| A very rare case of polysplenia syndrome with congenital diffuse pulmonary arteriovenous fistulas. | |
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MedLine Citation:
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PMID: 16562797 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A five-year-old girl patient was admitted with cyanosis and dyspnea, which started from birth. She had small telangiectatic lesions on her face and cerebral arteriovenous malformation, but no family history of hereditary hemorrhagic telangiectasia. Contrast echocardiography and pulmonary angiography demonstrated diffuse pulmonary arteriovenous fistulas. The patient was diagnosed as polysplenia syndrome characterized with left atrial isomerism, interrupted inferior vena cava, azygous continuation to the superior vena cava, and hepatic veins draining to the right atrium. In contrast to the other polysplenia syndrome cases, in this patient, pulmonary arteriovenous fistulas were not associated with cavopulmonary anastomoses or liver disease. |
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Authors:
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Dolunay Gürses; Zülal Ulger; Ertürk Levent; A Ruhi Ozyürek |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: The Turkish journal of pediatrics Volume: 48 ISSN: 0041-4301 ISO Abbreviation: Turk. J. Pediatr. Publication Date: 2006 Jan-Mar |
Date Detail:
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Created Date: 2006-03-27 Completed Date: 2006-04-25 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0417505 Medline TA: Turk J Pediatr Country: Turkey |
Other Details:
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Languages: eng Pagination: 96-9 Citation Subset: IM |
Affiliation:
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Department of Pediatric Cardiology, Ege University Faculty of Medicine, izmir, Turkey. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple* Arteriovenous Malformations / complications* Child, Preschool Female Heart Defects, Congenital* Hemodynamics Humans Pulmonary Artery / abnormalities* Pulmonary Veins / abnormalities* Spleen / abnormalities* Syndrome |
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