| Coronary artery disease in the Hurler syndrome. Qualitative and quantitative analysis of the extent of coronary narrowing at necropsy in six children. | |
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MedLine Citation:
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PMID: 6781324 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The amount of cross-sectional area luminal narrowing in each 5 mm segment of each of the four major epicardial coronary arteries (right, left main, left anterior descending and left circumflex) is described at necropsy in six children (aged 3 to 16 years) with the Hurler syndrome. In five patients at least one of the four major coronary arteries was narrowed 76 to 100 percent, and in four of these five patients all four major arteries were narrowed to this extent. Of the 24 major coronary arteries in the six patients, 17 (71 percent) were narrowed 76 to 100 percent at some point. A total of 182 segments were examined from the 24 major coronary arteries, and the extent of narrowing was as follows: 96 to 100 percent, 14 (8 percent); 76 to 95 percent, 61 (34 percent); 51 to 75 percent, 59 (32 percent); 26 to 50 percent, 39 (21 percent) and 0 to 25 percent, 9 (5 percent). By applying a score of 1 to 4 to each 5 mm segment according to its category of narrowing (1 = 0 to 25 percent; 2 = 26 to 50 percent; 3 = 51 to 75 percent and 4 = 76 to 100 percent), the 182 segments had a total score of 570 and a mean score of 3.2, indicating that each segment was narrowed an average of about 67 percent in cross-sectional area. Thus, narrowing of the major epicardial coronary arteries at necropsy is usually diffuse and severe in the Hurler syndrome, which is the cause of the most severe coronary narrowing in childhood. |
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Authors:
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F C Brosius; W C Roberts |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The American journal of cardiology Volume: 47 ISSN: 0002-9149 ISO Abbreviation: Am. J. Cardiol. Publication Date: 1981 Mar |
Date Detail:
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Created Date: 1981-04-24 Completed Date: 1981-04-24 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0207277 Medline TA: Am J Cardiol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 649-53 Citation Subset: AIM; IM |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Arterial Occlusive Diseases / complications*, pathology Autopsy Child Child, Preschool Coronary Disease / complications*, pathology Humans Mucopolysaccharidosis I / complications*, pathology |
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