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Pulmonary alveolar proteinosis – current and future therapeutical strategies
Current opinion in pulmonary medicine, 2023Purpose of review We discuss the most recent advances in the treatment of pulmonary alveolar proteinosis (PAP), an ultra-rare syndrome. Recent findings Whole lung lavage (WLL) remains the gold standard of treatment for PAP syndrome.
L. Jehn, F. Bonella
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PULMONARY ALVEOLAR PROTEINOSIS
Revista sanitaria de investigación, 2023La proteinosis alveolar pulmonar (PAP) es una enfermedad intersticial muy infrecuente que se produce por un acúmulo excesivo de los lípidos y proteínas que componen el surfactante alveolar.
García Esteban Ana +5 more
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Pulmonary Alveolar Proteinosis
Critical Reviews in Computed Tomography, 2003Case 1: A 52-year-old Caucasian retired police officer presented 3 years prior to diagnosis with the chief complaint of inability to exercise due to breathlessness. The patient was a long-distance walker who had given up this recreational activity due to dyspnea on exertion.
Jeffrey A. Whitsett +2 more
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Pulmonary alveolar proteinosis
Clinics in Chest Medicine, 2004Pulmonary 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
Presneill, JJ +3 more
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Pulmonary Alveolar Proteinosis
Respiration, 1974A case of alveolar proteinosis of the lungs with sarcoidal reaction of the lymph nodes is reported. The diagnosis was established by histological examination of lung tissue secured during thoracotomy.
Krystyna Smigla, Jerzy Kurgan
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Pulmonary Alveolar Proteinosis
Radiology, 1969Since the original description of pulmonary alveolar proteinosis in 1958 (19), more than 100 cases have been reported. This article indicates the wide spectrum of roentgenographic changes (other than the well known batwing appearance) by reference to 5 patients in the San Francisco area who were examined recently.
W. M. Macleod, Jean M. Davidson
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Pulmonary Alveolar Proteinosis
New England Journal of Medicine, 1958WITHIN the past five years, and at an increasing rate within recent months, we have encountered 27 cases of a remarkable disease of the lung that consists of the filling of the alveoli by a PAS-positive proteinaceous material, rich in lipid. This material appears to be produced by the lining cells, which slough into the lumen, ultimately becoming ...
Samuel H. Rosen +4 more
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European Respiratory Journal
Extract Autoimmune pulmonary alveolar proteinosis (aPAP) is associated with autoantibodies against granulocyte–macrophage colony-stimulating factor (GM-CSF) in serum, causing the disruption of GM-CSF signal and the functional failure of alveolar ...
F. Bonella, E. Manali, S. Papiris
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Extract Autoimmune pulmonary alveolar proteinosis (aPAP) is associated with autoantibodies against granulocyte–macrophage colony-stimulating factor (GM-CSF) in serum, causing the disruption of GM-CSF signal and the functional failure of alveolar ...
F. Bonella, E. Manali, S. Papiris
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Pediatric Pulmonology, 2020
To the Editor, Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces, which interferes with gas exchange.
U. Ozcelik +11 more
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To the Editor, Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces, which interferes with gas exchange.
U. Ozcelik +11 more
semanticscholar +1 more source
Pharmacotherapy options in pulmonary alveolar proteinosis.
Expert Opinion on Pharmacotherapy, 2020INTRODUCTION Pulmonary alveolar proteinosis (PAP) is a heterogeneous group of rare diseases characterized by the abnormal production and impaired degradation of pulmonary surfactant as a result of malfunctioning of alveolar macrophages.
S. Antoniu +3 more
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