In 1963, five cases of alpha1-antitrypsin deficiency were reported in the scientific literature, as well as an attempt to treat pulmonary alveolar proteinosis by a massive washing of the lung (whole lung lavage). Now, fifty years later, it seems the ideal moment not only to commemorate these publications, but also to point out the influence both papers had in the following decades and how knowl...

Hermansky-Pudlak Syndrome (HPS) is a rare autosomal recessive disorder presenting with oculocutaneous albinism, bleeding diathesis and lysosomal accumulation of ceroid lipofuscin which leads to interstitial fibrosis in lung. Pulmonary fibrosis which is usually associated with HPS-1 and HPS-4 subtypes usually manifests in the third/fourth decades of life representing with giant lamellar bodies o...

Miliary mottling on chest radiography is seen in miliary tuberculosis, certain fungal infections, sarcoidosis, coal miner’s pneumoconiosis, silicosis, hemosiderosis, fibrosing alveolitis, acute extrinsic allergic alveolitis, pulmonary eosinophilic syndrome, pulmonary alveolar proteinosis, and rarely in hematogenous metastases from the primary cancers of the thyroid, kidney, trophoblasts, and so...

THIS CASE STUDY DESCRIBES THE ASSOCIATION BETWEEN TWO RARE ENTITIES: pulmonary alveolar proteinosis (PAP) and embryonic rhabdomyosarcoma of the prostate in a 54 year old male presenting with pulmonary symptoms and obstructive urinary symptoms. The prostate tumour was treated with a radical cystectomy and adjuvant chemotherapy and radiotherapy. The patient did not wish to have treatment for his ...

Recently Rosen and coworkers1 reported an unusual histologic pattern in the lungs consisting of alveolar deposition of a granular proteinoid material. They designated their findings as “pulmonary alveolar proteinosis.” These changes were found in biopsy or autopsy specimens of the lung which were referred primarily for tissue diagnosis. One of these patients was under our care. Since their desc...

Pulmonary alveolar proteinosis (PAP) is a progressive lung disease characterized by accumulated surfactant-like lipoproteinaceous material in the alveoli and distal bronchioles. This accumulation is the result of impaired clearance by alveolar macrophages. PAP has been described in 11 solid organ transplant recipients, 9 of whom were treated with mammalian target of rapamycin inhibitors. We rep...

We describe a rare case of pulmonary alveolar proteinosis in a young woman with dyspnea and progressive hypoxaemia due to the alveolar deposition of insoluble, surfactant-like material. Routine treatment includes whole-lung-lavage (WLL) using double-lumen-tubes for selective lavaging of each lung. We performed three whole-lung-lavages and used veno-venous extracorporeal membrane oxygenation (v-...