Resumo Objetivo: Este estudo com acompanhamento de longo prazo visou a avaliar o quadro clínico, os achados laboratoriais, o perfil histológico, os tratamentos e os resultados de crianças e adolescentes com hepatite autoimune. Métodos: Foram analisados os prontuários médicos de 828 crianças e adolescentes com HAI. Foi usado um questionário para coletar os dados anônimos sobre o quadro clínico, os achados bioquímicos e histológicos e os tratamentos. Resultados: De todos os pacientes, 89,6% tinham hepatite autoimune-1 e 10,4% hepatite autoimune-2. O sexo feminino foi predominante nos dois grupos. A idade média no início dos sintomas foi 111,5 (6; 210) e 53,5 (8; 165) meses nos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. Foi observado início clínico agudo em 56,1% e 58,8% e sintomas insidiosos em 43,9% e 41,2% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. A probabilidade de insuficiência hepática foi 1,6 vezes maior para hepatite autoimune-2; 3,6% e 10,6% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente, apresentaram insuficiência hepática fulminante; o risco foi 3,1 vezes maior para hepatite autoimune-2. Os níveis de gamaglobulina e imunoglobulina G foram significativamente maiores nos pacientes com hepatite autoimune-1, ao passo que os níveis de imunoglobulina A e C3 foram menores em pacientes com hepatite autoimune-2; 22,4% dos pacientes apresentaram cirrose e a remissão bioquímica foi atingida em 76,2%. A taxa de sobrevida atuarial foi de 93,0%. Um total de 4,6% pacientes foram submetidos a transplante de fígado e 6,9% morreram (hepatite autoimune-1: 7,5%; hepatite autoimune-2: 2,4%). Conclusões: Nesta grande série clínica de crianças e adolescentes brasileiros, a hepatite autoimune-1 foi mais frequente e os pacientes com hepatite autoimune-2 mostraram maiores taxas de remissão da doença com respostas mais rápidas aos tratamentos. Os pacientes com hepatite autoimune-1 apresentaram maior risco de óbito.

Abstract Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

ABSTRACT Objective To evaluate the frequency of DQ2.5 and DQ8 alleles using the Tag-single-nucleotide polymorphism (Tag-SNP) technique in individuals with type 1 diabetes mellitus (T1DM) and celiac disease (CD) in southern Brazil. Materials and methods In a prospective design, we performed the search for DQA1*0501 and DQB1*0201 alleles for DQ2.5 and DQB1*0302 for DQ8 through Real-Time Polymerase Chain Reaction (RT-PCR) technique, using TaqMan Genotyping Assays (Applied Biosystems, USA). The diagnosis of CD was established by duodenal biopsy and genotypic determination performed by StepOne Software v2.3. Allelic and genotypic frequencies were compared between groups using Chi-square and Fisher’s exact tests and the multiple comparisons using Finner’s adjustment. Results Three hundred and sixty two patients with a median age of 14 years were divided into 3 groups: T1DM without CD (264); T1DM with CD (32) and CD without T1DM (66). In 97% of individuals with T1DM and CD and 76% of individuals with CD without T1DM, respectively, the alleles DQ2.5 and/or DQ8 were identified (p < 0.001). DQ2.5 was more common in individuals with CD (p = 0.004) and DQ8 was more common in individuals with type 1 diabetes (p = 0.008). Conclusions The evaluation of the alleles for DQ2.5 and DQ8 by Tag-SNP technique showed a high negative predictive value among those with T1DM, similar to that described by the conventional technique. The high frequency of DQ8 alleles in individuals with T1DM did not allow differentiating those at higher risk of developing T1DM.

OBJETIVOS: Estimar a prevalência de anticorpos contra hepatite A (anti-VHA) em grupo de crianças e adolescentes de laboratório público e privado em Porto Alegre e comparar com estudo realizado na década anterior. MÉTODOS: Entre 2007 e 2008 foi realizado estudo transversal onde foram incluídas, consecutivamente, 465 amostras de soros de crianças e adolescentes entre 1 e 19 anos de idade para determinar a prevalência de anticorpos anti-VHA total. As amostras foram fornecidas por laboratório público (grupo 1), que atende somente Sistema Único de Saúde, e por laboratório privado (grupo 2), representando os estratos socioeconômicos mais baixo e mais alto, respectivamente. O teste foi realizado em único laboratório (eletroquimioluminescência, Roche Diagnostics). Resultados > 20 UI/L foram considerados positivos. RESULTADOS: A soroprevalência de anti-VHA no grupo 1 foi de 37,6% e o percentual de positividade aumentou conforme a idade, variando de 19,4% entre 1-4 anos a 54,1% entre 15-19 anos. No grupo 2, a frequência de anti-VHA foi de 46,1% e foi inversamente relacionada à idade, caindo de cerca de 50,0% nas faixas etárias menores para 29,1% aos 15-19 anos. Houve diminuição significativa na prevalência do anti-VHA nas crianças de 5-9 anos do grupo 1 (p = 0,03), quando comparadas com estudo realizado na década de 1990. CONCLUSÕES: Os resultados sugerem queda na endemicidade da hepatite A em Porto Alegre na última década e indicam maior suscetibilidade à doença em crianças e adolescentes, principalmente no estrato socioeconômico mais baixo.

OBJECTIVES: To estimate the prevalence of anti-hepatitis A virus (anti-HAV) antibodies in serum samples from children and adolescents obtained at two clinical pathology laboratories in the city of Porto Alegre, south of Brazil, and to compare findings to those of a study carried out in the 1990s. METHODS: In this cross-sectional study conducted between 2007 and 2008, 465 serum samples obtained from subjects aged 1-19 years were consecutively tested to determine the prevalence of total anti-HAV antibodies. Samples were provided by a public laboratory (group 1) that serves the Unified Health System exclusively, meant to represent the lowest socioeconomic strata, and by a private laboratory (group 2), meant to represent the higher socioeconomic classes. Tests were performed at a single laboratory using commercially available electrochemiluminescence kits. Antibody levels > 20 UI/L were considered positive. RESULTS: The seroprevalence of anti-HAV in Group 1 was 37.6%. The percentage of anti-HAV reactivity increased from 19.4% in the 1-to-4 group to 54.1% in the 15-to-19 group. In Group 2, overall anti-HAV positivity was 46.1% and was inversely correlated with age, declining from roughly 50% in the youngest groups to 29.1% in the 15-to-19 group. Comparison of sample findings to those reported in a 1990s study showed a significant reduction in anti-HAV prevalence among 5-to-9-year-olds in group 1 (p = 0.03). CONCLUSIONS: The results suggest that the endemicity of hepatitis A in Porto Alegre has been declining over the past decade, and that children and adolescents, particularly those in the lowest socioeconomic strata, are more susceptible to the disease.

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%) 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%) pacientes. RESULTADOS: Quarenta e um (80,4%) pacientes apresentaram insuficiência pancreática (EL-1 fecal < 100 µg/g), sendo 17 (41,5%) homozigotos, 14 heterozigotos (34,1%) e 10 sem ∆F508 (24,4%). 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%) apresentaram EL-1 fecal > 200 µg/g, e 5/10 (50%) utilizavam enzimas. CONCLUSÕES: A atividade de EL-1 fecal < 100 µg/g, indicativa de insuficiência pancreática, apresentou-se em 17/17 (100%) 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%) 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-∆F508 (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 ∆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.

OBJETIVO: Determinar a concentração plasmática de zinco em crianças e adolescentes cirróticos e investigar a associação entre esses resultados e a ingestão dietética de zinco, os dados antropométricos e a gravidade da doença hepática. MÉTODOS: As concentrações plasmáticas de zinco foram avaliadas por espectrofotometria de absorção atômica em 30 crianças e adolescentes com cirrose (105,0±60,0 meses; 22 meninas) e 27 hígidas e sem doença hepática (122,3±47,3 meses, 14 meninas). Os dados relacionados ao zinco dietético foram avaliados por registro de ingestão alimentar de 3 dias. A antropometria incluiu peso, altura, espessura da dobra cutânea, circunferência braquial e área muscular do braço. A gravidade da doença hepática foi classificada de acordo com os critérios de Child-Pugh, MELD e PELD. RESULTADOS: As concentrações plasmáticas de zinco dos indivíduos controles e dos pacientes foram 105,69±19,46 e 75,44±24,45 μg/dL, respectivamente (p < 0,001). Nenhuma associação foi encontrada entre os índices antropométricos, a ingestão dietética e o zinco plasmático. Houve diferença estatística nas concentrações de zinco plasmático entre pacientes Child-Pugh B+C e controles (p < 0,001), e com relação ao PELD, entre pacientes abaixo e acima do ponto de corte 15 (p = 0,002). CONCLUSÃO: A prevalência de hipozincemia foi de 43% para pacientes com cirrose. A baixa concentração plasmática de zinco foi associada com a doença hepática grave; entretanto, a hipozincemia esteve presente mesmo em pacientes Child-Pugh A. Portanto, a suplementação de zinco deve ser considerada para os pacientes pediátricos cirróticos.

OBJECTIVE: To determine plasma zinc concentrations in children and adolescents with cirrhosis and to investigate the association between these results and dietary zinc intake, anthropometric data, and severity of liver disease. METHODS: Plasma zinc concentration was assessed by atomic absorption spectrophotometry in 30 children and adolescents with cirrhosis (105.0±60.0 months; 22 girls) and 27 without liver disease (122.3±47.3 months, 14 girls). Dietary zinc data were evaluated by 3-day food intake records. Anthropometry measures included height, weight, skinfold thickness, brachial circumference, and upper arm muscle size. Severity of liver disease was classified according to the Child-Pugh, MELD, and PELD criteria. RESULTS: The mean (± standard deviation) plasma zinc concentrations in control subjects and patients were 105.69±19.46 and 75.44±24.45 μg/dL, respectively (p < 0.001). No associations were found between anthropometric measures, dietary zinc intake, and plasma zinc concentration. There was statistical difference related to plasma zinc concentrations between Child-Pugh B + C patients and control subjects (p < 0.001), and concerning PELD, between patients below the cutoff score of 15 and those above (p = 0.002). CONCLUSION: The prevalence of hypozincemia was 43% for patients with cirrhosis. Although low plasma zinc concentration was associated with more severe liver disease, it was present even in some Child-Pugh A patients. Therefore, zinc supplementation should be considered for cirrhotic children.

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%) ocorreram de 1982 a 1989, 46 (41,1%) de 1990 a 1999 e 28 (25,0%) a partir de 2000. Em 12 (10,7%) 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% 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% em 2 anos, diminuindo progressivamente até 15,3% 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%) 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.

OBJECTIVE: We evaluated the seroprevalence for measles, mumps, and rubella in school-age children (6-12 years old) before and after the administration of three triple combined viral vaccines. METHODS: In two municipal schools of Rio Grande do Sul, Brazil, 692 blood samples were collected before vaccination and 636 samples 21 to 30 days after vaccination during 1996. IgG antibody seropositivity was investigated by enzyme-linked immunosorbent assay (measles and mumps with Enzygnost [Behring, Marburg, Germany]; rubella with Rubenostika [Organon Teknica, Boxtel, the Netherlands]). The vaccines compared were: A: E-Zagreb, L-Zagreb, and Wistar RA 27/3 (Tresivac); B: Moraten, J-Lynn, and Wistar RA 27/3 (M-M-R II); and C: Schwarz, Urabe AM-9, and Wistar RA 27/3 (Trimovax). RESULTS: Before vaccination, 79.2% [95% confidence interval (CI) = 76.0%-82.2%] of the samples were positive for measles, 69.4% (95% CI = 65.8%-72.8%) for mumps, and 55.4% (95% CI = 51.6%-59.2%) for rubella. After vaccination with the A, B, and C vaccines, seropositivity was 100.0%, 99.5%, and 100.0%, respectively for measles; 99.5%, 94.5%, and 92.0% for mumps; and 92.6%, 91.3%, and 88.6% for rubella. CONCLUSIONS: About one-fifth (20.8%) of the schoolchildren who could have been vaccinated against measles at age 9 months had levels of antibodies insufficient for protection. In the sample of schoolchildren without previous vaccination against mumps and rubella, high proportions of susceptible levels were found. All vaccines were immunogenic, but vaccine A yielded a seroconversion rate of 99.5% for the mumps component, which was significantly higher than the other two vaccines (P < 0.01).

OBJETIVO: Se evaluó la seroprevalencia para sarampión, paperas y rubéola en niños en edad escolar (6-12 años) antes y después de la administración de tres vacunas triples antivirales combinadas. MÉTODOS: Se colectaron 692 muestras de sangre antes de la vacunación y 636 muestras entre 21 y 30 días después de la vacunación a niños de dos escuelas municipales de Rio Grande do Sul, Brasil, durante 1996. Se investigó la seropositividad de anticuerpos de la clase IgG mediante un ensayo de inmunoadsorción enzimática tipo ELISA (sarampión y paperas con Enzygnost [Behring, Marburgo, Alemania] y rubéola con Rubenostika [Organon Teknica, Boxtel, Países Bajos]). Las vacunas comparadas fueron: a) E-Zagreb, L-Zagreb y Wistar RA 27/3 (Tresivac); b) Moraten, J-Lynn y Wistar RA 27/3 (M-M-R II); y c) Schwarz, Urabe AM-9 y Wistar RA 27/3 (Trimovax). RESULTADOS: Antes de la vacunación, 79,2% (intervalo de confianza [IC] 95%: 76,0 a 82,2) de las muestras fueron positivas para sarampión, 69,4% (IC 95%: 65,8 a 72,8) para paperas y 55,4% (IC 95%: 51,6 a 59,2) para rubéola. Después de la vacunación con las vacunas A, B y C, la seropositividad fue de 100%, 99,5% y 100%, respectivamente para sarampión; de 99,5%, 94,5% y 92,0% para paperas; y de 92,6%, 92,3% y 88,6% para rubéola. CONCLUSIONES: Alrededor de un quinto (20,8%) de los escolares que pudieron haber sido vacunados contra el sarampión a los 9 meses de edad tenían niveles de anticuerpos insuficientes para protegerlos. En la muestra de escolares sin vacunación previa contra paperas y rubéola se encontró una alta proporción de niños susceptibles. Todas las vacunas fueron inmunogénicas, pero la vacuna A produjo una tasa de seroconversión de 99,5% para el componente de paperas, significativamente mayor que la de las otras dos vacunas (P < 0,01).

OBJECTIVE: Analyze the changes in the seminiferous epithelium in rats with carbon tetrachloride-induced cirrhosis (CCl4). MATERIALS AND METHODS: Forty-eight male Wistar rats aged 45-50 days, weighing 150-180 grams were used. Twenty-two rats underwent CCl4-induced cirrhosis with CCl4 0.25 mL/Kg weekly intragastrically once a week, during 10 weeks. Additionally, they had a 44% food restriction diet (Group 1). The control group was divided in two subgroups: 13 rats had a 44% food restriction diet and no CCl4 (Group 2) and 10 rats were not submitted to CCl4 or food restriction (Group 3). After 10 weeks, the rats were sacrificed and liver sections were collected for histological analysis. The testicular analysis was carried out to evaluate the frequency of tubules in stages VIII and XIV. RESULTS: The mean rates of stage VIII in animals with food restriction plus CCl4-induced cirrhosis and food restriction without CCl4 were significantly different from animals without either food restriction or CCl4 (18.1 ± 5.5%, 20.5 ± 2.5% and 13.4 ± 3.5%, respectively, p = 0.002). The mean rate of stage VIII in rats with cirrhosis was not significantly different from rats without cirrhosis (18.1 ± 5.5% and 17.4 ± 4.6% respectively). The mean frequency of stage XIV in rats with cirrhosis was significantly greater than rats without cirrhosis (4.7 ± 2.3% and 6.8 ± 1.9% respectively, p = 0.027). CONCLUSION: Animals with CCl4-induced cirrhosis and food restriction have shown alterations in spermatogenic cycle that were not seen in rats without CCl4-induced cirrhosis and food restriction.

OBJETIVOS: testar a hipótese de que a mucosa do intestino delgado proximal de crianças com diarréia persistente apresenta alterações morfométricas e estereológicas proporcionais ao estado nutricional, utilizando captura de imagens através de microscópio acoplado ao computador. MÉTODOS: estudo transversal incluindo 65 pacientes pediátricos, com idade entre quatro meses e cinco anos, com diarréia de mais de 14 dias de duração, avaliados pelos escores z, divididos em eutróficos = z > 2 DP e desnutridos = z < -2DP; eutróficos = z > 2 DP, risco nutricional = z < -1DP e desnutridos = z < -2DP; e de maneira contínua em ordem decrescente, utilizando-se as tabelas do NCHS. Após a captura das imagens através do programa Scion Image, foram medidas a altura dos vilos, a profundidade das criptas, a espessura da mucosa, a espessura total da mucosa e a relação vilo/cripta (100 aumentos) e a altura do enterócito, altura do núcleo e do bordo em escova (500 aumentos). A análise estereológica foi feita através de arcos ciclóides. RESULTADOS: para os escores z P/I, P/E e E/I, divididos em duas categorias de estado nutricional, não houve diferença estatisticamente significante quanto às medidas da altura dos vilos, profundidade das criptas, espessura da mucosa, espessura total da mucosa e relação vilo/cripta. A altura do enterócito foi a característica que apresentou maior diferença entre os grupos eutróficos e desnutridos, para os índices P/I e P/E, em 500 aumentos, sem atingir significância estatística. No entanto, para três categorias de estado nutricional, a análise morfométrica digitalizada mostrou diferença estatisticamente significante para a relação vilo/cripta entre eutróficos e desnutridos leves e entre eutróficos e desnutridos moderados e graves (p=0,048). A relação vilo/cripta foi maior nos eutróficos. Através do coeficiente de correlação linear de Spearman, a altura do enterócito, a altura do núcleo do enterócito e a do bordo em escova mostraram clara associação com os índices P/I (r=0,25;p=0,038) e P/E (r=0,029; p=0,019). A altura do núcleo do enterócito e a do bordo em escova mostraram, ainda, associação com o índice P/E. CONCLUSÕES: as correlações encontradas entre o estado nutricional e a morfometria das variáveis da mucosa do intestino delgado relacionaram-se ao peso dos pacientes. Embora estas associações tenham sido de magnitude fraca a moderada, há uma tendência à diminuição do tamanho do enterócito, do seu núcleo e do bordo em escova, à medida que aumenta o grau de desnutrição.

OBJECTIVES: to test the hypothesis that the proximal small intestines of children with persistent diarrhea present morphometric and stereologic changes proportional to their nutritional status, using microscope images stored in a computer. METHODS: cross-sectional study with 65 pediatric patients, whose ages ranged from 4 months to 5 years, with persistent diarrhea for over 14 days. The nutritional assessment was performed according to the z-scores for weight/age (W/A), weight/height (W/H) and height/age (H/A) ratios, divided into: well-nourished = z > 2SD and malnourished = z < -2SD; well-nourished = z > 2SD, nutritional risk = z < -1SD and malnourished = z < -2SD; and continuously, in descending order, using the NCHS charts. After obtaining the computer images using the software Scion Image, villous height, crypt depth, mucosal thickness, total mucosal thickness, and villous/crypt ratio were measured in the fragments of the small intestinal mucosa, enlarged 100 times. When images were enlarged 500 times, enterocyte height, nuclear height and brush-border height were measured. Stereologic analysis was performed using cycloid arcs. RESULTS: for W/A, W/H and H/A z-scores, divided into two nutritional status categories, no statistically significant difference was observed in regard to villous height, crypt depth, mucosal thickness, total mucosal thickness and villous/crypt ratio. Enterocyte height presented the most significant difference between well-nourished and malnourished groups, for W/A and W/H ratios, with a 500x enlargement, although this difference was not statistically significant. When z-scores were subdivided into three nutritional status categories, a digital morphometric analysis showed a statistically significant difference for villous/crypt ratio between the well-nourished and slightly malnourished group and the well-nourished and mild to severe malnourished group (p = 0.048). The villous/crypt ratio was higher among well-nourished children. Using the Spearman coefficient, the variables enterocyte height, height of enterocyte nucleus and brush-border height presented a clear association with the W/A ratio (r = 0.25; p = 0.038), W/H ratio (r = 0.029; p = 0.019). The height of the enterocyte and the brush-border height were associated with W/H ratio. CONCLUSIONS: the observed associations between nutritional status and the analyzed small intestinal mucosa variables showed a positive correlation with patients' weight. Although these associations were of a slight to moderate magnitude, we observed a tendency of enterocyte size reduction, as well as a reduction in the size of its nucleus and brush-border, as the level of malnutrition increases.

OBJETIVO: verificar os níveis plasmáticos de vitamina D de pacientes colestáticos crônicos e relacionar com estado nutricional, tempo de colestase e uso de suplemento vitamínico. MÉTODOS: estudo transversal controlado, cujo fator em estudo é colestase crônica e o desfecho, o nível plasmático de vitamina D. Pacientes entre quatro meses a 18 anos, atendidos na unidade de gastroenterologia pediátrica do HCPA; como controles, crianças eutróficas da mesma faixa etária. Foi coletado sangue para as dosagens por radioimunoensaio, e realizadas avaliação antropométrica, pesquisa de tempo de colestase e uso de suplemento vitamínico. RESULTADOS: foram avaliadas 22 crianças e adolescentes com colestase crônica e 17 controles. O valor médio de vitamina D entre os pacientes foi de 13,7 ± 8,39 ng/ml, enquanto que nos controles foi de 25,58 ± 16,73 ng/ml (p = 0,007). A prevalência de hipovitaminose D, entre os pacientes, foi de 36%. A mediana do tempo de colestase foi de um ano. A avaliação antropométrica (NCHS) demonstrou 36% de desnutrição pelo peso, e 41% para estatura. Na avaliação antropométrica pelo escore z, obtivemos prevalência de desnutrição para os critérios altura/idade e peso/idade de 33,3% e 23,8%, respectivamente. Avaliado peso em relação altura, não observamos valores abaixo de dois desvios padrão. Não foi observada relação entre o estado nutricional, o uso de suplemento oral e os níveis plasmáticos de vitamina D CONCLUSÕES: os níveis plasmáticos de vitamina D em colestáticos foram menores do que os dos controles, sem relação com estado nutricional, tempo de colestase e/ou uso suplementação vitamínica.

OBJECTIVE: to verify blood levels of vitamin D in patients with chronic cholestasis, and relate them to nutritional status, length of time since the onset of cholestasis and use of vitamin supplement. METHODS: controlled cross-sectional study with chronic cholestasis as study factor and blood levels of vitamin D as outcome. The study included patients aged between 4 months and 18 years, who were cared for at the Pediatric Gastroenterology Unit of Hospital de Clínicas de Porto Alegre. Controls were eutrophic children in the same age range. Blood was collected for radioimmunoassay. Anthropometric analyses were performed, as was determination of the length of time since the onset of cholestasis and use of vitamin supplement. RESULTS: twenty-two patients and 17 controls were evaluated. Average vitamin D level in patients was 13.7 ± 8.39 ng/ml, compared to 25.58 ± 16.73 ng/ml in controls (p = 0.007). Prevalence of hypovitaminosis D in patients was 36%. Median of period of time since the onset of cholestasis was 1 year with variation of 6 months to the 25th percentile and 3.9 years to the 75th percentile. Anthropometric evaluation (NCHS) showed 36% of malnutrition by weight and 41% by height. Anthropometric evaluation according to Z score showed 33.3% and 23.8% prevalence of malnutrition for the criteria height/age and weight/age, respectively. The evaluation of weight regarding height did not show values below two standard deviations. No relationship was found between nutritional state, use of oral vitamin supplement and blood levels of vitamin D. CONCLUSIONS: blood levels of vitamin D in patients with cholestasis were lower than those of controls, but were not related to nutritional status, period of time since the onset of cholestasis or use of vitamin supplement.

OBJETIVO: determinar a freqüência de deficiência da proteína C (PC), proteína S (PS) e antitrombina (AT) e das mutações fator V Leiden (FVL), G20210A, no gene da protrombina (PTR), e C677T da metileno-tetraidrofolato redutase (MTFR) em crianças e adolescentes com trombose da veia porta (TVP), e definir o padrão hereditário de uma eventual deficiência. MÉTODOS: durante o período de dois anos, foi investigada a presença de distúrbios trombofílicos em 14 crianças e adolescentes (grupo 1) com TVP, seus pais (grupo 2, n = 25) e dois grupos controles constituídos, o primeiro, por crianças e adolescentes sem hepatopatia, pareados por idade com as crianças do grupo 1 (n = 28), e o outro, por pacientes com cirrose (n = 24). Os pacientes com TVP foram investigados do ponto de vista clínico, laboratorial, endoscópico e com biópsia de fígado. O diagnóstico da trombose foi realizado por ultra-sonografia abdominal com Doppler e/ou estudo angiográfico. RESULTADOS: a freqüência da deficiência de PC, PS e AT nos pacientes com TVP foi de 6/14 (42,9%) (p < 0,05 versus controles sem hepatopatia), 3/14 (21,4%) (p > 0,05) e 1/14 (7,1%) (p > 0,05), respectivamente. Nos pacientes com cirrose, a freqüência da deficiência de PC, PS e AT foi de 14/24 (58,3%), 7/24 (29,2%) e 11/24 (45,8%), respectivamente (p < 0,05 versus controles sem hepatopatia). A deficiência dessas proteínas não foi identificada nos pais dos pacientes com TVP e em nenhum controle sem hepatopatia. A mutação G20210A da PTR foi identificada em um paciente com TVP e em um controle sem hepatopatia (p = 0,999). A mutação C677T da MTFR foi observada na forma homozigótica, em 3/14 (21,4%) dos pacientes com TVP, e em 5/28 (17,9%) controles (p = 0,356). Em nenhum paciente ou controle sem hepatopatia, foi identificado o FVL. CONCLUSÕES: metade das crianças e adolescentes com TVP apresentou deficiência de uma ou mais proteínas inibidoras da coagulação, principalmente da proteína C, mas esta deficiência não parece ser de origem genética. Os distúrbios trombofílicos hereditários não representaram papel importante como causa da TVP nas crianças e adolescentes. Nos pacientes cirróticos, houve maior freqüência de deficiência das proteínas na presença de doença de maior intensidade.

OBJECTIVE: to determine the frequency of protein C, protein S and antithrombin deficiency, and factor V Leiden, prothrombin G20210A, and methylenetetrahydrofolate reductase C677T mutations in children and adolescents with portal vein thrombosis, as well as assessing the hereditary character of this disorders. METHODS: a two-year study was carried out to determine the frequency of thrombophilic disorders in children and adolescents with portal vein thrombosis (n = 14), their parents (n = 24), and two control groups, one age-matched children and adolescents free of liver disease (n = 28) and another group with cirrhosis (n = 24). The portal vein thrombosis patients were investigated by clinical and laboratory means, esophagogastroduodenal endoscopy and liver biopsies. The presence of portal vein thrombosis was assessed by Doppler ultrasonography and/or angiographic analysis. RESULTS: the frequency of protein C, protein S and antithrombin deficiency was 6/14 (42.9%) (p < 0.05 versus. controls), 3/14 (21.4%) (p > 0.05) and 1/14 (7.1%) (p > 0.05) of children and adolescents with portal vein thrombosis, respectively. The frequency of protein C, protein S and antithrombin deficiency in cirrhotic patients was 14/24 (58.3%), 7/24 (29.2%) and 11/24 (45.8%), respectively (p < 0.05 versus controls free of liver disease). None of the portal vein thrombosis parents or controls presented protein C, protein S or antithrombin deficiency. One portal vein thrombosis patient and one control (p = 0.999) presented prothrombin G20210A mutation. The homozygous form of methylenetetrahydrofolate reductase C677T mutation was observed in 3/14 (21.4%) patients with portal vein thrombosis and in 5/28 (17.9%) (p = 0.356) controls. None of the patients or controls presented the factor V Leiden. CONCLUSIONS: half of the children and adolescents with portal vein thrombosis presented deficiency of one or more coagulation inhibitor proteins, mainly protein C, but this deficiency does not seem to be an inherited condition. The hereditary prothrombotic disorders do not seem to play a vital role in thrombosis in patients with portal vein thrombosis of this study. In the cirrhotic patients, there was a higher frequency of protein deficiency when the disease was more intense.

Objetivo: avaliar a prevalência de hepatite viral A (HVA) em crianças e adolescentes portadores de doenças crônicas do fígado, em um serviço de hepatologia pediátrica. Métodos: entre maio de 1999 e fevereiro de 2001, foi estudada a prevalência de anticorpos anti-HVA total em 60 crianças e adolescentes, entre 1 e 16 anos de idade, portadoras de hepatopatias crônicas, provenientes da unidade de gastroenterologia pediátrica e programa de transplante hepático infantil do serviço de pediatria do Hospital de Clínicas de Porto Alegre. O anti-HVA, realizado através de um teste laboratorial comercialmente disponível em nosso meio (Abbott - MEIA HAVAB - sistema AXSYM), foi determinado e relacionado com a idade, com o sexo, com a cor, com o diagnóstico etiológico da hepatopatia e com a renda familiar dos pacientes. Resultados: apenas uma criança de 1 ano, portadora de atresia biliar, foi excluída do estudo por apresentar anti-HVA indeterminado, em duas ocasiões. Das 59 crianças restantes, 14 (24%) apresentavam resultados positivos para o anti-HVA total. As idades dos pacientes com anti-HVA positivos variaram de 1 a 16 anos (x= 7,7 anos e mediana 8,5 anos). Não houve diferença significante entre idade, sexo e cor entre os grupos positivo e negativo. A renda familiar foi menor no grupo dos pacientes anti-HVA positivo, mas não mostrou diferença estatística significante. A diferença de prevalência de anti-HVA entre as etiologias das hepatopatias está, provavelmente, relacionada à idade mais do que ao diagnóstico. Conclusões: na população estudada, a maioria (76%) das crianças e adolescentes portadoras de hepatopatias crônicas é suscetível à infecção pelo vírus A, podendo apresentar, portanto, um curso mais grave e complicações, se adquirirem HVA. Sugerimos, então, que esses pacientes devam receber a vacina inativada contra HVA.

Objective: to evaluate the prevalence of Hepatitis A virus (HAV) in children and adolescents with chronic liver disease in a unit of pediatric hepatology. Methods: between May 1999 and February 2001, we studied the prevalence of anti-HAV in 60 children and adolescents with chronic liver disease, aged between 1 and 16 years, from the Unit of Pediatric Hepatology of the Hospital de Clínicas de Porto Alegre. The total anti-HAV was determined by a commercially available competitive ELISA method (Abbott), and compared with age, sex, race, etiologic diagnosis and family income of each patient. Results: a one-year old child was not included in the study because she presented twice with undetermined anti-HAV results. Among the other 59 patients, 14 (24%) presented a positive result of total anti-HAV. The ages of test-positive subjects varied between 1 and 16 years old (mean = 7.7 years, median = 8.5). The differences between positive and negative groups in relation to age, sex and race were not statistically significant. Family income was lower in anti-HAV positive patients, but this difference was not significant. The differences between the etiologies of liver diseases were probably more related to the age than to the etiologies of the diseases. Conclusions: in the studied population, the majority (76%) of children and adolescents with chronic liver disease are susceptible to hepatitis A virus infection and, consequently, they could present a more severe disease or even fulminant hepatitis A. We strongly suggest that these subjects receive Hepatitis A inactivated vaccine.

The high incidence of Hepatitis A and B in institutionalized patients with Down Syndrome (DS) is not fully understood. Under poor hygienic conditions, immunological alterations might predispose individuals to these infections. Sixty three DS children between 1 and 12 years old living at home with their families were examined for anti-HAV and compared to age-matched controls (64 healthy children). This cross-sectional study was carried out from May, 1999, to April, 2000, at the Hospital de Clínicas of Porto Alegre, southern Brazil. Groups were compared in terms of age, sex, skin color, and family income (> R$ 500 and < R$ 500/ month) by the chi-square test, with Yates' correction and for the prevalence of anti-HAV (Fisher's exact test). In the DS group (n=63), the mean age was 4.4 ± 3.3 years, 94% of the patients were white and 51% were female. Family income was <= R$ 500/month in 40 cases (63%). In the control group (n=64), the mean age was 4.8 ± 2.7 years, 81% of the patients were white and 56% were female. Family income was <= R$ 500 in 20 patients (31%). DS children's families had a significantly lower income (P<0.0005). In the DS group there were 6 positive (9.5%) anti-HAV cases, and all came from low-income families (less than R$ 500/ month). In the control group, 3 cases (4.7%) were positive for anti-HAV (two were from a low-income family and one was from a higher income family). These differences were not significant. Our data indicate that Hepatitis A is not a special risk for mentally retarded DS outpatients, even in a developing country like Brazil.

Objetivo: determinar a prevalência de má absorção de lactose e sua associação com a cor da pele e com a idade em crianças e adolescentes de escolas públicas do município de Porto Alegre. Métodos: foi realizado um estudo transversal, que incluiu 225 indivíduos de 8 a 18 anos, alunos de duas escolas públicas do município de Porto Alegre. A seleção dos alunos ocorreu através de sorteio. Os participantes foram classificados segundo a cor da pele (brancos e não-brancos), e a faixa etária (8 a 12 e 13 a 18 anos). A má absorção de lactose foi diagnosticada através do teste do hidrogênio expirado após ingestão de 250ml de leite de vaca integral industrializado. O teste teve duração de 3 horas, com coletas em jejum e aos 60, 120 e 180 minutos após a ingestão do leite. Foi considerado como critério de positividade o aumento 20 ppm na concentração de hidrogênio em relação ao nível basal. Resultados: foram estudados 225 alunos, com uma média e desvio-padrão de idade de 12,2 2,0 anos. Cento e trinta e quatro indivíduos eram do sexo feminino (59,6%). Cento e cinqüenta e quatro alunos eram de cor branca (68,4%) e os restantes, de cor não-branca. A má absorção de lactose foi evidenciada em 19/225 casos (8,4%). Ela foi diagnosticada em 8/154 alunos de cor branca (5,2%) e em 11/71 alunos de cor não-branca (15,5%) (p= 0,02). Em relação à faixa etária, ocorreram 15/143 casos de má absorção nos alunos entre 8 a 12 anos (10,5%), e 4/82 casos entre 13 e 18 anos (4,9%) (p= 0,227). Conclusões: a prevalência de má absorção de lactose encontrada em alunos de escolas públicas de Porto Alegre é significativa, especialmente se considerarmos que foram utilizadas doses fisiológicas do dissacarídeo (250 ml de leite) para o diagnóstico. A taxa de má absorção foi maior entre as crianças de cor não-branca em relação às crianças de cor branca, confirmando a influência racial na hipolactasia primária do tipo adulto. Não foi encontrada associação entre má absorção de lactose e faixa etária.

Objective: To determine the prevalence of lactose malabsorption and its association with skin color and age in children and teenagers attending public schools in Porto Alegre, Brazil. Methods: A cross-sectional study was performed with 225 subjects between 8 and 18 years attending two public schools in Porto Alegre, Brazil. Patients were randomly selected. Subjects were classified according to skin color (white and non-white) and age group (8 to 12 and 13 to 18 years). Lactose malabsorption was diagnosed using the breath hydrogen test after ingestion of 250 ml of whole cow milk. The test lasted for 3 hours, with collections after fasting and 60, 120, and 180 minutes after milk ingestion. Malabsorption was determined in the presence of increase of >20 ppm in hydrogen concentration regarding the basal levels. Results: Two-hundred and twenty-five students were studied, with a mean age ± standard deviation of 12.2 ± 2.0 years. The subjects consisted of 134 females (59.6%); 154 white (68.4%); and 71 nonwhite. Lactose malabsorption was observed in 19/225 cases (8.4%). It was diagnosed in 8/154 white patients (5.2%) and in 11/71 non-white patients (15.5%) (p = 0.02). Regarding the age group, we found 15/143 cases of malabsorption in students between 8 and 12 years (10.5%), and 4/82 cases in students between 13 and 18 years (4.9%) (p = 0.227). Conclusions: The prevalence of lactose malabsorption in students attending public schools in Porto Alegre is significant, especially if we consider that the physiological doses (250 ml of milk) were used for diagnosis. The malabsorption rate was higher among non-white children, which confirms the influence of race on primary adult type hypolactasia. No association was observed between lactose malabsorption and age group.

Objectives: Varicella has more serious consequences in adolescents and adults. Recent reports from Europe and Asia show an increasing number of adolescents and young adults being seronegative. As there is only limited data on varicella zoster virus (VZV) seroprevalence in Brazil and to facilitate the strategy for varicella vaccination we conducted a VZV seroprevalence study in Brazil. Methods: This population-based, cross sectional seroepidemiology study was performed in 4 different regions of Brazil. The studied population was stratified according to gender, age and socioeconomic status. VZV IgG antibodies were analyzed by ELISA. Results: 3,879 subjects aged 1Œ40 years were included into the study. The overall anti-VZV seropositivity rate across all age groups and centers in Brazil was 85.4%. There was a strong age relationship. Especially in the South East and South seroprevalence was low in the age group 1-5 years (44.5% and 57.8%, respectively) while in the North the rate was 88.9%. Overall, Varicella infection was independent of the socioeconomic level, but in the youngest age groups (1-10 years) seroprevalence rates were significantly lower in the high/medium socioeconomic class for most regions. Clinical history of chickenpox correlates well with anti-VZV seropositivity with a predictive value of 95.1% Conclusions: In preadolescence a substantial proportion of the Brazilian population is susceptible to Varicella infection, and a considerable part of the adolescents and young adults remain VZV-seronegative and are thus also at risk

Objetivos: La varicela tiene consecuencias más graves en adolescentes y adultos. Relatos recientes de Europa y Asia muestran un número creciente de adolescentes y adultos jóvenes seronegativos. Como son pocos los datos sobre la prevalencia de antiVZV en Brasil y con el objetivo de definir estrategias para la inmunización contra la varicela, realizamos un estudio de seroprevalencia para el VZV (virus varicela Zoster) en diferentes partes de Brasil. Método: Este estudio seroepidemiológico transversal y poblacional fue realizado en cuatro diferentes regiones de Brasil. La población estudiada fue estratificada por sexo, edad y situación socioeconómica. Fueron analizados por el método Elisa anticuerpos IgG, antiVZV. Resultados: Fueron incluidos en el estudio 3 879 individuos de 1 a 40 años. La tasa de seropositivos de antiVZV en todos los grupos etarios y centros en Brasil fue de 85,4%. Se evidenció una fuerte correlación con la edad. Especialmente en el sureste y sur la seroprevalencia fue menor en el grupo etario de 1-5 años (44,5% y 57,8%, respectivamente. En la región norte esta tasa fue de 88,9%. Conclusiones: En la preadolescencia una proporción substancial de la población brasileña es susceptible a la varicela y una parte considerable de adolescentes y adultos jóvenes se mantienen seronegativos y, consecuentemente, en riesgo