RESUMEN
La dermatomiositis es una miopatía inflamatoria con una incidencia de 0.5-1 caso por 100.000 habitantes. Se caracteriza por lesiones cutáneas típicas y debilidad muscular, aunque ocasionalmente podemos encontrar formas amiopáticas. La asociación con malignidad ha sido ampliamente descrita en la literatura, habiéndose detectado tumores hasta en un tercio de los casos. La presencia del anticuerpo anti-TIF-1-gamma presenta tasas más altas de neoplasia comparada con otras formas sin este anticuerpo, lo que obliga a realizar un despistaje tumoral. A continuación, presentamos 2 casos de dermatomiositis asociada a anticuerpo TIF-1-gamma asociadas a malignidad. (AU)
Dermatomyositis is an inflammatory myopathy with an incidence of 0.5-1 case per 100,000 inhabitants. It is characterized by hallmark cutaneus findings and muscle weakness, although we can occasionally find amyopathic forms. The association with malignancy has been widely described in the literature, as tumors have been detected in up to a third of the cases. The presence of the anti-TIF-1-gamma antibody presents higher rates of neoplasia compared to other forms without this antibody, so it is mandatory to do a tumor screening. Next, we present 2 cases of TIF-1-gamma antibody-associated dermatomyositis associated with malignancy. (AU)
Asunto(s)
Humanos , Enfermedades Musculares , Síndromes Paraneoplásicos , Dermatomiositis/diagnóstico , Dermatomiositis/terapiaRESUMEN
INTRODUCCIÓN Y OBJETIVOS: El embarazo en cicatriz de cesárea previa (ECC) es una entidad poco frecuente que puede tener graves consecuencias. Hasta la fecha no existen esquemas estandarizados de tratamiento y su manejo óptimo sigue siendo controvertido. Nuestro objetivo es realizar una revisión de la literatura publicada sobre el manejo del ECC y proponer un algoritmo. También exponemos tres casos de ECC resueltos con diferentes tratamientos en el Hospital Universitario Infanta Elena MÉTODOS: Búsqueda de la literatura en bases de datos utilizando las palabras clave: "embarazo en cicatriz cesárea"," gestación ectópica en cicatriz cesárea", "tratamiento", "manejo". RESULTADOS: Las opciones terapéuticas pueden ser médicas, quirúrgicas o una combinación de ambas. Los tratamientos quirúrgicos tienen altas tasas de éxito, sin embargo, son más invasivos y no están exentos de riesgo. La combinación de tratamientos parece aumentar la tasa de éxito, no obstante, podría implicar un mayor riesgo de efectos secundarios y costes. CONCLUSIONES: El manejo de los ECC debe de ser individualizado, basado en la evidencia científica, en los medios disponibles y la experiencia de los profesionales en los distintos procedimientos, guiándonos por el tipo de ECC y su grado de vascularización e invasión, grosor del miometrio, niveles de beta-hCG, presencia de actividad cardiaca, clínica y estabilidad hemodinámica de la paciente. Deben tenerse en cuenta las circunstancias y patología intercurrente de la mujer, así como su deseo genésico o de preservación del útero.
INTRODUCTION AND OBJECTIVES: Cesarean scar pregnancy (CSP) is a rare entity that can cause serious consequences. Up to now, there are no standardized treatment schemes, and its optimal management remains controversial. Our objetive is to review the literature regarding CSP management and propose an algorithm. We also present three cases of CSP resolved with different treatments at Hospital Universitario Infanta Elena. METHODS: Literature search in databases using the following keywords: pregnancy with cesarean section, ectopic pregnancy with cesarean section, treatment, management. RESULTS: The therapeutic options can be medical, surgical or a combination of both. Surgical treatments have high success rates; however, they are more invasive and are not without risk. The combination of treatments seems to increase the success rate; however, it could imply a higher risk of side effects and costs. CONCLUSIONS: The management of CSP must be individualized; based on scientific evidence, on the means available, and on the experience of the professionals in the different procedures; guided by the type of CSP and its degree of vascularization and invasion, by the thickness of the myometrium, beta-hCG levels, presence of cardiac activity, and by clinical and hemodynamic stability of the patient. The circumstances and intercurrent pathology of the patient must be considered, as well as her desire for future pregnancy or preservation of the uterus.
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Embarazo Ectópico/terapia , Cesárea/efectos adversos , Cicatriz/etiología , Cicatriz/terapia , Embarazo Ectópico/cirugía , Embarazo Ectópico/tratamiento farmacológico , Metotrexato/uso terapéutico , Cicatriz/cirugía , Cicatriz/tratamiento farmacológico , Embolización de la Arteria Uterina , Ultrasonido Enfocado de Alta Intensidad de Ablación , HisterectomíaRESUMEN
OBJETIVO: Reportar el caso de una torsión de útero, trompas y ambos ovarios a nivel de cuello uterino. Describir la patogenia, sintomatología y tratamiento, como un caso infrecuente y potencialmente grave, en la práctica clínica diaria. MATERIAL Y MÉTODOS: Analizar el curso de esta extremadamente rara patología, en una mujer postmenopáusica de 59 años. Se trata de un caso raro de torsión uterina en un útero no gestante, reportando los hallazgos en los exámenes previos a la cirugía y los hallazgos intraoperatorios e histopatológicos postcirugía. La resonancia magnética fue fundamental en el diagnóstico de la paciente y la laparotomía realizada demostró una torsión de cuerpo uterino de 360 ° a nivel de cérvix, y un gran mioma pediculado con signos de necrosis. Hemos revisado los últimos artículos de esta rara patología RESULTADOS Y DISCUSIÓN: La paciente fue operada. Se realizó una histerectomía total mediante una laparotomía media supra e infraumbilical. Los ovarios no tenían signos de necrosis. CONCLUSIONES: Debemos tener en cuenta esta posibilidad diagnóstica, en pacientes con aumento de tamaño uterino y clínica de dolor abdominal, ya que aunque es muy infrecuente, su diagnóstico es importante, ya que puede ser peligroso para la vida de la paciente.
OBJECTIVE: To report a case of torsion of the uterus, fallopian tubes and both ovaries around the uterine collum and the symptomatology, pathogenesis and treatment of this uncommon pathology. MATERIAL AND METHODS: The article analyses the course of this extremely rare pathology in a postmenopausical woman of 59 years. It is a rare case like a non gravid uterine torsion in a postmenopausical woman. We have the exams previous to the surgery, the finds intraoperative and the histolopathological reports postsurgery. RMN was the most important in diagnosis and the laparotomy showed a 360° an uterus increased on size and a big pedunculated myoma with signs of necrosis We have reviewed the latest articles on this strange pathology RESULTS AND DISCUSSION: The patient was operated. A infra-supra medium laparotomy was practiced and a total hysterectomy was made. Ovarians had not signs of necrosis. CONCLUSIONS: We must take into account, in patients with pathologies that increase uterine size and abdominal pain, the possibility of uterine torsion. Although it is a very infrequent pathology, its diagnosis is important, since it can be dangerous for the life of the patient
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Anomalía Torsional/etiología , Neoplasias Uterinas/complicaciones , Mioma/complicaciones , Anomalía Torsional/cirugía , Anomalía Torsional/diagnóstico por imagen , Neoplasias Uterinas/cirugía , Neoplasias Uterinas/diagnóstico , Imagen por Resonancia Magnética , Histerectomía , Mioma/cirugía , Mioma/diagnósticoRESUMEN
Introducción: Las disecciones de arteriales cervicales (DAC) provocan hasta el 20% de los ictus isquémicos en menores de 45 años. El beneficio de los tratamientos de reperfusión en fase aguda no está plenamente clarificado. Métodos: Revisión retrospectiva de pacientes con DAC ingresados en un centro terciario de ictus desde 2010 hasta 2015. Recogemos las características basales, clínicas, los tratamientos, el pronóstico funcional y la mortalidad. Resultados: Se registraron 35 DAC (23 carotídeas/12 vertebrales). La edad media fue de 43,5 ± 9,5 años y el 67,7% fueron varones. En 10 casos (32,3%) hubo antecedente de un traumatismo. Los factores de riesgo más frecuentes fueron la hipertensión arterial (29%) y el tabaquismo (35,5%). La presentación clínica más frecuente fue el infarto cerebral en 29 pacientes (93,5%). La mediana de puntuación National Institute of Health Stroke Scale basal fue de 6 (0-41). El método diagnóstico más empleado fue la angio-TC (74,2%), seguido de resonancia magnética (64,5%) y arteriografía cerebral (45,6%). Siete pacientes (22,6%) fueron tratados con fibrinólisis intravenosa y 11 (35,5%) con tratamiento endovascular (TEV) ± fibrinólisis intravenosa. A los 3 meses, la independencia funcional (escala de Rankin 0-2) fue del 57,1% y del 63,6%, respectivamente. Falleció un paciente (3,2%). Conclusiones: La forma de presentación más frecuente de la DAC fue el infarto cerebral. Estos casos pueden beneficiarse de terapias de reperfusión, con un pronóstico similar al resto de enfermos con ictus isquémicos. Se requieren registros más extensos para conocer mejor la respuesta a los tratamientos de reperfusión en fase aguda en este grupo de pacientes
Introduction: Cervical artery dissection (CAD) is responsible for up to 20% of all ischaemic strokes in patients younger than 45. The benefits of acute-phase reperfusion therapy in these patients have yet to be confirmed. Methods: We conducted a retrospective review of patients with CAD admitted to a comprehensive stroke centre between 2010 and 2015. We recorded baseline clinical characteristics, treatments, functional outcomes, and mortality. Results: We identified 35 cases of CAD (23 carotid/12 vertebral); mean age was 43.5 ± 9.5 years and 67.7% were men. Ten patients (32.3%) had a history of trauma. The most frequent risk factors were arterial hypertension (29%) and smoking (35.5%). The most common clinical presentation was ischaemic stroke (29 patients, 93.5%). The median baseline National Institute of Health Stroke Scale score was 6 (range, 0-41). The most frequently used diagnostic method was CT angiography (74.2%), followed by MRI (64.5%) and digital subtraction angiography (45.6%). Seven patients (22.6%) were treated with intravenous fibrinolysis and 11 (35.5%) with endovascular treatment plus intravenous fibrinolysis; at 3 months, functional independence (modified Rankin Scale scores 0-2) was achieved by 57.1% and 63.6% of these cases, respectively. One patient died (3.2%). Conclusions: In our sample, the most common form of presentation of CAD was ischaemic stroke. Reperfusion therapy seems to be a safe and effective option for these patients, and outcomes resemble those of other patients with ischaemic stroke. Larger comparative studies are necessary to better assess response to reperfusion therapy in acute ischaemic stroke
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Infarto Cerebral/terapia , Reperfusión/métodos , Accidente Cerebrovascular/terapia , Disección de la Arteria Carótida Interna/terapia , Isquemia Encefálica/terapia , Estudios Retrospectivos , Disección de la Arteria Vertebral/terapia , Procedimientos Endovasculares/métodos , Terapia Trombolítica/métodosRESUMEN
INTRODUCTION: Cervical artery dissection (CAD) is responsible for up to 20% of all ischaemic strokes in patients younger than 45. The benefits of acute-phase reperfusion therapy in these patients have yet to be confirmed. METHODS: We conducted a retrospective review of patients with CAD admitted to a comprehensive stroke centre between 2010 and 2015. We recorded baseline clinical characteristics, treatments, functional outcomes, and mortality. RESULTS: We identified 35 cases of CAD (23 carotid/12 vertebral); mean age was 43.5 ± 9.5 years and 67.7% were men. Ten patients (32.3%) had a history of trauma. The most frequent risk factors were arterial hypertension (29%) and smoking (35.5%). The most common clinical presentation was ischaemic stroke (29 patients, 93.5%). The median baseline National Institute of Health Stroke Scale score was 6 (range, 0-41). The most frequently used diagnostic method was CT angiography (74.2%), followed by MRI (64.5%) and digital subtraction angiography (45.6%). Seven patients (22.6%) were treated with intravenous fibrinolysis and 11 (35.5%) with endovascular treatment plus intravenous fibrinolysis; at 3 months, functional independence (modified Rankin Scale scores 0-2) was achieved by 57.1% and 63.6% of these cases, respectively. One patient died (3.2%). CONCLUSIONS: In our sample, the most common form of presentation of CAD was ischaemic stroke. Reperfusion therapy seems to be a safe and effective option for these patients, and outcomes resemble those of other patients with ischaemic stroke. Larger comparative studies are necessary to better assess response to reperfusion therapy in acute ischaemic stroke.
Asunto(s)
Disección de la Arteria Carótida Interna/complicaciones , Fibrinolíticos/uso terapéutico , Reperfusión/métodos , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/etiología , Enfermedad Aguda , Adulto , Angiografía/métodos , Procedimientos Endovasculares/métodos , Femenino , Humanos , Masculino , Estudios Retrospectivos , Factores de Riesgo , España , Accidente Cerebrovascular/diagnóstico por imagen , Resultado del TratamientoRESUMEN
INTRODUCCIÓN: En España existe actualmente escasa información sobre el manejo de los pacientes con psoriasis en la práctica clínica diaria de los dermatólogos. OBJETIVO: El objetivo de esta encuesta de opinión fue recoger información de los dermatólogos españoles expertos en el manejo de los pacientes con psoriasis sobre los protocolos que realizan en su práctica clínica habitual. MATERIAL Y MÉTODOS: Encuesta de opinión realizada mediante cuestionario on line remitido a 75 dermatólogos expertos en el manejo de la psoriasis. El cuestionario, diseñado específicamente para la encuesta de opinión, incluía 12 preguntas sobre diferentes aspectos de la práctica clínica en el tratamiento de la psoriasis moderada-grave. RESULTADOS: La tasa de respuesta fue del 96% (n = 72). Los biológicos fueron la opción más usada como monoterapia. El 64,3% de los encuestados señaló que sus pacientes permanecen 1-2 años con terapias sistémicas clásicas antes de la transición a biológicos, y el principal determinante para decidir la transición fue el control inestable de la actividad de la enfermedad. El 85,7% dio importancia «alta» o «muy alta» a considerar una puntuación PASI < 3 como objetivo terapéutico. Los fármacos de elección más consensuados fueron etanercept en población pediátrica (78,6%), adalimumab y etanercept en artritis psoriásica (64,3%) y ustekinumab en pacientes con frecuentes ausencias domiciliarias (78,6%), baja adherencia (71,4%) e historia de esclerosis múltiple o enfermedades desmielinizantes (64,3%). CONCLUSIÓN: Esta encuesta de opinión proporciona una perspectiva única sobre las opiniones de una muestra representativa de los dermatólogos expertos en cuanto al tratamiento actual de la psoriasis con fármacos biológicos en España
BACKGROUND: There is currently little information available on the management of patients with psoriasis in the daily clinical practice of dermatologists in Spain. OBJECTIVE: The aim of this study was to survey a group of Spanish dermatologists with particular expertise in the management of psoriasis to determine their opinions on the protocols used in routine clinical practice. MATERIAL AND METHODS: A cross-sectional study based on an online survey about the management of psoriasis sent to 75 dermatologists. The survey, which was specifically designed for the study, included 12 questions on different aspects of clinical practice in the treatment of moderate to severe psoriasis. RESULTS: The response rate was 96% (n = 72). Biologics were the most widely used monotherapy option. In total, 64.3% of respondents reported that their patients used conventional systemic therapies for 1 to 2 years before switching to a biologic drug and that the main reason for the switch was unstable control of disease activity. Overall, 85.7% assigned a "high" or "very high" importance to the use of a Psoriasis Area Severity Index score of < 3 as a treatment goal. The drugs of choice among the respondents were etanercept for pediatric patients (78.6%), adalimumab and etanercept for patients with psoriatic arthritis (64.3%), and ustekinumab in patients frequently away from home (78.6%) and patients with a history of multiple sclerosis, demyelinating diseases (64.3%), or poor adherence to treatment (71.4%). CONCLUSION: This study provides a unique overview of the opinions of a representative sample of expert dermatologists on the current use of biologics for the treatment of psoriasis in Spain
Asunto(s)
Humanos , Masculino , Femenino , Niño , Adulto , Psoriasis/tratamiento farmacológico , Psoriasis/epidemiología , Pautas de la Práctica en Medicina/estadística & datos numéricos , Antiinflamatorios/uso terapéutico , Productos Biológicos/uso terapéutico , Estudios Transversales , España/epidemiología , Índice de Severidad de la Enfermedad , Selección de Paciente , Inmunosupresores/uso terapéuticoRESUMEN
BACKGROUND: There is currently little information available on the management of patients with psoriasis in the daily clinical practice of dermatologists in Spain. OBJECTIVE: The aim of this study was to survey a group of Spanish dermatologists with particular expertise in the management of psoriasis to determine their opinions on the protocols used in routine clinical practice. MATERIAL AND METHODS: A cross-sectional study based on an online survey about the management of psoriasis sent to 75 dermatologists. The survey, which was specifically designed for the study, included 12questions on different aspects of clinical practice in the treatment of moderate to severe psoriasis. RESULTS: The response rate was 96% (n=72). Biologics were the most widely used monotherapy option. In total, 64.3% of respondents reported that their patients used conventional systemic therapies for 1 to 2years before switching to a biologic drug and that the main reason for the switch was unstable control of disease activity. Overall, 85.7% assigned a "high" or "very high" importance to the use of a Psoriasis Area Severity Index score of <3 as a treatment goal. The drugs of choice among the respondents were etanercept for pediatric patients (78.6%), adalimumab and etanercept for patients with psoriatic arthritis (64.3%), and ustekinumab in patients frequently away from home (78.6%) and patients with a history of multiple sclerosis, demyelinating diseases (64.3%), or poor adherence to treatment (71.4%). CONCLUSION: This study provides a unique overview of the opinions of a representative sample of expert dermatologists on the current use of biologics for the treatment of psoriasis in Spain.
Asunto(s)
Pautas de la Práctica en Medicina/estadística & datos numéricos , Psoriasis/tratamiento farmacológico , Adulto , Antiinflamatorios/uso terapéutico , Productos Biológicos/uso terapéutico , Niño , Contraindicaciones de los Medicamentos , Estudios Transversales , Manejo de la Enfermedad , Sustitución de Medicamentos , Femenino , Encuestas de Atención de la Salud , Humanos , Inmunosupresores/uso terapéutico , Masculino , Selección de Paciente , Psoriasis/epidemiología , Índice de Severidad de la Enfermedad , España/epidemiologíaRESUMEN
Introducción: La problemática de los trastornos del movimiento (TM) es compleja y la duración y frecuencia de las consultas presenciales puede estar limitada por problemas de espacio y tiempo. Analizamos el funcionamiento de un servicio de atención por correo electrónico institucional para médicos de Atención Primaria (MAP) y pacientes en la Unidad de Trastornos del Movimiento (UTM). Métodos: Se revisaron retrospectivamente los correos electrónicos enviados y recibidos en un periodo de 4 meses, un año tras su implantación. La dirección se proporcionaba en consulta y mediante sesiones informativas a los MAP del área. Se analizaron datos clínicos y demográficos de los pacientes, tipo de interlocutor, número de consultas, motivo y actuaciones derivadas de ellas. Resultados: Del 1 de enero al 30 de abril de 2015 se recibieron 137 correos de 63 pacientes (43% varones; edad 71 ± 10,5 años) diagnosticados de enfermedad de Parkinson (76%), parkinsonismos atípicos (10%) y otros (14%), y se enviaron 116 respuestas. En 20 casos (32%) fueron redactados por el paciente, en 38 (60%) por sus familiares y en 5 (8%) por MAP. Los motivos de consulta fueron clínicos en 50 casos (80%): deterioro clínico (16; 32%), nuevos síntomas (14; 28%), efectos secundarios o dudas sobre medicación (20; 40%). Como consecuencia, se adelantó una cita programada en 9 casos (14%), mientras que el resto se solucionaron por correo electrónico. En 13 (20%), el motivo de consulta fue burocrático: relacionado con citas (11, 85%) y solicitud de informe (2, 15%). La satisfacción fue generalizada, sin constituir una sobrecarga asistencial excesiva para los facultativos responsables. Conclusiones: La implantación de una consulta por correo electrónico es factible en UTM, facilita la comunicación médico-paciente y la continuidad asistencial con Atención Primaria (AU)
Introduction: The clinical problems of patients with movement disorders (MD) are complex, and the duration and frequency of face-to-face consultations may be insufficient to meet their needs. We analysed the implementation of an e-mail-based query service for our MD unit's patients and their primary care physicians (PCPs). Methods: We retrospectively reviewed all consecutive emails sent and received over a period of 4 months, one year after implementation of the e-mail inquiry system. All patients received the during consultations, and PCPs, during scheduled informative meetings. We recorded and later analysed the profile of the questioner, patients' demographic and clinical data, number of queries, reason for consultation, and actions taken. Results: From 1 January 2015 to 30 April 2015, the service received 137 emails from 63 patients (43% male, mean age 71 ± 10.5) diagnosed with Parkinson's disease (76%), atypical parkinsonism (10%), and others (14%); 116 responses were sent. Twenty (32%) emails were written by patients, 38 (60%) by their caregivers, and 5 (8%) by their PCPs. The reasons for consultation were clinical in 50 cases (80%): 16 (32%) described clinical deterioration, 14 (28%) onset of new symptoms, and 20 (40%) side effects or concerns about medications. In 13 cases (20%), the query was bureaucratic: 11 were related to appointments (85%) and 2 were requests for clinical reports (15%). In response, new appointments were scheduled in 9 cases (14%), while the rest of the questions were answered by email. Patients were satisfied overall and the additional care burden on specialists was not excessive. Conclusions: Implementing an e-mail-based consultation system is feasible in MD units. It facilitates both communication between neurologists and patients and continued care in the primary care setting (AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Enfermedad de Parkinson/epidemiología , Correo Electrónico , Atención Primaria de Salud , Consulta Remota , Correo Electrónico/tendencias , Estudios Retrospectivos , Telemedicina/métodosRESUMEN
The gastrointestinal (GI) tract plays a central role in the absorption, distribution, metabolism, and excretion of flavonoids, which ultimately define the health effects of these bioactives. These aspects are modulated by the interactions of flavonoids with other dietary components, environmental factors, the host, and the GI microbiota. Flavonoid can target molecules in the luminal content, the different GI tract cell types, and the microbiota. Importantly, flavonoid actions at the GI tract can have an impact systemically, e.g. on glucose homeostasis, lipid and energy metabolism, or cardiovascular risk factors. The beneficial actions of flavonoids at the GI include their capacity to: i) protect the intestinal epithelium against pharmacological insults and food toxins; ii) modulate the activity of enzymes involved in lipid and carbohydrate absorption; iii) maintain the intestinal barrier integrity; iv) modulate the secretion of gut hormones; v) modulate the GI tract immune system; vi) exert potential anti-colorectal cancer activity; and vii) shape microbiota composition and function. Further understanding of the mechanisms mediating the effects of flavonoids on the intestine (and its microbiota) is of critical importance given the relevance of the GI tract on sustaining overall health and of the widespread recommendations of increasing the intake of plant bioactives.
Asunto(s)
Flavonoides/metabolismo , Tracto Gastrointestinal/metabolismo , Enfermedades Gastrointestinales/metabolismo , Enfermedades Gastrointestinales/microbiología , Enfermedades Gastrointestinales/patología , Microbioma Gastrointestinal , Tracto Gastrointestinal/microbiología , Tracto Gastrointestinal/patología , Salud , HumanosRESUMEN
INTRODUCTION: The clinical problems of patients with movement disorders (MD) are complex, and the duration and frequency of face-to-face consultations may be insufficient to meet their needs. We analysed the implementation of an e-mail-based query service for our MD unit's patients and their primary care physicians (PCPs). METHODS: We retrospectively reviewed all consecutive emails sent and received over a period of 4 months, one year after implementation of the e-mail inquiry system. All patients received the during consultations, and PCPs, during scheduled informative meetings. We recorded and later analysed the profile of the questioner, patients' demographic and clinical data, number of queries, reason for consultation, and actions taken. RESULTS: From 1 January 2015 to 30 April 2015, the service received 137 emails from 63 patients (43% male, mean age 71±10.5) diagnosed with Parkinson's disease (76%), atypical parkinsonism (10%), and others (14%); 116 responses were sent. Twenty (32%) emails were written by patients, 38 (60%) by their caregivers, and 5 (8%) by their PCPs. The reasons for consultation were clinical in 50 cases (80%): 16 (32%) described clinical deterioration, 14 (28%) onset of new symptoms, and 20 (40%) side effects or concerns about medications. In 13 cases (20%), the query was bureaucratic: 11 were related to appointments (85%) and 2 were requests for clinical reports (15%). In response, new appointments were scheduled in 9 cases (14%), while the rest of the questions were answered by email. Patients were satisfied overall and the additional care burden on specialists was not excessive. CONCLUSIONS: Implementing an e-mail-based consultation system is feasible in MD units. It facilitates both communication between neurologists and patients and continued care in the primary care setting.
Asunto(s)
Comunicación , Correo Electrónico/estadística & datos numéricos , Enfermedad de Parkinson/complicaciones , Médicos de Atención Primaria , Derivación y Consulta/estadística & datos numéricos , Especialización , Anciano , Femenino , Humanos , Masculino , Estudios RetrospectivosRESUMEN
Gaucher Disease type 1 (GD1) is a lysosomal disorder that affects many systems. Therapy improves the principal manifestations of the condition and, as a consequence, many patients show a modified phenotype which reflects manifestations of their disease that are refractory to treatment. More generally, it is increasingly recognised that information as to how a patient feels and functions [obtained by patient- reported outcome measurements (PROMs)] is critical to any comprehensive evaluation of treatment. A new set of management goals for GD1 in which both trends are reflected is needed. To this end, a modified Delphi procedure among 25 experts was performed. Based on a literature review and with input from patients, 65 potential goals were formulated as statements. Consensus was considered to be reached when ≥75% of the participants agreed to include that specific statement in the management goals. There was agreement on 42 statements. In addition to the traditional goals concerning haematological, visceral and bone manifestations, improvement in quality of life, fatigue and social participation, as well as early detection of long-term complications or associated diseases were included. When applying this set of goals in medical practice, the clinical status of the individual patient should be taken into account.
Asunto(s)
Enfermedad de Gaucher/complicaciones , Enfermedad de Gaucher/terapia , Calidad de Vida , Consenso , Manejo de la Enfermedad , Europa (Continente)/epidemiología , Enfermedad de Gaucher/epidemiología , Enfermedad de Gaucher/psicología , HumanosRESUMEN
No disponible
Asunto(s)
Humanos , Masculino , Adulto , Enfermedades de la Piel/genética , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/etiología , Cuero Cabelludo/anomalías , Cuero Cabelludo/patología , Cuero Cabelludo/fisiopatología , Anamnesis/métodos , Examen Físico/instrumentación , Examen Físico/métodos , Examen Físico , Tomografía Computarizada por Rayos X/instrumentación , Tomografía Computarizada por Rayos X/métodos , Tomografía Computarizada por Rayos X , Radiografía/instrumentación , Radiografía/métodos , RadiografíaRESUMEN
INTRODUCCIÓN: La enseñanza de la bioética se ha incorporado a los planes de estudio de medicina y al programa para médicos residentes. Sin embargo, la transmisión de conocimientos basados en la práctica clínica habitualmente no se realiza de una manera bien estructurada. OBJETIVO: Valorar los conocimientos de ética de los residentes de pediatría españoles y analizar su relación con la formación recibida durante la licenciatura y el periodo de residencia. MATERIAL Y MÉTODOS: Se diseñó una encuesta con 20 preguntas tipo test destinada a evaluar conocimientos éticos básicos con repercusión sobre la clínica. Se valoraron la formación recibida durante el pregrado y la residencia, y los principales conflictos éticos afrontados. RESULTADOS: Se obtuvieron 210 encuestas remitidas desde 20 hospitales: 47 correspondientes a (R1), 49 a (R2), 57 a (R3) y 57 a (R4). La media de respuestas correctas fue de 16,8. No hubo diferencias entre los residentes de distinto año ni entre los que manifestaron haber recibido o no formación específica. Se contabilizaron más fallos en preguntas relacionadas con el consentimiento informado, ley de autonomía del paciente, los principios implicados en la calidad de vida, la sistemática del análisis de casos y la dimensión de la justicia distributiva. CONCLUSIONES: La limitación del esfuerzo terapéutico se ha identificado como el principal problema ético en la práctica clínica. Gran parte de los conocimientos sobre ética se adquieren en el pregrado y varían poco durante la residencia, lo que hace necesarios mayores esfuerzos organizativos y docentes durante este periodo
INTRODUCTION: Bioethics has been recently incorporated in to the educational programs of both medical students and medical residents as part of their curriculum. However, its training based on clinical practice is not well structured. OBJECTIVE: To evaluate the knowledge of bioethics in Spanish paediatric residents, and to analyse how this relates to the medical education during graduate and post-graduate training. MATERIAL AND METHODS: A questionnaire with 20 multiple choice questions was designed to evaluate the knowledge in basic ethics with potential implications in clinical practice. We evaluated the education received during graduate and post-graduate training, and the main ethical conflicts faced. RESULTS: A total of 210 completed questionnaires were received from medical residents in paediatrics from 20 different Spanish hospitals, of whom 47 of these were first year residents (R1), 49 were second year residents (R2), 57 were third year residents (R3), and the remaining 57 were final year residents (R4). The mean number of correct answers was 16.8 out of 20. No differences were found between residents in different years of training, nor were there any differences between the group that had received specific training in bioethics versus those who had not. Residents were more likely to give wrong answers related with informed consent, the law on the freedom of the patient, principles of quality of life, the case analysis system, and the dimension of distributive justice. CONCLUSIONS: Limitation of therapeutic efforts was identified as the main ethical problem faced in clinical practice by Spanish residents in paediatrics. Most of the knowledge of bioethics is acquired during graduate training, and improved very little throughout the period of medical residence. Our results suggest that efforts are required in organising and structuring the education in bioethics during the training of residents in paediatrics
Asunto(s)
Humanos , Pediatría/ética , Especialización/tendencias , Educación Médica/tendencias , Bioética/educación , Conocimientos, Actitudes y Práctica en Salud , Evaluación EducacionalRESUMEN
INTRODUCTION: Bioethics has been recently incorporated in to the educational programs of both medical students and medical residents as part of their curriculum. However, its training based on clinical practice is not well structured. OBJECTIVE: To evaluate the knowledge of bioethics in Spanish paediatric residents, and to analyse how this relates to the medical education during graduate and post-graduate training. MATERIAL AND METHODS: A questionnaire with 20 multiple choice questions was designed to evaluate the knowledge in basic ethics with potential implications in clinical practice. We evaluated the education received during graduate and post-graduate training, and the main ethical conflicts faced. RESULTS: A total of 210 completed questionnaires were received from medical residents in paediatrics from 20 different Spanish hospitals, of whom 47 of these were first year residents (R1), 49 were second year residents (R2), 57 were third year residents (R3), and the remaining 57 were final year residents (R4). The mean number of correct answers was 16.8 out of 20. No differences were found between residents in different years of training, nor were there any differences between the group that had received specific training in bioethics versus those who had not. Residents were more likely to give wrong answers related with informed consent, the law on the freedom of the patient, principles of quality of life, the case analysis system, and the dimension of distributive justice. CONCLUSIONS: Limitation of therapeutic efforts was identified as the main ethical problem faced in clinical practice by Spanish residents in paediatrics. Most of the knowledge of bioethics is acquired during graduate training, and improved very little throughout the period of medical residence. Our results suggest that efforts are required in organising and structuring the education in bioethics during the training of residents in paediatrics.
Asunto(s)
Bioética/educación , Internado y Residencia , Pediatría/educación , Encuestas y CuestionariosRESUMEN
The N-acylhydrazone (NAH) analogues N-methyl 2-thienylidene 3,4-benzoylhydrazine (LASSBio-785) and N-benzyl 2-thienylidene 3,4-benzoylhydrazine (LASSBio-786) were prepared from 2-thienylidene 3,4-methylenedioxybenzoylhydrazine (LASSBio-294). The ability of LASSBio-785 and LASSBio-786 to decrease central nervous system activity was investigated in male Swiss mice. LASSBio-785 or LASSBio-786 (30â mg/kg, ip) reduced locomotor activity from 209 ± 26 (control) to 140 ± 18 (P < 0.05) or 146 ± 15 crossings/min (P < 0.05), respectively. LASSBio-785 (15 or 30â mg/kg, iv) also reduced locomotor activity from 200 ± 15 to 116 ± 29 (P < 0.05) or 60 ± 16 crossings/min (P < 0.01), respectively. Likewise, LASSBio-786 (15 or 30â mg/kg, iv) reduced locomotor activity from 200 ± 15 to 127 ± 10 (P < 0.01) or 96 ± 14 crossings/min (P < 0.01), respectively. Pretreatment with flumazenil (20â mg/kg, ip) prevented the locomotor impairment induced by NAH analogues (15â mg/kg, iv), providing evidence that the benzodiazepine (BDZ) receptor is involved. This finding was supported by the structural similarity of NAH analogues to midazolam. However, LASSBio-785 showed weak binding to the BDZ receptor. LASSBio-785 or LASSBio-786 (30â mg/kg, ip, n = 10) increased pentobarbital-induced sleeping time from 42 ± 5 (DMSO) to 66 ± 6 (P < 0.05) or 75 ± 4â min (P < 0.05), respectively. The dose required to achieve 50% hypnosis (HD50) following iv injection of LASSBio-785 or LASSBio-786 was 15.8 or 9.5â mg/kg, respectively. These data suggest that both NAH analogues might be useful for the development of new neuroactive drugs for the treatment of insomnia or for use in conjunction with general anesthesia.
Asunto(s)
Hidrazinas/farmacología , Hidrazonas/farmacología , Hipnóticos y Sedantes/farmacología , Actividad Motora/efectos de los fármacos , Receptores de GABA/efectos de los fármacos , Tiofenos/farmacología , Animales , Hidrazinas/química , Hidrazonas/química , Masculino , Ratones , Receptores de GABA/fisiología , Tiofenos/químicaRESUMEN
The N-acylhydrazone (NAH) analogues N-methyl 2-thienylidene 3,4-benzoylhydrazine (LASSBio-785) and N-benzyl 2-thienylidene 3,4-benzoylhydrazine (LASSBio-786) were prepared from 2-thienylidene 3,4-methylenedioxybenzoylhydrazine (LASSBio-294). The ability of LASSBio-785 and LASSBio-786 to decrease central nervous system activity was investigated in male Swiss mice. LASSBio-785 or LASSBio-786 (30 mg/kg, ip) reduced locomotor activity from 209 ± 26 (control) to 140 ± 18 (P < 0.05) or 146 ± 15 crossings/min (P < 0.05), respectively. LASSBio-785 (15 or 30 mg/kg, iv) also reduced locomotor activity from 200 ± 15 to 116 ± 29 (P < 0.05) or 60 ± 16 crossings/min (P < 0.01), respectively. Likewise, LASSBio-786 (15 or 30 mg/kg, iv) reduced locomotor activity from 200 ± 15 to 127 ± 10 (P < 0.01) or 96 ± 14 crossings/min (P < 0.01), respectively. Pretreatment with flumazenil (20 mg/kg, ip) prevented the locomotor impairment induced by NAH analogues (15 mg/kg, iv), providing evidence that the benzodiazepine (BDZ) receptor is involved. This finding was supported by the structural similarity of NAH analogues to midazolam. However, LASSBio-785 showed weak binding to the BDZ receptor. LASSBio-785 or LASSBio-786 (30 mg/kg, ip, n = 10) increased pentobarbital-induced sleeping time from 42 ± 5 (DMSO) to 66 ± 6 (P < 0.05) or 75 ± 4 min (P < 0.05), respectively. The dose required to achieve 50% hypnosis (HD50) following iv injection of LASSBio-785 or LASSBio-786 was 15.8 or 9.5 mg/kg, respectively. These data suggest that both NAH analogues might be useful for the development of new neuroactive drugs for the treatment of insomnia or for use in conjunction with general anesthesia.
Asunto(s)
Animales , Masculino , Ratones , Hidrazinas/farmacología , Hidrazonas/farmacología , Hipnóticos y Sedantes/farmacología , Actividad Motora/efectos de los fármacos , Receptores de GABA/efectos de los fármacos , Tiofenos/farmacología , Hidrazinas/química , Hidrazonas/química , Receptores de GABA/fisiología , Tiofenos/químicaRESUMEN
No disponible
Asunto(s)
Humanos , Niño , Unidades de Cuidado Intensivo Pediátrico/normas , Humanización de la Atención , Cuidados Críticos/tendencias , Calidad de la Atención de Salud/tendencias , Relaciones Profesional-Familia , Relaciones Profesional-Paciente , Atención Integral de Salud , Chaperones Médicos/normasRESUMEN
Introducción: Existe una creciente preocupación por las complicaciones neurológicas asociadas a las cardiopatías congénitas y a la cirugía cardiaca infantil. Material y métodos: Estudio observacional retrospectivo de casos y controles de las cirugías cardiacas infantiles y su postoperatorio en cuidados intensivos durante un período de 10 años. Se seleccionaron dos controles por cada caso, ajustados por el mismo grado de complejidad quirúrgica. Resultados: Se estudiaron 900 cirugías. Se detectaron 38 complicaciones neurológicas (4,2%), de las cuales 21 (55,3%) implicaban al sistema nervioso periférico y 17 (44,7%) al sistema nervioso central. Las complicaciones del sistema nervioso central (1,9% del total) fueron 8 convulsiones, 4 accidentes cerebrovasculares, 4 encefalopatías hipóxico-isquémicas y un déficit neurológico reversible. Un 35,3% mostró afectación al alta y un 17,6% falleció. Se encontró una diferencia estadísticamente significativa en el tiempo de circulación extracorpórea (p=0,009), el tiempo de isquemia (p=0,12), los días de estancia en cuidados intensivos (p=0,001), días de ventilación mecánica (p=0,004) y días de soporte inotrópico (p=0,001). Conclusiones: La incidencia de complicaciones neurológicas en nuestra serie es similar a la descrita previamente. Las convulsiones son la manifestación clínica más común. Las complicaciones del sistema nervioso central se asocian con un aumento de la morbilidad, una mayor estancia hospitalaria y un mayor consumo de recursos. Es necesario establecer medidas en el pre y post-operatorio, así como durante la cirugía, encaminadas a su prevención y diagnóstico precoz(AU)
Introduction: There has been an increasing concern over the neurological complications associated with congenital heart disease and cardiac surgery. Material and methods: We performed a retrospective, case-control, observational review of the postoperative period in the intensive care unit of patients undergoing cardiac surgery over the past 10 years. We selected 2 control patients for each case, matched for surgical complexity. Results: A total of 900 patients were reviewed. We found 38 neurological complications (4.2%), of which 21 (55.3%) were in the peripheral nervous system and 17 (44.7%) in the central nervous system. The complications involving the central nervous system (1.9% of total) consisted of 8 seizures, 4 cerebrovascular accidents, 4 hypoxic-ischemic encephalopathy events, and 1 reversible neurological deficit. At the time of discharge, 35.3% were symptomatic and 17.6% had died. Patients with neurological complications had a longer bypass time (P=.009), longer aortic cross time (P=.012), longer hospitalization in intensive care (P=.001), longer duration of mechanical ventilation (P=.004) and an increased number of days under inotropic support (P=.001). Conclusions: Our incidence of neurological complications after cardiac surgery is similar to that previously described. Clinical seizures are the most common complication. Central nervous system complications are associated with a higher morbidity and hospitalization time. Units caring for patients with congenital heart disease must implement neurological monitoring during and after cardiac surgery to prevent and to detect these complications earlier(AU)
Asunto(s)
Humanos , Masculino , Femenino , Niño , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/terapia , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Cardiopatías/complicaciones , Cardiopatías/cirugía , Sistema Nervioso/patología , Cardiopatías Congénitas/etiología , Cardiopatías Congénitas/fisiopatología , Estudios Retrospectivos , Modelos Logísticos , Oportunidad RelativaRESUMEN
INTRODUCTION: There has been an increasing concern over the neurological complications associated with congenital heart disease and cardiac surgery. MATERIAL AND METHODS: We performed a retrospective, case-control, observational review of the postoperative period in the intensive care unit of patients undergoing cardiac surgery over the past 10 years. We selected 2 control patients for each case, matched for surgical complexity. RESULTS: A total of 900 patients were reviewed. We found 38 neurological complications (4.2%), of which 21 (55.3%) were in the peripheral nervous system and 17 (44.7%) in the central nervous system. The complications involving the central nervous system (1.9% of total) consisted of 8 seizures, 4 cerebrovascular accidents, 4 hypoxic-ischemic encephalopathy events, and 1 reversible neurological deficit. At the time of discharge, 35.3% were symptomatic and 17.6% had died. Patients with neurological complications had a longer bypass time (P=.009), longer aortic cross time (P=.012), longer hospitalization in intensive care (P=.001), longer duration of mechanical ventilation (P=.004) and an increased number of days under inotropic support (P=.001). CONCLUSIONS: Our incidence of neurological complications after cardiac surgery is similar to that previously described. Clinical seizures are the most common complication. Central nervous system complications are associated with a higher morbidity and hospitalization time. Units caring for patients with congenital heart disease must implement neurological monitoring during and after cardiac surgery to prevent and to detect these complications earlier.