Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia.

BACKGROUND: Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis. AIMS: To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia. METHODS: All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninety-day mortality was also evaluated. Prognostic factors related to both endpoints also were studied. RESULTS: One-year patient survival for NA was 97.1%, versus 80.8% for A1, versus 52% for A2, versus 13.6 for A3 (p < 0.001). The presence of ascites increased mortality by 17 times. In the multivariate analysis, clinically detectable ascites (HR 3.14, 95% CI 1.14-8.60, p = 0.026), lower sodium (HR 1.15, 95% CI 1.04-1.27, p = 0.006), higher bilirubin (HR 1.06, 95% CI 1.00-1.12, p = 0.023), and higher PELD score (HR 1.05, 95% CI 1.02-1.08, p = 0.001) were all associated with decreased survival. Lower serum sodium (HR 1.20, 95% CI 1.09-1.32, p < 0.001) and higher PELD score (HR 1.03, 95% CI 1.001-1.063, p = 0.043) were associated with increased 90-day mortality. CONCLUSIONS: Clinically detectable ascites is associated with decreased 1-year survival of children with biliary atresia. These patients should be treated with caution and prioritized for liver transplantation.

The first episode of spontaneous bacterial peritonitis is a threat event in children with end-stage liver disease.

OBJECTIVE: Studies on native liver survival (NLS) after the first episode of spontaneous bacterial peritonitis (SBP) are rare. Our objective was to evaluate NLS in children up to 1 year after SBP. METHODS: A historical cohort study of 18 children followed after the first episode of SBP was conducted. NLS, in-hospital mortality, causes of death, and rate of multidrug-resistant organisms were reported. RESULTS: Biliary atresia was the most prevalent diagnosis (72.2%); all were Child-Pugh C, and the median age was 1.0 year. The probability of NLS was 77.8, 27.8, and 11.1% at 1, 3 and 6 months, respectively. At 9 months, no child had the native liver. In-hospital mortality was 38.9%, and the main causes of death were septic shock and acute-on-chronic liver failure. Escherichia coli was the predominant organism cultured. Multidrug-resistant organisms were not detected. The cumulative probability of NLS was 77.8% at 1 month, 27.8% at 3 months, and 11.1% at 6 months. At 9-month follow-up, none of children had their native liver. Ascites PMN count cell more than 1000 cells/mm, positive ascites culture, and prolonged international normalized ratio reached a significant value as predictive factors of NLS and were selected for multivariate analysis. We did not identify independent predictors of survival. CONCLUSION: Development of SBP was a late event in children and had a high effect on NLS.

Glycogen storage disease type I: clinical and laboratory profile / Doença de depósito de glicogênio tipo I: perfil clínico e laboratorial

OBJECTIVES: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. METHODS: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. RESULTS: Twenty-one patients were included (median age 10 years, range 1-25 years), all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1-132 months), and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008). CONCLUSIONS: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients. .

OBJETIVOS: Caracterizar o perfil clínico, laboratorial e antropométrico de uma amostra de pacientes brasileiros com doença de depósito de glicogênio tipo I tratados em um ambulatório de referência para erros inatos do metabolismo. MÉTODOS: Este foi um estudo ambulatorial transversal com base em uma estratégia de amostragem de conveniência. Foram avaliados os dados com relação ao diagnóstico, tratamento, parâmetros antropométricos e acompanhamento. RESULTADOS: Foram incluídos 21 pacientes (idade média de 10 anos, faixa 1-25 anos de idade), e todos se encontravam em terapia de amido de milho cru. A idade média na época do diagnóstico foi de sete meses (faixa, 1-32 meses), e 19 pacientes foram submetidos a biópsia hepática para confirmação do diagnóstico. Sobrepeso, baixa estatura, hepatomegalia e nódulos hepáticos foram fatores presentes em 16 de 21, quatro de 21, nove de 14 e três de 14 pacientes, respectivamente. Foi encontrada uma correlação entre os escores z para peso para idade e IMC para idade (r = 0,561; p = 0,008). CONCLUSÕES: O diagnóstico da doença de depósito de glicogênio tipo I tem sido tardio no Brasil. A maioria dos pacientes foi submetida a confirmação do diagnóstico, apesar de o quadro clínico característico e os métodos moleculares poderem fornecer um diagnóstico definitivo de forma menos invasiva. Obesidade é um efeito colateral da terapia com amido de milho e parece estar associada a crescimento nesses pacientes. .

Glycogen storage disease type I: clinical and laboratory profile.

OBJECTIVES: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. METHODS: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. RESULTS: Twenty-one patients were included (median age 10 years, range 1-25 years), all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1-132 months), and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r=0.561; p=0.008). CONCLUSIONS: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients.

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.

Use of monoclonal faecal elastase-1 concentration for pancreatic status assessment in cystic fibrosis patients.

OBJECTIVE: To assess the concentration of faecal elastase-1 (EL-1) in pediatric patients with cystic fibrosis with mutation DeltaF508. METHODS: Cross-sectional study with samples collected consecutively from 51 patients aged 4 months to 17 years old (mean 9.11±4.74); 32 (62.8%) patients were male. Clinical-demographic data were collected, as well as data on the type of mutation. Exocrine pancreatic insufficiency was established by the activity of faecal EL-1 < 200 µg/g. EL-1 was quantified through the monoclonal ELISA method (ScheBo Biotech AG, Germany). Pancreatic supplements were used in 46 (90.2%) patients. RESULTS: Forty-one (80.4%) patients presented with pancreatic insufficiency (EL-1 fecal < 100 µg/g): 17 (41.5%) were homozygous, 14 were heterozygous (34.1%) and 10 were non-DeltaF508 (24.4%). Regarding the mutation, there was a statistically significant association of homozygosity with faecal EL-1 concentration < 100 µg/g (p = 0.010). All patients considered to be pancreatic insufficient (n = 41) by the test were using pancreatic supplements. Ten (19.6%) presented faecal EL-1 > 200 µg/g, and 5/10 (50%) used enzymes. CONCLUSIONS: The activity of faecal EL-1 < 100 µg/g, indicating pancreatic insufficiency, was observed in 17/17 (100%) of homozygous patients, as expected, and was less frequent in patients who were heterozygous for DeltaF508 and in patients without the mutation. There was no association of faecal EL-1 concentration with age and sex of patients. The test was standardized, is easy to execute, and can be used to assess the pancreatic status of patients with cystic fibrosis.

Utilidade da concentração da elastase-1 fecal monoclonal na avaliação da função pancreática nos pacientes com fibrose cística / Use of monoclonal faecal elastase-1 concentration for pancreatic status assessment in cystic fibrosis patients

OBJETIVO: Avaliar a concentração da elastase-1 (EL-1) fecal em pacientes pediátricos com fibrose cística, portadores da mutação ∆F508. MÉTODOS: Estudo transversal com amostras colhidas consecutivamente de 51 pacientes com idade entre 4 meses e 17 anos (média 9,11±4,74), sendo 32 (62,8 por cento) pacientes do sexo masculino. Houve coleta de dados clínico-demográficos e do tipo de mutação. A insuficiência pancreática exócrina foi definida pela atividade da EL-1 fecal < 200 µg/g. A quantificação da EL-1 foi realizada pelo método ELISA monoclonal (ScheBo Biotech AG, Germany). A suplementação pancreática foi utilizada em 46 (90,2 por cento) pacientes. RESULTADOS: Quarenta e um (80,4 por cento) pacientes apresentaram insuficiência pancreática (EL-1 fecal < 100 µg/g), sendo 17 (41,5 por cento) homozigotos, 14 heterozigotos (34,1 por cento) e 10 sem ∆F508 (24,4 por cento). Ao considerar a mutação, houve associação estatisticamente significativa entre os homozigotos e a concentração da EL-1 fecal < 100 µg/g (p = 0,010). Todos os pacientes considerados insuficientes pancreáticos (n = 41) pelo teste utilizavam suplemento pancreático. Dez (19,6 por cento) apresentaram EL-1 fecal > 200 µg/g, e 5/10 (50 por cento) utilizavam enzimas. CONCLUSÕES: A atividade de EL-1 fecal < 100 µg/g, indicativa de insuficiência pancreática, apresentou-se em 17/17 (100 por cento) dos homozigotos, conforme o esperado, sendo menos frequente nos heterozigotos para ∆F508 e nos pacientes com ausência dessa mutação. Não houve relação entre a concentração da EL-1 fecal com idade e sexo dos pacientes. O teste foi padronizado, é de fácil execução e poderá ser utilizado para avaliação da função pancreática dos pacientes com fibrose cística.

OBJECTIVE: To assess the concentration of faecal elastase-1 (EL-1) in pediatric patients with cystic fibrosis with mutation ∆F508. METHODS: Cross-sectional study with samples collected consecutively from 51 patients aged 4 months to 17 years old (mean 9.11±4.74); 32 (62.8 percent) patients were male. Clinical-demographic data were collected, as well as data on the type of mutation. Exocrine pancreatic insufficiency was established by the activity of faecal EL-1 < 200 µg/g. EL-1 was quantified through the monoclonal ELISA method (ScheBo Biotech AG, Germany). Pancreatic supplements were used in 46 (90.2 percent) patients. RESULTS: Forty-one (80.4 percent) patients presented with pancreatic insufficiency (EL-1 fecal < 100 µg/g): 17 (41.5 percent) were homozygous, 14 were heterozygous (34.1 percent) and 10 were non-∆F508 (24.4 percent). Regarding the mutation, there was a statistically significant association of homozygosity with faecal EL-1 concentration < 100 µg/g (p = 0.010). All patients considered to be pancreatic insufficient (n = 41) by the test were using pancreatic supplements. Ten (19.6 percent) presented faecal EL-1 > 200 µg/g, and 5/10 (50 percent) used enzymes. CONCLUSIONS: The activity of faecal EL-1 < 100 µg/g, indicating pancreatic insufficiency, was observed in 17/17 (100 percent) of homozygous patients, as expected, and was less frequent in patients who were heterozygous for ∆F508 and in patients without the mutation. There was no association of faecal EL-1 concentration with age and sex of patients. The test was standardized, is easy to execute, and can be used to assess the pancreatic status of patients with cystic fibrosis.

Stress ulcer prophylaxis in pediatric intensive care units.

OBJECTIVE: To assess use of stress ulcer prophylaxis in patients admitted to five pediatric intensive care units (PICUs) in Porto Alegre, Brazil. METHODS: This was a multicenter, prospective, cross-sectional observational study. PICUs were visited on randomly defined days between April 2006 and February 2007, and the medical records of admitted patients were reviewed. Patients whose records had been previously assessed were excluded, as were those with upper gastrointestinal bleeding on admission. Data were collected on age, gender, admission diagnosis, severity of illness, administration of stress ulcer prophylaxis, rationale for prophylaxis, and first-line prophylactic agent of choice. Variables were described as absolute and relative frequencies, mean and standard deviation, or median and interquartile range as appropriate. Pearson's chi-square test for linear trend or Fisher's exact test were used to assess possible associations. The level of significance was set at 5% (p ≤ 0.05). RESULTS: 398 patients (57% male) were assessed [median age, 16 months (IQR 4-65); median length of PICU stay, 4 days (IQR 1-9)]. Respiratory illness was the main reason for admission (32.7%). Most patients received stress ulcer prophylaxis (77.5%; range, 66-91%). Mechanical ventilation (22.3%) was the most common rationale provided, followed by informal routine use of prophylaxis (21.4%). Only one of the participating PICUs had a specific care protocol for use of stress ulcer prophylaxis. Ranitidine was the most commonly used drug (84.5% of cases). Evidence of minor gastrointestinal bleeding was found in 3% of patients; none had clinically significant bleeds. CONCLUSIONS: Administration of stress ulcer prophylaxis is a common practice in the participating PICUs, with ranitidine the most commonly used drug. Among the various rationales provided, mechanical ventilation and informal routine use were the most prevalent.

Profilaxia para úlcera de estresse em pacientes internados em UTI pediátrica / Stress ulcer prophylaxis in pediatric intensive care units

OBJETIVO: Avaliar a utilização de profilaxia para úlcera de estresse (UE), em pacientes internados, de cinco unidades de terapia intensiva pediátrica (UTIP) de Porto Alegre (RS). MÉTODOS: Estudo multicêntrico, prospectivo, transversal, observacional. Foram avaliados os prontuários dos pacientes internados em dia definido para visitação, entre abril de 2006 e fevereiro de 2007, excluindo os avaliados em visitas anteriores e aqueles com hemorragia digestiva alta na admissão. Foram avaliados a idade, o gênero, o diagnóstico na admissão, a gravidade da doença, o uso de profilaxia para UE, a sua justificativa e o medicamento profilático utilizado como primeira escolha. As variáveis foram descritas como frequências absoluta e relativa, ou média e desvio padrão/mediana, e intervalo interquartil (IQ). Os testes qui-quadrado de Pearson, de tendência linear, ou exato de Fisher foram utilizados para avaliar as associações. O nível de significância adotado foi de 5 por cento, sendo estatisticamente significativo p < 0,05. RESULTADOS: Foram avaliados 398 pacientes, sendo 57 por cento do gênero masculino. A mediana de idade foi de 16 meses (IQ4-65) e mediana de permanência em UTIP foi de 4 dias (IQ1-9). O principal motivo de internação foi doença respiratória (32,7 por cento). Usaram profilaxia 77,5 por cento dos pacientes, variando de 66 a 91 por cento; a ventilação mecânica (22,3 por cento) foi a justificativa mais prevalente, seguida de rotina informal do serviço (21,4 por cento). Apenas uma das UTIP tinha protocolo assistencial para profilaxia de UE. A ranitidina foi o medicamento mais empregado (84,5 por cento). CONCLUSÕES: O uso de profilaxia para UE foi prática frequente nas UTIP avaliadas, sendo a ranitidina a droga de escolha. Entre as justificativas, a ventilação mecânica e o uso baseado em rotinas institucionais foram as mais prevalentes.

OBJECTIVE: To assess use of stress ulcer prophylaxis in patients admitted to five pediatric intensive care units (PICUs) in Porto Alegre, Brazil. METHODS: This was a multicenter, prospective, cross-sectional observational study. PICUs were visited on randomly defined days between April 2006 and February 2007, and the medical records of admitted patients were reviewed. Patients whose records had been previously assessed were excluded, as were those with upper gastrointestinal bleeding on admission. Data were collected on age, gender, admission diagnosis, severity of illness, administration of stress ulcer prophylaxis, rationale for prophylaxis, and first-line prophylactic agent of choice. Variables were described as absolute and relative frequencies, mean and standard deviation, or median and interquartile range as appropriate. Pearson's chi-square test for linear trend or Fisher's exact test were used to assess possible associations. The level of significance was set at 5 percent (p < 0.05). RESULTS: 398 patients (57 percent male) were assessed [median age, 16 months (IQR 4-65); median length of PICU stay, 4 days (IQR 1-9)]. Respiratory illness was the main reason for admission (32.7 percent). Most patients received stress ulcer prophylaxis (77.5 percent; range, 66-91 percent). Mechanical ventilation (22.3 percent) was the most common rationale provided, followed by informal routine use of prophylaxis (21.4 percent). Only one of the participating PICUs had a specific care protocol for use of stress ulcer prophylaxis. Ranitidine was the most commonly used drug (84.5 percent of cases). Evidence of minor gastrointestinal bleeding was found in 3 percent of patients; none had clinically significant bleeds. CONCLUSIONS: Administration of stress ulcer prophylaxis is a common practice in the participating PICUs, with ranitidine the most commonly used drug. Among the various rationales provided, mechanical ventilation and informal routine use were the most prevalent.

Biliary atresia: we still operate too late.

OBJECTIVE: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. METHODS: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. RESULTS: Of the 112 cases of children with biliary atresia studied, 38 (33.9%) occurred between 1982 and 1989, 46 (41.1%) between 1990 and 1999 and 28 (25.0%) after 2000. Portoenterostomy was not performed for 12 cases (10.7%). Age at surgery ranged from 25 to 297 days (median: 80.5; IQR25-75: 61.3-109.0 days); for 20.5% of cases, the age was below 60 days. There was no age difference at diagnosis for the three decades in the study. Patients from the countryside (median: 87.0; IQR25-75: 69.0-115.0 days) were referred significantly later (p = 0.007) than those living in Porto Alegre and the metropolitan area (median: 68.0; IQR25-75: 55.5-98.0 days). The ratio of patients younger than 60 days was significantly lower (p = 0.013) for those from the countryside. Survival periods with native liver for all patients was 46.2% in 2 years, falling continuously until reaching 15.3% in 20 years. Patients operated before reaching 60 days of age had longer survival periods with native livers (log rank < 0.0001). CONCLUSIONS: Late performance of portoenterostomy was a constant in the past 25 years, and this delay led to shorter survival periods with native livers for biliary atresia patients.

Atresia biliar: continuamos operando tarde / Biliary atresia: we still operate too late

OBJETIVO: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. MÉTODOS: Foram revisados os prontuários dos pacientes operados entre 1982 e 2007, residentes no Rio Grande do Sul. RESULTADOS: Dos 112 casos estudados de crianças com atresia biliar, 38 (33,9 por cento) ocorreram de 1982 a 1989, 46 (41,1 por cento) de 1990 a 1999 e 28 (25,0 por cento) a partir de 2000. Em 12 (10,7 por cento) casos, não foi realizada a portoenterostomia. A idade na cirurgia variou de 25 a 297 dias (mediana: 80,5; IIQ25-75: 61,3-109,0 dias); em 20,5 por cento dos casos, a idade das crianças foi menor do que 60 dias. Não houve diferença na idade, no momento do diagnóstico, entre as 3 décadas. Os pacientes do interior do estado (mediana: 87,0; IIQ25-75: 69,0-115,0 dias) foram encaminhados significativamente (p = 0,007) mais tarde do que os da região metropolitana de Porto Alegre (RS) (mediana: 68,0; IIQ25-75: 55,5-98,0 dias). A proporção de pacientes com menos de 60 dias foi significativamente menor (p = 0,013) nos oriundos do interior. A sobrevida com fígado nativo do total dos pacientes foi de 46,2 por cento em 2 anos, diminuindo progressivamente até 15,3 por cento em 20 anos. Os pacientes operados com menos de 60 dias tiveram maior sobrevida com fígado nativo (log rank < 0,0001). CONCLUSÕES: A portoenterostomia se manteve tardia nos últimos 25 anos, e esse atraso determinou menor tempo de sobrevida com fígado nativo nos pacientes com atresia biliar.

OBJECTIVE: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. METHODS: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. RESULTS: Of the 112 cases of children with biliary atresia studied, 38 (33.9 percent) occurred between 1982 and 1989, 46 (41.1 percent) between 1990 and 1999 and 28 (25.0 percent) after 2000. Portoenterostomy was not performed for 12 cases (10.7 percent). Age at surgery ranged from 25 to 297 days (median: 80.5; IQR25-75: 61.3-109.0 days); for 20.5 percent of cases, the age was below 60 days. There was no age difference at diagnosis for the three decades in the study. Patients from the countryside (median: 87.0; IQR25-75: 69.0-115.0 days) were referred significantly later (p = 0.007) than those living in Porto Alegre and the metropolitan area (median: 68.0; IQR25-75: 55.5-98.0 days). The ratio of patients younger than 60 days was significantly lower (p = 0.013) for those from the countryside. Survival periods with native liver for all patients was 46.2 percent in 2 years, falling continuously until reaching 15.3 percent in 20 years. Patients operated before reaching 60 days of age had longer survival periods with native livers (log rank < 0.0001). CONCLUSIONS: Late performance of portoenterostomy was a constant in the past 25 years, and this delay led to shorter survival periods with native livers for biliary atresia patients.

Amplification of bacterial DNA does not distinguish patients with ascitic fluid infection from those colonized by bacteria.

OBJECTIVE: To evaluate 16S ribosomal RNA (rRNA) gene amplification to diagnose spontaneous bacterial peritonitis (SBP). PATIENTS AND METHODS: According to a retrospective protocol, 31 patients with portal hypertensive ascites (serum to ascites albumin gradient > or = 1.1 g/dL) were studied. Ascitic fluid was analyzed as follows: Gram stain, aerobic and anaerobic cultures, polymorphonuclear cell count, and biochemical tests. Bacterial DNA was detected by polymerase chain reaction. RESULTS: There were 8 episodes of SBP and 4 episodes of bacterascites (BA). Culture was positive in 4 of 8 cases of SBP and bacterial DNA was positive in 7 of 8 cases of SBP. Bacterial DNA was positive in 3 of 4 cases of BA and in 8 of 28 cases of culture-negative non-neutrocytic ascites (CNNNA). The PELD score, serum to albumin ascites gradient, and mortality showed no statistical difference between patients with CNNNA and the result of the bacterial DNA analysis. CONCLUSIONS: Although the 16S rRNA gene amplification was better than culture to diagnose SBP, bacterial DNA does not seem to allow a distinction between ascites infection and ascites colonization.

The role of food restriction on CCl4-induced cirrhosis model in rats.

Effects of food restriction on susceptibility to the toxic effect of some chemicals are controversial. In order to identify an exposure model that could maximize cirrhosis and minimize mortality rate, this study aimed to evaluate the effect of food restriction on tetrachloride carbon (CCl(4))-induced cirrhosis model in rats. Fifty-three male Wistar rats received CCl(4) 0.25 ml/kg weekly intragastrically once a week. Thirty-three had 44% food restriction (group 1); 10 rats had 25% food restriction (group 2); and 10 rats received ad libitum food (group 3). After 10 weeks, the animals were sacrificed and liver sections were collected for histology. Of the 53 animals enrolled for the study, 22 (41.5%) died before completing 10-week CCl(4). Mortality rate was significantly higher in group 1 compared to other groups (p<0.05). Cirrhosis was significantly more prevalent in group 1 than in group 3 (p<0.01), but without significant difference between groups 1 and 2 (p=0.624). We concluded that food restriction is an important issue to be considered when establishing a CCl(4)-induced cirrhosis model in rats. Moreover, there is an ideal range of food intake that predisposes to liver damage without increasing mortality leading to a more effective model.

Infected and noninfected ascites in pediatric patients.

OBJECTIVES: To determine the prevalence of spontaneous bacterial peritonitis, ascites with bacterial infection and noninfected ascites in pediatric patients with portal hypertensive ascites and to compare the clinical and laboratory features of infected and noninfected ascites. METHODS: Forty-one episodes of portal hypertensive ascites (serum-ascites albumin gradient >1.1 g/dL) in 31 patients were studied. Median age was 2.9 years. Twenty-four (77.4%) patients were cirrhotic and 20 (83.3%) were classified as Child-Pugh C. Median pediatric end-stage liver disease score was 18.5. The following ascites features were assessed: polymorphonuclear neutrophil cell count, cytology, pH, concentration of glucose, lactic dehydrogenase, total protein and albumin, Gram stain and bacteriological culture. Blood was sampled for complete blood count, coagulation studies, liver and renal function tests. Groups were compared by Mann-Whitney and chi tests (P < 0.05). RESULTS: Noninfected ascites were observed in 29 of 41 samples, spontaneous bacterial peritonitis in eight of 41 and ascites with bacterial infection in four of 41. The most prevalent clinical features were fever, voluminous ascites and encephalopathy, but there were no significant differences in the clinical features of the groups. All patients with infected ascites were cirrhotic. There was no statistical difference in Child-Pugh or pediatric end-stage liver disease status between patients with infected and noninfected ascites. Culture of ascetic fluid was positive in four of eight cases of spontaneous bacterial peritonitis. Gram-negative rods were the most prevalent bacteria cultured. Except for serum albumin, no statistical differences in biochemical markers were observed between patients with infected and noninfected ascites. CONCLUSIONS: The prevalence of infected ascites was 29.2%. With the exception of serum albumin, there were no differences in the clinical and biochemical features of patients with infected ascites and noninfected ascites.

Ânus anterior: está ou não associado com a constipação crônica na criança? / Is anterior anus associated to chronic constipation in children?

Com o objetivo de estudar a associação entre deslocamente ventral do ânus, através da medida do índice ânus-genital e a presença de constipação crônica em crianças, foram estudadas 195 crianças atendidas no ambulatório de Gastroenterologia Pediátrica do HCPA, com idade entre 10 dias e 9 anos e 10 meses, sendo 94 meninas e 101, meninos. Foram consideradas constipadas aquelas que não evacuavam há mais de 3 dias e que apresentavam fezes disquézicas ou cibalosas. Ânus anteriorizado foi definido como IAG menor do que o desvio-padrão daquele obtido da média das crianças controle não constipadas (IAG<0,40 em meninos e <0,24 em meninas). Foram identificadas 58/195 crianças (29,7 por cento) com constipação crônica. Não houve diferença estatísticamente significativa em relação a sexo (30,9 por cento) ou quanto à presença de ânus anterior. Não houve correlação estatisticamente significativa entre presença de ânus anteriorizado e constipação, ou entre constipação e as medidas do IAG

Evoluçäo dos pacientes pediátricos avaliados para transplante hépatico / Follow-up of pediatric patients evaluated for liver transplantation

Objetivo: analisar a evoluçäo de pacientes pediátricos avaliados para Transplante Hepático. Métodos: Foram revisados os prontuários das primeiras 65 crianças e adolescentes portadores de hepatopatias crônicas, com idades de 5 meses a 19 anos (x=6,8 anos), que foram avaliados, de agosto de 1994 a março de 1996, para realizar transplante de fígado. Os dados colhidos foram referenes às características demográficas dos pacientes, causa da hepatopatia, avaliaçäo psicossocial dos pacientes e de seus responsáveis e avaliaçäo clínico-laboratorial. De acordo com a gravidade da doença, os pacientes foram classificados como ativos (aguardando doaçäo), em avaliaçäo, inativos (hepatopatia compensada) e excluídos por motivos psicossociais, médicos ou por má indicaçäo...