Figure 3

Pathological features. A: Lung histological specimen from a 9 months old boy, post-mortem examination. Diffuse intra alveolar accumulation of granular eosinophilic proteinaceous material with rare cholesterol clefts and normal interalveolar septa typical of PAP. There was no inflammation and no fibrosis. HES (×100). B: Lung histological specimen from a 9 years old girl, OLB. Numerous intra-alveolar cholesterol clefts with giant cells associated with inflammatory cells and fibrosis, typical of PICG. Lesions predominantly observed in the subpleural part of the lung parenchyma. Fatty involution of the visceral pleura (*). Tiny foci of PAP were also observed. HES (×100). C: Lung histological specimen from a 25 years old woman, post-mortem examination. Lung sample displayed both PAP on the upper part of the figure (*) and PICG with cholesterol clefts, giant cells granuloma, interstitial fibrosis and inflammation in the lower part of the figure. HES (×200). D: Liver histological specimen from a 2 years and 9 months old boy. Micro nodular cirrhosis, steatosis, ductular proliferation without active inflammation. HES (×100).