Table 1.
Clinical and biochemical features in patients with anti-FH associated hemolytic uremic syndrome in two 6-year cohorts.
| Variable | 2007-12 (n = 119) | 2013-18 (n = 317) | Whole cohort (n = 436) | P |
|---|---|---|---|---|
| Boys | 91 (76.5) | 211 (66.6) | 302 (69.3) | 0.048 |
| Age, years | 7.9 ± 3.6 | 7.6 ± 3.2 | 7.7 ± 3.3 | 0.26 |
| Time to presentation, daysa | 18.0 ± 18.2 | 12.4 ± 12.1 | 13.8 ± 14.1 | 0.001 |
| Duration of oligoanuria, days | 11.7 ± 11.2 | 6.7 ± 9.9 | 8.0 ± 4.5 | < 0.001 |
| Anuria | 52 (43.7) | 79 (24.9) | 131 (30.0) | < 0.001 |
| Prodromal illness | ||||
| Febrile illness | 63 (52.9) | 175 (55.2) | 238 (54.6) | 0.74 |
| Diarrhea, dysentery | 10 (8.4) | 19 (6.0) | 29 (6.7) | 0.24 |
| Upper respiratory tract infection | 6 (5.0) | 39 (12.3) | 45 (10.3) | 0.032 |
| Jaundice, elevated transaminases | 24 (20.2) | 138 (43.5) | 162 (37.2) | < 0.001 |
| Seizures | 46 (38.7) | 71 (22.4) | 117 (26.8) | < 0.001 |
| Stage 2 hypertension | 84 (70.6) | 154 (48.6) | 238 (54.6) | < 0.001 |
| Hemoglobin, g/dl | 5.5 ± 1.3 | 5.2 ± 1.3 | 5.3 ± 1.3 | 0.67 |
| Platelet count, × 103/mm3 | 63.9 ± 39.3 | 58.5 ± 39.1 | 59.9 ± 39.1 | 0.57 |
| Reticulocyte count, % | 11.0 ± 9.5 | 8.7 ± 6.6 | 9.2 ± 7.3 | 0.10 |
| Nephrotic range proteinuria | 36 (30.3) | 211 (66.6) | 247 (56.7) | < 0.001 |
| Blood creatinine, mg/dl | 5.84 ± 2.67 | 5.46 ± 3.08 | 5.56 ± 2.98 | 0.28 |
| Lactate dehydrogenase, IU/L | 3042.0 ± 2701.4 | 3582.5 ± 2873.1 | 3,447 ± 2837.4 | 0.005 |
| Complement C3, mg/dl | 70.0 ± 28.6 | 71.2 ± 27.9 | 70.9 ± 28.1 | 0.84 |
| Anti-FH antibody, AU/ml* | 7330.5 ± 2017.4 | 11847.4 ± 1,140.1 | 10,633.2 ± 998.5 | 0.005 |
Normal range: hemoglobin 11.0–15.8 g/dl, platelet count 190–590 × 103/mm3, reticulocyte count 3.6–11%, blood creatinine: 0.17–1.01 mg/dl, lactate dehydrogenase 195–314 IU/L, C3 90–130 mg/dl.
a
Days between disease onset and evaluation.
Data shown as N (%), mean ± standard deviation or
*
mean ± standard error of mean; AU arbitrary units; FH factor H.