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Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family

Objective: This article describes the diagnosis, clinical and microscopic (histopathology and ultrastructural) features and treatment of a new family with hereditary gingival fibromatosis (HGF) and highlights the importance of this genetic condition. Study Design: To characterize the pattern of inhe...

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Autores principales: Pego, Sabina-Pena-Borges, Coletta, Ricardo D., Mendes, Danilo-Cangussu, de Faria, Paulo-Rogério, Melo-Filho, Mário R., Alves, Lucas-Rodrigues, Martelli-Júnior, Hercílio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medicina Oral S.L. 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4393976/
https://www.ncbi.nlm.nih.gov/pubmed/25475776
http://dx.doi.org/10.4317/medoral.20170
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author Pego, Sabina-Pena-Borges
Coletta, Ricardo D.
Mendes, Danilo-Cangussu
de Faria, Paulo-Rogério
Melo-Filho, Mário R.
Alves, Lucas-Rodrigues
Martelli-Júnior, Hercílio
author_facet Pego, Sabina-Pena-Borges
Coletta, Ricardo D.
Mendes, Danilo-Cangussu
de Faria, Paulo-Rogério
Melo-Filho, Mário R.
Alves, Lucas-Rodrigues
Martelli-Júnior, Hercílio
author_sort Pego, Sabina-Pena-Borges
collection PubMed
description Objective: This article describes the diagnosis, clinical and microscopic (histopathology and ultrastructural) features and treatment of a new family with hereditary gingival fibromatosis (HGF) and highlights the importance of this genetic condition. Study Design: To characterize the pattern of inheritance and the clinical features, members of a new family with HGF were examined. The pedigree was reliably constructed including the four latest generations of family. Hematoxylin and eosin staining and ultrastructural analysis were performed with the gingival tissue. Results: Examination of the family pedigree revealed that the patient III-2 represent the index patient of this family (initial patient with a mutation), which was transmitted to her daughter through an autosomal dominant mode of inheritance. The affected patients showed a generalized gingival overgrowth. The patient was treated with surgical procedures of gingivectomy and gingivoplasty. The diagnosis was confirmed by histopathology examination that showed a well-structured epithelium with elongated and thin papillae inserted in fibrous connective tissue with increased amount of collagen. The ultrastructural aspects of the tissue show collagen fibrils exhibiting their typically repeating banding pattern with some fibrils displaying loops at their end. Moreover, it was possible to seen in some regions fibrillar component presenting tortuous aspects and loss of the alignment among them. Conclusions: This HGF frequently resulted in both esthetic and functional problems. The genetic pattern of this Brazilian family suggested a new mutation, which was later transmitted by an autosomal dominant trait. Key words:Gingival fibromatosis, genetic disease, pedigree, ultrastructure.
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spelling pubmed-43939762015-04-13 Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family Pego, Sabina-Pena-Borges Coletta, Ricardo D. Mendes, Danilo-Cangussu de Faria, Paulo-Rogério Melo-Filho, Mário R. Alves, Lucas-Rodrigues Martelli-Júnior, Hercílio Med Oral Patol Oral Cir Bucal Research Objective: This article describes the diagnosis, clinical and microscopic (histopathology and ultrastructural) features and treatment of a new family with hereditary gingival fibromatosis (HGF) and highlights the importance of this genetic condition. Study Design: To characterize the pattern of inheritance and the clinical features, members of a new family with HGF were examined. The pedigree was reliably constructed including the four latest generations of family. Hematoxylin and eosin staining and ultrastructural analysis were performed with the gingival tissue. Results: Examination of the family pedigree revealed that the patient III-2 represent the index patient of this family (initial patient with a mutation), which was transmitted to her daughter through an autosomal dominant mode of inheritance. The affected patients showed a generalized gingival overgrowth. The patient was treated with surgical procedures of gingivectomy and gingivoplasty. The diagnosis was confirmed by histopathology examination that showed a well-structured epithelium with elongated and thin papillae inserted in fibrous connective tissue with increased amount of collagen. The ultrastructural aspects of the tissue show collagen fibrils exhibiting their typically repeating banding pattern with some fibrils displaying loops at their end. Moreover, it was possible to seen in some regions fibrillar component presenting tortuous aspects and loss of the alignment among them. Conclusions: This HGF frequently resulted in both esthetic and functional problems. The genetic pattern of this Brazilian family suggested a new mutation, which was later transmitted by an autosomal dominant trait. Key words:Gingival fibromatosis, genetic disease, pedigree, ultrastructure. Medicina Oral S.L. 2015-03 2014-12-05 /pmc/articles/PMC4393976/ /pubmed/25475776 http://dx.doi.org/10.4317/medoral.20170 Text en Copyright: © 2015 Medicina Oral S.L. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research
Pego, Sabina-Pena-Borges
Coletta, Ricardo D.
Mendes, Danilo-Cangussu
de Faria, Paulo-Rogério
Melo-Filho, Mário R.
Alves, Lucas-Rodrigues
Martelli-Júnior, Hercílio
Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title_full Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title_fullStr Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title_full_unstemmed Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title_short Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
title_sort hereditary gingival fibromatosis: clinical and ultrastructural features of a new family
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4393976/
https://www.ncbi.nlm.nih.gov/pubmed/25475776
http://dx.doi.org/10.4317/medoral.20170
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