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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.