Document Detail


New syndrome of spondylospinal thoracic dysostosis with multiple pterygia and arthrogryposis.
MedLine Citation:
PMID:  9066887     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a "new" syndrome of spondylospinal thoracic dysostosis with a short curved spine and fusion of the spinous processes, short thorax with "crab-like" configuration of the ribs, pulmonary hypoplasia, severe arthrogryposis and multiple pterygia, and hypoplastic maxilla and mandible in two siblings. This appears to be an autosomal recessive lethal trait. A literature review revealed two reports of four similar or related cases.
Authors:
V P Johnson; L D Keppen; M S Carpenter; B B Randall; P E Newby
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  69     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1997 Mar 
Date Detail:
Created Date:  1997-06-24     Completed Date:  1997-06-24     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  73-8     Citation Subset:  IM    
Affiliation:
Department of Obstetrics/Gynecology, University of South Dakota School of Medicine, Sioux Falls, USA.
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MeSH Terms
Descriptor/Qualifier:
Arthrogryposis / radiography*
Dysostoses / radiography*
Female
Humans
Infant, Newborn
Pterygium / radiography*
Syndrome
Thoracic Vertebrae / radiography*

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


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