Abstract Fanconi anemia is a rare autosomal recessive disease. In this disease, cytokine pathways can induce the bone marrow failure that is observed in individuals with Fanconi anemia. Interleukin IL-17 exhibits a protective effect in organisms because it induces neutrophil recruitment and shows a pathological role in several models of autoimmune diseases, periodontal disease, cancer, allograft rejection, and graft versus host disease. Polymorphisms in the IL17A and IL17RA genes were evaluated from DNA in saliva, comparing individuals with or without Fanconi anemia, using models of genotypic transmission (additive, dominant, and recessive). Polymorphisms in the IL17A and IL17RA genes (rs2241044 [C allele], rs879577 [C allele], rs9606615 [T allele], and rs2241043 [C allele]) were risk factors for developing Fanconi anemia. We also performed an analysis of gene markers with clinical variables in the Fanconi group. Polymorphisms in the IL17A gene (rs3819025 [A allele] and rs2275913 [G allele], respectively) were associated with an age of less than 20 years (p = 0.026; RP 0.65) and the female sex (p = 0.043; RP 0.88). The IL17RA gene was also associated with age and the presence of leukoplakia (a potentially malignant oral disorder). An age of less than 20 years was associated with rs917864 (T allele; p = 0.036; RP 0.67). The presence of leukoplakia was associated with rs17606615 (T allele; p = 0.042; RP 0.47). To our knowledge, this is the first study that associates IL17A and IL17RA gene polymorphisms with Fanconi anemia and examines rs2241044 polymorphisms in scientific literature thus far.

Abstract: The objective of this study is to present a tool to help understand how variables associated with oral cancer prevention relate to each other in a social network. A search of the Scopus database was performed using terms related to oral cancer and prevention from 2000 to 2020. The keywords were used as nodes and were analyzed using NodeXL, which produced the network graphic analysis. From the 1004 publications available, 4038 different keywords were obtained and then grouped into 75 constructs based on conceptual similarity. The most influential nodes were risk factors, comorbidities, epidemiology, and treatment. However, topics such as technology, telemedicine, self-examination, and diagnostic delay remain far removed from central relations. Network analysis enabled us to observe the bias of biological and basic science in the field and identify a need for studies concerning primary prevention, behavioral interventions, and inequalities in oral cancer.

Abstract: This study aimed to describe the telediagnosis of oral lesions through a telehealth program offered in the State of Paraná, southern Brazil. This report included all oral medicine-related teleconsultations between January 2015 and December 2019. Primary care dentists from the public health services of the state were trained to use the Telessaúde Brasil Redes platform, a national telehealth program that provides telediagnostic support through teleconsultations with specialists. Clinical information and images of oral lesions were solicited to each teleconsultation request. An oral medicine specialist evaluated the cases and provided diagnostic hypotheses, management, and referral suggestions. Finally, dentists were invited to evaluate the services. A total of 162 cases were submitted by 44 dentists. The patient's main complaint was described in 98.8% of cases, while the duration/evolution and the type of lesions were 64.81% and 40.12%, respectively. No images were attached to 19 (11.70%) patients. The information sent was sufficient for diagnostic reasoning in 125 (77.16%) requests. The specialist considered 78 cases (48.1%) to be resolved in primary care. Among respondents (45.7%), dentists considered that the teleconsultation “totally attended” their needs in 92% of requests. The description of the telehealth platform usage in oral medicine in Paraná showed the need to improve the participation of primary care dentists and the quality of clinical information provided as barriers to be overcome, aiming for the best usage of the platform.

Abstract Objective: To analyze if the oral health conditions in children and adolescents are associated with hemophilia (PROSPERO-42020168192). Material and Methods: The search strategy was performed in PubMed, Scopus, Lilacs/BBO, Web of Science, Cochrane, and Grey literature databases. Two independent researchers assessed the risk of bias in these studies by the Newcastle-Ottawa Scale. For the meta-analysis, the clinical conditions data were extracted as numerical variables according to their indexes, such as dental caries experience (dmft/DMFT), gingival condition (Modified Gingival Index - IGM), and oral hygiene (Plaque Index - PI). The quality of the evidence of the meta-analysis was evaluated by the GRADE tool (GRADEproGDT). Results: From a total of 431 studies, 27 were included, and 10 were included in the meta-analysis. The studies presented a moderate risk of bias, ranging from 2 to 7 points. The dental caries experience in primary (-0.62; CI95%: -1.68–0.43) and permanent dentitions (-0.05; CI95%: -0.69–0.59), gingival condition (-0.12; CI95%: -0.27–0.03), and oral hygiene (0.36; CI95%: -0.06–0.77) did not differ between the groups. Conclusion: Based on studies with very weak evidence, there were no differences in the oral health conditions of children and adolescents with and without hemophilia.

ABSTRACT Introduction: Fanconi anemia (FA) is a rare genetic disease characterized by congenital malformations and bone marrow failure. One of the most common oral diseases in individuals with FA is periodontitis and adequate self-perception of periodontal status could contribute to its prevention and early detection. Aim: To compare oral health self-perception, measured by a questionnaire, with the clinical oral condition of patients with FA. Methods and Results: Fifty-six patients with FA, over 11 years of age, answered a questionnaire about dental history and self-reported oral health. Decayed, missing, and filled teeth (DMFT), Visible Plaque Index (VPI) and Gingival Bleeding Index (GBI) were measured. The median age of participants was 21 years (min 11, max 44), 31 (55%) were females and 25 (45%) males. Thirty-five (62.5%) participants rated their oral condition as satisfactory and 7 (12.5%) participants reported tooth mobility, 10 (17.9%) exposed roots and 21 (37.5%) gingival bleeding. Clinical examination detected average DMFT = 5.23, VPI = 31.36% and GBI = 33.77%. The gingival bleeding report was more frequent among individuals with higher GBI (p = 0.014). The DMFT was higher in those who had already undergone dental treatments (p = 0.031). There was an association between participants who presented dental caries and who rated their oral health as poor (p = 0.03). The question “Do your gums bleed easily?” had good accuracy in the evaluation of periodontal disease (p = 0.68). Conclusion: Oral health self-perception of individuals with FA about gingival inflammation was associated with their gingival bleeding index.

Resumen Objetivo: Describir la incidencia y el puntaje de la mucositis oral (MO) y las morbilidades relacionadas en individuos sometidos a trasplante de células madre hematopoyéticas (TCMH) a lo largo del período de inmunosupresión. Métodos: Los sujetos con enfermedades onco / hematológicas, mayores de 14 años, sometidos a TCMH alogénico fueron evaluados diariamente por la presencia y clasificación de OM, nivel de dolor, disfagia, disgeusia y xerostomía. El examen comenzó dos días antes de la infusión de células madre hematopoyéticas y finalizó veinte días después. La OM se clasificó de acuerdo con la escala de la OMS y se utilizó la escala analógica visual (EVA) para medir el nivel de dolor. Resultados: Se reclutaron 23 individuos, el 83% con enfermedades malignas y el 91% con OM. La mediana del grado máximo de OM fue 3 y el nivel máximo de dolor fue 9. Hubo una mediana de 11 días de uso de medicación opioide. Los sujetos que tuvieron el mayor número de días con dolor en la boca alcanzaron el grado máximo de OM y el mayor número de días y el uso de opioides. Conclusión: Hubo una alta incidencia y puntuaciones más altas de OM, pérdida de masa corporal y dolor en esta muestra.

Abstract Aim: To describe the oral mucositis (OM)` incidence and score, and related morbidities in individuals submitted to Hematopoietic Stem Cell Transplantation (HSCT) throughout the immunosuppression period of time. Methods: Subjects with onco / hematological diseases, older than 14 years, submitted to allogeneic HSCT were daily evaluated by the presence and classification of OM, pain level, dysphagia, dysgeusia and xerostomia. The examination started two days before the infusion of hematopoietic stem cells and ended twenty days later. The OM was classified according to the WHO scale and visual analog scale (VAS) was used to measure pain level. Results: Twenty-three individuals were recruted, 83% with malignant diseases and 91% had OM. The median of maximum OM degree was 3 and the maximum pain level was 9. There was a median of 11 days of opioid medication use. The subjects who had the highest mean number of days with mouth pain reached the maximum degree of OM and higher number of days and opiod use. Conclusion: There was a high incidence and high scores of OM, loss of body mass and pain in this sample.

ABSTRACT: The peripheral ossifying fibroma (POF) is a rare, reactive and inflammatory gingival overgrowth, arising on the interdental papilla, from cells derived of the periodontal ligament. Usually its diameter is less than 1.5 cm. It’s uncommon in newborns, frequently occurring in teenagers and young adult. The purpose of this report is to describe a case of FOP in a six months old baby. She had a nodular lesion, located on the gingival mucosa of the lower incisor area, of approximately 1.5 cm; it was firm, smooth, pedunculated, with an equal color of the underlying mucosa. A neonatal tooth had been removed four months back. Although FOP is a rare injury, it can be found in infants with less than one year of age, which is why it is important to make the right diagnosis, and an opportune treatment, to avoid further complications due to its early establishment and rapid growth.

RESUMEN: El fibroma osificante periférico (FOP) es una hipertrofia benigna de los tejidos. Se localiza en la región de la papila interdental, proveniente de células que derivan del ligamento periodontal. Generalmente mide menos de 1,5cm de diámetro, siendo muy raro en recién nacidos. El objetivo de la presentación de este caso clínico es describir un caso de FOP en un bebé de 6 meses de edad. La niña presentaba una lesión nodular localizada en la mucosa gingival, en el área de incisivos inferiores, de aproximadamente 1,5cm diámetro, de consistencia firme, lisa, y coloración igual a la de la mucosa subyacente. Un diente neonatal había sido removido a los 2 meses de nacida. El FOP es una lesión poco común y que raramente puede encontrarse en bebés menores de un año de edad; por este motivo es importante realizar el diagnóstico adecuado, para evitar cualquier complicación debido a su establecimiento precoz y rápido crecimiento.

Studies assessing the prevalence of oral lesions show that 5.2% to 12.8% of biopsy specimens are from children and adolescents. In Brazil, there are few studies analyzing the distribution of oral lesions in that population. Therefore, the aim of this study was to evaluate the distribution of oral and maxillomandibular lesions of adolescents, admitted to the Department of Oral Diagnosis of the Federal University of Paraná (UFPR) from 1994 to 2013. A study based on the review of the charts was done and the lesions were classified in 11 categories: salivary glands disease, dental pathology, gingival and periodontal pathology, odontogenic cysts, odontogenic tumors, non odontogenic cysts, bone pathology, mucosal pathology, connective tissue pathology, malignant tumors and other pathology. Variables including age, sex, ethnicity, diagnosis and lesion location were also evaluated. Upon analysis, 376 lesions were identified, most in girls, 51.9% and 77.1 % of patients were white. The most frequent site was the lower lip followed by the gingiva. Mucocele was the most common lesion (27.6%), followed by fibroepithelial hyperplasia (8.2%) and pyogenic granuloma (5.3%). Although there is a wide range of pathologies that can occur in the oral cavity, traumatic injuries are the most common in this age group.

Los estudios que evalúan la prevalencia de lesiones orales muestran que entre el 5,2% hasta el 12,8% de las muestras de biopsia corresponden a niños y adolescentes. En Brasil, hay pocos estudios que hayan analizado la distribución de las lesiones orales enesa población. Por lo tanto, el objetivo de este estudio fue evaluar la distribución de las lesiones orales y maxilofaciales de adolescentes, ingresados en el Servicio de Diagnóstico Oral de la Universidad Federal de Paraná (UFPR) entre 1994 a 2013. Se llevó a cabo la revisión de historias clínicas y las lesiones halladas fueron clasificadas en 11 categorías: enfermedad de glándulas salivales, patología dental, patología gingival y periodontal, quistes odontogénicos, tumores odontogénicos, quistes no odontogénicos, patología ósea, patología de la mucosa, patología del tejido conectivo, tumores malignos y otras patologías. También se evaluaron otras variables como edad, género, etnia, diagnóstico y localización de la lesión. Luego del análisis, se identificaron 376 lesiones, la mayoría en niñas (51,9%), siendo el 77,1% de los pacientes de raza blanca. La localización más frecuente fue el labio inferior seguida de la encía. El mucocele fue la lesión más común (27,6%), seguido de hiperplasia fibroepitelial (8,2%) y granuloma piógeno (5,3%). Aunque existe una amplia serie de patologías que pueden ocurrir en la cavidad oral, las lesiones traumáticas son las más comunes en este grupo de edad.

A Anemia de Fanconi é uma desordem genética recessiva e rara caracterizada por uma instabilidade cromossômica que induz a alterações congênitas nos indivíduos. Anemia aplásica pela falência progressiva da medula óssea, neoplasias malignas como leucemia mielóide aguda, tumores de fígado e carcinoma espinocelular, são algumas das possíveis evoluções da Anemia de Fanconi. Algumas dessas doenças desenvolvem-se principalmente após o transplante de medula óssea. O objetivo desta revisão crítica da literatura é discorrer sobre as principais manifestações bucais e sua implicação na saúde dos indivíduos doentes da Anemia de Fanconi. Os achados clínicos bucais descritos na literatura incluem alterações periodontais, como gengivite e periodontite agressiva; úlcera aftosa recorrente e lesão traumática. A atrofia papilar, macroglossia, pigmentação melânica e o carcinoma espinocelular são as manifestações bucais mais comuns na língua. Um aumento do risco ao desenvolvimento de neoplasias malignas em indivíduos com Anemia de Fanconi é relatado, sendo este progressivo após o transplante de medula óssea. Nas radiografias são observadas anomalias dentárias como a presença de supranumerários, agenesias dentárias, giroversão e transposição dentária. O fluxo salivar e alguns componentes salivares também estão alterados. Devido à suscetibilidade aumentada ao desenvolvimento do câncer nessa população específica, torna-se importante para o cirurgião dentista conhecer as manifestações bucais comuns e as lesões cancerizáveis para realizar um diagnóstico precoce nos indivíduos com Anemia de Fanconi.

Fanconi Anemia is a recessive and rare genetic disorder, characterized by chromosomal instability that induces congenital alterations in individuals. Aplastic anemia due to the progressive failure of the bone marrow, malignant neoplasias such as acute myeloid leukemia, liver tumors and squamous cell carcinoma are some of the possible evolutions of Fanconi Anemia. Some of these diseases develop mainly after bone marrow transplantation. The aim of this critical review of the literature was to discourse about the main oral manifestations and their involvement in the health of individuals who are ill with Fanconi Anemia. The clinical oral findings described in the literature include periodontal changes, such as gingivitis and aggressive periodontitis, recurrent aphthous ulcers and traumatic lesions. Papillary atrophy, macroglossia, melanic pigmentation and squamous cell carcinoma are the most common oral manifestations on the tongue. An increased risk for the development of malignant neoplasias in individuals with Fanconi Anemia has been reported, and this is progressive after bone marrow transplantation. In radiographs, dental anomalies such as the presence of supernumerary teeth, tooth agenesis, tooth rotation and transposition of teeth are observed. Salivary flow and some salivary components are also altered. Due to the increased susceptibility to the development of cancer in this specific population, it is important for the dentist to know the common oral manifestations and potentially cancerous lesions, in order to make an early diagnosis in individuals with Fanconi Anemia.

Long-term survivors of hematopoietic stem cell transplantation are recognized as a risk group for malignization. Malignant oral neoplasms are increasingly being reported in the literature as a consequence of lesions of chronic graft-versus-host disease, and prolonged multidrug treatment to control its manifestations. This report describes a 43-year-old patient who, after allogeneic bone marrow transplantation, developed an oral squamous cell carcinoma secondary to the use of azathioprine, cyclosporine, prednisone, and tacrolimus, associated with multiorgan chronic graft-versus-host disease involving the oral mucosa, skin, eyes, and liver. This report aims to discuss the possible role of immunosuppressant therapy for chronic graft-versus-host disease on the development of oral squamous cell carcinoma, and the relevance of a close oral follow-up of patients to detect dysplastic or malignant alterations at an early stage.

O avanço verificado no enfrentamento de neoplasias malignas por meio dos sistemas de saúde envolve melhorias nas áreas de vigilância, organização de redes de assistência, programas específicos voltados às prevenções primária e secundária e, obviamente, aos avanços técnico-científicos que caracterizam a abordagem diagnóstica e terapêutica. Embora seja notável o reconhecimento de avanços no manejo de neoplasias malignas em todas as áreas citadas, o câncer da boca permanece com indicadores de morbidade e mortalidade que parecem não acompanhar o acúmulo científico no conhecimento da doença. O presente manuscrito objetiva discutir os motivos desse descompasso, a necessidade de reorientação de prioridades na abordagem do câncer da boca e sua efetivação como política pública de saúde.

Progress in cancer management by health systems involves improvements in surveillance, organization of healthcare services, specific programs focused on primary and secondary prevention, and scientific and technical advances in diagnosis and treatment. Despite well-known progress in the management of malignant neoplasms in all the above areas, oral cancer displays persistently high morbidity and mortality rates, apparently failing to reflect the accumulated scientific knowledge on the disease. The current article discusses the reasons for this mismatch, the need for redefining priorities in oral cancer management, and the implementation of such priorities as a public health policy.

O avanço verificado no enfrentamento de neoplasias malignas por meio dos sistemas de saúde envolve melhorias nas áreas de vigilância, organização de redes de assistência, programas específicos voltados às prevenções primária e secundária e, obviamente, aos avanços técnico-científicos que caracterizam a abordagem diagnóstica e terapêutica. Embora seja notável o reconhecimento de avanços no manejo de neoplasias malignas em todas as áreas citadas, o câncer da boca permanece com indicadores de morbidade e mortalidade que parecem não acompanhar o acúmulo científico no conhecimento da doença. O presente manuscrito objetiva discutir os motivos desse descompasso, a necessidade de reorientação de prioridades na abordagem do câncer da boca e sua efetivação como política pública de saúde.

Progress in cancer management by health systems involves improvements in surveillance, organization of healthcare services, specific programs focused on primary and secondary prevention, and scientific and technical advances in diagnosis and treatment. Despite well-known progress in the management of malignant neoplasms in all the above areas, oral cancer displays persistently high morbidity and mortality rates, apparently failing to reflect the accumulated scientific knowledge on the disease. The current article discusses the reasons for this mismatch, the need for redefining priorities in oral cancer management, and the implementation of such priorities as a public health policy.

OBJETIVO: Odontodisplasia regional (OR) é uma condição rara caracterizada por achados clínicos, radiográficos e microscópicos bem distintos. É o resultado de um distúrbio de desenvolvimento bem localizado afetando tanto o ectoderma quanto o mesoderma odontogênico. Este trabalho descreveu os achados clínicos e radiográficos de um caso de OR e os tratamentos. DESCRIÇÃO DO CASO: Um menino de 15 anos de idade apresentou um caso incomum de OR envolvendo dois quadrantes dos maxilares no lado direito da face. CONCLUSÃO: Há necessidade de uma equipe multidisciplinar complexa para reabilitar inteiramente um paciente com OR. O tratamento de OR é controverso e varia de acordo com as características e resposta do paciente.

PURPOSE: Regional odontodysplasia (RO) is a rare condition characterized by distinctive clinical, radiographic and microscopic findings. It is presumed to be the result of a developmental disturbance that locally affects the odontogenic ectodermal and mesodermal tissues. This report describes the clinical and radiographic findings of RO and its treatment. CASE DESCRIPTION: A 15-year-old boy presented with an uncommon case of RO involving two quadrants of the jaws on the right side of his face. CONCLUSION: A complex multidisciplinary team is required to fully rehabilitate a patient affected by RO. The treatment of RO is controversial and varies according to the individual patient.

Investigation in oral cancer comprises many different fields such as epidemiology, risk factors, biological markers, diagnostic testing, screening, treatment and prognosis. Although many researchers have dealt with the oral cancer problem, it is unknown if any public policy is capable of reducing its incidence around the world under the scrutiny of the scientific method. This paper aims to briefly review and discuss the literature regarding oral cancer public policies and to screen the evidence of controlled implementations of oral health policies that have been able to diminish oral cancer incidence around the globe.

A anemia de Fanconi (AF) é uma doença autossômica recessiva heterogênea que pode causar uma variedade de alterações congênitas e de desenvolvimento. As características mais importantes da AF são a falência progressiva de medula óssea e o desenvolvimento de condições malignas, como leucemia mielóide aguda e tumores sólidos. Este artigo relata o caso de um paciente de 12 anos com AF atendido no Serviço de Estomatologia e Prevenção do Câncer Bucomaxilofacial do Hospital São Lucas (Brasil), que havia sido submetido a transplante de medula óssea (TMO) aos 5 anos e apresentaava lesões características da doença do enxerto versus hospedeiro (DEVH) crônica. O paciente foi regularmente acompanhado no Serviço e, 11 anos após a realização do TMO, desenvolveu um carcinoma epidermóide de língua, considerado com sendo uma condição não-tratável. O pactiente faleceu por asfixia poucos meses depois aos 16 anos. As razões para a ocorrência dessa condição maligna ainda são incertas, podendo estar relacionadas com a instabilidade cromossômica característica da AF, com fatores relacionados ao TMO ou com a DEVH. O acompanhamento sistemático desses pacientes permite a realização de abordagens terapêuticas precoces e menos agressivas quando necessário.

Fanconi anemia (FA) is an autosomal recessive disorder that might cause a variety of congenital and developmental abnormalities. The most important features of FA are progressive bone marrow failure and development of malignancies, particularly acute myeloid leukemia and solid tumors. This paper reports the case of a 12-year-old patient with FA assisted at the Stomatology and Bucomaxillofacial Cancer Prevention Service of São Lucas Hospital, Brazil, who had been submitted to bone marrow transplantation (BMT) at the age of 5 and exhibited oral lesions characteristic of chronic graft versus host disease (GVHD). The patient was treated and followed-up for the oral lesions. Eleven years after the BMT, he developed squamous cell carcinoma of the tongue with an aggressive behavior, which was considered an untreatable condition. The patient died few months later from asphyxia at the age of 16. The reasons for development of these malignant conditions are unknown. However, chromosomal instability typically observed in FA cases, BMT factors and GVHD have been considered. Systematic follow-up of these patients allows early and less invasive therapeutic approaches.