The Experts below are selected from a list of 264 Experts worldwide ranked by ideXlab platform
David Montani - One of the best experts on this subject based on the ideXlab platform.
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ESC CardioMed - Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Edmund Lau, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease
Advances in Pulmonary Hypertension, 2015Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:As a rare form of Pulmonary hypertension, the pathophysiology and clinical presentation of Pulmonary Veno-Occlusive Disease (PVOD) has long remained poorly understood. In this review, we will discu...
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Pulmonary Veno-Occlusive Disease: advances in clinical management and treatments
Expert review of respiratory medicine, 2011Co-Authors: Alice Huertas, Barbara Girerd, Marc Humbert, Peter Dorfmüller, Dermot S. O'callaghan, David MontaniAbstract:Pulmonary Veno-Occlusive Disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic Pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic background and hemodynamic profile. A definite diagnosis of PVOD necessitates a surgical biopsy, but since it represents a high-risk procedure in these patients, it is contraindicated. Therefore, a noninvasive diagnostic approach using chest high-resolution computed tomography, arterial blood gas analysis, Pulmonary function tests and bronchoalveolar lavage is helpful to detect PVOD. PVOD is characterized by a poor prognosis and the possibility of developing severe Pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice.
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Pulmonary veno occlusive Disease
European Respiratory Journal, 2009Co-Authors: Barbara Girerd, David Montani, Xavier Jaïs, Peter Dorfmüller, Edmund M T Lau, Laurent SavaleAbstract:Pulmonary Veno-Occlusive Disease (PVOD) is a rare form of Pulmonary hypertension (PH) characterised by preferential remodelling of the Pulmonary venules. In the current PH classification, PVOD and Pulmonary capillary haemangiomatosis (PCH) are considered to be a common entity and represent varied expressions of the same Disease. The recent discovery of biallelic mutations in the EIF2AK4 gene as the cause of heritable PVOD/PCH represents a major milestone in our understanding of the molecular pathogenesis of PVOD. Although PVOD and Pulmonary arterial hypertension (PAH) share a similar clinical presentation, with features of severe precapillary PH, it is important to differentiate these two conditions as PVOD carries a worse prognosis and life-threatening Pulmonary oedema may occur following the initiation of PAH therapy. An accurate diagnosis of PVOD based on noninvasive investigations is possible utilising oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest. No evidence-based medical therapy exists for PVOD at present and lung transplantation remains the preferred definitive therapy for eligible patients.
Marc Humbert - One of the best experts on this subject based on the ideXlab platform.
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ESC CardioMed - Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Edmund Lau, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease
Advances in Pulmonary Hypertension, 2015Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:As a rare form of Pulmonary hypertension, the pathophysiology and clinical presentation of Pulmonary Veno-Occlusive Disease (PVOD) has long remained poorly understood. In this review, we will discu...
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Pulmonary Veno-Occlusive Disease: advances in clinical management and treatments
Expert review of respiratory medicine, 2011Co-Authors: Alice Huertas, Barbara Girerd, Marc Humbert, Peter Dorfmüller, Dermot S. O'callaghan, David MontaniAbstract:Pulmonary Veno-Occlusive Disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic Pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic background and hemodynamic profile. A definite diagnosis of PVOD necessitates a surgical biopsy, but since it represents a high-risk procedure in these patients, it is contraindicated. Therefore, a noninvasive diagnostic approach using chest high-resolution computed tomography, arterial blood gas analysis, Pulmonary function tests and bronchoalveolar lavage is helpful to detect PVOD. PVOD is characterized by a poor prognosis and the possibility of developing severe Pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice.
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Occult alveolar haemorrhage in Pulmonary Veno-Occlusive Disease
The European respiratory journal, 2006Co-Authors: Anne Rabiller, A Resten, F Capron, G Simonneau, Xavier Jaïs, A. Hamid, Florence Parent, R Haque, Olivier Sitbon, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) is a rare cause of Pulmonary arterial hypertension that affects predominantly post-capillary Pulmonary vessels. A major concern with PVOD is the poor response to available therapies and the risk of Pulmonary oedema with continuous intravenous epoprostenol. The present authors hypothesised that alveolar haemorrhage may be a characteristic feature of Pulmonary Veno-Occlusive Disease, as compared with other forms of Pulmonary arterial hypertension that predominantly involve pre-capillary Pulmonary arteries. This paper reports a series of 19 patients with either PVOD (n = 8) or idiopathic Pulmonary arterial hypertension (IPAH; n = 11) who underwent bronchoalveolar lavage. Cytological analyses were performed and differential counts were made on Perls-stained preparations. The Golde score was used to assess alveolar haemorrhage. As compared with IPAH, PVOD was characterised by a higher percentage of haemosiderin-laden macrophages (40+/-37 versus 3+/-6%), resulting in elevated Golde scores (81+/-88 versus 4+/-10). It was concluded that occult alveolar haemorrhage is a common feature of Pulmonary Veno-Occlusive Disease. Detecting occult alveolar haemorrhage may be of interest in the diagnostic approach of Pulmonary Veno-Occlusive Disease.
Barbara Girerd - One of the best experts on this subject based on the ideXlab platform.
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ESC CardioMed - Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease and Pulmonary capillary haemangiomatosis
ESC CardioMed, 2018Co-Authors: David Montani, Barbara Girerd, Edmund Lau, Marc HumbertAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary haemangiomatosis (PCH) are rare causes of Pulmonary hypertension, and they are now known to represent manifestations of a common Disease characterized by fibrous remodelling of the small Pulmonary veins and endothelial cell proliferation within the Pulmonary capillaries. Heritable PVOD and PCH are caused by the same genetic defect of biallelic mutations of the EIF2AK4 gene. Other risk factors associated with the development of PVOD/PCH include chemotherapy treatment, organic solvent exposure, and systemic sclerosis.
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Pulmonary Veno-Occlusive Disease
Advances in Pulmonary Hypertension, 2015Co-Authors: David Montani, Barbara Girerd, Marc HumbertAbstract:As a rare form of Pulmonary hypertension, the pathophysiology and clinical presentation of Pulmonary Veno-Occlusive Disease (PVOD) has long remained poorly understood. In this review, we will discu...
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granulysin in Pulmonary veno occlusive Disease
European Respiratory Journal, 2013Co-Authors: Frederic Perros, Barbara Girerd, Nicolas Raymond, Alice Huertas, Sylvia Cohenkaminsky, Natalia Gambaryan, Isabelle Klingelschmitt, Olaf Mercier, Elie Fadel, G SimonneauAbstract:Background: Pulmonary Veno-Occlusive Disease (PVOD) shares many similarities with precapillary Pulmonary Arterial Hypertension (PAH), from risk factors to clinical or hemodynamic presentation. The need to establish a correct diagnosis of PVOD is justified by the worse prognosis of these patients and by their risk of developing severe Pulmonary edema with specific PAH therapy. Aims and objectives: To show that the cytolytic subpopulation of inflammatory cells is differently regulated in PAH and PVOD patients and to identify a biological tool to distinguish these two entities. Methods: The functional status of the cytolytic compartment was studied through epigenetic analysis of the Granulysin (GNLY), a powerful effector for these subpopulations in explanted lungs and in PBMC. Flow cytometry allowed analysis of circulating cytolytic cells and GNLY contents. A GNLY-specific ELISA allowed measurement of GNLY serum concentrations. Results: A decrease in GNLY demethylation in the DNA extracted from PBMC and lungs was found in PVOD but not in PAH. This was associated with a decrease in populations and subpopulations of cytotoxic T-(CTL), and Natural Killer T-(NKT) cells, and an increase of Natural Killer-(NK) populations. Despite the reduced GNLY-containing cells in PVOD, GNLY serum levels were higher, suggesting these cells were wasting their content. Furthermore, the increase of GNLY concentration in the serum of PVOD was higher than in PAH patients. Conclusions: PVOD is characterized by alterations of circulating cytotoxic cell-subpopulations and by epigenetic dysregulation within the GNLY gene. Our findings may be helpful to develop needed biological tools in order to screen for suspected PVOD in patients with Pulmonary hypertension.
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cytotoxic cells and granulysin in Pulmonary arterial hypertension and Pulmonary veno occlusive Disease
American Journal of Respiratory and Critical Care Medicine, 2013Co-Authors: Frederic Perros, Barbara Girerd, Nicolas Raymond, Alice Huertas, Sylvia Cohenkaminsky, Natalia Gambaryan, Isabelle Klingelschmitt, Olaf Mercier, Elie Fadel, G SimonneauAbstract:Rationale: Pulmonary arterial hypertension (PAH) and Pulmonary Veno-Occlusive Disease (PVOD) both display occlusive remodeling of the Pulmonary vasculature responsible for increased Pulmonary vascular resistances. Cytotoxic T (CTL), natural killer (NK), and natural killer T (NKT) cells play a critical role in vascular remodeling in different physiological and pathological conditions. Granulysin (GNLY) represents a powerful effector protein for all these subpopulations.Objectives: To analyze the cytolytic compartment of inflammatory cells in patients with PAH and PVOD.Methods: The overall functional status of the cytolytic compartment was studied through epigenetic analysis of the GNLY gene in explanted lungs and in peripheral blood mononuclear cells. Flow cytometry technology allowed analysis of specific circulating cytolytic cells and GNLY contents. A GNLY-specific ELISA allowed measurement of GNLY serum concentrations.Measurements and Main Results: A decrease in GNLY demethylation in the gDNA extracted ...
Charles Gregory Elliott - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary capillary hemangiomatosis and Pulmonary veno occlusive Disease
Clinics in Chest Medicine, 2016Co-Authors: Neal F Chaisson, Mark W Dodson, Charles Gregory ElliottAbstract:Abstract This article provides an overview of Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic Pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH.
Andrew G Nicholson - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary veno occlusive Disease and Pulmonary capillary hemangiomatosis a clinicopathologic study of 35 cases
The American Journal of Surgical Pathology, 2006Co-Authors: Sylvie Lantuejoul, B. Corrin, Margaret Burke, Mary N Sheppard, Andrew G NicholsonAbstract:Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary capillary hemangiomatosis (PCH) are rare causes of Pulmonary hypertension, regarded by some as distinct entities. However, their presentations are similar and both are associated with poor prognoses. We therefore reviewed 38 specimens [autopsies
