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Table 2 Cardiac and renal parameters

From: Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease

Cardiac measures

Females with ERT with indication

Females w/o ERT with indication

Females with ERT without indication

Females w/o ERT without indication

Total

Dyspnea [n]

54 (43.6)***

14 (18.7)

0 (0.0)

1 (4.8)

69 (30.9)

NYHA class [n]

 none

29 (28.2)

31 (45.6)*

1 (50.0)

9 (56.3)

70 (37.0)

 I

34 (33.0)

25 (36.8)

1 (50.0)

6 (37.5)

66 (34.9)

 II

27 (26.2)

11 (16.2)

0 (0.0)

1 (6.3)

39 (20.6)

 III

13 (12.6)**

1 (1.5)

0 (0.0)

0 (0.0)

14 (7.4)

 IV

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

LV diastolic diameter [mm]

45.8 ± 5.5

46.2 ± 4.4

42.5 ± 3.5

46.4 ± 7.2

46.0 ± 5.3

LV systolic diameter [mm]

28.8 ± 6.1

28.5 ± 4.7

24.0 ± 1.4

29.7 ± 4.9

28.7 ± 5.5

LVDDi [cm/m2]

2.79 ± 0.33

2.83 ± 0.68

2.58 ± 0.12

2.78 ± 0.42

2.81 ± 0.49

Septal diameter [mm]

12.3 ± 3.6****

9.6 ± 2.0

9.5 ± 2.1

8.4 ± 1.5

11.0 ± 3.3

LVH [n]

64 (55.7)****

14 (18.9)

0 (0.0)

0 (0.0)

78 (37.0)

Posterior wall diameter [mm]

11.3 ± 3.1****

9.4 ± 1.9

10.5 ± 0.7

8.9 ± 1.6

10.4 ± 2.8

RWT [cm]

0.53 ± 0.17****

0.41 ± 0.09

0.48 ± 0.11

0.38 ± 0.10

0.47 ± 0.15

ECG abnormalities [n]

42 (36.5)****

7 (10.0)

1 (50.0)

1 (5.3)

51 (24.8)

Pacemaker [n]

18 (14.5)***

0 (0.0)

0 (0.0)

0 (0.0)

18 (8.0)

Myocardial infarction [n]

7 (6.9)*

0 (0.0)

0 (0.0)

0 (0.0)

7 (3.5)

Renal measures

 Albumin/creatinine-ratio [mg/gCreatinine]

48 [0–4773]

37 [0–734]

7 [7]

9 [0–226]

39 [0–4773]

 Albuminuria [n]

60 (61.9)

30 (58.8)

0 (0.0)

6 (40.0)

96 (58.5)

  Microalbuminuria (30–300 mg/gCreatinine) [n]

40 (66.7)*

27 (90.0)

0 (0.0)

6 (100.0)

73 (76.0)

  Macroalbuminuria (>300 mg/gCreatinine) [n]

20 (33.3)

3 (10.0)

0 (0.0)

0 (0.0)

23 (24.0)

 Cystatin C [mg/l]

1.03 ± 0.36****

0.78 ± 0.19

0.65 ± 0.18

0.71 ± 0.09

0.90 ± 0.32

 eGFRcys [ml/min/1.73 m2]a

80.4 ± 26.3****

103.6 ± 20.7

116.1 ± 20.3

114.5 ± 13.6

92.6 ± 26.7

 Creatinine [mg/dl]

0.99 ± 0.93***

0.75 ± 0.15

0.64 ± 0.11

0.65 ± 0.09

0.87 ± 0.71

 eGFRcreat [ml/min/1.73 m2]a

81.2 ± 25.3****

98.6 ± 19.1

108.8 ± 12.3

113.8 ± 12.6

90.4 ± 24.8

 eGFRcreat < 90 ml/min/1.73 m2 [n]

82 (66.1)***

28 (36.8)

0 (0.0)

0 (0.0)

110 (49.1)

 Hb [mg/dl]

13.3 ± 1.0

13.4 ± 0.99

13.1 ± 0.31

13.3 ± 1.1

13.3 ± 1.02

 Dialysis [n]

2 (1.6)

0 (0.0)

0 (0.0)

0 (0.0)

2 (0.9)

 Kidney transplantation [n]

1 (0.8)

0 (0.0)

0 (0.0)

0 (0.0)

1 (0.5)

Manifestations in different organs justifying ERT (per patient) [n]

2.5 [1–5]****

1 [1–3]

-

-

2 [0–5]

  1. Categorical data are presented as n and are % of total in parenthesis. Otherwise data is presented as mean ± standard deviation or median [range]. eGFR: estimated glomerular filtration rate (aexcluding patients with dialysis and/or kidney transplant); Hb Hemoglobin. eGFRcreat and eGFRcys are calculated via the CKD-EPI formulas according to Levey et al. 2009 and Inker et al. 2012, respectively. LVDDi: left ventricular diastolic diameter index; LVH: left ventricular hypertrophy (>12 mm septal diameter); NYHA New York Heart Association, RWT relative wall thickness. * p < 0.05, ** p < 0.01, *** p < 0.001, **** p < 0.0001