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Table 1 Participant characteristics by age 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

Age 0–19 yrs 20–39 yrs 40–59 yrs > 60 yrs Chi-square test
Total 74 109 89 70  
Sex
 Male 32 41 35 40  
 Female 42 68 54 30  
Dermatological manifestations a) (n = 340) *
 D1 24 9 4 3  
 D2 17 25 13 9  
 D3 13 42 47 38  
 D4 18 33 25 20  
Neurological manifestations b) (n = 333)
 N0 33 45 37 25  
 N1 20 47 33 29  
 N2 18 15 16 15  
Bone manifestations c) (n = 331) *
 B0 32 56 46 23  
 B1 16 27 30 37  
 B2 25 21 10 8  
Clinical stage d) (n = 342) *
 Stage 1, 2 18 24 8 7  
 Stage 3, 4 9 29 41 26  
 Stage 5 47 56 40 37  
  1. *P < 0.05
  2. a) Dermatological manifestations
  3. D1: Pigmented macules and few neurofibromas
  4. D2: Pigmented macules and many neurofibromas
  5. D3: Numerous neurofibromas (> 1000)
  6. D4: Functional impairment or severe physical distress due to plexiform neurofibromas, or malignant peripheral nerve sheath tumour
  7. b) Neurological manifestations
  8. N0: No neurological symptoms
  9. N1: Neurologic symptoms (e.g., paralysis or pain)
  10. N2: Severe/progressive neurological symptoms
  11. c) Bone manifestations
  12. B0: No bone lesions
  13. B1: Mild/moderate bone lesions (e.g., deformity in spine that does not require surgical treatment)
  14. B2: Severe bone lesions
  15. d) Classification of severity
  16. Stage 1: D1 and N0 and B0
  17. Stage 2: (D1 or D2) and (N0 or N1) and (B0 or B1)
  18. Stage 3: D3 and N0 and B0
  19. Stage 4: D3 and (N1 or B1)
  20. Stage 5: D4 or N2 or B2