Document Detail


Pulmonary capillary hemangiomatosis. A report of three cases and a review of the literature.
MedLine Citation:
PMID:  2675708     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension. At present, only eleven cases have been reported. This report describes the clinical and pathologic findings in three additional cases who presented to the University of Pittsburgh for heart-lung transplantation and integrates the clinical features of all fourteen cases. Clinically, this disorder should be suspected in a patient who presents with pulmonary hypertension, hemoptysis, a reticulonodular infiltrate on chest radiograph, a lung scan showing inhomogeneously enhanced perfusion (particularly in the lower lobes), and pulmonary angiography showing increased peripheral vascularity corresponding to both the radiographic infiltrate and the areas of enhanced perfusion on lung scan. Histologic features consist of nodular proliferation of capillary-sized vessels that infiltrate the pulmonary interstitium, vascular walls, and lumens as well as the alveolar septa. The vascular invasion results in a secondary veno-occlusive phenomenon that explains the clinical confusion with pulmonary veno-occlusive disease.
Authors:
C N Faber; S A Yousem; J H Dauber; B P Griffith; R L Hardesty; I L Paradis
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.; Review    
Journal Detail:
Title:  The American review of respiratory disease     Volume:  140     ISSN:  0003-0805     ISO Abbreviation:  Am. Rev. Respir. Dis.     Publication Date:  1989 Sep 
Date Detail:
Created Date:  1989-10-19     Completed Date:  1989-10-19     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0370523     Medline TA:  Am Rev Respir Dis     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  808-13     Citation Subset:  AIM; IM    
Affiliation:
Department of Medicine, University of Pittsburgh, School of Medicine, Pennsylvania 15260.
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MeSH Terms
Descriptor/Qualifier:
Adult
Capillaries / pathology
Female
Hemangioma* / complications,  pathology,  radiography
Humans
Hypertension, Pulmonary / etiology
Lung / blood supply,  pathology,  radiography
Lung Neoplasms* / complications,  pathology,  radiography
Middle Aged
Grant Support
ID/Acronym/Agency:
HL-37553/HL/NHLBI NIH HHS

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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