Document Detail


'Epiphyseal dysgenesis' in Laurence-Moon-Biedl-Bardet syndrome.
MedLine Citation:
PMID:  9702668     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Ten patients with Laurence-Moon-Biedl-Bardet syndrome were investigated. They all belonged to one large family within which several intermarriages had taken place. We found that, apart from the already described abnormalities, these patients suffered from dysgenesis of the following epiphyses: vertebrae, proximal femur, proximal tibia, capitellum, tarsal navicular, and the distal epiphysis of the first metatarsal. The degree of polydactyly, a prominent feature of this syndrome, varied from patient to patient, ranging from a wide fifth metatarsal or metacarpal to a complete sixth digit. As in other epiphyseal dysgenesis, a pseudoepiphysis of the distal first metacarpal was present. One patient (the eldest) was tetraparetic due to severe spinal stenosis.
Authors:
G Moses; C Howard; J Bar-Ziv; S Dekel; M Nyska
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric orthopaedics. Part B     Volume:  7     ISSN:  1060-152X     ISO Abbreviation:  J Pediatr Orthop B     Publication Date:  1998 Jul 
Date Detail:
Created Date:  1998-10-30     Completed Date:  1998-10-30     Revised Date:  2011-10-14    
Medline Journal Info:
Nlm Unique ID:  9300904     Medline TA:  J Pediatr Orthop B     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  193-8     Citation Subset:  IM    
Affiliation:
Department of Orthopaedic and Traumatic Surgery, Ichilov Hospital, Jerusalem, Israel.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Child, Preschool
Diagnosis, Differential
Epiphyses / abnormalities*
Female
Humans
Israel
Laurence-Moon Syndrome / diagnosis*,  physiopathology
Male
Pedigree
Prognosis
Range of Motion, Articular

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


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