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Table 4 NF1-associated deaths by selected underlying cause, stratified by age

From: Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

People died at age < 40 years

 

Number

  

Underlying cause of death (ICD-9 and ICD-10 Codes )

Observ.

Expect.

PMRs

95% CI

Neurofibromatosis (ICD-9: 237.7; ICD-10: Q85.0)

59

-

  

   Neoplasms, excluding neurofibromatosis (ICD-9: 140-239, excluding 237.7; ICD-10: C00-D48)

77

26.1

2.9

(2.33 - 3.68)

Malignant Neoplasms (ICD-9: 140-208; ICD-10: C00-C97)

68

24.6

2.8

(2.15 - 3.51)

Connective and other soft tissue (ICD-9:171; ICD-10: C49)

21

0.9

23.4

(14.45 - 35.71)

Brain (ICD-9:191; ICD-10: C71)

19

2.5

7.5

(4.50 - 11.67)

Other and unspecified parts of nervous system (ICD-9:192; ICD-10: C70; C72)

3

0.1

32.7

(6.58 - 95.68)

Neoplasm without specification of site (ICD-9:199; ICD-10: C80)

4

0.6

6.8

(1.82 - 17.37)

Benign Neoplasms (ICD-9:210-229; ICD-10: D10-D36)

2

0.2

11.0

(1.23 - 39.54)

Neoplasms of uncertain behavior and unspecified nature, excluding neurofibromatosis (ICD-9: 235-239, excluding 237.7; ICD-10: D37-D48)

7

1.4

5.1

(2.04 - 10.50)

TOTAL

151

   

People died at age ≥ 40 years

 

Number

  

Underlying cause of death (ICD-9 and ICD-10 Codes )

Observ.

Expect.

PMRs

95% CI

Neurofibromatosis (ICD-9: 237.7; ICD-10: Q85.0)

91

-

  

Connective and other soft tissue (ICD-9:171; ICD-10: C49)

14

0.7

20.8

(11.35 - 34.87)

Benign Neoplasms (ICD-9:210-229; ICD-10: D10-D36)

5

0.6

8.7

(2.79 - 20.22)

Neoplasms of uncertain behavior and unspecified nature, excluding neurofibromatosis (ICD-9: 235-239, excluding 237.7; ICD-10: D37-D48)

10

4.4

2.3

(1.09 - 4.19)

Diseases of the respiratory system (ICD-9: 460-519; ICD-10: J00-J99)

30

18.2

1.6

(1.11 - 2.35)

Obstructive chronic bronchitis (ICD-9: 491.2; ICD-10: J44.8)

13

6.4

2.0

(1.08 - 3.47)

Diseases of the musculoskeletal system and connective tissue (ICD-9:710-739; ICD-10: M00-M99)

5

1.4

3.6

(1.15 - 8.34)

TOTAL

380

   
  1. Italy, 1995-2003 and 2006.