Background
Methods
Study design
Outcome measurement – Quality of life
Reference data: Comparison data for each participating country
France
Germany
The Netherlands
Poland
Sweden
UK
Statistics
Results
Variables | Categories | Number | % |
---|---|---|---|
Diagnosis | Turner syndrome | 301 | 28.9 |
Klinefelter Syndrome | 218 | 21.0 | |
47,XYY | 1 | 0.0 | |
46,XX CAH | 226 | 21.7 | |
46,XX DSD - XX gonadal dysgenesis - XX ovotesticular DSD - XX, males | 27 | 2.6 | |
46,XY DSD - complete/partial XY gonadal dysgenesis - complete/partial androgen insensitivity syndrome - androgen synthesis defects - severe hypospadias - others | 222 | 21.3 | |
45,X/46XY | 45 | 4.3 | |
Age | mean (SD) | 32.4 (13.6) | |
≤ 19 years | 193 | 18.6 | |
20–24 years | 185 | 17.8 | |
25–44 years | 458 | 44.0 | |
45–64 years | 178 | 17.1 | |
≥ 65 years | 26 | 2.5 | |
Gender | Female | 717 | 68.9 |
Male | 311 | 29.9 | |
Other than female or male | 12 | 1.2 | |
Education | Low (ESISCED 1–2) | 202 | 19.4 |
Middle (ESISCED 3–5) | 453 | 43.6 | |
High (ESISCED 6–7) | 266 | 25.6 | |
Other | 63 | 60.6 | |
Not available | 56 | 5.4 | |
Feeling about household income | Living comfortable on present income | 339 | 32.6 |
Coping with present income | 393 | 37.8 | |
Finding it (very) difficult to live on present income | 128 | 12.3 | |
Other | 16 | 1.5 | |
Not available | 164 | 15.8 | |
Relationship status | Being single | 246 | 23.7 |
Having a partner | 382 | 36.7 | |
Living with parents | 321 | 30.9 | |
Other | 37 | 3.6 | |
Not available | 54 | 5.2 | |
Health status | How is your health in general? Would you say it is … | ||
Very bad | 13 | 1.3 | |
Bad | 72 | 6.9 | |
Fair | 282 | 27.1 | |
Good | 494 | 47.5 | |
Very good | 123 | 11.8 | |
Not available | 56 | 5.4 | |
Do you have any longstanding illness? (apart from your condition) | |||
No | 461 | 44.3 | |
Don’t know or not available | 100 | 9.6 | |
Yes | 479 | 46.1 | |
If yes, physical health problem | 316 | – | |
Mental health problem | 37 | – | |
Both (physical and mental) | 83 | – | |
Not available | 43 | – |
WHOQOL-BREF domain | Overall | Cronbach’s α (std.) | Standard error Cronbach’s α | Guttman’s lambda 6 | ||
---|---|---|---|---|---|---|
Domain | n | Mean | SD | |||
1040 | ||||||
Physical health | ||||||
Range 0–100 | 984 | 70.1 | 18.4 | 0.82 | 0.015 | 0.82 |
Range 4–20 | 15.2 | 2.9 | ||||
Psychological | ||||||
Range 0–100 | 988 | 63.9 | 18.1 | 0.83 | 0.015 | 0.82 |
Range 4–20 | 14.2 | 2.9 | ||||
Social relationships | ||||||
Range 0–100 | 987 | 62.6 | 20.9 | 0.71 | 0.030 | 0.64 |
Range 4–20 | 14.0 | 3.3 | ||||
Environment | ||||||
Range 0–100 | 986 | 73.0 | 14.7 | 0.80 | 0.014 | 0.79 |
Range 4–20 | 15.7 | 2.4 |
Comparison to the reference population
Country | WHOQOL-BREF | Reference population | Study participants (dsd-LIFE) | p-value | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|
n | Mean | SD | n | Mean | SD | Mean | SD | ||||
[0–100] | [0–100] | [4-20] | [4-20] | ||||||||
France | |||||||||||
Physical health | Healthy | 12,186 | 80.2 | 11.0 | 249 | 71.2 | 16.8 | 15.4 | 2.7 | 4.1 E-36 | |
Psychological | 12,186 | 67.7 | 11.0 | 251 | 65.7 | 17.4 | 14.5 | 2.8 | 4.5 E-03 | ||
Social relationships | 12,186 | 75.4 | 11.0 | 250 | 65.7 | 19.6 | 14.5 | 3.1 | 1.9 E-41 | ||
Environment | – | – | – | 249 | 73.9 | 14.6 | 15.7 | 2.3 | – | ||
Physical health | Non-healthy | 4192 | 66.4 | 19.4 | 249 | 71.2 | 16.8 | 15.4 | 2.7 | 1.4 E-04 | |
Psychological | 4192 | 64.9 | 13.0 | 251 | 65.7 | 17.4 | 14.5 | 2.8 | n.s.* | ||
Social relationships | 4192 | 71.5 | 19.4 | 250 | 65.7 | 19.6 | 14.5 | 3.1 | 3.9 E-06 | ||
Environment | – | – | – | 249 | 73.3 | 14.6 | 15.7 | 2.3 | – | ||
Germany | |||||||||||
Physical health | Healthy | 2052 | 76.9 | 17.7 | 238 | 73.0 | 19.3 | 15.7 | 3.1 | 1.3 E-03 | |
Psychological | 2055 | 74.0 | 15.7 | 239 | 65.0 | 19.3 | 14.4 | 3.1 | 4.6 E-16 | ||
Social relationships | 2048 | 71.8 | 18.5 | 239 | 62.3 | 22.2 | 14.0 | 3.6 | 2.9 E-13 | ||
Environment | 2053 | 70.4 | 14.2 | 239 | 75.1 | 14.6 | 16.0 | 2.3 | 1.6 E-06 | ||
Physical health | Non-healthy (physical) | 261 | 53.4 | 20.3 | 238 | 73.0 | 19.3 | 15.7 | 3.1 | 2.0 E-25 | |
Psychological | 261 | 62.7 | 16.3 | 239 | 65.0 | 19.3 | 14.4 | 3.1 | n.s.* | ||
Social relationships | 261 | 68.0 | 16.9 | 239 | 62.3 | 22.2 | 14.0 | 3.6 | 1.3 E-03 | ||
Environment | 261 | 67.2 | 13.4 | 239 | 75.1 | 14.6 | 16.0 | 2.3 | 7.4 E-10 | ||
Physical health | Non-healthy (mental) | 98 | 58.5 | 17.7 | 238 | 73.0 | 19.3 | 15.7 | 3.1 | 5.1 E-10 | |
Psychological | 98 | 52.0 | 19.5 | 239 | 65.0 | 19.3 | 14.4 | 3.1 | 4.4 E-08 | ||
Social relationships | 98 | 50.9 | 26.5 | 239 | 62.3 | 22.2 | 14.0 | 3.6 | 6.3 E-05 | ||
Environment | 98 | 59.3 | 18.8 | 239 | 75.1 | 14.6 | 16.0 | 2.3 | 3.4 E-15 | ||
Netherlands | |||||||||||
Physical health | Healthy | 218 | 15.2 | 2.6 | 229 | 67.3 | 20.2 | 14.8 | 3.2 | n.s.* | |
Psychological | 218 | 14.4 | 2.0 | 230 | 61.9 | 18.1 | 13.9 | 2.9 | n.s.* | ||
Social relationships | 218 | 15.4 | 2.9 | 230 | 62.6 | 21.0 | 14.0 | 3.4 | 3.9 E-06 | ||
Environment | 218 | 15.8 | 2.0 | 230 | 72.6 | 15.1 | 15.6 | 2.4 | n.s.* | ||
Physical health | Non-healthy | 410 | 11.8 | 3.0 | 229 | 67.3 | 20.2 | 14.8 | 3.2 | 4.7 E-29 | |
Psychological | 410 | 10.5 | 2.5 | 230 | 61.9 | 18.1 | 13.9 | 2.9 | 1.4 E-46 | ||
Social relationships | 410 | 12.8 | 3.5 | 230 | 62.6 | 21.0 | 14.0 | 3.4 | 3.3 E-05 | ||
Environment | 410 | 13.5 | 2.5 | 230 | 72.6 | 15.1 | 15.6 | 2.4 | 1.3 E-22 | ||
Poland | |||||||||||
Physical health | Healthy | 438 | 15.8 | 2.2 | 107 | 69.1 | 14.0 | 15.1 | 2.2 | 2.5 E-03 | |
Psychological | 438 | 13.8 | 2.5 | 107 | 63.5 | 17.4 | 14.2 | 2.8 | n.s.* | ||
Social relationships | 438 | 14.9 | 3.0 | 107 | 59.5 | 19.1 | 13.5 | 3.1 | 4.5 E-05 | ||
Environment | 438 | 13.1 | 2.4 | 107 | 71.0 | 13.1 | 15.4 | 2.1 | 1.2 E-17 | ||
Physical health | Non-healthy | 470 | 13.1 | 2.7 | 107 | 69.1 | 14.0 | 15.1 | 2.2 | 7.0 E-12 | |
Psychological | 470 | 12.5 | 2.6 | 107 | 63.5 | 17.4 | 14.2 | 2.8 | 6.8 E-09 | ||
Social relationships | 470 | 13.4 | 3.1 | 107 | 59.5 | 19.1 | 13.5 | 3.1 | n.s.* | ||
Environment | 470 | 12.8 | 2.4 | 107 | 71.0 | 13.1 | 15.4 | 2.1 | 5.8 E-23 | ||
Sweden | |||||||||||
Physical health | – | – | – | 119 | 70.3 | 18.0 | 15.3 | 2.9 | – | ||
Psychological | – | – | – | 119 | 63.7 | 17.3 | 14.2 | 2.8 | – | ||
Social relationships | – | – | – | 119 | 61.3 | 20.6 | 13.8 | 3.3 | – | ||
Environment | – | – | – | 119 | 72.5 | 14.0 | 15.6 | 2.2 | – | ||
United Kingdom | |||||||||||
Physical health | Healthy | 1324 | 76.5 | 16.2 | 42 | 64.1 | 20.1 | 14.3 | 3.2 | 1.5 E-06 | |
Psychological | 1324 | 67.8 | 15.6 | 42 | 60.4 | 17.5 | 13.7 | 2.8 | 2.6 E-03 | ||
Social relationships | 1324 | 70.5 | 20.7 | 42 | 57.9 | 24.0 | 13.3 | 3.8 | 1.2 E-04 | ||
Environment | 1324 | 68.2 | 13.8 | 42 | 68.5 | 18.3 | 15.0 | 2.9 | n.s.* | ||
Physical health | Non-healthy | 1864 | 57.0 | 22.7 | 42 | 64.1 | 20.1 | 14.3 | 3.2 | 4.4 E-02 | |
Psychological | 1864 | 58.3 | 19.3 | 42 | 60.4 | 17.5 | 13.7 | 2.8 | n.s.* | ||
Social relationships | 1864 | 62.8 | 23.2 | 42 | 57.9 | 24.0 | 13.3 | 3.8 | n.s.* | ||
Environment | 1864 | 64.9 | 16.9 | 42 | 68.5 | 18.3 | 15.0 | 2.9 | n.s.* |
QOL per five diagnosis groups
WHOQOL-BREF domain | Turner Syndrome | Klinefelter Syndrome | CAH | XY-DSD female | XY-DSD male |
p
| ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Domain | n | Mean | SD | n | Mean | SD | n | Mean | SD | n | Mean | SD | n | Mean | SD | |
301
|
213
|
221
|
194
|
93
| ||||||||||||
Physical health | ||||||||||||||||
Range 0–100 | 283 | 71.5 | 16.8 | 200 | 66.4 | 19.4 | 211 | 68.1 | 18.9 | 187 | 73.9 | 17.9 | 86 | 73.1 | 18.1 | < 0.001 |
Range 4–20 | 15.4 | 2.7 | 14.6 | 3.1 | 14.9 | 3.0 | 15.8 | 2.9 | 15.7 | 2.9 | ||||||
Psychological | ||||||||||||||||
Range 0–100 | 284 | 63.7 | 16.1 | 201 | 63.3 | 17.8 | 211 | 65.6 | 18.4 | 188 | 64.6 | 18.9 | 87 | 63.2 | 20.2 | 0.663 |
Range 4–20 | 14.2 | 2.6 | 14.1 | 2.8 | 14.5 | 2.9 | 14.3 | 3.0 | 14.1 | 3.2 | ||||||
Social relationships | ||||||||||||||||
Range 0–100 | 283 | 65.9 | 18.6 | 201 | 59.4 | 21.9 | 211 | 64.8 | 20.5 | 188 | 62.6 | 20.7 | 87 | 57.2 | 23.4 | < 0.001 |
Range 4–20 | 14.5 | 3.0 | 13.5 | 3.5 | 14.4 | 3.3 | 14.0 | 3.3 | 13.2 | 3.7 | ||||||
Environment | ||||||||||||||||
Range 0–100 | 283 | 73.9 | 12.9 | 201 | 69.9 | 14.9 | 211 | 74.1 | 15.7 | 187 | 75.0 | 15.4 | 87 | 72.2 | 13.8 | 0.005 |
Range 4–20 | 15.8 | 2.1 | 15.2 | 2.4 | 15.9 | 2.5 | 16.0 | 2.5 | 15.6 | 2.2 |
Variance in QOL
Physical health (n = 960) | Psychological (n = 963) | Social relationships (n = 962) | Environment (n = 961) | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Variable | Categories | ß | 95% CI | p-value | p R2 (%) | ß | 95% CI | p-value | p R2 (%) | ß | 95% CI | p-value | p R2 (%) | ß | 95% CI | p-value | p R2 (%) |
Age | −0.1 | −0.2, −0.0 | 0.006 | 5 | −0.0 | −0.1, 0.1 | 0.875 | 2 | −0.2 | −0.3, −0.1 | 0.006 | 5 | 0.0 | −0.0, 0.1 | 0.360 | 1 | |
Feeling about households income | 14 | 14 | 10 | 33 | |||||||||||||
Living comfortable on present income | 0.0 | 0.0 | 0.0 | 0.0 | |||||||||||||
Coping with present income | −3.4 | −5.3, − 1.5 | < 0.001 | −3.0 | −5.2, − 0.9 | 0.006 | −3.4 | −6.0, − 0.8 | 0.009 | −4.5 | −6.2, −2.8 | < 0.001 | |||||
(Very) difficult to live on present income | −9.0 | − 11.8, −6.2 | < 0.001 | −6.9 | − 10.0, − 3.8 | < 0.001 | − 5.2 | −9.0, − 1.5 | 0.006 | −12.2 | −14.6, −9.7 | < 0.001 | |||||
Relationship status | 1 | 2 | 15 | 0 | |||||||||||||
Being single | 0.0 | 0.0 | 0.0 | 0.0 | |||||||||||||
Having a partner | 0.3 | −2.0, 2.5 | 0.806 | 2.9 | 0.4, 5.4 | 0.025 | 9.6 | 6.5, 12.6 | < 0.001 | −0.5 | −2.5, 1.5 | 0.637 | |||||
Living with parents | − 0.5 | −3.1, 2.2 | 0.727 | 1.2 | −1.8, 4.2 | 0.425 | 3.2 | −0.4, 6.7 | 0.081 | −0.2 | −2.5, 2.2 | 0.887 | |||||
Other | −1.6 | −6.6, 3.5 | 0.544 | 1.1 | −4.5, 6.7 | 0.697 | −0.9 | −7.6, 5.9 | 0.796 | −2.0 | −6.5, 2.4 | 0.366 | |||||
Health status | 77 | 81 | 62 | 63 | |||||||||||||
Very bad | 0.0 | 0.0 | 0.0 | 0.0 | |||||||||||||
Bad | 16.0 | 7.9, 24.2 | < 0.001 | 7.9 | −1.3, 17.0 | 0.092 | 5.8 | −5.3, 16.7 | 0.305 | 14.1 | 6.9, 21.4 | < 0.001 | |||||
Fair | 28.4 | 20.7, 26.1 | < 0.001 | 15.4 | 6.8, 24.0 | < 0.001 | 11.6 | 1.3, 22.0 | 0.027 | 17.9 | 11.1, 24.6 | < 0.001 | |||||
Good | 41.7 | 34.0, 49.4 | < 0.001 | 25.6 | 17.0, 34.2 | < 0.001 | 21.9 | 11.6, 32.2 | < 0.001 | 26.0 | 19.2, 32.8 | < 0.001 | |||||
Very good | 52.3 | 44.3, 60.2 | < 0.001 | 38.3 | 29.4, 47.2 | < 0.001 | 31.8 | 21.1, 42.5 | < 0.001 | 34.1 | 27.1, 41.1 | < 0.001 | |||||
Diagnosis | 3 | 1 | 7 | 2 | |||||||||||||
Turner | 0.0 | 0.0 | 0.0 | 0.0 | |||||||||||||
Klinefelter | −0.7 | −3.3; 1.9 | 0.593 | 1.8 | −1.1, 4.7 | 0.221 | −4.4 | −7.9, −1-0 | 0.012 | −1.8 | −4.1, 0.5 | 0.124 | |||||
CAH | −3.2 | −5.6; −0.7 | 0.012 | 1.7 | −1.0, 4.5 | 0.219 | −1.6 | −4.9, 1.8 | 0.360 | 0.4 | −1.8, 2.6 | 0.698 | |||||
XY-DSD female | 1.1 | −1.5; 3.7 | 0.398 | −0.7 | −3.6, 2.2 | 0.630 | −4.8 | −8.3, −1.4 | 0.006 | 0.2 | −2.1, 2.4 | 0.893 | |||||
XY-DSD male | 3.2 | −0.2; 6.6 | 0.066 | 0.9 | −2.9, 4.7 | 0.647 | −7.9 | −12.5, −3.4 | < 0.001 | −0.6 | − 3.6, 2.4 | 0.678 | |||||
Model fit | R2 (%) | 45 | R2 (%) | 28 | R2 (%) | 23 | R2 (%) | 33 |