Table 1 Patient characteristics.

From: Predictors of objective cognitive impairment and subjective cognitive complaints in patients with Fabry disease

 

All

Men

Women

Intergroup comparison

Classical (a)

Non-classical (b)

Classical (c)

Non-classical (d)

P

Post hoc

Patients, n (%)

81

17 (21.0%)

11 (13.6%)

43 (53.1%)

10 (12.3%)

—

—

Age in years, mean (±SD)

44.5 (±14.3)

38.6 (±13.5)

58.0 (±11.2)

43.5 (±13.9)

43.9 (±13.0)

3.5 * 10−3

a,c < b

History of ERT, n (%)

48 (59.3%)

17 (100.0%)

3 (27.3%)

27 (62.8%)

1 (10.0%)

5.3 * 10−7

b,c,d < a, d < c

Currently on ERT, n (%)

43 (53.1%)

15 (88.2%)

2 (18.2%)

25 (58.1%)

1 (10.0%)

3.2 * 10−5

d < a,c, b < a

   Replagal/Fabrazyme, n/n

11/32

5/10

0/2

6/19

0/1

—

—

   Time on ERT in years, median (range)

8.6 (0.1–16.0)

12.4 (1.6–16.0)

9.5 (6.4–12.5)

7.6 (0.1–13.6)

0.2

6.4 * 10−2

—

Current psychiatric medication, n (%)

15 (18.5%)

2 (11.8%)

3 (27.3%)

9 (20.9%)

1 (10.0%)

6.5 * 10−1

—

   Antidepressants†, n (%)

7 (8.6%)

1 (5.9%)

2 (18.2%)

3 (7.0%)

1 (10.0%)

6.4 * 10−1

—

   Benzodiazepines, n (%)

9 (11.1%)

1 (5.9%)

1 (9.1%)

7 (16.3%)

0 (0.0%)

5.9 * 10−1

—

Unemployed%, n (%)

32 (39.5%)

9 (52.9%)

5 (45.5%)

15 (34.9%)

3 (30.0%)

5.4 * 10−1

—

   Unfit for work$, n (%)

20 (24.7%)

7 (41.2%)

2 (18.1%)

10 (23.3%)

1 (10.0%)

3.1 * 10−1

—

Single#, n (%)

30 (37.0%)

9 (52.9%)

4 (36.4%)

14 (32.6%)

3 (30.0%)

5.1 * 10−1

—

Years of education, mean (±SD)

13.8 ± 3.0

14.4 ± 2.8

13.9 ± 4.9

13.3 ± 2.7

14.9 ± 1.8

3.5 * 10−1

—

Depression*, n (%)

22 (27.2%)

3 (17.6%)

3 (27.3%)

12 (27.9%)

4 (40.0%)

6.6 * 10−1

—

Burnout*, n (%)

12 (14.8%)

1 (5.9%)

0 (0.0%)

7 (16.3%)

4 (40.0%)

5.8 * 10−2

—

Smoking, n (%)

36 (44.4%)

6 (35.3%)

6 (54.5%)

21 (48.8%)

3 (30.0%)

5.6 * 10−1

—

Hypertension, n (%)

24 (29.6%)

2 (11.8%)

7 (63.6%)

14 (32.6%)

1 (10.0%)

1.5 * 10−2

‡

Diabetes mellitus type 2, n (%)

3 (3.7%)

0 (0.0%)

1 (9.1%)

2 (4.7%)

0 (0.0%)

1.0 * 100

—

Dyslipidemia, n (%)

11 (13.6%)

1 (5.9%)

4 (36.4%)

6 (14.0%)

0 (0.0%)

8.1 * 10−2

—

eGFR in ml/min/1.73 m2, median (range)

94.6 (11.4–141.0)

105.6 (25.4–141.0)

77.3 (11.4–109.9)

94.0 (45.6–131.1)

95.4 (73.6–118.3)

4.1 * 10−3

b < a,c,d

   eGFR <60 ml/min/1.73 m2, n (%)

11 (13.6%)

2 (11.8%)

4 (36.4%)

5 (11.6%)

0 (0.0%)

1.2*10−1

—

Albuminuria in mg/day, median (range)

32 (3–2761)

60 (5–921)

100 (7–2761)

21 (3–1426)

13.5 (4–1422)

7.4 * 10−2

—

   Albuminuria >A1

41 (50.6%)

12 (70.6%)

7 (63.6%)

21 (48.8%)

1 (10.0%)

1.5 * 10−2

d < a

LVMI in gr/m2, median (range)

62.7 (33.4–139.6)

78.3 (45.9–139.5)

64.7 (50.1–136.9)

55.9 (36.6–119.1)

44.7 (33.4–77.6)

1.5 * 10−4

c < a, d < a,b

LVMI >upper ref limit, n (%)

24/74 (32.4%)

9/17 (52.9%)

3/8 (37.5%)

11/39 (28.2%)

1/10 (10.0%)

1.2 * 10−1

—

   Cardiac fibrosis

23/72 (31.9%)

6/17 (35.3%)

2/6 (33.3%)

14/39 (35.9%)

1/10 (10.0%)

4.4 * 10−1

—

LysoGb3 before ERT in nmol/L, median (range)

8.2 (0.6–150.3)

99.0 (36.8–150.3)

5.0 (1.2–16.5)

7.8 (1.3–22.6)

1.9 (0.6–5.0)

5.5 * 10−11

b,c,d < a, d < c

  1. Continuous variables are presented as median (range) or mean (±SD) and discrete variables as number (percentages). Intergroup differences were tested, results <0.05 are in bold. If <0.05 then post-hoc tests were performed. For representation of the results of the post-hoc analyses we allocated a letter (a,b,c or d) to each subgroup.
  2. ERT = enzyme replacement therapy, eGFR = estimated glomerular filtration rate, LVMI = left ventricular mass index.
  3. †Antidepressants taken for neuropathic pain not included, %Includes three retirees, $Includes three patients regarded partially unfit for work, #Divorced, widowed, or no romantic relationship.
  4. *History of or current, as diagnosed by a general practitioner, psychologist or psychiatrist, ‡Post-hoc Fisher’s exact test was not significant after Bonferroni-Holm correction.
  5. Upper reference limit LVM: ♂ = 79/♀ = 75. Normal range lysoGb3 = 0.3–0.6 nmol/L. Albuminuria >A1 = > 30 mg/day.
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