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Table 5 Pulmonary function parameters in patients with BHDS ( VC, vital capacity; FEV1,-force

From: Novel folliculin gene mutations in Polish patients with Birt–Hogg–Dubé syndrome

Family

Patient

VC% pred

VC (L)

FEV1 (%pred)

FEV1 (L)

TLC (%pred)

TLC (L)

RV (%pred))

RV(L)

Tl,co

Tl,co (%pred)

TVPG (mmHg)

1

1.1

85

3.83

87

3.1

86

5.62

86

1.79

7.77

81

21

2

2.1

120

3.5

110

2.5

No

No

No

No

No

No

No

 

2.2

121

3.6

103

2.5

No

No

No

No

No

No

No

 

2.3

110

4.1

100

3.6

No

No

No

No

No

No

No

3

3.1

80

1.94

68

1.3

96

4.06

133

2.43

3.75

60

28

4

4.1

90

3.0

91

2.4

104

5.22

121

2.22

5.65

81

21

5

5.1

91

3.0

86

2.4

103

5.57

129

2.42

8.08

93

No

 

5.2

86

3.15

102

2.58

102

5.55

86

1.53

9.25

102

No

6

6.1

102

3.65

95

2.35

116

5.65

132

2.55

5.97

80

26

7

7.1

        

No

No

No

8

8.1

116

3.37

116

2.7

123

6.2

127

2.3

5.88

73

21

 

8.2

120

4.99

120

4.19

129

6.88

130

1.89

6.99

72

No

9

9.1

102

3.63

99

2.83

112

5.84

120

2.21

6.78

81

22

10

10.1

96

3.65

86

2.69

113

5.79

133

2.3

7.49

85

21

 

10.2

           

Mean (SD)

 

102.0 ± 15.1

3.45 ± 0.69

96.9 ± 14.6

2.68 ± 0.66

108.4 ± 12.8

5.64 ± 0.7

119.7 ± 18.33

2.16 ± 0.32

6.76 ± 1.53

80.8 ± 11.48

22.86 ± 2.9