Enfermedad venooclusiva pulmonar y hemangiomatosis capilar pulmonar. Article (PDF Available) in Medicina Clínica (6) · January A hemangiomatose capilar pulmonar é uma doença rara, caracterizada por proliferação de . Pulmonary capillary hemangiomatosis with atypical endotheliosis. Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis: A case report and literature review. XIONG Xianliang et al., Journal of Central South.

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Archivos de Bronconeumologia http: Other types of articles such as reviews, editorials, special articles, clinical reports, and letters to the Editor are also published in the Journal.

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It is a monthly Journal that publishes a total of 12 issues, which contain these types of articles to different extents. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team.

The Journal is published both in Spanish and English. Therefore, the submission of pu,monar written in either Spanish or English is welcome. Translators working for the Journal are in charge of the corresponding translations.

Manuscripts will be submitted electronically using the following web site: See more Access to any published article, in either language, is possible through the Journal web page as well as from Pubmed, Science Direct, hemangiomattosis other international databases. Furthermore, the Journal is also present in Twitter and Facebook. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published.

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SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Pulmonary capillary hemangiomatosis PCH is a low-grade pulmonary malignancy due to interstitial proliferation of capillary-like vessels occurring in patients of any age or sex.

Prognosis is poor, with an estimated mean survival of 36 months. A year old man, former smoker 39 pack-yearspresented with worsening dyspnoea upon exertion and fatigue lasting 4 years.

Pulmonary capillary hemangiomatosis

Electrocardiographic examination showed a PR interval of milliseconds, with pulmonary P waves, right bundle branch block, and a heart rate of 94 bpm. Findings of llaboratory tests were unremarkable. Complete pulmonary function tests were not performed, as the patient was not compliant.

Spirometry showed a mild obstructive ventilatory defect not reversible upon broncho-dilation. Standard chest X-ray showed non-specific hilar congestion not shown. Echocardiography revealed severe hypokinesia of the right ventricle along with a marked dilation of the right atrium and an estimated systolic pulmonary hemanngiomatosis pressure of 70 mmHg.

Right heart catheterization was refused. Thromboembolic pulmonary disease was ruled out by contrast medium computed tomography CT Fig. Main imaging findings are shown in Fig. The patient was started on oral therapy with carvedilol The patient was referred to a lung transplantation center.

Pulmonary capillary haemangiomatosis | Radiology Reference Article |

Now, over 50 months after diagnosis, his clinical condition is still seriously compromised, though stable. Volumetric reconstruction bone filter HRCT of the upper a and lower lung lobes b showing multifocal smooth interlobular septal tickening white arrows and bilateral mild pleural effusion black arrows. Endothelial cells stain positive with the CD31 monoclonal antibody d. We reported a case of PCH with an atypically long clinical course 6 years from clinical onset along with a non-specific radiologic pattern.

PCH is characterized by alveolar wall thickening due to capillary proliferation. Infiltration and compression of pulmonary veins by new capillaries can result in secondary PVOD.


Orphanet: Hemangiomatosis capilar pulmonar

Clinicians and radiologists should bear PCH in mind, as early identification calilar improve patient management. PCH pjlmonar like a low-grade non-metastatic vascular neoplasm, with a slow progressive clinical course. Prognosis is poor and lung transplantation is the best option. The authors declare that they have no conflict of interests. Please cite this article as: Previous article Next article. February Pages e5-e12 Pages Letter to the Editor. This item has received.

Now, over 50 months after diagnosis, his clinical condition is still seriously compromised, though stable. Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: Report of 2 new cases and review of 35 cases from the literature. Medicine Baltimore81pp.

Safety and efficacy of epoprostenol therapy in pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Circ J, 76pp. RadioGraphics, 27pp. Different sizes of centrilobular ground glass opacities in chest high resolution computed tomography of patients with pulmonary veno-occlusive disease and patients with pulmonary capillary hemangiomatosis.

Cardiovasc Path, 22pp. Three-dimensional structure of pulmonary capillary vessels in patients with pulmonary hypertension. Hemangio,atosis,pp. Subscribe to our Newsletter.

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