The Experts below are selected from a list of 396 Experts worldwide ranked by ideXlab platform
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.
H N Castro - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary capillary Hemangiomatosis. A rare cause of pulmonary hypertension: the first Brazilian case
Sociedade Brasileira de Pneumologia e Tisiologia, 2005Co-Authors: H N Castro, Mara Rúbia Fernades De ,figueiredo, Santos, Norma Selma, Nogueira, Teresa Neuma Albuquerque Gomes, Uchoa, Maria Da Penha, Holanda, Marcelo AlcântaraAbstract:A hemangiomatose capilar pulmonar é uma doença rara, caracterizada por proliferação de capilares que invadem o interstício pulmonar e o septo alveolar. Documentamos o primeiro caso brasileiro: um paciente do sexo masculino, de 21 anos, com hipertensão pulmonar grave, que evoluiu para óbito. Na tomografia computadorizada de alta resolução apresentava pequenas opacidades intersticiais nodulares maldefinidas, bilateralmente. Foi realizada biópsia pulmonar post-mortem e encontrada intensa proliferação multifocal de capilares nas paredes alveolares, septos interlobulares e tecido conjuntivo peribrônquico. O diagnóstico de hemangiomatose capilar pulmonar deve ser considerado nos pacientes com hipertensão pulmonar e alterações sugestivas na tomografia computadorizada de alta resolução.Pulmonary capillary Hemangiomatosis is a rare disorder characterized by a proliferation of capillaries that invade the pulmonary interstitium and alveolar septae. Herein, we report the first Brazilian case of pulmonary capillary Hemangiomatosis. A 21-year-old man presented with severe pulmonary hypertension that eventually resulted in his death. Upon admission, a computed tomography scan of the chest revealed diffuse ill-defined bilateral pulmonary nodules. A postmortem lung biopsy revealed pronounced multifocal proliferation of capillaries in the alveolar walls, interlobular septa and peribronchial connective tissue. A diagnosis of pulmonary capillary Hemangiomatosis should be considered in patients presenting pulmonary hypertension and suspicious changes on high-resolution computed tomography scans
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hemangiomatose capilar pulmonar uma rara causa de hipertensao pulmonar primeiro caso brasileiro pulmonary capillary Hemangiomatosis a rare cause of pulmonary hypertension the first brazilian case
2005Co-Authors: H N Castro, Figueiredo Mrf, N S Santos, Nogueira Tnag, M P Uchoa, Marcia Alcântara Holanda, Rubia Fernandes De Figueiredo, Teresa Neuma AlbuquerqueAbstract:Pulmonary capillary Hemangiomatosis is a rare disorder characterized by a proliferation of capillaries that invade the pulmonary interstitium and alveolar septae. Herein, we report the first Brazilian case of pulmonary capillary Hemangiomatosis. A 21-year-old man presented with severe pulmonary hypertension that eventually resulted in his death. Upon admission, a computed tomography scan of the chest revealed diffuse ill-defined bilateral pulmonary nodules. A postmortem lung biopsy revealed pronounced multifocal proliferation of capillaries in the alveolar walls, interlobular septa and peribronchial connective tissue. A diagnosis of pulmonary capillary Hemangiomatosis should be considered in patients presenting pulmonary hypertension and suspicious changes on high-resolution computed tomography scans. Descritores: Hemangioma capilar/etiologia; Hemangioma capilar/diagnostico; Hipertensao pulmonar/complicacoes; Tomografia computadorizada de emissao; Relato de caso
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Hemangiomatose capilar pulmonar, uma rara causa de hipertensão pulmonar: primeiro caso brasileiro Pulmonary capillary Hemangiomatosis. A rare cause of pulmonary hypertension: the first Brazilian case
Sociedade Brasileira de Pneumologia e Tisiologia, 2005Co-Authors: H N Castro, N S Santos, Mara Rúbia Fernades De ,figueiredo, Teresa Neuma Albuquerque Gomes Nogueira, Maria Da Penha Uchoa, Marcelo Alcântara HolandaAbstract:A hemangiomatose capilar pulmonar é uma doença rara, caracterizada por proliferação de capilares que invadem o interstício pulmonar e o septo alveolar. Documentamos o primeiro caso brasileiro: um paciente do sexo masculino, de 21 anos, com hipertensão pulmonar grave, que evoluiu para óbito. Na tomografia computadorizada de alta resolução apresentava pequenas opacidades intersticiais nodulares maldefinidas, bilateralmente. Foi realizada biópsia pulmonar post-mortem e encontrada intensa proliferação multifocal de capilares nas paredes alveolares, septos interlobulares e tecido conjuntivo peribrônquico. O diagnóstico de hemangiomatose capilar pulmonar deve ser considerado nos pacientes com hipertensão pulmonar e alterações sugestivas na tomografia computadorizada de alta resolução.Pulmonary capillary Hemangiomatosis is a rare disorder characterized by a proliferation of capillaries that invade the pulmonary interstitium and alveolar septae. Herein, we report the first Brazilian case of pulmonary capillary Hemangiomatosis. A 21-year-old man presented with severe pulmonary hypertension that eventually resulted in his death. Upon admission, a computed tomography scan of the chest revealed diffuse ill-defined bilateral pulmonary nodules. A postmortem lung biopsy revealed pronounced multifocal proliferation of capillaries in the alveolar walls, interlobular septa and peribronchial connective tissue. A diagnosis of pulmonary capillary Hemangiomatosis should be considered in patients presenting pulmonary hypertension and suspicious changes on high-resolution computed tomography scans
Hunter D Best - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary veno-occlusive disease: Two children with gradual disease progression
Elsevier, 2017Co-Authors: Ronald W. Day, Hunter D Best, Parker W. Clement, Aimee O. Hersh, Susan M. Connors, Kelli L. Sumner, Mouied AlashariAbstract:Pulmonary veno-occlusive disease and pulmonary capillary Hemangiomatosis are rare forms of pulmonary vascular disease. We report two cases of affected children who had evidence of pulmonary hypertension 3–5 years before developing radiographic findings of pulmonary veno-occlusive disease or pulmonary capillary Hemangiomatosis. Both patients experienced a moderate decrease in pulmonary arterial pressure during acute vasodilator testing. Both patients experienced an improvement in six-minute walk performance without an increase in pulmonary edema when treated with targeted therapy for pulmonary hypertension. In some patients, pulmonary veno-occlusive disease and pulmonary capillary Hemangiomatosis may progress slowly over a period of months to years. A favorable acute vasodilator response may identify patients who will tolerate, and demonstrate transient clinical improvement with, medical therapy
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eif2ak4 mutations in pulmonary capillary Hemangiomatosis
Chest, 2014Co-Authors: Hunter D Best, Kelli Sumner, Wendy K Chung, Lynette M Brown, Alain C Borczuk, Eric D Austin, Erika B. RosenzweigAbstract:Background Pulmonary capillary Hemangiomatosis (PCH) is a rare disease of capillary proliferation of unknown cause and with a high mortality. Families with multiple affected individuals with PCH suggest a heritable cause although the genetic etiology remains unknown. Methods We used exome sequencing to identify a candidate gene for PCH in a family with two affected brothers. We then screened 11 unrelated patients with familial (n = 1) or sporadic (n = 10) PCH for mutations. Results Using exome sequencing, we identified compound mutations in eukaryotic translation initiation factor 2 α kinase 4 ( EIF2AK4 ) (formerly known as GCN2 ) in both affected brothers. Both parents and an unaffected sister were heterozygous carriers. In addition, we identified two EIF2AK4 mutations in each of two of 10 unrelated individuals with sporadic PCH. EIF2AK4 belongs to a family of kinases that regulate angiogenesis in response to cellular stress. Conclusions Mutations in EIF2AK4 are likely to cause autosomal-recessive PCH in familial and some nonfamilial cases.
Miriam D. Post - One of the best experts on this subject based on the ideXlab platform.
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pulmonary capillary Hemangiomatosis a rare cause of pulmonary hypertension
Archives of Pathology & Laboratory Medicine, 2015Co-Authors: Mary C Okeefe, Miriam D. PostAbstract:Pulmonary capillary Hemangiomatosis is a rare cause of pulmonary hypertension characterized by extensive proliferation of pulmonary capillaries within alveolar septae. Clinical presentation is nonspecific and includes dyspnea, cough, chest pain, and fatigue. Radiology shows diffuse centrilobular ground-glass opacities. Pulmonary capillary Hemangiomatosis is clinically and radiographically indistinguishable from peripheral venoocclusive disease, making microscopic diagnosis essential. Histologically, pulmonary capillary Hemangiomatosis shows an abnormal proliferation of small, thin-walled capillaries that expand the alveolar septae. The endothelial cells that comprise these lesions are cytologically bland and show no mitotic activity. Pulmonary capillary Hemangiomatosis is important to recognize because prostacyclin therapy, a mainstay in the treatment of pulmonary hypertension, has been reported to cause sudden respiratory distress and death in these patients. Prognosis of this disease remains poor, and t...
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pulmonary capillary Hemangiomatosis a rare cause of pulmonary hypertension
Archives of Pathology & Laboratory Medicine, 2015Co-Authors: Mary C Okeefe, Miriam D. PostAbstract:Pulmonary capillary Hemangiomatosis is a rare cause of pulmonary hypertension characterized by extensive proliferation of pulmonary capillaries within alveolar septae. Clinical presentation is nonspecific and includes dyspnea, cough, chest pain, and fatigue. Radiology shows diffuse centrilobular ground-glass opacities. Pulmonary capillary Hemangiomatosis is clinically and radiographically indistinguishable from peripheral venoocclusive disease, making microscopic diagnosis essential. Histologically, pulmonary capillary Hemangiomatosis shows an abnormal proliferation of small, thin-walled capillaries that expand the alveolar septae. The endothelial cells that comprise these lesions are cytologically bland and show no mitotic activity. Pulmonary capillary Hemangiomatosis is important to recognize because prostacyclin therapy, a mainstay in the treatment of pulmonary hypertension, has been reported to cause sudden respiratory distress and death in these patients. Prognosis of this disease remains poor, and the only definitive treatment is lung transplantation.
Dominique Musset - One of the best experts on this subject based on the ideXlab platform.
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high resolution ct of the chest in four patients with pulmonary capillary Hemangiomatosis or pulmonary venoocclusive disease
American Journal of Roentgenology, 1998Co-Authors: B. Dufour, Sophie Maitre, Gérald Simonneau, Frederique Capron, Marc Humbert, Dominique MussetAbstract:OBJECTIVE: Clinical differentiation of isolated pulmonary hypertensive arteriopathy from pulmonary capillary Hemangiomatosis or pulmonary venoocclusive disease can be difficult on a clinical basis alone. Differentiation is important because misdiagnosis of pulmonary capillary Hemangiomatosis or pulmonary venoocclusive disease may lead to severe vasodilator-induced pulmonary edema. The objective of our study was to determine whether high-resolution CT of the chest could distinguish pulmonary capillary Hemangiomatosis or pulmonary venoocclusive disease from isolated pulmonary hypertensive arteriopathy. CONCLUSION: Pulmonary hypertension in patients who also have pulmonary capillary Hemangiomatosis or pulmonary venoocclusive disease shows characteristics on high-resolution CT that are not seen in patients with isolated pulmonary hypertensive arteriopathy.
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pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary Hemangiomatosis
American Journal of Respiratory and Critical Care Medicine, 1998Co-Authors: Marc Humbert, Sophie Maitre, Dominique Musset, B Rain, Frederique Capron, Gérald SimonneauAbstract:Continuous intravenous epoprostenol (prostacyclin) produces hemodynamic and symptomatic responses and improves survival in patients with severe primary pulmonary hypertension refractory to conventional medical therapy. However, it has been recently shown that short-term infusion of epoprostenol can produce pulmonary edema in pulmonary veno-occlusive disease, presumably because of increased pulmonary perfusion in the presence of downstream vascular obstruction. We describe two additional cases of pulmonary edema complicating continuous intravenous epoprostenol in patients displaying severe pulmonary hypertension and pulmonary capillary Hemangiomatosis, a rare condition characterized by the proliferation of thin-walled microvessels in the alveolar walls. This report indicates that epoprostenol therapy should not be used in patients with severe pulmonary hypertension secondary to pulmonary capillary Hemangiomatosis.