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Table 1 Demographics and clinical characteristics of the study population

From: Patients affected with Fabry disease have an increased incidence of progressive hearing loss and sudden deafness: an investigation of twenty-two hemizygous male patients

  Patient  

  Age  

Acroparesthesia

Kidney function (51Cr-EDTA

Glomerular Filtration

Rate in mL/min/1.73 m2)

Left ventricular

hypertrophy

Central Nervous

System involvement

Hearing status (0–5)

* right ear / left ear

1

50

+

Transplant

+

+ (lacuna on MRI)

2/2

2

26

+

Normal (98.7)

-

-

0/0

3

40

+

Transplant

-

-

0/1

4

59

+

Severe renal insufficiency (20.9)

+

+ (TIA)

2/2

5

24

+

Normal (87)

-

-

0/0

6

34

+

Transplant

+

-

0/2

7

37

-

Severe renal insufficiency (23.5)

-

+ (stroke)

2/5

8

29

+

Normal (111.8)

-

+ (TIA)

0/0

9

48

+

Transplant

+

+ (Stroke)

5/2

10

40

-

Transplant

+

-

0/0

11

61

-

Peritoneal dialysis

+

Not evaluated

1/3

12

31

+

Hemodialysis

+

+ (stroke)

1/1

13

21

+

Normal (127)

-

Not evaluated

0/0

14

50

+

Hemodialysis

+

+ (lacuna on MRI)

2/2

15

39

-

Severe renal insufficiency (16.4)

+

-

0/0

16

23

+

Normal (111)

-

+ (TIA)

2/2

17

64

+

Moderate kidney insufficiency (41.2)

+

+ (lacuna)

0/1

18

36

+

Normal (98.7)

-

-

0/0

19

57

+

Moderate kidney insufficiency (59.7)

+

+ (TIA)

5/1

20

35

-

Transplant

+

-

1/1

21

28

+

Mild kidney insufficiency (79.2)

-

-

0/0

22

25

+

Normal (114.2)

-

-

0/0

  1. Hearing impairment: 0: Normal hearing status (0–25 dB HL), 1: mild impairment (26–40 dB HL); 2: moderate impairment (41–55 dB HL), 3: severe deafness (56–70 dB HL), 4: profound deafness (91–110 dB HL), 5: >110 dB HL (cophosis)