pulmonary alveolar proteinosis, (pap) is a rare disease of unknown etiology, characterized by accumulation of intra-alveolar proteinaceous material which is rich in lipid and positive on periodic acid-schiff stain. two clinically different pediatric types have been defined as congenital pap which is fulminant and fatal, and a late-onset pap which is similar to the adult form and less severe. ei...

INTRODUCTION Pulmonary alveolar proteinosis is a rare pulmonary disease characterized by excessive alveolar accumulation of surfactant due to defective alveolar clearance by macrophages. There are only a few published case reports of pulmonary alveolar proteinosis occurring in association with solid cancers. To the best of our knowledge, there are no previously reported cases of pulmonary alveo...

REFERENCES 1 Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958; 258: 123–1143. 2 Kitamura T, Tanaka N, Watanabe J, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte-macrophage colony stimulating factor. J Exp Med 1999; 190: 875–880. 3 Kitamura T, Uchida K, Tanaka N, et al. Serological diagno...

Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material derived from alveolar surfactant in the alveoli, with a consequent deterioration in gas exchange. Pathogenesis is related to impaired phagocytic function of alveolar macrophages. In recent years, a new treatment for pulmonary alveolar proteinosis-consisting of subcutaneous administra...

1. Ramirez-R J. Pulmonary Alveolar Proteinosis: Treatment by Massive Bronchopulmonary Lavage. Arch Intern Med. 1967;119(2):147-156. doi:10.1001/archinte.1967.00290200071003 CrossRef Google Scholar

1 Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958; 258: 1123–1142. 2 Kitamura T, Tanaka N, Watanabe J, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med 1999; 190: 875–880. 3 Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress ...

Pulmonary alveolar proteinosis is a rare idiopathic diffuse airspace disease characterized by intra-alveolar accumulation of large quantities of lipoproteinaceous material, with preservation of the lung interstitium. The clinical course of pulmonary alveolar proteinosis is variable. Spontaneous resolution is known to occur in up to a quarter of the cases. We describe two patients with untreated...

Extensive pulmonary fibrosis is a rare occurrence in pulmonary alveolar proteinosis. We report 2 cases that have interesting implications. A female patient was diagnosed with autoimmune pulmonary alveolar proteinosis that evolved over 7 years into diffuse fibrosis. In a male patient with diffuse fibrosis we incidentally detected electron microscopic features of alveolar surfactant accumulation...

silicosis is considered to be among the occupational lung diseases and associated with sandblasting, mining, quarrying and tunneling. acute silicosis is usually progressive diseaseand despite treatment with corticosteroids it leads to cardio-respiratory failure and death. alveolar silicoproteinosis is one of it’s acute presentations due to exposure to silica dust and lungs filling with proteina...

BACKGROUND Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for ...