Clinical utility of investigations in triple-negative thrombocytosis: A real-world, multicentre evaluation of UK practice.

Diagnosis of essential thrombocythaemia (ET) is challenging in patients lacking JAK2/CALR/MPL mutations. In a retrospective evaluation of 320 patients with 'triple-negative thrombocytosis', we assessed utility of bone marrow histology (90.9% of patients) and myeloid gene panel (MGP, 55.6%). Supportive histology ('myeloproliferative neoplasm-definite/probable', 36.8%) was associated with higher platelet counts and varied between centres. 14.6% MGP revealed significant variants: 3.4% JAK2/CALR/MPL and 11.2% other myeloid genes. Final clinical diagnosis was strongly predicted by histology, not MGP. 23.7% received cytoreduction (17.6% under 60 years). Real-world 'triple-negative' ET diagnosis currently depends heavily on histology; we advocate caution in MGP-negative cases and that specific guidelines are needed.

Exploring the Molecular Aspects of Myeloproliferative Neoplasms Associated with Unusual Site Vein Thrombosis: Review of the Literature and Latest Insights.

Myeloproliferative neoplasms (MPNs) are the leading causes of unusual site thrombosis, affecting nearly 40% of individuals with conditions like Budd-Chiari syndrome or portal vein thrombosis. Diagnosing MPNs in these cases is challenging because common indicators, such as spleen enlargement and elevated blood cell counts, can be obscured by portal hypertension or bleeding issues. Recent advancements in diagnostic tools have enhanced the accuracy of MPN diagnosis and classification. While bone marrow biopsies remain significant diagnostic criteria, molecular markers now play a pivotal role in both diagnosis and prognosis assessment. Hence, it is essential to initiate the diagnostic process for splanchnic vein thrombosis with a JAK2 V617F mutation screening, but a comprehensive approach is necessary. A multidisciplinary strategy is vital to accurately determine the specific subtype of MPNs, recommend additional tests, and propose the most effective treatment plan. Establishing specialized care pathways for patients with splanchnic vein thrombosis and underlying MPNs is crucial to tailor management approaches that reduce the risk of hematological outcomes and hepatic complications.

The World Health Organization Intersectoral Global Action Plan on Epilepsy and Other Neurological Disorders and the headache revolution: from headache burden to a global action plan for headache disorders.

The World Health Organization (WHO) Intersectoral Global Action Plan on Epilepsy and Other Neurological Disorders was developed by WHO to address the worldwide challenges and gaps in provision of care and services for people with epilepsy and other neurological disorders and to ensure a comprehensive, coordinated response across sectors to the burden of neurologic diseases and to promote brain health across life-course. Headache disorders constitute the second most burdensome of all neurological diseases after stroke, but the first if young and midlife adults are taken into account. Despite the availability of a range of treatments, disability associated with headache disorders, and with migraine, remains very high. In addition, there are inequalities between high-income and low and middle income countries in access to medical care. In line with several brain health initiatives following the WHOiGAP resolution, herein we tailor the main pillars of the action plan to headache disorders: (1) raising policy prioritization and strengthen governance; (2) providing effective, timely and responsive diagnosis, treatment and care; (3) implementing strategies for promotion and prevention; (4) fostering research and innovation and strengthen information systems. Specific targets for future policy actions are proposed. The Global Action Plan triggered a revolution in neurology, not only by increasing public awareness of brain disorders and brain health but also by boosting the number of neurologists in training, raising research funding and making neurology a public health priority for policy makers. Reducing the burden of headache disorders will not only improve the quality of life and wellbeing of people with headache but also reduce the burden of neurological disorders increasing global brain health and, thus, global population health.

Health equity, care access and quality in headache - part 1.

Current definitions of migraine that are based mainly on clinical characteristics do not account for other patient's features such as those related to an impaired quality of life, due to loss of social life and productivity, and the differences related to the geographical distribution of the disease and cultural misconceptions which tend to underestimate migraine as a psychosocial rather than neurobiological disorder.Global differences definition, care access, and health equity for headache disorders, especially migraine are reported in this paper from a collaborative group of the editorial board members of the Journal of Headache and Pain. Other components that affect patients with migraine, in addition to the impact promoted by the migraine symptoms such as stigma and social determinants, are also reported.

Recomendaciones de “no hacer” en la consulta de Atención Primaria / Recommendations for “not to do” in primary care practice

Introducción: en los últimos años se ha observado un exceso de prácticas clínicas innecesarias en Pediatría que carecen de suficiente evidencia científica y generan costos significativos, además de resultar molestas para el paciente y no aportar beneficios adicionales. Por ello, diversas sociedades e instituciones científicas han propuesto recomendaciones para evitar estas intervenciones ineficaces y científicamente injustificadas. Material y métodos: el proyecto fue coordinado por el grupo de trabajo PrevInfad de la Asociación Española de Pediatría de Atención Primaria (AEPap). Se desarrolló en dos fases a través del intercambio de correos electrónicos. En la primera, se solicitó a los coordinadores que divulgaran el proyecto y recopilaran propuestas de intervenciones innecesarias. En la segunda, se realizó la selección de las recomendaciones mediante el método Delphi y dos rondas de votación. Resultados: en la primera fase se obtuvieron 40 propuestas de recomendaciones de “no hacer”. En la primera ronda de la segunda fase se aceptaron 7 propuestas. Cinco recomendaciones fueron rechazadas y 28 fueron consideradas dudosas y pasaron a una segunda valoración. En la segunda ronda se aceptó una recomendación adicional. En total, se aprobaron 8 recomendaciones de “no hacer”. Conclusiones: la AEPap elaboró una lista de recomendaciones de “no hacer” en Pediatría en colaboración con sus grupos de trabajo y asociaciones federadas utilizando la metodología Delphi para el consenso entre expertos. Estas recomendaciones buscan mejorar la calidad asistencial y promover la utilización adecuada de recursos en la Atención Primaria pediátrica (AU)

Introduction: in recent years, there has been an excess of unnecessary clinical practices in pediatrics that lack sufficient scientific evidence and generate significant costs, in addition to being bothersome and not providing additional benefits. For this reason, various scientific societies and institutions have proposed recommendations to avoid these ineffective and scientifically unjustified interventions.Material and methods: the project was coordinated by the PrevInfad working group of the Spanish Association of Primary Care Pediatrics (AEPap). It was developed in two phases through the exchange of e-mails. In the first phase, the coordinators were asked to disseminate the project and to collect proposals for unnecessary interventions. In the second phase, the recommendations were selected using the Delphi method and two rounds of voting.Results: in the first phase, 40 proposals for 'do not do' recommendations were obtained. In the first round of the second phase, seven proposals were accepted. Five recommendations were rejected and 28 were considered doubtful and passed to a second assessment. One additional recommendation was accepted in the second round. In total, 8 'do not do' recommendations were approved.Conclusions: the AEPap developed a list of 'do not do' recommendations in pediatrics in collaboration with its working groups and federated associations using the Delphi methodology for consensus among experts. These recommendations aim to improve the quality of care and promote the appropriate use of resources in pediatric primary care. (AU)

Lipidation and PEGylation Strategies to Prolong the in Vivo Half-Life of a Nanomolar EphA4 Receptor Antagonist.

The EphA4 receptor tyrosine kinase plays a role in neurodegenerative diseases, inhibition of nerve regeneration, cancer progression and other diseases. Therefore, EphA4 inhibition has potential therapeutic value. Selective EphA4 kinase inhibitors are not available, but we identified peptide antagonists that inhibit ephrin ligand binding to EphA4 with high specificity. One of these peptides is the cyclic APY-d3 (ßAPYCVYRßASWSC-NH2), which inhibits ephrin-A5 ligand binding to EphA4 with low nanomolar binding affinity and is highly protease resistant. Here we describe modifications of APY-d3 that yield two different key derivatives with greatly increased half-lives in the mouse circulation, the lipidated APY-d3-laur8 and the PEGylated APY-d3-PEG4. These two derivatives inhibit ligand induced EphA4 activation in cells with sub-micromolar potency. Since they retain high potency and specificity for EphA4, lipidated and PEGylated APY-d3 derivatives represent new tools for discriminating EphA4 activities in vivo and for preclinical testing of EphA4 inhibition in animal disease models.

Opciones terapéuticas para la retirada de “superpegamentos” de la piel / Therapeutic options for removing superglues from the skin

El cianocrilato es un compuesto adhesivo que se utiliza en la fabricación de “superpegamentos”. En contacto con tejidos de algodón o lana puede producir quemaduras térmicas. Por el contrario, en contacto sobre piel sana se han descrito dermatitis de contacto o alérgica pero no quemaduras. Caso clínico. Niña de 8 años que acude a urgencias tras haber derramado de manera accidental un “superpegamento” directamente sobre una mano y ambas extremidades inferiores, permaneciendo con el pegamento en contacto con la piel durante 3 horas sin poder retirarlo en domicilio. En la exploración destacaba gran eritema en varias regiones subyacentes al pegamento, sin ampollas, heridas ni sangrado. El pegamento se retiró bajo sedoanalgesia con lavavajillas líquido diluido en agua tibia, sin apreciarse solución de continuidad ni quemadura. Conclusiones. Existen numerosas medidas para la retirada de adhesivos de la piel. Escoger la opción terapéutica viene determinado, entre otros factores, por la presencia de lesiones cutáneas bajo el adhesivo. La aplicación de acetona está contraindicada en pieles con lesiones debido a que puede empeorar la irritación de las mismas. La demora hasta la retirada del adhesivo puede influir en el éxito del tratamiento; así como en la aparición de posibles secuelas (AU)

Cyanoacrylate is an adhesive used as a component in most of “super glues”. It can cause thermal burns if in contact with wool or cotton fabric. Nevertheless, in contact with intact skin it can cause contact or allergic dermatitis, but no thermal burns have been described.Clinical case: 8-year-old girl who had suffered an accidental fall of one of these glues directly on one of her hands and both lower extremities, and who had remained with the glue in contact with the skin for 3 hours. On examination we observed erythema on several regions underlying the glue, without blisters, wounds or bleeding. The glue was removed with warm water and detergent. No open wounds or burns were noticed.Conclusions: There are different ways to remove adhesives from the skin. Choosing the correct therapeutic option is determined, among other factors, by the presence of skin lesions under the adhesive. The use of acetone is contraindicated in injured skin because it can worsen the irritation. The delay in the removal of the adhesive can influence the success of the treatment and the type of treatment to be applied. (AU)

Terapias complementarias en migraña y variables relacionadas. Encuesta Europea / Complementary therapies in migraine and related variables. European Survey

Objetivo: valorar el uso de terapias alternativas no farmacológicas en migraña y variables relacionadas. La migraña afecta a una de cada diez personas en el mundo, con aumento creciente y mayor prevalencia entre mujeres de zonas urbanas. Aunque los tratamientos habituales son farmacológicos, se ha incrementado el uso de terapias alternativas no farmacológicas en migraña y variables relacionadas. Materiales y métodos: estudio observacional transversal mediante encuesta anónima vía web a 3.342 pacientes de países europeos sobre el uso de terapias alternativas para tratamiento de migraña. Variables de estudio: edad, género, país, tipo de localidad, nivel de estudios, ámbito rural o urbano y respuesta al tratamiento. Resultados: la utilización de terapias alternativas en migraña es un fenómeno creciente, con mayor uso en pacientes entre 41-60 años y estudios superiores. Conclusiones: en migraña se observan diferencias por edad, género, nivel cultural y país de procedencia. El uso de la medicina complementaria como alternativa terapéutica en la migraña es poco habitual y se debería indagar con mayor profundidad estableciendo relaciones que permitan apoyar al paciente afectado y priorizar las de mayor evidencia científica..(Au)

Objective: to evaluate the use of alternative non-pharmacological therapies in migraine and related variables. Migraine affects one in ten people worldwide, with a growing increase and a higher prevalence among women in urban areas. Although the usual treatments are pharmacological, the use of alternative techniques is increasing. Materials and methods: cross-sectional observational study by anonymous web survey of 3,342 patients from European countries on the use of alternative therapies. Study variables: age, gender, country, type of locality, educational level, rural or urban setting and treatment response. Results: the use of alternative therapies in migraine treatment is a growing phenomenon, with greater use in patients between 41-60 years of age and higher education. Conclusions: in Migraine, differences are observed by age, gender, cultural level and country of origin. The use of complementary medicine as an therapeutic alternative in migraine is uncommon and should be investigated in greater depth, establishing relationships that allow supporting the affected patient and prioritizing, if possible, some over others with scientific evidence..(Au)

Aspectos preventivos en migraña y trabajo. Encuesta europea / Preventive Aspects for Migraine and the workplace: A European Survey

INTRODUCCIÓN: La promoción de la salud en el trabajo integra las iniciativas en salud y seguridad en ámbito ocupacional, con mejoras personales, incremento de productividad y menores riesgos y gastos sociales, especialmente en migraña, como enfermedad neurológica con prevalencia estimada en el 11% de la población. El objetivo de este trabajo fue conocer las condiciones preventivas de los trabajadores con migraña y las opciones de gestión preventiva en sus empresas. MÉTODO: Estudio observacional transversal realizado en 3.342 pacientes de España, Italia, Francia, Portugal, Irlanda, Reino Unido, Alemania y otros países de la UE mediante encuesta anónima en la web de la European Migraine & Headache Alliance (EMHA-web), entre septiembre de 2018 y enero de 2019. RESULTADOS: Estrés laboral y uso de PVD son los riesgos más referidos por los trabajadores con migraña. El 43.71% de trabajadores desconoce el tipo de Servicio de Prevención de su empresa, 49.06% no dispone de servicio médico; el 67.67% no ha tenido impedimentos de acceso laboral por migraña, ni despido o no renovación del contrato, pero el 42,14% tuvo algún conflicto por pérdida de productividad; el 26,54% desconoce el concepto de especial sensibilidad o no lo ha solicitado por migraña; un 55,42% no se ha sentido comprendido ni apoyado por su empresa en sus limitaciones por migraña, pero sí por los compañeros. CONCLUSIÓN: Se observa una deficiente información preventiva y escaso uso de las opciones de gestión adaptativa en las empresas para personas con migraña

INTRODUCTION: Workplace health promotion integrates initiatives in health and safety in the occupational field, with personal improvements, increased productivity and lower risks and social cost, especially with respect to migraine headaches, a neurological disorder affecting approximately 11% of the population. The objective of this study was to know the preventive resources available to workers with migraine headaches and the preventive management options in their companies. METHOD: Cross-sectional observational study of 3,342 patients from Spain, Italy, France, Portugal, Ireland, United Kingdom, Germany and other European Union countries, conducted through an anonymous survey on the web of the European Migraine & Headache Alliance (EMHA-web), from September 2018 to January 2019. RESULTS: Occupational stress (77.65%) and use of computer monitors (63.87%) are the most common risks described by workers with migraine. About. 43.71% of workers are not familiar with the type of occupational health service present in their company, 49.06% do not have a medical service; 67.67% reported no work-related limitations due to migraine, neither dismissal nor non-renewal of their contract (88.29%), but 42.14% had experienced some conflict due to decreased productivity; 26.54% were unaware of the concept of vulnerable workers or had not requested this status because of their migraine (63.8%), nor had they demanded job accommodations (67.64%) or job change (80.89%); 55.42% did not feel understood or supported by their company in their limitations due to migraine, although they did feel they were supported by their colleagues (63.07%). CONCLUSION: We found that preventive resources and information were deficient, and that there was little use of adaptive management options for workers with migraine in their companies

Procedimiento de colocación del catéter de bomba de insulina / Procedure for placing an insulin pump catheter

La terapia con Infusión Subcutánea Continua de Insulina (ISCI) o bomba de insulina puede ser una buena alternativa terapéutica para las personas con diabetes tipo 1 en situaciones concretas. El aprendizaje de esta técnica ha de realizarse mediante un programa de educación sanitaria impartido por un equipo multidisciplinar, con el que se capacita al paciente en el manejo del dispostivo y en cuidado de la zona de la inserción de la cánula. Cumpliendo estas condiciones básicas, la persona puede obtener beneficios, tanto en el control glucémico como en la reducción de la incidencia de hipoglucemias y, por lo tanto, una mejoría en su calidad de vida.El objetivo de este artículo consiste en explicar las indicaciones, el equipo necesario y el procedimiento de autoaplicacion de la bomba de insulina,así como las posibles complicaciones asociadas, con el fin de garantizarlas máximas condiciones de seguridad y las mínimas incomodidades para la persona que sigue este tipo de tratamiento (AU)

Continuous subcutaneous insulin infusion (CSII) therapy or an insulinpump can be a good therapeutic alternative for people with type 1 diabetes in specific situations.The learning of this technique must be performed by means of a health education programme taught by a multidisciplinary team, in which thepatient is taught to manage the device and to care for the cannula’s insertion area. If the patient adheres to these basic conditions, he/she can reap the benefits, both in glycemic control and in the reduction of the incidence of hypoglycaemias, which in turn improves his or her quality of life.The objective of this article is to explain the indications, the equipment that is needed and the self-application procedure for the insulin pump, as well as possible complications, in order to guarantee the best safety conditions and to decrease the discomfort experienced by the patient who undergoes this type of treatment (AU)

Primer contacto con una bomba de insulina. Caso clínico / First contact with an insulin pump. Clinical case

Las bombas de insulina son unos dispositivos que infunden la insulina de forma similar a la secreción pancreática, continuamente y con infinidad de posibilidades para adaptarse a horarios, comidas y actividad física, otorgando a los diabéticos la posibilidad de llevar una vida con muchas menos ataduras debido a su enfermedad. Además de la libertad que les proporciona, esta terapia permite un control más exacto de las glucemias, mejorando notablemente el autocontrol general y con ello la calidad de vida de estas personas, reduciendo la aparición de complicaciones a largo plazo. Aunque todos los diabéticos reciben a lo largo de su enfermedad infinidad de pautas y recomendaciones para un óptimo control, los pacientes incluidos en este protocolo tienen que estar especialmente concienciados e implicados en su autocontrol, ya que el tratamiento les exige una gran implicación, tanto para aprender a utilizar el dispositivo como para tomar decisiones una vez se está utilizando. Se presenta el caso clínico de una paciente diabética tipo 1 de 21 años de evolución, durante un día de ingreso en el hospital para llevar a cabo la implantación de un infusor continuo de insulina subcutánea (bomba de insulina). Para ello, elaboramos el plan de cuidados de la paciente siguiendo los pasos del método científico y apoyándonos en la taxonomía NANDA, así como en la NOC y NIC para delimitar objetivos e intervenciones enfermeras, respectivamente (AU)

Insulin pumps are devices that inject insulin continuously into the body in a similarway to the pancreatic secretion, and with endless possibilities to adjust schedules, meals and physical activities, giving diabetics the chance to lead a life with many less restraints due totheir illness. Besides giving them this freedom, the therapy allows more precise control of blood glucose, significantly improving the overall self-control and thus the quality of life of the diabetics, reducing the appearance of long-term complications. Although all diabetics receive many guidelinesand recommendations for optimal control throughout their illness patients included in this protocolmust be especially aware and involved in self-control, since the treatment demands more involvement both when learning to use the device as well as making decisions once they are using it. We present the case of a type 1 diabetic patient, twenty-one years of onset, who while admitted to hospital for a day, had a continuous subcutaneous insulin infusion pump implanted (insulin pump). To this end, we developed a patient care plan of patient following the steps of scientific method and relying on the NANDA taxonomy and on the NOC and NIC to design goals and nursing interventions, respectively (AU)

Evaluación de aspectos bioéticos en los profesionales de la salud / Evaluation of bioethical aspects in health professionals

Objetivo. Evaluar el nivel de formación, conocimientos y percepción acerca de diversos aspectos bioéticos entre los profesionales de la salud. Método. Estudio descriptivo, trasversal, mediante cuestionario validado, realizado en el año 2005 en el Hospital Universitario Marqués de Valdecilla. La población de estudio fueron los profesionales del equipo de salud que trabajaban en el área de cardiología de dicho hospital. Resultados. Del total de sujetos de estudio (186 profesionales), se recogieron 117 encuestas, lo que significa el 63,5% de la población a estudio, 58 enfermeras (49,5%), 23 médicos (19,6%) y 34 auxiliares (28,2%). El 64,6% carece de formación en bioética, y los de una antigüedad profesional superior a 20 años son los más formados en dicha disciplina (p = 0,019). El 45,7% conoce la existencia (p = 0,004) y contenido (p = 0,03) de la Ley 41/2002. El 12,8% cree que los usuarios conocen sus derechos, y los que no tienen formación en bioética son los que mayoritariamente creen que se respeta siempre o habitualmente la confidencialidad (p = 0,002) y la intimidad (p = 0,039) del usuario. El 37,8% refiere enfrentarse en su quehacer profesional con problemas bioéticos. Conclusión. Los miembros del equipo de salud presentan un déficit de conocimientos paralelo a unos niveles de escasa formación en bioética y una percepción de insuficiente respeto a los derechos básicos del usuario. Se hace necesario potenciar la formación, así como vías ágiles de información en las instituciones que permitan una real adaptación a los requerimientos éticos y legales en cada momento

Objective. To evaluate training in bioethics, as well as knowledge and perceptions of bioethical issues, among health professionals. Method. A descriptive cross-sectional study was performed in 2005 by means of a validated questionnaire. The study population consisted of the health team working in the cardiology area in the Marqués de Valdecilla University Hospital. Results. There were 186 health professionals, and 117 questionnaires were returned, representing 63.5% of the study population: 58 nurses (49.5%), 23 physicians (19.6%), and 34 nursing auxiliaries. More than half (64.6%) had no training in bioethics. The health professionals with the most training in bioethics were those with more than 20 years' experience (p = 0.019). Less than half (45.7%) knew of the existence (p = 0.004) or the content (p = 0.03) of law 41/2002. A total of 12.8% believed that users knew their rights. Health professionals with no bioethics training were those most likely to believe that users' confidentiality (p = 0.002) and privacy (p = 0.039) were always or usually respected. More than one third (37.8%) reported facing bioethical problems in their professional activity. Conclusion. The members of the health team have insufficient knowledge of bioethics parallel to low levels of training in this discipline and perceive insufficient respect for users' basic rights. Training in bioethics should be increased, and information pathways should be established in institutions to allow continual adaptation to ethical and legal requirements