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OBJECTIVE: Translating and cross-culturally adapting the CFAbd-Score, Cystic Fibrosis (CF) Abdominal Score, to use in Brazilian spoken Portuguese. The CFAbd-Score is a questionnaire for assessing CF-related abdominal symptoms and their influence on the quality of life (QoL). It comprises 28 questions on five domains: abdominal pain, bowel movements, eating and appetite, gastroesophageal reflux symptoms, and the impact of gastrointestinal (GI) symptoms on QoL. METHOD: Cross-cultural adaptation included assessment of conceptual and item equivalence, semantic, operational, and measurement equivalence. Content validity was assessed. The validation and psychometric analysis phase included 97 people with CF (pwCF), median age:14.58y (IQR 9/19), and 105 healthy individuals, 15.10y (IQR 9/20). Exploratory factor analysis (FA) identified retained factors. Internal consistency of the extracted domains was evaluated using Cronbach's α, and the Kaiser-Meyer-Olkin test (KMO) was used to check the sample adequacy. Bartlett's test tested the null hypothesis that the correlation matrix is an identity matrix. RESULTS: All items were considered relevant to the construct and good semantic equivalence of the version was recognized. FA showed the appropriate weight of all items and good internal consistency, with Cronbach's alpha 0.89. Bartlett's test significance level (p < 0.001) and KMO coefficient of 0.72 indicated good adequacy for structure. Internal consistency coefficients (Cronbach's alpha) were good for abdominal pain: 0.84; abdominal bloating: 0.73; flatulence: 0.76; heartburn: 0.81, and low for reflux: 0.54. CONCLUSION: The CFAbd-Score was adapted to the Brazilian spoken Portuguese and demonstrated content and semantic equivalence. The final version showed appropriate validity, and internal consistency, preserving the psychometric properties of the original version.

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BACKGROUND: Children who experience alkaline injury are at risk for the development of esophageal strictures and the need for esophageal dilations. OBJECTIVE: We aimed to assess predictors for a higher number of esophageal dilatations in children following alkali ingestion. METHODS: Single-center retrospective cohort study including children who underwent esophagogastroduodenoscopy (EGD) after alkali ingestion. Possible predictive factors for the need for esophageal dilatations were evaluated. RESULTS: A total of 34 patients were included, and 19 were female (55.9%). The median age at the time of the accidents was 20.6 months (IQR 15-30.7). All alkali ingestions were accidental, in all cases involving liquid products, and most (24/34; 70%) occurred at the child's home. Homemade liquid soap was the agent in half of the cases. The most frequently reported symptom at presentation was vomiting (22/34, 64.7%). The median follow-up time was 3.2 years (IQR 1.1-7.4). On follow-up, the median number of esophageal dilatations required for these patients was 12.5 (IQR 0-34). Among demographic factors, male gender (P=0.04), ingestion of homemade products (P<0.01), and accidents happening outside of the household environment (P=0.02) were associated with a greater number of esophageal dilations on follow-up. An endoscopic classification Zargar of 2B or higher (P=0.03), the presence of stricture at the time of the second EGD (P=0.01), and gastroesophageal reflux disease (GERD) as a late complication (P=0.01) were also associated with a greater number of esophageal dilations on long term follow-up. CONCLUSION: Beyond the endoscopic classification severity - a well-known risk factor for the strictures after alkali ingestions, we found that male gender, accidents with homemade products, and accidents occurring outside the household environment were significantly associated with a greater number of esophageal dilatations in the long-term follow-up of children following alkali ingestion.

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ABSTRACT Background: Children who experience alkaline injury are at risk for the development of esophageal strictures and the need for esophageal dilations. Objective: We aimed to assess predictors for a higher number of esophageal dilatations in children following alkali ingestion. Methods: Single-center retrospective cohort study including children who underwent esophagogastroduodenoscopy (EGD) after alkali ingestion. Possible predictive factors for the need for esophageal dilatations were evaluated. Results: A total of 34 patients were included, and 19 were female (55.9%). The median age at the time of the accidents was 20.6 months (IQR 15-30.7). All alkali ingestions were accidental, in all cases involving liquid products, and most (24/34; 70%) occurred at the child's home. Homemade liquid soap was the agent in half of the cases. The most frequently reported symptom at presentation was vomiting (22/34, 64.7%). The median follow-up time was 3.2 years (IQR 1.1-7.4). On follow-up, the median number of esophageal dilatations required for these patients was 12.5 (IQR 0-34). Among demographic factors, male gender (P=0.04), ingestion of homemade products (P<0.01), and accidents happening outside of the household environment (P=0.02) were associated with a greater number of esophageal dilations on follow-up. An endoscopic classification Zargar of 2B or higher (P=0.03), the presence of stricture at the time of the second EGD (P=0.01), and gastroesophageal reflux disease (GERD) as a late complication (P=0.01) were also associated with a greater number of esophageal dilations on long term follow-up. Conclusion: Beyond the endoscopic classification severity - a well-known risk factor for the strictures after alkali ingestions, we found that male gender, accidents with homemade products, and accidents occurring outside the household environment were significantly associated with a greater number of esophageal dilatations in the long-term follow-up of children following alkali ingestion.

RESUMO Contexto: Crianças que sofrem lesões cáusticas correm alto risco de desenvolver estenose esofágica e necessidade de dilatações esofágicas. Objetivo: Objetivamos avaliar preditores de necessidade de maior número de dilatações esofágicas em crianças, após uma ingestão cáustica. Métodos: Estudo de coorte retrospectivo de centro único incluindo crianças submetidas a esofagogastroduodenoscopia (EGD) após ingestão cáustica. Foram avaliados possíveis fatores preditivos para a necessidade de dilatações esofágicas. Resultados: Foram incluídos 34 pacientes, 19 do sexo feminino (55,9%). A idade mediana no momento dos acidentes foi de 20,6 meses (IQR 15-30,7). Todas as ingestões cáusticas foram incidentais, de substâncias líquidas, e a maioria dos acidentes (24/34; 70%) ocorreu no domicílio da criança. Em metade dos casos, a substância ingerida foi um sabão caseiro. O sintoma mais reportado na apresentação foi vômito (22/34 -64,7%). O tempo médio de acompanhamento foi de 3,2 anos (IQR 1,1- 7,4). No seguimento, o número médio de dilatações esofágicas necessárias foi de 12,5 (IQR 0-34). Entre os fatores demográficos, o sexo masculino (P=0,04), acidentes com produtos caseiros (P=<0,01) e a localização do acidente fora do ambiente domiciliar (P=0,02) foram associados a um maior número de dilatações esofágicas no seguimento. A classificação endoscópica Zargar 2B ou mais (P=0,03), a presença de estenose na segunda EGD (P=0,01) e a DRGE como complicação tardia (P=0,01) também se associaram a maior número de dilatações esofágicas no acompanhamento a longo prazo. Conclusão: Além da gravidade da classificação endoscópica - fator de risco bem conhecido para as estenoses após ingestão de cáusticos, observamos que o sexo masculino, os acidentes com produtos caseiros e os acidentes ocorridos fora do ambiente doméstico foram fatores significativamente associados a um maior número de dilatações esofágicas em acompanhamento em longo prazo de crianças após ingestão de soda cáustica.

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Severe acute liver failure (SALF) is a rare condition in children. Up to 50% of the cryptogenic causes of SALF are associated with autoimmune hepatitis (AIH). This report presents a 5-year-old girl with progressive jaundice for 10 days. Her 1999 AIH diagnostic score totaled 11 points, compatible with probable AIH. She fulfilled the SALF criteria and the King's College criteria for liver transplantation, despite treatment with corticosteroids, and underwent the transplant, but died in the immediate postoperative period due to massive bleeding. Subsequently, the liver-kidney microsome type 1 result was 1:80, increasing the AIH score to 13 points. The final diagnosis was probable AIH type 2, associated with SALF. The biopsy of the explanted liver was compatible with fulminant hepatitis. This report highlights the difficult diagnosis of AIH in SALF, limitations of the diagnostic criteria for SALF in indications for emergency transplantation, and the uncertain therapeutic response produced by corticosteroids.

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Abstract Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.

Resumo Objetivo: Avaliar os dados demográficos, o genótipo e o quadro clínico de pacientes pediátricos que apresentam síndrome da obstrução intestinal distal (DIOS) e os fatores associados à recidiva da DIOS. Métodos: Casuística de 10 pacientes (média de 13,2 anos) monitorados em um centro de referência e avaliados de forma retroativa. Os dados analisados incluíram idade, sexo, genótipo da fibrose cística, íleo meconial no nascimento, estado de hidratação, exacerbação pulmonar, colonização por Pseudomonas aeruginosa, insuficiência pancreática (IP), IMC nos episódios, manifestações clínicas da DIOS, estudos de diagnóstico por imagem realizados, manejo agudo da DIOS, terapia de manutenção e recidiva no acompanhamento. Resultados: Todos os pacientes apresentaram dois exames de cloreto no suor positivos e 09/10 também apresentaram estudo do genótipo. O genótipo mais comum identificado foi a homozigose da mutação delta F508. Em sete casos foi mencionado um histórico de íleo meconial. Todos os pacientes apresentaram insuficiência pancreática. O diagnóstico da DIOS teve como base achados clínicos e de imagem; 85% do número total de episódios foram tratados com sucesso com laxantes osmóticos orais e/ou terapia retal (enema de glicerina ou irrigação salina). A recidiva foi observada em 5 de 10 pacientes. Conclusão: Neste primeiro relatório da DIOS pediátrica na América do Sul, a presença de dois fatores de risco na ocorrência da DIOS foi universal: insuficiência pancreática e genótipo associado a doença grave. O histórico de íleo meconial no nascimento esteve presente na maioria dos pacientes, bem como no subgrupo com recidiva da DIOS. O diagnóstico dependeu principalmente do quadro clínico e do diagnóstico por imagem abdominal. As práticas de manejo de episódios variaram, provavelmente refletiram as mudanças no tratamento dessa síndrome ao longo do tempo.

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OBJECTIVE: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. METHODS: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. RESULTS: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. CONCLUSION: In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.

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Resumo Objetivo A adição de frutooligossacarídeos e galactooligossacarídeos a fórmulas infantis pode diminuir a consistência fecal e aumentar a frequência das evacuações. O objetivo do presente estudo foi determinar o efeito do galactooligossacarídeo em crianças com constipação crônica. Métodos Entre 2010 e 2012, 20 pacientes constipados (4-16 anos), atendidos numa unidade básica de saúde, completaram ensaio clínico duplo cego, placebo-controlado e de delineamento crossover. Onze pacientes receberam galactooligossacarídeo (1,7 g) por 30 dias, seguidos por 15 dias de washout, e, após, placebo (maltodextrina) por 30 dias; nove pacientes receberam placebo 30 dias, seguidos de 15 dias de washout e 30 dias de galactooligossacarídeo (1,7 g). Os desfechos primários foram frequência semanal de evacuações, esforço evacuatório e consistência fecal, classificada por escala numérica elaborada para este estudo e compilada no primeiro, 15̊ e 30̊ dias de cada período de crossover. Análise estatística foi feita por método de análise de variância (Anova) para medidas repetidas. Resultados Intensidade dos sintomas nos grupos foi semelhante no início do estudo (p = 0,45). Durante a ingestão de galactooligossacarídeo constatou-se maior frequência de evacuações, p < 0,0001, menor dificuldade evacuatória, p < 0,0001 e diminuição da consistência fecal, p = 0,0014. Efeitos colaterais não foram referidos durante a ingestão do prebiótico. Conclusão Durante a ingestão de galactooligossacarídeo os sintomas clínicos da constipação em crianças e adolescentes foram significantemente aliviados.

Abstract Objective Fructooligosacharides and galactooligosacharides soften fecal bolus and increase frequency of depositions when added to infant formula. This study aimed to determine the effects of galactooligosaccharide in pediatric patients with chronic constipation. Methods From 2010 to 2012, 20 constipated patients (4-16 years of age) attended to at a primary healthcare unit were enrolled in a double-blinded, placebo-controlled crossover trial. Eleven children ingested galactooligosaccharide (1.7 g) for 30 days, followed by a 15-day washout period, and a 30-day period of placebo (maltodextrin). Nine patients ingested maltodextrin for 30 days, followed by 15-day washout period, and galactooligosaccharide (1.7 g) for 30 days. Constipation symptoms were considered as primary outcomes: bowel movements/week, straining during defecation, and stool consistency. Outcome symptoms were ranked according to a numerical scale elaborated for this study. Data were recorded at baseline, and on days 15 and 30 of each 30-day crossover period. Repeated-measures analysis of variance (ANOVA) was used to analyze symptoms along time. Results At baseline, there was no significant difference in symptoms severity between groups (p = 0.45). Galactooligosaccharide ingestion was related to increase of the bowel movement frequency, p < 0.0001; relief of defecation straining, p < 0.0001; and decrease in stool consistency, p = 0.0014, compared to placebo ingestion. Patients reported no side effects from galactooligosaccharide. Conclusion Galactooligosaccharide was effective at improving clinical symptoms in this group of constipated children.

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OBJECTIVE: Fructooligosacharides and galactooligosacharides soften fecal bolus and increase frequency of depositions when added to infant formula. This study aimed to determine the effects of galactooligosaccharide in pediatric patients with chronic constipation. METHODS: From 2010 to 2012, 20 constipated patients (4-16 years of age) attended to at a primary healthcare unit were enrolled in a double-blinded, placebo-controlled crossover trial. Eleven children ingested galactooligosaccharide (1.7 g) for 30 days, followed by a 15-day washout period, and a 30-day period of placebo (maltodextrin). Nine patients ingested maltodextrin for 30 days, followed by 15-day washout period, and galactooligosaccharide (1.7 g) for 30 days. Constipation symptoms were considered as primary outcomes: bowel movements/week, straining during defecation, and stool consistency. Outcome symptoms were ranked according to a numerical scale elaborated for this study. Data were recorded at baseline, and on days 15 and 30 of each 30-day crossover period. Repeated-measures analysis of variance (ANOVA) was used to analyze symptoms along time. RESULTS: At baseline, there was no significant difference in symptoms severity between groups (p=0.45). Galactooligosaccharide ingestion was related to increase of the bowel movement frequency, p<0.0001; relief of defecation straining, p<0.0001; and decrease in stool consistency, p=0.0014, compared to placebo ingestion. Patients reported no side effects from galactooligosaccharide. CONCLUSION: Galactooligosaccharide was effective at improving clinical symptoms in this group of constipated children.

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OBJECTIVE: The aim of this study was to assess clinical features and colonic transit patterns in Brazilian children with refractory constipation.METHODS: From 2010 to 2013, 79 constipated patients received follow-up care in a tertiary hospital. Of these patients, 28 (aged 8-14 years) were refractory to conventional therapy and underwent a simplified visual method of nuclear colonic transit study, by ingestion of a liquid meal containing 9.25 MBq/kg of 99mTc-phytate. Abdominal static images were taken immediately and at two, six, 24, 30, and 48 h after ingestion for qualitative analysis of the radio marker progression through the colon.RESULTS: Two patterns of colonic transit were found: slow colonic transit (SCT,n = 14), when images at 48 h showed a larger part of the tracer remained in proximal and transverse colon, and distal retention (DR, n = 14), when after 30 h, the radio isotope passed the transverse colon and was retained in the rectosigmoid up to 48 h. The SCT and DR group included, respectively, nine and ten males; median ages in the nuclear study of 11 and 10 years, p = 0.207; median duration of constipation of seven and six years, p = 0.599. Constipation appearing during first year age (p = 0.04) and report of soft stools (p = 0.02) were more common in SCT patients. Palpable abdominal fecal impaction was found only in DR group. Appendicostomy for antegrade continence enema was successful in 4/12 (30%) of SCT patients (median follow-up: 2.4 years).CONCLUSION: Nuclear transit study distinguished two colonic dysmotility patterns and was useful for guiding refractory patients to specific therapies.

OBJETIVO: Avaliar as características clínicas e os padrões de trânsito intestinal em crianças brasileiras com constipação refratária.MÉTODOS: De 2010 a 2013, 79 pacientes constipados receberam acompanhamento em um hospital terciário. Desses pacientes, 28 (entre 8-14 anos) foram identificados como terapia refratária a convencional e passaram por um método visual simplificado de estudo nuclear do trânsito intestinal, com ingestão de uma refeição líquida contendo 9,25 MBq/Kg de fitato-99mTc. Imagens estáticas abdominais foram tiradas imediatamente e em duas, seis, 24, 30 e 48 horas após a ingestão para uma análise qualitativa da progressão do marcador radioativo pelo cólon.RESULTADOS: Foram encontrados dois padrões de trânsito intestinal: trânsito intestinal lento (STC, n = 14), quando as imagens de 48 horas mostraram que grande parte do marcador permaneceu no cólon proximal e transversal; e retenção distal (DR, n = 14), quando, após 30 horas, o radioisótopo havia passado o cólon transverso e estava retido no retossigmoide até 48 horas. O grupo STC e o grupo DR incluíram, respectivamente, nove e 10 meninos; idade média no momento do NTS: 11 e 10 anos, p = 0,207; duração média de constipação: sete e seis anos, p = 0,599. Sintomas de constipação durante o primeiro ano de idade (p = 0,04) e relatos de fezes moles (p = 0,02) foram mais comuns em pacientes com STC. Observou-se impactação fecal abdominal palpável apenas no grupo DR. A apendicostomia para enema anterógrado para continência foi bem-sucedida em 4/12 (305) pacientes com STC (acompanhamento médio: 2,4 anos).CONCLUSÃO: O estudo nuclear do trânsito diferenciou dois padrões de dismotilidade intestinal e foi útil para orientar pacientes refratários a terapias específicas.

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OBJECTIVE: The aim of this study was to assess clinical features and colonic transit patterns in Brazilian children with refractory constipation. METHODS: From 2010 to 2013, 79 constipated patients received follow-up care in a tertiary hospital. Of these patients, 28 (aged 8-14 years) were refractory to conventional therapy and underwent a simplified visual method of nuclear colonic transit study, by ingestion of a liquid meal containing 9.25 MBq/kg of (99m)Tc-phytate. Abdominal static images were taken immediately and at two, six, 24, 30, and 48h after ingestion for qualitative analysis of the radio marker progression through the colon. RESULTS: Two patterns of colonic transit were found: slow colonic transit (SCT, n=14), when images at 48h showed a larger part of the tracer remained in proximal and transverse colon, and distal retention (DR, n=14), when after 30h, the radio isotope passed the transverse colon and was retained in the rectosigmoid up to 48h. The SCT and DR group included, respectively, nine and ten males; median ages in the nuclear study of 11 and 10 years, p=0.207; median duration of constipation of seven and six years, p=0.599. Constipation appearing during first year age (p=0.04) and report of soft stools (p=0.02) were more common in SCT patients. Palpable abdominal fecal impaction was found only in DR group. Appendicostomy for antegrade continence enema was successful in 4/12 (30%) of SCT patients (median follow-up: 2.4 years). CONCLUSION: Nuclear transit study distinguished two colonic dysmotility patterns and was useful for guiding refractory patients to specific therapies.

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