| An unusual case of Klippel-Trénaunay-Weber syndrome presenting with portosystemic encephalopathy. | |
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MedLine Citation:
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PMID: 18797123 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report a unique male patient presenting with portosystemic encephalopathy (PSE) due to intrahepatic portohepatic venous (PHV) shunts. He was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS) based on the findings of a hemitruncal port-wine stain with subcutaneous arteriovenous fistulae and varicose veins in the legs. However, limb-hypertrophy, which is one of the most cardinal manifestations of KTWS, was absent, and in KTWS, PSE is quite a rare clinical manifestation. Hence, the clinical picture of this patient was unusual. Our clinical observation suggests that KTWS can be one of the underlying disorders causing PSE. |
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Authors:
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Masahide Yazaki; Kazuma Kaneko; Kana Tojo; Daigo Miyazaki; Yasuhiro Shimojima; Kazuhiko Ueda; Shu-ichi Ikeda |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2008-09-16 |
Journal Detail:
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Title: Internal medicine (Tokyo, Japan) Volume: 47 ISSN: 1349-7235 ISO Abbreviation: Intern. Med. Publication Date: 2008 |
Date Detail:
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Created Date: 2008-09-17 Completed Date: 2009-01-07 Revised Date: 2009-04-16 |
Medline Journal Info:
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Nlm Unique ID: 9204241 Medline TA: Intern Med Country: Japan |
Other Details:
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Languages: eng Pagination: 1621-5 Citation Subset: IM |
Affiliation:
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Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto. mayazaki@hsp.md.shinshu-u.ac.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Angiography Hepatic Encephalopathy / etiology, pathology* Humans Klippel-Trenaunay-Weber Syndrome / complications, diagnosis*, pathology* Male Middle Aged Port-Wine Stain / etiology Tomography, X-Ray Computed |
| Comments/Corrections | |
Comment In:
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Intern Med. 2009;48(6):493-4; author reply 495
[PMID:
19293555
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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