Document Detail


Proteus syndrome: an expanded phenotype.
MedLine Citation:
PMID:  3605210     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on 11 new cases of Proteus syndrome to illustrate the broad range of the phenotype in this hamartomatous dysplasia. The cardinal manifestations of this sporadic disorder are hemihypertrophy, macrodactyly, exostoses, scoliosis, cavernous hemangiomas, lipomas, linear sebaceous nevi, and deeply rugated soles of the feet. Intelligence is usually normal. The differential diagnosis includes Klippel-Trenaunay-Weber and partial lipodystrophy syndromes.
Authors:
R D Clark; D Donnai; J Rogers; J Cooper; M Baraitser
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  27     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1987 May 
Date Detail:
Created Date:  1987-07-24     Completed Date:  1987-07-24     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:  99-117     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Child, Preschool
Exostoses / genetics
Extremities / pathology
Female
Growth Disorders / diagnosis*,  genetics
Hamartoma Syndrome, Multiple / diagnosis*,  genetics
Hemangioma, Cavernous / genetics
Humans
Lipoma / genetics
Male
Neoplasms, Multiple Primary / diagnosis*
Nevus / genetics
Phenotype
Scoliosis / genetics

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


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