Pulmonary veno-occlusive disease: Two children with gradual disease progression
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 pulmonar...
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| Format: | Article |
| Language: | English |
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Elsevier
2017-01-01
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| Series: | Respiratory Medicine Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213007116301861 |
| _version_ | 1818298316296814592 |
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| author | Ronald W. Day Parker W. Clement Aimee O. Hersh Susan M. Connors Kelli L. Sumner D. Hunter Best Mouied Alashari |
| author_facet | Ronald W. Day Parker W. Clement Aimee O. Hersh Susan M. Connors Kelli L. Sumner D. Hunter Best Mouied Alashari |
| author_sort | Ronald W. Day |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-13T04:33:23Z |
| format | Article |
| id | doaj.art-d1b587f5552f4f8f82d3d899893a9941 |
| institution | Directory Open Access Journal |
| issn | 2213-0071 |
| language | English |
| last_indexed | 2024-12-13T04:33:23Z |
| publishDate | 2017-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Respiratory Medicine Case Reports |
| spelling | doaj.art-d1b587f5552f4f8f82d3d899893a99412022-12-21T23:59:30ZengElsevierRespiratory Medicine Case Reports2213-00712017-01-0120C828610.1016/j.rmcr.2016.12.007Pulmonary veno-occlusive disease: Two children with gradual disease progressionRonald W. Day0Parker W. Clement1Aimee O. Hersh2Susan M. Connors3Kelli L. Sumner4D. Hunter Best5Mouied Alashari6University of Utah Department of Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, UT 84113, USAUniversity of Utah Department of Pathology, 15 North Medical Drive, Suite 1100, Salt Lake City, UT 84132, USAUniversity of Utah Department of Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, UT 84113, USAVascular Biology Program of Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USAARUP Institute for Clinical and Experimental Pathology, 500 Chipeta Way, Salt Lake City, UT 84108, USAARUP Institute for Clinical and Experimental Pathology, 500 Chipeta Way, Salt Lake City, UT 84108, USAUniversity of Utah Department of Pathology, 15 North Medical Drive, Suite 1100, Salt Lake City, UT 84132, USAPulmonary 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.http://www.sciencedirect.com/science/article/pii/S2213007116301861Pulmonary arterial hypertensionPulmonary capillary hemangiomatosisPulmonary veno-occlusive disease |
| spellingShingle | Ronald W. Day Parker W. Clement Aimee O. Hersh Susan M. Connors Kelli L. Sumner D. Hunter Best Mouied Alashari Pulmonary veno-occlusive disease: Two children with gradual disease progression Respiratory Medicine Case Reports Pulmonary arterial hypertension Pulmonary capillary hemangiomatosis Pulmonary veno-occlusive disease |
| title | Pulmonary veno-occlusive disease: Two children with gradual disease progression |
| title_full | Pulmonary veno-occlusive disease: Two children with gradual disease progression |
| title_fullStr | Pulmonary veno-occlusive disease: Two children with gradual disease progression |
| title_full_unstemmed | Pulmonary veno-occlusive disease: Two children with gradual disease progression |
| title_short | Pulmonary veno-occlusive disease: Two children with gradual disease progression |
| title_sort | pulmonary veno occlusive disease two children with gradual disease progression |
| topic | Pulmonary arterial hypertension Pulmonary capillary hemangiomatosis Pulmonary veno-occlusive disease |
| url | http://www.sciencedirect.com/science/article/pii/S2213007116301861 |
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