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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.