Is neurodevelopment impaired in Brazilian children with intestinal failure on prolonged parenteral nutrition? A single center study.

PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.

Validação de um questionário de avaliação nutricional subjetiva global / Validation of a subjective global assessment questionnaire

Resumo Objetivo Validar o questionário de Avaliação Nutricional Subjetiva Global (ANSG) para a população de crianças e adolescentes brasileiros. Métodos Estudo transversal, feito com 242 pacientes, de 30 dias a 13 anos, atendidos em unidades pediátricas de um hospital terciário, com doenças agudas e tempo de permanência mínima de 24 horas hospitalizados. Após autorização dos autores do estudo original foram cumpridas as seguintes etapas para obtenção da validação dos instrumentos de ANSG: tradução (backtranslation), validade de critério concorrente e preditiva e confiabilidade interobservador. As variáveis em estudo foram: idade, sexo, peso e comprimento ao nascer, prematuridade e antropometria (peso, estatura, índice de massa corporal, circunferência braquial, dobra cutânea tricipital e dobra cutânea subescapular). O desfecho principal considerado foi necessidade de internação/reinternação até 30 dias após a alta hospitalar. Os testes estatísticos usados foram: Anova, Kruskal-Wallis, Mann-Whitney, qui-quadrado e coeficiente Kappa. Resultados De acordo com a classificação do ANSG, 80% dos pacientes foram classificados como bem nutridos, 14,5% moderadamente desnutridos e 5,4% gravemente desnutridos. A validade concorrente mostrou fraca a regular correlação do ANSG com as medidas antropométricas usadas (p < 0,001). Quanto ao poder preditivo, o desfecho principal associado ao ANSG foi tempo de internação/reinternação. Os desfechos secundários associados foram: tempo de permanência na unidade após ANSG, peso e comprimento ao nascer e prematuridade (p < 0,05). A confiabilidade interobservador mostrou boa concordância entre os avaliadores (Kappa = 0,74). Conclusão Este estudo validou o método de ANSG nessa amostra de pacientes pediátricos hospitalizados e possibilitou seu uso para fins de aplicação clínica e de pesquisa na população brasileira.

Abstract 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 24 h. 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.

Changing pattern of indications of endoscopic retrograde cholangiopancreatography in children and adolescents: a twelve-year experience.

BACKGROUND: There are few data regarding endoscopic retrograde cholangiopancreatography (ERCP) usefulness in children and adolescents. We reviewed the long-term experience with diagnostic and therapeutic ERCP in a tertiary single center in Southern Brazil. METHODS: A retrospective chart review of patients aged 0-18 years who had undergone ERCPs from January 2000 to June 2012 was done. Data on demographics, indications, diagnosis, treatments, and complications were collected. RESULTS: Seventy-five ERCPs were performed in 60 patients. The median age of the patients at the procedure was 13.9 years (range: 1.2-17.9). Of the 60 patients, 47 (78.3%) were girls. Of all ERCPs, 48 (64.0%) were performed in patients above 10 years and 35 (72.9%) of them were in girls. ERCP was indicated for patients with bile duct obstruction (49.3%), sclerosing cholangitis (18.7%), post-surgery complication (12%), biliary stent (10.7%), choledochal cyst (5.3%), and pancreatitis (4%). The complication rate of ERCP was 9.7% involving mild bleeding, pancreatitis and cholangitis. Patients who had therapeutic procedures were older (13.7±3.9 vs. 9.9±4.9 years; P=0.001) and had more extrahepatic biliary abnormalities (82% vs. 50%; P=0.015) than those who had diagnostic ERCPs. A marked change in the indications of ERCPs was found, i.e., from 2001 to 2004, indications were more diagnostic and from 2005 therapeutic procedures were predominant. CONCLUSIONS: Diagnostic ERCPs are being replaced by magnetic resonance cholangiopancreatography and also by endoscopic ultrasound. All these procedures are complementary and ERCP still has a role for therapeutic purposes.

Gastric yield pressure and gastric yield volume to assess anti-reflux barrier in a porcine model.

Anti-reflux barrier (ARB) resistance may be useful to test new treatments for gastroesophageal reflux (GER). The ARB has been estimated by increasing gastric yield pressure (GYP) and gastric yield volume (GYV) in animal models but has not been validated. This study aimed to develop an experimental model suitable for assessing the ARB resistance to increasing intragastric pressure and volume and its reproducibility in a seven-day interval. Ten two-month-old female Large-White swine were studied. Intragastric pressure and volume were recorded using a digital system connected to a Foley catheter inserted through gastrostomy into the stomach. GYP and GYV were defined as the gastric pressure and volume able to yield gastric contents into the esophagus detected by esophageal pH. A sudden pH drop below 3 sustained during 5 min was considered diagnostic for gastric yield. Animals were studied again after seven days. On days 0 and 7, there were no significant differences for GYP (mean ± SD = 7.66 ± 3.02 mmHg vs. 7.07 ± 3.54 mmHg, p = .686) and GYV (636.70 ± 216.74 ml vs. 608.30 ± 276.66 ml; p = .299), respectively. Concordance correlation coefficient (ρc) was significant for GYP (ρc = 0.634, 95% CI = 0.141-0.829, p = .006), but not for GYV (ρc = 0.291, 95% CI = -0.118 to 0.774, p = .196). This study demonstrated an experimental model, assessing the ARB resistance. GYP seems to be a more reliable parameter than GYV for assessment of ARB resistance.

Ghrelin, leptin and insulin in cirrhotic children and adolescents: relationship with cirrhosis severity and nutritional status.

OBJECTIVE: Ghrelin, leptin, and insulin concentrations are involved in the control of food intake and they seem to be associated with anorexia-cachexia in cirrhotic patients. The present study aimed to investigate the relationship between the nutritional status and fasting ghrelin, leptin and insulin concentrations in pediatric cirrhotic patients. METHODS: Thirty-nine patients with cirrhosis and 39 healthy controls aged 0-15 years matched by sex and age were enrolled. Severity of liver disease was assessed by Child-Pugh classification, and Pediatric for End Stage Liver Disease (PELD) or Model for End-stage Liver Disease (MELD) scores. Blood samples were collected from patients and controls to assay total ghrelin, acyl ghrelin, leptin and insulin by using a commercial ELISA kit. Anthropometry parameters used were standard deviation score of height-for-age and triceps skinfold thickness-for-age ratio. A multiple linear regression analysis was used to determine the correlation between dependent and independent variables. RESULTS: Acyl ghrelin was significantly lower in cirrhotic patients than in controls [142 (93-278) pg/mL vs 275 (208-481) pg/mL, P=0.001]. After multiple linear regression analysis, total ghrelin and acyl ghrelin showed an inverse correlation with age; acyl ghrelin was associated with the severity of cirrhosis and des-acyl ghrelin with PELD or MELD scores ≥15. Leptin was positively correlated with gender and anthropometric parameters. Insulin was not associated with any variable. CONCLUSION: Low acyl ghrelin and high des-acyl ghrelin concentrations were associated with cirrhosis severity, whereas low leptin concentration was associated with undernourishment in children and adolescents with cirrhosis.

Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience.

PURPOSE: Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period. METHODS: Patients with achalasia were identified from a database built during the period of January 2000-January 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described. RESULTS: Thirteen patients were studied; median age was 7 (1-14) years. Most frequent symptoms were vomiting (84.6 %) and dysphagia (69.2 %). Weight loss occurred in 46.0 % of patients and chronic cough in 46.1 %. Associated disorders were Down's syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up. CONCLUSION: Achalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.

Refluxo gastroesofágico em crianças com asma persistente: uma coexistência silenciosa? / Gastroesophageal reflux and persistent asthma in children: is there a silent coexistence?

Objetivo: Avaliar a prevalência de refluxo gastroesofágico (RGE) em uma amostra de pacientes pediátricos com asma persistente, atendidos em serviço ambulatorial de Pneumologia Pediátrica, comparar as características clínicas do RGE entre os pacientes com e sem RGE detectável por meio da pHmetria intra-esofágica prolongada. Métodos: Foi conduzido um estudo transversal observacional com pacientes de idades entre 5 e 18 anos e com diagnóstico clínico de asma persistente, de acordo com as definições estabelecidas no III Consenso Brasileiro no Manejo da Asma. O diagnóstico de RGE foi definido pelo estudo da pHmetria intra-esofágica prolongada (pHmetria), considerado positivo quando o valor do índice de refluxo apresentou percentual maior ou igual a 5%. Resultados: A amostra foi totalizada em 38 pacientes. A média da idade dos pacientes foi de 9,5 anos e 23 desses pertenciam ao sexo masculino (60%). A prevalência de pHmetrias alteradas foi de 47,3%. A ocorrência de sintomas atribuíveis ao RGE não demonstrou diferenças entre os pacientes com pHmetrias alteradas e normais. Conclusões: A prevalência de RGE nesta amostra de pacientes foi semelhante às relatadas na literatura. Os sintomas atribuíveis ao RGE não foram úteis para a diferenciação dos pacientes com pHmetrias positivas ou negativas.

Objective: To evaluate the prevalence of gastroesophageal reflux (GER) in a sample of patients within the age of 5 – 18 years with persistent asthma assisted in a Pediatric Pulmonary Department; to compare the clinical features of GER among patients with or without GER diagnosed by a distal esophageal pH probe study Methods: Patients with persistent asthma diagnosed by criteria of III Consenso Brasileiro no Manejo da Asma and aged 5-18 years were allocated in a cross-sectional observational study. The diagnosis of GER was defined with a reflux index of 5% or higher of acid reflux by means of a distal esophageal pH probe Results: Thirty-eight patients were evaluated, mean age was 9.5 years and 60% were male. The prevalence of positive pHmetry examinations was 47.3%. The occurrence of GER symptoms has not presented significant differences between patients with or without GER diagnosed by pHmetry. Conclusions: In this study, clinical symptoms of GER have not shown usefulness to define the presence of GER in these patients. The prevalence of GER in this sample was similar to those related in literature.

Acalasia na doença de Chagas é diferente de acalasia idiopática? Experiência do Hospital de Clínicas de Porto Alegre / Is achalasia in Chagas'disease different from idiopathic achalasia? Experience of the Hospital de Clínicas de Porto Alegre

O presente trabalho tem como objetivo avaliar as diferenças entre a acalasia chagásica e a idiopática em pacientes do Hospital de Clínicas de Porto Alegre, através da análise de achados epidemiológicos, clínicos, radiológicos e manométricos. Foram estudados pacientes encaminhados ao Hospital de Clínicas de Porto Alegre, entre novembro de 1996 e dezembro de 2001, com suspeita de acalasia, posteriormente, confirmada por manometria esofágica. Além das características manométricas e radiológicas, os pacientes foram avaliados quanto a idade, sexo, sintomas e tempo de evolução. Entre 51 pacientes, nove (18 por cento) tiveram sorologia positiva para doença de Chagas e 42 (82 por cento) sorologia negativa. Indivíduos com sorologia negativa foram considerados portadores de acalasia idiopática. Pacientes com acalasia chagásica tinham média de idade de 62 mais ou menos 15 anos e os com idiopática 43 mais ou menos 18 anos (P<0,02). O período de evolução dos sintomas em pacientes com acalasia chagásica foi de 74 mais ou menos 47 meses e nos idiopáticos 49 mais ou menos 35 meses (P<0,05). Disfagia, regurgitação, dor torácica e emagrecimento, valores do esfíncter esofágico inferior (pressão basal, pressão e duração de relaxamento pós-deglutição e comprimento total) e do corpo esofágico (amplitude e duração das ondas pós-deglutição) foram similares em ambos os grupos). As únicas diferenças estatisticamente significativas encontradas entre os dois grupos foram a média de idade e o período de evolução dos sintomas, maiores nos pacientes chagásicos. Esses dados permitem especular sobre uma maior tolerância aos sintomas nos pacientes com idade mais avançada.

Colestase neonatal e infecçäo por citomegalovírus: formas de apresentaçäo clínica e histopatológica / Neonatal cholestasis and cytomegalovirus infection: clinical and histopathologic forms

A infecçäo pelo citomegalovírus é sintomática em apenas 10 por cento dos casos. Os achados mais freqüentes säo a colestase e a hepatoesplenomegalia. Foram estudados 10 pacientes que apresentavam quadro de colestase neonatal associada à infecçäo pelo citomegalovírus. A maioria apresentava aumento de aminotransferases e alteraçöes discretas na funçäo hepática. Os achados histopatológicos foram de normalidade, hepatite de células gigantes, proliferaçäo de ductos biliares (confundindo com a atresia de vias biliares extra-hepáticas) e ductopenia. A evoluçäo dos pacientes foi variável, desde desaparecimento dos sintomas (2 pacientes) até óbito (2 pacientes). Pela possibilidade do quadro histopatológico se confundir com o da atresia de vias biliares extra-hepáticas, é necessário que a investigaçäo da causa da colestase neonatal, incluindo a pesquisa para infecçäo pelo citomegalovírus, seja realizada o mais rápido possível.