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pubmed-article:9740273pubmed:abstractTextA case of non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension is reported. The patient was a 7-year old boy who presented with hematemesis, cyanosis, hypoxemia and orthodeoxia. His liver pathology was compatible with non-cirrhotic portal fibrosis. His pulmonary angiography showed arteriovenous shunting and pulmonary arterial hypertension (mean pulmonary artery pressure 34 mmHg). His sister also had non-cirrhotic portal fibrosis with neither hypoxemia nor orthodeoxia. This report raises the possibility of non-cirrhotic portal fibrosis having a genetic etiology.lld:pubmed
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pubmed-article:9740273pubmed:authorpubmed-author:VivatvakinBBlld:pubmed
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pubmed-article:9740273pubmed:pagination76-9lld:pubmed
pubmed-article:9740273pubmed:dateRevised2007-11-15lld:pubmed
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pubmed-article:9740273pubmed:year1998lld:pubmed
pubmed-article:9740273pubmed:articleTitleNon-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension.lld:pubmed
pubmed-article:9740273pubmed:affiliationDepartment of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.lld:pubmed
pubmed-article:9740273pubmed:publicationTypeJournal Articlelld:pubmed
pubmed-article:9740273pubmed:publicationTypeCase Reportslld:pubmed
pubmed-article:9740273pubmed:publicationTypeResearch Support, Non-U.S. Gov'tlld:pubmed