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Abstract(s)
Este trabalho tem como objetivo enfatizar as manifestações bucais das síndromes de
Apert e Crouzon e apontar informações importantes para o plano de tratamento destes
doentes. Foi realizada uma busca nas bases de dados PubMed, Scielo e BVS-Bireme,
com o intuito de revisar a literatura científica a cerca do tema. Das craniossinostoses
sindrômicas, as mais frequentes são a Síndrome de Apert e a Síndrome de Crouzon,
ambas acontecem devido a mutações no gene que codifica o recetor do Fator de
Crescimento de Fibroblastos, isto resulta num fechamento precoce das suturas
cranianas, podendo por sua vez ocasionar uma hipertensão intracraniana. O tratamento
destes pacientes, deve ser realizado em ordem inter e multidisciplinar, e o diagnóstico
precoce é de extrema importância para melhorar a qualidade de vida dos portadores e
dos seus familiares.
This work aims to emphasize the oral manifestations of the Apert and Crouzon syndromes and to point out important information for the treatment of these patients. A search was made in the databases PubMed, Scielo and BVS-Bireme, with the intention of reviewing the scientific literature on the subject. Among the syndromic craniosynostoses, the most frequent are Apert Syndrome and Crouzon Syndrome, both occur due to mutations in the gene encoding the Fibroblast Growth Factor receptor, this results in an early closure of the cranial sutures, which can cause an intracranial hypertension. The treatment of these patients must be performed in an inter and multidisciplinary order, and early diagnosis is extremely important to improve the quality of life of these patients and their families.
This work aims to emphasize the oral manifestations of the Apert and Crouzon syndromes and to point out important information for the treatment of these patients. A search was made in the databases PubMed, Scielo and BVS-Bireme, with the intention of reviewing the scientific literature on the subject. Among the syndromic craniosynostoses, the most frequent are Apert Syndrome and Crouzon Syndrome, both occur due to mutations in the gene encoding the Fibroblast Growth Factor receptor, this results in an early closure of the cranial sutures, which can cause an intracranial hypertension. The treatment of these patients must be performed in an inter and multidisciplinary order, and early diagnosis is extremely important to improve the quality of life of these patients and their families.
Description
Keywords
Acrocefalossindactilia Síndrome de Apert Disostose craniofacial Craniossinostose Síndrome de Crouzon Acrocephalosyndactylia Apert´s syndrome Dysostoses craniofacial Craniosynostosis Crouzon´s syndrome