Skip to main content

Table 1 Patient characteristics

From: Depressive symptoms in Fabry disease: the importance of coping, subjective health perception and pain

 

All

Men

Women

Classical

Non-classical

Classical

Non-classical

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)

Currently on ERT, n (%)

43 (53.1%)

15 (88.2%)

2 (18.2%)

25 (58.1%)

1 (10.0%)

Years treated with ERT, median (range)

1.6 (0.0–16.0)

12.4 (1.5–16.0)

0.0 (0.0–14.2)

1.6 (0.0–13.6)

0.0 (0.0–0.3)

Unemployeda, n (%)

32 (39.5%)

9 (52.9%)

5 (45.5%)

15 (34.9%)

3 (30.0%)

 Unfit for workb, n (%)

20 (24.7%)

7 (41.2%)

2 (18.1%)

10 (23.3%)

1 (10.0%)

Singlec, n (%)

30 (37.0%)

9 (52.9%)

4 (36.4%)

14 (32.6%)

3 (30.0%)

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

Depressiond, n (%)

22 (27.2%)

3 (17.6%)

3 (27.3%)

12 (27.9%)

4 (40.0%)

Burnoutd, n (%)

12 (14.8%)

1 (5.9%)

0 (0.0%)

7 (16.3%)

4 (40.0%)

Current psychiatric medication, n (%)

15 (18.5%)

2 (11.8%)

3 (27.3%)

9 (20.9%)

1 (10.0%)

 Antidepressants, n (%)

7 (8.6%)

1 (5.9%)

2 (18.2%)

3 (7.0%)

1 (10.0%)

 Benzodiazepines, n (%)

9 (11.1%)

1 (5.9%)

1 (9.1%)

7 (16.3%)

0 (0.0%)

Loneliness, n (%)

11 (13.6%)

2 (11.8%)

2 (18.2%)

6 (14.0%)

1 (10.0%)

Comorbidity, n (%)

40 (49.4%)

8 (47.1%)

10 (90.9%)

19 (44.2%)

3 (30.0%)

Left ventricular hypertrophye,f, n (%)

45 (55.6%)

13 (76.5%)

4 (36.4%)

24 (55.8%)

4 (40.0%)

Cardiac fibrosis, n (%)

23/72 (31.9%)

6/17 (35.3%)

2/6 (33.3%)

14/39 (35.9%)

1/10 (10.0%)

eGFR< 60 ml/min, n (%)

11 (13.6%)

2 (11.8%)

4 (36.4%)

5 (11.6%)

0 (0.0%)

Fazekas scoree,g, median (range)

1 (0–6)

0 (0–6)

1 (0–3)

1 (0–6)

0.5 (0–2)

Complications, n (%)

27 (33.3%)

7 (41.2%)

6 (54.5%)

14 (32.6%)

0 (0.0%)

 Cardiac, n (%)

14 (17.3%)

4 (23.5%)

4 (36.4%)

6 (14.0%)

0 (0.0%)

 Renal, n (%)

4 (4.9%)

1 (5.9%)

2 (18.2%)

1 (2.3%)

0 (0.0%)

 Stroke, n (%)

10 (12.3%)

2 (11.8%)

2 (18.2%)

6 (14.0%)

0 (0.0%)

  1. Continuous variables are presented as median (range) or mean (±SD) and discrete variables as number (percentages)
  2. ERT enzyme replacement therapy, eGFR estimated glomerular filtration rate
  3. aIncludes three retirees
  4. bIncludes three patients regarded partially unfit for work
  5. cUnmarried, divorced or widowed
  6. dHistory of or current, as diagnosed by a general practitioner, psychologist or psychiatrist
  7. eMRIs were unavailable in seven patients (three non-classical men, four classical women) due to presence of an MRI non-compatible pacemaker or ICD (n = 6) and due to claustrophobia (n = 1)
  8. fIf MRI of the heart was not available then presence of left ventricular hypertrophy on echocardiography was used
  9. gIn one patient the brain MRI was performed in a different hospital
Back to article page

Orphanet Journal of Rare Diseases

ISSN: 1750-1172