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Table 2 Stage progression rates and incidence rates of stage progression of NF1 from 2009 to 2012 by age and clinical stage at registration in 2008

From: Evaluation of neurofibromatosis type 1 progression using a nationwide registry of patients who submitted claims for medical expense subsidies in Japan between 2008 and 2012

 

No. of cases (A)

No. of cases with renewal claim (B)

No. of cases with no renewal claims

Renewal rate (B/A)

No. of cases with stage progression (C)

No. of cases with dermatological progression

No. of cases with neurological progression

No. of cases with bone progression

Person-years (D) a)

Stage progression rate (C/A) b)

Incidence rate per 100 person-years (C/D)

Total

342

205

137

60%

30

13

16

8

245.0

19%

12.2

Age group

 0–19 yrs

74

47

27

64%

7

1

4

4

28.0

26%

25.0

 20–39 yrs

109

65

44

60%

10

6

7

1

89.0

19%

11.2

 40–59 yrs

89

53

36

60%

6

4

2

1

77.5

12%

7.7

  > 60 yrs

70

40

30

57%

7

2

3

2

50.5

21%

13.9

Clinical stage

 Stage 1, 2

57

19

38

33%

10

6

7

2

63.0

18%

15.9

 Stage 3, 4

105

67

38

64%

20

7

9

6

182.0

19%

11.0

 Stage 5

180

119

61

66%

    

 

Age×clinical stage

 0–19 yrs

           

  Stage 1, 2

18

7

11

39%

3

0

2

1

20.0

17%

15.0

  Stage 3, 4

9

5

4

56%

4

1

2

3

8.0

44%

50.0

  Stage 5

47

35

12

74%

    

 

 20–39 yrs

  Stage 1, 2

24

9

15

38%

6

5

4

1

26.5

25%

22.6

  Stage 3, 4

29

20

9

69%

4

1

3

0

62.5

14%

6.4

  Stage 5

56

36

20

64%

    

 

 40–59 yrs

  Stage 1, 2

8

2

6

25%

1

1

1

0

10.5

13%

9.5

  Stage 3, 4

41

23

18

56%

5

3

1

1

67.0

12%

7.5

  Stage 5

40

28

12

70%

    

 

> 60 yrs

  Stage 1, 2

7

1

6

14%

0

6.0

0%

0.0

  Stage 3, 4

26

19

7

73%

7

2

3

2

44.5

27%

15.7

  Stage 5

37

20

17

54%

    

 
  1. a) Sum of observation periods of Stage 1 to Stage 4 patients at registration
  2. b) Total number of Stage 1 to Stage 4 patients at registration was used as the denominator to calculate the rate