Cystic partially differentiated nephroblastoma: a rare pediatric renal tumor-case report.

Cystic partially differentiated nephroblastoma is a rare renal tumor of childhood. It is part of a spectrum of multicystic renal tumors that also includes cystic nephroma and cystic Wilms' tumor. We present a case of cystic partially differentiated nephroblastoma, highlighting the clinical and imaging diagnostic challenge. Although the histological diagnostic criteria for all these 3 entities are well established, they are clinically and radiologically indistinguishable. Cystic partially differentiated nephroblastoma is often observed in male children under 2 years old. Typical clinical presentations include abdominal masses, abdominal pain and/or hematuria. Patients should be treated according to tumor histology and stage.

Pulmonary radiographic findings in young children with cystic fibrosis.

BACKGROUND: Pulmonary evaluation is one of the greatest challenges in children with cystic fibrosis who are younger than 6 years. Although chest CT can provide the most information for initial and progressive changes in cystic fibrosis, the radiation exposures can lead to significant cumulative exposure in children as they are followed with serial scanning to monitor early and progressive disease. Therefore the systematized study of chest radiographs using the Brasfield score has been used scientifically with the aim of evaluating the evolution of pulmonary abnormalities in children with cystic fibrosis. OBJECTIVE: This study was performed to assess the radiologic findings in children younger than 6 years with cystic fibrosis. We used the Brasfield score to compare radiographs performed in patients with Pseudomonas aeruginosa vs. oxacillin-sensitive Staphylococcus aureus and to compare radiographs in children with early vs. delayed diagnosis. MATERIALS AND METHODS: A total of 254 chest radiographs from 67 children who had undergone material cultures of the airways as part of routine care were evaluated in this cross-sectional study. The statistical analysis was performed by the Kruskal-Wallis test, with a significance level of 5%. RESULTS: Approximately 35.8% of chest radiographs had a Brasfield score lower than 21 points, which is compatible with potentially irreversible pulmonary disease. Brasfield scores decreased (this score decreases with increasing disease severity) in older children, and both bacterial colonization with Pseudomonas and later diagnosis were associated with lower (greater disease) scores. CONCLUSION: The evaluation of radiographs using the Brasfield score demonstrated the most important pulmonary findings in cystic fibrosis and identified the age group when these alterations began to appear more pronounced.

Murine model to study brain, behavior and immunity during hepatic encephalopathy.

AIM: To propose an alternative model of hepatic encephalopathy (HE) in mice, resembling the human features of the disease. METHODS: Mice received two consecutive intraperitoneal injections of thioacetamide (TAA) at low dosage (300 mg/kg). Liver injury was assessed by serum transaminase levels (ALT) and liver histology (hematoxylin and eosin). Neutrophil infiltration was estimated by confocal liver intravital microscopy. Coagulopathy was evaluated using prolonged prothrombin and partial thromboplastin time. Hemodynamic parameters were measured through tail cuff. Ammonia levels were quantified in serum and brain samples. Electroencephalography (EEG) and psychomotor activity score were performed to show brain function. Brain edema was evaluated using magnetic resonance imaging. RESULTS: Mice submitted to the TAA regime developed massive liver injury, as shown by elevation of serum ALT levels and a high degree of liver necrosis. An intense hepatic neutrophil accumulation occurred in response to TAA-induced liver injury. This led to mice mortality and weight loss, which was associated with severe coagulopathy. Furthermore, TAA-treated mice presented with increased serum and cerebral levels of ammonia, in parallel with alterations in EEG spectrum and discrete brain edema, as shown by magnetic resonance imaging. In agreement with this, neuropsychomotor abnormalities ensued 36 h after TAA, fulfilling several HE features observed in humans. In this context of liver injury and neurological dysfunction, we observed lung inflammation and alterations in blood pressure and heart rate that were indicative of multiple organ dysfunction syndrome. CONCLUSION: In summary, we describe a new murine model of hepatic encephalopathy comprising multiple features of the disease in humans, which may provide new insights for treatment.

Role of Doppler ultrasonography evaluation of superior mesenteric artery flow volume in the assessment of Crohn's disease activity / Contribuição da medida do volume de fluxo da artéria mesentérica superior pelo Doppler na caracterização da atividade inflamatória em pacientes com doença de Crohn

Objective To investigate superior mesenteric artery flow measurement by Doppler ultrasonography as a means of characterizing inflammatory activity in Crohn's disease. Materials and Methods Forty patients were examined and divided into two groups – disease activity and remission – according to their Crohn's disease activity index score. Mean superior mesenteric artery flow volume was calculated for each group and correlated with Crohn's disease activity index score. Results The mean superior mesenteric artery flow volume was significantly greater in the patients with active disease (626 ml/min ± 236 × 376 ml/min ± 190; p = 0.001). As a cut off corresponding to 500 ml/min was utilized, the superior mesenteric artery flow volume demonstrated sensitivity of 83% and specificity of 82% for the diagnosis of Crohn's disease activity. Conclusion The present results suggest that patients with active Crohn's disease have increased superior mesenteric artery flow volume as compared with patients in remission. Superior mesenteric artery flow measurement had a good performance in the assessment of disease activity in this study sample. .

Evaluation of inflammatory activity in Crohn's disease and ulcerative colitis.

Crohn's disease and ulcerative colitis evolve with a relapsing and remitting course. Determination of inflammatory state is crucial for the assessment of disease activity and for tailoring therapy. However, no simple diagnostic test for monitoring intestinal inflammation is available. Noninvasive markers give only indirect assessments of disease activity. Histopathological or endoscopical examinations accurately assess inflammatory activity, but they are invasive, time consuming and expensive and therefore are unsuitable for routine use. Imaging procedures are not applicable for ulcerative colitis. The usefulness of ultrasound and Doppler imaging in assessing disease activity is still a matter of discussion for Crohn's disease, and an increased interest in computed tomography enterograph (CTE) has been seen, mainly because it can delineate the extent and severity of bowel wall inflammation, besides detecting extraluminal findings. Until now, the available data concerning the accuracy of magnetic resonance enterography in detecting disease activity is less than CTE. Due to this, clinical activity indices are still commonly used for both diseases.

Intestinal permeability and antigliadin antibody test for monitoring adult patients with celiac disease.

Celiac disease causes chronic inflammation of the intestinal mucosa and reduces surface absorption; after the withdrawal of gluten from the diet, there are clinical and histologic improvements. The intestinal permeability test and serologic tests are useful for confirming the diagnosis and monitoring patients. The goal of this study is to compare the antigliadin antibody (AGA) test with the intestinal permeability test for celiac patients on a gluten-free diet. The sample consisted of 22 celiac patients who were antigliadin immunoglobulin A-positive before treatment. After 12 months on a gluten-free diet, AGA testing was repeated and the intestinal permeability test was performed. A control group was composed of 11 healthy individuals. AGA remained positive in 40.9% of celiac patients, and the mean urinary lactulose excretion was 10.27%, that of mannitol was 10.18%, and the lactulose/mannitol ratio was 1.02. In the subgroup in which antigliadin became negative (59.1%), the value for lactulose was 3.79%, that for mannitol was 11.12%, the lactulose/mannitol ratio was 0.38, and the p value was less than 0.0001, 0.66, and less than 0.0001, respectively. When the two celiac subgroups were compared with the control group, the urinary lactulose excretion and the lactulose/mannitol ratio was less in the control group, whereas urinary mannitol excretion was greater. The p values were less than 0.0001 for the three variables, suggesting persistent lesions in mucosa of both subgroups, although to a lesser degree for those that became AGA negative. It is concluded that intestinal permeability allows a more precise clinical physiopathologic correlation than antigliadin and offers more information for the monitoring of these patients.

Aspectos clínicos e histológicos de 34 casos de doença celíaca no adulto / Clinical and histological aspects of 34 adult celiac disease cases

A doença celíaca é uma enteropatia induzida pela dieta contendo glúten, cuja prevalência e incidência vêm aumentando progressivamente. Além das formas clássicas de apresentação da doença com a prevenção da diarréia, formas menos típicas constituem uma parcela importante em várias casuísticas. É objetivo do estudo avaliar o comportamento da doença baseado em dados clínicos e propedêuticos, sobretudo a biópsia intestinal. Para tanto, 34 pacientes foram inclídos neste estudo. Os critérios utilizados para o diagnóstico foram baseados no Consenso da Sociedade Européia de Gastroenterologia e Hepatologia Pediátrica e Nutrição, realizado em 1989. Do ponto de vista clínico, as formas de manifestação da doença foram divididas em clássica, subclínica, silenciosa e latente. Os padrões histopatológicos foram divididos em infiltrativo, hiperplásico, destrutivo e hipoplásico. A maioria absoluta dos pacientes (91,2por cento) apresentava a forma clássica da doença. A forma subclínica estava presente em apenas três pacientes e as formas silenciosa e latente não foram observadas na cauística. Os auto-amticorpos, o ácido fólico e o teste do hidrogênio expirado apresentaram níveis de sensibilidade entre 60 e 81por cento. O padrão histológico destrutivo foi o mais freqüente, perfazendo 76,5por cento dos casos: o hiperplásico, 20,6por cento; e o infiltrativo, 2,9por cento. Formas clínicas menos típicas e forma histólogicas com alterações menos exuberantes ou que não proporcionem distorções na arquitetura vilositária podem resultar em acurácia diagnóstica menor. A utilização dos auto-anticorpos e de outros exames de triagem podedem contribuir para a reversão desse cenário