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Table 3 Summaries of 5-year survival from study entry according to potential prognostic factors (23 deceased patients)

From: Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Prognostic factors n 5-year survival percentages P-value*
Gender    0.7
   Women 141 86  
   Men 127 91  
Age at diagnosis    0.4
   0-15 years 22 81  
   16-30 years 59 92  
   31-45 years 115 89  
   46-60 years 61 82  
   >61 years 11 100  
Age at entry    0.03
   18-30 years 32 91  
   31-45 years 85 94  
   46-60 years 104 89  
   >61 years 47 77  
Nationality    0.7
   Netherlands 99 90  
   Germany 48 93  
   US 69 85  
   UK 20 67  
   Other° 32 93  
Disability level at study entry    0.002
   No wheelchair use or respiratory support ª 127 95  
   Wheelchair use 34 91  
   Use of respiratory support 39 89  
   Both wheelchair use and respiratory support 68 74  
RHS score at study entry*    0.002
   1 85 74  
   2 85 95  
   3 88 97  
  1. * Log rank test for the Kaplan-Meier curves, overall p-values of univariate analyses. ° Due to small groups Canada and Australia were classified into the category "other". ª 'Respiratory support 'includes partial and fulltime, invasive and non-invasive respiratory support. *RHS score was divided into tertiles for comparison. 1) < 23, 2) 23-30, 3)>30.