 (74) | 32 (34) | 10 (14) | 4 (6) | .18 | .21 | .03 |
Plasma, infusion or exchange | 34 (63) | 21 (32) | 7 (10) | 3 (4) | .28 | .34 | .80 |
Plasma and drugs acting on the coagulation cascade | 6 (63) | 0 (32) | 2 (10) | 0 (4) | .07 | .32 | .009 |
Plasma and drugs acting on the immune system | 14 (63) | 11 (32) | 1 (10) | 0 (4) | .20 | .37 | .14 |
Plasma and both categories of drugs | 9 (63) | 0 (32) | 0 (10) | 1 (4) | .02 | .20 | NA |
Supportive treatment | 59 (76) | 32 (39) | 7 (14) | 3 (6) | .58 | .03 | .02 |
Outcome of the first episode | |||||||
Complete remission | 28 (81) | 7 (40) | 12 (14) | 3 (6) | .05 | <.001 | <.001 |
Partial remission | 30 (81) | 12 (40) | 1 (14) | 1 (6) | .44 | .028 | .085 |
Dialysis | 19 (81) | 9 (40) | 1 (14) | 2 (6) | .91 | .17 | .20 |
Death | 4 (81) | 12 (40) | 0 (14) | 0 (6) | <.001 | .39 | .02 |
Treatment or outcome . | No mutation, no. . | CFH mutation, no. . | MCP mutation, no. . | IF mutation, no. . | P1 . | P2 . | P3 . |
---|---|---|---|---|---|---|---|
Treatment of the first episode | |||||||
No treatment | 11 (74) | 2 (34) | 4 (14) | 2 (6) | .18 | .21 | .03 |
Plasma alone or in combination with drugs | 63 (74) | 32 (34) | 10 (14) | 4 (6) | .18 | .21 | .03 |
Plasma, infusion or exchange | 34 (63) | 21 (32) | 7 (10) | 3 (4) | .28 | .34 | .80 |
Plasma and drugs acting on the coagulation cascade | 6 (63) | 0 (32) | 2 (10) | 0 (4) | .07 | .32 | .009 |
Plasma and drugs acting on the immune system | 14 (63) | 11 (32) | 1 (10) | 0 (4) | .20 | .37 | .14 |
Plasma and both categories of drugs | 9 (63) | 0 (32) | 0 (10) | 1 (4) | .02 | .20 | NA |
Supportive treatment | 59 (76) | 32 (39) | 7 (14) | 3 (6) | .58 | .03 | .02 |
Outcome of the first episode | |||||||
Complete remission | 28 (81) | 7 (40) | 12 (14) | 3 (6) | .05 | <.001 | <.001 |
Partial remission | 30 (81) | 12 (40) | 1 (14) | 1 (6) | .44 | .028 | .085 |
Dialysis | 19 (81) | 9 (40) | 1 (14) | 2 (6) | .91 | .17 | .20 |
Death | 4 (81) | 12 (40) | 0 (14) | 0 (6) | <.001 | .39 | .02 |
IF mutation group has not been included in statistical analysis. The numbers of patients for whom data were available are reported in parentheses. Supportive treatment includes dialysis, blood transfusions, or concentrated red blood cell infusion. Plasma treatment includes infusion of 10 to 20 mL/kg/day and/or exchange of 1 to 2 plasma vol/day (or 30-40 mL/kg/day) for a total of 2 to 36 treatments in 2 days to 6 weeks. Complete remission is defined as normalization of both hematologic parameters and of renal function. Partial remission is defined as normalization of hematologic parameters with renal sequelae.
P1 indicates no mutation versus CFH mutation; P2, no mutation versus MCP mutation; P3, CFH mutation versus MCP mutation; NA, not applicable. Significant P values are in italics.