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Table 1 Patients' characteristics

From: A role for MCP-1/CCR2 in interstitial lung disease in children

No

Sex

Age [years]

Interstitial lung disease

Diagnosis finding

Radiographic findings

Fibrotic changes (CT)

ILD Score*

Dyspnoe

Cough

Cyanosis

Exercise Intolerance

Failure to thrive

Medication

FVC [% of pred.]

TLC % [of pred.]

1

F

7

LIP

CT, LB

• diffuse interstitial involvement

+

4

++

+

+

+

+

CS, AZT

34

56

     

• reticular-nodular pattern

          
     

• follicular bronchiolitis

          

2

M

14

U-ILD, IPH

CT, BAL

patchy interstitial involvement

-

2

+

-

-

-

-

CS

77

89

3

M

8

U-ILD

CT, LB

• ground-glass opacity

+

3

++

-

-

+

-

 

46

74

4

M

4

IPH

CT, BAL, LB

interstitial involvement

-

3

+

-

-

-

-

 

77

168

5

M

16

U-ILD

CT, BAL

interstitial involvement

+

2

+

-

-

+

+

 

76

95

6

F

7

U-ILD

CT, BAL

interstitial involvement

-

2

+

+

-

-

+

AZT

50

68

7

M

4

CPI

CT, LB

• diffuse infiltrates

+

3

+

-

-

+

+

AZT

58

64

     

• ground-glass opacity

          

8

F

3

NSIP

CT, LB

• interstitial involvement

+

3

++

-

+

+

+

CS

n.d.

n.d.

     

• alveolar infiltrates

          

9

M

8

Sarcoidosis

CT, BAL, LB

• interstitial involvement

+

2

++

+

-

+

+

CS

56

63

     

• perivascular nodules

          

10

F

8

U-ILD

CT, BAL, LB

ground-glass opacity

-

1

-

+

-

+

-

 

76

87

11

F

8

CPI

CT, LB

• interstitial involvement

+

2

+

+

-

+

+

CS

37

74

     

• ground-glass opacity

          

12

M

9

U-ILD

CT

interstitial involvement

-

2

+

-

-

-

-

 

70

98

13

M

5

NSIP

CT, LB

• interstitial involvement

+

3

++

-

-

+

-

CS

61

76

     

• ground-glass opacity

          

14

F

6

U-ILD

CT

reticular-nodular pattern

+

3

++

+

-

-

-

AZT

60

68

15

F

4

U-ILD

CT

interstitial involvement

+

2

+

+

-

-

+

 

n.d.

n.d.

16

M

12

U-ILD

CT

interstitial involvement

-

2

+

-

-

-

-

 

68

75

17

M

3

PAP†

CT, BAL, LB

• ground glass opacity

-

4

+++

+

+

+

+

CS

n.d.

n.d.

18

M

6

NSIP

CT, BAL, LB

• alveolar infiltrates

+

4

+++

+

+

+

+

CS, AZT

63

72

   

PAP

 

• ground glass opacification

          

19

F

3

PAP†

CT, BAL, LB

• ground glass opacity

+

4

++

-

+

+

+

CS

n.d.

n.d.

     

• alveolar infiltrates

          

20

F

9

NSIP

CT, LB

• interstitial involvement

+

3

++

+

+

+

+

CS, AZT

55

74

     

• honeycombing

          

21

M

7

U-ILD

CT

reticular-interstitial pattern

+

3

+

+

-

+

+

AZT, MT

38

59

22

M

7

Cholesterol

CT, BAL, LB

• interstitial involvement

+

4

+++

+

+

+

+

CS

16

24

   

pneumonitis†

 

• reticular-interstitial pattern

          

23

M

4

U-ILD

CT, LB

• interstitial involvement

-

2

+

-

-

+

+

CS

102

99

     

• honeycombing

          

24

M

8

U-ILD

CT

interstitial involvement

-

2

+

+

-

+

-

CS

63

78

25

M

7

NSIP

CT, LB

• interstitial involvement

+

3

+

+

-

+

-

CS

60

76

  1. ILD-NC: children with interstitial lung disease without systemic corticosteroid treatment; ILD-C: children with interstitial lung disease with systemic corticosteroid treatment;
  2. U-ILD: undefined/idiopathic interstitial lung disease: no specific diagnosis could be made; PAP: pulmonary alveolar proteinosis; CGD: chronic granulomatous disease; IPH: idiopathic pulmonary hemosiderosis; LIP: lymphocytic interstitial pneumonia; CPI: Chronic pneumonitis of infancy
  3. CS: corticosteroids, AZT: azathioprine, MT: methotrexat
  4. n.d.: lung function testing not done (children < 5 years); † symbolizes patients who died due to respiratory failure.
  5. CT: Computed tomography; BAL: Bronchoalveolar lavage; LB: Lung biopsy
  6. * ILD score according to Fan[41]