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Pulmonary alveolar proteinosis is a rare disorder with decreased clearance of surfactant from the pulmonary alveoli resulting in progressive respiratory distress. Whole-lung lavage remains an important procedure for symptomatic relief in patients with pulmonary alveolar proteinosis. If a double-lumen tube can be placed, lavage of one side of the lung is performed through one lumen while the pat...

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alv...

INTRODUCTION Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihil...

2009;136;571-577 Chest Sara R. Greenhill and Darrell N. Kotton Pulmonary Alveolar Proteinosis http://chestjournal.chestpubs.org/content/136/2/571.full.html and services can be found online on the World Wide Web at: The online version of this article, along with updated information ISSN:0012-3692 ) http://chestjournal.chestpubs.org/site/misc/reprints.xhtml ( of the copyright holder. may be repro...

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP i...

INTRODUCTION Pulmonary alveolar proteinosis (PAP) is a relatively rare lung disorder, probably under diagnosed, characterized by the accumulation of lipoproteinaceosus material in the lung alveoli. The primary (acquired or idiopathic) form occurs in more than 90% of all cases. Whole lung lavage is considered the golden standard of treatment. In this report, we describe a rare case of pulmonary ...

Pulmonary alveolar proteinosis (PAP) has been recognized for almost half a century. At least three separate pathophysiologic mechanisms may lead to the characteristic feature of PAP: the excessive accumulation of surfactant lipoprotein in pulmonary alveoli, with associated disturbance of pulmonary gas exchange. The prognosis for adult patients with PAP varies, but disease-specific survival rate...

T wenty-four years ago, Rosen et al’ described the entity they called “pulmonary alveolar proteinosis” (PAP). The characteristics ofthis lung disease were that alveoli were filled with granular proteinaceous material which was periodic acid Schiff-positive and that the alveolar septae were relatively normal except for increased numbers of cuboidal “septal cells” (type 2 pneumocytes) in the alve...