The Experts below are selected from a list of 252 Experts worldwide ranked by ideXlab platform
C Medrano - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary Alveolar Proteinosis: prolonged spontaneous remission in two patients
The European respiratory journal, 1991Co-Authors: Ma Martinez-lopez, G Gomez-cerezo, C Villasante, F Molina, S Diaz, J Cobo, C MedranoAbstract:Pulmonary Alveolar Proteinosis is a rare idiopathic diffuse airspace disease characterized by intra-Alveolar accumulation of large quantities of lipoproteinaceous material, with preservation of the lung interstitium. The clinical course of Pulmonary Alveolar Proteinosis is variable. Spontaneous resolution is known to occur in up to a quarter of the cases. We describe two patients with untreated Pulmonary Alveolar Proteinosis who experienced complete clinical, functional and radiographic resolution. In follow-up periods of fourteen and six years, both patients have remained asymptomatic.
Ma Martinez-lopez - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary Alveolar Proteinosis: prolonged spontaneous remission in two patients
The European respiratory journal, 1991Co-Authors: Ma Martinez-lopez, G Gomez-cerezo, C Villasante, F Molina, S Diaz, J Cobo, C MedranoAbstract:Pulmonary Alveolar Proteinosis is a rare idiopathic diffuse airspace disease characterized by intra-Alveolar accumulation of large quantities of lipoproteinaceous material, with preservation of the lung interstitium. The clinical course of Pulmonary Alveolar Proteinosis is variable. Spontaneous resolution is known to occur in up to a quarter of the cases. We describe two patients with untreated Pulmonary Alveolar Proteinosis who experienced complete clinical, functional and radiographic resolution. In follow-up periods of fourteen and six years, both patients have remained asymptomatic.
Bruno Crestani - One of the best experts on this subject based on the ideXlab platform.
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Rituximab therapy in autoimmune Pulmonary Alveolar Proteinosis
The European respiratory journal, 2009Co-Authors: Raphael Borie, Marie-pierre Debray, C. Lainé, Michel Aubier, Bruno CrestaniAbstract: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.
Bruce C. Trapnell - One of the best experts on this subject based on the ideXlab platform.
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inhaled molgramostim therapy in autoimmune Pulmonary Alveolar Proteinosis
The New England Journal of Medicine, 2020Co-Authors: Bruce C. Trapnell, Francesco Bonella, Etsuro Yamaguchi, Ilaria Campo, Cliff Morgan, Yoshikazu Inoue, Stephane Jouneau, Elisabeth Bendstrup, Spyros Papiris, Erdogan CetinkayaAbstract:Abstract Background Autoimmune Pulmonary Alveolar Proteinosis (aPAP) is a rare disease characterized by progressive surfactant accumulation and hypoxemia. It is caused by disruption of granulocyte–...
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Pulmonary Alveolar Proteinosis
Nature Reviews Disease Primers, 2019Co-Authors: Bruce C. Trapnell, Matthias Griese, Koh Nakata, Francesco Bonella, Ilaria Campo, John Hamilton, Tisha Wang, Cliff Morgan, Vincent Cottin, Cormac MccarthyAbstract:Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by the accumulation of Alveolar surfactant and dysfunction of Alveolar macrophages. PAP results in progressive dyspnoea of insidious onset, hypoxaemic respiratory failure, secondary infections and Pulmonary fibrosis. PAP can be classified into different types on the basis of the pathogenetic mechanism: primary PAP is characterized by the disruption of granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling and can be autoimmune (caused by elevated levels of GM-CSF autoantibodies) or hereditary (due to mutations in CSF2RA or CSF2RB , encoding GM-CSF receptor subunits); secondary PAP results from various underlying conditions; and congenital PAP is caused by mutations in genes involved in surfactant production. In most patients, pathogenesis is driven by reduced GM-CSF-dependent cholesterol clearance in Alveolar macrophages, which impairs Alveolar surfactant clearance. PAP has a prevalence of at least 7 cases per million individuals in large population studies and affects men, women and children of all ages, ethnicities and geographical locations irrespective of socioeconomic status, although it is more-prevalent in smokers. Autoimmune PAP accounts for >90% of all cases. Management aims at improving symptoms and quality of life; whole-lung lavage effectively removes excessive surfactant. Novel pathogenesis-based therapies are in development, targeting GM-CSF signalling, immune modulation and cholesterol homeostasis. Pulmonary Alveolar Proteinosis comprises a group of diseases with different pathogenetic mechanisms but a common outcome — the progressive accumulation of Alveolar surfactant in the lungs and dysfunction of Alveolar macrophages, which result in respiratory failure and increased risk of secondary infections.
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Pulmonary Alveolar Proteinosis
The New England journal of medicine, 2003Co-Authors: Bruce C. Trapnell, Jeffrey A. Whitsett, Koh NakataAbstract:In acquired Pulmonary Alveolar Proteinosis, lipids and proteins accumulate within the alveoli because Alveolar macrophages cannot catabolize surfactants. Surprisingly, Alveolar macrophages require granulocyte–macrophage colony-stimulating factor (GM-CSF) to perform this function. Autoantibodies against GM-CSF may cause Pulmonary Alveolar Proteinosis.
J Cobo - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary Alveolar Proteinosis: prolonged spontaneous remission in two patients
The European respiratory journal, 1991Co-Authors: Ma Martinez-lopez, G Gomez-cerezo, C Villasante, F Molina, S Diaz, J Cobo, C MedranoAbstract:Pulmonary Alveolar Proteinosis is a rare idiopathic diffuse airspace disease characterized by intra-Alveolar accumulation of large quantities of lipoproteinaceous material, with preservation of the lung interstitium. The clinical course of Pulmonary Alveolar Proteinosis is variable. Spontaneous resolution is known to occur in up to a quarter of the cases. We describe two patients with untreated Pulmonary Alveolar Proteinosis who experienced complete clinical, functional and radiographic resolution. In follow-up periods of fourteen and six years, both patients have remained asymptomatic.
