Double heterozygous pathogenic mutations in KIF3C and ZNF513 cause hereditary gingival fibromatosis / 国际口腔科学杂志·英文版

Hereditary gingival fibromatosis (HGF) is a rare inherited condition with fibromatoid hyperplasia of the gingival tissue that exhibits great genetic heterogeneity. Five distinct loci related to non-syndromic HGF have been identified; however, only two disease-causing genes, SOS1 and REST, inducing HGF have been identified at two loci, GINGF1 and GINGF5, respectively. Here, based on a family pedigree with 26 members, including nine patients with HGF, we identified double heterozygous pathogenic mutations in the ZNF513 (c.C748T, p.R250W) and KIF3C (c.G1229A, p.R410H) genes within the GINGF3 locus related to HGF. Functional studies demonstrated that the ZNF513 p.R250W and KIF3C p.R410H variants significantly increased the expression of ZNF513 and KIF3C in vitro and in vivo. ZNF513, a transcription factor, binds to KIF3C exon 1 and participates in the positive regulation of KIF3C expression in gingival fibroblasts. Furthermore, a knock-in mouse model confirmed that heterozygous or homozygous mutations within Zfp513 (p.R250W) or Kif3c (p.R412H) alone do not led to clear phenotypes with gingival fibromatosis, whereas the double mutations led to gingival hyperplasia phenotypes. In addition, we found that ZNF513 binds to the SOS1 promoter and plays an important positive role in regulating the expression of SOS1. Moreover, the KIF3C p.R410H mutation could activate the PI3K and KCNQ1 potassium channels. ZNF513 combined with KIF3C regulates gingival fibroblast proliferation, migration, and fibrosis response via the PI3K/AKT/mTOR and Ras/Raf/MEK/ERK pathways. In summary, these results demonstrate ZNF513 + KIF3C as an important genetic combination in HGF manifestation and suggest that ZNF513 mutation may be a major risk factor for HGF.

Hereditary gingival fibromatosis: a case report / Fibromatosis gingival hereditaria: informe de caso

Introduction: Hereditary gingival fibromatosis is a rare disorder with a genetic component that may appear during tooth replacement. This condition can cause functional and aesthetic problems such as malocclusions, diastemas, pain when chewing, dental caries, periodontal disease, delayed eruption, among others. Objective: To report the multidisciplinary treatment provided to a patient with hereditary gingival fibromatosis. Case Report: This report describes the treatment carried out in a thirteen-year-old male patient presenting generalized increase in gingival volume associated with functional and aesthetic compromise and delayed eruption of permanent teeth. After diagnosis, a multidisciplinary intervention was proposed, involving periodontal and pediatric dentistry procedures, which improved the quality of life of the patient both functionally and aesthetically. Conclusion: Hereditary gingival fibromatosis not only affects the dental eruption process, but also causes aesthetic and emotional alterations in the patient. The periodontal procedures significantly improved the appearance, function, and the psychological state of the patient.

Introducción: La fibromatosis gingival hereditaria es una altera-ción poco común, asociada a un componente genético que en ocasiones se hace evidente en el recambio dentario. Este padecimiento puede generar problemas funcionales y estéticos como maloclusiones, diastemas, dolor al masticar, caries, enfermedad periodontal, erupción tardía, entre otros. Objetivo: Reportar el caso clínico con manejo interdisciplinario en un paciente con fibromatosis gingival hereditaria. Reporte de Caso: Se expone el tratamiento realizado en un paciente de trece años, sexo masculino, con aumento de volumen gingival generalizado con compromiso funcional y estético, conjugado con retraso en la erupción de dientes permanentes. Tras diagnóstico se plantea la intervención multidisciplinaria, integrando áreas como periodoncia y odontopediatría; los procedimientos ejecutados permitieron mejorar la calidad de vida desde el punto de vista funcional y estético. Conclusión: La fibromatosis gingival hereditaria no solo desencadena alteración en proceso eruptivo dental, sino también alteraciones estéticas y emocionales en el paciente que la padece. Los procedimientos perio-dontales realizados permitieron de forma categórica la mejora de la estética, función y estado psicológico del paciente.

Generalized hereditary gingival fibromatosis in a child: clinical, histopathological and therapeutic aspects

Hereditary gingival fibromatosis (HGF) is a rare genetic condition characterized by slow and progressive gingival enlargement. The gingival overgrowth often delays teeth eruption and may cause serious functional and aesthetic problems. We reported a case of a 10-year-old female child presenting a generalized gingival enlargement covering almost all the maxillary and mandibular teeth and resulted in problems for swallowing, speaking, and poor aesthetics. An incisional biopsy was performed and revealed a hypocellular and hypovascular dense collagenous tissue covered by squamous epithelium exhibiting acanthosis and elongated rete ridges. The diagnosis was HGF. The treatment instituted was an association of gingivectomy with a rigorous program of oral hygiene and follow-up. Herein, we describe a rare non-syndromic case of generalized HGF, including clinical and microscopical features, as well as highlighting the importance of correct diagnosis of this genetic condition.

Hereditary gingival fibromatosis: a three-generation case report / 华西口腔医学杂志

Hereditary gingival fibromatosis (HGF) is a familial hereditary disease; while it is rare and usually benign, it is also characterized by the slow and progressive development of gingival tissue. This paper reports on the clinical examina-tion and history of HGF in a family of patients.

Eficacia inhibitoria del aceite esencial de Cymbopogon Citratus sobre cepas de Porphyromona Gingivalis: Estudio in vitro / Eficácia inhibitória do óleo essencial de Cymbopogon Citratus em cepas de Porphyromona Gingivalis: Estudio in vitro / Inhibitory effect of the essential oil of Cymbopogon Citratus on the strain of Porphyromona Gingivalis: In Vitro Study

Las plantas con propiedades terapéuticas utilizadas en la asistencia sanitaria tradicional han sido estudiadas ampliamente en los últimos años y constituyen una alternativa importante frente a diferentes tratamientos odontológicos.Objetivo: De-terminar el efecto inhibitorio del aceite esencial de Cymbopogon Citratus (CC) a diferentes concentraciones sobre cepas de Porphyromonas Gingivalis (Pg). Materiales y métodos: Estudio experimental, in vitro. La muestra fue constituida por 24 cajas Petri con cultivos de Pg ATCC® 33277™ en Agar Mueller Hinton, cada una con 5 discos correspondiente a los 5 grupos de estudio siendo: G1 aceite esencial de CC al 100%; G2 aceite esencial de CC al 50%; G3 aceite esencial de CC al 75%; G4 clorhexidina 0,12% (control positivo); G5 suero fisiológico (control negativo). A las 24 horas de incubación se efectúo la medición de los halos utilizando la regla Antibiotic Zone Scale (mm) con los parámetros de sensibilidad deter-minados por la escala de Duraffourd et al., 1986 siendo: Nula (-) < 8mm, Sensible (+) >8mm ≤14mm, Muy sensible (++) >14 ≤ 20mm y Sumamente sensible (+++) > 20mm. Se utilizó para el análisis los test de ANOVA y Tukey con un nivel de significancia del 5%. Resultados: El aceite esencial de CC mostró un halo de inhibición de 14mm, 12mm y 10mm a concentraciones de 100%, 50% y 75% respectivamente. La Clorhexidina 0,12% se observó valores de inhibición de 17mm. Se observó diferencia estadísticamente significativa entre los grupos (< 0.001). Conclusión: El aceite esencial de CC al 100%, 50% y 75% demostraron ser sensibles, frente a la Porphyromona Gingivalis.

Plants with therapeutic properties used in traditional healthcare have been studied in recent years and are an important alternative to different dental treatments. Objective: To determine the inhibitory effect of the essential oil of Cymbopogon Citratus (CC) at different levels on the Porphyromonas Gingivalis (Pg) strain. Materialsandmethods: Experimental study, in vitro. The sample consisted of 24 Petri dishes with cultures of Pg ATCC® 33277 ™ in Mueller Hinton Agar, each with 5 discs corresponding to the 5 study groups being: G1 essential oil of 100% CC; G2 50% CC essential oil; G3 75% CC essential oil; G4 chlorhexidine 0.12% (positive control); G5 physiological saline (negative control). At 24 hours of incubation, the halos are measured using the Antibiotic Zone Scale (mm) with the sensitivity parameters determined by the Duraffourd scale et al, 1986 being: Null (-) <8 mm, Sensitive (+) > 8 mm ≤14 mm, Very sensitive (++) > 14 ≤ 20mm and Extremely sensitive (+++)> 20mm. It was used for the ANOVA and Tukey tests analysis with a level of significance of 5%. Results: The CC essential oil showed a halo of inhibition of 14 mm, 12 mm and 10 mm at concentrations of 100%, 50% and 75% respectively. Chlorhexidine 0.12% haunted values of 17mm inhibition. There was a statistically significant difference between the groups (<0.001). Conclusion: The essential oil of CC 100%, 50% and 75% proved to be sensitive, against the Porphyromona Gingivalis.

Plantas com propriedades terapêuticas utilizadas nos cuidados se saúde tradicionais têm sido estudadas extensivamente nos últimos anos e são uma alternativa importante para diferentes tratamentos dentários. Objetivo: Determinar o efeito inibitó-rio do óleo essencial de Cymbopogon Citratus (CC) em diferentes concentrações em cepas de Porphyromonas Gingivalis(Pg). Materiais e métodos: Estudo experimental, in vitro. A amostra foi constituída por 24 placas de Petri com culturas de Pg ATCC® 33277 ™ em Ágar Mueller Hinton, cada uma com 5 discos correspondentes aos 5 grupos de estudo sendo: G1 óleo essencial de 100% CC; G2 50% de óleo essencial de CC; G3 75% de óleo essencial de CC; Clorexidina G4 0,12% (controle positivo); G5 soro fisiológico (controle negativo). Após 24 horas de incubação a medição dos halos foi realizada utilizando a regra Antibiotic Zone Scale (mm) com parâmetros de sensibilidade determinados pela escala de Duraffourd et al, 1986 sendo: nulo. (-) <8 milímetros, sensível (+ )> 8mm ≤14mm, Muito sensível (++)> 14 ≤ 20mm e Extremamen-te sensível (+++)> 20mm. Os testes ANOVA e Tukey com nível de significância de 5% foram utilizados para a análise. Resultados: O óleo essencial de CC apresentou halo de inibição de 14mm, 12mm e 10mm nas concentrações de 100%, 50% e 75%, respectivamente. Valores de inibição de clorexidina 0,12% de 17mm foram observados. Observamos diferença estatisticamente significante entre os grupos (<0,001). Conclusão: O óleo essencial de 100% CC, 50% e 75% mostrou-se sensível, contra o Porphyromona Gingivalis.

Fibromatosis gingival hereditaria: una rara enfermedad. Reporte de una familia / Hereditary Gingival Fibromatosis: A Rare Disease. A Family Report / Fibromatose gengival hereditária uma estranha doença: reporte de uma família

Resumen Introducción: la fibromatosis gingival hereditaria es un desorden genético raro que produce un sobre-crecimiento gingival y el desplazamiento dental asociado, la patogénesis y la base molecular de la enfermedad sigue siendo desconocida. Dado que es una enfermedad poco frecuente, es importante que el profesional en el área de la salud oral conozca las características clínicas, histológicas y genéticas de la enfermedad con el objetivo de realizar un correcto diagnóstico, plan de tratamiento y orientación sobre la condición de la patología. Presentación del caso: se reporta informe de una familia con tres generaciones afectadas con fibromatosis gingival hereditaria, en la que se describen las características clínicas, histopatológicas y tratamiento. Conclusión: la fibromatosis gingival hereditaria es un trastorno poco frecuente que genera diversos grados de aumento en el volumen gingival, los compromisos estéticos y funcionales a menudo requieren intervención quirúrgica, histológicamente es común la presencia de abundantes haces de colágeno y fibroblastos.

Abstract Introduction: Hereditary gingival fibromatosis is a rare genetic disorder that produces a gingival overgrowth and the associated dental displacement, the pathogenesis and the molecular basis of the disease remains unknown. Given that it is a rare disease, it is important that the professionals in the field of oral health know the clinical, histological and genetic characteristics of the disease in order to make a correct diagnosis, a treatment plan and the guidance on the condition of the pathology. Case presentation: Report of a family with three generations affected with hereditary gingival fibromatosis, in which the clinical, histopathological and treatment characteristics are described. Conclusions: Hereditary gingival fibromatosis is a rare disorder that generates varying degrees of increase in gingival volume, aesthetic and functional compromises often require surgical intervention, the presence of abundant bundles of collagen and fibroblasts is histologically common.

Resumo Introdução: a fibromatose gengival hereditária é uma desordem genética estranha que produz um so-brecrescimento gengival e o deslocamento dental associado, a patogênese e a base molecular da doença segue sendo desconhecida. Dado que é uma doença pouco frequente, é importante que o profissional na área da saúde oral conheça as características clínicas, histológicas e genéticas da doença com o objetivo de realizar um correto diagnóstico, plano de tratamento e orientação sobre a condição da patologia. Apresentação do caso: reporta-se o informe de uma família com três gerações afetados com fibromatose gengival hereditária, no que se descrevem as características clínicas, histopatológicas e tratamento. Conclusão: a fibromatose gengival hereditária é um transtorno pouco frequente que gera diversos graus de aumento no volume gengival, os compromissos estéticos e funcionais frequentemente requerem intervenção cirúrgica, histologicamente é comum a presença de abundantes feixes de colágeno e fibroblastos.

Immunohistochemical Analysis of Cell Proliferation and Bcl-2 Expression in Drug-Induced Gingival Overgrowth

Objective: To compare the rate of cell proliferation and expression of antiapoptotic protein Bcl-2 between drug-induced gingival overgrowth (DIGO) and clinical healthy gingiva (CHG) and to establish associations with histopathological features. Material and Methods: Twenty specimens of DIGO and 20 CHG specimens were submitted to morphological and immunohistochemical analysis by light microscopy. Cell proliferation (Ki-67) and the expression of Bcl-2 were evaluated in epithelial cells and spindle-shaped mononuclear cells of the connective tissue by establishing the labeling index (LI). Results: In epithelial tissue, the mean LI for Ki-67 was 17.2% in DIGO and 21.71% in CHG (p = 0.137). The mean LIs for Bcl-2 in epithelial tissue were 14.67% and 10.24% in DIGO and CHG, respectively (p = 0.026). In connective tissue, DIGO and CHG specimens exhibited low LIs for Ki-67 and Bcl-2, with mean values of less than 0.5% in both groups. No significant differences in the LIs for Ki-67 or Bcl-2 in epithelial tissue were observed according to the degree of collagenization, degree of vascularization and intensity of inflammatory infiltration (p > 0.05). No significant correlations were observed between the LIs for Ki-67 and Bcl-2 (p > 0.05). Conclusion: The present results suggest that the pathogenesis of DIGO does not involve increased proliferation or decreased apoptosis of fibroblasts. On the other hand, the morphological pattern of elongated epithelial cristae observed in DIGO could mainly be due to the inhibition of keratinocyte apoptosis and not to increased proliferation of these cells.

Fibroma gingival de células gigantes de tamaño inusual / Gingival giant cell fibroma of unusual size

Giant cell fibroma is a benign oral fibrous tumor and it is typically an asymptomatic sessile or pedunculated mass that is usually less than 1 cm in diameter. The lesion consists of uninflamed fibrous tissue in which there are numerous large uninucleated or multinucleated spindle- and stellate-shaped cells with prominent basophilic cytoplasm. The purpose of this paper is to report a case of a gingival giant cell fibroma of abnormal size. A 31-year-old white woman was referred to the dental service for evaluation of a growth on the mandibular gingival. The intraoral examination revealed a 3.0 × 1.5 cm exophytic gingival mass located in the lingual gingiva of the right mandibular permanent first and second molars. The differential diagnosis included peripheral ossifying fibroma, peripheral giant cell granuloma, and giant cell fibroma. Complete surgical excision of the lesion was performed and the diagnosis of giant cell fibroma was made. No complications or recurrence of the lesion have been noted after 4 years of follow-up. Although giant cell fibromas are benign lesions in which simple surgical excision is curative, it is very important that dental and medical professionals recognize it in light of the frequency of occurrence and the need for a precise diagnosis(AU)

El fibroma de células gigantes es un tumor fibroso benigno de la mucosa bucal que típicamente se presenta como una masa asintomática sésil o pediculada generalmente menos de 1 cm de diámetro. La lesión consiste en tejido fibroso no inflamado en el que se encuentran numerosas células fusiformes y estrelladas de gran tamaño, mononucleares o multinucleadas con prominente citoplasma basófilo. El propósito de este trabajo es describir el caso de un fibroma gingival de células gigantes de tamaño inusual. Una mujer blanca de 31 años de edad se presentó al servicio dental para la evaluación de un crecimiento en la encía mandibular. El examen clínico intrabucal reveló una masa gingival exofítica de 3,0 cm x 1,5 cm situado en la encía lingual en el área de los primeros y segundos molares permanentes mandibulares del lado derecho. El diagnóstico diferencial incluyó fibroma osificante periférico, granuloma periférico de células gigantes y fibroma de células gigantes. Se realizó la escisión quirúrgica completa de la lesión y el diagnóstico definitivo fue de fibroma de células gigantes. No se han observado complicaciones o recurrencia de la lesión después de 4 años de seguimiento. Aunque los fibromas de células gigantes son lesiones benignas en las que la escisión quirúrgica simple es curativa, es muy importante que los profesionales dentales y médicos reconozcan la necesidad de un diagnóstico preciso en vista de la frecuencia de aparición(AU)

Extensa fibromatose gengival idiopática: relato de caso / Extensive Idiopathic Gingival Fibromatosis: A Case Report

A Fibromatose Gengival (FG) é descrita como uma condição bucal rara, clinicamente manifestada por um crescimento lento, progressivo, difuso e benigno dos tecidos gengivais. Essa condição pode se manifestar de forma isolada, em associação a outras doenças sistêmicas ou como componente de síndromes. A FG pode ter uma etiologia identificável ou ser idiopática. Em função da severidade de cada caso, pode acarretar transtornos funcionais e estéticos significativos, sobretudo, relacionado à dificuldade de higienização, fala e deglutição, devido à formação de grandes massas teciduais na gengiva. O presente trabalho propõe a revisão dos aspectos clínicos, diagnósticos e terapêuticos da Fibromatose Gengival e relata um caso severo desta doença que foi tratada cirurgicamente... (AU)

The Gingival Fibromatosis (GF) is described as a rare oral condition, clinically manifested by a slow, progressive, diffuse, and benign growth of gingival tissues. This condition can manifest itself in isolation, in combination with other systemic diseases or as a component of syndromes. GF may have an identifiable etiology or be idiopathic. Depending on the severity of each case, GF may result in significant functional and aesthetic disorders, mainly related to the difficulty of cleaning, speech and swallowing due to formation of large gingival tissue masses. This paper proposes a review of the clinical features, diagnosis and treatment of gingival fibromatosis and reports a severe case of this condition that was surgically treated... (AU)

Fibromatosis Gingival Idiopática Unilateral: Reporte de un Caso con Cuatro Años de Seguimiento / Unilateral Idiopathic Gingival Fibromatosis: Case Report With Four-Year Follow-Up

La fibromatosis gingival es una enfermedad rara que se caracteriza por aumento del tejido gingival por proliferación fibrosa, es de crecimiento lento, cubriendo en algunos casos la totalidad de los dientes comprometidos. Esta nosología comprende un grupo heterogéneo de patologías de causas no determinadas. El objetivo de este trabajo es presentar un caso clínico de una paciente de 13 años con FGI Unilateral, su diagnóstico, tratamiento y seguimiento durante cuatro años. Presenta al examen clínico intraoral, agrandamiento anormal de su encía tanto superior como inferior, afectando solamente los hemiarcos del sector izquierdo. A la palpación es indolora y de consistencia fibrosa. No se encontraron factores locales que justifiquen dicho aumento. El examen físico elimina la posibilidad de otras patologías asociadas, no refiriendo recibir medicaciones inmunosupresivas, antiepilépticos o antihipertensivas. No tiene antecedentes familiares. La paciente fue sometida a extirpación quirúrgica del tejido hiperplasiado y el material obtenido enviado a estudio histopatológico. Después del tratamiento quirúrgico y una buena higiene oral con exámenes de control efectuados durante cuatro años no se observó recurrencia. A pesar de ser una patología poco frecuente el odontólogo debe conocer la fisiopatología y comprender que el manejo de ésta enfermedad es a través de un equipo multidisciplinario. El diagnóstico precoz y el tratamiento oportuno logran la recuperación psicológica, estética y funcional de los pacientes.

Gingival fibromatosis is a rare disease characterized by progressive enlargement of the gingiva caused by an increase in submucosal connective tissue. The enlargement may potentially cover the exposed tooth surfaces. Idiopathic gingival fibromatosis (IGF) is a heterogeneous group of disorders with no definite cause. The aim of this study is to present a 13-year-old female patient with unusual clinical forms of Unilateral FGI with a complete analysis of the features of the clinical diagnosis, treatment and follow-up for four years. Intraoral examination revealed severe gingival overgrowth involving both the upper arch and the lower arch, affecting on the left side. The right side of the mouth was unaffected. There was no significant pain. The lesion was diffuse, firm and fibrotic. Her medical and family history was also non-contributory. The patient was not receiving any antiepileptic, antihypertensive, or immunosuppressive medications that could contribute to the gingival enlargement. Histopathological examination showed hyperparakeratinized stratified squamous epithelium, presence of a thickened acanthotic epithelium and focal hyperplasia. Underlying connective tissue stroma was collagenous with numerous lymphoplasmacytic infiltrate. Unusual findings include the presence of calcified particles. Bevel gingivectomy was taken to remove gingival overgrowth. This case is thus a rare and atypical presentation of gingival fibromatosis. The patient was advised to maintain good oral hygiene to minimize the effect of inflammation on fibroblasts. In our case, even after four years of follow-up, no recurrence of gingival overgrowth was observed. Unilateral IGF is a relatively rare condition with poorly understood etiopathogenesis and recurrence rates. The cases should be treated with a multidisciplinary management. Treatment appreciably improved the patient's psychological, aesthetics and masticatory competence.

Enamel renal syndrome with associated amelogenesis imperfecta, nephrolithiasis, and hypocitraturia: A case report

Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a case of amelogenesis imperfecta of the enamel agenesis variety with nephrolithiasis in a 21-year-old male patient who complained of small teeth. The imaging modalities employed were conventional radiography, cone-beam computed tomography, and renal sonography. Such cases are first encountered by dentists, as other organ or metabolic diseases are generally hidden. Hence, cases of amelogenesis imperfecta should be subjected to advanced diagnostic modalities, incorporating both dental and medical criteria, in order to facilitate comprehensive long-term management.

Crescimentos gengivais: relato de caso / Gingival overgrowth: case reports

As lesões nos tecidos moles da cavidade oral, principalmente aquelas relacionadas ao aumento gengival, são comumente encontradas pelos cirurgiões-dentistas. Dentre estas condições, que geram aumento gengival e podem ser encontradas em tecidos moles, temos: a hiperplasia fibrosa inflamatória (HFI), a fibromatose gengival hereditária (FGH) e o granuloma piogênico (GP). A HFI se enquadra entre as lesões benignas do tecido mole decorrentes de traumas crônicos, geralmente de uma prótese mal adaptada. A FGH é descrita como uma condição bucal rara, clinicamente manifestada por um crescimento lento, progressivo, difuso e benigno dos tecidos gengivais. O GP é considerado um processo proliferativo reacional não neoplásico, podendo acometer pele e mucosas. O presente estudo relatou estas três condições de tecido mole que geram aumento gengival.

Unilateral Gingival Fibromatosis: a case report / Fibromatose Gengival Unilateral: um relato de caso

The gingival fibromatosis is a slow and progressive benign proliferation, which affects the gingival tissues. It may present a genetic inheritance and association with some syndromes. There are conservative and radical treatments, ranging from hygiene care to bloc resection of the affected bone. This case scenario is a 07 year-old child, who presented a nodular unilateral hyperplastic lesion in the right mandible, with sessile base and approximately 5 cm in its largest diameter. The patient presented difficulty of lip closure and slight swelling in the right area of the face. The tomographic image showed infiltration in buccal and lingual cortical of right mandible and tooth displacement. After lesion removal, the histopathologic diagnosis of fibromatosis was confirmed, with no relapse after 20 months of follow-up.

A Fibromatose Gengival é uma proliferação benigna, lenta e progressiva, que afeta os tecidos gengivais. Pode apresentar herança genética e associação à uma série de síndromes. Existem tratamentos conservadores e radicais, desde cuidados com higiene à ressecção em bloco do tecido ósseo afetado. Este é o caso clínico de uma criança, que apresentou lesão hiperplásica unilateral em mandíbula, com deslocamento dentário. Após remoção, o diagnóstico de hiperplasia gengival foi confirmado e não há recidiva da lesão até o momento.

Prevalencia de lesiones benignas y desórdenes potencialmente malignos que afectan la mucosa bucal en pacientes adultos Facultad de Odontoloía UCV / Injury prevalence of benign and potencially malignant disorders affecting the mouth in patients mucosal Faculty of Dentistry UCV

Se realiza un estudio de tipo documental, retrospectivo, transversal. La población del presente estudio estuvo representada por un total de 9.000 historias diagnosticadas en el Laboratorio Central de Histopatología Bucal "Dr. Pedro Tinoco" de la Facultad de Odontología de la U.C.V., durante el período 1988-2008. La muestra evaluada quedó conformada por un total de Cuatro Mil Ciento Sesenta y Seis (4.166) casos, los cuales fueron seleccionados de manera intencional no probabilística a partir de la población antes mencionada siguiendo criterios de inclusión y exclusión. La prevalencia de Lesiones Benignas y Desórdenes Potencialmente Malignos que afectan la Mucosa Bucal es alta (46,2%) con respecto a la muestra total estudiada (9000 casos). De acuerdo al Grupo Etario observamos un intervalo de edad entre 15 y 97 años, una media de 47,3 años y Desv. Tip de 16,5 (Y ± s : 47,3 ± 16,5). En relación a la distribución por Género, en nuestro estudio existe un predominio de las lesiones de la mucosa bucal por el sexo femenino (69,7%). En base al diagnóstico clínico se identificaron 77 lesiones diferentes. El Fibroma Traumático fue la lesión más común con 1042 casos (25,01%), seguida por la Leucoplasia con 764 casos (18,33%), la Hiperplasia Fibrosa por Prótesis Dental con 447 casos (10,73%). En referencia a las diferentes localizaciones anatómicas mayormente afectadas en este estudio: el Reborde Alveolar ocupó el primer lugar (1134 casos; 27,2%), seguida por Los Carrillos (639 casos; 15,33%), Labio Inferior (522 casos; 12,5%), Encías (493 casos; 11,8%).En referencia al grado de Concordancia global entre el Diagnóstico Clínico e Histopatológico fue del 75,9 % de los casos (3.161 casos de 4.166). Situación que nos permite asumir que la concordancia global es satisfactoria.

A study of documentary, retrospective, cross. The study population was represented by a total of 9,000 stories diagnosed at the Central Laboratory of Oral Histopathology "Dr. Pedro Tinoco " in the Faculty of Dentistry at UCV, during the period 1988-2008 . The sample studied was composed of a total of Four Hundred Sixty Six Thousand (4,166 ) cases , which were selected intentionally not random from the population above following inclusion and exclusion criteria . Injury prevalence Benign and Potentially Malignant Disorders affecting Mucosa is high ( 46.2 %) compared to the total study sample (9000 cases). According to Age Group observed an age range between 15 and 97 years , an average of 47.3 years and 16.5 Desv.Tip (Y ± s : 47.3 ± 16.5). Regarding the Gender distribution in our study there is a prevalence of oral mucosal lesions in females (69.7 %). Clinical diagnoses based on 77 different lesions were identified. The Traumatic fibroma was the most common injury in 1042 patients (25.01%), followed by leukoplakia with 764 cases (18.33%), Fibrous Hyperplasia By Dental Implants with 447 cases (10.73%). Referring to different anatomical locations most affected in this study: Alveolar Flange ranked first (1134 cases , 27.2 %), followed by The Cheeks (639 cases , 15.33%), Lower Lip (522 cases; 12.5%), Gum (493 cases, 11.8%). Referring to the degree of overall concordance between the Clinical and Histopathological diagnosis was 75.9% of cases (3,161 of 4,166 cases). This situation allows us to assume that the overall agreement is satisfactory.

Fibromixoma odontogénico en el maxilar superior: reporte de un caso clínico y revisión de la literatura / Fibromixoma odontogenic in the maxilla: report of a case and literture review

El Fibromixoma Odontogénico es una variante del Mixoma Odontogénico. Se describe como una lesión intraósea agresiva derivada del tejido conjuntivo embrionario asociada con la odontogénesis1 constituida principalmente por grandes cantidades de tejido fibroso celular maduro. Su origen es controvertido, aparece en el esqueleto facial, afectando con mayor frecuencia a la mandíbula2. A continuación se presenta el caso clínico de un paciente de sexo femenino de 36 años de edad que presentó un aumento de volumen a nivel del ápice de diente 1.6 ,en la que se realizó un curetaje logrando la completa resección de la lesión, el resultado del informe patológico da el diagnóstico de Fibromixoma de origen Odontogénico

The Odontogenic Fibromyxoma is a variant of the Odontogenic Myxoma. It is described as an agressive intraoseous lesion that derives from the embrionary connective tissue associated with the odontogenesis, constituted by great amounts of celular mature fibrous tissue. It has a controverted origin, appears in the facial skeleton affecting more frecuently the mandible. We present a case of a 36 year old female who consulted with an increase of volume in relation to the 1.6 theet where we practiced a curetaje obtaining a complete resection of the lesion, the results of the patologic inform gives the diagnostic of odontogenic fibromyxoma

Diferencias histomorfológicas entre tejido gingival clínicamente sano de niños y adultos jóvenes / Differences between histomorphologic clinically healthy gingival tissue of children and young adults

Existe controversia con respecto a la influencia del factor histomorfológico en la menor severidad en la respuesta inflamatoria del tejido gingival de la población infantil con respecto a la población adulta ante la biopelícula dental. El objetivo de ésta investigación fue identificar las diferencias histomorfológicas entre el tejido gingival clínicamente sano de niños y adultos jóvenes. Se realizaron exodoncias de dientes temporales y permanentes sanos con su tejido gingival adherido a niños de 6 a 10 y adultos de 18 a 25 años de edad, para posterior análisis histomorfológico bajo microscopía de luz. El área total del epitelio de unión fue mayor en los niños presentando mayor grosor y número de capas en la zona apical. Se observó además, infiltrado celular inflamatorio adyacente al epitelio de unión, mayor cantidad de vasos, predominio de densidad laxa en las fibras del tejido conectivo y paraqueratinización del epitelio bucal. No se encontraron diferencias en la amplitud de los tejidos ni en su espesor con respecto a los epitelios bucal y surcular, tampoco en las dimensiones del tejido conectivo ni en el grosor de la capa córnea. El presente estudio confirmó la existencia de diferencias histomorfológicas entre los tejidos gingivales de niños y adultos jóvenes en condiciones de salud clínica, resaltando la importancia del factor histomorfológico, como uno de los parámetros que podrían influir en la respuesta de los tejidos gingivales a la biopelícula dental a diferentes de edades

The effect of the histomorphologic features in the minor severity of the inflammatory response in gingival tissues against the dental biofilm in childhood is matter of controversy. The objective was to identify the differences between the histomorphologic characteristics in healthy gingival tissues of children and young adults. Extraction of healthy temporal and permanent teeth and its gingival tissues were carried out in children of 6 -10 years old and adults of 18 - 25 years old, the histological samples were processed and analyzed using light microscopy. Children showed a bigger area of the junctional epithelium, more thickness of the apical area as well as a high number of union layers; presence of inflammatory cell infiltrated around the junctional ephytelium, increased number of blood vessels, predominance of laxe density of the connective tissue and pharaqueratinization of the oral ephytelium. There were no differences in the width and thickness of the oral epithelium and surcular tissues, the width of the connective tissue and the thickness of the stratum corneum did not showed differences. The present study confirmed the occurrence of histomorphologic differences in healthy gingival tissues between children and young adults, highlighting the importance of this parameter as one of the characteristics that could influence the gingival response to the dental biofilm at different age

Fibromatosis gingival hereditaria una inusual enfermedad genética: reporte de caso / Hereditary gingival fibromatosis a rare genetic disease: case report

Asociada a factores genéticos, que se caracterizada por aumento en el tamaño del tejido gingival, el cual genera dificultades emocionales, estéticas y funcionales. En el presente artículo se reporta un caso de una paciente femenina de 13 años con aumento generalizado en el volumen de la encía, que cubre casi todos los dientes, la historia familiar fue muy importante para el diagnóstico de fibromatosis gingival hereditaria, ya que la madre y un hermano presentaron la misma manifestación.

Hereditary gingival fibromatosis is a rare disorder, associated with genetic factors, characterized by various degrees of attached gingival overgrowth, which generates emotional, aesthetic and functional disorders. This article reports the case of a 13-year-old female who presented a generalized severe gingival overgrowth, involving the maxillary and mandibular arches and covering almost the whole dentition. The family history was very important for the diagnosis of hereditary gingival fibromatosis, as the mother and a brother had the same disorder.

Fibroma odontogênico periférico: relato de caso de uma lesão rara mimetizando uma lesão reacional / Peripheral odontogenic fibroma: case report of a rare tumor mimicking a gingival reactive lesion

Objetivo: Relatar um caso de um tumor raro imitando uma lesão gengival inflamatória tendo em foco não apenas as suas características, mas realizando um diagnóstico diferencial aprofundado com as lesões inflamatórias; a fim de colaborar para um diagnóstico mais eficiente por parte dos clínicos. Descrição do caso: Um paciente do sexo feminino, de 20 anos de idade apresentava uma massa gengival séssil e assintomática - entre os dentes 32 e 33 - com duração aproximada de 1 ano. Após biópsia excisional, a análise histológica revelou um neoplasma odontogênico caracterizado pela presença de ilhas de epitélio odontogênico aparentemente inativo, localizado em um estroma fibro-mixóide. Características consistentes com o diagnóstico de fibroma odontogênico periférico. Conclusão: O fibroma odontogênico periférico é freqüentemente confundido com uma lesão reacional inflamatória. No entanto, a presença de particularidades de cada lesão pode auxiliar o clínico, a fim de se obter um diagnóstico mais preciso. A biópsia excisional é o tratamento mais indicado. Porém, o prognóstico do fibroma odontogênico periférico pode ser diferente, devido a sua raridade e pouca informação sobre seu acompanhamento de longo prazo.

Purpose: To report a rare tumor mimicking a gingival inflammatory lesion focusing not only on its characteristics, but making a deepened differential diagnosis with inflammatory lesions, in terms to collaborate for an accurate diagnosis as possible by the clinicians. Case description: A 20-year-old woman presented an asymptomatic sessile gingival mass - between teeth 32 and 33 - with 1 year of duration. An excisional biopsy was performed and the histological examination revealed an odontogenic neoplasm characterized by the presence of islands of apparently inactive odontogenic epithelium, in a fibromyxoid stroma; all features consistent with the diagnosis of peripheral odontogenic fibroma. Conclusion: Peripheral odontogenic fibroma is often misdiagnosed as an inflammatory lesion. However, the existence of particularities of each lesion can guide the clinical to a more efficient diagnosis. Excisional biopsy is their treatment of choice, whereas; the peripheral odontogenic fibroma prognosis may be different given its rarity and its few data about long-term follow-up.

Gingival fibromatosis with multiple unusual findings: report of a rare case / 国际口腔科学杂志·英文版

Gingival fibromatosis is a rare disease, especially its syndromic form. Here, we review the literatures on gingival fibromatosis and briefly summarize some characters on clinical, etiological, genetic and histopathological aspects. We also present a rare case of gingival fibromatosis with multiple unusual findings in a 21-year-old man. And we differentiate it from some well-known syndromes including gingival fibromatosis. Maybe it implies a new syndrome within the spectrum of those including gingival fibromatosis.

Fibroma traumático: dos presentaciones clínicas una misma entidad / Traumatic fibroma: two clinical presentations same entity

El Fibroma Traumático (FT) se considera la patología reactiva de tejido conjuntivo más frecuente de la cavidad bucal, cuya etiología es el trauma crónico. Es más frecuente en la segunda década de la vida, se presenta sin predilección por grupo étnico o género y aparece en áreas frecuentemente traumatizadas como carrillos, bordes laterales de lengua y labio inferior. Se presentan dos casos, ambos pacientes presentados en este trabajo fueron de sexo femenino, la primera de ellas de 59 años de edad y portadora de prótesis total superior, que mostró al examen clínico una lesión particular donde la presión mecánica de la prótesis ocasionó un crecimiento tumoral de 1cm de diámetro, color rosa pálido, lisa y brillante que se desprende del paladar duro a través de un pedículo. Esta lesión al examen clínico podría pasar desapercibida por la forma como se apoya sobre la mucosa del paladar. La otra paciente mostraba la lesión típica que caracteriza al FT, patología tumoral, sésil, en labio superior lado izquierdo, antre la comisura y la línea media del paciente, aproximadamente 6mm de diámetro, color rojizo y erosionada en la superficie, asociada a fricción crónica posterior a un trauma. La conducta a seguir para ambos casos fue extirpación quirúrgica y estudio histopatológico, obteniéndose como diagnóstico definitivo FT. La importancia de estos casos se basa en que ambas patologías aunque tienen el mismo diagnóstico histopatológico poseen presentaciones clínicas completamente distintas y una de ellas inusual, esto probablemente originado por el agente causal

Traumatic fibrosis (TF) is considered the reactive connective tissue disease more common in the oral cavity, the etiology is chronic trauma. It is more common in the second decade of life, is presented without preference for gender or ethnic group and appears frequently traumatized areas such as cheeks, lateral edges of tongue and lower lip. Both cases was in female patients, the first is 59 years old and superior total prosthesis, clinical examination showed a particular injury where the mechanical pressure of the prosthesis caused a tumor growth completely flattened 1cm in diameter, pale pink, smooth and bright that it appears the hard palate through a pedicle. This injury to the clinical examination may go unnoticed by the way is based on the mucosa of the palate. The other patient showed the typical lesion characteristic of the FT, tumor pathology, sessile on the upper lip, approximately 6mm of diameter, reddish and eroded on the surface friction associated with chronic post-trauma. The conduct to be followed in both cases was surgical excision and histopathology, obtained as a definitive diagnosis: TF. The importance of these cases is based on both disorders but have the same histopathological diagnosis have completely different clinical presentations and one of them is unusual, this probably originated by the causal agent