| Total | Mortality group | Surviving group |  |
---|---|---|---|---|
 | N | N | N | P value |
 | 471 | 14 (3.0%) | 457 (97.0%) |  |
Sex, n (%) | ||||
 Male | 232 (49.3) | 8 (57.1) | 224 (49.0) |  |
 Female | 239 (50.7) | 6 (42.9) | 233 (51.0) | 0.5970 |
Age at endpoint (death or end of queried period), years | ||||
 Mean (SD) | 20.9 (13.3) | 32.4 (17.3) | 20.4 (13.0) |  < 0.0001 |
 Median (IQR) | 17.8 (11.5–27.2) | 32.8 (23.4–43.8) | 17.5 (11.3–26.4) | 0.0006 |
Cohorts by age at endpoint, n (%) | ||||
 < 1 year | 2 (0.4) | 1 (7.1) | 1 (0.2) |  |
 1–18 years old | 235 (49.9) | 2 (14.3) | 233 (51.0) |  |
 19–40 years old | 196 (41.6) | 6 (42.9) | 190 (41.6) |  |
 > 40 years old | 38 (8.1) | 5 (35.7) | 33 (7.2) |  < 0.0001 |
Age at enrollment (date of CIC acquisition), years | ||||
 Mean (SD) | 15.0 (13.8) | 29.7 (17.8) | 14.5 (13.4) |  < 0.0001 |
 Median (IQR) | 10.9 (4.1–21.9) | 31.6 (16.6–42.6) | 10.8 (4.0–21.3) | 0.0015 |
Cohorts by age at enrollment, n (%) | ||||
 < 1 year | 36 (7.6) | 1 (7.1) | 35 (7.7) |  |
 1–18 years old | 279 (59.2) | 3 (21.4) | 276 (60.4) |  |
 19–40 years old | 129 (27.4) | 5 (35.7) | 124 (27.1) |  |
 > 40 years old | 27 (5.7) | 5 (35.7) | 22 (4.8) |  < 0.0001 |
 ≤ 18 years old | 315 (66.9) | 4 (28.6) | 311 (68.1) |  |
 > 18 years old | 156 (33.1) | 10 (71.4) | 146 (31.9) | 0.0034 |
Survived time from enrollment to endpoint, years | ||||
 Mean (SD) | 5.8 (3.4) | 2.6 (2.1) | 5.9 (3.4) | 0.0003 |
 Median (IQR) | 5.5 (3.1–8.1) | 2.2 (0.7–3.4) | 5.6 (3.2–8.1) | 0.0002 |
 Total | 2749.11 | 2712.29 | 36.82 |  |
Comorbidity (ICD-9-CM diagnostic codes queried), n (%) | ||||
 Neurological symptoms | ||||
  Epilepsy (345.x) | 361 (76.6) | 10 (71.4) | 351 (76.8) | 0.7479 |
  Cerebral degenerations (331.x) | 8 (1.7) | 0 (0.0) | 8 (1.8) | 1.0000 |
  Multiple sclerosis (340.x) | 24 (5.1) | 1 (7.1) | 23 (5.0) | 0.5242 |
  Infantile cerebral palsy (343.x) | 47 (10.0) | 2 (14.3) | 45 (9.8) | 0.6405 |
  Other congenital nervous system anomalies (742.x) | 142 (30.1) | 3 (21.4) | 139 (30.4) | 0.5679 |
 TANDs | ||||
  Dementias (290.x, 291.x, 294.x) | 31 (6.6) | 1 (7.1) | 30 (6.6) | 1.0000 |
  Psychotic conditions (293.x–299.x) | 73 (15.5) | 1 (7.1) | 72 (15.8) | 0.7062 |
  Neurotic disorders (300.x) | 22 (4.7) | 0 (0.0) | 22 (4.8) | 1.0000 |
  Depression (311.x) | 6 (1.3) | 0 (0.0) | 6 (1.3) | 1.0000 |
  ADHD (314.x) | 31 (6.6) | 0 (0.0) | 31 (6.8) | 0.6136 |
  Developmental delays (315.x–319.x) | 151 (32.1) | 1 (7.1) | 150 (32.8) | 0.0442 |
 Any malignant neoplasms (140.x–195.x) | 167 (35.5) | 5 (35.7) | 162 (35.4) | 1.0000 |
  Oral and pharyngeal cancers (140.x–149.x) | 34 (7.2) | 1 (7.1) | 33 (7.2) | 1.0000 |
  Cardiac cancer (164.1) | 5 (1.1) | 0 (0.0) | 5 (1.1) | 1.0000 |
  Urological cancers (189.x) | 11 (2.3) | 1 (7.1) | 10 (2.2) | 0.2851 |
  Brain cancer (191.x) | 18 (3.8) | 0 (0.0) | 18 (3.9) | 1.0000 |
 Benign tumours |  |  |  |  |
  Benign skin tumours (216.x) | 14 (3.0) | 0 (0.0) | 14 (3.1) | 1.0000 |
  Benign urological tumours (223.x) | 22 (4.7) | 3 (21.4) | 19 (4.2) | 0.0231 |
  Benign brain tumours (225.x) | 13 (2.8) | 1 (7.1) | 12 (2.6) | 0.3280 |
 Other systemic manifestations |  |  |  |  |
  Any renal diseases (403.x, 404.x, 580.x-586.x) | 30 (6.4) | 3 (21.4) | 27 (5.9) | 0.0527 |
  Myocardial infarction (410.x, 411.x) | 1 (0.2) | 0 (0.0) | 1 (0.2) | 1.0000 |
  Cerebrovascular diseases (430.x-433.x, 435.x) | 2 (0.4) | 0 (0.0) | 2 (0.4) | 1.0000 |
  Peptic ulcers (531.x-534.x) | 10 (2.1) | 1 (7.1) | 9 (2.0) | 0.2627 |
  Diabetes mellitus (250.x) | 5 (1.1) | 0 (0.0) | 5 (1.1) | 1.0000 |
  LAM (516.4) | 3 (0.7) | 0 (0.0) | 3 (0.0) | 1.0000 |