[Neuroimaging in epileptic encephalopathies in infants]. / Neuroimagen en las encefalopatias epilepticas del lactante.

Magnetic resonance plays a vital role in the aetiological diagnosis of epileptic encephalopathies, since it is capable of identifying specific aetiological patterns or patterns which are suggestive of different conditions. We review the main magnetic resonance findings that are observed in symptomatic epileptic encephalopathies.

TITLE: Neuroimagen en las encefalopatias epilepticas del lactante.La resonancia magnetica desempeña un papel crucial en el diagnostico etiologico de las encefalopatias epilepticas, al poder identificar patrones etiologicamente especificos o sugestivos de diferentes entidades. Se revisan los principales hallazgos por resonancia magnetica que se objetivan en las encefalopatias epilepticas sintomaticas.

[Reversible alterations in the neuroimages associated with vigabatrine treatment in infants with epileptic spasms]. / Alteraciones reversibles en la neuroimagen asociadas al tratamiento con vigabatrina en lactantes con espasmos epilepticos.

INTRODUCTION: Vigabatrin (VGB) is a first-line drug for the treatment of infantile spasms. Recently, several reports claim the existence of abnormalities in magnetic resonance imaging (MRI) (particularly affecting basal ganglia, and visible in T2 and diffusion sequences) in infants with spasms that were receiving high doses of VGB (> 100 mg/kg/day), which appear to be reversible after withdrawal of treatment. CASE REPORTS: We present two cases with an epileptic encephalopathy in the first year of life and seizures consisting of infantile spasms. Both were treated with several antiepileptic drugs, including VGB up to a maximum dosage of 200 mg/kg/day. At the age of 11 and 28 months, respectively, MRI images showed marked signal hyperintensities on T2-sequences on bilateral globus pallidus, thalamus, posterior portion of the brainstem and dentate nuclei, also visible on diffusion sequences. Both had previous unaltered MRI studies. After excluding an underlying metabolic disease, VGB withdrawal is decided, appreciating the reversibility of those lesions in a prospective MRI study, three months later. CONCLUSIONS: We must consider and carefully evaluate findings on brain MRI in infants receiving VGB at high doses for treatment of spasms. The apparent cytotoxic effect on basal ganglia could simulate metabolic/mitochondrial diseases. By knowing this effect of VGB and its typical MRI features, unnecessary testing can be avoided in young infants with epileptic encephalopathies, including complex procedures like muscle biopsy or a new metabolic screening.

TITLE: Alteraciones reversibles en la neuroimagen asociadas al tratamiento con vigabatrina en lactantes con espasmos epilepticos.Introduccion. La vigabatrina (VGB) es un farmaco de primera linea para el tratamiento de espasmos infantiles. Diversos estudios han hallado anomalias en la resonancia magnetica (RM) cerebral, que afectaban particularmente a los ganglios de la base, y especialmente en secuencias de difusion, en lactantes con espasmos que recibian VGB en altas dosis (> 100 mg/kg/dia), y se ha observado la desaparicion de las lesiones tras la retirada de dicho tratamiento. Casos clinicos. Se presentan dos casos clinicos con inicio de una encefalopatia epileptica en el primer año de vida y crisis en forma de espasmos infantiles. Ambos recibieron tratamiento con distintos farmacos, entre ellos VGB hasta dosis de 200 mg/kg/dia. Con 11 y 28 meses de vida, respectivamente, aparecian imagenes en la RM cerebral con una marcada hiperintensidad de señal en secuencias ponderadas en T2 en ambos palidos, talamos, porcion posterior del tronco encefalico y nucleos dentados, que asociaban restriccion en secuencias de difusion. Ambos disponian de estudios previos de RM, sin alteraciones. Tras excluir una metabolopatia subyacente, se decidio la retirada de la VGB y tres meses despues, en una RM de control, se aprecio la total reversibilidad de dichas lesiones. Conclusiones. Deben evaluarse con cautela los hallazgos de la RM cerebral en lactantes que reciban VGB en altas dosis para el tratamiento de espasmos. Su aparente efecto citotoxico sobre los ganglios de la base podria simular metabolopatias/enfermedades mitocondriales. Conocer este efecto de la VGB y sus caracteristicas tipicas en la RM puede evitar pruebas innecesarias, como una biopsia muscular o un nuevo cribado metabolico.

All children with malignant rhabdoid tumors should be treated initially with intensive chemotherapy.

Malignant rhabdoid tumors (MRT) of soft tissues are aggressive tumors, which can be detected in almost any part of the body. MRT are rare, and very few cases have been reported in the literature. Prognosis of these tumors is extremely poor despite intensive therapy. Some risk factors such as young age or disseminated disease are associated with an aggressive and almost always lethal clinical course. Some clinicians even recommend initial palliative care due to this outcome. We report a case of metastatic MRT in a 6-month-old child with excellent initial response to chemotherapy.

Fibrosis quística en imágenes. Clasificación de Bhalla para la tomografía computarizada en pacientes pediátricos / Cystic fibrosis in images: the Bhalla scoring system for computed tomography in paediatric patients

Para el seguimiento de la evolución del daño pulmonar en los pacientes con fibrosis quística (FQ) se han desarrollado distintos sistemas de clasificación o puntuación mediante la radiografía de tórax y la tomografía computarizada de alta resolución (TCAR). La TCAR es una técnica más sensible que la radiografía de tórax para evaluar la anatomía, sin embargo, en pacientes pediátricos el uso de la tomografía computarizada (TC) debe reducirse al mínimo necesario y utilizar sistemas de protección radiológica y reducción de dosis. Uno de los sistemas de clasificación mediante TCAR más empleados es el propuesto por Bhalla en 1991, que facilita la evaluación de la gravedad y la evolución de estos pacientes en función de los distintos hallazgos radiológicos. Presentamos diferentes ejemplos de estos criterios para la TCAR, observados al revisar los estudios de un grupo de 48 pacientes (AU)

To monitor the course of lung damage in patients with cystic fibrosis (CF) using the different chest X-ray and high resolution computed tomography (HRCT) scoring systems that have been developed. The HRCT technique is more sensitive than chest radiography to evaluate the anatomy. However, in paediatric patients, the use of CT should be kept to a minimum, and guidelines for radiation protection and dose reduction should be applied. One of the most used classification systems for HRCT is the one proposed by Bhalla in 1991, which helps in the assessment of the severity and course of the disease in these patients depending on the different imaging findings. We present various examples of these criteria for HRCT, observed while reviewing a group of 48 paediatric patients (AU)

[Cystic fibrosis in images: the Bhalla scoring system for computed tomography in paediatric patients]. / Fibrosis quística en imágenes. Clasificación de Bhalla para la tomografía computarizada en pacientes pediátricos.

To monitor the course of lung damage in patients with cystic fibrosis (CF) using the different chest X-ray and high resolution computed tomography (HRCT) scoring systems that have been developed. The HRCT technique is more sensitive than chest radiography to evaluate the anatomy. However, in paediatric patients, the use of CT should be kept to a minimum, and guidelines for radiation protection and dose reduction should be applied. One of the most used classification systems for HRCT is the one proposed by Bhalla in 1991, which helps in the assessment of the severity and course of the disease in these patients depending on the different imaging findings. We present various examples of these criteria for HRCT, observed while reviewing a group of 48 paediatric patients.

Linfohistiocitosis hemofagocítica familiar: hallazgos neurorradiológicos / Familial hemophagocytic lymphohistiocytosis: neuroradiological findings

La linfohistiocitosis hemofagocítica (LHH) es un síndrome clínico raro, posiblemente infradiagnosticado, más frecuente en niños. Pueden ser cuadros agresivos y evolucionar en poco tiempo a fallo multiorgánico letal. Simula cuadros de sepsis infecciosa, aunque con peor respuesta y evolución. Se debe sospechar ante un niño pequeño con fiebre persistente de origen desconocido, afectación del estado general, hepatoesplenomegalia, citopenias, aumento de triglicéridos y ferritina y disminución del fibrinógeno. En la resonancia magnética cerebral se puede apreciar un realce difuso leptomeníngeo y perivascular, áreas parcheadas hiperintensas en T2 en sustancia blanca de ambos hemisferios cerebrales y atrofia cerebral. La secuencia de difusión es útil en la estadificación de las lesiones. Presentamos un caso clínico de LHH familiar de evolución mortal y realizamos una revisión bibliográfica de las características clínicas, anatomopatológicas y radiológicas de esta entidad (AU)

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure.…HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease (AU)

[Familial hemophagocytic lymphohistiocytosis: Neuroradiological findings]. / Linfohistiocitosis hemofagocítica familiar: hallazgos neurorradiológicos.

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure....HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease.

Accuracy of hippocampal network activity is disrupted by neuroinflammation: rescue by memantine.

Understanding how the hippocampus processes episodic memory information during neuropathological conditions is important for treatment and prevention applications. Previous data have shown that during chronic neuroinflammation the expression of the plasticity related behaviourally-induced immediate early gene Arc is altered within the CA3 and the dentate gyrus; both of these hippocampal regions show a pronounced increase in activated microglia. Low doses of memantine, a low to moderate affinity open channel uncompetitive N-Methyl-d-aspartate receptor antagonist, reduce neuroinflammation, return Arc expression to control levels and attenuate cognitive deficits induced by lipopolysaccharide. Here we investigate whether neuroinflammation affects the accuracy of information processing in the CA3 and CA1 hippocampal regions and if this is modified by memantine treatment. Using the immediate early gene-based brain-imaging method called cellular analysis of temporal activity by fluorescence in situ hybridization, it is possible to detect primary transcripts at the genomic alleles; this provides exceptional temporal and cellular resolution and facilitates the mapping of neuronal activity. Here, we use this method to compare the neuronal populations activated by two separate experiences in CA1 and CA3 and evaluate the accuracy of information processing during chronic neuroinflammation. Our results show that the CA3 pyramidal neuron activity is not stable between two exposures to the same environment context or two different contexts. CA1 networks, however, do not differ from control conditions. These data suggest that during chronic neuroinflammation, the CA3 networks show a disrupted ability to encode spatial information, and that CA1 neurons can work independently of CA3. Importantly, memantine treatment is able to partially normalize information processing in the hippocampus, suggesting that when given early during the development of the pathology memantine confers neuronal and cognitive protection while indirectly prevents pathological microglial activation.

Widespread expression of inducible NOS and citrulline in lupus nephritis tissues.

OBJECTIVE: Examine the presence of functional inducible nitric oxide synthase (iNOS) in lupus nephritis lesions. METHODS: Seventeen kidney biopsies from patients with lupus nephritis and an equal number of normal control kidney biopsies were examined for the presence of iNOS and endothelial nitric oxide synthase (eNOS) and citrulline by using immunohistochemical methods. Additionally, iNOS and eNOS mRNAs were examined by reverse transcription -PCR amplification of total renal RNA. RESULTS: All biopsies expressed constitutive eNOS, but in contrast to normal kidney biopsies, 70% of the lupus biopsies also expressed iNOS mRNA and the cognate protein. Eight positive biopsies corresponded to class IV lupus nephritis, which also had a high degree of citrullination. CONCLUSIONS: The data indicate that functional iNOS activity is present in glomeruli as part of the inflammatory process in the kidney; therefore the products of iNOS could play a role in the pathogenesis of lupus nephritis.

Extracutaneous sporotrichosis in a patient with liver cirrhosis.

We report an unusual case of disseminated cutaneous sporotrichosis with oral mucous and tracheal involvement in a forty-year-old male with a history of heavy drinking and liver cirrhosis. We also review the literature and other similar published cases.

Extracutaneous sporotrichosis in a patient with liver cirrhosis

Se presenta el caso clínico de un paciente de 40 años de edad conantecedente de alcoholismo intenso y cirrosis hepática que desarrolloesporotricosis cutánea diseminada con afectación a mucosa oral y tráquea.Se comenta la literatura correspondiente(AU)

We report an unusual case of disseminated cutaneous sporotrichosis with oralmucous and tracheal involvement in a forty-year-old male with a history ofheavy drinking and liver cirrhosis. We also review the literature and othersimilar published cases(AU)

La necesidad de un informe de enfermería al alta o traslado en una residencia geriátrica / The need of a nursing report at discharge or transfer to a geriatric residence

Frecuentemente el personal de enfermería se encuentra que al ingreso de un anciano la transmisión de información es deficiente o escasa para el abordaje integral e individualizado de éste. Con el objetivo de salvaguardar este problema y mejorar la continuidad asistencial de los ancianos dados de alta a su domicilio o trasladados a otro centro asistencial, el equipo de enfermería de la Residencia Clínica “Los Robles” ha elaborado un instrumento de registro que denominamos “Informe de enfermería al alta o traslado de una residencia geriátrica”. En el mismo se pretende recoger de una forma esquemática, ágil y completa la realidad y situación biopsicosocial del anciano garantizando una adecuada transmisión de la información y la continuidad de los cuidados de enfermería. Dicha hoja se elaboró basándonos en gran medida en el Modelo de Necesidades de Virginia Henderson (1). En ella se recogen los aspectos básicos del anciano a través de una serie de ítems cerrados y abiertos, abarcando tanto el estado basal del anciano como los procesos agudos si los hubiere, estando dirigida tanto al personal sanitario del centro asistencial de destino como a los propios cuidadores del anciano en el caso de traslado a domicilio

Introduction: As people get older, certain changes happen in the brain that become even more pronounced when the elderly person falls ill. Therefore it is very important to carry out exercises that stimulate the brain of elderly hospitalised people with special focus on the main cerebral functions of orientation and language. Our aims will be directed towards enhancing, stimulating and exercising two main cognitive functions: orientation and language in their diverse forms. Methods and resources: This work was developed in the San Rafael University hospital in Granada. The activities carried out were of orientation and language. Variables: age, sex, main diagnostic, Barthel Index and the Lobo (MEC) mini cerebral test. Results: The sample is constituted by 20 patients with an average age of 84.5 years old. We can note two main predominances: a greater number of women (75% from the total sample) and significant prevalence of neurological diseases (40%). During the initial MEC, before accomplishing the exercises, 35% of the sample showed a moderate cognitive deterioration, whereas minor deterioration prevailed during the subsequent MEC after the exercises, with a 35%. Within orientation exercises, patients obtained higher marks in the temporal-spatial ones. Concerning language exercises, marks reached an acceptable standard overall. Discussions and conclusions: The aged patients showed an acceptable level in temporal and spatial orientation and verbal repetition. It is essential the periodical practice of stimulating cognitive activities to improve aged patients’ life standards

Tricobezoar gástrico en una niña diagnosticada de anorexia nerviosa purgativa / No disponible

El bezoar es una concreción de carácter variado, que puede encontrase en el estómago o intestino de hombre u otros animales. Los que se componen fundamentalmente por retos de pelos son los denominados tricobezoares. Esta entidad se observa fundamentalmente en menores de 30 años de edad y es más frecuente en las mujeres (9:1) y en personas con alteraciones del comportamiento alimentario. El propósito de este trabajo es documentar un caso de tricobezoar en una niña de 9 años que se presentó con síntomas inespecíficos que se confundieron con los de la anorexia nerviosa de la que previamente había sido diagnosticada (AU)

A bezoar is a collection of diverse aetiology that can be found in stomach or intestine of men or other animals. Those made basically of hair are called trichobezoar. This rare pathology is more usual in the group of age under 30 years old and also in women (9:1), basically if they associate eating disorders. Our objective was to relate a case of trichobezoar in a 9 year-old-girl, with non-specific sintomatology that was confounded with the clinic of anorexia nervosa, which she had been previously diagnosed (AU)

TRAF4 functions as an intermediate of GITR-induced NF-kappaB activation.

Members of the tumor necrosis factor receptor (TNFR) family regulate the activation, differentiation, and function of many cell types, including cells of the immune system. TNFR-associated factors (TRAFs) function as adapter molecules controlling signaling pathways triggered by TNFR family members, such as activation of nuclear factor kappaB (NF-kappaB). Despite intensive research, the function of TRAF4 in signaling pathways triggered by TNFR-related proteins remains enigmatic. Intriguingly, our functional studies indicated that TRAF4 augments NF-kappaB activation triggered by glucocorticoid-induced TNFR (GITR), a receptor expressed on T cells, B cells, and macrophages. Further analyses revealed that TRAF4-mediated NF-kappaB activation downstream of GITR depends on a previously mapped TRAF-binding site in the cytoplasmic domain of the receptor and is inhibited by the cytoplasmic protein A20. GITR is thought to inhibit the suppressive function of regulatory T cells (Treg cells) and to promote activation of T cells. Taken together, our studies provide the first indications that TRAF4 elaborates GITR signaling and suggest that TRAF4 can modulate the suppressive functions of Treg cells.

Importancia de los registros en la cura de las úlceras por presión / Importance of the registers in the cure of the pressure ulcers

En el trabajo que desarrollamos a continuación queremos señalar la importancia de Los registros en la cura de las ulceras por presión (UPP) ya que estos nos han permitido realizar un total seguimiento tanto del paciente como de las úlceras que presenta, y conseguir una transmisión eficaz de la información sobre la evolución y tratamiento de las mismas, que es válida tanto para el personal fijo como para el nuevo o eventual. Para lograr este resultado se debe hacer una previa valoración de la úlcera además de una valoración integral del estado del paciente. Los datos obtenidos se registrarán en una primera hoja única al inicio del estudio (hoja de valoración).En un segundo modelo de hoja (hoja de evolución) se registrará periódicamente la evolución de las UPP cada vez que se realice la cura, y se hará un seguimiento fotográfico quincenal de las mismas. Al observar el registro, se obtiene una rápida y fácil visualización de la evolución de la úlcera desde el inicio del estudio hasta la fecha y del tratamiento y medidas preventivas adoptadas. A pesar de no ser un objetivo buscado, la utilización de los registros nos ha servido para unificar criterios de actuación del personal de Enfermería ante la prevención y el tratamiento de las UPP (AU)

Effects of dietary nitrogen manipulation on ammonia volatilization from manure from Holstein heifers.

Decomposition of livestock manure produces gaseous ammonia. Dietary manipulation is one means to reduce N in manure and ammonia volatilization. The effects of dietary crude protein concentration on N intake, N and urinary urea-N excretion, and ammonia volatilization were measured. Eight Holstein heifers (body weight = 260 to 488 kg) were fed a total mixed ration containing either 9.6 or 11.0% crude protein in a crossover design. Oatlage and concentrate were fed at 77:23 (dry matter basis), and soybean meal was used to alter total dietary crude protein. Seven-day adjustment periods preceded 5-d collection periods. Indwelling urinary catheters were inserted 2 d prior to the collection periods. Daily feces and acidified urine were collected, stirred, and subsampled for total Kjeldahl N, urinary urea N, dry matter, P, K, and ash. Urine collection tubes were split during period 2 to allow for collection of unacidified samples for urea N and total N determinations. Unacidified urine and fecal samples were combined (1:1.3) for collection of volatilized ammonia. Remaining slurries were extracted for total and urea N. Increased dietary crude protein concentration increased N intake, N excretion, urea-N excretion, and N excreted in the urine by the heifers. Dietary manipulation of N intake by reduction of 14.0% (dry matter basis) resulted in a 28.1% decrease in ammonia emission and decreases in the urea N, total N, and percentage N excreted in the urine of 29.6, 19.8, and 7.4%, respectively. Ammonia volatilization was dependent on N quantity and form in the urine.