Training of children's and adolescents' family members in home parenteral nutrition care / Capacitação dos familiares de crianças e adolescentes para os cuidados com nutrição parenteral domiciliar

ABSTRACT Objective: To report the experience of the training in home parenteral nutrition (PN) directed to family members of children and adolescents participating in a multidisciplinary intestinal rehabilitation program of a tertiary public hospital. Methods: Cross-sectional descriptive study with family caregivers of patients from the Intestinal Rehabilitation Program of Hospital de Clínicas de Porto Alegre, RS, Brazil, from July/2014 to January/2017. Inclusion criteria: family members of children aged 30 days to 17 years and estimated PN use ≥8 weeks; and family members motivated to care for the child. The training covered: hand washing and disinfection; infusion pump handling; and central venous catheter (CVC) and PN care. Outcomes assessed: catheter-related bloodstream infection (CRBSI) rate, accidental CVC exit, end of PN infusion with more than 60minutes of delay or advance compared to the time predicted, mechanical obstruction, bleeding in the CVC insertion site, and death. Results: Twenty-seven family members of 17 children were trained. Their median age was 28 (18-60) years, and 63% were mothers. The mean CRBSI rate was 1.7/1,000 days of CVC use, and 29.4% of patients had at least one episode of accidental CVC exit. There were no complications related to PN infusion, bleeding, or death. Conclusions: The training of family caregivers allowed the safe implementation of home PN, with the active participation of families, making the procedure feasible in the public health system in Brazil.

RESUMO Objetivo: Apresentar a experiência da capacitação de familiares de crianças e adolescentes participantes de um programa multiprofissional de reabilitação intestinal de um hospital público terciário para uso de nutrição parenteral (NP) no domicílio. Métodos: Estudo descritivo transversal com familiares cuidadores de pacientes do Programa de Reabilitação Intestinal do Hospital de Clínicas de Porto Alegre, RS, Brasil, entre julho/2014 e janeiro/2017. Critérios de inclusão: familiares das crianças com idades entre 30dias e 17 anos e previsão de uso de NP≥8 semanas; e familiares que demonstrassem motivação para os cuidados da criança. Acapacitação contemplou: lavagem e higienização das mãos; manuseio da bomba de infusão; cuidados com cateter venoso central (CVC) e com solução de NP. Desfechos avaliados foram: taxa de infecção de corrente sanguínea relacionada ao CVC (ICSRC), saída acidental do CVC, término da infusão da NP com atraso ou adiantamento maior que 60minutos em comparação ao previsto, obstrução mecânica, sangramento do sítio de inserção do CVC e óbito. Resultados: Foram capacitados 27 familiares de 17 crianças, com mediana de idade de 28 (18-60) anos, dos quais 63% eram mães. A taxa média de ICSRC observada foi 1,7/1.000 dias de uso de CVC, e a saída acidental do CVC ocorreu em 29,4% dos pacientes. Não foram observadas complicações referentes à infusão da NP, a sangramento ou a óbito. Conclusões: A capacitação de familiares cuidadores possibilitou a execução de maneira segura da NP no domicílio com participação ativa das famílias, tornando o procedimento viável no sistema público de saúde no Brasil.

TRAINING OF CHILDREN'S AND ADOLESCENTS' FAMILY MEMBERS IN HOME PARENTERAL NUTRITION CARE.

OBJECTIVE: To report the experience of the training in home parenteral nutrition (PN) directed to family members of children and adolescents participating in a multidisciplinary intestinal rehabilitation program of a tertiary public hospital. METHODS: Cross-sectional descriptive study with family caregivers of patients from the Intestinal Rehabilitation Program of Hospital de Clínicas de Porto Alegre, RS, Brazil, from July/2014 to January/2017. Inclusion criteria: family members of children aged 30 days to 17 years and estimated PN use ≥8 weeks; and family members motivated to care for the child. The training covered: hand washing and disinfection; infusion pump handling; and central venous catheter (CVC) and PN care. Outcomes assessed: catheter-related bloodstream infection (CRBSI) rate, accidental CVC exit, end of PN infusion with more than 60minutes of delay or advance compared to the time predicted, mechanical obstruction, bleeding in the CVC insertion site, and death. RESULTS: Twenty-seven family members of 17 children were trained. Their median age was 28 (18-60) years, and 63% were mothers. The mean CRBSI rate was 1.7/1,000 days of CVC use, and 29.4% of patients had at least one episode of accidental CVC exit. There were no complications related to PN infusion, bleeding, or death. CONCLUSIONS: The training of family caregivers allowed the safe implementation of home PN, with the active participation of families, making the procedure feasible in the public health system in Brazil.

Associations of birth mode with cord blood cytokines, white blood cells, and newborn intestinal bifidobacteria.

The associations of Cesarean delivery with offspring metabolic and immune-mediated diseases are believed to derive from lack of mother-to-newborn transmission of specific microbes at birth. Bifidobacterium spp., in particular, has been hypothesized to play a health-promoting role, yet little is known about how delivery mode modifies colonization of the newborn by this group of microbes. The aim of this research was to examine the presence of Bifidobacterium in meconium and in the transitional stool, and to assess cytokine levels and hematological parameters in the venous cord blood of infants born by elective, pre-labor Cesarean section vs. vaginal delivery in Southern Brazil. We recruited 89 mother-newborn pairs (23 vaginal delivery and 66 elective cesarean delivery), obtained demographic information from a structured questionnaire and clinical information from medical records. We obtained umbilical cord venous blood and meconium samples following delivery and the transitional stool (the first defecation after meconium) before discharge. We determined plasma levels of IL-1ß, IL-10, IL-6, GM-CSF, IL-5, IFN-γ, TNF-α, IL-2, IL-4 and IL-8 in the cord blood, and presence of stool Bifidobacterium by real time PCR. Compared to vaginally-delivered neonates, Cesarean-delivered neonates had a lower leukocyte count (p = 0.037), lower hemoglobin (p = 0.04), and lower levels of the cytokine GM-CSF (p = 0.009) in the cord blood. Moreover, Bifidobacterium was detected less often in the transitional stool of Cesarean-delivered neonates compared to vaginally-delivered neonates (p = 0.001). The results indicate that pre-labor Cesarean birth may be associated with microbial and hematological alterations in the neonate. The clinical significance of these findings remains to be determined in larger prospective birth cohort studies.

Avaliação nutricional em crianças e adolescentes com cirrose: estudo transversal de duas séries / Nutritional assessment in children and adolescents with cirrhosis: cross-sectional study of two series

Introdução: A cirrose caracteriza-se por uma alteração crônica do parênquima hepático que frequentemente leva à desnutrição em crianças e adolescentes. A intervenção nutricional deve ser feita precocemente, o que requer um cuidadoso acompanhamento desses pacientes. Objetivos: Comparar os resultados da avaliação nutricional de crianças e adolescentes cirróticos realizada em dois períodos de tempo distintos. Métodos: Foram utilizados bancos de dados oriundos de duas pesquisas conduzidas com pacientes pediátricos com cirrose. Após a aplicação de critérios de inclusão e exclusão, 67 crianças e adolescentes foram avaliados em duas séries com intervalo de aproximadamente uma década entre elas. As duas séries tiveram as variáveis antropométricas estatura para idade (E/I) e dobra cutânea tricipital para idade (DCT/I) avaliadas de acordo com os padrões da Organização Mundial de Saúde. A gravidade da doença foi avaliada pelos modelos Pediatric End-stage Liver Disease (PELD)/ Model for End-stage Liver Disease (MELD) e pelo escore Child-Pugh. O nível de significância foi estabelecido em 5%. Resultados: Os resultados da avaliação do estado nutricional dos pacientes nas duas séries não mostraram diferença estatisticamente significativa. Na série 1, 22,6% dos pacientes apresentaram desnutrição, e 27,8% na série 2 (p = 0,955). Conclusões: Podemos concluir que nas duas séries avaliadas, separadas por aproximadamente uma década, o percentual de desnutrição e a gravidade da cirrose se mantiveram estáveis (AU)

Introduction: Cirrhosis is characterized by a chronic alteration of the liver parenchyma that often leads to malnutrition in children and adolescents. Nutritional intervention should be performed early, requiring careful follow-up of these patients. Objectives: To compare the nutritional assessment of cirrhotic pediatric patients performed in two separate periods of time. Methods: This research used two different databases originated from studies conducted with pediatric patients with cirrhosis. After applying inclusion and exclusion criteria, 67 children and adolescents were assessed in two series of tests performed within a time range of approximately a decade. Both series had standard deviation score for height-for-age (SDS-H/A), standard deviation score for triceps skinfold-for-age and (SDS-TSF/A), calculated according to the standards established by the World Health Organization. Disease severity was evaluated by the Pediatric End-stage Liver Disease (PELD)/Model for End-stage Liver Disease (MELD) and by the Child-Pugh score. Results were considered significant at p < 0.05. Results: The present study did not find any statistically significant difference for the nutritional status of the researched subjects in any of the series. In the first series, 22.6% of patients were undernourished, compared to 27.8% in the second one (p = 0.955). Conclusions: We can conclude that in both series of tests conducted with an interval of about a decade from each other the percentage of malnutrition and the severity of cirrhosis remained stable (AU)

Serum proinflammatory cytokines and nutritional status in pediatric chronic liver disease.

AIM: To evaluate the nutritional status and its association with proinflammatory cytokines in children with chronic liver disease. METHODS: We performed a cross-sectional study with 43 children and adolescents, aged 0 to 17 years, diagnosed with chronic liver disease. All patients regularly attended the Pediatric Hepatology Unit and were under nutritional follow up. The exclusion criteria were fever from any etiology at the time of enrollment, inborn errors of the metabolism and any chronic illness. The severity of liver disease was assessed by Child-Pugh, Model for End-stage Liver Disease (MELD) and Pediatric End Stage Liver Disease (PELD) scores. Anthropometric parameters were height/age, body mass index/age and triceps skinfold/age according to World Health Organization standards. The cutoff points for nutritional status were risk of malnutrition (Z-score < -1.00) and malnutrition (Z-score < -2.00). Interleukin-1ß (IL-1ß), IL-6 and tumor necrosis factor-α levels were assessed by commercial ELISA kits. For multivariate analysis, linear regression was applied to assess the association between cytokine levels, disease severity and nutritional status. RESULTS: The median (25(th)-75(th) centile) age of the study population was 60 (17-116)-mo-old, and 53.5% were female. Biliary atresia was the main cause of chronic liver disease (72%). With respect to Child-Pugh score, cirrhotic patients were distributed as follows: 57.1% Child-Pugh A, a mild presentation of the disease, 34.3% Child-Pugh B, a moderate stage of cirrhosis and 8.6% Child-Pugh C, were considered severe cases. PELD and MELD scores were only above the cutoff point in 5 cases. IL-6 values ​​were increased in patients at nutritional risk (34.9%) compared with those who were well-nourished [7.12 (0.58-34.23) pg/mL vs 1.63 (0.53-3.43) pg/mL; P = 0.02], correlating inversely with triceps skinfold-for-age z-score (rs = -0.61; P < 0.001). IL-6 levels were associated with liver disease severity assessed by Child-Pugh score (P = 0.001). This association remained significant after adjusting for nutritional status in a linear regression model. CONCLUSION: High IL-6 levels were found in children with chronic liver disease at nutritional risk. Inflammatory activity may be related to nutritional status deterioration in these patients.

Validation of a subjective global assessment questionnaire.

OBJECTIVE: To validate the Subjective Global Nutritional Assessment (SGNA) questionnaire for Brazilian children and adolescents. METHODS: A cross-sectional study with 242 patients, aged 30 days to 13 years, treated in pediatric units of a tertiary hospital with acute illness and minimum hospitalization of 24h. After permission from the authors of the original study, the following criteria were observed to obtain the validation of SGNA instruments: translation and backtranslation, concurrent validity, predictive validity, and inter-observer reliability. The variables studied were age, sex, weight and length at birth, prematurity, and anthropometry (weight, height, body mass index, upper arm circumference, triceps skinfold, and subscapular skinfold). The primary outcome was considered as the need for admission/readmission within 30 days after hospital discharge. Statistical tests used included ANOVA, Kruskal-Wallis, Mann-Whitney, chi-square, and Kappa coefficient. RESULTS: According to SGNA score, 80% of patients were considered as well nourished, 14.5% moderately malnourished, and 5.4% severely malnourished. Concurrent validity showed a weak correlation between the SGNA and anthropometric measurements (p<0.001). Regarding predictive power, the main outcome associated with SGNA was length of admission/readmission. Secondary outcomes associated included the following: length of stay at the unit after SGNA, weight and length at birth, and prematurity (p<0.05). The interobserver reliability showed good agreement among examiners (Kappa=0.74). CONCLUSION: This study validated the SGNA in this group of hospitalized pediatric patients, ensuring its use in the clinical setting and for research purposes in the Brazilian population.

Cesarean delivery and metabolic risk factors in young adults: a Brazilian birth cohort study.

BACKGROUND: Cesarean delivery (CD) perturbs the assembly of the neonatal gut microbiome and has been associated with child and adult obesity. However, it is still unknown whether CD is associated with metabolic risk factors in young adults. OBJECTIVE: We investigated the association of CD and metabolic risk factors in young adults in a cohort study who were 23-25 y of age at follow-up. DESIGN: We used data from a cohort study in Ribeirão Preto, Brazil. Baseline data on 6827 singleton pregnancies were collected in 1978-1979, and a sample of 2063 subjects were followed up 23-25 y later (2002-2004). Information on the type of delivery, birth weight, maternal age, parity, maternal schooling, and maternal smoking was obtained after birth. Anthropometric data, biochemical measurements, and information on participant schooling and smoking history were collected at 23-25 y of age. A linear regression was performed to assess the association between CD and biochemical measurements in early adulthood, controlling for a minimum set of confounders that were identified in a directed acyclic graph. RESULTS: The mean ± SD age of the subjects was 23.9 ± 0.71 y, and 51.8% of the sample were women. The CD rate was 32.0% and was more common in older (P < 0.001) and more educated mothers (P < 0.001). Compared with vaginal delivery, CD was associated with higher body mass index (BMI) after multivariable adjustment (P < 0.001) but not with glucose, homeostasis model assessment of insulin resistance, the quantitative insulin-sensitivity check index, total cholesterol, LDL cholesterol, HDL cholesterol, or triglycerides (all P > 0.05). CONCLUSION: In our sample of Brazilian adults, CD was associated with higher BMI but not with other metabolic risk factors.

Cesarean section and increased body mass index in school children: two cohort studies from distinct socioeconomic background areas in Brazil.

BACKGROUND: Recent studies have raised controversy regarding the association between cesarean section and later obesity in the offspring. The purpose of this study was to assess the association of cesarean section with increased body mass index (BMI) and obesity in school children from two Brazilian cities with distinct socioeconomic backgrounds. METHODS: Two birth cohorts respectively born in 1994 in Ribeirao Preto, a wealthy city in Southeast, and in 1997/98 in Sao Luis, a less wealthy city in Northeast of Brasil, were evaluated. After birth, 2,846 pairs of mothers-newborns were evaluated in Ribeirao Preto and 2,542 in Sao Luis. In 2004/05, 790 children aged 10/11 years were randomly reassessed in Ribeirao Preto and 673 at 7/9 years in Sao Luis. Information on type of delivery, maternal and child characteristics, socioeconomic position and anthropometric measurements were collected after birth and at school age. Obesity was defined as BMI ≥ 95th percentile at school age. RESULTS: Obesity rate was 13.0% in Ribeirao Preto and 2.1% in Sao Luis. Cesarean section was associated with obesity and remained significant after adjustment only in Ribeirao Preto [OR = 1.74 (95% CI: 1.04; 2.92)]. The association between cesarean section and BMI remained significant after adjustment for maternal schooling, maternal smoking during pregnancy, duration of breastfeeding, gender, birth weight and gestational age, type of school and, only in Sao Luis, pre-pregnancy maternal weight. In Ribeirao Preto children born by cesarean section had BMI 0.31 kg/m2 (95% CI: 0.11; 0.51) higher than those born by vaginal delivery. In Sao Luis BMI of children born by cesarean section was 0.28 kg/m2 higher (95% CI: 0.08; 0.49) than those born by vaginal delivery. CONCLUSION: A positive association between cesarean section and increased BMI z-score was demonstrated in areas with different socioeconomic status in a middle-income country.

Risco nutricional e desnutrição em crianças e adolescentes com cirrose: o papel da avaliação nutricional / Nutritional risk and malnutrition in children and adolescents with cirrhosis: the role of nutritional assessment

Introdução: A desnutrição é um importante fator que interfere no prognóstico de crianças e adolescentes com cirrose. Este estudo tem como objetivo avaliar o estado nutricional e a adequação da ingesta alimentar de crianças e adolescente com cirrose. Métodos: Estudo transversal, realizado com 39 crianças e adolescentes cirróticos com idade entre 0-15 anos. A gravidade da doença hepática foi avaliada pelo critério de Child-Pugh e escores Pediatric End-Stage Liver Disease e/ou Model for End-Stage Liver Disease. A classificação do estado nutricional foi determinada de acordo com os padrões WHO (2009) e Frisancho (2008). A avaliação da ingesta alimentar foi realizada a partir de um registro alimentar de três dias. Resultados: As médias e desvios padrão dos escores z para os parâmetros Peso/Idade (P/I), Índice de Massa Corporal/Idade (IMC/I), Estatura/Idade (E/I), Circunferência do Braço/Idade (CB/I) e Dobra Cutânea Tricipital/Idade (DCT/I) foram respectivamente -0,53 (±1,17), 16,8 (±2,53), -1,22 (±1,20), -1,04 (±1,61) e -0,99 (±1,67), caracterizando cerca de 44% como desnutridos; sendo que 69% destes eram desnutridos graves (abaixo do escore-z -3,00). A ingesta alimentar média dos cirróticos (33/39), excluindo aqueles em aleitamento materno, dieta enteral e/ou restrição dietética, comparada com a RDI para idade foi de 112% (±36), sendo que a maioria 78,4% (26/33) apresentou uma ingesta maior ou igual a 80% da recomendação para a idade. Conclusão: A associação de parâmetros antropométricos, clínicos e dietéticos deve ser utilizada para que se possa chegar a um diagnóstico nutricional coerente e intervenção nutricional efetiva.

Introduction: Malnutrition is an important factor affecting the prognosis of children and teenagers with cirrhosis. This study aims to evaluate the nutritional status and adequacy of food intake by children and adolescents with cirrhosis. Methods: Cross-sectional study of 39 cirrhotic children and adolescents aged 0-15 years. The severity of liver disease was evaluated by Child-Pugh scores and Pediatric End-Stage Liver Disease and/or Model for End-Stage Liver Disease. The nutritional status was determined according to WHO standards (2009) and Frisancho (2008). The evaluation of food intake was made by recording food intake for three days. Results: The means and standard deviations of z scores for the parameters weight/age (W/A), body mass index/age (BMI/A), Height/Age (H/A), arm circumference/age (A /I), and triceps skinfold/Age (TS/A) were respectively -0.53 (± 1.17), 16.8 (± 2.53), -1.22 (± 1.20), -1.04 (± 1.61), and -0.99 (± 1.67), characterizing about 44% as malnourished, 69% of whom as severely malnourished (z-score <3.00). The mean dietary intake of cirrhotic patients (33/39), excluding those in breast-feeding, enteral feeding and/or dietary restriction, as compared with the RDI for age was 112% (± 36), most of which (78.4% , 26/33) with an intake > 80% as recommended for their age. Conclusion: A combination of anthropometric, clinical and dietary factors should be used so that a coherent nutritional diagnosis and effective nutritional intervention can be made.

Intestinal permeability assessed by 51Cr-EDTA in rats with CCl4 - induced cirrhosis.

CONTEXT: The straight relationship between cirrhosis and impaired intestinal barrier has not been elucidated yet. OBJECTIVES: To verify (51)Cr-EDTA-intestinal permeability in rats with CCl(4)-induced cirrhosis and controls. METHOD: Fifty male Wistar rats weighing 150-180 g were separated in three groups: 25 animals received CCl(4) 0.25 mL/kg with olive oil by gavage with 12 g/rat/day food restriction for 10 weeks (CCl(4)-induced cirrhosis); 12 received the same food restriction for 10 weeks (CCl(4)-non exposed). Other 13 rats received indomethacin 15 mg/kg by gavage as positive control of intestinal inflammation. RESULTS: The median (25-75 interquartile range) (51)Cr-EDTA-IP values of cirrhotic and CCl(4)-non exposed rats were 0.90% (0.63-1.79) and 0.90% (0.60-1.52) respectively, without significant difference (P = 0.65). Animals from indomethacin group showed (51)Cr-EDTA-IP, median 7.3% (5.1-14.7), significantly higher than cirrhotic and CCl(4)-non exposed rats (P<0.001). CONCLUSION: This study showed the lack of difference between (51)Cr-EDTA-intestinal permeability in rats with and without cirrhosis. Further studies are necessary to better clarify the relationship between intestinal permeability and cirrhosis.

Intestinal permeability assessed by 51Cr-EDTA in rats with CCl4 - induced cirrhosis / Permeabilidade intestinal ao 51-Cr-EDTA em ratos com cirrose induzida por CCl4

CONTEXT: The straight relationship between cirrhosis and impaired intestinal barrier has not been elucidated yet. OBJECTIVES: To verify 51Cr-EDTA-intestinal permeability in rats with CCl4-induced cirrhosis and controls. METHOD: Fifty male Wistar rats weighing 150-180 g were separated in three groups: 25 animals received CCl4 0.25 mL/kg with olive oil by gavage with 12 g/rat/day food restriction for 10 weeks (CCl4-induced cirrhosis); 12 received the same food restriction for 10 weeks (CCl4-non exposed). Other 13 rats received indomethacin 15 mg/kg by gavage as positive control of intestinal inflammation. RESULTS: The median (25-75 interquartile range) 51Cr-EDTA-IP values of cirrhotic and CCl4-non exposed rats were 0.90 percent (0.63-1.79) and 0.90 percent (0.60-1.52) respectively, without significant difference (P = 0.65). Animals from indomethacin group showed 51Cr-EDTA-IP, median 7.3 percent (5.1-14.7), significantly higher than cirrhotic and CCl4-non exposed rats (P<0.001). CONCLUSION: This study showed the lack of difference between 51Cr-EDTA-intestinal permeability in rats with and without cirrhosis. Further studies are necessary to better clarify the relationship between intestinal permeability and cirrhosis.

CONTEXTO: A relação direta entre cirrose e alterações na barreira intestinal ainda não foi devidamente esclarecida. OBJETIVO: Verificar a permeabilidade intestinal ao 51Cr-EDTA em ratos com cirrose induzida por tetracloreto de carbono (CCl4) e controles. MÉTODO: Cinquenta ratos Wistar machos pesando 150-180 g foram separados em três grupos: 25 animais receberam CCl4 0,25 mL/kg diluído em óleo de oliva por gavagem com restrição dietética de 12 g/rato/dia por 10 semanas (grupo cirrose induzida por CCl4); 12 receberam a mesma restrição dietética por 10 semanas (grupo não exposto ao CCl4). Outros 13 ratos receberam indometacina 15 mg/kg por gavagem como controle positivo de inflamação intestinal. RESULTADOS: A mediana (intervalo interquartil 25-75) dos valores de permeabilidade intestinal ao 51Cr-EDTA dos grupos cirrose induzida por CCl4 e não exposto ao CCl4 foram 0,90 por cento (0,63-1,79) e 0,90 por cento (0,60-1,52), respectivamente, sem significância estatística (P = 0,65). Os animais do grupo indometacina apresentaram uma mediana de permeabilidade intestinal ao 51Cr-EDTA de 7,3 por cento (5,1-14,7), sendo significativamente maior do que os grupos cirrose induzida por CCl4 e não exposto ao CCl4 (P<0,001). CONCLUSÃO: Este estudo não demonstrou diferenças entre a permeabilidade intestinal ao 51Cr-EDTA em ratos com e sem cirrose. Mais estudos são necessários para melhor esclarecer a relação entre a permeabilidade intestinal e cirrose.

Terapia nutricional em crianças e adolescentes com cirrose: uma visão atual / Nutritional therapy in children and adolescents with cirrhosis: current status

A anorexia e o hipermetabolismo são aspectos clínicos importantes em crianças com cirrose. Embora muitas das complicações da cirrose sejam semelhantes àquelas encontradas em adultos, a etiologia e a história natural da progressão da doença e o tratamento clínico em pacientes pediátricos podem ser significativamente diferentes. As alterações metabólicas da doença hepática crônica agravada pela anorexia e desnutrição podem ter implicações negativas no crescimento e desenvolvimento infantil. Crianças com cirrose de evolução progressiva são frequentemente desnutridas e, no entanto, os métodos comumente empregados para avaliação nutricional têm uso limitado nestes pacientes. Mesmo que a importância do estado nutricional sobre o prognóstico destes pacientes seja clara, poucos estudos sobre a terapia nutricional nas hepatopatias da infância têm sido realizados. A avaliação nutricional em crianças com cirrose hepática deve incluir uma completa história clínica e dietética, medidas antropométricas e parâmetros laboratoriais. A recomendação nutricional na cirrose infantil pode variar de acordo com o estado nutricional, idade e quadro clínico. Como a doença hepática crônica em crianças pode impactar significativamente sobre o estado nutricional e consequentemente no crescimento e desenvolvimento, o objetivo deste artigo é revisar os aspectos clínicos e fisiopatológicos envolvidos no diagnóstico e manejo nutricional da cirrose hepática em pacientes pediátricos.

Anorexia and hypermetabolism are disorders of paramount importance in children with cirrhosis. Although many complications caused by cirrhosis in children are similar to those found in adults, the etiologic spectrum and natural history of this disease progression and its clinical management in pediatric patients may be significantly different. The metabolic changes caused by chronic liver disease aggravated by anorexia and malnutrition can affect child growth and development. Malnutrition is common in children with cirrhosis and the methods commonly used for their nutritional assessment are limited. Although the importance of the nutritional status on the prognosis of these patients is clear, there are few studies about nutritional therapy in children with cirrhosis. Nutritional assessment in children with liver cirrhosis should include full clinical and nutritional history, anthropometric measurements, and laboratory parameters. The nutritional recommendation for cirrhosis in children may vary depending on age and nutritional and clinical status. Because chronic liver disease in children may have a significant impact on nutritional status, growth, and development, the objective of this study is to review the clinical and pathophysiological aspects involved in the diagnosis and nutritional management of liver cirrhosis in children.

[Predominant respiratory symptoms in indications for prolonged esophageal pH-monitoring in children]. / Predomínio de manifestações respiratórias na indicação de pHmetria esofágica prolongada em crianças.

BACKGROUND: An increasing prevalence of extraesophageal complications of gastroesophageal reflux (GER) has been seen. Evaluation of clinical profiles of patients submitted to prolonged esophageal pH monitoring could help to identify the patients who could benefit from the early diagnosis of GER. OBJECTIVE: To evaluate the population, indications and results of esophageal pH-monitoring for the diagnosis of GER in a pediatric gastroenterology clinic. PATIENTS AND METHODS: Data from 190 children and adolescents who had esophageal pH-monitoring were analyzed. A descriptive analysis of the population was performed and the children were categorized in five groups according to the age. The equipment used was a Digitrapper MKIII (Synetics) and all data were analyzed by using a software EsopHogram 5.7. Reflux index was established for analysis. RESULTS: The most frequent indications for the exam were: asthma (26.8%), apnea/cyanosis (20%), recurrent pulmonary infections (18.4%), wheezing infant syndrome (15.8%) and chronic cough/horseness (11%). The most frequent groups of age for the respiratory symptoms were: asthma--above 5 years (92.2%); apnea/cyanosis--under 3 months (55.3%); recurrent pulmonary infections and wheezing infant syndrome--3 to 12 months (31.4% and 83.3%). Reflux index of patients with positive exam for GER was significantly higher in patients under 2 years than above 2 years of age. CONCLUSION: Respiratory symptoms were predominant among the indications for esophageal pH-monitoring in children. The knowledge of the clinical profile of the patients who had esophageal pH monitoring could help to improve the technical quality of the exam.

Predomínio de manifestações respiratórias na indicação de pHmetria esofágica prolongada em crianças / Predominant respiratory symptoms in indications for prolonged esophageal pH-monitoring in children

RACIONAL: Observa-se um aumento na prevalência de complicações extra-esofágicas do refluxo gastroesofágico. A avaliação do perfil de pacientes submetidos a pHmetria esofágica prolongada poderia auxiliar na identificação de pacientes que poderiam se beneficiar do diagnóstico precoce de refluxo gastroesofágico. OBJETIVO: Avaliar a população de demanda, indicações e resultados dos exames de pHmetria esofágica prolongada na avaliação de refluxo gastroesofágico em um Serviço de Gastroenterologia Pediátrica. PACIENTES E MÉTODOS: Analisaram-se os dados de 190 crianças e adolescentes que realizaram pHmetria esofágica. Realizou-se análise descritiva da população de estudo. As crianças foram categorizadas em cinco grupos conforme a idade. Utilizou-se o aparelho Digitrapper MKIII (Synetics) e software EsopHogram versão 5.7. O parâmetro do exame considerado para análise foi o índice de refluxo. RESULTADOS: As indicações mais freqüentes para o exame foram: asma (26,8 por cento), apnéia/cianose (20 por cento), pneumonias de repetição (18,4 por cento), síndrome do bebê chiador (15,8 por cento) e tosse crônica/rouquidão (11 por cento). Os grupos etários mais freqüentes nas indicações respiratórias foram: asma - maior de 5 anos (92,2 por cento); apnéia/cianose - menor de 3 meses (55,3 por cento); pneumonias de repetição e síndrome do bebê chiador - 3 a 12 meses (31,4 por cento e 83,3 por cento). Os índices de refluxo das crianças com pHmetria positiva para refluxo gastroesofágico foram significativamente mais elevados nas crianças menores de 2 anos, em relação às maiores de 2 anos. CONCLUSÃO: Houve predomínio de manifestações respiratórias nas indicações de pHmetria esofágica. O conhecimento do perfil de pacientes com indicação de pHmetria esofágica pode contribuir para a melhoria da qualidade técnica do exame.

Report of two cases of children with Budd-Chiari Syndrome successfully treated with streptokinase

Two children with Budd-Chiari syndrome were successfully submitted to thrombolytic therapy. This study suggests that streptokinase is safe and effective in the treatment of this syndrome and should be considered as primary treatment in case of early diagnosedacute disease in view of the poor prognosis and the agressiveness of surgical treatment currently available.

Aspectos fisiopatológicos do refluxo gastroesofágico na criança / Physiopathologic aspects of gastroesophageal reflux in infancy

O refluxo gastroesofágico na infância constitui-se num problema freqüente dentro do meio pediátrico. Em revisao de literatura, sao apresentados e discutidos alguns aspectos fisiopatológicos envolvidos na gênese do refluxo gastroesofágico na criança, dentre eles: os que favorecem os episódios de refluxo com maior freqüência, os que interferem no mecanismo de "clearance" esofágico, os que possibilitam o refluxo ser mais agressivo ou os que facilitam aspiraçao para as vias aéreas superiores. Foram apresentadas algumas questoes: o refluxo gastroesofágico na criança é diferente do adulto; a gênese é multifatorial e o refluxo gastroesofágico faz parte de um distúrbio da motilidade do trato gastrointestinal como um todo.

Glicogenose hepática na infância: achados clínicos e laboratoriais em 20 pacientes / Hepatic glycogenosis in childhood: clinical and laboratory findings in 20 patients

Foram estudadas 20 crianças com quadro clínico e exames laboratoriais de glicogenose hepática. Catorze eram do sexo masculino e seis do feminino. Em relaçao à idade de início dos sintomas, notou-se variaçao desde o nascimento até 24 meses e em relaçao à idade do diganóstico, a variaçao foi de dois a 81 meses. A hepatomegalia foi encontrada em todos os pacientes, a diarréia em 65 por cento deles (13/20) e crises convulsivas em 50 por cento (10/20). A avaliaçao nutricional mostrou maior comprometimento da estatura que do peso. Nos achados laboratoriais destacaram-se a elevaçao das transaminases hepáticas (12/19), a hipercolesterolemia (8/14), a hiperuricemia (6/17) e a hipoglicemia (6/20). A funçao hepática nao estava comprometida na maioria dos casos. No teste do glucagon a resposta foi variável. O estudo histoenzimológico, realizado em 15 pacientes, mostrou os seguintes achados: Tipo VI (déficit de fosforilase) em sete pacientes, Tipo I (déficit de glicose-6-fosfatase) em dois pacientes, Tipo IV (déficit da enzima ramificadora) em um paciente e em cinco pacientes o estudo nao permitiu a classificaçao. O achado de hipoglicemia em poucos pacientes pode ser explicado pelo pequeno número de glicogenose Tipo I neste estudo, provavelmente devido ao fato de que este tipo é o de diagnóstico mais fácil, com menor necessidade de encaminhamento a serviços especializados.