Skip to main content

Table 4 Results of Kaplan–Meier analysis, log-rank test, and Cox regression survival analysis showing bivariate (unadjusted) and multivariate (adjusted) HRs for variables associated with mortality

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

 

Mortality, n (%)

Total patients

Log-rank test

Crude HR (95% CI)

P value

Adjusted HR (95% CI)

P value

Enrollment age

 ≤ 18

4 (1.26)

315

 

1.00

 

1.00

 

 > 18

10 (6.42)

156

0.0003

6.54 (2.03–21.04)

0.0016

5.97 (1.74–20.47)

0.0045

Sex

 Males

8 (3.44)

232

 

1.00

 

1.00

 

 Females

6 (2.52)

239

0.6011

0.75 (0.26–2.18)

0.6023

0.69 (0.24–2.02)

0.4971

Comorbidity

 Neurological symptoms

  Epilepsy

   No

4 (3.64)

110

 

1.00

 

1.00

 

   Yes

10 (2.78)

361

0.3575

0.58 (0.18–1.87)

0.3633

0.84 (0.25–2.82)

0.7829

  Cerebral degenerations

   No

14 (3.0)

463

 

1.00

 

1.00

 

   Yes

0 (0.0)

8

0.6026

–

–

–

–

  Multiple sclerosis

   No

13 (2.9)

447

 

1.00

 

1.00

 

   Yes

1 (4.2)

24

0.9193

1.11 (0.14–8.52)

0.9193

1.24 (0.12–12.52)

0.8554

  Infantile cerebral palsy

  No

12 (2.8)

424

 

1.00

 

1.00

 

  Yes

2 (4.3)

47

0.7852

1.23 (0.27–5.52)

0.7855

6.03 (0.97–37.46)

0.0540

  Other congenital nervous system anomalies

   No

11 (3.3)

329

 

1.00

 

1.00

 

   Yes

3 (2.1)

142

0.2588

0.49 (0.13–1.75)

0.2689

0.78 (0.17–3.69)

0.7567

 TANDs

  Dementias

   No

13 (2.96)

440

 

1.00

 

1.00

 

   Yes

1 (3.22)

31

0.9931

1.01 (0.13–7.72)

0.993

0.5 (0.06–3.97)

0.5119

  Psychotic conditions

   No

13 (3.3)

398

 

1.00

 

1.00

 

   Yes

1 (1.4)

73

0.3052

0.36 (0.05–2.76)

0.3261

–

–

  Neurotic disorders

   No

14 (3.1)

449

 

1.00

 

1.00

 

   Yes

0 (0.0)

22

0.3789

–

–

–

–

  Depression

   No

14 (3.0)

465

 

1.00

 

1.00

 

   Yes

0 (0.0)

6

0.6456

–

–

–

–

  ADHD

   No

14 (3.2)

440

 

1.00

 

1.00

 

   Yes

0 (0.0)

31

0.2862

–

–

–

–

  Developmental delays

   No

13 (4.1)

320

 

1.00

 

1.00

 

   Yes

1 (0.7)

151

0.0270

0.14(0.02–1.07)

0.0583

0.24 (0.02–2.34)

0.2195

 Any malignant neoplasms

  No

9 (2.96)

304

 

1.00

 

1.00

 

  Yes

5 (3.00)

167

0.5822

0.73 (0.24–2.22)

0.5836

0.62 (0.19–2.07)

0.4413

  Oral & pharyngeal cancer

   No

13 (3.0)

437

 

1.00

 

1.00

 

   Yes

1 (2.9)

34

0.7634

0.73(0.1–5.63)

0.7647

1.14 (0.1–12.48)

0.9161

  Cardiac cancer

   No

14 (3.0)

466

 

1.00

 

1.00

 

   Yes

0 (0.0)

5

0.7087

–

–

–

–

  Urological cancer

   No

13 (2.8)

460

   

1.00

 

   Yes

1 (9.1)

11

0.2763

2.94 (0.38–22.49)

0.2993

1.87 (0.17–21.26)

0.6121

  Brain cancer

   No

14 (3.1)

453

 

1.00

 

1.00

 

   Yes

0 (0.0)

18

0.4122

–

–

–

–

 Benign tumours

  Benign skin tumour

       

   No

14 (3.1)

457

 

1.00

 

1.00

 

   Yes

0 (0.0)

14

0.4934

–

–

–

–

  Benign urological tumour

   No

11 (2.4)

449

 

1.00

 

1.00

 

   Yes

3 (13.6)

22

0.0016

6.11 (1.70–22.03)

0.0057

3.34 (0.72–15.64)

0.1250

  Benign brain tumour

   No

13 (2.8)

458

 

1.00

 

1.00

 

   Yes

1 (7.7)

13

0.3333

2.63 (0.34–20.1)

0.3519

5.85 (0.52–66.02)

0.1533

 Other systemic manifestations

  Any renal diseases

   No

11 (2.50)

441

 

1.00

 

1.00

 

   Yes

3 (10.00)

30

0.0149

4.29 (1.19–15.41)

0.0256

2.25 (0.53–9.51)

0.2688

  Myocardial infarctions

   No

14 (2.98)

470

 

1.00

 

1.00

 

   Yes

0 (0.00)

1

0.8690

–

–

–

 

  Cerebrovascular diseases

       

   No

14 (2.98)

469

 

1.00

 

1.00

 

   Yes

0 (0.00)

2

0.8429

–

–

–

 

  Peptic ulcers

   No

13 (2.82)

461

 

1.00

 

1.00

 

   Yes

1 (10.00)

10

0.3113

2.74 (0.36–21.08)

0.3316

1.75 (0.18–17.48)

0.6327

  Diabetes mellitus

   No

14 (3.00)

466

 

1.00

 

1.00

 

   Yes

0 (0.00)

5

0.6947

–

–

–

 

  LAM

   No

14 (3.00)

468

 

1.00

 

1.00

 

   Yes

0 (0.00)

3

–

–

–

–

 
  1. Kaplan–Meier estimates of survival and log rank test were used to initially identify those variables associated with mortality. Crude hazard ratio (HR) and their P values were calculated by using the Cox proportional hazards regression model. Adjusted hazard ratio (HR) and their P values were calculated by using the stepwise Cox proportional hazards regression model. TAND, TSC-associated neuropsychiatric disorders; ADHD, attention-deficit hyperactivity disorder; LAM, lymphangioleiomyomatosis
Back to article page

Orphanet Journal of Rare Diseases

ISSN: 1750-1172