Possible ocular involvement in pulmonary alveolar proteinosis
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چکیده
منابع مشابه
Possible ocular involvement in pulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterised by lipoproteinaceous material accumulation in the alveoli. In the most frequent form of the disease (acquired PAP), a neutralising auto-antibody against granulocytemacrophage colony-stimulating factor (GM-CSF) causes defects in the functioning of alveolar macrophages, including impairment of the catabolism of surfactant l...
متن کاملPulmonary Alveolar Proteinosis
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متن کاملPulmonary alveolar proteinosis.
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...
متن کاملPulmonary alveolar proteinosis.
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.
متن کاملPrimary pulmonary alveolar proteinosis.
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...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2006
ISSN: 0903-1936,1399-3003
DOI: 10.1183/09031936.06.00038906