Fissuras lábio-palatinas : incidência e prevalência da patologia no Hospital de Clinicas de Porto Alegre : um estudo de 10 anos

As fissuras lábio-palatinas estão entre as anomalias congênitas mais comuns. Realizou-se um estudo no Hospital de Clínicas de Porto Alegre (HCPA) com o objetivo de avaliar a sua prevalência e analisar possíveis fatores predisponentes. Foram estudados todos os casos de Hssura lábio-palatiria nascidos no hospital no período de fevereiro de 1983 até julho de 1993. Foram utilizados dados do Estudo Colaborativo Latino-Americano de Malformações Congênitas (ECLAMC) e do Serviço do Arquivo Médico e Informação de Saúde (SAMIS) do H CP A. Ocorreram 41 casos de fendas lábiopalatinas em 31.058 nascimentos, correspondendo a uma prevalência de 1/757,5 nascimentos. Os casos foram descritos pelo método LAHSHAL, um novo sistema de documentação, que, por ser simples e facilmente memorizado, facilita a descrição clínica e seu uso em trabalhos de pesquisa.The cleft lip and palate is among the most frequent congenital anomalies. This study was done at the ''Hospital de Clínicas de Porto Alegre (HCPA)" aiming to evaluate possible risk factors involved in its genesis and its prevalance. All cases of cleft lip and palate, who were born at the hospital during the period of February,1983 until July, 1993, were accepted at the study. Data was provided by the ''Estudo Colaborativo Latino-Americano de Malformações Congênitas (ECLAMC)" and the "Serviço do Arquivo Médico e lnformação de Saúde (SAMJS)" at the HCPA. We found 41 cleft cases in 31.058 children born, corresponding to a prevalence of 1/757,5 deliveries. The cleft forms were described using the LAHSHAL method, a new description system that is simple and quickly memorized. It provides good clinical description and makes research easier

Valor preditivo da incorporação da estimativa de volume e extensão do câncer de próstata na ressonância magnética ao nomograma de Kattan para radioterapia externa

Objetivo: Determinar se os resultados de exames de ressonância magnética (RM) e imagens espectroscópicas por RM podem melhorar as previsões feitas com o nomograma de Kattan para a radioterapia. Métodos: O conselho de revisão institucional aprovou este estudo retrospectivo de acordo com a HIPAA (Lei de Portabilidade e Responsabilidade de Seguro de Saúde dos Estados Unidos). Foram incluídos noventa e nove homens que se submeteram a RM endorretal e espectroscopia por RM antes da radioterapia por feixe externo para câncer de próstata (janeiro de 1998 a junho de 2007). Preditores lineares foram calculados com as variáveis de entrada da amostra do estudo e os coeficientes de Kattan originais. O preditor linear é um valor ponderado único que combina informações de todas as variáveis de previsão em um modelo em que o peso de cada valor é sua associação com o resultado. Dois radiologistas analisaram todas as imagens de RM de forma independente, para determinar a extensão da doença. Um terceiro leitor independente sanou as discrepâncias. A falha bioquímica foi definida como um nível sérico de antígeno específico da próstata no soro de 2 ng/mL (2 mg/L) ou mais acima do nadir. Modelos de Cox de risco proporcional foram usados para determinar as probabilidades de falha do tratamento (falha bioquímica) em cinco anos. Um modelo incluiu apenas os dados do nomograma de Kattan; o outro incorporou também os resultados de imagem. O desempenho de discriminação de todos os modelos foi determinado com análises de curva das características operacionais do receptor (ROC). Essas análises foram seguidas por uma avaliação de reclassificação de risco líquido. Resultados: As áreas sob a curva ROC para o nomograma de Kattan e o modelo que incorporava descobertas por imagens de RM foram de 61,1% (intervalo de confiança de 95%: 58,1%, 64,0%) e 78,0% (intervalo de confiança de 95%: 75,7%, 80,4%), respectivamente. A comparação de desempenho demonstrou que o modelo que incorporava resultados de exames de imagem teve desempenho significativamente melhor que o modelo com variáveis clínicas isoladamente (P <.001). De maneira geral, a inclusão de resultados de imagem levou à melhoria na classificação de risco de cerca de 28%, variando do mínimo de aproximadamente 16% ao máximo de 39%, dependendo da alteração de risco considerada importante. Conclusão: Os dados de imagem de RM melhoram a previsão de falha bioquímica com o nomograma de Kattan após radioterapia por feixe externo para câncer de próstata. O número necessário de imagens para melhorar a previsão de falha bioquímica em um paciente variou de três a seis.Purpose: To determine whether magnetic resonance (MR) imaging and MR spectroscopic imaging findings can improve predictions made with the Kattan nomogram for radiation therapy. Methods: The institutional review board approved this retrospective HIPAAcompliant study. Ninety-nine men who underwent endorectal MR and MR spectroscopy before external-beam radiation therapy for prostate cancer (January 1998 to June 2007) were included. Linear predictors were calculated with input variables from the study sample and the Kattan original coefficients. The linear predictor is a single weighted value that combines information of all predictor variables in a model, where the weight of each value is its association with the outcome. Two radiologists independently reviewed all MR images to determine extent of disease; a third independent reader resolved discrepancies. Biochemical failure was defined as a serum prostate- specific antigen level of 2 ng/mL (2 mg/L) or more above nadir. Cox proportional hazard models were used to determine the probabilities of treatment failure (biochemical failure) in five years. One model included only the Kattan nomogram data; the other also incorporated imaging findings. The discrimination performance of all models was determined with receiver operating characteristics (ROC) curve analyses. These analyses were followed by an assessment of net risk reclassification. Results: The areas under the ROC curve for the Kattan nomogram and the model incorporating MR imaging findings were 61.1% (95% confidence interval: 58.1%, 64.0%) and 78.0% (95% confidence interval: 75.7%, 80.4%), respectively. Comparison of performance showed that the model with imaging findings performed significantly better than did the model with clinical variables alone (P < .001). Overall, the addition of imaging findings led to an improvement in risk classification of about 28%, ranging from approximately a minimum of 16% to a maximum of 39%, depending on the risk change considered important. Conclusion: MR imaging data improve the prediction of biochemical failure with the Kattan nomogram after external-beam radiation therapy for prostate cancer. The number needed to image to improve the prediction of biochemical failure in one patient ranged from three to six

Eficiência das dosagens enzimáticas em fibroblastos no diagnóstico de erros inatos do metabolismo

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Eficiência das dosagens enzimáticas em fibroblastos no diagnóstico de erros inatos do metabolismo

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Liver fat accumulation after islet transplantation and graft survival

Our objective is to evaluate if there is an association between liver fat accumulation after islet transplantation (ITx) and graft survival. A cohort study was conducted in 34 subjects with type 1 diabetes postallogeneic ITx. Liver fat content was evaluated by magnetic resonance imaging (MRI) (change in liver signal intensity on inphase and opposed-phase images). Kaplan–Meier curves and Cox regression analysis were performed with islet dysfunction duration as the dependent variable and fat liver content as an independent one. Values of p < 0.05 were significant (SSPS®18.0 and MedCalc®12.5). Patients’ mean age was 40 ± 8 years (diabetes duration: 31 ± 12 years; male: 41%). Islet survival did not differ in patients without (51 months, 95% CI 40–62 months) or with steatosis (48 months, 95% CI 38–58 months; p = 0.55) during islet dysfunction period. Nevertheless, survival curves appear to separate late in the follow-up, and after 40 months steatosis was associated with shorter graft survival (p log rank = 0.049). This association remained (RR 23.5, 95% CI 1.1–516.0; p = 0.045) after adjustments for possible confounding factors. In this sample of subjects with type 1 diabetes submitted to ITx, steatosis was not associated with islet failure in the whole cohort. However, in subjects with functional islets after 40 months, a shorter graft survival was observed in those with steatosis during the islet dysfunction period, even after adjustments to variables known to be associated with islet failure

Liver fat accumulation after islet transplantation and graft survival

Our objective is to evaluate if there is an association between liver fat accumulation after islet transplantation (ITx) and graft survival. A cohort study was conducted in 34 subjects with type 1 diabetes postallogeneic ITx. Liver fat content was evaluated by magnetic resonance imaging (MRI) (change in liver signal intensity on inphase and opposed-phase images). Kaplan–Meier curves and Cox regression analysis were performed with islet dysfunction duration as the dependent variable and fat liver content as an independent one. Values of p < 0.05 were significant (SSPS®18.0 and MedCalc®12.5). Patients’ mean age was 40 ± 8 years (diabetes duration: 31 ± 12 years; male: 41%). Islet survival did not differ in patients without (51 months, 95% CI 40–62 months) or with steatosis (48 months, 95% CI 38–58 months; p = 0.55) during islet dysfunction period. Nevertheless, survival curves appear to separate late in the follow-up, and after 40 months steatosis was associated with shorter graft survival (p log rank = 0.049). This association remained (RR 23.5, 95% CI 1.1–516.0; p = 0.045) after adjustments for possible confounding factors. In this sample of subjects with type 1 diabetes submitted to ITx, steatosis was not associated with islet failure in the whole cohort. However, in subjects with functional islets after 40 months, a shorter graft survival was observed in those with steatosis during the islet dysfunction period, even after adjustments to variables known to be associated with islet failure