Biochemical and Microbiological Aspects of the Oral Cavity of Children and Young People with Neurological Impairment and Oropharyngeal Dysphagia.

The components and the salivary flow have a direct influence on the composition of the oral microbiota of children and young people with oropharyngeal dysphagia, and studies have already demonstrated the excessive accumulation of supragingival dental calculus in individuals with enteral nutrition. This study aimed to compare the oral hygiene, biochemical, and microbiological aspects of the oral cavity of children and young people with neurological impairment and oropharyngeal dysphagia. Forty children and young people with neurological impairment and oropharyngeal dysphagia were enrolled and divided into two groups: group I, encompassing 20 participants fed via gastrostomy; and group II, encompassing 20 participants fed via the oral route. Oral hygiene and salivary pH and flow were assessed, and a polymerase chain reaction was performed to evaluate the messenger RNA expressions of Porphyromonas gingivalis, Tanerella forsythia, and Treponema denticola. In groups I and II, the mean Oral Hygiene Index-Simplified scores were 4 and 2, respectively, showing a significant difference; the mean Calculus Index scores were 2 and 0, respectively, showing a significant difference; and the mean pH was 7.5 and 6.0, respectively, showing a significant difference. Bacterial analysis indicated no association between the two groups. It can be concluded that children and young people who use gastrostomy had a poorer oral hygiene, greater dental calculus deposition, and higher salivary pH. The saliva of patients in both groups contained Porphyromonas gingivalis, Tanerella forsythia, and Treponema denticola.

Comportamento alimentar de crianças com transtorno do espectro autista / Eating behavior of children with autism spectrum disorder

RESUMO Objetivo: O objetivo deste estudo foi analisar o comportamento alimentar de crianças e adolescentes com transtorno do espectro autista. Métodos: Participaram deste estudo 21 crianças e adolescentes com TEA, na faixa etária de 2 a 14 anos de idade, de ambos os sexos. Foi aplicado um questionário sobre o comportamento alimentar com os 21 pais e/ou responsáveis das crianças. Esse instrumento é composto por 53 questões, distribuídas nas seguintes categorias: Motricidade na mastigação; Seletividade alimentar; Aspectos comportamentais; Sintomas gastrointestinais; Sensibilidade sensorial e Habilidades nas refeições. Resultados: A análise dos dados obtidos revela que as crianças com TEA apresentaram maiores alterações no comportamento alimentar nas categorias Seletividade alimentar (34,4%), Aspectos comportamentais (27,1%) e Motricidade na mastigação (21,9%). E houve correlação entre a categoria Motricidade na mastigação com todas as outras categorias. Houve também correlação entre seletividade alimentar com aspectos comportamentais e aspectos comportamentais com sensibilidade sensorial e habilidades nas refeições. Conclusão: Portanto, é possível observar que, no comportamento alimentar de crianças e adolescentes com TEA, há uma tendência a seletividade alimentar, comportamentos habituais durante as refeições e dificuldades motoras no que se refere à mastigação e à ingestão dos alimentos.

ABSTRACT Objective: The objective of this study was to analyze feeding behavior in children with autism spectrum disorder. Methods: Twenty-one children with autism spectrum disorder (ASD) participated in this study, aged 2 to 14 years old, of both sexes. A questionnaire on feeding behavior was applied to the 21 parents and/or responsible of the children. This instrument consists of 53 questions, distributed in the following categories: Motricity in chewing; Food selectivity; Behavioral aspects; Gastrointestinal symptoms; Sensory sensitivity and Skills in meals. Results: The analysis of the data obtained reveals that children with ASD showed greater changes in eating behavior in the categories Food selectivity (34.4%), Behavioral aspects (27.1%) and Motricity in mastication (21.9%). And there was a correlation between the chewing motricity category with all other categories. There was also a correlation between food selectivity with behavioral aspects and behavioral aspects with sensory sensitivity and mealtime skills. Conclusion: Therefore, it is possible to observe that in the eating behavior of children with autism spectrum disorder there is a tendency towards food selectivity, habitual behaviors during meals and motor difficulties with regard to chewing and food intake.

CEDNIK syndrome in a Brazilian patient with compound heterozygous pathogenic variants.

CEDNIK (Cerebral Dysgenesis, Neuropathy, Ichthyosis, and Keratoderma) syndrome is a neuro ichthyotic syndrome characterized by a clinical constellation of features including severe developmental delay, microcephaly, and facial dysmorphism. Here, we report the clinical and molecular characterization of a patient with CEDNIK syndrome harboring two compound heterozygous variants in the SNAP29 gene. The patient presents a combination of a loss-of-function SNAP29 mutation and a ∼370 kb 22q11.2 deletion, each of these genetic variants inherited from one of the parents. This report provides detailed data of a patient with unprecedented genetic events leading to the CEDNIK phenotype and may contribute to the elucidation of this rare condition.

Interrater reliability in the temporal quantitative analysis of oropharyngeal swallowing using a specific software / Confiabilidade entre avaliadores na análise quantitativa temporal da deglutição orofaríngea por meio de software específico

ABSTRACT Purpose This study aims to analyze inter-judge reliability by measuring a few temporal parameters of swallowing using a specific software. Methods Six databases that include the results of reliability tests performed in prior studies by the present research group were employed. The datasets were obtained using the same measurement method and include data obtained based on puree (level 3) consistency and liquid (level 0) consistency according to the International Dysphagia Diet Standardization Initiative. In this study, the reliabilities corresponding to the total oral transit time (TOTT), initiation of the pharyngeal response time (PRT), and the pharyngeal transit time (PTT) were measured using the same software; the evaluations performed by a single rater for all datasets were used as the benchmark, and evaluations performed by new raters for each dataset were also included. The intra-class correlation coefficient (ICC) with a 95% confidence interval was employed. Results A total of 244 videofluoroscopic swallowing study images were analyzed. In all analyses, the ICCs were >0,75 and showed excellent agreement between the senior and junior raters. The TOTT for level 3 showed ICCs from 0.936 to 1.000 and that for level 0 showed ICCs from 0.997 to 1.000. Further, the PRT showed ICCs from 0.916 to 1.000 for level 3 and from 0.978 to 1.000 for level 0. The PTT showed ICCs from 0.848 to 1.000 for level 3 and from 0.984 to 1.000 for level 0. Conclusion The reliabilities obtained using this specific software for the TOTT, PRT, and PTT showed excellent agreement.

RESUMO Objetivo analisar a confiabilidade inter-juízes por meio da mensuração de alguns parâmetros temporais da deglutição por meio de um software específico. Método Foram utilizados seis bancos de dados que incluem os resultados dos testes de confiabilidade realizados em estudos anteriores do presente grupo de pesquisa. Os bancos de dados foram obtidos usando o mesmo método de medição e incluem dados obtidos na consistência purê (nível 3) e líquida (nível 0). Neste estudo, as confiabilidades correspondentes ao tempo de trânsito oral total (TTOT), tempo de início de resposta faríngea (IRF) e tempo de trânsito faríngeo (TTF) mensuradas no mesmo software; as avaliações realizadas por um único avaliador para todos os bancos de dados foram usadas como referência, e as avaliações realizadas por novos avaliadores para cada banco de dados também foram incluídas. Foi utilizado o coeficiente de correlação intraclasse (ICC) com intervalo de confiança de 95%. Resultados Foram analisadas 244 imagens de estudos videofluoroscópicos da deglutição. Em todas as análises, os ICCs foram >0,75, mostrando excelente concordância entre avaliadores. O TTOT do nível 3 apresentou ICCs de 0,936 a 1.000 e o do nível 0 apresentou ICCs de 0,997 a 1,000. Além disso, o IRF mostrou ICCs de 0,916 a 1,000 para o nível 3 e de 0,978 a 1,000 para o nível 0. O TTF mostrou ICCs de 0,848 a 1,000 para o nível 3 e de 0,984 a 1,000 para o nível 0. Conclusão As confiabilidades obtidas com este software específico para o TTOT, IRF e TTF mostraram excelente concordância.

Interrater reliability in the temporal quantitative analysis of oropharyngeal swallowing using a specific software.

PURPOSE: This study aims to analyze inter-judge reliability by measuring a few temporal parameters of swallowing using a specific software. METHODS: Six databases that include the results of reliability tests performed in prior studies by the present research group were employed. The datasets were obtained using the same measurement method and include data obtained based on puree (level 3) consistency and liquid (level 0) consistency according to the International Dysphagia Diet Standardization Initiative. In this study, the reliabilities corresponding to the total oral transit time (TOTT), initiation of the pharyngeal response time (PRT), and the pharyngeal transit time (PTT) were measured using the same software; the evaluations performed by a single rater for all datasets were used as the benchmark, and evaluations performed by new raters for each dataset were also included. The intra-class correlation coefficient (ICC) with a 95% confidence interval was employed. RESULTS: A total of 244 videofluoroscopic swallowing study images were analyzed. In all analyses, the ICCs were >0,75 and showed excellent agreement between the senior and junior raters. The TOTT for level 3 showed ICCs from 0.936 to 1.000 and that for level 0 showed ICCs from 0.997 to 1.000. Further, the PRT showed ICCs from 0.916 to 1.000 for level 3 and from 0.978 to 1.000 for level 0. The PTT showed ICCs from 0.848 to 1.000 for level 3 and from 0.984 to 1.000 for level 0. CONCLUSION: The reliabilities obtained using this specific software for the TOTT, PRT, and PTT showed excellent agreement.

Oral hygiene and oral microbiota in children and young people with neurological impairment and oropharyngeal dysphagia.

This study compared the oral hygiene and oral microbiota in children and young people with neurological impairment and oropharyngeal dysphagia with and without gastrostomy. Forty children and young people participated in this study: 19 females and 21 males, aged 2 to 22 years (mean age 8.6 years). Participants were divided into two groups: group I (GI = 20) with gastrostomy and group II (GII = 20) without gastrostomy (with oral feeding). Oral hygiene was assessed using the Simplified Oral Hygiene Index (SOHI). Analysis of two bacteria, Streptococcus mutans and Streptococcus sobrinus, was performed by collecting saliva using an oral swab, then mRNA expression was evaluated using the polymerase chain reaction (PCR) technique. The oral hygiene index had a general median of 2.2, and the two groups were statistically different (Group I: median 2.9 and Group II: median 2.0) (p = 0.01751). Bacterial analysis indicated 13 individuals with S. mutans and none with S. sobrinus. Of the 13 individuals with S. mutans, 6 were from Group I and 7 from Group II. Those with gastrostomy had worse oral hygiene, and both groups harbored the bacterium S. mutans.

Electrical, taste, and temperature stimulation in patients with chronic dysphagia after stroke: a randomized controlled pilot trial.

The objective of present study was compare a traditional swallowing therapy program with a new combined swallowing therapy program including neuromuscular electrical stimulation in patients with oropharyngeal dysphagia after stroke. This pilot study included eight patients with chronic oropharyngeal dysphagia after stroke. These patients underwent traditional therapy with gustative-thermic-tactile stimulation (group A), or a new combined program adding neuromuscular electrical stimulation (group B). Study participants were evaluated before and after the intervention using fiberoptic endoscopic evaluation of swallowing with temporal measures of posterior oral spillage and whiteout time, functional oral intake scale and a visual analog scale classifies an individual's swallowing ability. The two groups did not differ in terms of posterior oral spillage time, whiteout time and functional oral intake scale. Subjects in group B exhibited significant increases in visual analog scale scores. However, both groups demonstrated improvement with decreases in posterior oral spillage time, increased whiteout time, and increased functional oral intake scale and visual analog scale scores. There was no difference in the parameters studied in both therapeutic programs in individuals with chronic oropharyngeal dysphagia after stroke.

Influence of Thermal and Gustatory Stimulus in the Initiation of the Pharyngeal Swallow and Bolus Location Instroke.

INTRODUCTION/OBJECTIVE: The aim of this study is to analyze the influence of sour taste and cold temperature in the initiation of the pharyngeal swallow (IPS) and bolus location at pharyngeal swallow onset in individuals after stroke. METHODS: Cross-sectional prospective study. The study included 52 individuals with unilateral ischemic stroke. Each individual was assessed by videofluoroscopic swallowing study with 5ml of paste bolus offering four different stimuli (natural, cold, sour, and sour-cold). The individuals were divided into two groups according to the offer sequence. Group 1 (G1) - received a randomized sequence of stimuli (24 individuals), and Group 2 (G2) -the stimuli were offered in the following order: natural, cold, sour, and sour-cold(28 individuals). The IPS time and bolus location at pharyngeal swallow onset were analyzed. The bolus location at pharyngeal swallow onset was defined using six different levels. RESULTS: Individuals in G1 did not show a significant difference in IPS time between stimuli. However, individuals in G2 presented a significantly shorter IPS time with the sour and sour-cold stimuli than with natural or cold stimuli. The bolus location at pharyngeal swallow onset did not show significant difference between stimuli in both groups. On the other hand, in the G2 it was observed higher frequency of swallowing with sour-cold stimulus at level 1 (the bolus head was located in any region between the fauces pillar and the point where the tongue crosses the inferior border of the mandible). CONCLUSION: The sour and sour-cold stimuli influenced the IPS time when they were offered in a sequential order. Moreover, both the IPS time and bolus location at pharyngeal swallow onset were not influenced by the sour and sour cold-bolus when offered in a random sequence.

Oral transit time in children with neurological impairment indicated for gastrostomy. / Tempo de trânsito oral na criança com acometimento neurológico indicada à gastrostomia.

PURPOSE: The objective of the present study was to describe the total oral transit time (TOTT) of children with neurological impairment (CNI) and with an indication of gastrostomy. METHOD: A cross-sectional clinical study was conducted on 15 children (10 male and 5 female ranging in age from 1 to 14 years; mean 5.7 years) with CNI and gastrostomy indication. The patients were monitored by a Multidisciplinary Group of Pediatric Gastroenterology of Universidade de Marília - UNIMAR, which 13 of them with previous exclusive oral feeding and 2 fed by a nasogastric tube. Swallowing was analyzed by videofluoroscopy swallowing study and 19 images of TOTT were obtained using specific software, with analysis of pureed food (13 images) and liquid (six images). TTOT was categorized as short or long based on definitions already evidenced in the literature. RESULTS: The mean and standard deviation of TOTT values was 10.75 s and 11.76 s for pureed food and 4.22 s and 1.54 s for liquid food. CONCLUSION: The total oral transit time of pureed or liquid consistency was long in children with neurological involvement and with an indication of gastrostomy.

OBJETIVO: O objetivo deste estudo foi descrever o tempo de trânsito oral total (TTOT) da deglutição em diferentes consistências de alimento na criança com acometimento neurológico (CAN) e com indicação de gastrostomia. MÉTODO: Estudo clínico transversal incluiu 15 indivíduos com CAN e indicação de gastrostomia, sendo 10 do sexo masculino e cinco do sexo feminino, 13 com alimentação via oral exclusiva e dois com sonda nasogástrica, faixa etária de um a 14 anos, média de 5,7 anos, acompanhados no Grupo Multidisciplinar de Gastroenterologia Pediátrica da Universidade de Marília-UNIMAR. A deglutição foi analisada por estudo videofluoroscópico da deglutição. Foram mensuradas 19 imagens do tempo de trânsito oral total (TTOT) da deglutição, por software específico, utilizando alimento pastoso (13 imagens) e alimento líquido (seis imagens). O TTOT foi categorizado em curto ou longo baseado em definições já evidenciadas na literatura. RESULTADOS: A média e o desvio padrão para o TTOT foram, respectivamente, 10,75s e 11,76s para o pastoso e 4,22s e 1,54s para o líquido. CONCLUSÃO: O tempo de trânsito oral total é longo nas consistências pastosa e líquida em crianças com acometimento neurológico e com indicação de gastrostomia.

Fiberoptic endoscopic findings of oropharyngeal swallowing of different food consistencies in Amyotrophic Lateral Sclerosis. / Achados videoendoscópicos da deglutição em diferentes consistências de alimento na Esclerose Lateral Amiotrófica.

PURPOSE: This study aimed to compare the fiberoptic endoscopic findings of oropharyngeal swallowing of distinct food consistencies in Amyotrophic Lateral Sclerosis (ALS). METHODS: This was a retrospective clinical study of a convenience sample of 20 individuals (13 males and seven females aged 34 to 78 years old) with a diagnosis of ALS and oropharyngeal dysphagia confirmed by clinical and objective evaluation of swallowing, regardless of the bulbar or skeletal type and of the time of neurological diagnosis. The fiberoptic endoscopic evaluation of swallowing (FEES) of the liquid (N = 18), thickened liquid (N = 19) and pureed samples (N = 20) in a volume of 5 ml were analyzed. Data related to posterior oral spillage, pharyngeal residues, laryngeal penetration and/or aspiration after swallowing the three food consistencies were analyzed statistically by the Friedman ANOVA test. RESULTS: No impairment of laryngeal sensitivity was found in this population. There was no statistically significant difference in posterior oral spillage, penetration and/or aspiration between food consistencies. There was a statistically significant difference only related to pharyngeal residues of the thickened liquid and pureed consistency. CONCLUSION: Among the fiberoptic endoscopic findings of swallowing in ALS, only pharyngeal residues had a higher frequency depending on the consistency of food.

OBJETIVO: Este estudo teve por objetivo comparar os achados videoendoscópicos da deglutição orofaríngea em distintas consistências de alimento na Esclerose Lateral Amiotrófica (ELA). MÉTODO: Estudo clínico retrospectivo com amostra de conveniência. Foram incluídos 20 indivíduos com diagnóstico de ELA e disfagia orofaríngea confirmada por avaliação clínica e objetiva de deglutição, independentemente do tipo, bulbar ou esquelética, e tempo de diagnóstico neurológico, 13 do sexo masculino e sete do sexo feminino, faixa etária variando de 34 a 78 anos, média de 57 anos. Foram analisados os achados da videoendoscopia de deglutição (VED) nas consistências líquida (N=18), líquida espessada (N=19) e pastosa (N=20) no volume de cinco ml. Os achados sobre escape oral posterior, resíduos faríngeos, penetração laríngea e/ou aspiração foram comparados nas três consistências de alimento e a análise estatística utilizou o teste ANOVA de Friedman. RESULTADOS: Não foi encontrada alteração na sensibilidade laríngea nessa população. Não houve diferença estatística significativa entre as consistências de alimento na presença de escape oral posterior, penetração e/ou aspiração. Houve diferença estatística significativa somente com resíduos faríngeos na consistência líquida espessada e pastosa na ELA. CONCLUSÃO: Dentre os achados videoendoscópicos da deglutição na ELA, somente o resíduo faríngeo teve maior frequência na dependência da consistência de alimento.