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IntroductionThe anterior elements of the spine, particularly the odontoid processes, are a rare location for osteoblastomas. Pseudomalignant osteoblastomas are themselves rare histologic types and are also extremely rare in this location. Most osteoblastomas are Enneking stage 2 lesions; less frequently, they can be more aggressive with extra-capsular extension (Enneking stage 3). En bloc resection is recommended for aggressive lesions, but the literature is less clear regarding the approach to stage 2 tumors, particularly those with pseudomalignant histologic features.Case reportA 6-year-old male child presented with a type III pathologic fracture of the odontoid. The fracture healed but upon 6-month follow-up CT scanning, an expansile lesion was detected. Surgical biopsy revealed an osteoblastoma which was treated with intralesional excision. Meanwhile, the excised specimen showed histological features of a pseudomalignant osteoblastoma. Despite this diagnosis, no further treatment was undertaken. At a 10-year follow-up, the patient was free from pain and had full range of motion of the cervical spine; no recurrence was detected.ConclusionThis unique case of odontoid osteoblastoma illustrates that malignant behavior may not be predicted only by the presence of pseudomalignant features on histology.

Nonalcoholic fatty liver disease (NAFLD) can be seen as a manifestation of overnutrition. The muscle is a central player in the adaptation to energy overload, and there is an association between fatty-muscle and -liver. We aimed to correlate muscle morphology, mitochondrial function and insulin signaling with NAFLD severity in morbid obese patients. Liver and deltoid muscle biopsies were collected during bariatric surgery in NAFLD patients. NAFLD Activity Score and Younossi's classification for nonalcoholic steatohepatitis (NASH) were applied to liver histology. Muscle evaluation included morphology studies, respiratory chain complex I to IV enzyme assays, and analysis of the insulin signaling cascade. A healthy lean control group was included for muscle morphology and mitochondrial function analyses. Fifty one NAFLD patients were included of whom 43% had NASH. Intramyocellular lipids (IMCL) were associated with the presence of NASH (OR 12.5, p<0.001), progressive hepatic inflammation (p = 0.029) and fibrosis severity (p = 0.010). There was a trend to an association between IMCL and decreased Akt phosphorylation (p = 0.059), despite no association with insulin resistance. In turn, hepatic steatosis (p = 0.015) and inflammation (p = 0.013) were associated with decreased Akt phosphoryation. Citrate synthase activity was lower in obese patients (p = 0.047) whereas complex I (p = 0.040) and III (p = 0.036) activities were higher, compared with controls. Finally, in obese patients, complex I activity increased with progressive steatosis (p = 0.049) and with a trend with fibrosis severity (p = 0.056). In morbid obese patients, presence of IMCL associates with NASH and advanced fibrosis. Muscle mitochondrial dysfunction does not appear to be a major driving force contributing to muscle fat accumulation, insulin resistance or liver disease. Importantly, insulin resistance in muscle might occur at a late point in the insulin signaling cascade and be associated with IMCL and NAFLD severity.

Background: Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease where liver biopsy remains the gold standard for diagnosis. Here we aimed to evaluate the role of circulating adiponectin, leptin, and insulin-like growth factor 1 (IGF-1) levels as non-invasive NAFLD biomarkers and assess their correlation with the metabolome. Materials and Methods: Leptin, adiponectin, and IGF-1 serum levels were measured by ELISA in two independent cohorts of biopsy-proven obese NAFLD patients and healthy-liver controls (discovery: 38 NAFLD, 13 controls; validation: 194 NAFLD, 31 controls) and correlated with clinical data, histology, genetic parameters, and serum metabolomics. Results: In both cohorts, leptin increased in NAFLD vs. controls (discovery: AUROC 0.88; validation: AUROC 0.83; p < 0.0001). The leptin levels were similar between obese and non-obese healthy controls, suggesting that obesity is not a confounding factor. In the discovery cohort, adiponectin was lower in non-alcoholic steatohepatitis (NASH) vs. non-alcoholic fatty liver (AUROC 0.87; p < 0.0001). For the validation cohort, significance was attained for homozygous for PNPLA3 allele c.444C (AUROC 0.63; p < 0.05). Combining adiponectin with specific serum lipids improved the assay performance (AUROC 0.80; p < 0.0001). For the validation cohort, IGF-1 was lower with advanced fibrosis (AUROC 0.67, p < 0.05), but combination with international normalized ratio (INR) and ferritin increased the assay performance (AUROC 0.81; p < 0.01). Conclusion: Serum leptin discriminates NAFLD, and adiponectin combined with specific lipids stratifies NASH. IGF-1, INR, and ferritin distinguish advanced fibrosis.

Somatostatin analogs (SSAs) are used as part of standard treatment for advanced neuroendocrine tumors (NETs). The mechanisms behind the antiproliferative action of SSAs remain largely unknown, but a connection with the mammalian target of rapamycin (mTOR) signaling pathway has been suggested. Our purpose was to evaluate the activation status of the AKT/mTOR pathway in advanced metastatic NETs and identify biomarkers of response to SSA therapy. Expression of phosphatase and tensin homolog (PTEN), phosphorylated (p)-AKT(Ser473), and p-S6(Ser240/244) was evaluated using immunohistochemistry in archival paraffin samples from 23 patients. Expression levels were correlated with clinicopathological parameters and progression-free survival under treatment with SSAs. A positive association between p-AKT and p-S6 expression was identified (P = 0.01) and higher expression of both markers was observed in pancreatic NETs. AKT/mTOR activation was observed without the loss of PTEN expression. Tumors showing AKT/mTOR signaling activation progressed faster when treated with SSAs: higher expression of p-AKT or p-S6 predicted a median progression-free survival of 1 month vs 26.5 months for lower expression (P = 0.02). Constitutive activation of the AKT/mTOR pathway was associated with shorter time-to-progression in patients undergoing treatment with SSAs. Larger case series are needed to validate whether p-AKT(Ser473) and p-S6(Ser240/244) can be used as prognostic markers of response to therapy with SSAs.

Prospective studies concerning liver disease in pediatric cystic fibrosis patients are scarce. The present study aimed to describe the prevalence and clinical expression of cystic fibrosis - related liver disease, in a cohort of 62 pediatric patients. Descriptive study, resulting from the prospective evaluation, between 1994 and 2009, of 62 pediatric patients (age <18 years) with cystic fibrosis. The follow-up protocol included a clinical assessment every 2 months, liver function tests every 6 months and annual liver ultrasonography. The cumulative prevalence of liver disease was 11.2% (7/62 cases). All patients had ΔF508 mutation and pancreatic insufficiency, none had meconium ileus. The liver involvement became clinically evident at a mean age of 8 years (3-15 years), revealed by hepatomegaly or hepatosplenomegaly (3 cases) and/ or abnormalities of liver function tests (3 cases) changes of liver ultrasound (7 cases) with evidence of portal hypertension (2 cases). Four patients were submitted to liver biopsy; biliary fibrosis was documented in one case, focal biliary cirrhosis in 2 cases and multilobular cirrhosis in another case. Within a median 11.6 years follow-up period (all patients under UDCA therapy after liver disease diagnosis), progression of liver disease was observed in 2 patients; one patient developed refractory variceal bleeding and progressive hepatic failure, requiring liver transplant. The results of the present study agree with those of previous pediatric studies, further documenting clinical expression of liver disease in CF patients, which is usually detected in the first decade of life and emphasize the contribution of ultrasound to early diagnosis of liver involvement. Moreover, although advanced liver disease is a relatively rare event, early isolated liver transplantation may have to be considered at this age group.

Our objective was to evaluate the histopathological features of chronic hepatitis C of 64 liver biopsies and to correlate this with the route of transmission of hepatitis C virus, the genotype of HCV, and the patient's age. Moderate chronic hepatitis was the most frequently observed (62.5%). Cirrhosis was observed in 14 patients (21.9%) and was more frequently found among patients over 40 years of age (34.3% vs. 6.9%, P = 0.025). The mean histopathological activity index (HAI) was significantly higher in the sporadic (10+/-3.1) than the posttransfusional (7.5+/-3.7) and the intravenous drug use (IVDU) groups (6.3+/-2.8) (P<0.02). Moreover the sporadic group showed more fibrosis (P<0.04) than the posttransfusional group. No liver cirrhosis was found in the IVDU group. The overall prevalence of HCV variants was: 54.7% type 1b, 4.6% type 1a, 37.5% type 2c, 1.6% type 2b, 1.6% type 2. The genotype distribution showed no relation to the HAI, hepatitis activity (grade), and fibrosis (stage) of the liver disease. In conclusion, the sporadic route of transmission of HCV was related to a more severe chronic hepatic disease, a finding that could influence future antiviral therapies. The predominance of HCV type 1b in this study reflects the higher frequency of this variant in our area. Our data suggests that the ultimate consequence of HCV chronic infection depends on patient age rather than on HCV genotype.

O problema da qualidade nos serviços prestados é visível em todo o tipo de empresas. Actualmente são vários os estudos dirigidos a diversas companhias aéreas, no sentido de determinar dimensões de qualidade do serviço e perceber quais delas têm maior impacto sobre a percepção dos clientes. Com este trabalho procuramos analisar e avaliar as percepções dos consumidores, o que requeria identificar as dimensões da qualidade de serviço e a percepção dos consumidores da TAAG - Linhas Aéreas de Angola, tendo como base diversos trabalhos por nós analisados. Apresentando-se como relevante pelo facto de existirem poucos (ou nenhum) trabalhos sobre a companhia aérea por nós seleccionada. Para o efeito, foi aplicado um questionário adaptado para os transportes aéreos baseado no modelo SERVPERF. Com a ajuda do Software SPSS, aplicámos a análise factorial e em seguida os testes da ANOVA e Qui-Quadrado para responder às hipóteses colocadas. Os resultados mostram cinco dimensões da qualidade percebida do serviço: protecção e segurança, comodidade (bem-estar), fiabilidade, prestatividade e empatia. Destes factores, apenas a fiabilidade e protecção e segurança têm um impacto positivo na percepção da qualidade, sendo a fiabilidade a mais importante e a protecção e segurança vista como a menos importante. O que torna os nossos resultados diferentes dos apresentados por outros autores. Por outro lado, a percepção da qualidade do serviço varia apenas em termos de frequência de voo, idade e rendimento. Quanto às outras variáveis: género, nível educacional, ocupação e estado civil, não parecem exercer influência significativa na percepção da qualidade do serviço prestado.

Este trabalho aborda a avaliação da satisfação dos estudantes como elemento fundamental para que as Instituições de Ensino Superior (IES) possam identificar os fatores considerados relevantes pelos estudantes para a escolha de uma IES, bem como os fatores ligados à experiência académica a que estes dão maior importância, e com os quais estão mais ou menos satisfeitos. Isso permitirá às IES traçar estratégias que permitam corrigir possíveis erros, melhorando assim a qualidade dos seus serviços, sendo estes, os principais objetivos do trabalho.Este estudo apresenta-se de extrema relevância uma vez que não se verificam estudos dirigidos especificamente aos estudantes, que busquem recolher a informação sobre os diversos serviços prestados pelas IES, e também pelo fato da informação destes ser relevante para a melhoria do sistema de ensino das instituições em particular, e do país, em geral.Para atingir estes objetivos e poder responder às questões de pesquisa, foi utilizado o método misto, em que se procurou através de ferramentas de âmbito qualitativo validar os resultados da pesquisa quantitativa, e teve ainda um carácter exploratório e descritivo. Da população – os estudantes da VIª Região Académica – extraiu-se um grupo 1032 estudantes para responderem a um questionário estruturado, mas destes, apenas puderam ser validados 255 questionários.Os resultados revelam que os estudantes consideram como sendo o fator mais importante para entrar no ensino superior a possibilidade de obter conhecimentos que permitam uma carreira aliciante, sendo o fator “Ter uma boa reputação” a razão da escolha de determinada instituição em particular. Embora estes resultados sejam semelhantes a alguns estudos realizados em outros países, foi possível notar a existência de um gap entre a importância e satisfação dos estudantes para com os fatores ligados a experiência académica.

MARIANA VERDELHO MACHADO, DUARTE M.S. FERREIRA, RUI E. CASTRO, ANA RITA SILVESTRE, TERESINHA EVANGELISTA, JOÃO COUTINHO, FÁTIMA CAREPA, ADÍLIA COSTA, CECÍLIA M. P. RODRIGUES, and HELENA CORTEZ-PINTO7.1 INTRODUCTIONNonalcoholic fatty liver disease (NAFLD) is a condition characterized by fat accumulation in the liver, not related with alcohol consumption. It represents a wide spectrum of pathological subgroups, from benign simple steatosis to nonalcoholic steatohepatitis (NASH), which can progress to hepatic cirrhosis [1], and is associated with overall and liver-related increased mortality [2]. Two of the main risk factors for developing NAFLD are insulin resistance and obesity, in which the peripheral adipose tissue reservoir capacity is overwhelmed, allowing ectopic fat accumulation.

Our objective was to estimate the effect of interferon (IFN) on the evolution of fibrosis in chronic hepatitis C and the significance of the N-terminal propeptide of procollagen type III (PIIIP) as a marker of fibrogenesis. One hundred seventeen patients, 72 male (61%) and 45 female (39%), with a mean age of 40.7+/-11.9 years were treated with a2b-IFN, 3 to 5 MU, for 12 months: sustained responders (SR = 44), relapsers (RR = 35), and nonresponders (NR = 38). Liver biopsies were performed before treatment and 1 year after cessation of IFN for evaluation of the histological activity index (HAI). Serum PIIIP was obtained at the time of liver biopsy, at the beginning, during, and end of therapy and during the follow-up. The normal value in 29 healthy individuals was 0.37+/-0.18 U/L. Staging was reduced in 58% of SR, 12.5% of RR, and 11.5% of NR. There was a correlation between PIIIP and the HAI before (n = 71, r(s) = 0.41, P < 0.0004) and after IFN (n = 71, r(s) = 0.58, P < 0.0001). The SR had a better improvement in grading (90.3%; P < 0.05) and staging (58%; P < 0.001). The correlation of the HAI parameters with the variation of PIIIP showed significance only for fibrosis (r(s) = 0.36, P < 0.002) and portal inflammation (r(s) = 0.35, P < 0.01). PIIIP normalized only in patients whose fibrosis improved (P < 0.01). At the end of therapy, PIIIP had a predictive value in the distinction of SR from RR (PPV, 64; PNV, 55.6). During the follow-up, PIIIP remained lower in SR compared with RR and NR (P < 0.002). The response to a-IFN improved liver inflammation and fibrosis. Serum PIIIP is a useful noninvasive method to evaluate serially fibrogenesis in chronic hepatitis C treated with IFN.