BACKGROUND Pulmonary alveolar proteinosis is a rare lung disorder, which was first reported as idiopathic condition in 1958. The prevalence of acquired pulmonary alveolar proteinosis has been estimated to be 0.37 per 100,000 population. The cause of this condition is not entirely clear. We present alveolar proteinosis in a case recently treated for pulmonary Pneumocystis carinii infection. CA...

AIMS For the diagnosis of pulmonary alveolar proteinosis from bronchoalveolar lavage specimens it is normally necessary to make an ultrastructural examination. However, this is thought to be impractical for bronchoalveolar lavage specimens that have been routinely fixed in ethanol. In the present study, bronchoalveolar lavage cytology smears on slide glasses were examined directly ultrastructur...

INTRODUCTION Pulmonary alveolar proteinosis is a rare condition that requires treatment by whole-lung lavage. We report a case of severe pulmonary alveolar proteinosis and discuss a safe and effective strategy for the anaesthetic management of patients undergoing this complex procedure. CASE PRESENTATION A 34-year-old Caucasian man was diagnosed with severe pulmonary alveolar proteinosis. He ...

Pulmonary alveolar proteinosis is a rare idiopathic lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli, which impairs gas transfer and decreases the ventilation/perfusion ratio, and can lead to respiratory failure. Whole lung lavage is the most effective therapy for pulmonary alveolar proteinosis, but may not be tolerated by patients with severe resp...

BACKGROUND The congenital form of pulmonary alveolar proteinosis due to colony stimulating factor 2 receptor alpha gene mutations is a rare disease with only a few cases reported worldwide. In this study we report a new case of pulmonary alveolar proteinosis with a novel variant in colony stimulating factor 2 receptor alpha gene. CASE PRESENTATION A 5-year-old Saudi boy presented with a histo...

Pulmonary alveolar proteinosis is a disease caused by increased accumulation and impaired clearance of surfactant by alveolar macrophages. This narrative review summarizes the role of therapeutic whole-lung lavage in the management of pulmonary alveolar proteinosis. We describe the preprocedural evaluation, indications, and anesthetic considerations, along with step-by step technical aspects of...

Pulmonary alveolar proteinosis is a rare cause of respiratory distress in neonates. We present a 4 month old infant who presented with progressive respiratory distress since birth and failure to thrive. He was initially treated as a case of diffuse alveolar disease but on open lung biopsy was diagnosed as pulmonary alveolar proteinosis. The child expired at 7 months of age.

Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis is characterized by the accumulation of granular proteinaceous material within the alveoli of the lung. It is well established that patients with pulmonary alveolar proteinosis have a high incidence of complicating pulmonary infections, which suggests that the function of the alveolar macrophages is abnormal. To investigate the function of these cells, they were o...