| Enrollment age cohorts | P value | Sex cohorts | P value | ||
---|---|---|---|---|---|---|
Enrollment age ≤ 18 | Enrollment age > 18 | Males | Females | |||
Survival time after enrollment (mortality group) | ||||||
 Mean (SD) | 3.7 (2.8) | 2.2 (1.9) | 0.2743 | 1.6 (1.5) | 4.0 (2.2) | 0.0356 |
 Median (IQR) | 3.8 (1.8–5.5) | 2.0 (0.7–3.0) | 0.2579 | 1.3 (0.3–2.7) | 3.2 (2.0–6.7) | 0.0707 |
Mortality rates and IRR | ||||||
 Death/total subjects | 4/315 (1.27) | 10/156 (6.41) |  | 8/232 (3.45) | 6/239 (2.51) |  |
 Person-years survived for all subjects | 2038.64 | 711.53 |  | 1385.37 | 1364.79 |  |
 MR per 1000 person-years | 1.96 | 14.05 |  | 5.77 | 4.40 |  |
  95% CI | 1.88–2.05 | 13.06–15.13 |  | 5.48–6.09 | 4.17–4.64 |  |
 Crude IRR | 1.00 | 7.16 | 0.0009 | 1.00 | 0.76 | 0.6136 |
  95% CI |  | 2.25–22.84 |  |  | 0.26–2.19 |  |
 Adjusted IRR | 1.00 | 6.60 | 0.0025 | 1.00 | 0.72 | 0.5461 |
  95% CI |  | 1.95–22.4 |  |  | 0.25–2.09 |  |
Comorbidities, n (% of cohort) | ||||||
 Neurological symptoms | ||||||
  Epilepsy | 259 (82.2) | 102 (65.4) | < 0.0001 | 182(78.4) | 179(74.9) | 0.3848 |
  Cerebral degenerations | 4 (1.3) | 4 (2.6) | 0.4491 | 4 (1.7) | 4 (1.7) | 1.00 |
  Multiple Sclerosis | 14 (4.4) | 10 (6.4) | 0.3781 | 13 (5.6) | 11 (4.6) | 0.68 |
  Infantile cerebral palsy | 44 (14.0) | 3 (1.9) |  < .0001 | 23 (9.9) | 24 (10.0) | 1.00 |
  Other congenital nervous system anomalies | 110 (34.9) | 32 (20.5) | 0.0013 | 67 (28.9) | 75 (31.4) | 0.62 |
 TANDs | ||||||
  Dementias | 10 (3.2) | 21 (13.5) |  < 0.0001 | 19(8.2) | 12(5) | 0.1948 |
  Psychotic conditions | 42 (13.3) | 31 (19.9) | 0.065 | 42 (18.1) | 31 (13.0) | 0.13 |
  Neurotic disorders | 6 (1.9) | 16 (10.3) | 0.0001 | 12 (5.2) | 10 (4.2) | 0.67 |
  Depression | 1 (0.3) | 5 (3.2) | 0.0167 | 2 (0.9) | 4 (1.7) | 0.68 |
  ADHD | 31 (9.8) | 0 (0.0) | < 0.0001 | 17 (7.3) | 14 (5.9) | 0.58 |
  Development delays | 135 (42.9) | 16 (10.3) |  < 0.0001 | 78 (33.6) | 73 (30.5) | 0.49 |
 Any malignant neoplasms | 116 (36.8) | 51 (32.7) | 0.4135 | 77(33.2) | 90(37.7) | 0.3359 |
  Oral and pharyngeal cancer | 29 (9.2) | 5 (3.2) | 0.0218 | 17 (7.3) | 17 (7.1) | 1.00 |
  Cardiac cancer | 4 (1.3) | 1 (0.6) | 1.0000 | 2 (0.9) | 3 (1.3) | 1.00 |
  Urological cancer | 4 (1.3) | 7 (4.5) | 0.0469 | 6 (2.6) | 5 (2.1) | 0.77 |
  Brain cancer | 11 (3.5) | 7 (4.5) | 0.6146 | 7 (3.0) | 11 (4.6) | 0.47 |
 Benign tumours |  |  |  |  |  |  |
  Benign skin tumours | 6 (1.9) | 8 (5.1) | 0.0796 | 6 (2.6) | 8 (3.3) | 0.79 |
  Benign urological tumours | 5 (1.6) | 17 (10.9) |  < 0.0001 | 5 (2.2) | 17 (7.1) | 0.01 |
  Benign brain tumours | 8 (2.5) | 5 (3.2) | 0.7667 | 7 (3.0) | 6 (2.6) | 0.78 |
 Other systemic manifestations | ||||||
  Any renal diseases | 7 (2.2) | 23 (14.7) |  < 0.0001 | 13(5.6) | 17(7.1) | 0.5734 |
  Myocardial infarction | 1 (0.3) | 0 (0.0) | 1.0000 | 1(0.4) | 0(0) | 0.4926 |
  Cerebrovascular diseases | 1 (0.3) | 1 (0.6) | 0.5532 | 1(0.4) | 1(0.4) | 1.0000 |
  Peptic ulcers | 4 (1.3) | 6 (3.8) | 0.0893 | 5(2.2) | 5(2.1) | 1.0000 |
  Diabetes mellitus | 1 (0.3) | 4 (2.6) | 0.0434 | 2(0.9) | 3(1.3) | 1.0000 |
  LAM | 0 (0.0) | 3 (0.0) | 1.0000 | 1 (0.4) | 2 (0.9) | 1.0000 |