Reduced circulating levels of sTWEAK are associated with NAFLD and may affect hepatocyte triglyceride accumulation.

CONTEXT: Non-alcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome and is strongly associated with obesity, dyslipidaemia and altered glucose regulation. Previous data demonstrated that low circulating levels of tumour necrosis factor weak inducer of apoptosis (sTWEAK) were associated with obesity, diabetes and insulin resistance, all traits associated with an increased risk of NALFD. Circulating sTWEAK levels are expected to be reduced in the presence of NAFLD. OBJECTIVE: We aimed to explore the relationship between NAFLD and circulating sTWEAK levels in obese patients, and to evaluate the effect of sTWEAK on hepatocyte triglyceride accumulation.Design setting and patients:This is an observational case-control study performed in n=112 severely obese patients evaluated for NAFLD by abdominal ultrasound and n=32 non-obese patients without steatosis. Serum sTWEAK concentrations were measured by ELISA. Multivariable analyses were performed to determine the independent predictors of NAFLD. We analysed TWEAK and Fn14 protein expression in liver biopsies by western blotting and immunohistochemistry. An immortalized primary human hepatocyte cell line (HHL) was used to evaluate the effect of sTWEAK on triglyceride accumulation. RESULTS: We observed a reduction in serum circulating sTWEAK concentrations with the presence of liver steatosis. On multivariable analysis, lower sTWEAK concentrations were independently associated with the presence of NAFLD (odds ratio (OR)=0.023; 95% confidence interval: 0.001-0.579; P<0.022). In human hepatocytes, sTWEAK administration reduced fat accumulation as demonstrated by the reduction in palmitic acid-induced accumulation of triglyceride and the decreased expression of cluster of differentiation 36 (CD36) and perilipin 1 and 2 (PLIN1 and PLIN2) genes. CONCLUSIONS: Decreased sTWEAK concentrations are independently associated with the presence of NAFLD. This is concordant with the observation that TWEAK reduces lipid accumulation in human liver cells.

[Neuro-Behçet: clinicopathological findings in an autopsy case]. / Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia.

INTRODUCTION: Behçet's disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in this study. CASE REPORT: A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobule extended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammation in small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. CONCLUSIONS: The necropsy studies of patients with neuro-Behcet's disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem.

Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia / Neuro-behçet: Clinicopathological findings in an autopsy case

Introducción. La enfermedad de Behçet (EB) la describió por primera vez en 1937 Hulusi Behçet como la tríada deúlceras orales, úlceras genitales y uveítis. Involucra a casi todos los tejidos y órganos sin excepción, y la tasa de afectación del sistema nervioso central es del 5 al 30%. Los estudios histopatológicos de casos de necropsias son escasos, a pesar de que la causa de muerte se constata en este estudio. Caso clínico. Varón de 30 años que comenzó con EB con afectación neurológicade evolución progresiva y fatal. El estudio autópsico mostró en el examen externo úlceras orales y escrotales. El encéfalo presentaba marcado edema cerebral, meninges opacas y un infarto hemorrágico frontal izquierdo con extensión hacia el ventrículo lateral homólogo, y el tronco del encéfalo, infartos hemorrágicos confluentes y hemorragia del cuarto ventrículo. En el examen de los órganos restantes destacaban múltiples infartos pulmonares bilaterales. El estudio histológico del encéfalo mostró, de forma generalizada, predominante en el tronco del encéfalo, vasos de pequeño y mediano calibre rodeados por una corona de linfocitos y neutrófilos, con intensa extravasación eritrocitaria perivascular. Conclusiones. Los estudios denecropsias de pacientes con neurobehçet son escasos. Presentamos los hallazgos clínicos y patológicos de un paciente con EB con afectación neurológica, en el que destaca una afectación vascular cerebral predominante en el tronco del encéfalo

Introduction. Behçet’s disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in thisstudy. Case report. A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobuleextended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammationin small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. Conclusions. The necropsy studies of patients with neuro-Behçet’s disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem

Prevalencia de la patología fúngica en biopsias y autopsias realizadas en el Hospital Universitario Joan XXIII de Tarragona / Prevalence of fungal infections detected from biopsies and autopsies in the past 11 years at the University Hospital Joan XXIII in Tarragona, Spain

La patología infecciosa, favorecida por distintos factores, es cada vez más frecuente en nuestros pacientes. Dentro de ésta, la patología debida a hongos está en aumento. Se han descrito más de 300 especies de hongos patógenos para el hombre. Para conocer la prevalencia de la patología fúngica en nuestro hospital (de nivel C y con 330 camas), se ha realizado un estudio retrospectivo de la presencia de micosis en biopsias y autopsias en los últimos once años (1994-2004). De un total de 78.310 biopsias y 753 autopsias, un 0,24% mostraron la presencia de hongos (0,21% en biopsias y 4,25% en autopsias), siendo la localización más común la piel y las mucosas (66%). De los hongos observados, un 61% correspondía al género Candida, seguido de Aspergillus (10%) y zigomicetos (5%). La patología de base más importante fue la enfermedad pulmonar obstructiva crónica, seguida de diabetes y sida. Cabe destacar la observación de un aumento del número de casos con la edad del paciente, siendo la octava década de la vida la que presentaba mayor incidencia de infección fúngica. El tratamiento mejoraba la evolución de los pacientes, si bien las patologías de base son factores muy importantes en la evolución. Las micosis sistémicas presentaban el peor pronóstico con un 91% de mortalidad. En consecuencia, un diagnóstico rápido con intervención del patólogo es muy importante de cara al pronóstico(AU)

Infectious diseases caused by fungal pathogens have increased in the past 10 years. More than 300 pathogenic fungal species have been incriminated as the etiologic agents. We carried out a retrospective study (1994-2004) to evaluate the prevalence of mycoses at the University Hospital Joan XXIII (330 beds). This report found 0.24% of the studied cases (78,310 biopsies and 753 autopsies) were diagnosed as fungal infections (0.21% of the total studied biopsy and 4.25% of the whole autopsies). Skin and mucose were involved in 66% of cases, followed by other less affected anatomical areas. 61% of studied cases were caused by Candida spp (the most frequent in our environment), followed by Aspergillus spp (10%) and the Zygomycetes (5%). The most important underlying illness was obstructive chronic pulmonary disease followed by diabetes and AIDS. The incidence of mycoses increased with the patient's age, especially those patients in their 80s. Antifungal management improved the clinical outcome of the patient but predisposing factors are crucial for diagnosis. Systemic mycoses have poor prognosis with 91% of fatal outcome. Thus, it is important to perform a rapid diagnosis of the fungal infections a diagnostic area in which pathology could play a major role(AU)

[Discrepancies between clinical and pathological diagnosis in a polyvalent intensive care service]. / Discrepancias entre el diagnóstico clínico y el anatomopatológico en un Servicio de Cuidados Intensivos Polivalente.

OBJECTIVES: Analyze the frequency and spectrum of the most relevant diseases found in the necropsic study. Assess the association between stay in Intensive Care Unit (ICU) less than 24 hours and rate of diagnostic errors. MATERIAL AND METHODS: Retrospective, observational study during a 46 month period in a polyvalent ICU. The differences between the clinical and pathological diagnoses were established based on Goldman's classification. RESULTS: A total of 85 autopsies out of 520 exitus (16.3%) were done. Five patients were excluded due to incomplete information. Of the 80 cases, we found 30 patients with major errors, 21 with therapeutic and prognostic repercussion, 9 in which the therapeutic strategy had not been modified. The most frequently found diagnosis in type I error was bacterial infection followed by cardiovascular disease. Major error rate with therapeutic repercussion was superior in patients with a stay in the ICU less than 24 hours (40% vs 21%; p < 0.05). CONCLUSIONS: Autopsy continues to be a useful tool to assess quality of clinical diagnosis. The diagnostic errors with therapeutic repercussion are bacterial infections and cardiovascular disease. Patients with a stay less than 24 hours have a higher rate of type I diagnostic errors.

Discrepancias entre el diagnóstico clínico y el anatomopatológico en un Servicio de Cuidados Intensivos Polivalente / Discrepancies between clinical and pathological diagnosis in a Polyvalent Intensive Care Service

Objetivos. Analizar la frecuencia y espectro de las patologías más relevantes encontradas en el estudio necrópsico. Valorar la asociación entre estancia en la Unidad de Cuidados Intensivos (UCI) inferior a 24 horas y la tasa de errores diagnósticos. Material y métodos. Estudio retrospectivo observacional durante un período de 46 meses en una UCI polivalente. Las diferencias entre el diagnóstico clínico y anatomopatológico se establecieron en función de la clasificación de Goldman. Resultados. Se realizaron 85 autopsias de un total de 520 exitus (16,3%). Cinco pacientes fueron excluidos por información incompleta. De los 80 casos, encontramos 30 pacientes con errores mayores, 21 con repercusión terapéutica y pronóstica, y 9 en los que la estrategia terapéutica no se hubiera modificado. El diagnóstico más frecuentemente encontrado en el error tipo I fue la infección bacteriana seguida de la patología cardiovascular. La tasa de errores mayores con repercusión terapéutica fue superior en los pacientes con una estancia en UCI inferior a 24 horas (40% frente a 21%; p < 0,05). Conclusiones. La autopsia continúa siendo una herramienta útil para evaluar la calidad del diagnóstico clínico. Los errores diagnósticos con repercusión terapéutica son las infecciones bacterianas y la patología cardiovascular. Los pacientes con una estancia en la UCI inferior a 24 horas presentan una tasa mayor de errores diagnósticos tipo I

Objectives. Analyze the frequency and spectrum of the most relevant diseases found in the necropsic study. Assess the association between stay in Intensive Care Unit (ICU) less than 24 hours and rate of diagnostic errors. Material and methods. Retrospective, observational study during a 46 month period in a polyvalent ICU. The differences between the clinical and pathological diagnoses were established based on Goldman's classification. Results. A total of 85 autopsies out of 520 exitus (16.3%) were done. Five patients were excluded due to incomplete information. Of the 80 cases, we found 30 patients with major errors, 21 with therapeutic and prognostic repercussion, 9 in which the therapeutic strategy had not been modified. The most frequently found diagnosis in type I error was bacterial infection followed by cardiovascular disease. Major error rate with therapeutic repercussion was superior in patients with a stay in the ICU less than 24 hours (40% vs 21%; p < 0.05). Conclusions. Autopsy continues to be a useful tool to assess quality of clinical diagnosis. The diagnostic errors with therapeutic repercussion are bacterial infections and cardiovascular disease. Patients with a stay less than 24 hours have a higher rate of type I diagnostic errors

Adenocarcinoma gástrico pobremente diferenciado con rasgos rabdoides vimentinanegativo y colonizado por candidas / Poor differentiated gastric adenocarcinoma with rhabdoid features vimentin-negative and colonized by candidas

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Carcinoma adenoescamoso gástrico en un paciente de raza caucásica / Adenosquamous gastric carcinoma in Caucasian patient

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