Avances en tumores del estroma gastrointestinal: ¿hacia dónde vamos?

Gastrointestinal Stromal Sarcomas (GIST) are mesenchymal neoplasms whose incidence accounts for 1-2% of digestive tumors, being located in the stomach (55-60%) and small intestine (30%). The advances in its knowledge and management succeeded in the last years have being spectacular. This review aims to summarize the most important of them for surgeons. We identified four areas of interest: molecular oncology, laparoscopic approach, management of GIST located at unusual locations, and management of advanced GIST. Advances in the field of molecular oncology lead to the discovery of new oncogenic mutations making the term Wil Type GIST obsolete. Moreover, these advances allow for the development of 2 new drugs: Avapritinib and Ripretinib, that added to the previous 3 commercially available drugs (imatinib, sunitinib and regorafenib) make possible the management of GIST with resistant mutations. The principles of the surgical management of primary GIST are well stablished which laparoscopic approach must accomplish. This approach is limited by 2 main factors: location and size. The diagnosis of GIST in unusual locations as esophagus, duodenum, rectum of out of the gastrointestinal tract (EGIST), implies an extraordinary therapeutic challenge, being imperative to manage them by surgeons and oncologist among others in the setting of a multidisciplinary team. The management of advanced/metastatic GIST has changed in a revolutionary fashion because surgery is now part of its treatment as adjuvant to tyrosine kinase inhibitors.

Los tumores del estroma gastrointestinal (GIST) suponen el 1-2% de los tumores digestivos, siendo su localización más frecuente el estómago (55-60%) y el intestino delgado (30%). Los avances más importantes sucedidos en los últimos años se centran en cuatro áreas: biología molecular, abordaje quirúrgico laparoscópico, manejo técnico del GIST en localizaciones inusuales y tratamiento e integración de la cirugía en el manejo del GIST avanzado. Los avances en el conocimiento de la biología molecular del GIST han dado lugar a la progresiva identificación de nueva mutaciones oncogénicas que hacen del concepto wild type obsoleto. Estos avances han permitido el desarrollo de dos nuevos fármacos, avapritinib y ripretinib, lo que permite el tratamiento de pacientes con mutaciones resistentes a las tres líneas terapéuticas clásicas. El tratamiento quirúrgico del GIST se rige por unos principios técnicos bien establecidos que el abordaje laparoscópico debe cumplir, abordaje que queda limitado por dos factores clave: localización y tamaño. El GIST de localización infrecuente (esófago, duodeno o recto, o extradigestivo) supone un reto terapéutico. Estos pacientes deben ser manejados en un contexto multidisciplinario. La cirugía queda integrada en el manejo del GIST avanzado, considerándose como adyuvante a los inhibidores de la tirosina cinasa.

Simultaneous pancreas-kidney transplantation: Early complications and long-term outcomes - a single-center experience.

INTRODUCTION: This study aimed to assess the prevalence and severity of complications after simultaneous pancreas-kidney transplantation (SPKT) and to evaluate its influence on both grafts' long-term results. METHODS: This was an observational, retrospective study including 39 consecutive SPKT cases from 2000-2018. Complications were classified into kidney-related and pancreas-related. The severity of complications was assessed using the modified Clavien-Dindo scale. Kaplan-Meier curve analysis and log-rank tests were used. Cox regression was performed for the multivariate analysis. RESULTS: All 39 recipients had long-term type I diabetes. Twenty-one (53.8%) patients suffered a Clavien-Dindo ≥IIIa complication. Most complications were pancreas-related, with 17 (43.6%) patients suffering from one. Kidney-related major complications were seen in 11 (28.2%) patients. Patient survival at one, five, and 15 years was 89.7%, 87.1%, and 83.9%, respectively; kidney survival was 87.1%, 81.4%, and 73.6%, respectively; and pancreas survival was 76.9%, 71.3%, and 72%, respectively. Pancreas graft survival was influenced by the presence of major postoperative complications; patients and kidney graft survival were not. CONCLUSIONS: Complications after SPKT influence pancreas graft survival. Despite the high rate of complications, our results suggest that patient and kidney graft survival may not be affected by complications.

Controlled donation after circulatory death up to 80 years for liver transplantation: Pushing the limit again.

Our main objective was to compare liver transplant (LT) results between donation after circulatory death (DCD) and donation after brainstem death (DBD) in our hospital and to analyze, within the DCD group, the influence of age on the results obtained with DCD donors aged >70 years and up to 80 years. All DCD-LTs performed were analyzed prospectively. The results of the DCD group were compared with those of a control group who received a DBD-LT immediately after each DCD-LT. Later, the results obtained within the DCD group were analyzed according to the age of the donors, considering 2 subgroups with a cut-off point at 70 years. Survival results for LT with DCD and super rapid recovery were not inferior to those obtained in a similar group of patients transplanted with DBD livers. However, the cost of DCD was a higher rate of biliary complications, including ischemic cholangiopathy. Donor age was not a negative factor.

Identification of two clusters within schizophrenia with different structural, functional and clinical characteristics.

BACKGROUND: Several biologically distinct subgroups may coexist within schizophrenia, which may hamper the necessary replicability to translate research findings into clinical practice. METHODS: Cortical thickness, curvature and area values and subcortical volumes of 203 subjects (121 schizophrenia patients, out of which 64 were first episodes), 60 healthy controls and 22 bipolar patients were used to identify clusters using principal components and canonical discriminant analyses. Regional glucose metabolism using positron emission tomography, P300 event related potential, baseline clinical data and percentage of improvement with treatment were used to validate possible clusters based on MRI data. RESULTS: All the controls, the bipolar patients and most of the schizophrenia patients were grouped in a cluster (cluster A). A group of 24 schizophrenia patients (12 first episodes), characterized by large intrinsic curvature values, was identified (cluster B). These patients, but not those in cluster A, showed reduced thalamic and cingulate glucose metabolism in comparison to controls, as well as a worsening of negative symptoms at follow-up. Patients in cluster A showed a significant putaminal metabolic increase, which was not observed for those in cluster B. P300 amplitude was reduced in patients of both clusters, in comparison to controls. CONCLUSIONS: Results of this study support the existence of a biologically distinct group within the schizophrenia syndrome, characterized by increased cortical curvature values, reduced thalamic and cingulate metabolism, lack of the expected increased putaminal metabolism with antipsychotics and persistent negative symptoms.

Greater clinical and cognitive improvement with clozapine and risperidone associated with a thinner cortex at baseline in first-episode schizophrenia.

Cortical thickness may be useful as a treatment response predictor in first-episode (FE) patients with schizophrenia, although this possibility has been scarcely assessed. In this study we assessed the possible relation between cortical thickness in regions of interest selected because of previously reported structural alterations in schizophrenia and clinical and cognitive changes after two years of treatment with risperidone or clozapine in 31 neuroleptic-naïve FE patients with schizophrenia (16 of them treated with clozapine and 15 with risperidone). Using the last-observation-carried-forward (LOCF), a larger improvement in positive, negative and total symptoms was predicted by the amount of baseline cortical thinning in the right prefrontal cortex (pars orbitalis). After two years of treatment, cognitive status was reassessed in the 17 patients (11 on clozapine) who had not dropped out. Working memory improvement after reassessment was associated with a greater baseline cortical thinning in the left prefrontal cortex (pars orbitalis), and verbal memory improvement with a greater baseline cortical thinning in the left pars triangularis. Significant but weak cortical thickness decrease from baseline to follow-up was observed in patients in comparison to controls (left pars triangularis and opercularis, and left caudal middle frontal areas). These results may support a positive predictive role for cortical thinning in the frontal region with regard to clinical and cognitive improvement with clozapine and risperidone in FE patients with schizophrenia.

Is there a bilingual advantage in the ANT task? Evidence from children.

Bilinguals have been shown to outperform monolinguals in a variety of tasks that do not tap into linguistic processes. The origin of this bilingual advantage has been questioned in recent years. While some authors argue that the reason behind this apparent advantage is bilinguals' enhanced executive functioning, inhibitory skills and/or monitoring abilities, other authors suggest that the locus of these differences between bilinguals and monolinguals may lie in uncontrolled factors or incorrectly matched samples. In the current study we tested a group of 180 bilingual children and a group of 180 carefully matched monolinguals in a child-friendly version of the ANT task. Following recent evidence from similar studies with children, our results showed no bilingual advantage at all, given that the performance of the two groups in the task and the indices associated with the individual attention networks were highly similar and statistically indistinguishable.

Action sentences activate sensory motor regions in the brain independently of their status of reality.

Some studies have reported that understanding concrete action-related words and sentences elicits activations of motor areas in the brain. The present fMRI study goes one step further by testing whether this is also the case for comprehension of nonfactual statements. Three linguistic structures were used (factuals, counterfactuals, and negations), referring either to actions or, as a control condition, to visual events. The results showed that action sentences elicited stronger activations than visual sentences in the SMA, extending to the primary motor area, as well as in regions generally associated with the planning and understanding of actions (left superior temporal gyrus, left and right supramarginal gyri). Also, we found stronger activations for action sentences than for visual sentences in the extrastriate body area, a region involved in the visual processing of human body movements. These action-related effects occurred not only in factuals but also in negations and counterfactuals, suggesting that brain regions involved in action understanding and planning are activated by default even when the actions are described as hypothetical or as not happening. Moreover, some of these regions overlapped with those activated during the observation of action videos, indicating that the act of understanding action language and that of observing real actions share neural networks. These results support the claim that embodied representations of linguistic meaning are important even in abstract linguistic contexts.

Discourse-based emotional consistency modulates early and middle components of event-related potentials.

In this study, participants read stories describing emotional episodes with either a positive or negative valence (Experiment 1). Following each story, participants were exposed to short sentences referring to the protagonist, and the event-related potential (ERP) for each sentence's last word was recorded. Some sentences described the protagonist's emotion, either consistent or inconsistent with the story; others were neutral; and others involved a semantically anomalous word. Inconsistent emotions were found to elicit larger N100/P200 and N400 than consistent emotions. However, when participants were exposed to the same critical sentences in a control experiment (Experiment 2) in which the stories had been removed, emotional consistency effects disappeared in all ERP components, demonstrating that these effects were discourse-level phenomena. By contrast, the ordinary N400 effect for locally anomalous words in the sentence was obtained both with and without story context. In conclusion, reading stories describing events with emotional significance determines strong and very early anticipations of an emotional word.

Subcortical and cortical gray matter differences between Kraepelinian and non-Kraepelinian schizophrenia patients identified using voxel-based morphometry.

The long-term outcome of schizophrenia patients may differ depending on their brain structure. This would be reflected in significant structural differences between poor-outcome (i.e., Kraepelinian) and non-Kraepelinian patients. To assess this possibility, we have evaluated the degree of deviation in brain structure in Kraepelinian patients with respect to controls and non-Kraepelinian schizophrenia patients. We used voxel-brain morphometry (VBM) to assess the differences in gray matter volume across the brain in the Kraepelinian group with respect to the healthy controls and non-Kraepelinian patients. Twenty-six Kraepelinian and 18 non-Kraepelinian schizophrenia patients and 41 healthy controls were included. With respect to the healthy controls, the Kraepelinian patients showed a very significant decrease in gray matter in the frontal, occipital, and limbic cortices, and, at a subcortical level, bilaterally in the striatum and thalamus. In comparison with the non-Kraepelinian patients, the Kraepelinian individuals continued to show a similar subcortical decrease. Thus, Kraepelinian patients may be characterized by a distinct pattern of brain abnormalities, in particular, in subcortical regions.

Técnica de Nikaidoh para la transposición de grandes arterias con estenosis pulmonar y comunicación interventricular / Nikaidoh procedure for the correction of transposition of the great arteries, ventricular septal defect and pulmonary stenosis

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[Experience with the Norwood operation for hypoplastic left heart syndrome]. / Resultados de la intervención de Norwood para el síndrome del corazón izquierdo hipoplásico.

INTRODUCTION AND OBJECTIVES: To describe our experience and to identify risk factors for in-hospital mortality. METHODS: Between October 1991 and June 2005, 42 children underwent the Norwood procedure. In the first 30 patients, pulmonary circulation was established using a modified Blalock-Taussig shunt (Group 1), while a right ventricle to pulmonary artery conduit was used in the remaining 12 (Group 2). Preoperative anatomic features and procedural factors were analyzed with respect to their impact on mortality. Postoperatively, data were collected on arterial blood pressure, arterial and venous oxygen saturation, arterial pH, venous pCO2, the PaO2/FiO2 ratio, tissue oxygen extraction, and dead space fraction. The association between each individual variable and mortality was investigated. RESULTS: Thirty patients (71.4%) had both aortic and mitral atresia, eight (19%) had either aortic or mitral atresia, and four (9.5%) had no valvular atresia. There was no statistically significant difference in postoperative mortality between the groups 1 and 2 (12/22 [54.5%] vs 7/12 [58.3%]; P=.56). The only significant risk factor for in-hospital mortality was a longer cardiopulmonary bypass time (P=.01) and, for intraoperative mortality, primary rather than delayed sternal closure (P=.004). Venous pCO2, the mean dead space fraction, and tissue oxygen extraction all tended to be higher among infants who died, but the difference was not statistically significant. CONCLUSIONS: Use of a right ventricle to pulmonary artery conduit did not improve postoperative survival. Both a long cardiopulmonary bypass time and primary sternal closure were associated with increased mortality.

Resultados de la intervención de Norwood para el síndrome del corazón izquierdo hipoplásico / Experience with the norwood operation for hypoplastic left heart syndrome

Introducción y objetivos. Describir nuestra experiencia e identificar factores de riesgo de mortalidad hospitalaria. Métodos. Entre octubre de 1991 y junio de 2005 intervinimos a 42 niños con la técnica de Norwood. Los 30 primeros recibieron una fístula de Blalock-Taussig (grupo 1) y los 12 restantes, un conducto entre el ventrículo derecho y la arteria pulmonar (grupo 2). Se analizaron los factores anatómicos y de la técnica con respecto a la mortalidad. Se recogieron variables del postoperatorio, incluidas la presión arterial, la saturación arterial y venosa de oxígeno, el pH arterial, la pCO2 venosa, la relación PaO2/FiO2, la extracción tisular de oxígeno y el espacio muerto, para estudiar su asociación con la mortalidad. Resultados. En total, 30 (71,4%) pacientes tenían atresia aórtica y mitral; 8 (19%) tenían atresia aórtica o mitral y 4 (9,5%) no tenían atresia. No hubo diferencias significativas en la mortalidad postoperatoria entre los grupos 1 y 2 (12/22 [54,5%] frente a 7/12 [58,3%]; p = 0,56). El único factor de riesgo de mortalidad hospitalaria fue un tiempo de circulación extracorpórea prolongado (p = 0,01), y el de mortalidad intraoperatoria, el cierre primario del esternón (p = 0,004). La pCO2 venosa, el espacio muerto pulmonar y la extracción tisular de oxígeno fueron superiores en los niños fallecidos, pero las diferencias no fueron significativas. Conclusiones. El uso de un conducto entre el ventrículo derecho y la arteria pulmonar no mejoró la supervivencia postoperatoria. Un tiempo de circulación extracorpórea prolongado y el cierre primario del esternón se asociaron con un aumento de la mortalidad (AU)

Introduction and objectives. To describe our experience and to identify risk factors for in-hospital mortality. Methods. Between October 1991 and June 2005, 42 children underwent the Norwood procedure. In the first 30 patients, pulmonary circulation was established using a modified Blalock-Taussig shunt (Group 1), while a right ventricle to pulmonary artery conduit was used in the remaining 12 (Group 2). Preoperative anatomic features and procedural factors were analyzed with respect to their impact on mortality. Postoperatively, data were collected on arterial blood pressure, arterial and venous oxygen saturation, arterial pH, venous pCO2, the PaO2/FiO2 ratio, tissue oxygen extraction, and dead space fraction. The association between each individual variable and mortality was investigated. Results. Thirty patients (71.4%) had both aortic and mitral atresia, eight (19%) had either aortic or mitral atresia, and four (9.5%) had no valvular atresia. There was no statistically significant difference in postoperative mortality between the groups 1 and 2 (12/22 [54.5%] vs 7/12 [58.3%]; P=.56). The only significant risk factor for in-hospital mortality was a longer cardiopulmonary bypass time (P=.01) and, for intraoperative mortality, primary rather than delayed sternal closure (P=.004). Venous pCO2, the mean dead space fraction, and tissue oxygen extraction all tended to be higher among infants who died, but the difference was not statistically significant. Conclusions. Use of a right ventricle to pulmonary artery conduit did not improve postoperative survival. Both a long cardiopulmonary bypass time and primary sternal closure were associated with increased mortality (AU)

A database for medical image management.

MEDIMAN (Medical Image MANagement) is a web information system (WIS) for medical image management and processing currently used by neuroscientists and clinicians at several medical and research centres in Spain for research and clinical trials. While developing the MEDIMAN database (DB) we encountered several design challenges unlike those arising in traditional DBs. This paper describes the development of MEDIMAN focusing on the database and the use of the database development process proposed in Midas, a model-driven framework for WIS development. Special attention is given to the design decisions made at each stage to address the challenges encountered.

Neuroimagen en los trastornos del movimiento / Neuroimaging in movement disorders

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[Risk factors associated with arterial switch operation for transposition of the great arteries]. / Factores de riesgo de la corrección anatómica para la transposición de grandes arterias.

INTRODUCTION AND OBJECTIVES: The present study was undertaken to determine the risk factors for early mortality following an arterial switch operation. PATIENTS AND METHOD: From January 1994 through October 2003, 78 pediatric patients underwent surgical repair. Simple transposition was present in 48 patients (61.5%), 29 (37.2%) had an associated ventricular septal defect, and one had a Taussig-Bing anomaly. The risk factors analyzed were: the patient's age and weight at the time of the intervention, repair of a coexisting ventricular septal defect, coronary artery anatomical pattern, duration of cardiopulmonary bypass, duration of aortic cross-clamping, and duration of circulatory arrest. All factors were evaluated for strength of association with the duration of mechanical ventilation, the length of intensive care unit stay, and mortality. RESULTS: Overall, the early mortality rate was 9% (7/78). Some 14 patients (17.9%) underwent simultaneous repair of a ventricular septal defect. Patients with an intramural coronary artery (n=3, 3.8%) or a single coronary ostium (n=5, 6.4%) were the only ones who had a significant (P<.05) mortality risk, at 50% (4/8). Circulatory arrest was implemented in 53 (68%) patients. There were significant correlations between the duration of circulatory arrest and the ventilator support time (r=0.3, P<.05) and the duration of stay in the intensive care unit (r=0.3, P<.05). CONCLUSIONS: The risk of early death was increased when more complex coronary artery anatomical variants were present. As the period of circulatory arrest lengthened, the mechanical ventilation time and duration of intensive care unit stay increased.

Factores de riesgo de la corrección anatómica para la transposición de grandes arterias / Risk factors associated with arterial switch operation for transposition of the great arteries

Introducción y objetivos. Este estudio se realizó para determinar los factores de riesgo que pueden influir en la mortalidad precoz después de la corrección anatómica. Pacientes y método. Entre enero de 1994 y octubre de 2003 intervenimos a 78 pacientes; 48 (61,5%) eran transposiciones simples, 29 (37,2%) presentaban asociada una comunicación interventricular y 1 tenía una anomalía de Taussing-Bing. Se analizaron la edad y el peso en el momento de la intervención, el cierre o no de la comunicación interventricular, la anatomía coronaria y los tiempos de circulación extracorpórea, la anoxia miocárdica y la parada circulatoria. Evaluamos la relación entre estas variables con los tiempos de ventilación mecánica, la estancia en la unidad de cuidados intensivos pediátricos y la mortalidad. Resultados. De los 78 niños fallecieron 7 (9%). En 14 (17,9%) se cerró, además, una comunicación interventricular. Los que presentaron una arteria coronaria intramural (n = 3, 3,8%) o tenían un orificio coronario único (n = 5, 6,4%) fueron los que tuvieron una mayor mortalidad (4/8, 50%) (p < 0,05). En 53 niños (68%) se realizó parada circulatoria; el tiempo de parada se correlacionó de forma directa tanto con las horas de ventilación mecánica (r = 0,3; p < 0,05) como con los días de estancia (r = 0,3; p < 0,05). Conclusiones. Las variantes más complejas en la anatomía coronaria se asociaron con un mayor riesgo de muerte precoz. La duración de la parada circulatoria influyó en los tiempos de ventilación mecánica y en la estancia en cuidados intensivos

Introduction and objectives. The present study was undertaken to determine the risk factors for early mortality following an arterial switch operation. Patients and method. From January 1994 through October 2003, 78 pediatric patients underwent surgical repair. Simple transposition was present in 48 patients (61.5%), 29 (37.2%) had an associated ventricular septal defect, and one had a Taussig-Bing anomaly. The risk factors analyzed were: the patient's age and weight at the time of the intervention, repair of a coexisting ventricular septal defect, coronary artery anatomical pattern, duration of cardiopulmonary bypass, duration of aortic cross-clamping, and duration of circulatory arrest. All factors were evaluated for strength of association with the duration of mechanical ventilation, the length of intensive care unit stay, and mortality. Results. Overall, the early mortality rate was 9% (7/78). Some 14 patients (17.9%) underwent simultaneous repair of a ventricular septal defect. Patients with an intramural coronary artery (n=3, 3.8%) or a single coronary ostium (n=5, 6.4%) were the only ones who had a significant (P<.05) mortality risk, at 50% (4/8). Circulatory arrest was implemented in 53 (68%) patients. There were significant correlations between the duration of circulatory arrest and the ventilator support time (r=0.3, P<.05) and the duration of stay in the intensive care unit (r=0.3, P<.05). Conclusions. The risk of early death was increased when more complex coronary artery anatomical variants were present. As the period of circulatory arrest lengthened, the mechanical ventilation time and duration of intensive care unit stay increased