Table 6.
Clinical and biological data at onset of the 30 patients with anti-FH associated aHUS.
| ID | Sex | Ethnicity | Population | Disease onset | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Age (years) | Disease triggers | Hb (14–18 g/dl) | LDH (266–500 U/L) | Platelets (150–400 × 103/ul) | Creatinine (0.5–0.8 mg/dl)* | Dialysis (yes/no) | ||||
| 1 | F | Caucasian (Eu) | Italian | 7.0 | Vomiting | 4.5 | na | 38,000 | 2.7 | Yes |
| 2 | M | Caucasian (Eu) | Italian | 7.7 | Flu like | 6.3 | 4,185 | 64,000 | 1.6 | No |
| 3 | F | Caucasian (Eu) | Portuguese | 7.6 | Resp. infection | 8.5 | 538 | 78,000 | 4.9 | Yes |
| 4 | F | Caucasian (Eu) | German | 8.0 | None | 6 | 2,464 | 16,000 | 1.56 | Yes |
| 5 | M | Caucasian (Eu) | Italian | 8.8 | Vomiting | 6 | na | 167,000 | 9.4 | Yes |
| 6 | M | Caucasian (Eu) | Italian | 6.2 | Flu like | 6.9 | na | 30,000 | 6.9 | Yes |
| 7 | M | Caucasian (Eu) | Italian | 5.7 | None | 6.5 | 3,650 | 58,000 | 2.4 | No |
| 8 | M | Caucasian (USA) | American | 6.7 | na | na | na | na | na | Yes |
| 9 | F | Caucasian (Eu) | Serbian | 7.6 | Vomiting | 10.4 | 5,120 | 48,000 | 7 | Yes |
| 10 | F | Caucasian (Eu) | Serbian | 6.6 | Resp. infection | 6 | 2,022 | 124,000 | 1.25 | No |
| 11 | F | Caucasian (Eu) | Italian | 5.4 | Vomiting | 7 | 2,930 | 42,000 | 2.8 | Yes |
| 12 | M | Caucasian (Eu) | Italian | 7.8 | Vomiting | 6.8 | 2,866 | 96,000 | 1.9 | Yes |
| 13 | F | Caucasian (Eu) | Turkish | 12.8 | None | 10.6 | 1,767 | 12,000 | 2.6 | No |
| 14 | F | Caucasian (Eu) | Bulgarian | 15.5 | Vomiting, fever | 9 | 3,597 | 79,000 | 3.7 | Yes |
| 15 | M | Caucasian (Eu) | Polish | 11.0 | Vomiting | 7.8 | 1,908 | 43,000 | 4.3 | Yes |
| 16 | F | Caucasian (USA) | American | 10.5 | Vomiting, fever | 8 | na | 29,000 | 11 | Yes |
| 17 | F | Caucasian (USA) | American | 5.3 | Flu like | 8 | 1,422 | 120,000 | 3 | Yes |
| 18 | M | Caucasian (Eu) | Italian | 10.1 | Vomiting | 5.2 | 3,770 | 70,000 | 7 | No |
| 19 | F | Caucasian (Eu) | Italian | 8.8 | na | 7.2 | na | 60,000 | 4 | Yes |
| 20 | F | Caucasian (Eu) | Dutch | 30.2 | Cesarean | 9.5 | 3,362 | 40,000 | 1.8 | No |
| 21 | F | Hispanic | Argentine | 17.6 | Oral contraceptive | na | na | na | na | Yes |
| 22 | M | Caucasian (Eu) | Polish | 7.6 | Resp. infection | 9 | na | na | 9 | Yes |
| 23 | M | Asian | Japanese | 9.2 | Vomiting, fever | 6.5 | 2,569 | 15,000 | 2 | Yes |
| 24 | M | Jewish | Israeli | 5 | None | 4.8 | 1,091 | 76,000 | 1.5 | Yes |
| 25 | M | Persian | Iranian | 15.0 | Resp. infection | 8 | 7,752 | 23,000 | 5.7 | Yes |
| 26 | M | Hispanic | Argentine | 6.5 | None | 9 | na | 18,000 | 3.5 | Yes |
| 27 | M | Caucasian (Eu) | Italian | 7.1 | Resp. infection | 6.6 | na | 52,000 | 4.7 | Yes |
| 28 | F | Caucasian (Eu) | Italian | 8.1 | Vomiting, fever | na | na | na | na | Yes |
| 29 | M | African-Arab | Yemenian | 1.3 | Resp. infection | 5 | 1,916 | 150,000 | 0.6 | No |
| 30 | M | Caucasian (Eu) | Belorussian | 6.9 | Vomiting | 5.8 | 5,619 | 62,000 | 2.33 | Yes |
| Median [IQR] | Mean ± SD | Mean ± SD | Mean ± SD | Mean ± SD | % Yes | |||||
| Overall | 7.6 [6.6–9.9] | 7.3 ± 1.4 | 3,081.5 ± 1,736 | 61.923 ± 41,218 | 5.7 ± 3.3 | 77 | ||||
| FHR1 deficiency | Yes | 7.6 [6.6–9.2] | 7.1 ± 1.3 | 3,010 ± 1,696 | 66,272 ± 42,332 | 6.5 ± 3.4 | 76 | |||
| No | 8.8 [6.9–12.8] | 8.2 ± 2.1 | 3,693.0 ± 2,723.8 | 38,000 ± 26,683 | 3.3 ± 1 | 80 | ||||
| P-value | 0.4 | 0.23 | 0.61 | 0.21 | 0.4 | 0.7 | ||||
| LPVs | Yes | 7.1 [5.7–8.8] | 5.6 ± 0.9 | 2,521.8 ± 1,325.0 | 62,750 ± 40,679 | 3.3 ± 1 | 56 | |||
| No | 7.8 [6.9–10.1] | 7.6 ± 1.3 | 3,281.4 ± 1,832.7 | 61,555 ± 42,621 | 6.5 ± 3.4 | 86 | ||||
| P-value | 0.32 | 0.34 | 0.42 | 0.95 | 0.38 | 0.18 | ||||
The Patient ID, the sex (F, female; M, male), the ethnicity, the population of origin, the age at the disease onset, the observed triggers, hemoglobin (Hb), lactate dehydrogenase (LDH), platelet count, serum creatinine and the need for dialysis are shown. na, not available. Normal values are in brackets.
*
Normal values for children between 5 and 10 years. For children < 1–5 years, 0.3–0.5 mg/dl; for children >10 years/adults, 0.5–1.2 mg/dl.