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Table 2 Characteristics and assessment of the PAP cohort

From: Assessment and management of pulmonary alveolar proteinosis in a reference center

 

n.

%

m ± sd

 

73

  

Age

  

41 ± 12.8 (yrs)

Gender

   

Male

52

71

 

Female

21

29

 

Smoking status

   

Current

16

22

 

Former

33

45

 

Never

24

33

 

Exposures 1

26

36

 

Symptoms at enrollment

   

Symptomatic

69

95

 

dyspnea2

49

67

 

cough

23

31

 

fever

8

11

 

sputum production

1

1

 

Asymptomatic

4

5

 

Diagnostic procedures 3

69

  

HR CT Scan

51

74

 

Biopsy

41

56

 

BAL

32

46

 

GMAbs4

31

45

 

Infection at enrollment 4

14

19

 

Lung Function 5

   

FVC

57

 

75.50 ± 21.1 (% predicted)

FEV1

57

 

77.77 ± 18.49 (% predicted)

TLC

57

 

75.38 ± 14.77 (% predicted)

DLCO

57

 

51.83 ±17.90 (% predicted)

PaO2

57

 

64.46 ± 14.17 (mm Hg)

ΔA-aO2

57

 

41.44 ± 16.25 (mm Hg)

Exercise Sat.O2

57

 

-4.27% ± 1.93

Biomarkers 6

   

Cyfra 21-1

26

 

12.8 ± 14.6 (ng/mL) (nv: 0.0-3.3)

CEA

27

 

13.4 ± 13.4 (ng/mL) (nv: 0.0-5.0)

NSE

23

 

20.04 ± 6.9 (ng/mL) (nv: 0.0-15.0)

LDH

57

 

550.3 ± 248 (U/L) (nv: 230-460)

GMAbs

31

 

184.1 ± 175 (μg/ml) (nv < 3)

DSS 7

39

  

1

2

5

 

2

12

31

 

3

13

33

 

4

4

10

 

5

8

21

 
  1. 1Subjects exposed for work-related reasons to dust, smoke or gas of organic or inorganic origin. 2Patients may report more than one symptom.3No data available in 4/73 pts. More than one diagnostic tool was applied in 58 PAP patients (84%). Measurement of GMAbs began in 2004.4These include respiratory infections in 12 patients (4 Staph.aureus, 3 Str.pneumoniae, 1 Pn.jiroveci, 1 Kleb.pneumoniae, 1 Nocardia ast., 1 Serratia Marcesc., 1 Candida Alb) and 2 isolates of H.pilorii from gastric biopsy. 5Abbreviation legends: FVC: forced vital capacitiy; FEV1: forced expiratory flow in the first second; TLC: total lung capacity; DLCO: diffusing lung capacity for carbon monoxide; PaO2: arterial oxygen tension; ΔA-aO2: alveolar to arterial oxygen tension difference;%predicted: percentage of the predicted value; nv: normal value. 6Abbreviation legends: Cyfra 21-1: cytokeratin 19 fragment 21-1; CEA: carcinoembrionic antigen; NSE: neuron specific enolase; LDH: lactate dehydrogenase. 7 DSS: disease severity score[10].