Clinical Characteristics and Gene Mutations in 186 Cases of Kindler Syndrome / 中国医学科学院学报

Objective To investigate the clinical characteristics and genetic mutations in Kindler syndrome(KS)and provide a theoretical basis for the diagnosis and treatment of KS. Methods The clinical data of one case of KS from Peking Union Medical College Hospital and 185 cases reported in literature were collected. The gene mutation types,patient clinical data,and tumor characteristics were statistically analyzed. Results A total of 186 cases were enrolled,including 110 males and 76 females,with the mean age of(28±16)years. The data of gene mutation and specific clinical manifestations were available in 151 and 94 patients,respectively. The main clinical manifestations of KS included poikiloderma,occurrence of blister in childhood,and photosensitivity,and the secondary clinical manifestations included oral inflammation,palmoplantar keratoderma,webbing/pseudoainhum,dysphagia,urethral stricture and so on.Oral inflammation(r=0.234,P=0.023),palmoplantar keratoderma(r=0.325,P=0.001),webbing/pseudoainhum(r=0.247,P=0.016),dysphagia(r=0.333,P=0.001),urethral stricture(r=0.280,P=0.006)were significantly correlated with age,showing significantly higher incidence in the patients over 32 years old.Urethral stricture(χ2=11.292,P=0.001)and anal stenosis(χ2=4.014,P=0.045)were significantly correlated with sex,with higher incidence in males.Eighty different mutations were found in 151 patients,and the most common gene mutation was c.676C>T.Forty-one tumors occurred in 27 patients,among which squamous cell carcinoma accounted for 92.7%. The gene mutation site had no significant correlation with squamous cell carcinoma or patient country. Conclusions The c.676C>T in FERMT1 gene is the most common mutation in KS.The patients are prone to squamous cell carcinoma and mainly attacked at the exposure sites(hand and mouth).

Esofagitis eosinofílica y acalasia esofágica / Eosinophilic esophagitis and esophageal acalasia

Introducción: La esofagitis eosinofílica es una enfermedad emergente, caracterizada por infiltración del esófago por leucocitos eosinófilos. Sus principales síntomas son la disfagia y las frecuentes impactaciones de alimento en el esófago. Actualmente existen evidencias científicas que reconocen la enfermedad como causa posible de evolución no favorable en pacientes después de miotomía de Héller. Objetivo: Describir las evidencias biomoleculares que asocian la esofagitis eosinofílica y la acalasia esofágica. Métodos: Se realizó una revisión sistemática y crítica de las evidencias sobre los mecanismos biomoleculares asociados a la esofagitis eosinofílica y la acalasia esofágica. Se consultaron artículos publicados entre 2015 y 2020 e indexados en las bases de datos PubMed, SciELO, LILACS y Scopus. Análisis e integración de la información: Se discute sobre cuestiones medulares que han sido publicadas recientemente respecto al tema en cuestión. ¿Pueden coexistir la esofagitis eosinofílica y la acalasia esofágica? ¿Influye la esofagitis eosinofílica en el resultado del tratamiento de la acalasia esofágica? ¿Qué investigaciones serían necesarias para establecer la relación entre las dos enfermedades? Conclusiones: Los mecanismos celulares y biomoleculares desencadenados por la infiltración eosinofílica contextualizan la diferencia etiológica y fisiopatológica de la esofagitis eosinofílica y la acalasia esofágica, lo cual sustenta la evolución desfavorable posmiotomía de los pacientes y motiva la realización de estudios prospectivos y controlados con el fin de ofrecer una mejor calidad de vida(AU)

Introduction: Eosinophilic esophagitis is an emerging disease characterized by infiltration of the esophagus by eosinophilic leukocytes. Its main symptoms are dysphagia and frequent food impaction in the esophagus. Scientific evidence is now available that recognizes the disease as the possible cause of unfavorable evolution in patients undergoing Heller myotomy. Objective: Describe the biomolecular evidence associating eosinophilic esophagitis to esophageal achalasia. Methods: A systematic critical review was conducted of the evidence about biomolecular mechanisms associated to eosinophilic esophagitis and esophageal achalasia. The articles consulted were published in the databases PubMed, SciELO, LILACS and Scopus from 2015 to 2020. Data analysis and integration: A discussion is provided about crucial questions published recently concerning the study topic: Can eosinophilic esophagitis and esophageal achalasia coexist? Does eosinophilic esophagitis influence the result of esophageal achalasia treatment? What studies are required to establish the relationship between the two conditions? Conclusions: The cellular and biomolecular mechanisms triggered by eosonophilic infiltration contextualize the etiological and pathophysiological difference between eosinophilic esophagitis and esophageal achalasia. This explains the unfavorable post-myotomy evolution of patients and encourages the conduct of prospective controlled studies aimed at enhancing quality of life(AU)

Acalasia y su relación con el cáncer esofágico / Esophageal acalasia and its relationship with esophageal cancer

Resumen La acalasia es un trastorno motor primario de la musculatura lisa esofágica que se caracteriza por disfagia, pseudorregurgitación y baja de peso. El tratamiento puede ser endoscópico o quirúrgico. Sólo se conocen los resultados a largo plazo de la cirugía, mientras que los endoscópicos tienen aún un seguimiento muy corto y no permiten sacar conclusiones valederas. La acalasia es una lesión que tiene una probabilidad significativamente mayor de desarrollar un cáncer esofágico, ya sea de tipo epidermoide, por inflamación crónica y retención de comida en el esófago, o un adenocarcinoma, secundario a reflujo gastroesofágico, que aparece posterior a cualquier tratamiento. Las publicaciones muestran que alrededor de 3% a 4% de los pacientes presentan a largo plazo, sobre 10 a 15 años postratamiento, el desarrollo de un cáncer avanzado del esófago. Se concluye que es indispensable un seguimiento clínico y endoscópico en forma rutinaria a estos pacientes.

Achalasia is a primary motor disorder of the esophageal smooth muscle characterized by dysphagia, pseudoregurgitation, and weight loss. Treatment can be endoscopic or surgical. The long-term results are only known from surgery, while endoscopic results still have a very short follow-up and do not allow us to draw valid conclusions. Achalasia is a lesión that has a significantly higher probability of developing esophageal cancer, whether of the epidermoid type, due to chronic inflammation and food retention in the esophagus, or an adenocarcinoma, secondary to gastroesophageal reflux, which appears after any treatment. Publications show that about 3 to 4% of patients present in time, about 10 to 15 years after treatment, the development of advanced cancer of the esophagus. It is concluded that clinical and endoscopic follow-up is essential in these patients on a routine basis.

Rol del fonoaudiólogo en el tratamiento de la disfagia en usuarios con alta clínica por Covid-19: una revisión de literatura / Role of the speech therapist in the treatment of dysphagia in users with clinical discharge for COVID-19: a literature review

La enfermedad conocida como Covid-19fue declarada pandemia a principios del año 2020, atacando de manera sustancial el aparato respiratorio de los contagiados. La insuficiencia respiratoria progresiva generada por la enfermedad genera incluso, la muerte en los estados más graves de la enfermedad. Debido a esta alteración algunos pacientes pueden requerir ventilación mecánica. Muchas investigaciones relacionan la intubación con las disfagias. El fonoaudiólogo es quien ayuda en la rehabilitación de las alteraciones deglutorias, por lo que el objetivo de esta investigación fue revisar literatura especializada para recabar información sobre el papel del fonoaudiólogo en el tratamiento de la disfagia en pacientes con alta clínica por Covid-19.La metodología usada, definió una búsqueda específica en las bases de datos PubMed, Sciencedirect y en la Biblioteca Virtual de Salud (BSV). Se utilizó el término clave MeSH "Covid-19" luego el booleano de intersección "AND" y el término clave MeSH "Dysphagia" en seguida el booleano de intersección "OR" y el termino clave MeSH "Swallowing Disorder". Se encontraron un total de 42 artículos en distribuidos de la siguiente manera: PubMed (14), ScienceDirect (7), BSV (21). En la presente investigación solo fueron incluidos artículos correspondientes a estudios experimentales, cuasi experiméntales, estudios de casos, revisiones bibliográficas y ensayos clínicos donde se observa el papel del fonoaudiólogo en el trabajo de la disfagia en pacientes con alta clínica después de haber tenido Covid-19.Los resultados obtenidos relacionan la intubación prolongada a la disfagia, aunque los artículos hallados no son suficientes para realizar una generalización, sumado a que la calidad de los artículos encontrados no tienen el nivel de evidencia esperada, a pesar de eso, los resultados encontrados confirman al fonoaudiólogos especialistas en motricidad orofacial como un actor relevante tanto en la evaluación como en la intervención de pacientes que poseen disfagia debido a la intubación prolongada por Covid-19.

The disease known as Covid-19 was declared a pandemic at the beginning of 2020, substantially attacking the respiratory system of those infected. The progressive respiratory failure generated by the disease even leads to death in severe stages of the disease. Due to this alteration some users may require mechanical ventilation. Much research links intubation to dysphagia. The Speech Therapist is the one who helps in the rehabilitation of swallowing disorders, the objective of this research was to review specialized literature to gather information on the Role of the Speech Pathologist in the Treatment of Dysphagia in High Clinic Users due to (Covid-19).A specific search was carried out in the databases PubMed, Science direct and in the Virtual Health Library (BSV). The key term Mesh "Covid-19" was used then the intersection boolean "AND" and the key term Mesh "Dysphagia" then the intersection boolean "OR" and the key term Mesh "Swallowing Disorder". A total of 42 articles in distributed as follows: PubMed (14), ScienceDirect (7), BSV (21). In the present investigation only articles corresponding to experimental studies, quasi-experimental studies, case studies, bibliographic reviews were included, clinical trials, where the role of the speech therapist in the work of dysphagia in patients with clinical discharge after having had Covid-19 is observed.The results obtained relate prolonged intubation to dysphagia, although the articles found are not enough to make a generalization, added to the fact that the quality of the articles found does not have the expected level of evidence, despite that, the results found confirm the speech pathologist specializing in orofacial motor skills as a relevant actor both in the evaluation and in the intervention of users who have dysphagia due to prolonged intubation by Covid-19.

Physicians' behavior regarding non-acceptance of oral restriction (nil per os) by dysphagic patient with risk of laryngotracheal aspiration / Comportamento do médico diante da não aceitação de restrição da via oral pelo paciente disfágico com risco de aspiração laringotraqueal

ABSTRACT Objective: To define physician´s behavior in the face of a mentally capable elderly dysphagic patients at risk of pulmonary aspiration, who do not accept oral restriction. Methods: Observational, cross-sectional study, presenting a clinical case of an independent elderly with clinical complaints of dysphagia and laryngotracheal aspiration by flexible endoscopic evaluation of swallowing who rejected the proposal to restrict oral diet. A questionnaire about the patient's decision-making process was used to assess whether the physician was sympathetic and justify their answer, and if they are aware of hierarchy of ethical principles (recognition of the person´s value, autonomy, beneficence, nonmaleficence and justice), in the decision-making process, and which was the main principle that guided their decision. Results: One hundred participants were classified by time since graduation as Group I (less than 10 years) and Group II (more than 10 years). Of them, 60% agreed with the patient's decision, with no difference between the groups. The main reason was autonomy of patients, in both groups. Among those who were not sympathetic, the main argument was beneficence and nonmaleficence, considering the risk between benefit and harm. As to awareness about the hierarchy of principles, we did not find differences between the groups. Autonomy was the principle that guided those who were sympathetic with the patient's decision, and justice among those who didnot agree. Conclusion: Physicians were sympathetic with the patient's decision regarding autonomy, despite the balance between risks of beneficence and nonmaleficence, including death. We propose to formalize a non-compliance term.

RESUMO Objetivo: Definir o comportamento médico diante de paciente idoso disfágico com risco de aspiração laringotraqueal e mentalmente capaz que não aceita a restrição da via oral. Métodos: Estudo observacional, transversal. Apresentamos um caso clínico de idoso, que vive independente, com queixas clínicas e videoendoscopia da deglutição comprovando disfagia e aspiração, que recusou a proposta de restrição da via oral. Um questionário foi aplicado sobre o processo de decisão do paciente, procurando avaliar se o médico torna-se solidário, e que justifique sua resposta, e se tem ciência da hierarquia dos princípios éticos (reconhecimento do valor da pessoa, autonomia, beneficência, não maleficência e justiça), no processo de decisão e qual o principal princípio que norteia sua decisão. Resultados: Cem participantes foram classificados por tempo de formados em Grupo I (até 10 anos) e Grupo II (mais de 10 anos). Deles 60% tornaram-se solidários à decisão do paciente, sem diferença entre os grupos. O principal argumento foi a autonomia do paciente nos dois grupos. Entre os não solidários, foi o binômio beneficência e não maleficência, e o balanço do risco/benefício e malefício. Considerando a ciência sobre a hierarquia dos princípios que regem a decisão, não encontramos diferença entre eles. A autonomia foi o principal princípio na decisão entre os solidários e a justiça entre os não solidários. Conclusão: O médico foi solidário à decisão do paciente em respeito à sua autonomia, apesar dos riscos ponderados da beneficência e da maleficência, inclusive de morte. Propomos o termo de recusa de conduta formalizada.

Swallowing in Patients with Mental Disability - Analysis of 189 Swallowing Video Endoscopies

Abstract Introduction Feeding, swallowing and breathing are fundamental activities for the survival and well-being of humans; these functions are performed by themost complex neuromuscular unit of the human body, which, when altered, may raise morbidity and mortality rates. Objective To evaluate deglutition in patients with mental disability in order to determine the incidence and the severity of dysphagia. Methods A total of 189 institutionalized adult patients with mental disability were analyzed using a 3.2 mm flexible fiberscope (Machida, Japan 1995). The following food consistencies were tested: pasty, thickened liquid and liquid. Results Among the total of 189 patients, 101 (53.4%) were female aged between 14 and 55 years old. Most of them 120 (63.5%) had profound mental deficiency, 58 (30.7%) had severe mental deficiency, 9 (4.8%) had moderate mental deficiency, and 2 (1.1%) had mild mental deficiency. Gender and the degree of mental deficiency did not influence significantly the degree of dysphagia. Age, degree of disability and interaction between age groups and degrees of disability influenced significantly the degree of dysphagia. Younger patients are more likely to present more severe dysphagia. Stabilization occurs between 31 and 40 years of age, and above this age, a greater chance of less severe dysphagia, because the increase in the degree of mental deficiency decreases the probability of more severe dysphagia. Conclusion The population that mostly presented severe dysphagia was characterized by being mostly female, with profound mental deficiency, with an average age of 36.7 years. There was no relationship between gender and the degree of mental disability concerning the degree of dysphagia. (AU)

Desidratação, acidente vascular cerebral e disfagia: revisão sistemática da literatura / Dehydration, stroke and dysphagia: systematic review

RESUMO Objetivos trata-se de revisão sistemática da literatura científica sobre a associação entre o acidente vascular cerebral, desidratação e disfagia orofaríngea. Estratégia de pesquisa o levantamento bibliográfico foi realizado nas bases de dados científicos: MEDLINE, LILACS, SciELO, Web of Science e Cochrane. Critérios de seleção foram incluídos os estudos que preencheram os seguintes critérios de inclusão: ser artigo original, com resumo disponível; ter sido publicado entre os anos de 2001 e 2018 e nos idiomas português, inglês ou espanhol; abordar o tema desidratação em indivíduos após acidente vascular cerebral. A triagem e análise dos estudos foram realizadas por dois avaliadores independentes. Resultados dentre os 484 artigos localizados para a triagem, 43 foram selecionados para leitura completa e 18 foram incluídos na análise final. Foram descritos diferentes métodos de avaliação do estado de hidratação nos indivíduos após acidente vascular cerebral, tais como: análise da relação BUN /creatinina, osmolaridade plasmática, relação ureia/creatinina, gravidade específica da urina, coloração da urina, ingestão hídrica, balanço hídrico, bioimpedância elétrica, avaliação clínica, análise de eletrólitos isolados. A prevalência de desidratação em pacientes após acidente vascular cerebral, durante a internação, variou de 11% a 66% e está associada à gravidade e piora na evolução clínica. Conclusão foi possível compreender a complexidade do processo de mensuração do estado de hidratação em indivíduos após acidente vascular cerebral e sua associação com a disfagia. Estudos enfocando essa temática são de extrema relevância, visto a sua influência sobre a taxa de mortalidade e morbidade nesta população.

ABSTRACT Purpose this is a systematic review of scientific literature associated with stroke, dehydration and oropharyngeal dysphagia (OD). Research Strategy bibliographic survey was conducted in scientific databases: MEDLINE, LILACS, SciELO, Web of Science and Cochrane. Selection criteria studies that met the following inclusion criteria were included: being original article with summary available; have been published in the last seventeen years (2001-2018) and in Portuguese, English or Spanish languages; and addressing dehydration in individuals after stroke Screening and analysis of the studies were performed by two independent evaluators. Results among the 484 articles found for screening, 43 were selected for full reading and 18 articles were included in the final analysis. Different methods of assessing hydration status have been described in individuals post-stroke, such as ratio analysis blood urea nitrogen (BUN)/creatinine, plasma osmolality, urea / creatinine, urine specific gravity, urine color, water intake, water balance, bioelectrical impedance analysis (BIA), clinical evaluation and analysis of electrolytes. The prevalence of dehydration in post-stroke during hospitalization varied from 11% to 66% and is associated with severity and deterioration in the clinical evolution. Conclusion It was possible to understand the complexity of the measurement of hydration status in individuals after stroke and its association with dysphagia. Studies focusing on the association between dehydration and stroke are very important, due to its influence on mortality and morbidity in this population.

O ensino de situações: problema de clientes hospitalizados com disfagia orofaríngea e seus nexos com o cuidado em enfermagem e fonoaudiologia em um curso de residência / The teaching of situations: problem of hospitalized clients with oropharyngeal dysphagia and its nexuses with care in nursing and speech therapy in a residency course

Os estudos com base nas situações-problema de clientes com disfagia orofaríngea necessitam ser ampliados, por se acreditar que ainda existem dificuldades dos enfermeiros em identificar os distúrbios relacionados às alterações de deglutição. Assim, a integração dos conhecimentos da fonoaudiologia e da enfermagem pode resultar no desenvolvimento de diretrizes, protocolos de apoio e material de orientação aos profissionais de enfermagem e, portanto, prover medidas básicas de qualidade a estes clientes

Profile of patients using nasoenteric probe at the adult emergency service in a public hospital

Introduction: Part of the prognosis of hospitalized patient depends on nutritional status and the safety and efficacy of the feeding administration route. Therefore, the aim of this study was to identify data on the indication of nasoenteric tube (NET) prescription to analyze the profile of these patients. Methods: A retrospective cross-sectional study was carried out with data collection in medical records of patients over 18 years of age, of both sexes, treated at the Emergency Adult Service (EAS), using NET. Results: there was a predominance of females (51.9%); neurological disease was the most prevalent underlying disease, and 57.8% had more than one diagnosed disease. Malnutrition, bronchopneumonia, and dysphagia were present in 23.6%, 27% and 40% of the cases, respectively. There was a request for speech-language evaluation in only 8.7% of the patients. And 80.7% did not use NET prior to emergency care. Regarding the indications for the use of NET by group of underlying disease, low food intake was the most prevalent clinical reason described in the medical records, followed by dysphagia, with a higher prevalence of patients taking NET for nutritional reasons. There was no justification for prescription in 15.2% of the sample. There was an association between the variables bronchopneumonia and dysphagia (p = 0.01). Conclusion: It was verified that in the studied population there are risk factors for dysphagia. The analysis of the population profile in the present study contributed to increased knowledge and information about this population regarding the criteria for indication of the use of the alternative route. (AU)

Study on the Correlation between Barium Radiography and Pulmonary Infection rate in the Evaluation of Swallowing Function

OBJECTIVES: To compare the results respectively obtained from the utilization of 60% barium sulfate suspension and Iohexol as contrast agents for videofluoroscopic swallowing studies and the relationship between the clinical application of the two kinds of contrast agents and the incidence of pneumonia. METHODS: Sixty cases of stroke patients with dysphagia were selected in rehabilitation department of our hospital, and the gender, age, position of the disease, and stroke nature between groups had no significant difference. Among which, 30 patients who were administered 350 mgI/ml Iohexol, and the other 30 patients with 60% barium sulfate suspension as contrast agent. We performed videofluoroscopic swallowing studies with barium 60% versus Iohexol within 1 week after admission and 2 weeks after admission. RESULTS: After 2 weeks in hospital, the aspiration pneumonia incidence of two groups was statistically significant (p<0.05), the pneumonia incidence of Iohexol group was lower than barium sulfate group which might have a impossble relevance with barium aspiration. CONCLUSIONS: During the videofluoroscopic swallowing study of dysphagia after stroke, barium sulfate can enhance the pneumonia incidence, and Iohexol can be widely applied in videofluoroscopic swallowing study.

Chest CT findings in patients with dysphagia and aspiration: a systematic review / Achados de TC de tórax em pacientes com disfagia e aspiração pulmonar: uma revisão sistemática

ABSTRACT The objective of this systematic review was to characterize chest CT findings in patients with dysphagia and pulmonary aspiration, identifying the characteristics and the methods used. The studies were selected from among those indexed in the Brazilian Virtual Library of Health, LILACS, Indice Bibliográfico Español de Ciencias de la Salud, Medline, Cochrane Library, SciELO, and PubMed databases. The search was carried out between June and July of 2016. Five articles were included and reviewed, all of them carried out in the last five years, published in English, and coming from different countries. The sample size in the selected studies ranged from 43 to 56 patients, with a predominance of adult and elderly subjects. The tomographic findings in patients with dysphagia-related aspiration were varied, including bronchiectasis, bronchial wall thickening, pulmonary nodules, consolidations, pleural effusion, ground-glass attenuation, atelectasis, septal thickening, fibrosis, and air trapping. Evidence suggests that chest CT findings in patients with aspiration are diverse. In this review, it was not possible to establish a consensus that could characterize a pattern of pulmonary aspiration in patients with dysphagia, further studies of the topic being needed.

RESUMO O objetivo deste estudo foi realizar uma revisão sistemática dos achados de TC de tórax que caracterizem aspiração pulmonar em pacientes com disfagia, identificando as características e os métodos utilizados. Para a seleção dos estudos, foram utilizadas as bases de dados da Biblioteca Virtual em Saúde, LILACS, Indice Bibliográfico Español de Ciencias de la Salud, Medline, Biblioteca Cochrane, SciELO e PubMed. A busca foi realizada no período entre junho e julho de 2016. Foram incluídos e revisados cinco artigos, todos realizados nos últimos cinco anos, publicados em língua inglesa e oriundos de diferentes países. O tamanho da amostra nos estudos selecionados variou de 43 a 56 pacientes, com predominância de sujeitos adultos e idosos. Os achados tomográficos em pacientes com aspiração relacionada à disfagia foram variados, abrangendo bronquiectasias, espessamento da parede brônquica, nódulos pulmonares, consolidações, derrame pleural, atenuação em vidro fosco, atelectasias, espessamento septal, fibrose, aprisionamento aéreo, entre outros. As evidências sugerem que os achados de TC de tórax em pacientes que apresentam aspiração são diversificados. Nesta revisão, não foi possível estabelecer um consenso que pudesse caracterizar um padrão de aspiração pulmonar nos pacientes com disfagia, sendo importantes investigações futuras sobre o assunto.

Modificação da voz após deglutição: compatibilidade com achados da videofluoroscopia / Postswallow voice modification: compatibility with videofluoroscopy findings

RESUMO Objetivo Verificar se a modificação da voz após a deglutição relaciona-se com os dados do exame de videofluoroscopia. Método 27 indivíduos com disfagia orofaríngea realizaram a gravação da vogal sustentada /a/ antes e após a deglutição durante exame de videofluoroscopia. Utilizou-se a escala GRBAS e acrescentou-se o aspecto voz molhada para avaliação dos dados. Em relação ao exame, verificou-se estase de alimento em valéculas e recessos piriformes, penetração laríngea, aspiração traqueal e grau de disfagia. Resultados Houve diminuição do grau de alteração e astenia e aumento da tensão fonatória após a deglutição, sem diferença para o parâmetro voz molhada. Obteve-se sensibilidade e especificidade de ±50% para estase em recessos piriformes e valéculas; porém, sensibilidade de 80% para detecção de penetração e de 66-75% para aspiração e modificação da tensão fonatória, com 77-91% de valores preditivos negativos para os três parâmetros de avaliação, sem correlação com o grau de disfagia. Conclusão A modificação dos parâmetros da escala GRBAS após a deglutição apresentou boa compatibilidade com achados da videofluoroscopia.

ABSTRACT Purpose Verify whether voice modification after swallowing is associated with videofluoroscopic examination data. Methods 27 patients with oropharyngeal dysphagia underwent recording of sustained phonation of vowel /a/ before and after swallowing during videofluoroscopy. The GRBAS scale and the wet voice parameter were used to evaluate the data. Videofluoroscopy results showed stasis of food in the valleculae and piriform recesses, laryngeal penetration, tracheal aspiration, and degree of dysphagia. Results Decreased dysphonia grade and asthenia and increased strain were observed after swallowing, with no difference for the wet voice parameter. Sensitivity and specificity of ± 50% were observed for food stasis in the valleculae and piriform recesses. Sensitivity values of 80 and 66-75% were observed for detection of laryngeal penetration and tracheal aspiration and modification of vocal strain, respectively. Negative predictive values of 77-91% were found for the three assessment parameters with no correlation with the degree of oropharyngeal dysphagia. Conclusion Modification of the GRBAS scale parameters after swallowing showed good compatibility with videofluoroscopy findings.

Trastornos de la alimentación y deglución en niños con parálisis cerebral: condiciones clínicas acompañantes / Feeding and swallowing disorders in children with cerebral palsy: co-occurring clinical conditions

Introduction: Cerebral Palsy (CP) is frequently accompanied by other cooccurring clinical conditions (CCC). Feeding and swallowing disorders (FSD) are a common problem among CP population and tend to be more complex when associated to certain CCC. Objective: To quantify the frequency of CCC in a group of children with CP and FSD, of both genders, GMFCS I to V, between the age of 3 to 6 years. Patients and Methods: 49 patients diagnosed with CP and FSD underwent 5 assessments: 1) physiatric evaluation; 2) Gross Motor Functional Classification System (GMFCS); 3) Eating and Drinking Ability Classification System (EDACS); 4) videofluoroscopic swallowing study; and 5) sensory processing test.Results: All 49 patients had at least one of the CCC usually present in CP, with an average of 3,8 (from 1 to 6). All GFMCS and EDACS levels were affected, including mild ones. Some of the CCC showed high frequencies: intellectual disability (ID) (83,7 percent), sensory processing disorder (SPD) (75,5 percent), sialorrhea (71,4 percent), malnutrition (67,4 percent), altered alertness (67,4 percent) and constipation (61,2 percent).There was a high frequency of association between CCC. In more than 50 percent of the cases, the association of ID with most of the other conditions was the commonest.Conclusions: Children diagnosed with CP and FSD present with multiple CCC able to worsen their prognosis. All GMFCS and EDACS levels are affected. ID is the most frequent CCC, being also the most associated with the others.

Introducción: La Parálisis Cerebral (PC) cursa junto a numerosas condiciones clínicas acompañantes (CCA), destacando los trastornos de la alimentación y deglución (TAD). En PC numerosas CCA pueden interactuar con los TAD y complicarlos. Objetivo: Cuantificar la frecuencia de determinadas CCA en niños con PC, GMFCS I a V, de 3 a 6 años, portadores de TAD. Pacientes y Métodos: 49 pacientes con PC y TAD fueron sometidos a 5 evaluaciones: 1) valoración fisiátrica; 2) Gross Motor Functional Classification System (GMFCS); 3) Eating and Drinking Ability Classification System (EDACS); 4) videofluoroscopía (VFC); y 5) pauta de integración sensorial. Resultados: Todos los pacientes con PC y TAD presentaron alguna de las CCA consideradas, con un promedio de 3,8 por niño (rango 1-6), afectando a todos los niveles GMFCS y EDACS, incluidos los leves. Algunas tuvieron elevada frecuencia: déficit cognitivo (DC) (83,7 por ciento), trastorno de la integración sensorial (TIS) (75,5 por ciento), sialorrea (71,4 por ciento), desnutrición (67,4 por ciento), alerta alterada (67,4 por ciento) y constipación (61,2 por ciento). Hubo una alta frecuencia de asociación de CCA, siendo más común la asociación de DC con varias de las otras condiciones, en más del 50 por ciento de los casos. Conclusiones: Los niños con PC y TAD cursan con CCA múltiples que complican su cuadro clínico. Todos los niveles GMFCS y EDACS se ven afectados. El DC es la condición más frecuente y muestra más asociación con las otras.

Aberrant right subclavian artery-esophageal fistula: massive upper gastrointestinal hemorrhage secondary to prolonged intubation / Fístula de artéria subclávia direita anômala com esôfago - hemorragia digestiva alta maciça secundária a intubação gástrica prolongada

ABSTRACT Aberrant right subclavian artery-esophageal fistula is a rare but potentially fatal complication. It may be associated with procedures, such as tracheostomy and tracheal or esophageal intubation, and yields massive upper gastrointestinal bleeding difficult to identify and to control. A high index of suspicion is essential for early diagnosis and better prognosis. We report a rare case of a patient who survived after emergent surgical procedure for massive upper gastrointestinal bleeding secondary to aberrant right subclavian artery-esophageal fistula after prolonged intubation.

RESUMO A fístula de artéria subclávia direita anômala com o esôfago é uma complicação rara, mas potencialmente fatal. Pode estar associada a procedimentos como traqueostomia e intubação traqueal ou esofágica e originar hemorragia digestiva alta maciça, de difícil identificação e controle. Um elevado índice de suspeição é essencial para o diagnóstico precoce e a melhoria do prognóstico. Relatamos caso raro de doente que sobreviveu após intervenção cirúrgica emergente por hemorragia digestiva alta maciça secundária a fístula de artéria subclávia direita anômala com esôfago, após intubação gástrica prolongada.

Correlation between the severity of critically ill patients and clinical predictors of bronchial aspiration / Correlação entre a gravidade de pacientes críticos e preditores clínicos de risco para a broncoaspiração

Objective: To determine whether the severity of non-neurological critically ill patients correlates with clinical predictors of bronchial aspiration. Methods: We evaluated adults undergoing prolonged orotracheal intubation (> 48 h) and bedside swallowing assessment within the first 48 h after extubation. We collected data regarding the risk of bronchial aspiration performed by a speech-language pathologist, whereas data regarding the functional level of swallowing were collected with the American Speech-Language-Hearing Association National Outcome Measurement System (ASHA NOMS) scale and those regarding health status were collected with the Sequential Organ Failure Assessment (SOFA). Results: The study sample comprised 150 patients. For statistical analyses, the patients were grouped by ASHA NOMS score: ASHA1 (levels 1 and 2), ASHA2 (levels 3 to 5); and ASHA3 (levels 6 and 7). In comparison with the other patients, those in the ASHA3 group were significantly younger, remained intubated for fewer days, and less severe overall clinical health status (SOFA score). The clinical predictors of bronchial aspiration that best characterized the groups were abnormal cervical auscultation findings and cough after swallowing. None of the patients in the ASHA 3 group presented with either of those signs. Conclusions: Critically ill patients 55 years of age or older who undergo prolonged orotracheal intubation (≥ 6 days), have a SOFA score ≥ 5, have a Glasgow Coma Scale score ≤ 14, and present with abnormal cervical auscultation findings or cough after swallowing should be prioritized for a full speech pathology assessment.

Objetivo: Correlacionar a gravidade de pacientes críticos não neurológicos com preditores clínicos do risco de broncoaspiração. Métodos: Participaram do estudo adultos com histórico de intubação orotraqueal prolongada (> 48 h) e submetidos à avaliação da deglutição à beira do leito nas primeiras 48 h após a extubação. Dados relacionados a avaliação fonoaudiológica clínica do risco de aspiração broncopulmonar, nível funcional da deglutição por meio da escala American Speech-Language-Hearing Association National Outcome Measurement System (ASHA NOMS) e status de saúde pelo Sequential Organ Failure Assessment (SOFA) foram coletados. Resultados: A amostra do estudo foi composta por 150 pacientes. Para fins da análise estatística, os pacientes foram agrupados com base nos escores ASHA NOMS: ASHA1 (níveis 1-2), ASHA2 (níveis 3-5) e ASHA3 (níveis 6-7). Os indivíduos no grupo ASHA3 eram significativamente mais jovens, permaneceram intubados por menos tempo e apresentaram menor gravidade de quadro clínico geral (escore SOFA) do que os indivíduos nos demais grupos. Os preditores clínicos de broncoaspiração que melhor caracterizaram os grupos foram achados de ausculta cervical alterada e presença de tosse após a deglutição. O grupo ASHA3 não apresentou esses sinais. Conclusões: Pacientes críticos com idade ≥ 55 anos, período de intubação ≥ 6 dias, gravidade de quadro clínico geral (escore SOFA ≥ 5), escore na Escala de Coma de Glasgow ≤ 14, ausculta cervical alterada e tosse após a deglutição devem ser priorizados para a avaliação fonoaudiológica completa.

Broncoaspiración en el niño: rol diagnóstico de los estudios de imágenes / Aspiration in children. Role of diagnostic imaging studies

Aspiration is defined as the entrance of foreign material through the respiratory tract to the lungs. This syndrome is relatively frequent in children and often under diagnosed. It can occur at any age and may cause acute or chronic lung disease. Clinical symptoms depend on the amount and frequency of events, individual response, the kind of aspirated material and the underlying cause. There are different diseases that can cause aspiration. Swallowing disorders, often associated with neuromuscular diseases are a common causal factor. Imaging studies can play an important role in the diagnostic approach and may identify aspiration cause. Among these studies, the most important is videofluoroscopy which allows a dynamic and real-time study of swallowing. Chest radiography and computed tomography can support the diagnosis.

La broncoaspiración corresponde a la entrada de material extraño a la vía aérea y pulmones. Es un síndrome relativamente frecuente y muchas veces subdiagnosticado. Puede presentarse a cualquier edad y ser factor causal de enfermedad pulmonar aguda o crónica. El cuadro clínico dependerá del volumen y frecuencia de los eventos, la respuesta individual, el material aspirado y el factor causal. Las causas de broncoaspiración son variadas. Las alteraciones en los mecanismos de la deglución, muchas veces asociadas a enfermedades neuromusculares son un factor causal frecuente. Los estudios de imágenes juegan un rol importante en la aproximación diagnóstica y pueden orientar a la etiología. Dentro de estos el de mayor relevancia es la videofluoroscopía, que permite un estudio dinámico y en tiempo real de la deglución. La cintigrafía también puede ser de utilidad diagnóstica, al igual que la radiografía de tórax y la tomografía computada.

Vocal Variability Post Swallowing in Individuals with and without Oropharyngeal Dysphagia

Introduction Voice modification after swallowing may indicate changes in the transit of the bolus. Objective The aim of this study is to verify the use of perceptual voice analysis to detect oropharyngeal dysphagia. Study Design Case series. Methods Twenty-seven patients with dysphagia as diagnosed by videofluoroscopy and 25 without were evaluated. The sustained vowel /a/ was recorded before this exam and after swallowing different consistencies (pasty, liquid and solid). For the voice evaluation, the GRBAS scale (grade, roughness, breathiness, asthenia and strain) and the parameter "wet voice" were used. Three judges blinded to study group and time of emission performed voice analysis. Results Individuals with dysphagia showed significant decrease in grade of voice and asthenia and increase in strain after swallowing pasty substances, differing from individuals without dysphagia who showed no modification of the parameters after swallowing. The wet voice parameter showed no difference after swallowing in both study groups. Conclusion The decrease in grade and asthenia and increased strain are indicative of a swallowing disorder, indicating increased vocal strain to clean the vocal tract of food. The modification of vocal production after swallowing proved to be a trusted resource for detection of swallowing disorders. .

Fatores de risco relacionados à aspiração, pneumonia e função pulmonar, e qualidade de vida em acientes disfágicos com câncer de cabeça e pescoço / Risk factors related to aspiration, pneumonia and pulmonary functional and quality of life in dysphagic patients with head and neck cancer

Introdução: Identificar fatores que contribuem para a aspiração e o desenvolvimento de complicações pulmonares nos pacientes disfágicos após tratamento do câncer de cabeça e pescoço (CCP) poderá contribuir para uma intervenção fonoaudiológica mais efetiva, melhorando a qualidade de vida destes indivíduos. Objetivo: identificar fatores de risco relacionados à penetração/aspiração, pneumonia e à função pulmonar pós-tratamento e avaliar a qualidade de vida (QV) de pacientes tratados do CCP. Método: Trata-se de um estudo prospectivo, realizado no Departamento de Cirurgia de Cabeça e Pescoço e Otorrinolaringologia, com pacientes com diagnóstico de CCP, sem tratamento prévio, no período de fevereiro de 2012 a maio de 2014, independentes de sexo e idade. Foram coletadas informações da evolução clínica pulmonar e possíveis fatores de risco para a penetração/aspiração: dados demográficos e clínicos, consumo de tabaco e álcool, uso de drogas que mudam o pH gástrico, HAD – Escala de ansiedade e depressão hospitalar, apoio da família, adesão do paciente, índice de massa corporal (IMC), higiene oral, nível de atividade física (IPAQ) e nível de consciência (Escala de Glasgow). Os pacientes foram submetidos à avaliação videofluoroscópica da deglutição, prova de função pulmonar e aplicação dos questionários de QV (UW-QOL – QV da Universidade de Washington; SGRQ – Questionário do Hospital Saint George na doença respiratória; MDADI – Questionário de disfagia M.D. Anderson)...

Introduction: To identify factors that contribute to aspiration and the development of pulmonary complications in dysphagia patients following head and neck cancer (HNC) treatment, which may contribute to a more effective SLP therapy intervention, thus improving the quality of life of these individuals. Objective: To identify risk factors related to penetration/aspiration and pulmonary clinical outcome post-treatment, and also to assess the quality of life (QOL) of patients treated for HNC. Methods: This was a prospective study conducted at the Head and Neck and Otorhinolaryngology Department with patients diagnosed with HNC, without prior treatment, from February 2012 to May 2014, independent of sex and age. The following information relating to pulmonary clinical outcome and possible risk factors for penetration/aspiration were collected: demographic and clinical data, tobacco and alcohol consumption, the usage of drugs that change gastric pH, HAD - Hospital Anxiety and Depression scale, family support, patient adherence, body mass index (BMI), oral hygiene, level of physical activity (IPAQ) and level of consciousness (Glasgow Scale). Patients were submitted to videofluoroscopic swallowing assessment, pulmonary function test and application of QOL questionnaires (UW-QOL – QV, University of Washington; SGRQ, Saint George Hospital Questionnaire; MDADI, MD Anderson Dysphagia Inventory). Patients were assessed in four stages: pretreatment, 1 to 3 months post-treatment, 6 to 9 months post-treatment and 12 to 18 months post-treatment. Results: The study consisted of 38 patients (28 males; 73.7%), with a mean age of 60 years (27-85 years). History of aspiration pneumonia was observed in 4 patients (10.5%). About half of the patients (47.4%) were dysphagic in pretreatment...