Document Detail


Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis.
MedLine Citation:
PMID:  3987735     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In 1983 the Proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses after surgical interventions in the regions involved. Histology of the adipose tissue showed considerable anisocytosis and increased cell volume.
Authors:
J Mücke; H Willgerodt; R Künzel; D Brock
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of pediatrics     Volume:  143     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  1985 Mar 
Date Detail:
Created Date:  1985-06-20     Completed Date:  1985-06-20     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  320-3     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Connective Tissue / pathology
Female
Fingers / abnormalities
Humans
Hypertrophy
Infant
Keratosis / genetics
Lipomatosis / genetics*
Nevus, Pigmented / genetics
Phenotype
Skin Neoplasms / genetics
Syndrome
Toes / abnormalities

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


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