| Valvular heart disease in four patients with Maroteaux-Lamy syndrome. | |
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MedLine Citation:
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PMID: 1728449 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Maroteaux-Lamy syndrome is a lysosomal storage disease of mucopolysaccharide metabolism (MPS type VI) that may involve the mitral and aortic valves. Affected patients have other skeletal and oropharyngeal malformations that complicate anesthetic and surgical management. METHODS AND RESULTS: The present report describes the clinical, echocardiographic, and pathological findings in four patients with Maroteaux-Lamy syndrome. Two of three siblings underwent successful double-valve replacement for aortic and mitral valve stenoses. The third sibling, whose aortic and mitral valves were thick and fibrotic, died from septicemia after hip surgery. A fourth, unrelated patient also had successful double-valve replacement. CONCLUSIONS: Our experience emphasizes the potential difficulties in preoperative assessment and surgical treatment as well as the unique problems related to airway management in patients with this syndrome. |
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Authors:
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C T Tan; H V Schaff; F A Miller; W D Edwards; P S Karnes |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Circulation Volume: 85 ISSN: 0009-7322 ISO Abbreviation: Circulation Publication Date: 1992 Jan |
Date Detail:
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Created Date: 1992-02-06 Completed Date: 1992-02-06 Revised Date: 2010-03-24 |
Medline Journal Info:
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Nlm Unique ID: 0147763 Medline TA: Circulation Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 188-95 Citation Subset: AIM; IM |
Affiliation:
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Section of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aortic Valve Stenosis / etiology*, pathology, ultrasonography Echocardiography Echocardiography, Doppler Female Heart Valve Prosthesis Humans Male Mitral Valve Stenosis / etiology*, pathology, ultrasonography Mucopolysaccharidosis VI / complications*, pathology, ultrasonography |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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