Document Detail


The Dyggve-Melchio-Clausen syndrome.
MedLine Citation:
PMID:  401564     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two families with Dyggve-Melchior-Clausen syndrome are reported. In the first family, Jews from Morocco, six of 10 siblings are affected. In the second family, a consanguineous marriage of Arabs from Gaza, two of three children are affected. A description of the skeletal changes in patients ranging in age from 4 to 25 years is presented. The radiologic signs of generalized platyspondyly with double humped end plates and the lace-like appearance of thickened iliac crests are pathognomonic and distinctive of the syndrome. The diagnostic features of the disease are compared to those of Morguio's disease, spondyloepiphyseal dysplasia tarda, and spondylometaphyseal dysplasia.
Authors:
S Schorr; C Legum; M Ochshorn; M Hirsch; S Moses; E E Lasch; M El-Masri
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article    
Journal Detail:
Title:  AJR. American journal of roentgenology     Volume:  128     ISSN:  0361-803X     ISO Abbreviation:  AJR Am J Roentgenol     Publication Date:  1977 Jan 
Date Detail:
Created Date:  1977-02-26     Completed Date:  1977-02-26     Revised Date:  2008-02-15    
Medline Journal Info:
Nlm Unique ID:  7708173     Medline TA:  AJR Am J Roentgenol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  107-13     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Arm / radiography
Bone and Bones / radiography
Child
Child, Preschool
Female
Hip Joint / radiography
Humans
Male
Mucopolysaccharidoses / genetics,  pathology,  radiography*
Mucopolysaccharidosis IV / pathology
Pelvic Bones / radiography
Spine / radiography
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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