10-year experience of early arthritis clinic at a tertiary rheumatology center: achievements and challenges.

OBJECTIVES: To characterize patients evaluated in our Early Arthritis Clinic (EAC) in the first ten years; to assess diagnostic delay and its underlying causes; and to evaluate the level of agreement between the referring physician and the rheumatologist regarding the presence of referral criteria. METHODS: Cross-sectional study including patients attending EAC between 2012 and 2021. Demographic data, provenience, final diagnosis, referral criteria and time related to diagnosis delay were retrieved from clinical files and the Portuguese Registry of Rheumatic Patients (reuma.pt). Characteristics of the patients and the time variables were analysed with descriptive statistical analysis. The agreement between the referring physician and rheumatologist regarding the referral criteria was evaluated using Cohen's Kappa. RESULTS: A total of 440 patients (68.9% females, mean age of 54±16.7 years) were referred, mostly from primary care (71.6%). Inflammatory Rheumatic Disease was diagnosed in 65.7% of the patients, with 58.9% classified as early arthritis. The median time from onset of symptoms to referral for EAC was 76 days (IQR 33.5-144.0); the median time from referral to the first EAC was 34 (IQR 19.0-46.0) days, and the median time from onset of symptoms to first EAC was 114.5 (IQR 66.8-190.3) days (16.3 weeks). Only about 10% were observed by a Rheumatologist before six weeks after symptom onset. The level of agreement between the referring physician and the rheumatologist was slight to fair to clinical criteria and moderate to substantial to laboratory criteria. CONCLUSIONS: A significant delay still is observed in patients with early arthritis suspicion, being the time from onset of symptoms to referral is the most relevant. A low agreement between referral and Rheumatologists suggests that non-rheumatologists education/training is needed. Identifying the barriers that prevent the adequate referral of patients is necessary to define strategies to improve it.

Comportamento Auditivo e Validação no Diagnóstico Audiológico e Intervenção em Bebês e Crianças com Deficiência Auditiva / Auditory Behavior and Validation in Audiological Diagnosis and Intervention in Infants and Children with Hearing Impairment / Comportamiento auditivo y validación en el diagnóstico e intervención audiológicos en bebés y niños con deficiencia auditiva

Introdução: A atuação profissional com bebês e crianças pequenas com deficiência auditiva exige conhecimento e técnica específica no que diz respeito à prescrição e adaptação do aparelho de amplificação sonora individual (AASI) e ao processo de desenvolvimento de linguagem. Limitações e imprecisões ao longo do processo diagnóstico poderão comprometer todos os procedimentos subsequentes do processo de intervenção. Objetivo: Analisar a validação do processo de diagnóstico audiológico e intervenção em bebês e crianças com deficiência auditiva a partir da análise comparativa de exames audiológicos, comportamento auditivo e aplicação do princípio de verificação cruzada após adaptação de AASI. Método: Foram sujeitos da pesquisa 12 crianças de até 36 meses de idade, com diagnóstico de perda auditiva neurossensorial bilateral, selecionados a partir da disponibilidade de acesso ao serviço para a avaliação e agrupados em G1 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB até 35%) e G2 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB acima de 54%). Resultados: A média de idade do diagnóstico audiológico foi de 4,33 meses. Os resultados audiológicos de todas as crianças tiveram correspondência entre si, com exceção de dois sujeitos do G2. Conclusão: O comportamento auditivo não só permitiu a validação dos processos de diagnóstico e intervenção auditiva dos sujeitos da pesquisa, como também permitiu a identificação de comportamentos não compatíveis com a audibilidade devido ao uso inconsistente dos AASI. A aplicação dos instrumentos de acompanhamento de desenvolvimento mostrou-se adequada para o monitoramento do desenvolvimento de habilidades de audição e linguagem em crianças pequenas. (AU)

Introduction: Professional work with infants and young children with hearing impairment requires specific knowledge and technique regarding the prescription and adaptation of the individual sound amplification device (PSAD) and the language development process. Limitations and inaccuracies throughout the diagnostic process may compromise all subsequent procedures of the intervention process. Purpose: To analyze the validation of the process of audiological diagnosis and intervention in infants and children with hearing impairment based on the comparative analysis of audiological tests, auditory behavior and application of the cross-checking principle after adaptation of hearing aids. Method: The research subjects were 12 children aged up to 36 months, with a diagnosis of bilateral sensorineural hearing loss, selected from the availability of access to the service for the evaluation and grouped into G1 (subjects with Speech Intelligibility Index - SII 65 dB up to 35%) and G2 (subjects with Speech Intelligibility Index - SII 65 dB above 54%). Results: The average age of the audiological diagnosis was 4.33 months. The audiological results of all children corresponded to each other, except for two subjects from G2. Conclusion: The auditory behavior not only allowed the validation of the processes of diagnosis and auditory intervention of the research subjects, but also allowed the identification of behaviors that are not compatible with audibility due to the inconsistent use of hearing aids. The application of developmental monitoring instruments proved to be adequate for monitoring the development of hearing and language skills in young children. (AU)

Introducción: El trabajo profesional con lactantes y niños pequeños con discapacidad auditiva requiere conocimientos y técnica específicos respecto a la prescripción y adaptación del dispositivo individual de amplificación del sonido (PSAD) y el proceso de desarrollo del lenguaje. Las limitaciones e imprecisiones a lo largo del proceso de diagnóstico pueden comprometer todos los procedimientos posteriores del proceso de intervención. Propósito: Analizar la validación del proceso de diagnóstico e intervención audiológica en lactantes y niños con discapacidad auditiva a partir del análisis comparativo de pruebas audiológicas, conducta auditiva y aplicación del principio de cruce tras adaptación de audífonos. Método: Los sujetos de investigación fueron 12 niños de hasta 36 meses, con diagnóstico de hipoacusia neurosensorial bilateral, seleccionados de la disponibilidad de acceso al servicio para la evaluación y agrupados en G1 (sujetos con Índice de inteligibilidade del Habla - SII 65 dB hasta 35%) y G2 (sujetos com Índice de inteligibilidade del Habla - SII 65 dB por encima del 54%). Resultados:La edad promedio del diagnóstico audiológico fue de 4,33 meses. Los resultados audiológicos de todos los niños se correspondieron entre sí, a excepción de dos sujetos del G2. Conclusión: La conducta auditiva no sólo permitió validar los procesos de diagnóstico e intervención auditiva de los sujetos de investigación, sino que también permitió identificar conductas no compatibles con la audibilidad debido al uso inconsistente de audífonos. La aplicación de instrumentos de seguimiento del desarrollo demostró ser adecuada para controlar el desarrollo de las habilidades auditivas y lingüísticas en niños pequeños. (AU)

Phage therapy in gut microbiome.

Phage therapy, the use of bacteriophage viruses for bacterial infection treatment, has been around for almost a century, but with the increase in antibiotic use, its importance has declined rapidly. There has been renewed interest in revisiting this practice due to the general decline in the effectiveness of antibiotics, combined with improved understanding of human microbiota and advances in sequencing technologies. Phage therapy has been proposed as a clinical alternative to restore the gut microbiota in the absence of an effective treatment. That is due to its immunomodulatory and bactericidal effects against its target bacteria. In the gastrointestinal diseases field, phage therapy has been studied mainly as a promising tool in infectious diseases treatment, such as cholera and diarrhea. However, many studies have been conducted in non-communicable diseases, such as the targeting of adherent invasive Escherichia coli in Crohn's disease, the treatment of Clostridioides difficile in ulcerative colitis, the eradication of Fusobacterium nucleatum in colorectal cancer, the targeting of alcohol-producing Klebsiella pneumoniae in non-alcoholic fatty liver disease, or Enterococcus faecalis in alcohol-associated hepatitis. This review will summarize the changes in the gut microbiota and the phageome in association with some gastrointestinal and liver diseases and highlight the recent scientific advances in phage therapy as a therapeutic tool for their treatment.

Immune Response of an Oral Enterococcus faecalis Phage Cocktail in a Mouse Model of Ethanol-Induced Liver Disease.

Cytolysin-positive Enterococcus faecalis (E. faecalis) cause more severe alcohol-associated hepatitis, and phages might be used to specifically target these bacteria in a clinical trial. Using a humanized mouse model of ethanol-induced liver disease, the effect of cytolytic E. faecalis phage treatment on the intestinal and liver immune response was evaluated. The observed immune response was predominantly anti-inflammatory and tissue-restoring. Besides, live phages could be readily recovered from the serum, spleen, and liver following oral gavage in ethanol-fed mice. We also isolated 20 new phages from the sewage water; six of them exhibited a relatively broad host range. Taken together, the oral administration of cytolytic E. faecalis phages leads to the translocation of phages to the systemic circulation and appears to be safe, following chronic-binge ethanol administration. A cocktail of three phages covers the majority of tested cytolysin-positive E. faecalis strains and could be tested in a clinical trial.

Adherence to Remote Microphone System use at school in children and adolescents with hearing loss. / Adesão ao uso do Sistema de Microfone Remoto em estudantes com deficiência auditiva usuários de dispositivos auditivos.

PURPOSE: To identify relationships between Remote Microphone System (RMS) use in the classroom and the schools' and teachers' characteristics. METHODS: We analyzed 120 subjects aged 5 to 17 years with hearing loss who had received an RMS from a health service accredited by the Unified Health System (SUS). The teachers of RMS users were the other subjects in the study. We analyzed the patients' medical records and interviewed their parents/guardians at the follow-up visit to verify issues related to the RMS and its use at school. We contacted the schools over the phone and visited some of them. RESULTS: Of the students, 54% used the device at school; 22% involuntarily did not use it; and 24% voluntarily did not use it. The Speech Intelligibility Index pattern of those who used the RMS was similar to those who involuntarily did not use it. There was a significant difference between the type of school and educational level - 86% of regular school students and elementary school students tend to use the device more often (62%). CONCLUSION: Most subjects use the RMS at school. The students' educational level also interfered with the adherence to RMS use, as elementary school students had a higher adherence. The data suggest that the coordination between health services and schools favors RMS use. However, when the parents mediate this relationship, other factors interfere with the systematic RMS use in the school routine.

OBJETIVO: Identificar relações entre a utilização sistemática do Microfone Remoto (MR) em sala de aula de estudantes com deficiência auditiva e características das escolas e dos professores. MÉTODO: Foram analisados 120 sujeitos, entre cinco e 17 anos, com deficiência auditiva que foram adaptados MR em um Serviço de Saúde credenciada pelo Sistema Único de Saúde (SUS). Também foram sujeitos, professores de usuários de MR. Realizou-se uma análise de prontuários e no momento que os sujeitos compareceram para acompanhamento foi realizado entrevista com os pais/responsáveis para caracterizar rotina de utilização do MR na escola. Foram realizados contatos telefônicos e visitas presenciais em algumas escolas. RESULTADOS: Quanto ao uso, observa-se que a maioria dos sujeitos utilizava o dispositivo na escola. Aqueles que não utilizavam involuntariamente e voluntariamente consistiu na minoria dos sujeitos. Houve uma similaridade no padrão do Speech Intelligibility Index -SII dos sujeitos que 'usam' e 'não usam involuntariamente' o MR. Houve diferença significativa entre o tipo de escola e o nível de escolaridade, a maioria dos sujeitos que frequentavam escola regular e estavam matriculados no ensino fundamental I tendem a usar mais o dispositivo. CONCLUSÃO: A maioria dos sujeitos faz uso do MR na escola. O nível educacional do estudante também foi um fator que interferiu na adesão ao uso dos MR, com maior adesão em estudantes do Ensino Fundamental I. Os dados sugerem que a articulação entre serviço de saúde e escola favorece a utilização do MR, entretanto quando essa relação é intermediada pelos pais, outros fatores acabam interferindo no uso sistemático no cotidiano da escola.

Frequency and origin of the c.2090T>G p.(Leu697Trp) MYO3A variant associated with autosomal dominant hearing loss.

We recently described a novel missense variant [c.2090T>G:p.(Leu697Trp)] in the MYO3A gene, found in two Brazilian families with late-onset autosomal dominant nonsyndromic hearing loss (ADNSHL). Since then, with the objective of evaluating its contribution to ADNSHL in Brazil, the variant was screened in additional 101 pedigrees with probable ADNSHL without conclusive molecular diagnosis. The variant was found in three additional families, explaining 3/101 (~3%) of cases with ADNSHL in our Brazilian pedigree collection. In order to identify the origin of the variant, 21 individuals from the five families were genotyped with a high-density SNP array (~600 K SNPs- Axiom Human Origins; ThermoFisher). The identity by descent (IBD) approach revealed that many pairs of individuals from the different families have a kinship coefficient equivalent to that of second cousins, and all share a minimum haplotype of ~607 kb which includes the c.2090T>G variant suggesting it probably arose in a common ancestor. We inferred that the mutation occurred in a chromosomal segment of European ancestry and the time since the most common ancestor was estimated in 1100 years (CI = 775-1425). This variant was also reported in a Dutch family, which shares a 87,121 bp haplotype with the Brazilian samples, suggesting that Dutch colonists may have brought it to Northeastern Brazil in the 17th century. Therefore, the present study opens new avenues to investigate this variant not only in Brazilians but also in European families with ADNSHL.

Adesão ao uso do Sistema de Microfone Remoto em estudantes com deficiência auditiva usuários de dispositivos auditivos / Adherence to Remote Microphone System use at school in children and adolescents with hearing loss

RESUMO Objetivo Identificar relações entre a utilização sistemática do Microfone Remoto (MR) em sala de aula de estudantes com deficiência auditiva e características das escolas e dos professores. Método Foram analisados 120 sujeitos, entre cinco e 17 anos, com deficiência auditiva que foram adaptados MR em um Serviço de Saúde credenciada pelo Sistema Único de Saúde (SUS). Também foram sujeitos, professores de usuários de MR. Realizou-se uma análise de prontuários e no momento que os sujeitos compareceram para acompanhamento foi realizado entrevista com os pais/responsáveis para caracterizar rotina de utilização do MR na escola. Foram realizados contatos telefônicos e visitas presenciais em algumas escolas. Resultados Quanto ao uso, observa-se que a maioria dos sujeitos utilizava o dispositivo na escola. Aqueles que não utilizavam involuntariamente e voluntariamente consistiu na minoria dos sujeitos. Houve uma similaridade no padrão do Speech Intelligibility Index -SII dos sujeitos que 'usam' e 'não usam involuntariamente' o MR. Houve diferença significativa entre o tipo de escola e o nível de escolaridade, a maioria dos sujeitos que frequentavam escola regular e estavam matriculados no ensino fundamental I tendem a usar mais o dispositivo. Conclusão A maioria dos sujeitos faz uso do MR na escola. O nível educacional do estudante também foi um fator que interferiu na adesão ao uso dos MR, com maior adesão em estudantes do Ensino Fundamental I. Os dados sugerem que a articulação entre serviço de saúde e escola favorece a utilização do MR, entretanto quando essa relação é intermediada pelos pais, outros fatores acabam interferindo no uso sistemático no cotidiano da escola.

ABSTRACT Purpose To identify relationships between Remote Microphone System (RMS) use in the classroom and the schools' and teachers' characteristics. Methods We analyzed 120 subjects aged 5 to 17 years with hearing loss who had received an RMS from a health service accredited by the Unified Health System (SUS). The teachers of RMS users were the other subjects in the study. We analyzed the patients' medical records and interviewed their parents/guardians at the follow-up visit to verify issues related to the RMS and its use at school. We contacted the schools over the phone and visited some of them. Results Of the students, 54% used the device at school; 22% involuntarily did not use it; and 24% voluntarily did not use it. The Speech Intelligibility Index pattern of those who used the RMS was similar to those who involuntarily did not use it. There was a significant difference between the type of school and educational level - 86% of regular school students and elementary school students tend to use the device more often (62%). Conclusion Most subjects use the RMS at school. The students' educational level also interfered with the adherence to RMS use, as elementary school students had a higher adherence. The data suggest that the coordination between health services and schools favors RMS use. However, when the parents mediate this relationship, other factors interfere with the systematic RMS use in the school routine.

CRIg on liver macrophages clears pathobionts and protects against alcoholic liver disease.

Complement receptor of immunoglobulin superfamily (CRIg) is expressed on liver macrophages and directly binds complement component C3b or Gram-positive bacteria to mediate phagocytosis. CRIg plays important roles in several immune-mediated diseases, but it is not clear how its pathogen recognition and phagocytic functions maintain homeostasis and prevent disease. We previously associated cytolysin-positive Enterococcus faecalis with severity of alcohol-related liver disease. Here, we demonstrate that CRIg is reduced in liver tissues from patients with alcohol-related liver disease. CRIg-deficient mice developed more severe ethanol-induced liver disease than wild-type mice; disease severity was reduced with loss of toll-like receptor 2. CRIg-deficient mice were less efficient than wild-type mice at clearing Gram-positive bacteria such as Enterococcus faecalis that had translocated from gut to liver. Administration of the soluble extracellular domain CRIg-Ig protein protected mice from ethanol-induced steatohepatitis. Our findings indicate that ethanol impairs hepatic clearance of translocated pathobionts, via decreased hepatic CRIg, which facilitates progression of liver disease.

Bacterial Nosocomial Infections: Multidrug Resistance as a Trigger for the Development of Novel Antimicrobials.

Nosocomial bacterial infections are associated with high morbidity and mortality, posing a huge burden to healthcare systems worldwide. The ongoing COVID-19 pandemic, with the raised hospitalization of patients and the increased use of antimicrobial agents, boosted the emergence of difficult-to-treat multidrug-resistant (MDR) bacteria in hospital settings. Therefore, current available antibiotic treatments often have limited or no efficacy against nosocomial bacterial infections, and novel therapeutic approaches need to be considered. In this review, we analyze current antibacterial alternatives under investigation, focusing on metal-based complexes, antimicrobial peptides, and antisense antimicrobial therapeutics. The association of new compounds with older, commercially available antibiotics and the repurposing of existing drugs are also revised in this work.

Sodium fluoride in cardiovascular disorders: A systematic review.

BACKGROUND: 18-Fluorine sodium fluoride is a well-known radiotracer used for bone metastasis diagnosis. Its uptake correlation with cardiovascular (CV) risk was primarily suggested in oncological patients. Moreover, as a specific marker of microcalcification, it seems to correlate with CV disease progression and plaque instability. METHODS AND RESULTS: Our purpose was to systematically review clinical studies that characterized the use of this marker in CV conditions. In atherosclerosis, most studies report a positive correlation with the burden of CV risk factors and vascular calcification. A higher uptake was found in culprit plaques/rupture sites in coronary and carotid arteries and it was also linked to high-risk features in histology and intravascular imaging analysis of the plaques. In aortic stenosis, this tracer displayed an increasing uptake with disease severity. CONCLUSIONS: Sodium fluoride positron emission tomography is a promising non-invasive technique to identify high-risk plaques, which sets ground to a potential use of this tracer in evaluating atherosclerotic disease progression and degenerative changes in aortic valve stenosis. Nevertheless, there is a need for further prospective evidence that demonstrates this technique's value in predicting clinical events, adjusting treatment strategies, and improving patient outcomes.

Antioxidant and anti-inflammatory action (in vivo and in vitro) from the trunk barks of Cabreúva (Myrocarpus frondosus Allemao, Fabaceae).

ETHNOPHARMACOLOGICAL RELEVANCE: Myrocarpus frondosus, known as cabreúva, is a tree whose trunk barks are used in folk medicine as tea, syrup, ointments, and tinctures for the treatment of inflammation. However, there is no scientific evidence demonstrating this activity. AIM OF THE STUDY: The present investigation was focused on evaluating the antioxidant and anti-inflammatory activities of M. frondosus, using the in vitro model of RAW 264.7 macrophages induced by LPS and the in vivo model of mouse pleurisy induced by carrageenan. MATERIALS AND METHODS: M. frondosus trunk barks were dried at room temperature for seven days and subjected to exhaustive maceration with ethanol (70%) to obtain its crude extract (CE). CE was subjected to UPLC-HRMS analysis to establish its chemical profile. Its antioxidant activity was evaluated using the DPPH method, reducing power by the iron (III) to iron (II) reduction assay and the ß-carotene-linoleic acid bleaching assay. The RAW 264.7 macrophages were pretreated with the CE in a non-cytotoxic concentration and induced by LPS (1 µg/mL). After 24 h, using the supernatant, we evaluated the nitric oxide (NOx) and interleukin-6 (IL-6) levels. The anti-inflammatory effects of CE (at doses of 30, 100 and 300 mg/kg) were evaluated on leukocyte migration (total and differential), exudate concentrations, myeloperoxidase (MPO) and adenosine-deaminase (ADA) activities, NOx, tumor necrosis factor-α (TNF-α), and IL-6 levels, by using a murine model of neutrophilic inflammation. RESULTS: The UPLC-HRMS of CE revealed the presence of isoflavonones, including biochanin A and formononetin. CE exhibited good antioxidant activity by quenching and decreasing free radicals, as well as reducing pro-oxidant metals. CE did not show cytotoxicity at a concentration below 11 µg/mL and reduced the secretion of the pro-inflammatory NOx in the inflamed macrophages. In vivo assay revealed that CE caused a pronounced inhibition on leukocyte migration, and this inhibition was due to its ability to reduce neutrophil migration. Moreover, CE was also able to reduce the release of critical pro-inflammatory mediators such as MPO, NOx, TNF-α, and IL-6. CONCLUSIONS: All these findings indicate that M. frondosus exhibited antioxidant activity and anti-inflammatory effect.

Uso do sistema auxiliar de escuta com estudantes com deficiência auditiva: identificação de barreiras e facilitadores / Use of hearing assistance technology with students with hearing loss: identification of barriers and facilitators / Uso del sistema auxiliar de escucha com alunos com hipoacusia: identificación de barreras y facilitadores

Introdução: A relação sinal-ruído na sala de aula pode ser um vilão na inclusão na escola regular de alunos com deficiência auditiva, usuários de dispositivos eletrônicos, que utilizam a língua oral para se comunicar. Os recursos tecnológicos são determinantes para a melhor audibilidade de sons de fala em ambientes ruidosos e sua efetividade depende da adesão ao uso do dispositivo. Esse processo é determinado pela parceria entre profissionais da saúde, família e  escola. Objetivo: Identificar a relação entre a utilização consistente do sistema de microfone remoto (SMR) em estudantes com deficiência auditiva e o uso pelos professores, que favoreceram ou dificultaram sua adaptação e o desenvolvimento escolar desses estudantes. Método: Foram analisados 175 sujeitos entre 5 e 17 anos que receberam o SMR num serviço de saúde auditiva entre os anos de 2017 e 2018. Pais e professores de usuários também foram sujeitos do estudo. O funcionamento do SMR e a classificação quanto ao seu uso foram verificados. Resultado: Os indivíduos que mais 'usam' o SMR estão no ensino fundamental I, e os que 'não usam voluntariamente' estão no ensino médio e fundamental II. Considerando-se o tipo de escola, a maioria que 'não usa voluntariamente' o SMR está em escola ou sala para surdos com uso de libras e/ou tem intérprete na sala da escola regular. Conclusão: Houve associação entre uso do SMR e tipo de escola. Recomenda-se que o tipo de escola seja um critério de indicação do dispositivo. O nível educacional também foi uma variável determinante no uso do dispositivo na escola.

Introduction: The signal-to-noise ratio in the classroom can be a villain in the inclusion of hearing-impaired students in regular school, users of electronic devices, that use the oral language to communicate. The technological resources are determinant for better audibility of speech sounds in noisy environments and their effectiveness depends on adherence to the use of the device. This process is determined by the partnership between health professionals, family and school. Objective: Identify the relation between the use of remote microphone system (RMS) in hearing impaired students and the use by teachers, which favored or hindered their adaptation and the school development of hearing-impaired students. Method:175 subjects between 5 and 17 years of age who received the HAT in a hearing health service between the years 2017 and 2018 were analyzed. Parents and teachers of users were also subjects of the study. The functioning of the HAT and the classification regarding its use was verified. Result: Most of the individuals who 'use' HAT are in elementary school, and those who 'don't use it voluntarily' are in high school and middle school. Considering the type of school, most who 'don't use voluntarily' the HAT is in school or room for deaf students using sign language /or has interpreter in regular school. Conclusion:There was an association between the use of the HAT and the type of school. It is recommended that the type of school is a criterion for the indication of the device. The educational level was also a determinant variable in the use of the device at school.

Introducción: La relación señal-ruido en el aula puede ser un villano en la inclusión de estudiantes con discapacidad auditiva en la escuela normal, usuarios de dispositivos electrónicos que utilizan el lenguaje oral para comunicarse. Los recursos tecnológicos son cruciales para una mejor audibilidad de los sonidos del habla en ambientes ruidosos y su eficacia depende de la adherencia al uso del dispositivo. Este proceso está determinado por la asociación entre los profesionales de salud, familia y escuela. Objetivo: Identificar la relación entre el uso del sistema de micrófono remoto(SMR) en los alumnos con deficiencias auditivas y el uso por parte de los maestros, que han favorecido o dificultado su adaptación y el desarrollo escolar de los alumnos con deficiencias auditivas. Método: Se analizaron 175 sujetos de entre 5 y 17 años que recibieron el SMR en un servicio de salud auditiva entre los años 2017 y 2018. Los padres y maestros de los usuarios también fueron sujetos del estudio. Se ha comprobado el funcionamiento del SMR y la clasificación relativa a su uso. Resultado: Los individuos que "usan" más el SMR están en la escuela primaria, y los que "no usan voluntariamente" están en la secundaria. Considerando el tipo de escuela, la mayoría de los que "no usan voluntariamente" el SMR está en una escuela o sala para sordos con uso de libras y/o tiene un intérprete en la sala de la escuela regular. Conclusión: Hubo una asociación entre el uso del SMR y el tipo de la escuela. Se recomienda que el tipo de escuela sea un criterio para indicar el dispositivo. El nivel educativo, también fue una variable determinante en el uso del dispositivo en la escuela.

From intestinal dysbiosis to alcohol-associated liver disease.

Alcohol-associated intestinal dysbiosis and bacterial overgrowth can lead to a dysregulation of tryptophan metabolism and lower production of indoles. Several of these indole derivatives are aryl hydrocarbon receptor ligands that, in turn, are involved in antimicrobial defense via induction of interleukin-22 (IL-22). IL-22 increases the expression of intestinal regenerating islet-derived 3 (Reg3) lectins, which maintain low bacterial colonization of the inner mucus layer and reduce bacterial translocation to the liver. Chronic alcohol consumption is associated with reduced intestinal expression of Reg3ß and Reg3γ, increased numbers of mucosa-associated bacteria and bacterial translocation. Translocated microbial products and viable bacteria reach the liver and activate the innate immune system. Release of inflammatory molecules promotes inflammation, contributes to hepatocyte death and results in a fibrotic response. This review summarizes the mechanisms by which chronic alcohol intake changes the gut microbiota and contributes to alcohol-associated liver disease by changing microbial-derived metabolites.

Força muscular respiratória em crianças com deficiência auditiva e a sua relação com categorias de audição e linguagem / Respiratory muscular strength in children with hearing impairment and their relationship with hearing and language categories / Fuerza muscular respiratoria en niños con discapacidad auditiva y su relación com las categorías de audiencia y lengua

Introdução: crianças com deficiência auditiva possuem uma dificuldade maior no controle de voz, respiração e articulação, que se caracteriza por uma menor produção de sílabas por ciclo respiratório e maior esforço fonatório. Objetivo: analisar os dados da força muscular respiratória e a sua relação com as habilidades de audição e linguagem em crianças com deficiência auditiva. Métodos: participaram do estudo 50 crianças com deficiência auditiva, de ambos os sexos, com idades entre 7 e 12 anos usuárias de aparelho de amplificação sonora individual. As pressões respiratórias máximas foram mensuradas por meio do equipamento manovacuômetro e analisadas pelos valores dos preditos para PImáx e PEmáx, a audição e linguagem das crianças foram classificadas de acordo as categorias de audição e linguagem, a partir da aplicação dos instrumentos: GASP; ABFW-vocabulário; Word Association for Syllable Perception; Limiar de Reconhecimento de Sentenças. Resultados: constatou-se que crianças com deficiência auditiva apresentam fraqueza muscular respiratória em relação a crianças ouvintes, de acordo com valores preditos, independentemente do tipo de perda auditiva. Grande parte das crianças tem perda auditiva classificada pela melhor orelha como grau moderado (42%); entretanto, essas mesmas crianças apresentaram resultados com pequena diferença em porcentagem entre os resultados de pressão inspiratória máxima (n=11, 26%) acima do predito e 10 crianças (24%) abaixo do predito. As crianças avaliadas que possuem comunicação oral estão a 2% de diferença nos resultados entre PImáx e PEmáx. Conclusão: Pode-se afirmar que as crianças com deficiência auditiva apresentam fraqueza muscular respiratória independentemente do grau de perda auditiva, tipo de comunicação e classificação nas categorias de audição e de linguagem.

Introduction: hearing impaired children have greater difficulty in controlling voice, breathing and articulation; this is characterized by lower syllable production per respiratory cycle and greater phonatory effort. Objective: to analyze respiratory muscle strength data and its relationship with hearing and language skills in children with hearing impairment. Methods: fifty hearing impaired children were availed, from both genders, with ages between 7 and 12 years, hearing aid users. Maximum respiratory pressures were measured using the manuvacuometer equipment and analyzed by the predicted values for PImáx and PEmáx, hearing and language categories, sentences classified according to hearing and language categories, sentences perception in open set, from the application of the instruments: GASP; ABFW vocabulary; Word Association for Syllable Perception (WASP); Sentences Perception Threshold. Results: most of the children have hearing loss classified by the best ear as moderate (42%); however, the same subjects showed results with small difference between the PImax results (n= 11 subjects ­ 26%) above predicted and 10 subjects (24%) bellow. The availed children with oral communication present 2% of difference between PImax and PEmax results. Conclusion: It can be said that children with hearing impairment have respiratory muscle weakness regardless of the degree of hearing loss, type of communication and classification in the hearing and language categories.

Introducción: los niños con discapacidad auditiva tienen mayor dificultad para controlar la voz, la respiración y la articulación, que se caracteriza por una menor producción de sílabas por ciclo respiratorio y un mayor esfuerzo fonatorio Objetivo: analizar los datos de fuerza muscular respiratoria y su relacióncon las habilidades de audición y lenguaje en niños con discapacidad auditiva. Métodos: 50 niños con pérdida auditiva, de ambos sexos, con edades comprendidas entre 7 y 12 años, con un audífono individual, participaron en el estudio. Las presiones respiratorias máximas se midieron usando el equipo de manovacuómetro y se analizaron mediante los valores predichos para MIP y MEP, la audición y el lenguaje de los niños se clasificaron según las categorías de audición y lenguaje; de la aplicación de los instrumentos: GASP; Vocabulario ABFW; Asociación de palabras para la percepción silábica; Umbral de reconocimiento de oraciones. Resultados: se encontró que los niños con discapacidad auditiva tienen debilidad muscular respiratoria en relación con los niños con audición, independientemente del tipo de pérdida auditiva, de acuerdo con los valores pronosticados. La mayoría de los niños tienen pérdida auditiva clasificada por el mejor oído como moderada (42%); sin embargo, estos mismos niños mostraron resultados con una pequeña diferencia porcentual entre los resultados de la presión inspiratoria máxima (n = 11, 26%) por encima de lo previsto y 10 niños (24%) por debajo de lo previsto. Los niños evaluados que tienen comunicación oral tienen una diferencia del 2% en los resultados de MIP y MEP. Conclusión: Se puede decir que los niños con discapacidad auditiva tienen debilidad muscular respiratoria, independientemente del grado de pérdida auditiva, tipo de comunicación y clasificación en las categorías de audición y lenguaje.

Relationship between hearing capacity and performance on tasks of speech perception in children with hearing loss. / Relações entre medidas de capacidade auditiva e desempenho em tarefas de percepção da fala em crianças com deficiência auditiva.

PURPOSE: To establish the relationship between the performance on word recognition tasks, using words with and without sense and degree, and the configuration of hearing loss, by using Speech Inteligibility Index (SII) values as indicators, in children with hearing loss. METHODS: SII were established for 55 and 65 Decibel of Sound Pressure Level (dB SPL) input sounds of ten children presenting bilateral sensorineural hearing loss (SNHL), adapted with bilateral hearing aids, and who have oral language as the main mode of communication. The children were submitted to a word and nonsense-word repetition task of two or three intensity degrees. Their productions were analyzed according to the Word Association for Syllable Perception (WASP) Protocol. In the data analysis, the values of SII were compared with the results obtained in each analysis criterion. RESULTS: Pertaining to the words, there was statistically significant difference between the two types of stimuli in 55 dBSPL. As for the performance of consonants and point of articulation, there was a statistically significant difference between stimuli types in 65 and 55 dB SPL, and between intensities 65 and 55 dB SPL in nonsense words. CONCLUSION: Overall, there was no regularity in the relationship between hearing ability and performance in speech perception tasks. The results suggest that performance in the nonsense words recognition tasks was more related to intelligibility index than to words with meaning, possibly because it limits semantic closure strategies by the subject.

OBJETIVO: Estabelecer relações entre o desempenho em tarefa de reconhecimento de palavras com e sem sentido e grau e configuração da perda auditiva, utilizando valores de Índices de Inteligibilidade de Fala (SII) como indicadores, em crianças com deficiência auditiva. MÉTODO: Foram estabelecidos os SII para sons de entradas de 55 e 65 Decibéis Nível de Pressão Sonora (dBNPS) de dez crianças com perda auditiva neurossensorial usuárias de aparelho de amplificação sonora individual bilateralmente que têm a linguagem oral como principal modalidade de comunicação. As crianças foram submetidas à tarefa de repetição de palavras com e sem sentido em duas ou três diferentes intensidades. As emissões foram analisadas de acordo com o Protocolo Word Association for Syllable Perception (WASP). Na análise dos dados, o SII foi comparado com os resultados obtidos em cada critério de análise. RESULTADOS: Para o desempenho em palavras, houve diferença estatisticamente significante entre os dois tipos de estímulo em 55dBNPS. Para o desempenho em consoantes e ponto de articulação, houve diferença estatisticamente significante entre os tipos de estímulos em 65 e 55dBNPS e também entre as intensidades de 65 e 55 dBNPS nas palavras sem sentido. CONCLUSÃO: De modo geral, não houve regularidade na relação entre capacidade auditiva e desempenho em tarefas de percepção da fala. Os resultados sugerem que o desempenho nas tarefas de reconhecimento de palavras sem sentido tem maior relação com o índice de inteligibilidade do que as palavras com sentido, possivelmente por limitar as estratégias de fechamento semântico pelo sujeito.

Relações entre medidas de capacidade auditiva e desempenho em tarefas de percepção da fala em crianças com deficiência auditiva / Relationship between hearing capacity and performance on tasks of speech perception in children with hearing loss

RESUMO Objetivo Estabelecer relações entre o desempenho em tarefa de reconhecimento de palavras com e sem sentido e grau e configuração da perda auditiva, utilizando valores de Índices de Inteligibilidade de Fala (SII) como indicadores, em crianças com deficiência auditiva. Método Foram estabelecidos os SII para sons de entradas de 55 e 65 Decibéis Nível de Pressão Sonora (dBNPS) de dez crianças com perda auditiva neurossensorial usuárias de aparelho de amplificação sonora individual bilateralmente que têm a linguagem oral como principal modalidade de comunicação. As crianças foram submetidas à tarefa de repetição de palavras com e sem sentido em duas ou três diferentes intensidades. As emissões foram analisadas de acordo com o Protocolo Word Association for Syllable Perception (WASP). Na análise dos dados, o SII foi comparado com os resultados obtidos em cada critério de análise. Resultados Para o desempenho em palavras, houve diferença estatisticamente significante entre os dois tipos de estímulo em 55dBNPS. Para o desempenho em consoantes e ponto de articulação, houve diferença estatisticamente significante entre os tipos de estímulos em 65 e 55dBNPS e também entre as intensidades de 65 e 55 dBNPS nas palavras sem sentido. Conclusão De modo geral, não houve regularidade na relação entre capacidade auditiva e desempenho em tarefas de percepção da fala. Os resultados sugerem que o desempenho nas tarefas de reconhecimento de palavras sem sentido tem maior relação com o índice de inteligibilidade do que as palavras com sentido, possivelmente por limitar as estratégias de fechamento semântico pelo sujeito.

ABSTRACT Purpose To establish the relationship between the performance on word recognition tasks, using words with and without sense and degree, and the configuration of hearing loss, by using Speech Inteligibility Index (SII) values as indicators, in children with hearing loss. Methods SII were established for 55 and 65 Decibel of Sound Pressure Level (dB SPL) input sounds of ten children presenting bilateral sensorineural hearing loss (SNHL), adapted with bilateral hearing aids, and who have oral language as the main mode of communication. The children were submitted to a word and nonsense-word repetition task of two or three intensity degrees. Their productions were analyzed according to the Word Association for Syllable Perception (WASP) Protocol. In the data analysis, the values of SII were compared with the results obtained in each analysis criterion. Results Pertaining to the words, there was statistically significant difference between the two types of stimuli in 55 dBSPL. As for the performance of consonants and point of articulation, there was a statistically significant difference between stimuli types in 65 and 55 dB SPL, and between intensities 65 and 55 dB SPL in nonsense words. Conclusion Overall, there was no regularity in the relationship between hearing ability and performance in speech perception tasks. The results suggest that performance in the nonsense words recognition tasks was more related to intelligibility index than to words with meaning, possibly because it limits semantic closure strategies by the subject.

Further evidence for loss-of-function mutations in the CEACAM16 gene causing nonsyndromic autosomal recessive hearing loss in humans.

Mutations in the CEACAM6 gene were first described as causing autosomal dominant nonsyndromic hearing loss, but two splice-altering variants have been recently described as causing autosomal recessive nonsyndromic hearing loss. We describe the novel and extremely rare loss-of-function variant c.436 C > T/p.(Arg146Ter) in the CEACAM16 gene segregating with post-lingual progressive autosomal recessive hearing loss. This variant is predicted to significantly reduce the size of the wild type protein. Our results give additional support that loss-of-function variants in CEACAM16 cause autosomal recessive hearing loss in humans.