Table 1.
Characteristics of the patients
| Total cohort | Population cohort | Referral cohort | Year of diagnosis | ||
|---|---|---|---|---|---|
| 1999 or earlier | 2000 onwards | ||||
| Patients, n (%) | 97 | 78 | 19 | 25(26) | 72(74) |
| Female, n (%) | 86 (89) | 69 (93) | 17 (89) | 24 (96) | 62 (86) |
| Caucasian, n (%) | 77 (79) | 59 (80) | 15 (79) | 21 (84) | 56 (78) |
| Asian, n (%) | 12 (12) | 4 (16) | 8 (11) | ||
| African, n (%) | 7 (7) | 0 (0) | 7 (10) | ||
| Age at onset, mean (SD) | 28.8 (13) | 30.4 (14) | 26.3 (11) | 27.3 (12)a | 29.2 (13)b |
| Age at diagnosis, mean (SD) | 33.9 (15) | 33.9 (15) | 32.6 (14) | 29.3 (13) | 34.4 (15) |
| Age <16 years at onset, n (%) | 12 (12) | 4 (16) | 8 (11) | ||
| Age <41 years at onset, n (%) | 76 (78) | 58 (74) | 18 (95)* | 21 (93) | 55 (77) |
| Age >50 years at onset, n (%) | 11 (11) | 8 (11) | 1 (5) | 2 (8) | 9 (13) |
| Follow up time (years), mean (SD) | 11.7 (12) | 27.5 (13) | 6.2 (3) | ||
| Deceased (by end of 2013), n (%) | 9 (9) | 5 (6) | 4 (21)* | 9 (38) | 0 (0) |
| Disease onset 1999 or earlier, n (%) | 39 (42) | ||||
| Disease onset from 2000 onwards, n (%) | 55 (58) | ||||
aAvailable in 16 patients. bAvailable in 68 patients. *p < 0.05 for the referral compared to the population cohort