Document Detail


Dural ectasia is a common feature of the Marfan syndrome.
MedLine Citation:
PMID:  3189335     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Widening of the lumbosacral spinal canal was found in 63% of 57 patients with the Marfan syndrome and in none of 57 age- and sex-matched non-Marfan control patients, who underwent CT scanning for routine clinical indications. The bony abnormalities in mild cases consisted of thinning of the pedicles and taminae and erosion of the neural foraminae and were generally limited to L5 and S1. More severe changes were present in 13 patients, two of whom had associated neurologic signs, and included meningoceles or near total erosion of a pedicle. Presence and severity of vertebral abnormalities were associated with neither any other clinical feature nor overall phenotypic severity. Dural ectasia can be added to the list of pleiotropic manifestations of the Marfan syndrome.
Authors:
R E Pyeritz; E K Fishman; B A Bernhardt; S S Siegelman
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of human genetics     Volume:  43     ISSN:  0002-9297     ISO Abbreviation:  Am. J. Hum. Genet.     Publication Date:  1988 Nov 
Date Detail:
Created Date:  1988-12-01     Completed Date:  1988-12-01     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0370475     Medline TA:  Am J Hum Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  726-32     Citation Subset:  IM    
Affiliation:
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
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MeSH Terms
Descriptor/Qualifier:
Adult
Dilatation, Pathologic / radiography
Female
Humans
Male
Marfan Syndrome / genetics*,  pathology,  radiography
Phenotype
Spinal Canal / pathology,  radiography*
Tomography, X-Ray Computed
Grant Support
ID/Acronym/Agency:
RR 00722/RR/NCRR NIH HHS
Comments/Corrections

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