Role of fiberoptic endoscopic evaluation of swallowing (FEES) in children with suspected dysphagia.

OBJECTIVE: To assess FEES findings in defining oral feeding safety in children with suspected dysphagia, comparing them with clinical feeding evaluation results. METHODS: This study comprised a case series involving children with suspected dysphagia, referred for evaluation by otolaryngologists and speech-language pathologists (SLPs) at a Brazilian quaternary public university hospital. These children underwent both clinical evaluations and fiberoptic endoscopic evaluation of swallowing (FEES), with a comprehensive collection of demographic and clinical data. Subsequently, the authors performed a comparative analysis of findings from both assessments. RESULTS: Most patients successfully completed the FEES procedure (93.7%), resulting in a final number of 60 cases included in the study. The prevalence of dysphagia was confirmed in a significant 88% of these cases. Suspected aspiration on clinical SLP evaluation was present in 34 patients. Of these, FEES confirmed aspiration or penetration in 28 patients. Among the 35 patients with aspiration or penetration on FEES, 7 (20%) had no suspicion on SLP clinical assessment. All seven patients in whom clinical SLP evaluation failed to predict penetration/aspiration had neurological disorders. The median age of the children was 2.8 years, and 49 (81.6%) had neurological disorders, while 35 (58.3%) had chronic pulmonary disease. The most prevalent complaints were choking (41.6%) and sialorrhea (23.3%). CONCLUSION: FEES can diagnose structural anomalies of the upper aerodigestive tract and significantly contribute to the detection of aspiration and penetration in this group of patients with suspected dysphagia, identifying moderate and severe dysphagia even in cases where clinical assessment had no suspicion.

Initial evaluation of a multidisciplinary pediatric aerodigestive program in a Brazilian hospital: challenges and mitigation strategies.

OBJECTIVE: To identify clinical and epidemiological characteristics of children evaluated by the pediatric aerodigestive program at the beginning of its activity, describe challenges in follow-up, and suggest mitigation strategies. METHODS: A case series was conducted describing the first 25 patients discussed by the aerodigestive team from a Brazilian quaternary public university hospital between April 2019 and October 2020. The median follow-up was 37 months. RESULTS: During the study period 25 children were seen by the group and the median age at first assessment was 45.7 months old. Eight children had a primary airway abnormality, five had a tracheostomy. Nine children had genetic disorders and one had esophageal atresia. Dysphagia was present in 80% of the patients, 68% had a history of chronic or recurrent lung disease, 64% had a gastroenterological diagnosis and 56% had neurological impairment. Moderate to severe dysphagia was identified in 12 children and 7 of these had an exclusive oral diet at the time. The majority of children (72%) had 3 or more comorbidities. Following team discussion, a change in feeding strategy was suggested in 56% of the children. The most frequently ordered exam was pHmetry (44%) and gastrostomy was the surgical procedure with the longest waiting list. CONCLUSIONS: Dysphagia was the most frequent issue encountered in this initial group of aerodigestive patients. Pediatricians caring for these children must be involved in aerodigestive team discussions and hospital policies must be revised to facilitate access to exams and procedures needed for this population.

Initial evaluation of a multidisciplinary pediatric aerodigestive program in a Brazilian hospital: challenges and mitigation strategies

Abstract Objective: To identify clinical and epidemiological characteristics of children evaluated by the pediatric aerodigestive program at the beginning of its activity, describe challenges in followup, and suggest mitigation strategies. Methods: A case series was conducted describing the first 25 patients discussed by the aerodigestive team from a Brazilian quaternary public university hospital between April 2019 and October 2020. The median follow-up was 37 months. Results: During the study period 25 children were seen by the group and the median age at first assessment was 45.7 months old. Eight children had a primary airway abnormality, five had a tracheostomy. Nine children had genetic disorders and one had esophageal atresia. Dysphagia was present in 80% of the patients, 68% had a history of chronic or recurrent lung disease, 64% had a gastroenterological diagnosis and 56% had neurological impairment. Moderate to severe dysphagia was identified in 12 children and 7 of these had an exclusive oral diet at the time. The majority of children (72%) had 3 or more comorbidities. Following team discussion, a change in feeding strategy was suggested in 56% of the children. The most frequently ordered exam was pHmetry (44%) and gastrostomy was the surgical procedure with the longest waiting list. Conclusions: Dysphagia was the most frequent issue encountered in this initial group of aerodigestive patients. Pediatricians caring for these children must be involved in aerodigestive team discussions and hospital policies must be revised to facilitate access to exams and procedures needed for this population.

Arginine and glutamine supplementation on transthyretin levels in severely burned patients: A systematic review.

OBJECTIVE: The aim of this study was to investigate the influence of supplementation with the immunomodulators arginine and glutamine on transthyretin levels in burn patients. METHODS: This systematic review followed the protocol proposed and registered in PROSPERO (CRD42021239526) and was carried out following the PRISMA checklist for systematic reviews. Forty-four studies were evaluated. Of the 44, we included 6 for complete analysis. RESULTS: In five of the six clinical trials, glutamine was the most used immunomodulator (0.5 g·kg·d-1 or 12-14 g/d), followed by arginine in three of the clinical trials (10-14 g/d in adults or 2% of total energy value in children). The findings of the studies were that the patients who received either of these supplements presented the following results: increased transthyretin, lymphoproliferative response, and serum glutamine values, as well as shorter stay in the intensive care unit, a significant reduction in C-reactive protein values, and a tendency toward a faster healing of the burns compared with the control groups. CONCLUSION: In view of the content in the present review, it is possible to affirm that the supplementation of immunomodulators in burn patients is an effective strategy for their treatment, and that the adequate nutritional offer may be a predictor of a favorable outcome. However, regarding the increase in transthyretin values, this finding needs to be considered with reservations as the values can be altered by the inflammatory activity, and not necessarily related to the use of a supplement.

Reducing public health costs with infant formulas: oral food challenge and misdiagnosed or outgrown cow's milk protein allergy

Background: Accurate diagnosis and appropriate management of cow's milk protein allergy (CMPA) are crucial for avoiding unnecessary prescription of infant formulas. Here, we aimed to use the oral food challenge (OFC) for CMPA confirmation and for assessing development of natural tolerance to milk, in children with clinical CMPA diagnosis. We also assessed the economic impact in public health-care costs of reducing the prescription of infant formulas (provided by the São Paulo State's public health service, in Brazil, until two years of age) after ruling out CMPA diagnosis in children with negative OFC results. Methods: We reviewed medical records of 76 children [41 males, median age = 2.0 years (0.8-5.0)] who underwent OFC from January 2016 to June 2018, 30 of whom were ≤2 years old. Results: Before OFC, 52 (68.4%), 20 (26.3%) and five (5.3%) children were diagnosed with non-IgE-mediated, IgE-mediated and mixed CMPA, respectively. Most children were fed with aminoacid-based formulas (n=29, 38%). OFC was negative in 58 (76%) children, thus ruling out CMPA diagnosis. Out of 18 (24%) OFC-positive children, most (n=10, 56%) had gastrointestinal symptoms. After ruling out CMPA diagnosis, a mean of 152.3 formula cans (2,161.14 US dollars) were saved per children by the public health service. The total amount saved was 64,834.27 US dollars. Conclusions: OFC proved important not only for ruling out misdiagnosed CMPA, but also for preventing the indiscriminate use of infant formulas, which, in turn, had positive consequences for public health costs.

Objetivos: O diagnóstico preciso e o manejo apropriado da alergia à proteína do leite de vaca (alergia ao leite, AL) são cruciais para evitar a prescrição desnecessária de fórmulas infantis. Aqui, tivemos o objetivo de usar o teste de provocação oral aberto (TPOA) para a confirmação do diagnóstico de AL e para avaliar o desenvolvimento de tolerância natural ao leite, em crianças com diagnóstico clínico de AL. Também avaliamos o impacto nos custos de saúde pública da redução da prescrição de fórmulas infantis (fornecidas pelo serviço de saúde do estado de São Paulo até os dois anos de idade) após descartar o diagnóstico de AL em crianças com TPOA negativo. Métodos: Revisamos prontuários de 76 crianças [41 do sexo masculino, mediana de idade = 2. anos (0,8-5,0)] submetidas ao TPOA de janeiro de 2016 a junho de 2018, 30 das quais tinham dois anos de idade ou menos. Resultados: Antes do TPOA, 52 (68.4%), 20 (26.3%) e cinco (5.3%) crianças foram diagnosticadas com AL não mediada por IgE, mediada por IgE e de mecanismo misto, respectivamente.  A maioria das crianças era alimentada com fórmulas baseadas em aminoácidos (n=29, 38%). O TPOA foi negativo em 58 (76%) crianças, descartando, assim, o diagnóstico de AL. De 18 (24%) crianças positivas para o TPOA, a maioria (n=10, 56%) tinha sintomas gastrointestinais. Após descartar o diagnóstico de AL, uma média de 152,3 latas de fórmula (R$ 8.644,57) foram economizadas, por criança, pelo serviço público de saúde. O montante total economizado foi de R$ 259.337,09. Conclusão: O TPOA se mostrou importante, não apenas para descartar o diagnóstico de AL, como também para evitar o uso indiscriminado de fórmulas infantis, o que, por sua vez, teve consequências positivas para os custos de saúde pública.

Evaluation of growth and nutritional status in children and adolescents with extrahepatic portal vein obstruction and portal hypertension / Avaliação do estado nutricional e do crescimento em crianças e adolescentes com obstrução extra-hepática de veia porta e hipertensão portal

ABSTRACT Objective: This study aims to evaluate dietary intake, nutritional status, and growth rate in children and adolescents with extrahepatic portal vein obstruction and portal hypertension. Methods: Outpatients aged 1-18 years, diagnosed with extrahepatic portal vein obstruction and portal hypertension, who had no associated diseases, and who had not been subjected to a venous shunt were included in this study. Two evaluations were carried out in this study: an initial (evaluation 1) and a final evaluation (evaluation 2), with a three-month minimum interval between them. In each evaluation, dietary intake was analyzed comparing the results with recommended energy intake using the Harris & Benedict equation and participants' anthropometric data, such as weight, height, mid-arm muscle circumference, weight-for-age, height-for-age, and body mass index-for-age, based on the World Health Organization 2006 standards. Results: A total of 22 patients participated in this study. There was a significant improvement in weight, height, body mass index, and mid-arm muscle circumference measurements (p<0.001; p<0.001; p<0.017; p=0.0018 respectively) and in the relationship between dietary intake and energy recommended energy intake, according to the Harris & Benedict equation (p=0.0001) from the first and second evaluation. Conclusion: Extrahepatic portal vein obstruction and portal hypertension were not shown to be factors predisposing to malnourishment.

RESUMO Objetivo: Este estudo tem como objetivo avaliar a ingestão alimentar, o estado nutricional e a taxa de crescimento de crianças e adolescentes com obstrução extra-hepática de veia porta e hipertensão portal. Métodos: Pacientes ambulatoriais com idades entre 1 e 18 anos, diagnóstico de obstrução extra-hepática de veia porta e hipertensão portal, sem patologias associadas e que não foram submetidos a um shunt venoso, foram incluídos neste estudo. O estudo compreendeu duas avaliações, no início (avaliação 1) e uma avaliação final (avaliação 2) com um intervalo mínimo de três meses entre ambos. Cada avaliação analisou a ingestão dietética comparando os resultados com as recomendações de energia usando a equação de Harris & Benedict e os dados antropométricos dos participantes, tais como peso, altura, circunferência muscular do braço, peso por idade, altura por idade e índice de massa corpórea por idade utilizando a norma da Organização Mundial da Saúde de 2006. Resultados: Participaram do estudo 22 pacientes. Foi observada uma melhora significativa entre a primeira e a segunda avaliação para as medidas de peso, estatura, índice de massa corporal e circunferência muscular do braço (p<0,001; p<0,001; p<0,017; p=0,0018, respectivamente) e a relação de ingestão dietética e recomendação energética equação de Harris & Benedict (p=0,0001). Conclusão: Obstrução extra-hepática de veia porta e hipertensão portal não se mostraram como fatores predisponentes para desnutrição em nosso estudo.

Biliary atresia: the Brazilian experience.

OBJECTIVE: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia. METHODS: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers (1982-2008) and compared regarding decades of surgery. RESULTS: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). CONCLUSIONS: This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.

Atresia biliar: a experiência Brasileira / Biliary atresia: the Brazilian experience

OBJETIVO: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. MÉTODOS: Dados sobre portoenterostomia, transplante hepático (TxH), idade no último seguimento e sobrevida foram coletados dos prontuários de pacientes acompanhados em seis centros no Brasil (1982-2008) e comparados em relação às décadas do procedimento cirúrgico. RESULTADOS: Dos 513 pacientes, 76,4 por cento foram submetidos a portoenterostomia [idade: 60,0-94,7 (82,6±32,8) dias] e 46,6 por cento foram submetidos a TxH. Em 69 por cento dos casos, o TxH foi realizado após a portoenterostomia, enquanto em 31 por cento dos casos o TxH foi realizado como cirurgia primária. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008) e Sudeste (p = 0,0012), embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6 por cento. A sobrevida aumentou nas últimas décadas (anos 1980 versus 1990, p = 0,002; anos 1980 versus 2000, p < 0,001; anos 1990 versus 2000, p < 0,001). A sobrevida de 4 anos pós-portoenterostomia, com ou sem TxH, foi de 73,4 por cento, inversamente correlacionada à idade no momento da portoenterostomia (80, 77,7, 60,5 por cento para < 60, 61-90, > 90 dias, respectivamente). Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3 por cento). A sobrevida de 4 anos com fígado nativo foi de 36,8 por cento, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6 por cento para < 60, 61-90, > 90 dias, respectivamente). CONCLUSÕES: Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portadoras de atresia biliar ainda é um problema no Brasil, influenciando a sobrevida destes pacientes. Estratégias que proporcionam o encaminhamento precoce estão sendo desenvolvidas com o objetivo de reduzir a necessidade de transplante hepático nos primeiros anos de vida.

OBJECTIVE: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia. METHODS: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers (1982-2008) and compared regarding decades of surgery. RESULTS: Of 513 patients, 76.4 percent underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6 percent underwent LTx. In 69 percent of cases, LTx followed portoenterostomy, whereas in 31 percent of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6 percent. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4 percent, inversely correlated with age at portoenterostomy (80, 77.7, 60.5 percent for < 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3 percent). The 4-year native liver survival was 36.8 percent, inversely correlated with age at portoenterostomy (54, 33.3, 26.6 percent for < 60, 61-90, > 90 days, respectively). CONCLUSIONS: This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.

Partial internal biliary diversion through a cholecystojejunocolonic anastomosis--a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report.

BACKGROUND/PURPOSE: The purpose of the study was to describe the initial experience with a novel approach to the surgical treatment of progressive familial intrahepatic cholestasis (PFIC), avoiding the creation of a permanent stoma. METHODS: Two teenaged patients, aged 15 and 17 years, underwent partial internal biliary diversion to treat uncontrollable pruritus associated with PFIC. The surgical technique involved the creation of an isolated jejunal conduit, anastomosed proximally in a terminolateral fashion to the gallbladder and distally to the ascending colon. This operation combines the advantages of partially diverting the biliary flow from the enterohepatic cycle, avoiding an external biliary fistula. In one of the patients, this technique was used as a primary procedure, whereas in the other, a previous partial external diversion was converted to an internal diversion. RESULTS: Both patients had complete resolution of their pruritus and normalization of hepatic laboratory tests. One of the patients developed a mild choleretic diarrhea that can be controlled with eventual use of cholestyramine. No complications were observed related to this operation. CONCLUSIONS: Biliary diversion appears to be a very attractive surgical option for the treatment of PFIC in children with a normal gallbladder. Long-term follow-up is necessary to evaluate late results and eventual complications of this approach.