| Congenital absence of portal vein presenting as hepatopulmonary syndrome. | |
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MedLine Citation:
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PMID: 15670231 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed. |
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Authors:
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K M Cheung; C Y Lee; C T Wong; A K H Chan |
Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: Journal of paediatrics and child health Volume: 41 ISSN: 1034-4810 ISO Abbreviation: J Paediatr Child Health Publication Date: 2005 Jan-Feb |
Date Detail:
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Created Date: 2005-01-26 Completed Date: 2005-05-04 Revised Date: 2007-09-24 |
Medline Journal Info:
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Nlm Unique ID: 9005421 Medline TA: J Paediatr Child Health Country: Australia |
Other Details:
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Languages: eng Pagination: 72-5 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Caritas Medical Centre, Hong Kong, China. jennykmcheung@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Female Hepatopulmonary Syndrome / classification, etiology* Humans Portal Vein / abnormalities*, radiography Tomography, X-Ray Computed |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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