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.

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.

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.