| Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. | |
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MedLine Citation:
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PMID: 3609666 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In a series of 40 patients with Budd-Chiari syndrome, 5 (12%) were found to be afflicted with paroxysmal nocturnal hemoglobinuria. The clinico-pathological features in these 5 patients and in 26 well-documented previously reported cases could be ascribed to three groups of increasing severity: thrombosis limited to small-sized hepatic veins with no or transient ascites, partial thrombosis of large-sized hepatic veins with chronic ascites, and complete thrombosis of large-sized hepatic veins with a life-threatening course. These three groups did not differ with regard to sex, age, and duration and characteristics of paroxysmal nocturnal hemoglobinuria. In view of the relationship between prognosis and the extent of hepatic vein obstruction, we suggest that early therapy directed toward limiting the extension of thrombosis, or toward dissolving formed thrombi, should improve the prognosis of this severe complication of paroxysmal nocturnal hemoglobinuria. |
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Authors:
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D Valla; D Dhumeaux; G Babany; P Hillon; B Rueff; H Rochant; J P Benhamou |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Gastroenterology Volume: 93 ISSN: 0016-5085 ISO Abbreviation: Gastroenterology Publication Date: 1987 Sep |
Date Detail:
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Created Date: 1987-09-09 Completed Date: 1987-09-09 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0374630 Medline TA: Gastroenterology Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 569-75 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Budd-Chiari Syndrome / etiology*, pathology, therapy Female Hemoglobinuria, Paroxysmal / complications* Humans Liver / pathology Male Prognosis |
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