Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy
Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Skin and skeletal developmental malformations are common and may assume tremendous proportions. The syndrome is often mistaken for other, more co...
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Veröffentlicht in: | Oral surgery, oral medicine, oral pathology, oral radiology and endodontics oral medicine, oral pathology, oral radiology and endodontics, 1998, Vol.85 (1), p.82-85 |
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creator | Pinto, Pascal X Beale, Victoria Paterson, Anthony W |
description | Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Skin and skeletal developmental malformations are common and may assume tremendous proportions. The syndrome is often mistaken for other, more commonly recognized conditions, including neurofibromatosis. The soft-tissue masses in Proteus syndrome are not nerve tumors but are usually hamartomatous proliferations. The case report describes its varied manifestations, which include significant cranifoacial dysmorphism, and discusses the differential diagnosis and management. |
doi_str_mv | 10.1016/S1079-2104(98)90403-6 |
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Skin and skeletal developmental malformations are common and may assume tremendous proportions. The syndrome is often mistaken for other, more commonly recognized conditions, including neurofibromatosis. The soft-tissue masses in Proteus syndrome are not nerve tumors but are usually hamartomatous proliferations. 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The case report describes its varied manifestations, which include significant cranifoacial dysmorphism, and discusses the differential diagnosis and management.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Complex syndromes</subject><subject>Dentistry</subject><subject>Facial Asymmetry - etiology</subject><subject>Female</subject><subject>Humans</subject><subject>Mandible - abnormalities</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Proteus Syndrome - complications</subject><subject>Proteus Syndrome - pathology</subject><issn>1079-2104</issn><issn>1528-395X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1998</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpNkU1LxDAQhoMoun78hIUcRPRQTZo2abzIIn6BoKCCt5CmUxppm5pkV_bf23VX8TQD78MwMw9CU0rOKaH84oUSIZOUkuxUFmeSZIQlfAtNaJ4WCZP5-_bY_yJ7aD-ED0IIZ1Luol2ZiYynZILKZ-8izAMOy77yroNLPMNGB8AeBucjdjXWuNGd9tF1Orp_KP6yscEBFuABd7qvbDlvtccNdLZZDuCjd0OzPEQ7tW4DHG3qAXq7vXm9vk8en-4ermePCaSSxiQTXBqoNc2kYDmUFeRlpitDZV5k1NCCF0SQMiVcM6FNzlJeAKlFZYjhsmbsAJ2s5w7efc4hRNXZYKBtdQ_j2kpILnPKxAhON-C87KBSg7fjeUu1ecqYH29yHYxua697Y8MfllKWF2KFXa0xGI9aWPAqGAu9gcp6MFFVzipK1EqW-pGlViaULNSPLMXZN7ukhx8</recordid><startdate>1998</startdate><enddate>1998</enddate><creator>Pinto, Pascal X</creator><creator>Beale, Victoria</creator><creator>Paterson, Anthony W</creator><general>Mosby, Inc</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>1998</creationdate><title>Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy</title><author>Pinto, Pascal X ; Beale, Victoria ; Paterson, Anthony W</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-e291t-4769cefa149735ebde5b4adc195841c1868070b206a37ac53268e0f7dc0c69f33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1998</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Complex syndromes</topic><topic>Dentistry</topic><topic>Facial Asymmetry - etiology</topic><topic>Female</topic><topic>Humans</topic><topic>Mandible - abnormalities</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Proteus Syndrome - complications</topic><topic>Proteus Syndrome - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Pinto, Pascal X</creatorcontrib><creatorcontrib>Beale, Victoria</creatorcontrib><creatorcontrib>Paterson, Anthony W</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Oral surgery, oral medicine, oral pathology, oral radiology and endodontics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pinto, Pascal X</au><au>Beale, Victoria</au><au>Paterson, Anthony W</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy</atitle><jtitle>Oral surgery, oral medicine, oral pathology, oral radiology and endodontics</jtitle><addtitle>Oral Surg Oral Med Oral Pathol Oral Radiol Endod</addtitle><date>1998</date><risdate>1998</risdate><volume>85</volume><issue>1</issue><spage>82</spage><epage>85</epage><pages>82-85</pages><issn>1079-2104</issn><eissn>1528-395X</eissn><abstract>Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. 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source | MEDLINE; Elsevier ScienceDirect Journals |
subjects | Adult Biological and medical sciences Complex syndromes Dentistry Facial Asymmetry - etiology Female Humans Mandible - abnormalities Medical genetics Medical sciences Proteus Syndrome - complications Proteus Syndrome - pathology |
title | Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy |
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