Comparison of dehydration systems in quality and chemical effects on sunflower seeds.

The present work investigates the quality and the chemical effects of dehydration, using a novel dehydration system based on an electromagnetic induction and low pressures technique, comparing it with the thermo-solar drying system. High oleic sunflower seeds, which are an important oil seed crop, were used due to the fact that they have a special place in the food industry. The seed samples were exposed to electromagnetic induction and low pressures by 0.5 and 1 h, then several chemical characterizations were carried out, in the electrophoresis study, it was found that most proteins in the hull were degraded or denatured, some of them were lost during the time in the thermosolar dryer while in kernel keeps 94.9% of the concentration in control proteins. Otherwise, the electromagnetic induction dryer did not lose the most of proteins in the kernel keeping 99.1% in 0.5 h and 98.4% in 1 h, just degrading its concentration. Germination viability results did not show changes after 0.5 h in the electromagnetic fields, but they decreased in 1 h from 66 to 40% until the thermosolar method fell to 24% in 4 h, both analysis results change proportionally with the treatment time and moisture content and the amount of the oxygen.

Psychological profiles and prescription opioid misuse, craving, and withdrawal in people with chronic pain.

BACKGROUND: The negative consequences of prescription opioid misuse and opioid use disorder make it relevant to identify factors associated with this problem in individuals with chronic pain. This cross-sectional study aimed at identifying subgroups of people with chronic pain based on their psychological profiles, prescription opioid misuse, craving, and withdrawal. METHODS: The sample comprised 185 individuals with chronic pain. We performed hierarchical cluster analysis on impulsivity, anxiety sensitivity, pain acceptance, pain intensity, opioid misuse, craving, and withdrawal. RESULTS: The four-cluster solution was the optimal one. Misuse, craving, and anxiety sensitivity were higher among people in the Severe-problems cluster than among people in the other three clusters. Withdrawal was the highest in the High-withdrawal cluster. Impulsivity was higher among people in the Severe-problems and High-withdrawal clusters than those in the Moderate-problems and Mild-problems clusters. Pain acceptance was higher among people in the Mild-problems cluster than among people in the other three clusters. Anxiety sensitivity and misuse were higher among people in the Moderate-problems cluster than among people in the Mild-problems cluster. CONCLUSIONS: These results support that impulsivity, anxiety sensitivity, and pain acceptance are useful constructs to identify subgroups of people with chronic pain according to their level of prescription opioid misuse, craving, and withdrawal. The results of this study may help in selecting the early intervention most suitable for each of the identified profiles. SIGNIFICANCE: The psychological profile of individuals with chronic pain, prescription opioid misuse, craving, and withdrawal is characterized by fearing anxiety-related symptoms due to the catastrophic interpretation of such symptoms and reacting impulsively to negative moods. In contrast, participants with high pain acceptance had less prescription opioid misuse, craving, and withdrawal. The profiles identified in this study could help clinicians select targets for intervention among profiles with similar needs and facilitate early interventions to prevent opioid misuse onset or aggravation.

Evolución de los pacientes con insuficiencia cardiaca aguda secundaria a infarto agudo de miocardio tratados con ventilación mecánica no invasiva / Outcome of patients with acute heart failure secondary to acute myocardial infarction treated with noninvasive mechanical ventilation

Introducción y objetivos: La ventilación no invasiva (VNI) es capaz de reducir la necesidad de intubación endotraqueal y la mortalidad de los pacientes con insuficiencia cardiaca aguda (ICA). Sin embargo, de muchos de los ensayos clínicos se ha excluido a los pacientes con ICA secundaria a síndrome coronario agudo o infarto agudo de miocardio (SCA-IAM). El objetivo de este estudio es comparar la efectividad de la VNI entre pacientes con ICA desencadenada por SCA-IAM y por otras causas. Métodos: Estudio prospectivo de cohortes, durante un periodo de 20 años, de todos los pacientes con ICA tratados con VNI ingresados en una unidad de cuidados intensivos. Se agrupó a los pacientes por la presencia o ausencia de SCA-IAM como causante del evento de ICA. Se definió el fracaso de la VNI por la necesidad de intubación endotraqueal o muerte. Resultados: Se analizó a 1.009 pacientes, 403 (40%) con SCA-IAM y 606 (60%) con otras etiologías. La VNI fracasó en 61 casos (15,1%) del grupo de SCA-IAM y 64 (10,6%) del grupo sin SCA-IAM (p=0,031), sin diferencias en la mortalidad hospitalaria (el 16,6 y el 14,9%; p=0,478). Conclusiones: El SCA-IAM como causa desencadenante de la ICA no influye en el pronóstico de los pacientes con insuficiencia respiratoria aguda que precisan asistencia respiratoria no invasiva (AU)

Introduction and objectives: Noninvasive ventilation (NIV) has been shown to reduce the rate of endotracheal intubation and mortality in patients with acute heart failure (AHF). However, patients with AHF secondary to acute coronary syndrome/acute myocardial infarction (ACS-AMI) have been excluded from many clinical trials. The purpose of this study was to compare the effectiveness of NIV between patients with AHF triggered by ACS-AMI and by other etiologies. Methods: Prospective cohort study of all patients with AHF treated with NIV admitted to the intensive care unit for a period of 20 years. Patients were divided according to whether they had ACS-AMI as the cause of the AHF episode. NIV failure was defined as the need for endotracheal intubation or death. Results: A total of 1009 patients were analyzed, 403 (40%) showed ACS-AMI and 606 (60%) other etiologies. NIV failure occurred in 61 (15.1%) in the ACS-AMI group and in 64 (10.6%) in the other group (P=.031), without differences in in-hospital mortality (16.6% and 14.9%, respectively; P=.478). Conclusions: The presence of ACS-AMI as the triggering cause of AHF did not influence patients with acute respiratory failure requiring noninvasive respiratory support (AU)

[Recurrent stroke, acrocyanosis and livedo racemosa: is it always Sneddon's syndrome?] / Ictus recidivante, acrocianosis y livedo racemosa: ¿siempre síndrome de Sneddon?

TITLE: Ictus recidivante, acrocianosis y livedo racemosa: ¿siempre síndrome de Sneddon?

Diagnóstico de la embolia pulmonar. El punto de vista del médico clínico / Diagnosing pulmonary embolisms: the clinician's point of view

La tromboembolia pulmonar (TEP) es una enfermedad frecuente y potencialmente grave. Es necesario el conocimiento de los conceptos básicos que rigen la utilidad de las pruebas para poder realizar una aproximación correcta a su proceso diagnóstico. La TEP se caracteriza porque su diagnóstico es un ejemplo de la aplicación de las probabilidades condicionadas de Bayes a la clínica diaria. Para una correcta interpretación de las pruebas diagnósticas disponibles, es necesario analizar diferentes conceptos que son fundamentales para la toma de decisiones. Es preciso conocer el significado de los cocientes de probabilidad, los intervalos de confianza del 95% y los umbrales de decisión. Así, la utilidad de la determinación del dímero D, o la realización de una angiografía pulmonar mediante tomografía computarizada u otras pruebas de imagen, se debe a la capacidad de modificar la probabilidad pretest de padecer la enfermedad a una probabilidad posterior que se encuentre por encima o por debajo de los umbrales de acción. El objetivo de esta revisión es clarificar la secuencia diagnóstica de la enfermedad tromboembólica pulmonar, analizando las principales herramientas diagnósticas, clínicas, analíticas y de imagen, poniendo especial énfasis en los principios que rigen la medicina basada en la evidencia (AU)

Pulmonary thromboembolism is common and potentially severe. To ensure the correct approach to the diagnostic workup of pulmonary thromboembolism, it is essential to know the basic concepts governing the use of the different tests available. The diagnostic approach to pulmonary thromboembolism is an example of the application of the conditional probabilities of Bayes’ theorem in daily practice. To interpret the available diagnostic tests correctly, it is necessary to analyze different concepts that are fundamental for decision making. Thus, it is necessary to know what the likelihood ratios, 95% confidence intervals, and decision thresholds mean. Whether to determine the D-dimer concentration or to do CT angiography or other imaging tests depends on their capacity to modify the pretest probability of having the disease to a posttest probability that is higher or lower than the thresholds for action. This review aims to clarify the diagnostic sequence of thromboembolic pulmonary disease, analyzing the main diagnostic tools (clinical examination, laboratory tests, and imaging tests), placing special emphasis on the principles that govern evidence-based medicine (AU)

The Key Role of Pain Catastrophizing in the Disability of Patients with Acute Back Pain.

PURPOSE: This study investigated the role of anxiety sensitivity, resilience, pain catastrophizing, depression, pain fear-avoidance beliefs, and pain intensity in patients with acute back pain-related disability. METHOD: Two hundred and thirty-two patients with acute back pain completed questionnaires on anxiety sensitivity, resilience, pain catastrophizing, fear-avoidance beliefs, depression, pain intensity, and disability. RESULTS: A structural equation modelling analysis revealed that anxiety sensitivity was associated with pain catastrophizing, and resilience was associated with lower levels of depression. Pain catastrophizing was positively associated with fear-avoidance beliefs and pain intensity. Depression was associated with fear-avoidance beliefs, but was not associated with pain intensity. Finally, catastrophizing, fear-avoidance beliefs, and pain intensity were positively and significantly associated with acute back pain-related disability. CONCLUSION: Although fear-avoidance beliefs and pain intensity were associated with disability, the results showed that pain catastrophizing was a central variable in the pain experience and had significant direct associations with disability when pain was acute. Anxiety sensitivity appeared to be an important antecedent of catastrophizing, whereas the influence of resilience on the acute back pain experience was limited to its relationship with depression.

Consumo de recursos sanitarios en atención primaria de los prematuros tardíos. Estudio caso-control en un centro de salud urbano / Use of health care resources by late premature neonates in primary care. Case-control study in a health area

No disponible

Ossiculum terminale de Bergman. Una entidad poco conocida / Ossiculum terminale Bergmann. A poorly known entity

No disponible

An examination of the structural link between post-traumatic stress symptoms and chronic pain in the framework of fear-avoidance models.

BACKGROUND: The tendency to respond with fear and avoidance can be seen as a shared vulnerability contributing to the development of post-traumatic stress disorder (PTSD) and chronic pain. Although several studies have examined which specific symptoms of PTSD (re-experiencing, avoidance, emotional numbing and hyperarousal) are associated with chronic pain, none has considered this association within the framework of fear-avoidance models. METHODS: Seven hundred fourteen patients with chronic musculoskeletal pain were assessed. Of these, 149 patients were selected for the study based upon the following inclusion criteria: exposure to a traumatic event before the onset of pain (with scores equal to or higher than 8 points on the fear and hopelessness scales of the Stressful Life Event Screening Questionnaire Revised) and scores equal to or higher than 30 on the Davidson Trauma Scale. RESULTS: Structural equation modelling was used to test the association between PTSD symptoms and pain outcomes (pain intensity and disability) using the mediating variables considered in the fear-avoidance models. The results show that emotional numbing and hyperarousal symptoms, but neither re-experiencing nor avoidance, affected pain outcome via anxiety sensitivity (AS), catastrophizing and fear of pain. PTSD symptoms increased the levels of AS, which predisposes to catastrophizing and, in turn, had an effect on the tendency of pain patients to respond with more fear and avoidance. CONCLUSIONS: This study provides empirical support for the potential role of PTSD symptoms in fear-avoidance models of chronic pain and suggests that AS is a relevant variable in the relationship between both disorders.

Comparing the crystallization and rheological behavior of organogels developed by pure and commercial monoglycerides in vegetable oil.

We investigated the crystallization and rheological behavior of organogels developed with commercial (MSGC) and pure (MSGP) monoglycerides in safflower oil solutions (0.5% to 8% wt/wt). The MSGC was composed of 1-mono-stearoyl-glycerol (1-MSG, 37.7%) and 1-mono-palmitoyl-glycerol (1-MPG, 54.0%), and the MSGP essentially by 1-MSG (93.51%). The elastic (G') and loss (G″) moduli of the MSGC and MSGP-oil solutions were measured from 80°C until achieving 5°C, and then during isothermal conditions. The d(G')/d(time) rheograms, where d(G')/d(time) is the difference in G' between subsequent time-temperature conditions during cooling, followed closely the phase transition observed by the monoglycerides (MG). The d(G')/d(time) profile showed that the formation of the inverse lamellar α mesophase provided a limited structure to the vegetable oil. In contrast, the crystallization of the sub-α phase in the MSGC-oil system, and of the sub-α1 and sub-α2 phases in the MSGP-oil system structured the vegetable oil through the uptake and retention of oil within their microstructure. Additionally, smaller crystals formed the three-dimensional crystal structure in the MSGC organogels. This is in comparison with the larger crystal size observed in MSGP organogels. Nevertheless, for a similar MG concentration the MSGC organogels showed higher G' and solid fat content (SFC) than the MSGP organogels, and the differences were greater as the MG concentration increased. We consider that the mixed sub-α structure developed by 1-MSG and 1-MPG in the MSGC-oil systems favored the incorporation and retention of higher amounts of oil, in comparison with the sub-α1 and sub-α2 structures developed just by 1-MSG in the MSGP-oil systems.

Encuesta de conocimiento de precios de la actividad sanitaria: ¿sabemos cuánto cuestan nuestras decisiones? / Survey of knowledge about health care activity prices: do we know how much our decisions cost?

Objetivo: Medir el conocimiento del precio de nuestras decisiones en la práctica clínica habitual en un Área de Gestión Clínica Pediátrica de un hospital público. Material y métodos: Encuesta individual escrita y anónima al personal sanitario [pediatras (FEAs), médicos internos residentes (MIRs) y personal de enfermería (DUEs)] del Área de Gestión Clínica de Pediatría de un hospital universitario. Se preguntó por el precio de ítems de actividad sanitaria: consulta/hospitalización; pruebas de laboratorio; pruebas de imagen; pruebas microbiológicas; material y técnicas; y tratamientos. Las respuestas se analizaron frente a valores de referencia en nuestro hospital. Resultados: Se analizaron 97 cuestionarios. El precio global fue sobrevalorado un 5% del precio real, aunque el 56% de encuestados infravaloró dicho precio global. Agrupados en seis grupos de ítems, fármacos y consultas/hospitalizaciones se infravaloraron, mientras que el resto de grupos se sobrevaloraron. La suma del precio de todos los fármacos se infravaloró en el 10% y la de todas las modalidades de consulta/hospitalización en el 25%. Los otros cuatro grupos se sobrevaloraron: analíticas de sangre en el 589%, material de técnicas en el 128%, pruebas microbiológicas en el 81% y pruebas de imagen en el 57%. En el análisis global de encuesta, las comparaciones por grupos no mostraron diferencias estadísticas significativas para total de encuesta por sexos ni por grupos de edad, pero sí para tipo de profesional (ANOVA p=0,03). MIRes y FEAs infravaloraban la encuesta, mientras que DUEs la sobreestimaban. Conclusiones: Existe un gran desconocimiento en el valor económico de costes sanitarios y gran variabilidad en su apreciación, entre el personal que trabaja en el sistema público de salud

Objective: To measure degree of knowledge about how much daily clinical practice cost in a pediatric clinical hospital. Material and methods: Individual, anonymous and written surveys were carried out by pediatric physicians (FEAs), pediatric resident physicians (MIRes) and nurses (DUEs). All of them were workers in a pediatric department of a university hospital. Survey asked for price of different items used in daily practice: consultation/hospitalization, diagnostic laboratory test, imaging test, microbiological test, techniques, materials used for techniques and treatments. Responses were analyzed having in count reference values established in our hospital. Results: We analyzed 97 surveys. Global price survey was overvalued by 5%, although the 56% of the respondents underestimated global price. Grouped into six groups of items, drugs and consultations/hospitalizations were underestimated, while the rest of the items were overestimated. Drugs price were underestimated by 10%, and all different forms of consultation/hospitalization by 25%. Other four groups were overestimated: blood test in 589%, material used for the techniques in 128%, microbiological test in 81% and imaging tests in 57%. In overall analysis of survey, comparisons of groups showed no statistically significant differences for total survey by gender and by group of age. However, statistically differences were found in type of professional we analyzed (ANOVA p=0,03). FEAs and MIRes underestimated the survey while DUEs overestimated it. Conclusions: There is great ignorance on economic value of healthcare costs. There is, as well, great variability in their appreciation among the staff who works in the public health system

Empirical evidence of the validity of the Spanish version of the pain vigilance awareness questionnaire.

BACKGROUND: The Spanish version of the Pain Vigilance and Awareness Questionnaire has not been validated. PURPOSE: The aims of this study were to examine the factor structure of the Spanish version of the Pain Vigilance and Awareness Questionnaire and present empirical evidence regarding its validity. METHOD: A sample of 468 chronic back pain patients completed a battery of instruments to assess fear-avoidance beliefs, pain anxiety, pain catastrophizing, pain vigilance and awareness, pain acceptance, depression, anxiety, disability, and pain intensity. RESULTS: Confirmatory factor analysis supported the validity of a nine-item version with two subscales: Active Vigilance and Passive Awareness. Both subscales and the total score were positively and significantly correlated with other fear-related constructs: fear-avoidance beliefs, pain anxiety, and pain catastrophizing. Regression analyses showed that Active Vigilance and the two subscales of the Fear-Avoidance Beliefs Questionnaire were significantly associated with higher anxiety and that the Acceptance Activity Engagement subscale was significantly associated with lower anxiety. The Fear-Avoidance Beliefs Questionnaire-Physical subscale was associated with higher disability and the Acceptance Pain Willingness subscale was associated with lower disability. The Fear-Avoidance Beliefs Questionnaire-Work subscale was significantly associated with higher pain intensity and depression; the Acceptance Activity Engagement and Pain Willingness subscales were significantly associated with lower pain intensity and depression. CONCLUSION: The Spanish version of the Pain Vigilance and Awareness Questionnaire is a reliable and valid instrument. Pain Acceptance and Fear Avoidance beliefs are better predictors of adjustment to pain than pain hypervigilance.

Experiential avoidance and anxiety sensitivity as dispositional variables and their relationship to the adjustment to chronic pain.

Anxiety sensitivity has been included in the fear-avoidance model as a vulnerability factor to explain individual differences in fear of pain. Several studies have suggested that the relationship between anxiety sensitivity and some psychopathological disorders is mediated by experiential avoidance, an affect-related regulatory process that involves unwillingness to endure private experiences. The role of these constructs as vulnerability variables has not been investigated in chronic pain patients. The aim of this study was to investigate the role of anxiety sensitivity and experiential avoidance as dispositional variables in pain fear-avoidance. Two alternative hypothetical models were tested: one in which anxiety sensitivity and experiential avoidance would be independently associated with pain fear-avoidance; and second, one in which experiential avoidance would mediate the relationship between anxiety sensitivity and pain fear-avoidance. The sample was composed of 299 patients with chronic back pain. The postulated relationships were tested using LISREL 8.20 software (Scientific Software International, Chicago, IL, USA) and the generally weighted least squares. The structural equation modelling analyses showed that experiential avoidance and anxiety sensitivity were independently associated with pain fear-avoidance and that anxiety sensitivity had a stronger association with pain fear-avoidance than experiential avoidance. The alternative model, in which experiential avoidance mediates the relationship between anxiety sensitivity and pain fear-avoidance, gave a much worse fit. These results highlight the importance of both anxiety sensitivity and experiential avoidance as variables which could explain individual differences in pain fear-avoidance. Thus, in terms of prevention, it should be a priority to identify patients with increased anxiety sensitivity and experiential avoidance during the first stages of the development of chronic pain conditions.

Síndrome de Stevens-Johnson con afectación mucosapredominante / Stevens-Johnson's Syndrome with predominant mucous membrane involvement

Introducción. El síndrome de Stevens-Johnson es una reacción idiosincrásica mucocutánea secundaria mayoritariamente a fármacos y a ciertos agentes infecciosos. En algunas ocasiones, como los dos casos que aquí se presentan ,cursa con mínima afectación cutánea, predominando el compromiso mucoso en forma de ampollas, erosiones y costras. Caso clínico 1. Niño de 8 años con lesiones aftosas, vesículo-ampollosas y costrosas en mucosa oral, labial y endonasal; eritema violáceo periocular bilateral, hiperemia conjuntival y queratitis. Asocia fiebre los dos días previos y lesiones costrosas en la base del pene y margen anal. Había presentado un cuadro similar un mes antes, teniendo en común ambos episodios sendos tratamientos con cotrimoxazol e ibuprofeno. Caso clínico 2. Niño de 9 años con aftas en mucosa oral, labios con fisuras y costras; conjuntivitis, una lesión vesiculosa en pene y dos pequeñas lesiones vesiculosas en ambos brazos. Además, presentaba fiebre y lesiones compatibles con reactivación herpética en mejilla. Había recibido sucesivamente tratamientos con azitromicina y amoxicilina-clavulánico por infecciones respiratorias. Los estudios complementarios mostraron un ligero aumento de la proteína C reactiva en ambos casos y una inmunoglobulina M positiva para Mycoplasma pneumoniae en el caso 2. La evolución ha sido satisfactoria en ambos casos trastratamiento con fluido terapia intravenosa, colutorios y antibióticos tópicos. Conclusiones. El síndrome de Stevens-Johnson es un cuadro a tener en cuenta en el diagnóstico diferencial de las afecciones vesículo-ampollosas de las mucosas oral, conjuntival, anal y genital, que puede cursar sin la afectación cutánea característica (AU)

Introduction. Stevens-Johnson syndrome is an idiosyncratic mucocutaneous reaction caused mainly due to drugs and certain infectious agents. Sometimes, as the two cases described here, it is presented by minimal cutaneous manifestations mainly mucosal involvement, in the form of blisters, erosions and crusts. Clinical case 1. Eight years male having aphthous, vesiculobullous and crusted lesions in lips, oral and endonasal mucosa, bilateral periorbital violaceous erythema, conjunctival hyperemia and keratitis. Two days before the lesions described above, he associates fever and crusted lesions on the penis and anal margin. A similar picture was presented a month earlier, when he took cotrimoxazoleand ibuprofen, as well as he does in this episode. Case report 2. Nine years male with thrush in the oralmucosa, cracked and crusty lips, conjunctivitis, a vesicularlesion on the penis and two small vesicular lesions on botharms. He has also fever and an herpes reactivation lesions in cheek. He had been on treatment with azithromycin andamoxicillin-clavulanate for respiratory infections. Further studies showed a slight increase in C-reactive protein in both cases and an immunoglobulin M positive for Mycoplasma pneumoniae in case 2. Evolution has been satisfactory in both cases after treatment with intravenous fluids, rinses, and topical antibiotics. Conclusions. Stevens-Johnson syndrome is a condition to be considered in the differential diagnosis of vesiculobullous diseases of the oral, anal, genital and conjuntival mucosa that may occur without its characteristic skin involvement (AU)

Bioengineering RNA silencing across the life kingdoms.

RNA silencing negatively regulates gene expression at transcriptional and posttranscriptional levels, guided by small RNA molecules. It modulates core regulatory pathways across the eukaryotes, such as developmental processes or stress responses. The widespread existence of this phenomenon and the key pathways regulated have led to the development of a new technology based on the modification of gene expression, which has been applied successfully in different areas such as medicine or agriculture. Here we review the most important patents related to RNA silencing across the life kingdoms, including biotechnological applications into medicine, crop science and bioengineering.

[Cap myopathy: a case report]. / Miopatía en gorra: a propósito de un caso.

AIM: To report a new case of a little-known congenital myopathy. Cap myopathy is a rare congenital disease caused by an alteration in the structure of the fibre, with disorganised myofibrils at the edges. Since it was first described, only a few cases have been reported in the literature. CASE REPORT: We describe the case of a 16-year-old patient with a history of neonatal hypotonia and psychomotor retardation. At the age of 4 years, the patient presented myopathic facies with atrophied pectoral muscles, winged scapula and lumbar hyperlordosis. A myopathic pattern was observed in the electromyogram. A muscle biopsy showed a very marked predominance of type I fibres, atrophy in part of the population of this type and 20% of them had cap-shaped subsarcolemmal accumulations, which were intensely positive with DPNH and SDH; in the ultrastructural study they correspond to disorganised peripheral myofibrils with preservation of the Z band and the absence of A bands. These alterations are similar to those described in what is known as cap disease or cap myopathy. Today, the patient has mild proximal tetraparesis and moderate restrictive respiratory failure.

Miopatía en gorra: a propósito de un caso / Cap myopathy: a case report

Presentar un nuevo caso de una miopatía congénita poco conocida. La miopatía en gorra es una rara enfermedadcongénita, debida a una alteración en la estructura de la fibra, con las miofibrillas desorganizadas en la periferia. Desde su primera descripción, sólo se han publicado unos pocos casos. Caso clínico. Adolescente de 16 años con antecedentes de hipotonía neonatal y retraso psicomotor. A los 4 años presenta facies miopática con atrofia de pectorales, escápula aladae hiperlordosis lumbar. En el electromiograma se observa un patrón miopático. La biopsia muscular demuestra un predominio muy marcado de fibras tipo I, atrofia de parte de la población de este tipo y presencia en el 20% de ellas de acúmulos subsarcolemales en forma de casquete, intensamente positivos con DPNH y SDH; en el estudio ultraestructural correspondena miofibrillas periféricas desorganizadas con conservación de la banda Z y ausencia de bandas A. Estas alteraciones son similares a las descritas bajo el nombre de cap disease o miopatía ‘en gorra’. Actualmente el paciente presenta una tetraparesiaproximal leve y una insuficiencia respiratoria restrictiva moderada

To report a new case of a little-known congenital myopathy. Cap myopathy is a rare congenital diseasecaused by an alteration in the structure of the fibre, with disorganised myofibrils at the edges. Since it was first described, only a few cases have been reported in the literature. Case report. We describe the case of a 16-year-old patient with a history of neonatal hypotonia and psychomotor retardation. At the age of 4 years, the patient presented myopathic facies with atrophiedpectoral muscles, winged scapula and lumbar hyperlordosis. A myopathic pattern was observed in the electromyogram. A muscle biopsy showed a very marked predominance of type I fibres, atrophy in part of the population of this type and 20% of them had cap-shaped subsarcolemmal accumulations, which were intensely positive with DPNH and SDH; in the ultrastructuralstudy they correspond to disorganised peripheral myofibrils with preservation of the Z band and the absence of A bands. These alterations are similar to those described in what is known as cap disease or cap myopathy. Today, the patient has mild proximal tetraparesis and moderate restrictive respiratory failure

[Neurogenic stunned myocardium]. / Aturdimiento miocáirdico neurogénico.

The existence of stunned myocardium and reversible myocardial dysfunction is widely described and accepted in patients suffering ischemic heart disease. However, it cannot be exclusive to coronary disease. Classically, the appearance of electrocardiographic changes in the critical neurological disease has been described. However, at present, it seems to be observed that some of these patients with critical neurological disease could have variable grades of myocardial dysfunction, which is generally reversible in the surviving patients. This myocardial dysfunction, which could affect critically ill neurological patients, has traits similar to stunned myocardium generated in coronary patients since: a) it is generally associated to electrocardiographic changes, b) it can be accompanied by segmental contractility disorders and even c) it may be accompanied by a certain increase of cardiac biomarkers. Although its etiopathogeny is unknown, it could be related with the severity of the primary neurological disease. Its prophylaxis and prognosis are also unknown. It could be related with neurogenic edema, with hemodynamic instability, and could also play a very important role in brain death and in organ donation.