Abstract The aim of this study was to evaluate the impact of oral conditions and health-related quality of life (HRQoL) on oral health-related quality of life (OHRQoL) in children and adolescents with blood coagulation disorders and hemoglobinopathies (BCDH). The study was cross-sectional and included 61 individuals aged 2 to 18 years with BCDH. Exams for dental caries (dmft/DMFT index), oral hygiene (simplified oral hygiene index – OHI-S), and gingival health (modified gingival index – MGI) were performed. The pediatric quality of life inventory™ (PedsQL™) generic core scale and oral health scale were used to measure HRQoL and OHRQoL. Spearman’s correlation coefficient (ρ) and the Mann-Whitney test (α = 0.05) were conducted to assess the relationship between covariates and the PedsQL™ oral health scale. The mean PedsQL™ oral health scale score was 76.66 (SD = 21.36). Worse OHRQoL was correlated with poor oral hygiene (ρ = -0.383; p: 0.004), poor gingival health (ρ = -0.327; p = 0.014), and better HRQoL (ρ = 0.488; p < 0.001). Greater untreated dental caries experience was associated with worse OHRQoL (p = 0.009). Worse oral health status in children and adolescents with BCDH negatively impacts OHRQoL, and OHRQoL and quality of life analyzed from a generic perspective are positively correlated constructs in this population. healthrelated related (HRQoL (OHRQoL . (BCDH) crosssectional cross sectional 6 1 dmft/DMFT dmftDMFT dmft DMFT index, , index) simplified OHIS, OHIS OHI S OHI-S) modified MGI performed inventory PedsQL (PedsQL™ Spearmans Spearman s ρ MannWhitney Mann Whitney α 0.05 005 0 05 7666 76 66 76.6 SD 21.36. 2136 21.36 21 36 21.36) 0.383 0383 383 -0.383 0.004, 0004 0.004 004 0.004) 0.327 0327 327 -0.327 0.014, 0014 0.014 014 0.014) 0.488 0488 488 0.001. 0001 0.001 001 0.001) 0.009. 0009 0.009 009 0.009) population (BCDH OHI-S (PedsQL 0.0 00 766 7 76. 213 21.3 3 0.38 038 38 -0.38 000 0.00 0.32 032 32 -0.32 0.01 01 0.48 048 48 0. 21. 0.3 03 -0.3 0.4 04 4 -0. -0 -

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. disease IL17 IL 17 IL-1 diseases cancer rejection ILA A ILRA RA saliva additive, additive (additive dominant recessive. . recessive) rs (rs224104 C allele, allele , rs87957 rs960661 T rs224104 group rs3819025 (rs381902 rs227591 G respectively 2 0.026 0026 0 026 0.65 065 65 0.043 0043 043 0.88. 088 0.88 88 0.88) disorder. disorder disorder) rs91786 0.036 0036 036 0.67. 067 0.67 67 0.67) rs1760661 0.042 0042 042 0.47. 047 0.47 47 0.47) knowledge far IL1 1 IL- (rs22410 rs8795 rs96066 rs22410 rs381902 (rs38190 rs22759 0.02 002 02 0.6 06 6 0.04 004 04 08 0.8 8 rs9178 0.03 003 03 rs176066 0.4 4 (rs2241 rs879 rs9606 rs2241 rs38190 (rs3819 rs2275 0.0 00 0. rs917 rs17606 (rs224 rs87 rs960 rs224 rs3819 (rs381 rs227 rs91 rs1760 (rs22 rs8 rs96 rs22 rs381 (rs38 rs9 rs176 (rs2 rs2 rs38 (rs3 rs17 (rs rs3 rs1

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: 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 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.

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.