Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the "no evidence of disease activity" parameter.

INTRODUCTION: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions. OBJECTIVE: To determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the "no evidence of disease activity" (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters. PATIENTS AND METHODS: We performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions. RESULTS: The study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti-JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy. CONCLUSIONS: Natalizumab is highly effective as measured by the NEDA long-term remission parameter.

Evolución de una serie de pacientes con esclerosis múltiple remitente-recurrente tratados con natalizumab durante 7 años mediante el parámetro no evidencia enfermedad activa / Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the “no evidence of disease activity” parameter

Introducción: La efectividad y seguridad de natalizumab en pacientes con esclerosis múltiple remitente recurrente (EMRR) se demostró en ensayos clínicos. Sin embargo, por las limitaciones de estos es importante saber cómo se comporta en condiciones de práctica clínica a largo plazo.ObjetivoConocer la eficacia a largo plazo de natalizumab en pacientes con EMRR mediante la evaluación anual del no evidence of disease activity (NEDA), que incluye número de brotes, discapacidad medida con EDSS y parámetros de RM cerebral.Pacientes y métodosEstudio retrospectivo y multicéntrico (n = 3) de pacientes con EMRR tratados con una o más dosis de natalizumab. Se evaluó el estado NEDA cada año y la seguridad a partir del porcentaje de pacientes que discontinuaron y que presentaron efectos adversos.ResultadosIncluimos 89 pacientes, la mayoría recibieron tratamiento durante 2 a 4 años, con una duración del seguimiento de hasta 7 años. Natalizumab reduce significativamente la progresión radiológica y clínica de la enfermedad, así como la tasa anual de brotes, demostrándose su eficacia con el parámetro NEDA, 75,28% al primer año y 66,67% al séptimo año. Veinticinco pacientes (28,1%) han abandonado el estudio en una mediana de tiempo de 4 años, 14 pacientes (56%) por aparición de anticuerpos contra el virus JC, como causa única o asociada a otro motivo, 4 abandonos (16%) fueron por ineficacia, un paciente falleció a causa de LMP.ConclusionesNatalizumab presenta una alta eficacia medida mediante el parámetro de remisión NEDA a largo plazo. (AU)

Introduction: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions.ObjectiveTo determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the “no evidence of disease activity” (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters.Patients and methodsWe performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions.ResultsThe study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti–JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy.ConclusionsNatalizumab is highly effective as measured by the NEDA long-term remission parameter. (AU)

Energy, nitrogen partitioning, and methane emissions in dairy goats differ when an isoenergetic and isoproteic diet contained orange leaves and rice straw crop residues.

The aim of this study was to evaluate the effects of incorporating rice straw and orange leaves into the diets for goats. Ten Murciano-Granadina goats at mid lactation weighing 45 ± 0.3 kg were used in a crossover design. Two isoproteic and isoenergetic diets (180 g/kg DM and 17 MJ/kg DM, respectively) with alfalfa hay as forage source (33% of DM) were fed. A control diet (CON) incorporated barley as energy source and soy hulls as fiber component. The experimental diet (ORG) replaced barley and soy hulls with orange leaves (19% on DM basis), rice straw (12%, on DM basis) and soya oil (2%). Peas and horsebeans were the protein source in both diets. Each goat received the 2 treatments in 2 periods. Goats were fed the experimental diets and after 14 d on their respective treatments moved to individual metabolism cages for another 7 d. Subsequently, feed intake, total fecal and urine output and milk yield were recorded daily over the first 5 d. During the next 2 d ruminal fluid and blood samples were collected, and then individual gas-exchange measurements were recorded by a mobile open-circuit indirect calorimetry system using a head box. No differences in dry matter intake were detected, and apparent total-tract digestibility was greater in CON than ORG. Efficiency of metabolizable energy intake for milk and maintenance also was lower in response to ORG (0.65 vs. 0.63), with energy balance being negative (-12 kJ/kg of BW0.75) due to mobilization of fat (-16 g/animal vs. 68 g/animal for ORG and CON, respectively). Although actual milk yield was lower in goats fed ORG (2.32 vs. 2.06 kg/d, respectively), energy-corrected milk did not differ (2.81 kg/d on average). In terms of milk quality, milk fat content, and concentrations of monounsaturated (18.54 vs. 11.55 g/100 g milk fat) and polyunsaturated fatty acids (5.75 vs. 3.99 g/100 g milk fat) were greater in goats fed ORG. Based on various indices, the milk produced by ORG would be less atherogenic and thrombogenic than CON milk. Compared with CON, enteric CH4 emission was lower due to feeding ORG (reduction of 38 g CH4/kg milk fat). Data suggest that greater fat mobilization in goats fed ORG might have been due to the apparent lack of synchrony between degradable protein and carbohydrate and the lipogenic nutrients associated with the lower cereal content of the ORG diet. Thus, goats fed ORG seemed to rely more on fat depots to help meet energy requirements and reach optimal performance. As such, the lower content of glucogenic nutrients in ORG did not favor body fat deposition and partitioning of ME into body tissue. Overall, responses in terms of CH4 emissions and milk quality suggest that inclusion of rice straw and orange leaves in diets for small ruminants could be a valuable alternative to reuse, recycle and revalue agricultural by-products.

Development of a dynamic energy-partitioning model for enteric methane emissions and milk production in goats using energy balance data from indirect calorimetry studies.

The main objective of this study was to develop a dynamic energy balance model for dairy goats to describe and quantify energy partitioning between energy used for work (milk) and that lost to the environment. Increasing worldwide concerns regarding livestock contribution to global warming underscore the importance of improving energy efficiency utilization in dairy goats by reducing energy losses in feces, urine and methane (CH4). A dynamic model of CH4 emissions from experimental energy balance data in goats is proposed and parameterized (n = 48 individual animal observations). The model includes DM intake, NDF and lipid content of the diet as explanatory variables for CH4 emissions. An additional data set (n = 122 individual animals) from eight energy balance experiments was used to evaluate the model. The model adequately (root MS prediction error, RMSPE) represented energy in milk (E-milk; RMSPE = 5.6%), heat production (HP; RMSPE = 4.3%) and CH4 emissions (E-CH4; RMSPE = 11.9%). Residual analysis indicated that most of the prediction errors were due to unexplained variations with small mean and slope bias. Some mean bias was detected for HP (1.12%) and E-CH4 (1.27%) but was around zero for E-milk (0.14%). The slope bias was zero for HP (0.01%) and close to zero for E-milk (0.10%) and E-CH4 (0.22%). Random bias was >98% for E-CH4, HP and E-milk, indicating non-systematic errors and that mechanisms in the model are properly represented. As predicted energy increased, the model tended to underpredict E-CH4 and E-milk. The model is a first step toward a mechanistic description of nutrient use by goats and is useful as a research tool for investigating energy partitioning during lactation. The model described in this study could be used as a tool for making enteric CH4 emission inventories for goats.

Urgencias dermatológicas: estudio descriptivo en un hospital terciario / Dermatological emergencies: A descriptive study in a tertiary hospital

INTRODUCCIÓN: El número de pacientes que acude a los servicios de Urgencias para consultar enfermedades dermatológicas está incrementándose. Sin embargo, se desconoce hasta qué punto una enfermedad cutánea es lo suficientemente urgente como para requerir atención especializada de forma inmediata. Los objetivos de este estudio fueron determinar la prevalencia y el tipo de enfermedades dermatológicas atendidas en una consulta de Urgencias de Dermatología de un hospital terciario, conocer el perfil epidemiológico del paciente demandante de la consulta urgente y determinar la vía de acceso. MATERIAL Y MÉTODO: Estudio descriptivo prospectivo de los pacientes que acudieron al servicio de Urgencias de un hospital con afección dermatológica durante 5 meses. Se recogieron la filiación del paciente, las características epidemiológicas básicas, la procedencia, el diagnóstico dermatológico y el destino. RESULTADOS: Se atendió a 242 pacientes (49% mujeres, 51% hombres). La edad media fue de 49,2 años. Se realizaron 78 diagnósticos distintos, los cuales se clasificaron en 12 grupos, destacando en primer lugar los eccemas (17,8%), seguidos de miscelánea (17,4%), tumores y quistes (16,2%) y dermatosis infecciosas (15%). Se realizaron 7 ingresos (2,9%). CONCLUSIONES: Las entidades dermatológicas atendidas fueron muy variadas. Las 3enfermedades más frecuentes fueron la psoriasis, los eccemas y las toxicodermias. El paciente demandante de la consulta urgente fue una persona de edad media, sin predominio sexual. La mayoría de los pacientes acudieron por iniciativa propia. El motivo más frecuente de ingreso fue la eritrodermia psoriásica

INTRODUCTION: Nowadays, there is an increasing number of patients who seek emergency treatment for their dermatology complaints. However, it is unknown to what extent a skin disease is urgent enough to require immediate specialised care. Our aims were to assess the type and prevalence of the dermatological diseases treated in the division of Dermatology of a tertiary hospital, as well as to determine the main features of the patients with these disorders and the means of access to the Dermatology Department. MATERIAL AND METHOD: A descriptive and prospective study was conducted on patients with dermatological conditions diagnosed in the Dermatology outpatient clinic of a hospital during a 5-month period. For this purpose, the information corresponding to health cover, basic epidemiological characteristics, origin, diagnosis, and destiny of each patient was entered into a database. RESULTS: The dermatologist attended 242 patients, of whom 49% were women and 51% men. The mean age was 49.2 years. The 78 different diagnoses made were grouped into 12 categories to facilitate analysis. According to this classification, most patients had eccemas (17.8%), followed by miscellaneous (17.4%), tumours and cysts (16.2%), and infectious dermatoses (15%). In 7 (2.9%) cases, the patient was admitted to hospital. CONCLUSIONS: Dermatological diseases attended to in the Dermatology Department were varied. The most common diagnoses were psoriasis, eccemas, and toxicoderma. The profile of the patient seeking emergency dermatological care is a person between the fourth and fifth decade of the life, who arrives directly to the Emergency Department without requesting previous assessment by a Primary Care physician. The most common reason for admission was erythrodermic psoriasis

[Dermatological emergencies: A descriptive study in a tertiary hospital]. / Urgencias dermatológicas: estudio descriptivo en un hospital terciario.

INTRODUCTION: Nowadays, there is an increasing number of patients who seek emergency treatment for their dermatology complaints. However, it is unknown to what extent a skin disease is urgent enough to require immediate specialised care. Our aims were to assess the type and prevalence of the dermatological diseases treated in the division of Dermatology of a tertiary hospital, as well as to determine the main features of the patients with these disorders and the means of access to the Dermatology Department. MATERIAL AND METHOD: A descriptive and prospective study was conducted on patients with dermatological conditions diagnosed in the Dermatology outpatient clinic of a hospital during a 5 month period. For this purpose, the information corresponding to health cover, basic epidemiological characteristics, origin, diagnosis, and destiny of each patient was entered into a database. RESULTS: The dermatologist attended 242 patients, of whom 49% were women and 51% men. The mean age was 49.2 years. The 78 different diagnoses made were grouped into 12 categories to facilitate analysis. According to this classification, most patients had eccemas (17.8%), followed by miscellaneous (17.4%), tumours and cysts (16.2%), and infectious dermatoses (15%). In 7 (2.9%) cases, the patient was admitted to hospital. CONCLUSIONS: Dermatological diseases attended to in the Dermatology Department were varied. The most common diagnoses were psoriasis, eccemas, and toxicoderma. The profile of the patient seeking emergency dermatological care is a person between the fourth and fifth decade of the life, who arrives directly to the Emergency Department without requesting previous assessment by a Primary Care physician. The most common reason for admission was erythrodermic psoriasis.

How is an ideal satiating yogurt described? A case study with added-protein yogurts.

Protein is recognized as the macronutrient with the highest satiating ability. Yogurt can be an excellent basis for designing satiating food as it is protein-based food product. Five different set-type yogurts were formulated by adding extra skim milk powder (MP), whey protein concentrate (WPC), calcium caseinate (CAS) or a blend of whey protein concentrate with calcium caseinate (CAS-WPC). A control yogurt without extra protein content was also prepared. Differences in sensory perceptions (through CATA questions) were related to the consumers' expected satiating ability and liking scores (of several modalities). In addition, an "Ideal satiating yogurt" was included in the CATA question to perform a penalty analysis to show potential directions for yogurt reformulation and to relate sensory and non-sensory yogurt characteristics to satiating capacity.

High hydrostatic pressure treatment provides persimmon good characteristics to formulate milk-based beverages with enhanced functionality.

High hydrostatic pressure (HHP) applied during food processing can improve the retention of food quality attributes and nutritional values in comparison with pasteurization. Persimmon is a good source of bioactive compounds but it is a seasonal fruit that cannot be consumed throughout the year. The aim of this work was to compare the HHP and pasteurization treatments to formulate milk-based beverages containing this carotenoid rich ingredient and to evaluate their performance in these beverages. The carotenoid and tannin contents of persimmon were determined and the microstructure and rheology of the new milk-based persimmon beverages were examined. The results showed that HHP treatment favoured the release of carotenoids from the fruit matrix and precipitation of the tannins. The milk-based beverages prepared with the high-pressure persimmon presented the best rheological properties because unlike the untreated and pasteurized persimmon milk-based beverages, they did not form a gel-like structure or separate out.

Ataxia y nistagmo de origen infrecuente / Ataxia and nystagmus of unusual origin

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Impact of high hydrostatic pressure and pasteurization on the structure and the extractability of bioactive compounds of persimmon "Rojo Brillante".

Rojo Brillante is an astringent oriental persimmon variety with high levels of bioactive compounds such as soluble tannins, carotenoids, phenolic acids, and dietary fiber. The purpose of this study was to investigate the effects of high hydrostatic pressure (HHP) and pasteurization on the structure of the fruit and on the extractability of certain bioactive compounds. The microstructure was studied using light microscopy, transmission electron microscopy, and low temperature scanning electron microscopy, and certain physicochemical properties (carotenoid and total soluble tannin content, antioxidant activity, fiber content, color, and texture properties) were measured. The structural changes induced by HHP caused a rise in solute circulation in the tissues that could be responsible for the increased carotenoid level and the unchanged antioxidant activity in comparison with the untreated persimmon. In contrast, the changes that took place during pasteurization lowered the tannin content and antioxidant activity. Consequently, HHP treatment could improve the extraction of potentially bioactive compoundsxsts from persimmons. A high nutritional value ingredient to be used when formulating new functional foods could be obtained using HHP.

Analysis of direct, indirect, and intangible costs of epilepsy.

INTRODUCTION: A financial estimate has been made of the costs of epilepsy in adults. METHODS: A prospective, observational study, over a period of 6 months, on epileptic patients over 14 years-old. Patients with concomitant diseases that could influence the outcome of the epilepsy were excluded. The direct costs included: treatment received, number of visits to neurology, primary care, and emergencies, number of days admitted to hospital, number and type of diagnostic tests, use of transport to and from hospital, and psychopedagogic and social support due to the epilepsy. The indirect costs were analysed according to, loss of work productivity of the patients, taking into account families where the patient needed supervision due to epilepsy. The total costs were derived from the sum of the direct and indirect costs. The intangible costs were calculated according to QOLIE-10 questionnaire. RESULTS: The mean direct cost per patient was 1,055.2 €. The mean indirect financial costs came to 1,528.8 € per patient. The total cost associated to epilepsy was a mean of 2,584 € for each patient, mainly arising from loss of work days (p<.05). For intangible costs according to the QOLIE-10 scale a mean of 77.8 was obtained. CONCLUSIONS: The greatest percentage of costs associated to epilepsy is due to the work productivity loss by the patients. The costs of psychological and social suffering in epilepsy lead to a deterioration in the quality of life.

The c.859G>C variant in the SMN2 gene is associated with types II and III SMA and originates from a common ancestor.

Homozygous mutations of the telomeric SMN1 gene lead to degeneration of motor neurons causing spinal muscular atrophy (SMA). A highly similar centromeric gene (SMN2) can only partially compensate for SMN1 deficiency. The c.859G>C variant in SMN2 has been recently reported as a positive disease modifier. We identified the variant in 10 unrelated chronic SMA patients with a wide spectrum of phenotypes ranging from type II patients who can only sit to adult walkers. Haplotype analysis strongly suggests that the variant originated from a common ancestor. Our results confirm that the c.859G>C variant is a milder SMN2 allele and predict a direct correlation between SMN activity and phenotypic severity.

Respuesta a: Leucoencefalopatía con sustancia blanca evanescente de inicio en edad adulta / Hospital Povisa

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Leucoencefalopatía con sustancia blanca evanescente: caso clínico de inicio en adulto / No disponible

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Improving the quality of fresh-cut apples, pears, and melons using natural additives.

Improving the quality of different fresh-cut fruits by adding natural substances was studied. "Fuji" apples, "Flor de Invierno" pears, and "Piel de Sapo" melons were treated with calcium lactate, N-acetyl-L-cysteine, glutathione, and malic acid and stored for 4 wk at 4 degrees C. Instrumental texture (penetration), microstructure (light microscopy), acidity, soluble solids, color, pectinmethylesterase activity, and microflora were studied. The results showed that the combined treatment reinforced the cell walls strengthening the structure and texture of these fruits and maintained the L* and a* values throughout 4 wk of storage at 4 degrees C. The combination of additives provided low microbial counts in apples until the 4th week and in melons until the 2nd week. So, this combined treatment could be used to extend the shelf life of some fresh-cut fruits while preserving their quality.

Atresia yeyunal y fibrosis quística: presentación de un caso / Jejunal atresia and cystic fibrosis: report of a case

La atresia yeyunal es la causa más común de obstrucción intestinal en el recién nacido. Es una malformación congénita rara que se cree que puede ser causada por obstrucciones vasculares intestinales ocurridas en la vida intrauterina. En algunos casos se ha referido una base hereditaria autosómica recesiva. En niños con atresia yeyunal se ha observado un incremento de la frecuencia de fibrosis quística. Se presenta el caso de un recién nacido que falleció por atresia yeyunal; sus progenitores fueron diagnosticados como portadores heterocigotos de la mutación ΔF508 de fibrosis quística y se pudo confirmar molecularmente la enfermedad en él mediante el estudio en tejido parafinado. Se discuten las implicaciones de la asociación de atresia yeyunal y fibrosis quística en el manejo de los pacientes afectados y en el asesoramiento genético familiar

The most common cause of bowel obstruction in thenewborn is jejunal atresia. It´s a rare congenital malformation that it is believed could be caused by bowel vascular obstruction during intrauterine life. In some cases, an autosomal recesive inheritance has been referred. In infants affected by jejunal atresia an increased frequency of cystic fibrosis has been observed. A case is reported where a newborndied by jejunal atresia. His parents are both heterozygote for the cystic fibrosis allele ΔF508 and the disease was confirmed through molecular genetics study un parafined tissues. The implications on management of patients and familial genetic counselling, are discussed (AU)

Rehydration of freeze-dried and convective dried boletus edulis mushrooms: effect on some quality parameters.

Quality of rehydrated products is a key aspect linked to rehydration conditions. To assess the effect of rehydration temperature on some quality parameters, experiments at 20 and 70 degrees C were performed with convective dried and freeze-dried Boletus edulis mushrooms. Rehydration characteristics (through Peleg's parameter, k(1), and equilibrium moisture, W(e)), texture (Kramer), and microstructure (Cryo-Scanning Electron Microscopy) were evaluated. Freeze-dried samples absorbed water more quickly and attained higher W(e) values than convective dried ones. Convective dehydrated samples rehydrated at 20 degrees C showed significantly lower textural values (11.9 +/- 3.3 N/g) than those rehydrated at 70 degrees C (15.7 +/- 1.2 N/g). For the freeze-dried Boletus edulis, the textural values also exhibited significant differences, being 8.2 +/- 1.3 and 10.5 +/- 2.3 N/g for 20 and 70 degrees C, respectively. Freeze-dried samples showed a porous structure that allows rehydration to take place mainly at the extracellular level. This explains the fact that, regardless of temperature, freeze-dried mushrooms absorbed water more quickly and reached higher W(e) values than convective dried ones. Whatever the dehydration technique used, rehydration at 70 degrees C produced a structural damage that hindered water absorption; consequently lower W(e) values and higher textural values were attained than when rehydrating at 20 degrees C.