Table 1.
Epidemiological and clinical features of studied patients
| Feature | All patients | STEC-HUS | aHUS |
|---|---|---|---|
| Number | 37 | 26 (68.4 %) | 11 (28.9 %) |
| Gender | 20 M : 17 F | 13 M : 13 F | 7 M : 4 F |
| Age (years) mean ± SD (range) | 6.6 ± 4.8 (0.4–17.8) | 6.6 ± 5.3 (0.4–17.8) | 6.5 ± 3.8 (0.5–12.6) |
| Follow-up (years) mean ± SD (range) | 2.9 ± 0.9 (1.3–4.4) | 2.7 ± 0.9 (1.3–4.4) | 3.1 ± 1.0 (1.9–4.3) |
| Serotype | Not applicable | O157: 16 (61.5 %) | Not applicable |
| O26: 4 (15.4 %) | |||
| O5: 1 (3.8 %) | |||
| O104: 1 (3.8 %) | |||
| Not serotyped, but α-O157 negative: 4 (15.4 %) | |||
| Presentation | |||
| Diarrhea | 14 (34) | 12 (23) | 2 (11) |
| Bloody diarrhea | 9 (34) | 9 (23) | 0 (11) |
| Gastrointestinal symptoms without diarrhea | 8 (34) | 2 (23) | 6 (11) |
| Upper respiratory tract infection | 4 (34) | 1 (23) | 3 (11) |
| Oligo/anuria | 14 (34) | 8 (23) | 6 (11) |
| Headache | 1 (34) | 0 (23) | 1 (11) |
| Extrarenal complications | |||
| None | 27 (34) | 16 (23) | 11 (11) |
| Yes | 7 (34) | 7 (23) | 0 (11) |
| Neurological | 7 (34) | 7 (23) | 0 (11) |
| Intestinal (sigmoid stenosis, peritonitis) | 1 (34) | 1 (23) | 0 (11) |
| Chronic venous insufficiency thrombosis | 1 (34) | 1 (23) | 0 (11) |
| Treatment | |||
| Spontaneous remission | 9 (34) | 8 (23) | 1 (11) |
| Dialysis | 13 (34) | 13 (23) | 0 (11) |
| Plasma therapy | 3 (34) | 0 (23) | 3 (11) |
| Dialysis and plasma therapy | 9 (34) | 2 (23) | 7 (11) |
| Eculizumab | 5 (34) | 2 (23) | 3 (11) |
| Outcome | |||
| Normal renal function | 26 (33) | 18 (23) | 8 (10) |
| Hypertension1 and proteinuria2 | 8 (33) | 3 (23) | 5 (10) |
| Hypertension1 | 2 (33) | 1 (23) | 1 (10) |
| Proteinuria2 | 4 (33) | 4 (23) | 0 (10) |
| Maintenance treatment with eculizumab | 2 (33) | 0 (23) | 2 (10) |
| Relapses after this episode | 3 (33) | 0 (23) | 3 (10) |
| Genetic or acquired complement aberrations (see Table 2) | |||
| Total | 14/36 (38.9 %) | 7/25 (28.0 %) | 7/11 (63.3 %) |
| CFH | 2/36 (5.6 %) | 2/25 (8.0 %) | – |
| CD46 | 2/36 (5.6 %) | – | 2/11 (18.2 %) |
| C3 | 3/36 (8.3 %) | 2/25 (8.0 %) | 1/11 (9.1 %) |
| C3 and αFH | 1/36 (2.8 %) | – | 1/11 (9.1 %) |
| αFH | 6/36 (16.7 %) | 3/25 (12.0 %) | 3/11 (27.3 %) |
For clinical features, the numbers of patients with data available are reported in parentheses
αFH autoantibodies against factor H, aHUS atypical HUS, C3 complement component 3, CD46 membrane cofactor protein, CFH complement factor H, HUS hemolytic uremic syndrome, SD standard deviation, STEC-HUS Shiga-toxin producing Escherichia coli-induced HUS
1Hypertension: a systolic and/ or diastolic pressure ≥ 2.0 standard deviation scores compared with normal values for age, gender, and height
2Proteinuria: > 2 years old > 0.2 mg/mg (>22.6 mg/ mmol or 0.226 g/10 mmol); < 2 years old > 0.5 mg/mg (>56.6 mg/mmol or 0.566 g/10 mmol)