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Table 2 Clinical Characteristics of Patients in the Global aHUS Registry

From: The global aHUS registry: methodology and initial patient characteristics

Characteristic

Pediatric patients (n = 201)a

Adult patients (n = 315)a

Total (N = 516)a

Age at initial symptoms, years

n

198

310

508

 Mean (SD)

4.1 (3.9)

33.6 (18.3)

22.1 (20.4)

 Median (range)

2.9 (0.0–16.3)

31.1 (0.1–81.8)

19.2 (0.0–81.8)

 Age categories, n (%)

   <2

87 (43.9)

12 (3.9)

99 (19.5)

  2 to <5

40 (20.2)

9 (2.9)

49 (9.6)

  5 to <12

60 (30.3)

14 (4.5)

74 (14.6)

  12 to <18

11 (5.6)

11 (3.5)

22 (4.3)

  18 to <30

95 (30.6)

95 (18.7)

  30 to <40

71 (22.9)

71 (14.0)

  40 to <50

34 (11.0)

34 (6.7)

   ≥50

64 (20.6)

64 (12.6)

Age at diagnosis, years

n

201

313

514

 Mean (SD)

4.3 (4.0)

34.5 (17.9)

22.7 (20.5)

 Median (range)

3.1 (0.0–16.3)

31.5 (0.1–81.8)

19.7 (0.0–81.8)

 Age categories, n (%)

   <2

86 (42.8)

10 (3.2)

96 (18.7)

  2 to <5

39 (19.4)

6 (1.9)

45 (8.8)

  5 to <12

63 (31.3)

11 (3.5)

74 (14.4)

  12 to <18

13 (6.5)

14 (4.5)

27 (5.3)

  18 to <30

98 (31.3)

98 (19.1)

  30 to <40

71 (22.7)

71 (13.8)

  40 to <50

35 (11.2)

35 (6.8)

   ≥50

68 (21.7)

68 (13.2)

Disease duration from diagnosis to enrollment, years

n

201

313

514

 Mean (SD)

4.0 (4.1)

5.2 (7.8)

4.7 (6.6)

 Median (range)

2.4 (0.0–17.1)

1.6 (0.0–50.6)

1.9 (0.0–50.6)

Family history of aHUS, n (%)

41 (20.4)

58 (18.4)

99 (19.2)

History of PE/PI, n (%)

107 (53.2)

204 (64.8)

311 (60.3)

History of dialysis, n (%)

107 (53.2)

200 (63.5)

307 (59.5)

History of kidney transplantation, n (%)

25 (12.4)

76 (24.1)

101 (19.6)

History of eculizumab use prior to enrollment, n (%)

106 (52.7)

168 (53.3)

274 (53.1)

  1. aHUS atypical hemolytic uremic syndrome, PE/PI plasma exchange/plasma infusion, SD standard deviation
  2. aFor each parameter, percentages were calculated based on the number of patients with non-missing observations in each group