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) |