Understanding risks and consequences of pathogen infections on the physiological performance of outmigrating Chinook salmon.

The greatest concentration of at-risk anadromous salmonids is found in California (USA)-the populations that have been negatively impacted by the degradation of freshwater ecosystems. While climate-driven environmental changes threaten salmonids directly, they also change the life cycle dynamics and geographic distribution of pathogens, their resulting host-pathogen interactions and potential for disease progression. Recent studies have established the correlation between pathogen detection and salmonid smolt mortality during their migration to the ocean. The objective of the present study was to screen for up to 47 pathogens in juvenile Chinook salmon (Oncorhynchus tshawytscha) that were held in cages at two key sites of the Sacramento River (CA, USA) and measure potential consequences on fish health. To do so, we used a combination of transcriptomic analysis, enzymatic assays for energy metabolism and hypoxia and thermal tolerance measures. Results revealed that fish were infected by two myxozoan parasites: Ceratonova shasta and Parvicapsula minibicornis within a 2-week deployment. Compared to the control fish maintained in our rearing facility, infected fish displayed reduced body mass, depleted hepatic glycogen stores and differential regulation of genes involved in the immune and general stress responses. This suggests that infected fish would have lower chances of migration success. In contrast, hypoxia and upper thermal tolerances were not affected by infection, suggesting that infection did not impair their capacity to cope with acute abiotic stressors tested in this study. An evaluation of long-term consequences of the observed reduced body mass and hepatic glycogen depletion is needed to establish a causal relationship between salmon parasitic infection and their migration success. This study highlights that to assess the potential sublethal effects of a stressor, or to determine a suitable management action for fish, studies need to consider a combination of endpoints from the molecular to the organismal level.

Improving cause of death certification in the Philippines: implementation of an electronic verbal autopsy decision support tool (SmartVA auto-analyse) to aid physician diagnoses of out-of-facility deaths.

BACKGROUND: The majority of deaths in the Philippines occur out-of-facility and require a medical certificate of cause of death by Municipal Health Officers (MHOs) for burial. MHOs lack a standardised certification process for out-of-facility deaths and when no medical records are available, certify a high proportion of ill-defined causes of death. We aimed to develop and introduce SmartVA Auto-Analyse, a verbal autopsy (VA) based electronic decision support tool in order to assist the MHOs in certifying out-of-facility deaths. METHOD: We conducted a stakeholder consultation, process mapping and a pre-test to assess feasibility and acceptability of SmartVA Auto-Analyse. MHOs were first asked to conduct an open-ended interview from the family members of the deceased, and if they were not able to arrive at a diagnosis, continue the interview using the standardised SmartVA questionnaire. Auto-Analyse then presented the MHO with the three most likely causes of death. For the pilot, the intervention was scaled-up to 91 municipalities. We performed a mixed-methods evaluation using the cause of death data and group discussions with the MHOs. RESULTS: Of the 5649 deaths registered, Auto-Analyse was used to certify 4586 (81%). For the remaining 19%, doctors believed they could assign a cause of death based on the availability of medical records and the VA open narrative. When used, physicians used the Auto-Analyse diagnosis in 85% of cases to certify the cause of death. Only 13% of the deaths under the intervention had an undetermined cause of death. Group discussions identified two themes: Auto-Analyse standardized the certification of home deaths and assisted the MHOs to improve the quality of death certification. CONCLUSION: Standardized VA combined with physician diagnosis using the SmartVA Auto-Analyse support tool was readily used by MHOs in the Philippines and can improve the quality of death certification of home deaths.

Relapse rate and renal prognosis in ANCA-associated vasculitis according to long-term ANCA patterns.

Long-term observation of patients with ANCA-associated vasculitis (AAV) allows the identification of different longitudinal patterns of ANCA levels during follow-up. This study aimed to characterize these patterns and to determine their prognostic significance. All ANCA determinations performed in two university hospitals during a 2-year period were retrospectively reviewed. Patients were included in the analysis if they had high titers of anti-myeloperoxidase (anti-MPO) or anti-proteinase 3 (anti-PR3) antibodies at least once, ≥ 5 serial ANCA determinations and AAV diagnosed by biopsy or American College of Rheumatology (ACR) classification criteria. Patients' time-course ANCA patterns were classified as monophasic, remitting, recurrent or persistent. Associations between ANCA patterns and prognostic variables (relapse rate and renal outcome) were analysed by univariate and multivariate statistics. A total of 99 patients [55 with microscopic polyangiitis (MPA), 36 with granulomatosis with polyangiitis (GPA) and eight with eosinophilic granulomatosis with polyangiitis (EGPA)] were included. Median follow-up was 9 years. Among patients diagnosed with MPA or GPA, recurrent or persistent ANCA patterns were associated with a higher risk of clinical relapse [hazard ratio (HR) = 3·7, 95% confidence interval (CI) = 1·5-9·1 and HR = 2·9, 95% CI = 1·1-8·0, respectively], independently of clinical diagnosis or ANCA specificity. In patients with anti-MPO antibodies, the recurrent ANCA pattern was associated with worsening renal function [odds ratio (OR) = 5·7, 95% CI = 1·2-26·0]. Recurrent or persistent ANCA patterns are associated with a higher risk of clinical relapse. A recurrent ANCA pattern was associated with worsening renal function in anti-MPO-associated vasculitis.

Is there a link between depression, neurochemical asymmetry and cardiovascular function?

Although at present depression is one of the most disabling disorders in our social environment, the understanding of its pathogenesis and the resources for its treatment are still unsatisfactory. The importance of brain asymmetry in the pathogenesis of disorders in brain function, including mood disorders such as depression, is a highly unexplored, sometimes underrated, and even ignored topic. It is important to note that the basal and pathological functional lateralization must have an underlying neurochemical substrate. It is also necessary to indicate that the brain asymmetry extends to a neurovisceral integration whose behavior may also be lateralized. One of the most studied axis from the functional point of view is the brain-heart connection, in whose operation there are observations that suggest an asymmetric behavior in basal conditions that is modified by central and peripheral changes, as well as by pharmacological treatments. There are evidences that connect cardiovascular function, neurochemical asymmetries, and depression. A deep understanding of the bilateral behavior of the brain following pathophysiological changes in blood pressure as well as pharmacologically induced, can provide us with therapeutic suggestions for the treatment of depression. In this article, we analyze remarkable results of some representative selected contributions, with which we discuss our proposal on the relationship between hypertension, depression and neurochemical asymmetry.

Asymmetrical influence of a standard light/dark cycle and constant light conditions on the alanyl-aminopeptidase activity of the left and right retinas in adult male rats.

The retina acts as an independent clock informing the central pacemaker, the suprachiasmatic nucleus, under environmental light conditions, with consequences of such inputs for the central and peripheral nervous system. Differences in the behavior of the left and right retinas depending on environmental light conditions may influence the information projected to the brain hemispheres. The retina possesses neuropeptides that act as neurotransmitters or neuromodulators. Alanyl-aminopeptidase (AlaAP, EC 3.4.11.2) activity regulates some of these neuropeptides and therefore reflects their function. We analyzed AlaAP activity in the left and right retinas of adult male rats at successive time points under standard (12/12 h light/dark cycle) and nonstandard (constant light) conditions. AlaAP activity was measured fluorometrically using alanyl-beta-naphthylamide as the substrate. Under standard conditions, there were no differences in the left or right retina between time points, with the left retina predominating, particularly in the light period. In contrast, under constant light, no left versus right differences were observed, but significant differences between time points appeared. In comparison with standard conditions, constant conditions led to significantly higher AlaAP activity. Considering all the left retina data in comparison with all the right retina data, no correlation was found between the left and right retinas under standard conditions, but a significant positive correlation was observed under constant light. These results demonstrate an asymmetrical response of retinal AlaAP activity to changes in environmental light conditions, which may affect the functions in which the substrates of AlaAP are involved and the information projected to the brain hemispheres.

Efficacy and safety of bortezomib in refractory lupus nephritis: a single-center experience.

BACKGROUND AND OBJECTIVES: Resistant lupus nephritis (LN) has been associated with the persistence of long-lived plasma cells. Preliminary studies identified bortezomib as a potential treatment option for patients with refractory LN. The aim of this study was to analyze the efficacy and safety of bortezomib in the treatment of severe refractory LN. METHODS: This retrospective study included 12 female patients diagnosed for the first time with class IV or IV/V LN with acute or rapidly progressive kidney injury (n = 11) and/or severe nephrotic syndrome (n = 1) who showed resistance to induction therapy with cyclophosphamide, steroids, mycophenolate, and rituximab, and were treated with either intravenous or subcutaneous bortezomib plus intravenous dexamethasone. RESULTS: All patients with acute or rapidly progressive kidney injury showed a significant reduction in both biochemical and immunological activity after a mean of 6 (minimum 5, maximum 7) weekly cycles of bortezomib regimen, with a significant increase in C3 levels and a significant decrease of anti-ds DNA antibody titers, Systemic Lupus Erythematosus Disease Activity Index score, serum creatinine, and proteinuria. One patient (8.3%) achieved a complete response, and 10 patients (83.4%) achieved a partial response. During follow-up, all these patients maintained partial responses under treatment with mycophenolate and low-dose glucocorticoids. The patient with refractory nephrotic syndrome showed a partial response but relapsed 11 months after the end of bortezomib treatment and was resistant to treatment. A significant decrease in serum IgG levels after initiation of bortezomib treatment was observed in all patients, five of them (41.6%) showed hypogammaglobulinemia (<500 mg/dl), but no patient suffered from opportunistic infections; in only two patients (16.6%) hypogammaglobulinemia persisted at the end of follow-up. Two patients (16.6%) suffered from sensory neuropathy, which led to bortezomib treatment discontinuation. CONCLUSIONS: Bortezomib may be an effective option for refractory LN, but close monitoring must be performed for possible adverse events such as peripheral neuropathy and hypogammaglobulinemia.

Angiotensin II, dopamine and nitric oxide. An asymmetrical neurovisceral interaction between brain and plasma to regulate blood pressure.

Vital functions, such as blood pressure, are regulated within a framework of neurovisceral integration in which various factors are involved under normal conditions maintaining a delicate balance. Imbalance of any of these factors can lead to various pathologies. Blood pressure control is the result of the balanced action of central and peripheral factors that increase or decrease. Special attention for blood pressure control was put on the neurovisceral interaction between Angiotensin II and the enzymes that regulate its activity as well as on nitric oxide and dopamine. Several studies have shown that such interaction is asymmetrically organized. These studies suggest that the neuronal activity related to the production of nitric oxide in plasma is also lateralized and, consequently, changes in plasma nitric oxide influence neuronal function. This observation provides a new aspect revealing the complexity of the blood pressure regulation and, undoubtedly, makes such study more motivating as it may affect the approach for treatment.

Cystinyl and pyroglutamyl-beta-naphthylamide hydrolyzing activities are modified coordinately between hypothalamus, liver and plasma depending on the thyroid status of adult male rats.

The hypothalamus determinates metabolic processes in liver through endocrine and autonomic control. Hypothalamic neuropeptides, such as thyrotropin releasing hormone or vasopressin, have been involved in liver metabolism. The thyroid status influences metabolic processes including liver metabolism in modulating those hypothalamic peptides whose functional status is regulated in part by aminopeptidase activities. In order to obtain data for a possible coordinated interaction between hypothalamus, plasma and liver, of some aminopeptidase activities that may partially reflect the hydrolysis of those peptides, pyroglutamyl- (pGluAP) and cystinyl- (CysAP) beta-naphthylamide hydrolyzing activities were determined fluorimetrically, both in their soluble and membrane-bound forms, in eu- hypo- and hyperthyroid adult male rats. Hyperthyroidism and hypothyroidism were induced with daily subcutaneous injections of tetraiodothyronine (300 µg/kg/day) or with 0.03% methimazole in drinking water for 6 weeks. Results demonstrated significant changes depending on the type of enzyme and the thyroid status. The most striking changes were observed for CysAP in liver where it was reduced in hypothyroidism and increased in hyperthyroidism. Significant intra- and inter-tissue correlations were observed. While there were positive inter-tissue correlations between liver, plasma and hypothalamus in eu-and hypothyroid rats, a negative correlation between hypothalamus and liver was observed in hyperthyroidism. These results suggest the influence of thyroid hormones and an interactive role for these activities in the control of liver metabolism. The present data also suggest a role for CysAP and pGluAP activities in liver function linked to their activities in hypothalamus.

Bidirectional asymmetry in the neurovisceral communication for the cardiovascular control: New insights.

The cardiovascular control involves a bidirectional functional connection between the brain and heart. We hypothesize that this connection could be extended to other organs using endocrine and autonomic nervous systems (ANS) as communication pathways. This implies a neuroendocrine interaction controlling particularly the cardiovascular function where the enzymatic cascade of the renin-angiotensin system (RAS) plays an essential role. It acts not only through its classic endocrine connection but also the ANS. In addition, the brain is functionally, anatomically, and neurochemically asymmetric. Moreover, this asymmetry goes even beyond the brain and it includes both sides of the peripheral nervous and neuroendocrine systems. We revised the available information and analyze the asymmetrical neuroendocrine bidirectional interaction for the cardiovascular control. Negative and positive correlations involving the RAS have been observed between brain, heart, kidney, gut, and plasma in physiologic and pathologic conditions. The central role of the peptides and enzymes of the RAS within this neurovisceral communication, as well as the importance of the asymmetrical distribution of the various RAS components in the pathologies involving this connection, are particularly discussed. In conclusion, there are numerous evidences supporting the existence of a neurovisceral connection with multiorgan involvement that controls, among others, the cardiovascular function. This connection is asymmetrically organized.

[Brain asymmetry and dopamine: beyond motor implications in Parkinson's disease and experimental hemiparkinsonism]. / Asimetria cerebral y dopamina: mas alla de las implicaciones motoras en la enfermedad de Parkinson y hemiparkinsonismo experimental.

INTRODUCTION: Brain asymmetry could be defined as the existence of functional, anatomic or neurochemical differences between both hemispheres. It is a dynamic phenomenon, regulated by endogenous and exogenous factors. Its functional significance is poorly clarified and is only partially understood in very specific cases such as the relationship between the lateralized brain content of dopamine and its motor effects which is specially patent in Parkinson's disease. DEVELOPMENT: The asymmetric brain content of dopamine not only displays lateralized motor effects but also behavioral and autonomic asymmetric consequences. In fact, Parkinson's disease is characterized not only by unilateral motor symptoms that arise at the early stages, but has other non-motor symptoms such as autonomic or cognitive alterations that are also revealed asymmetrically. CONCLUSIONS: Brain asymmetry has been underestimated when analyzing the pathogeny of brain diseases and it has been partially studied only in some specific cases, such as Parkinson's disease. However, in order to appropriately understand some brain diseases such as Parkinson's disease, the need to consider this phenomenon has been highlighted.

TITLE: Asimetria cerebral y dopamina: mas alla de las implicaciones motoras en la enfermedad de Parkinson y hemiparkinsonismo experimental.Introduccion. La asimetria cerebral se puede definir como la existencia de diferencias funcionales, anatomicas o neuroquimicas entre los dos hemisferios cerebrales. Se trata de un fenomeno dinamico modulable por factores endogenos y exogenos. Su significado funcional esta apenas aclarado y solo lo esta en algunos casos muy concretos como, por ejemplo, la relacion existente entre el contenido cerebral lateralizado de dopamina y sus efectos motores, que se manifiesta especialmente en la enfermedad de Parkinson. Desarrollo. El contenido asimetrico cerebral de dopamina no solo da lugar a efectos motores lateralizados, sino que se extiende a consecuencias autonomicas y de conducta igualmente lateralizadas. De hecho, la enfermedad de Parkinson se caracteriza por sintomas motores unilaterales, que surgen en las fases iniciales de la enfermedad, y por otros sintomas no motores, como alteraciones autonomicas o cognitivas, que tambien se manifiestan de forma lateralizada. Conclusiones. La asimetria cerebral ha sido un aspecto infravalorado a la hora de analizar la patogenia de las enfermedades cerebrales, y solo en determinados casos, como en la enfermedad de Parkinson, se ha profundizado parcialmente en su estudio. Sin embargo, se ha puesto en evidencia que es necesario considerar este fenomeno para la adecuada comprension de algunas patologias cerebrales, como es el caso de la enfermedad de Parkinson.

Handedness and gender influence blood pressure in young healthy men and women: A pilot study.

OBJECTIVE: The type and level of sex steroids influence blood pressure (BP). It has been suggested that functional brain asymmetries may be influenced by sex hormones. In addition, there are inter-arm differences in BP not yet related with handedness. In this study, we hypothesize a possible association between sex hormones, handedness, and inter-arm differences in blood pressure. METHODS: To analyze this hypothesis, we measured BP in the left and right arm of the left and right handed adult young men and women in menstrual and ovulatory phase and calculated their mean arterial pressure (MAP). RESULTS: Significant differences depending on sex, arm, handedness or phase of the cycle were observed. MAP was mostly higher in men than in women. Remarkably, in women, the highest levels were observed in the left handed in menstrual phase. Interestingly, the level of handedness correlated negatively with MAP measured in the left arm of right-handed women in the ovulatory phase but positively with the MAP measured in the right arm of right-handed women in the menstrual phase. CONCLUSIONS: These results may reflect an asymmetrical modulatory influence of sex hormones in BP control.

Influence of thyroid disorders on the kidney expression and plasma activity of aminopeptidase A.

OBJECTIVE: Thyroid disorders may affect blood pressure and renal function modifying factors of the plasmatic and kidney renin-angiotensin system such as aminopeptidase A (AP A) that metabolizes angiotensin II to angiotensin III. We investigated the expression of AP A in the kidney, as well as its enzymatic activity in the plasma of euthyroid, hyperthyroid, and hypothyroid adult male rats. METHODS: Hyperthyroidism was induced by daily subcutaneous injections of tetraiodothyronine. Hypothyroid rats were obtained by administration of methimazole in drinking water. Expression of AP A was determined by Western blot analysis. Plasma AP A activity was measured fluorometrically using glutamyl-ß-naphthylamide as substrate. RESULTS: While hyperthyroid rats exhibited lower levels of plasma AP A activity than controls, the kidney of hyperthyroid animals expressed significantly higher AP A than controls and hypothyroid animals. CONCLUSIONS: A discrepancy between the high expression of AP A in kidney of hyperthyroid rats and the low activity of AP A measured in plasma and kidney of hyperthyroid animals was found. The posttranslational influence of environmental biochemical factors may be in part responsible for that divergence.

Shunt portosistémico congénito. Importancia del tratamiento precoz / Congenital portosistemic shunt. Importance of early treatment

Objetivos. El shunt porto-sistémico congénito (SPSC) es una patología infrecuente que puede producir complicaciones graves, como encefalopatía y tumores hepáticos con riesgo de degeneración maligna. La oclusión del shunt por cirugía o radiología intervencionista puede evitar, e incluso mejorar, las complicaciones. En algunos casos el trasplante hepático es la única opción. Describimos nuestra experiencia con esta patología. Pacientes y Método. Entre 1992 y 2013, ocho pacientes en edad pediátrica (cuatro varones y cuatro mujeres) fueron diagnosticados de un SPSC (cuatro extrahepáticos y cuatro intrahepáticos), de los cuales siete fueron diagnosticados después del año 2007. La mediana de edad al diagnóstico fue 5,5 años (1 mes-15 años). Cinco pacientes tenían patología asociada. Resultados. Cinco pacientes presentaban hiperamoniemia y afectación intelectual. Una niña debutó con coma. Cuatro pacientes presentaron tumoraciones hepáticas, incluyendo hiperplasia nodular focal/nódulos de regeneración (n=3), y adenomas (n=3). Una paciente con tumoraciones múltiples requirió una hepatectomía por síntomas compresivos. En dos pacientes se produjo degeneración a hepatocarcinoma, un niño de 5 años tratado con trasplante y otro en edad adulta tratado con hepatectomía. En un paciente de diagnóstico neonatal, el shunt cerró espontáneamente en seis meses. En cinco pacientes se ha realizado portografía directa con test de oclusión, realizándose cierre del shunt en tres casos por radiología intervencionista, uno con cirugía y en otro, con trasplante. Conclusiones. El tratamiento del SPSC ha de ser precoz para prevenir, e incluso revertir, las complicaciones, evitando el trasplante hepático. En la actualidad la radiología intervencionista juega un papel fundamental en la estrategia y el tratamiento de estos pacientes

AIM. Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. Patients and Methods. Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007. The mean age at diagnosis was 5.5 years (1 month-15 years). Five patients had associated comorbidities. Results. Five patients had developed hyperammonemia and intellectual impairment, one of those manifested with an onset of coma. Four patients have developed at diagnosis liver tumors, including focal nodular hyperplasia/regenerative nodules (n=3) and adenomas (n=3). One patient with multiple tumors required a hepatectomy owing to compressive symptoms. Two patients, developed malignant degeneration, a child under five years treated with liver transplantation and another in adulthood treated with hepatectomy. In one patient, diagnosed in the neonatal period, the shunt occlusion occurred spontaneously. Direct portography with the occlusion test was performed in five patients, the shunt was occluded with interventional radiology in three cases, surgery in one and liver transplantation in the remaining. Conclusions. The treatment of the SPSC must be early to prevent and even to reverse its complications, avoiding liver transplantation. Currently, interventional radiology is essential in the strategy to follow and treatment of these patients

Rotura de un aneurisma de arteria mesentérica superior en edad pediátrica: caso clínico y revisión de la literatura / Rupture of a superior mesenteric artery aneurysm in pediatric age: case report and literature review

Introducción. Los aneurismas esplácnicos son excepcionales en la edad pediátrica. La elevada mortalidad por rotura justifica su tratamiento, existiendo diversas opciones terapéuticas entre las que destacan la cirugía y, recientemente, el tratamiento endovascular. Caso clínico. Paciente de 11 años que presentó dolor abdominal súbito y caída del hematocrito. La angio-TC abdominal urgente mostró un aneurisma sacular de la arteria mesentérica superior (AMS) a 4 cm del ostium con disección de la luz y signos de sangrado activo. Se realizó una angiografía que confirmó el aneurisma. Se colocó un stent autoexpandible en el tronco principal de la arteria mesentérica superior con repleción del aneurisma con microcoils y Onyx, sin evidenciar perfusión residual de la falsa luz y comprobando una adecuada vascularización tanto distal como de las ramas yeyuno-ileales. Se instauró doble antiagregación con AAS y dipiridamol. Tras 24 meses de seguimiento se encuentra asintomática. Comentarios. El tratamiento endovascular es efectivo en el paciente pediátrico, incluso en situaciones de emergencia

Introduction. Splanchnic artery aneurysms are rare in children. High mortality from rupture justifies its treatment, with various therapeutic options among which stand out surgery and recently, endovascular treatment. Case report. A 11 year old girl presented with abdominal pain and sudden drop in hematocrit. The urgent abdominal CT angiography showed a saccular aneurysm of the superior mesenteric artery (SMA) at 4 cm from the ostium with dissection and active bleeding. A selective angiography was performed which confirmed the dissection. A self-expanding stent was placed in the main trunk of the SMA and a transcatheter coil and onyx embolization of the aneurysm was performed. The control angiogram showed no evidence of residual perfusion of the false lumen and demonstrated proper vascularization of the distal jejunumileal branches. Dual antiplatelet therapy with aspirin and dipyridamole was begun. After 24 months of follow-up the patient is asymptomatic. Comments. Endovascular treatment of a SMA aneurysm is effective in the pediatric patient, even in emergency situations

[Rupture of a superior mesenteric artery aneurysm in pediatric age: case report and literature review]. / Rotura de un aneurisma de arteria mesentérica superior en edad pediátrica: caso clínico y revisión de la literatura.

INTRODUCTION: Splanchnic artery aneurysms are rare in children. High mortality from rupture justifies its treatment, with various therapeutic options among which stand out surgery and recently, endovascular treatment. CASE REPORT: A 11 year old girl presented with abdominal pain and sudden drop in hematocrit. The urgent abdominal CT angiography showed a saccular aneurysm of the superior mesenteric artery (SMA) at 4 cm from the ostium with dissection and active bleeding. A selective angiography was performed which confirmed the dissection. A self-expanding stent was placed in the main trunk of the SMA and a transcatheter coil and onyx embolization of the aneurysm was performed. The control angiogram showed no evidence of residual perfusion of the false lumen and demonstrated proper vascularization of the distal jejunum-ileal branches. Dual antiplatelet therapy with aspirin and dipyridamole was begun. After 24 months of follow-up the patient is asymptomatic. COMMENTS: Endovascular treatment of a SMA aneurysm is effective in the pediatric patient, even in emergency situations.

INTRODUCCION: Los aneurismas esplácnicos son excepcionales en la edad pediátrica. La elevada mortalidad por rotura justifica su tratamiento, existiendo diversas opciones terapéuticas entre las que destacan la cirugía y, recientemente, el tratamiento endovascular. CASO CLINICO: Paciente de 11 años que presentó dolor abdominal súbito y caída del hematocrito. La angio-TC abdominal urgente mostró un aneurisma sacular de la arteria mesentérica superior (AMS) a 4 cm del ostium con disección de la luz y signos de sangrado activo. Se realizó una angiografía que confirmó el aneurisma. Se colocó un stent autoexpandible en el tronco principal de la arteria mesentérica superior con repleción del aneurisma con microcoils y Onyx, sin evidenciar perfusión residual de la falsa luz y comprobando una adecuada vascularización tanto distal como de las ramas yeyuno-ileales. Se instauró doble antiagregación con AAS y dipiridamol. Tras 24 meses de seguimiento se encuentra asintomática.. COMENTARIOS: El tratamiento endovascular es efectivo en el paciente pediátrico, incluso en situaciones de emergencia.

[Congenital portosistemic shunt. Importance of early treatment]. / Shunt portosistémico congénito. Importancia del tratamiento precoz.

AIM: Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. PATIENTS AND METHODS: Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007. The mean age at diagnosis was 5.5 years (1 month-15 years). Five patients had associated comorbidities. RESULTS: Five patients had developed hyperammonemia and intellectual impairment, one of those manifested with an onset of coma. Four patients have developed at diagnosis liver tumors, including focal nodular hyperplasia/regenerative nodules (n=3) and adenomas (n=3). One patient with multiple tumors required a hepatectomy owing to compressive symptoms. Two patients, developed malignant degeneration, a child under five years treated with liver transplantation and another in adulthood treated with hepatectomy. In one patient, diagnosed in the neonatal period, the shunt occlusion occurred spontaneously. Direct portography with the occlusion test was performed in five patients, the shunt was occluded with interventional radiology in three cases, surgery in one and liver transplantation in the remaining. CONCLUSIONS: The treatment of the SPSC must be early to prevent and even to reverse its complications, avoiding liver transplantation. Currently, interventional radiology is essential in the strategy to follow and treatment of these patients.

OBJETIVOS: El shunt porto-sistémico congénito (SPSC) es una patología infrecuente que puede producir complicaciones graves, como encefalopatía y tumores hepáticos con riesgo de degeneración maligna. La oclusión del shunt por cirugía o radiología intervencionista puede evitar, e incluso mejorar, las complicaciones. En algunos casos el trasplante hepático es la única opción. Describimos nuestra experiencia con esta patología. PACIENTES Y METODO: Entre 1992 y 2013, ocho pacientes en edad pediátrica (cuatro varones y cuatro mujeres) fueron diagnosticados de un SPSC (cuatro extrahepáticos y cuatro intrahepáticos), de los cuales siete fueron diagnosticados después del año 2007. La mediana de edad al diagnóstico fue 5,5 años (1 mes-15 años). Cinco pacientes tenían patología asociada. RESULTADOS: Cinco pacientes presentaban hiperamoniemia y afectación intelectual. Una niña debutó con coma. Cuatro pacientes presentaron tumoraciones hepáticas, incluyendo hiperplasia nodular focal/nódulos de regeneración (n=3), y adenomas (n=3). Una paciente con tumoraciones múltiples requirió una hepatectomía por síntomas compresivos. En dos pacientes se produjo degeneración a hepatocarcinoma, un niño de 5 años tratado con trasplante y otro en edad adulta tratado con hepatectomía. En un paciente de diagnóstico neonatal, el shunt cerró espontáneamente en seis meses. En cinco pacientes se ha realizado portografía directa con test de oclusión, realizándose cierre del shunt en tres casos por radiología intervencionista, uno con cirugía y en otro, con trasplante. CONCLUSIONES: El tratamiento del SPSC ha de ser precoz para prevenir, e incluso revertir, las complicaciones, evitando el trasplante hepático. En la actualidad la radiología intervencionista juega un papel fundamental en la estrategia y el tratamiento de estos pacientes.

Relationship of angiotensinase and vasopressinase activities between hypothalamus, heart, and plasma in L-NAME-treated WKY and SHR.

The renin-angiotensin system (RAS), vasopressin, and nitric oxide (NO) interact to regulate blood pressure at central and peripheral level. To improve our understanding of their interaction and their relationship with the hypothalamus and the cardiovascular system, we analyzed angiotensin- and vasopressin-metabolizing activities in hypothalamus (HT), left ventricle (LV), and plasma, collected from Wistar-Kyoto (WKY) and spontaneously hypertensive rats (SHR) treated or not with L-NAME [N(G)-nitro-L-arginine methyl ester], which inhibits the formation of NO and over-activates the sympathetic nervous system. Previous observations in WKY suggested higher formation of Ang III and Ang IV in the HT and higher availability of Ang II in plasma after L-NAME treatment. Our current results show higher formation of Ang IV and higher metabolism of vasopressin after treatment with L-NAME in the LV of WKY rats. In SHR treated with L-NAME, there is higher availability of Ang III in the HT leading to higher release of vasopressin together with lower formation of Ang 2-10. In their LV, however, there is an increase of vasopressinase. Interestingly, while the enzymatic activities in the HT and LV of WKY rats and control SHR are poorly correlated, they are well but inversely correlated in the L-NAME treated SHR. On the other hand, no significant correlations between enzymatic activities in HT or LV and plasma were noticed. Our results suggest that eNOS inhibition in SHR induces or enhances an inverse reciprocal interaction between HT and LV involving the RAS and vasopressin, which may be mediated by the autonomic nervous system.