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dc.contributor.CRUESPUniversidade Estadual de Campinaspt_BR
dc.typeArtigo de periódicopt_BR
dc.titleAbernethy malformation: One of the etiologies of hepatopulmonary syndromept_BR
dc.contributor.authorAlvarez, AEpt_BR
dc.contributor.authorRibeiro, AFpt_BR
dc.contributor.authorHessel, Gpt_BR
dc.contributor.authorBaracat, Jpt_BR
dc.contributor.authorRibeiro, JDpt_BR
unicamp.authorState Univ Campinas, Sch Med, Ctr Invest Pediat, Div Pediat Immunol Allergy & Pneumol,Dept Pediat, BR-13084020 Campinas, SP, Brazil State Univ Campinas, Sch Med, Dept Pediat, Div Pediat Gastroenterol, BR-13084020 Campinas, SP, Brazil State Univ Campinas, Sch Med, Dept Radiol, BR-13084020 Campinas, SP, Brazilpt_BR
dc.subjecthepatopulmonary syndromept_BR
dc.subjectAbernethy malformationpt_BR
dc.subjectportal veinpt_BR
dc.subjectcongenital absencept_BR
dc.subject.wosLung Perfusion Scanpt_BR
dc.subject.wosCongenital Absencept_BR
dc.description.abstractHepatopulmonary syndrome (HPS) is the clinical relationship between hepatic disease and the existence of pulmonary vascular dilatations, which can result in a range of arterial oxygenation abnormalities. It is probably caused by an alteration in the synthesis or metabolism of vasoactive pulmonary substances at a hepatic level, leading to vasodilatation of pulmonary vessels and diffusion perfusion defects. The Abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end-to-side or side-to-side shunt. Here, we report on a 5-year-and-11-month-old-boy who had started cyanosis at age 4 years and 11 months, and did not have any other pulmonary or cardiac signs or symptoms. In the investigation, arterial blood gases revealed a PaO2 of 41.4 mm Hg. The chest x-ray film and echo Doppler cardiography were normal, Nuclear scanning with Technetium 99m-labeled macroaggregated albumin showed the presence of arteriovenous shunt, at 47%. Abdominal echography revealed Abernethy malformation with an absence of portal vein. We concluded that the patient had HPS caused by Abernethy malformation, The possible mechanism is that in this malformation, there is a deviation in the blood that comes from the spleen to the vena cava without passing through the liver, so there is no metabolism of some substances which can be responsible for the imbalance between the vasodilatation and the vasoconstriction of the pulmonary circulation. Abernethy malformation must be included as one of the etiologies of hepatopulmonary syndrome. This is the first case described in the literature with this form of presentation. (C) 2002 Wiley-Liss,
dc.relation.ispartofPediatric Pulmonologypt_BR
dc.relation.ispartofabbreviationPediatr. Pulmonol.pt_BR
dc.publisher.cityNew Yorkpt_BR
dc.identifier.citationPediatric Pulmonology. Wiley-liss, v. 34, n. 5, n. 391, n. 394, 2002.pt_BR
dc.sourceWeb of Sciencept_BR
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