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Table 2 Associations of survival time, mortality rates (MR), incidence rate ratios (IRR), and comorbidities with diagnosis age or sex in tuberous sclerosis complex (TSC) patients

From: A population-based study to estimate survival and standardized mortality of tuberous sclerosis complex (TSC) in Taiwan

 

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

  1. P value was calculated using the T-test, Wilcoxon Rank Sum test, or chi-squared test/Fisher exact test, as appropriate, for comparisons between enrollment age cohorts or sex cohorts. Incidence rate ratio (IRR) was calculated by using a generalized linear model to perform Poisson regression analyses. Adjusted IRR was calculated after adjustments were made for comorbidities of epilepsy, dementias, renal diseases, and diabetes, using a generalized linear model. Abbreviations: MR: mortality rate; IRR: incidence rate ratio; SD: standard deviation; IQR: inter-quartile range; TAND: TSC-associated neuropsychiatric disorders; ADHD: attention-deficit hyperactivity disorder; LAM: lymphangioleiomyomatosis