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Table 1 Patient characteristics.

From: Autonomic neuropathy in Fabry disease: a prospective study using the Autonomic Symptom Profile and cardiovascular autonomic function tests

 

All patients

n = 48

Male patients

n = 15

non-ERT

n = 2

ERT

n = 13

non-ERT versus ERT, p-value

Female patients

n = 33

non-ERT

n = 16

ERT

n = 17

non-ERT versus ERT, p-value

Age, years median (range)

47 (12-73)

47 (21-66)

47.5 (46-49)

47 (21-66)

1.00

45 (12-73)

25.5 (12-52)

51 (22-73)

0.00

Enzyme activity, μmol/l/h median (range)

14 (0.0-59)

1.2 (0.0-6.4)

2.0 (1.5-2.5)

0.7 (0.0-6.4)

0.20

19.6 (4.1-59.3)

20 (13-51)

19 (4.1-59)

0.51

MSSI sum score median (range)

15.5 (1-47)

25 (5-47)

11 (5-17)

27 (10-47)

0.23

13 (1-35)

7 (1-18)

23 (3-35)

0.00

   general score median (range)

4 (0-11)

5 (2-11)

3.5 (2-5)

5 (2-11)

0.31

4 (0-8)

2 (0-5)

5 (1-8)

0.00

   neurological score median (range)

4 (0-13)

6 (0-13)

0.5 (0-1)

6 (3-13)

0.02

3 (0-12)

1.5 (0-7)

4 (1-12)

0.04

   cardiovascular score median (range)

6.5 (0-15)

9 (1-15)

7 (3-11)

9 (1-15)

0.69

4 (0-15)

1.5 (0-11)

9 (0-15)

0.07

   renal score median (range)

0 (0-12)

0 (0-12)

0

0 (0-12)

0.38

0 (0-12)

0

4 (0-12)

0.00

VAS most severe pain median (range)

3 (0-9)

4 (0-8)

1

4 (0-8)

0.31

2 (0-9)

0.5 (0-8)

5 (0-9)

0.20

 

n = 36

n = 10

n = 2

n = 8

 

n = 26

n = 9

n = 17

 

Systolic BP, mmHg mean (SD)

118 (12)

114 (13)

115 (20)

113 (13)

0.88

120 (11)

115 (8)

123 (12)

0.11

Diastolic BP, mmHg mean (SD)

71 (7)

71 (9)

74 (16)

70 (8)

0.68

71 (7)

70 (9)

72 (5)

0.46

Heart rate, bpm mean (SD)

65 (12)

68 (13)

61 (4)

70 (14)

0.40

64 (12)

66 (13)

63 (11)

0.47

  1. ERT = enzyme replacement therapy, MSSI = Mainz Severity Score Index, VAS = visual analogue scale, BP = blood pressure, bpm = beats per minute