Efectividad y seguridad de la cirugía para la epilepsia en niños. Experiencia de un hospital terciario en Ecuador / Safety and effectiveness of surgery for epilepsy in children. Experience of a tertiary hospital in Ecuador

Introducción: Existe suficiente evidencia sobre la utilidad de la cirugía como alternativa terapéutica para pacientes con epilepsia farmacorresistente, sin embargo este tratamiento es subutilizado especialmente en países en desarrollo. El objetivo de este trabajo fue determinar la efectividad y seguridad de la cirugía de epilepsia en un hospital terciario de Ecuador.MétodosSe describe el resultado de la cirugía de epilepsia en 27 niños y adolescentes en el Hospital Baca Ortiz, de Quito, Ecuador, teniendo en cuenta las siguientes variables antes y después de la cirugía: reducción de la frecuencia de crisis, Engel posquirúrgico, mejoría en la calidad de vida y presencia de complicaciones graves por la cirugía.ResultadosSe realizaron 21 callosotomías y seis cirugías resectivas. La frecuencia de crisis media se redujo de 465 mensual antes de la cirugía a 37,2 mensual después de la misma (p<0,001), mientras que la puntuación en la escala de calidad de vida aumentó de 12,6 a 37,2 puntos (p<0,001), el 72,7% de los pacientes mejoró la calidad de vida. Entre las cirugía resectivas, en dos epilepsias del lóbulo temporal y una del cuadrante posterior se logró Engel Ia, una hemisferotomía por encefalitis de Rasmusen quedó en Engel IIa y dos hamartomas hipotalámicos, uno logró Engel III y otro Engel Ia pero falleció a mediano plazo por complicación posquirúrgica. La otra complicación grave fue un hidrocéfalo que llevó a la muerte a un lactante con espasmos infantiles refractarios sometido a callosotomía.ConclusiónEl resultado favorable se observó en el 92,5% de los pacientes. (AU)

Introduction: There is sufficient evidence on the usefulness of surgery as a therapeutic alternative for patients with drug-resistant epilepsy; however this treatment is underutilized, especially in developing countries.MethodsWe describe the outcomes of epilepsy surgery in 27 paediatric patients at Hospital Baca Ortiz in Quito, Ecuador. Our analysis considered the following variables: reduction in seizure frequency, surgery outcome according to the Engel classification, improvement in quality of life, and serious complications due to surgery.Results21 corpus callosotomies and 6 resective surgeries were performed. The mean seizure frequency decreased from 465 per month before surgery to 37.2 per month thereafter (p<.001); quality of life scale scores increased from 12.6 to 37.2 (p<.001), and quality of life improved in 72.7% of patients. Regarding resective surgery, 2 patients with temporal lobe epilepsy and one with posterior quadrant epilepsy achieved Engel class IA, and one patient undergoing hemispherotomy due to Rasmussen encephalitis achieved Engel class IIA. Two patients underwent surgery for hypothalamic hamartoma: one achieved Engel III and the other, Engel IA; however, the latter patient died in the medium term due to a postoperative complication. The other major complication was a case of hydrocephalus, which led to the death of a patient with refractory infantile spasms who underwent corpus callosotomy.ConclusionsFavourable outcomes were observed in 92.5% of patients. (AU)

Safety and effectiveness of surgery for epilepsy in children. Experience of a tertiary hospital in Ecuador. / Efectividad y seguridad de la cirugía para la epilepsia en niños. Experiencia de un hospital terciario en Ecuador.

INTRODUCTION: There is sufficient evidence on the usefulness of surgery as a therapeutic alternative for patients with drug-resistant epilepsy; however this treatment is underutilized, especially in developing countries. METHODS: We describe the outcomes of epilepsy surgery in 27 paediatric patients at Hospital Baca Ortiz in Quito, Ecuador. Our analysis considered the following variables: reduction in seizure frequency, surgery outcome according to the Engel classification, improvement in quality of life, and serious complications due to surgery. RESULTS: 21 corpus callosotomies and 6 resective surgeries were performed. The mean seizure frequency decreased from 465 per month before surgery to 37.2 per month thereafter (p<.001); quality of life scale scores increased from 12.6 to 37.2 (p<.001), and quality of life improved in 72.7% of patients. Regarding resective surgery, 2 patients with temporal lobe epilepsy and one with posterior quadrant epilepsy achieved Engel class IA, and one patient undergoing hemispherotomy due to Rasmussen encephalitis achieved Engel class IIA. Two patients underwent surgery for hypothalamic hamartoma: one achieved Engel III and the other, Engel IA; however, the latter patient died in the medium term due to a postoperative complication. The other major complication was a case of hydrocephalus, which led to the death of a patient with refractory infantile spasms who underwent corpus callosotomy. CONCLUSIONS: Favourable outcomes were observed in 92.5% of patients.

Utilidad del Video EEG en un hospital pediátrico de nivel terciario durante el año 2015 / Utility Of Video EEG In A Pediatric Tertiary Hospital During 2015

Resumen Objetivo: Evaluar la utilidad del V-EEG en el diagnóstico diferencial de la epilepsia en un hospital pediátrico de nivel terciario durante el año 2015 Materiales y Métodos: Se realizó un estudio descriptivo sobre 90 pacientes evaluados en esta unidad durante el año 2015. Se recogieron datos de variables relacionadas con la indicación y los resultados del V-EEG, los cuales fueron analizados usando medidas de estadística descriptiva. Resultados: El 53,3% de los pacientes fueron masculinos. El promedio de edad es de 7,7 años con una desviación estándar de 4,7 años. El tiempo que transcurre desde la primera crisis hasta que el paciente acude a realizarse el V-EEG presenta una media de 4,3 años. 72 pacientes (80%) presentaron crisis epilépticas, 12 pacientes (13,3%) presentaron trastornos paroxísticos no epilépticos, mientras 6 niños (6,7%) no presentaron crisis durante el monitoreo. En el 93,3% de los casos el estudio fue exitoso. Conclusiones: Se demuestra la utilidad del monitoreo V-EEG para el diagnóstico diferencial de epilepsia.

Summary Objective: The aim was to evaluate the V-EEG usefulness in the differential diagnosis of epilepsy in a Third Level Children's Hospital during 2015. Materials and Methods: A descriptive study was performed over 90 patients in this unit during 2015. The data was obtained from variables related to indications and results of V-EEG, which were analyzed using descriptive statistics. Results: Fifty three percent of the patients were male. The mean age was 7.7 years (SD ± 4.7 years). The time measured between the first seizure and the V-EEG recording was 4,3 years. Seventy two patients (80%) had epileptic seizures, 12 patients (13,3%) had nonepileptic seizures, while six children (6.7%) had no seizures during the V-EEG monitoring. Ninety three percent of all recordings were successful. Conclusions: It was demonstrated the usefulness of V-EEG monitoring for the differential diagnosis of epilepsy.

Uricemia y síndrome metabólico en pacientes con hipertensión arterial / Uricemia and metabolic syndrome in patients with arterial hypertension

Objetivo. Los niveles de uricemia se han asociado con el síndrome metabólico (SM). Sin embargo, la relación entre estas 2 variables en pacientes con hipertensión arterial (HTA) esencial no ha sido estudiada. Pacientes y métodos. Estudio observacional, transversal, de 592 pacientes con HTA esencial. Para la definición de SM se emplearon >=3 criterios de la ATP-III. Se excluyeron a los pacientes con tratamiento hipouricemiante. Resultados. La prevalencia de SM fue del 52% (IC del 95%: 48-56%) y aumentó gradualmente a medida que se incrementaba la uricemia (uricemia: <=4,7mg/dl, 36%; uricemia >=6,8mg/dl, 70%; p<0,001). Los enfermos hipertensos con SM mostraron una uricemia media más elevada que los que no tenían esta comorbilidad (6,1±1,5mg/dl vs 5,4±1,3mg/dl; p<0,0001). La prevalencia de hiperuricemia (varones: >=7,0mg/dl; mujeres: >=6,0mg/dl) en los pacientes hipertensos que no recibían tratamiento diurético fue del 24,3% (en aquellos con SM, 40,5% frente a un 11,4% en los que no tenían SM; p<0,001). En el análisis multivariante los triglicéridos (OR: 1,008; IC del 95%: 1,004-1,012; p<0,001) y el índice de masa corporal (IMC) (OR: 1,118; IC del 95%: 1,059-1,181; p<0,001) fueron predictores independientes de la uricemia. Conclusiones. En los pacientes con HTA esencial, aproximadamente la mitad padecen SM y uno de cada 4 presenta hiperuricemia. El determinante más relevante del incremento de la concentración sérica de uratos es el aumento del IMC(AU)

Objective. Serum urate levels have been associated with metabolic syndrome (MS). However, the relationship between these two variables in patients with essential arterial hypertension has not been studied. Patients and methods. A Cross-sectional study in 592 patients with essential hypertension. The MS was defined according to the ATP-III criteria. We excluded patients with hypouricemic treatment. Results. The prevalence of MS was 52% (95% CI, 48-56%) and there was a graded increase with increasing serum urate (uricemia <=.7 mg/dl, 36%; uricemia >=6.8 mg/dl, 70%, P < 0.001). Hypertensive patients with MS showed a higher mean uricemia than those without this comorbidity (6.1 ± 1.5 mg/dl versus 5.4 ± 1.3 mg/dl, P <0.0001). The prevalence of hyperuricemia (men, > 7.0 mg/dL; women, >6.0 mg/dL) in hypertensive patients without diuretic treatment, was 24% (in those with MS 40% versus 11% without MS). In multivariate analysis, triglycerides (OR = 1.008, CI 95%: 1.004-1.012, P < 0.001) and body mass index (BMI) (OR = 1.118, CI 95%: 1.059-1.181, P < 0.001), were independent predictors of serum uric acid levels. Conclusions. In patients with essential hypertension, about half have MS and one out of four has hyperuricemia. The most important determinant of hyperuricemia is BMI(AU)

[Uricemia and metabolic syndrome in patients with arterial hypertension]. / Uricemia y síndrome metabólico en pacientes con hipertensión arterial.

OBJECTIVE: Serum urate levels have been associated with metabolic syndrome (MS). However, the relationship between these two variables in patients with essential arterial hypertension has not been studied. PATIENTS AND METHODS: A Cross-sectional study in 592 patients with essential hypertension. The MS was defined according to the ATP-III criteria. We excluded patients with hypouricemic treatment. RESULTS: The prevalence of MS was 52% (95% CI, 48-56%) and there was a graded increase with increasing serum urate (uricemia ≤ 4.7 mg/dl, 36%; uricemia ≥ 6.8 mg/dl, 70%, P < 0.001). Hypertensive patients with MS showed a higher mean uricemia than those without this comorbidity (6.1 ± 1.5 mg/dl versus 5.4 ± 1.3 mg/dl, P < 0.0001). The prevalence of hyperuricemia (men, > 7.0 mg/dL; women, > 6.0 mg/dL) in hypertensive patients without diuretic treatment, was 24% (in those with MS 40% versus 11% without MS). In multivariate analysis, triglycerides (OR = 1.008, CI 95%: 1.004-1.012, P < 0.001) and body mass index (BMI) (OR = 1.118, CI 95%: 1.059-1.181, P < 0.001), were independent predictors of serum uric acid levels. CONCLUSIONS: In patients with essential hypertension, about half have MS and one out of four has hyperuricemia. The most important determinant of hyperuricemia is BMI.

Suppression of guanylyl cyclase (beta1 subunit) expression impairs neurite outgrowth and synapse maturation in cultured cerebellar granule cells.

The increased expression of different soluble guanylyl cyclase (sGC) subunits during development is consistent with these proteins participating in the formation and establishment of interneuronal contacts. Functional sGC is generated by the dimerization of an alpha-subunit (sGCalpha1/2) with the beta1-subunit (sGCbeta1), and both depletion of the sGCbeta1 subunit and inhibiting sGC activity impair neurite outgrowth. Similarly, impairing sGC activity diminishes the amount of growth-associated protein (GAP-43) and synapsin I, two proteins that participate in axon elongation and synaptogenesis, suggesting a role for sGC in these processes. Indeed, fewer synapses form when sGC is inhibited, as witnessed by FM1-43 imaging and synapsin I immunostaining, and the majority of synapses that do form remain functionally immature. These findings highlight the importance of sGC in the regulation of neurite outgrowth and synapse formation, and in the functional maturation of cerebellar granule cells in vitro.

Recomendaciones específicas para el manejo del paciente con síndrome metabólico / Specific recommendations for the management of patients with metabolic syndrome

El término síndrome metabólico hace referencia a un acúmulo de factores de riesgo cardiovascular en cuya fisiopatología subyace la existencia de resistencia insulínica. Este trastorno metabólico se asocia a la presencia de obesidad. Un estado proinflamatorio y protrombótico contribuye al desarrollo de este cuadro. La consecuencia principal del síndrome es una mayor frecuencia en la incidencia de enfermedades cardiovasculares y en el desarrollo de diabetes mellitus tipo 2. El tratamiento consiste en la disminución de peso con medidas dietéticas y en la realización de ejercicio físico. Sin embargo, resulta también apropiado el empleo de fármacos específicos para cada uno de los factores de riesgo definitorios presentes. Rimonabant, un bloqueante selectivo de los receptores tipo 1 de los cannabinoides, que reduce el peso y mejora los factores de riesgo metabólico en pacientes obesos o con sobrepeso, podría convertirse en el primer tratamiento específico para este cuadro

The term metabolic syndrome refers to a group of cardiovascular disease risk factors whose underlying pathophysiology is thought to be related to insulin resistance. This common metabolic disorder is associated to the presence of obesity. A proinflammatory and prothrombotic state probably contributes to the syndrome. The primary consequence of this syndrome is the increased risk for cardiovascular disease and type 2 diabetes. The fundamental approach is weight reduction with dietary measures and increased physical activity. However, drug treatment is also appropriate for specific risk factors. Rimonabant, a selective cannabinoid type 1 receptor blocker, reduces body weight and improves metabolic risk factors in overweight or obese patients and could become the first specific drug treatment for this disorder (AU)

Two-proton radioactivity of 45Fe.

In an experiment at the SISSI-LISE3 facility of GANIL, the decay of the proton drip line nucleus 45Fe has been studied. Fragment-implantation events have been correlated with radioactive decay events in a 16x16 pixel silicon-strip detector. The decay-energy spectrum of 45Fe implants shows a distinct peak at (1.14+/-0.04) MeV with a half-life of T(1/2)=(4.7(+3.4)(-1.4)) ms. None of the events in this peak is in coincidence with beta particles. For a longer correlation interval, daughter decays of the two-proton daughter 43Cr can be observed after 45Fe implantation. The decay energy for 45Fe agrees nicely with several theoretical predictions for two-proton radioactivity.

Nramp2 expression is associated with pH-dependent iron uptake across the apical membrane of human intestinal Caco-2 cells.

The absorption of dietary non-heme iron by intestinal enterocytes is crucial to the maintenance of body iron homeostasis. This process must be tightly regulated since there are no distinct mechanisms for the excretion of excess iron from the body. An insight into the cellular mechanisms has recently been provided by expression cloning of a divalent cation transporter (DCT1) from rat duodenum and positional cloning of its human homologue, Nramp2. Here we demonstrate that Nramp2 is expressed in the apical membrane of the human intestinal epithelial cell line, Caco 2 TC7, and is associated with functional iron transport in these cells with a substrate preference for iron over other divalent cations. Iron transport occurs by a proton-dependent mechanism, exhibiting a concurrent intracellular acidification. Taken together, these data suggest that the expression of the Nramp2 transporter in human enterocytes may play an important role in intestinal iron absorption.

[Clenbuterol poisoning. Clinical and analytical data on an outbreak in Móstoles, Madrid]. / Intoxicación por clenbuterol. Datos clínicos y analíticos de un brote epidémico en Móstoles, Madrid.

OBJECTIVE: To report the clinical manifestations and analytical findings in an epidemic outbreak of acute food poisoning with clenbuterol. MATERIALS AND METHODS: The clinical manifestations, physical examination findings and results of complementary tests are reported of fifteen patients performed by veal liver contaminated with clenbuterol. The clinical course of patients at 72 hours is reported. A quantitative measurement of clenbuterol in urine specimens from patients and in a veal liver specimen was performed by high pressure liquid chromatography (HPLC). RESULTS: The male/female distribution of patients was 7/8 respectively, with age ranging from 6 to 44 years. Symptoms appeared after 30 minutes to 2 hours of having ingested veal liver in 93% of cases. Patients presented at the Emergency Department with tremors, palpitations, anxiety, malaise, nausea, and pruritus as the most common complaints. On physical examination tachycardia was noted in 100% of cases. The analytical data included mild hypokaliemia (66%) and leukocytosis (28%). Only one patient required hospital admission on account of an hypertensive crisis. After 72 hours, 67% of patients were asymptomatic. The remaining patients had mild symptoms which included headache, myalgia, asthenia and anorexia. Serum potassium values returned to normality (p < 0.05). Urine measurements of clenbuterol were positive for all analyzed cases (50 +/- 42 ng/ml). The concentration of clenbuterol in a veal liver sample was 500 ppb. CONCLUSION: Clenbuterol poisoning should be suspected when symptoms of adrenergic hyperstimulation occur after the ingestion of meal, usually liver. Common symptoms include tachycardia and mild hypopotasemia. Diagnosis is confirmed by quantitative measurement of clenbuterol in urine. Most patients improve spontaneously shortly afterwards.

[Spontaneous peritonitis in a cirrhotic patient with a cat: Pasteurella multocida infection of the ascitic fluid]. / Peritonitis espontánea en un cirrótico con gato: infección por Pasteurella multocida del líquido ascítico.

Spontaneous peritonitis due to Pasteurella multocida is exceptional. As far as we know only 11 other cases have been reported. We describe a 45 year old patient who presented with a spontaneous Pasteurella multocida peritonitis as the first complication of a previously undiagnosed cirrhosis. The patient used to play with his pet cat, not recalling having ever sustained any injury. Cultures of the cat's mouth grew the same strain of Pasteurella multocida than was found in the patient's ascitic fluid. The clinical findings of the previous cases, most of which were also related to non traumatic exposure to domestic animals, are here described. Pasteurella multocida in one potential agent in the cirrhotic patient presenting with spontaneous peritonitis, especially if in close contact with animals, cats being the most often carriers.