Table 6.
Severity of HUS according to sC5b-9 level and genetic background in Shiga toxin–positive patients with HUS
| Complement Abnormalities | Acute Phase | Last Follow-Upa | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Dialysis Required | CNS Manifestations | No CKDb | CKD Stage 1–4b | CKD Stage 5b | CKD Stage 1–4 versus No CKD | CKD Stage 5 versus No CKD | |||||
| Yes, N (%) | No, N (%) | OR; 95% CI; P-Valuec | Yes, N (%) | No, N (%) | OR; 95% CI; P-Valuec | n (%) | n (%) | n (%) | OR; 95% CI; P-Valuec | OR; 95% CI; P-Valuec | |
| Increased sC5b-9 (>420 ng/ml)d | 23/33 (70) | 15/25 (60) | 1.5; 0.5 to 4.6; 0.2 | 11/13 (85) | 27/45 (60) | 3.6; 0.7 to 18; 0.1 | 25/36 (69) | 9/18 (50) | 1/1 (100) | 2.3; 0.7 to 7; 0.2 | 2.3; 0 to 19; 0.4 |
| Pathogenic variant (n=4) | 1/42 (2) | 3/33 (9) | 0.2; 0 to 2.4; 0.2 | 1/17 (6) | 3/58 (5) | 1.1; 0.1 to 12; 0.9 | 2/48 (4) | 1/20 (5) | 1/1 (100) | 0.8; 0.1 to 9; >0.99 | 55; 1.8 to 1747; 0.06 |
| Variant of uncertain significance (n=8) | 5/43 (12) | 3/32 (9) | 1.2; 0 to 5.8; 0.9 | 0/17 (0) | 8/58 (14) | 0.2; 0 to 3; 0.2 | 7/48 (15) | 1/20 (5) | 0/1 (0) | 3.2; 0.3 to 28; 0.4 | 0.5 |
| No variant identified | 37/43 (86) | 26/32 (81) | 1.4; 0 to 5; 0.3 | 1/17 (6) | 11/58 (19) | 0.3; 0 to 2.2; 0.2 | 39/48 (81) | 18/20 (90) | 0/1 (0) | 0.5; 0.1 to 2.5; 0.4 | 0.13 |
HUS, hemolytic uremic syndrome; CNS, central nervous system; OR, odds ratio; 95% CI, 95% confidence interval.
a
Median (First and third quartiles): 47 months (20–65) (n=79 with six lost to follow-up). The CKD stage was documented at 3 year follow-up in 43 patients.
b
CKD stages according to Kidney Disease Improving Global Outcomes Guidelines 2012 (http://www.kdigo.org/clinical_practice_guidelines/pdf/CKD/KDIGO_2012_CKD_GL.pdf). See Supplemental Material for definition of CKD stages. No patient had CKD stage 4 at last follow-up.
c
P with Fisher exact test.
d
Samples before day 14 after admission.