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Table 2 Association of variables with death and loss of autonomy in EPM2A and NHLRC1-related LD: multivariable analysis

From: Prognostic value of pathogenic variants in Lafora Disease: systematic review and meta-analysis of patient-level data

Variables

Death (n = 109)

Loss of autonomy (n = 52)

HR (95% CI)

P value

HR (95% CI)

P value

EPM2A

MS/MS

Reference

-

Reference

-

MS/PT

0.37 (0.06–2.03)

0.25

0.27 (0.07–1.09)

0.066

PT/PT

0.58 (0.21–1.62)

0.29

0.74 (0.34–1.64)

0.46

Age at onset 18 + versus < 18 years

0.65 (0.10–4.29)

0.66

4.16 (0.87–19)

0.073

Geographic origin

Asian versus European

7.60 (2.6222.0)

 < 0.001

4.83 (1.5814.9)

0.006

African versus European

–*

–*

American versus European

3.91 (1.2612.2)

0.018

2.70 (0.90–8.11)

0.076

Variables

Death (n = 141)

Loss of autonomy (n = 64)

HR (95% CI)

P value

HR (95% CI)

P value

NHLRC1

MS/MS

Reference

Reference

MS/PT

0.56 (0.12–2.68)

0.47

1.24 (0.46–3.32)

0.68

PT/PT

2.66 (1.10–6.39)

0.029

2.12 (1.04–4.31)

0.038

Age at onset 18 + versus < 18 years

0.20 (0.02–1.69)

0.14

0.41 (0.10–1.76)

0.23

Geographic origin

Asian versus European

1.17 (0.31–4.37)

0.82

1.10 (0.15–7.82)

0.93

African versus European

3.78 (0.21–67.2)

0.37

9.23 (0.24–354)

0.23

American versus European

0.86 (0.07–10.5)

0.91

0.82 (0.12–5.36)

0.83

  1. Bold text indicates statistical significance
  2. *Estimation was not possible due to everyone surviving (n = 3)