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Table 2 Pathological features

From: Pulmonary alveolar proteinosis in children on La Réunion Island: a new inherited disorder?

  

Histological diagnosis specimen (n = 19/34)

Last available histological specimen (n = 14/34)

Age

 

30 months

7.3 years

median (IQR)

 

(5.3 months -10.3 years)

(6 years – 13.9 years)

Mode: n (%)

OLB

16 (84)

1 (7)

 

TBB

1 (5)

9 (65)

 

Autopsy

2 (11)

2 (14)

 

Native lung

0 (0)

2 (14)

Results: n (%)

PAP

19 (100)

13 (93)

 

+

7 (37)

6 (43)

 

++

3 (16)

5 (35)

 

+++

9 (47)

2 (14)

 

PICG

8 (42)

8 (57)

 

+

0 (0)

1 (13)

 

++

2 (25)

5 (62)

 

+++

6 (75)

2 (25)

 

Cholesterol clefts

14 (73)

14 (100)

 

+

5 (36)

5 (36)

 

++

3 (21)

4 (28)

 

+++

6 (43)

5 (36)

 

Fibrosis

8 (42)

10 (71)

 

+

1 (13)

1 (10)

 

++

2 (25)

3 (30)

 

+++

5 (62)

6 (60)

 

Inflammation

16 (84)

13 (93)

 

+

9 (56)

9 (69)

 

++

4 (25)

3 (23)

 

+++

3 (19)

1 (8)

  1. Definition of abbreviations: IQR = interquartile range; OLB = open lung biopsy; TBB = transbronchial biopsy; PAP = pulmonary alveolar proteinosis; PICG = pulmonary interstitial cholesterol granulomas. Data are presented at diagnosis and for the last available histological specimen. A semi-quantitative grading scale was used to score each lesion, with 0 indicating absence; + indicating rare or mild lesions; ++ indicating moderate or localized lesions and +++ indicating diffuse lesions. At diagnosis, PAP lesions were either isolated or associated with variable amounts of cholesterol clefts, cholesterol granulomas and fibrosis. Fibrosis was already observed at diagnosis in the 8 oldest cases of the series. At follow-up, although PAP lesions remained present but in a lesser extent, PICG and fibrosis lesions were more frequent.