COEXISTING ENDOGENOUS AND EXOGENOUS LIPOID PNEUMONIA AND PULMONARY ALVEOLAR PROTEINOSIS IN A PATIENT WITH NEURODEVELOPMENTAL DISEASE

We report a unique case of coexisting exogenous lipoid pneumonia, endo genous lipoid pneumonia (ELP), and pulmonary alveolar proteinosis (PAP ) in a 5-year-old patient with severe neurodevelopmental disease. The Patient Presented with gastroesophageal reflux and presumed chronic lu ng disease resulting from recurrent aspiration pneumonias and succumbe d to respiratory failure. The autopsy showed lipid-laden macrophages a nd periodic acid-Schiff-positive granular maternal in alveolar spaces and multilamellated structures within both alveolar macrophages and ex tracellular debris. These findings were similar to those in previous r eports of coexisting ELP and PAP in the setting of gastroesophageal re flux [1]. However, the present case differed by the presence of scatte red large osmiophilic extracellular lipid vacuoles. Besides strengthen ing the association between ELP and PAP and their relationship to gast roesophageal reflux, this case suggests that they may arise together w ith exogenous lipoid pneumonia, through related mechanisms, in the set ting of neurodevelopmental disease.