Green hospital pharmacy: A sustainable approach to the medication use process in a tertiary hospital. / [Artículo traducido] Farmacia hospitalaria ecológica: un enfoque sostenible del proceso de utilización de medicamentos en un hospital de tercer nivel.

BACKGROUND: Sustainable management of healthcare waste has a positive impact on the global environment. In order to reduce it, the sustainable practice of the pharmacotherapeutic process in all its stages is essential. OBJECTIVE: To analyse the sustainability strategies proposed by the pharmacy service to reduce drug waste derived from the pharmacotherapeutic process. SECONDARY OBJECTIVES: To analyse the stage of the pharmacotherapeutic process and the number and type of drugs involved. METHODS: The study was carried out in a tertiary-level hospital. To coordinate the proposals, a referent pharmacist from every pharmacy department area was selected. Four stages of the process were evaluated (management, validation, dispensing and compounding), patients concerned were classified as outpatients or inpatients, and drugs potentially involved were analysed by the administration route: Into oral or parenteral. RESULTS: Twenty eight ideas were proposed, which could affect more than 1200 drugs. 39.3% would affect the validation process, 17.9% the procurement management, 17.9% dispensing, and 7.1% the compounding. Implementation feasibility and acceptability of these proposals were evaluated. Those with the greatest potential were: Limiting the duration of treatments when possible, favouring the implementation of computer prescription order entry, favouring the use of the oral route over the parenteral route, and implementing computers in the preparation areas to avoid the use of paper guides. DISCUSION: In our study, many ideas have been proposed by hospital pharmacists to improve the sustainability of the medication use process. When assessing these proposals by impact and feasibility, according to our results, shorten as much as possible the duration of treatments, computerization of the medication use process, and oral administration over intravenous should be prioritized in order to reduce environmental impact.

Telepharmacy scorecard: Activity and quality indicators for the pharmaceutical care in a hospital pharmacy service.

OBJECTIVE: Telepharmacy, as a complementary activity to face-to-face pharmaceutical care in a Hospital pharmacy service, must have specific activity, effectiveness and quality indicators. The objectives of the  project were to design a scorecard of activity, effectiveness and quality  indicators that will make it possible to assess the situation and progress of Telepharmacy and enable continuous improvement. A tool is also provided to measure the indicators, and some recommendations are given for its  implementation. METHOD: The project, led by a panel of expert pharmacists, was developed in  2021 in four phases: a literature review, preliminary identification of quality  criteria and indicators, evaluation of indicators, adjustment of the proposal and definition of priority indicators, and drafting of descriptive files, as well as the  development and validation of a scorecard. The indicators were prioritized  based on their appropriateness, usefulness, relevance and feasibility. Finally,  the scorecard was submitted for evaluation by the members and Patient  Committee of the Spanish Hospital Pharmacy Society. RESULTS: The resulting scorecard consists of 50 indicators grouped into five  areas: General aspects (18); pharmacotherapeutic follow-up (12); home drug  delivery (15); patient information and education (2); and coordination with the  care team (3). A total of 31 were considered priority or essential  indicators, which are initially recommended for the implementation of a  Telepharmacy program. In contrast, 15 are general indicators, including  measurement of patient and professional satisfaction; 6 indicators refer to  pharmacotherapeutic follow-up; 1 is related to patient information and  education, and 2 correspond to care team coordination. CONCLUSIONS: The scorecard developed is a management tool for the implementation and evaluation of Telepharmacy in the Hospital pharmacy service. This tool enables assessing the initial situation, monitoring  implementation progress, measuring quality and performance, facilitating  decision-making and establishing an improvement plan.

OBJETIVO: La Telefarmacia, como actividad complementaria de la atención farmacéutica presencial en un servicio de farmacia de hospital, debe disponer de indicadores de calidad, actividad y efectividad específicos. Los objetivos del proyecto fueron definir los indicadores de calidad, actividad y efectividad de un cuadro de mando que permitan conocer la  situación y evolución de la Telefarmacia y ayuden a la toma de decisiones de mejora continua, además de diseñar una herramienta que permita medir los indicadores y establecer recomendaciones para su implantación.Método: El proyecto liderado por un grupo de expertos farmacéuticos se desarrolló durante el año 2021 en cuatro fases: revisión bibliográfica,  elaboración preliminar de criterios de calidad e indicadores, valoración de los indicadores y definición de indicadores prioritarios, la elaboración de fichas descriptivas, y el desarrollo y validación de una herramienta de cuadro de mando. Los indicadores se priorizaron en función de su adecuación, utilidad, pertinencia y factibilidad. Finalmente, el cuadro de mando fue sometido a la evaluación de los socios y del Comité de Pacientes de la Sociedad Española de Farmacia Hospitalaria. RESULTADOS: El cuadro de mando consta de 50 indicadores agrupados en cinco ámbitos: 18 de ellos sobre aspectos generales, 12 en el ámbito del  seguimiento farmacoterapéutico, 15 relacionados con la dispensación y entrega informada de medicamentos a distancia, 2 sobre formación e información a los  pacientes y 3 en relación con la coordinación con el equipo asistencial. Se  consideraron 31 de ellos prioritarios, siendo los recomendados inicialmente en  la implantación de un programa de Telefarmacia. De ellos, 15 son generales  (incluyendo la medida de satisfacción del paciente y el profesional), 6 son  indicadores de seguimiento, 1 de formación e información y 2 de coordinación  asistencial. CONCLUSIONES: El cuadro de mando desarrollado es una herramienta de  gestión para implantar y evaluar la Telefarmacia en los servicios de farmacia  hospitalaria, que permite conocer la situación inicial, monitorizar la  implantación, medir la calidad y el desempeño, facilitar la toma de decisiones y  establecer un plan de mejora.

Cuadro de mando de Telefarmacia: Indicadores deactividad y calidad de la atención farmacéuticade un servicio de farmacia de hospital / Telepharmacy scorecard: Activity and quality indicators for the pharmaceutical care in a hospital pharmacy service

Objetivo: La Telefarmacia, como actividad complementaria de la atención farmacéutica presencial en un servicio de farmacia de hospital, debedisponer de indicadores de calidad, actividad y efectividad específicos.Los objetivos del proyecto fueron definir los indicadores de calidad, actividad y efectividad de un cuadro de mando que permitan conocer la situación y evolución de la Telefarmacia y ayuden a la toma de decisiones demejora continua, además de diseñar una herramienta que permita medirlos indicadores y establecer recomendaciones para su implantación.Método: El proyecto liderado por un grupo de expertos farmacéuticos sedesarrolló durante el año 2021 en cuatro fases: revisión bibliográfica, elaboración preliminar de criterios de calidad e indicadores, valoración de losindicadores y definición de indicadores prioritarios, la elaboración de fichasdescriptivas, y el desarrollo y validación de una herramienta de cuadrode mando. Los indicadores se priorizaron en función de su adecuación,utilidad, pertinencia y factibilidad. Finalmente, el cuadro de mando fuesometido a la evaluación de los socios y del Comité de Pacientes de laSociedad Española de Farmacia Hospitalaria. Resultados: El cuadro de mando consta de 50 indicadores agrupadosen cinco ámbitos: 18 de ellos sobre aspectos generales, 12 en el ámbitodel seguimiento farmacoterapéutico, 15 relacionados con la dispensacióny entrega informada de medicamentos a distancia, 2 sobre formación einformación a los pacientes y 3 en relación con la coordinación con elequipo asistencial. Se consideraron 31 de ellos prioritarios, siendo losrecomendados inicialmente en la implantación de un programa de Telefarmacia. De ellos, 15 son generales (incluyendo la medida de satisfaccióndel paciente y el profesional), 6 son indicadores de seguimiento, 1 deformación e información y 2 de coordinación asistencial. (AU)

Objective: Telepharmacy, as a complementary activity to face-to-facepharmaceutical care in a Hospital pharmacy service, must have specificactivity, effectiveness and quality indicators. The objectives of the projectwere to design a scorecard of activity, effectiveness and quality indicators that will make it possible to assess the situation and progress ofTelepharmacy and enable continuous improvement. A tool is also provided to measure the indicators, and some recommendations are given forits implementation.Method: The project, led by a panel of expert pharmacists, was developed in 2021 in four phases: a literature review, preliminary identificationof quality criteria and indicators, evaluation of indicators, adjustment ofthe proposal and definition of priority indicators, and drafting of descriptive files, as well as the development and validation of a scorecard. Theindicators were prioritized based on their appropriateness, usefulness,relevance and feasibility. Finally, the scorecard was submitted for evaluation by the members and Patient Committee of the Spanish HospitalPharmacy Society Results: The resulting scorecard consists of 50 indicators grouped intofive areas: General aspects (18); pharmacotherapeutic follow-up (12);home drug delivery (15); patient information and education (2); and coordination with the care team (3). A total of 31 were considered priorityor essential indicators, which are initially recommended for the implementation of a Telepharmacy program. In contrast, 15 are general indicators, including measurement of patient and professional satisfaction;6 indicators refer to pharmacotherapeutic follow-up; 1 is related to patientinformation and education, and 2 correspond to care team coordination. (AU)

Factors associated with poor long-term adherence after completing a pulmonary rehabilitation programme in patients with chronic obstructive pulmonary disease.

BACKGROUND: Although pulmonary rehabilitation programmes (PRPs) benefit patients with chronic obstructive pulmonary disease (COPD), poor adherence to these programmes is common. OBJECTIVE: This study aimed to analyse the factors associated with poor long-term adherence after completing a PRP. METHOD: We conducted a retrospective study of 70 patients with COPD who performed an 8-week outpatient PRP that included 24 sessions of aerobic training, skeletal muscle resistance exercises, physiotherapy and COPD education. The study classified the patients into 2 groups: (1) long-term adherence and (2) long-term non-adherence to the PRP. We considered long-term non-adherence when the patient did not attend the 32 weeks follow-up visit after beginning the PRP. We measured the degree of dyspnoea, quality of life, physical activity, anxiety-depression status, submaximal exercise capacity and COPD exacerbations in both groups. RESULTS: The patients' median age was 69.6 [63.8-75.0] years, and 71.4% were men. The median forced expiratory volume in 1 second was 60.0 [47.7-68.0] % of that predicted. We observed total COPD exacerbations and severe COPD exacerbations in the last year in 32 (45.7%) and 22 (31.4%) patients, respectively. Dyspnoea, physical activity and quality of life significantly improved after completing the PRP. Long-term non-adherence to the PRP was observed in 32 (45.7%) patients. In the single regression model, severe COPD exacerbations (p = 0.04) and dyspnoea (p = 0.03) were associated with long-term non-adherence to the PRP. In the multiple regression model, only severe COPD exacerbations remained as an associated factor (OR 2.7; 95% CI 1.1-8,1; p = 0.04). CONCLUSION: Approximately half of patients who complete PRPs present long-term non-adherence to the programme. Severe COPD exacerbations are associated with long-term non-adherence.

Encuesta a profesionales sanitarios y pacientes sobre el papel del farmacéutico en la Unidad de Enfermedades Inflamatorias Inmunomediadas / The role of the pharmacist within an immune-mediated inflammatory disease unit: Results of a survey of healthcare providers and patients

Objetivo: Evaluar el interés y necesidad de que el farmacéutico desarrolle nuevas actividades propuestas, y potenciar o mantener otras que yase realizaban, antes de que la futura Unidad de Enfermedades Inflamatorias Inmunomediadas inicie su actividad en nuestro hospital. Además,priorizar la incorporación de las nuevas actividades en base a los resultados obtenidos.Método: Diseño observacional transversal unicéntrico mediante unaencuesta realizada en enero de 2020 a todos los profesionales sanitarios de los servicios clínicos implicados y a una muestra de pacientes, yestructurada en dos categorías: Acciones orientadas a la atención farmacéutica al paciente y Acciones orientadas a los profesionales de dichaUnidad. Cada ítem se puntuó de 0 a 10, siendo 10 el máximo interés/necesidad. Se aplicó una matriz de priorización para cuantificar y evaluar cada actividad e implantar las nuevas por orden de priorización.Resultados: Se completaron 90 encuestas (30 de pacientes y 60 deprofesionales). Se analizaron las medianas obtenidas de cada una de las20 actividades propuestas, alcanzándose valores entre 8 y 10. Se compararon valores: en el grupo de farmacéuticos versus médicos se obtuvieron más ítems con diferencias estadísticamente significativas que en elgrupo farmacéuticos versus enfermería, o farmacéuticos versus pacientes. (AU)

Objective: To evaluate the importance and need for pharmacists toexpand their role to new activities and to promote and maintain othersthey already carried out prior to the implementation of a new Immunemediated Inflammatory Diseases Unit to be created in our hospital; toprioritize the new activities incorporated based on the results obtained.Method: This was a single center cross-sectional based on a survey administered during January 2020 to all clinical healthcare providers due to bepart of the new unit, as well as to a sample of patients. It was structuredinto two categories: actions related to patients’ pharmaceutical care, andactions related to practitioners of the Immune-mediated Inflammatory Diseases Unit. Each item was assigned a score from 0 to 10, where 10 indicatedmaximum interest or need. A prioritization template was applied to quantifyand evaluate each activity and implement the new ones in order of priority.Results: A total of 90 responses were obtained (30 from patients and60 from healthcare workers). An analysis was performed of the medianscores of each of the 20 activities proposed, which ranged between 8 and10 points. When comparing the scores obtained, it was observed thatmore statistically significant differences were obtained in the pharmacists vsdoctors group than in the pharmacists vs nurses group, or the pharmacistsvs patients one. After prioritization, the first action taken was to implement electronic prescriptions for outpatients with immune-mediated inflammatorydiseases. (AU)

The role of the pharmacist within an immune-mediated inflammatory disease unit: Results of a survey of healthcare providers and patients.

OBJECTIVE: To evaluate the importance and need for pharmacists to expand  their role to new activities and to promote and maintain others they already  carried out prior to the implementation of a new Immunemediated Inflammatory Diseases Unit to be created in our hospital; to prioritize the new activities incorporated based on the results obtained. METHOD: This was a single center cross-sectional based on a survey  administered during January 2020 to all clinical healthcare providers due to be part of the new unit, as well as to a sample of patients. It was structured into two categories: actions related to patients' pharmaceutical  care, and actions related to practitioners of the Immune-mediated  Inflammatory Diseases Unit. Each item was assigned a score from 0 to 10,  where 10 indicated maximum interest or need. A prioritization template was  applied to quantify and evaluate each activity and implement the new ones in  order of priority. RESULTS: A total of 90 responses were obtained (30 from patients and 60 from  healthcare workers). An analysis was performed of the median scores of each  of the 20 activities proposed, which ranged between 8 and 10 points. When  comparing the scores obtained, it was observed that more statistically  significant differences were obtained in the pharmacists vs doctors group than  in the pharmacists vs nurses group, or the pharmacists vs patients one. After  prioritization, the first action taken was to implement electronic prescriptions  for outpatients with immune-mediated inflammatory diseases. CONCLUSIONS: The survey revealed the expectations of healthcare providers and patients regarding the role pharmacists should play in the newly created unit and provided an insight into the most valued activities. This information will be useful in prioritizing the implementation of the new activities to be carried out by the unit.

Objetivo: Evaluar el interés y necesidad de que el farmacéutico desarrolle nuevas actividades propuestas, y potenciar o mantener otras que ya se realizaban, antes de que la futura Unidad de Enfermedades Inflamatorias Inmunomediadas inicie su actividad en nuestro hospital. Además, priorizar la incorporación de las nuevas actividades en base a los  resultados obtenidos.Método: Diseño observacional transversal unicéntrico mediante una encuesta  realizada en enero de 2020 a todos los profesionales sanitarios de los servicios  clínicos implicados y a una muestra de pacientes, y estructurada en dos  categorías: Acciones orientadas a la atención farmacéutica al paciente y  Acciones orientadas a los profesionales de dicha Unidad. Cada ítem se puntuó  de 0 a 10, siendo 10 el máximo interés/necesidad. Se aplicó una matriz de  priorización para cuantificar y evaluar cada actividad e implantar las nuevas  por orden de priorización.Resultados: Se completaron 90 encuestas (30 de pacientes y 60 de profesionales). Se analizaron las medianas obtenidas de cada una de las 20  actividades propuestas, alcanzándose valores entre 8 y 10. Se compararon valores: en el grupo de farmacéuticos versus médicos se  obtuvieron más ítems con diferencias estadísticamente significativas que en el grupo farmacéuticos versus enfermería, o farmacéuticos versus pacientes. Tras la priorización, la primera acción fue implantar la prescripción electrónica en pacientes externos con enfermedades inflamatorias inmunomediadas.Conclusiones: La encuesta ha permitido conocer las expectativas de los  profesionales sanitarios y pacientes sobre la actividad del farmacéutico en  dicha Unidad, cuantificar las actividades más valoradas y priorizar la  implantación de nuevas actividades.

Relationship between air pollution levels in Madrid and the natural history of idiopathic pulmonary fibrosis: severity and mortality.

OBJECTIVE: We aimed to assess the relationship between major air pollutants and the natural history and mortality of idiopathic pulmonary fibrosis (IPF). METHODS: We conducted a retrospective cohort study from 2013 to 2019 among 52 patients with IPF from the pneumology department of a tertiary hospital. According to their geocoded residential address, each patient was assigned a mean concentration of carbon monoxide (CO), nitrogen dioxide, particulate matter 2.5 and 10, ozone, and sulfur dioxide, as measured at a single surveillance station in central Madrid, Spain. We analyzed forced vital capacity (FVC), CO diffusing capacity, 6-minute walking test, degree of dyspnea, radiologic pattern, and signs of pulmonary hypertension in all patients. RESULTS: Patients' mean age was 66 ± 10 years, and 79% were men. The mean predicted FVC was 78.9 ± 0.5%. Forty-two patients met the criteria for severe disease, and 18 patients died. Mortality was significantly associated with increased CO exposure (for each 0.1 mg/m2 increase: odds ratio 2.45, 95% confidence interval 1.39-4.56). We observed no association between any of the other investigated contaminants and IPF mortality or severity. CONCLUSIONS: Air pollution, specifically that caused by carbon monoxide, can increase mortality in patients with IPF.

Desarrollo de un cuestionario dirigido a conocer el proceso de consentimiento informado en investigación clínica desde la perspectiva del paciente / Informed consent process in clinical trials: development of a patient-reported questionnaire

OBJETIVO: Desarrollar un cuestionario en español dirigido a evaluar el proceso de información y obtención del consentimiento informado en investigación clínica desde la perspectiva del paciente. Con esta herramienta se pretende analizar en los pacientes que participan en un ensayo clínico los siguientes aspectos: la experiencia y desarrollo práctico del proceso de consentimiento informado, su nivel de satisfacción con dicho proceso y su nivel de comprensión del estudio. MÉTODO: Estudio de desarrollo, adaptación y validación de un cuestionario autocumplimentable para evaluar el proceso de consentimiento informado a través de la información obtenida de los pacientes. Los pasos seguidos fueron: revisión bibliográfica, generación de un pool de ítems, redacción del cuestionario, revisión por expertos, pilotaje, optimización y análisis de legibilidad. También se realizó una evaluación, selección, traducción y adaptación al español de una herramienta disponible en lengua inglesa que permitiese valorar la comprensión del paciente de la información. RESULTADOS: El cuestionario quedó conformado por cuatro apartados que permiten evaluar: 1) datos sociodemográficos, 2) aspectos prácticos relacionados con el desarrollo del proceso de consentimiento informado, 3) valoración del paciente del proceso (satisfacción, expectativas y motivaciones), 4) grado de comprensión. Para valorar la comprensión se seleccionó el cuestionario Quality of Informed Consent questionnaire, que fue traducido por tres traductores bilingües. Se incluyeron tres preguntas adicionales para evaluar la comprensión de conceptos relacionados con el equívoco terapéutico y el enmascaramiento de los tratamientos. La validez de contenido fue evaluada mediante consulta con un panel de expertos. En el análisis de legibilidad se obtuvo un valor de Índice de Flesch-Szigriszt de 64,34 equivalente a un grado de dificultad "normal" en la escala Inflesz. En el estudio piloto se entrevistó a 32 pacientes que mostraron no tener dificultades para comprender las preguntas ni problemas a la hora de utilizar las escalas de respuesta. El tiempo medio de cumplimentación del cuestionario fue de 16,6 minutos. CONCLUSIONES: La herramienta desarrollada es útil a la hora de conocer y valorar el proceso de consentimiento informado desde la perspectiva del paciente al que se le invita a participar en un estudio. Su aplicación podría resultar de ayuda a los investigadores para verificar que se ha seguido un adecuado proceso y para identificar aspectos concretos que son susceptibles de ser modificados y optimizados

OBJECTIVE: To develop a Spanish-language questionnaire aimed at evaluating patients' perception of the way they are briefed and their consent is obtained prior to participating in clinical trials. The tool was conceived to evaluate the following aspects: patients' personal experience, the way the informed consent process was implemented in practice, patients' level of satisfaction with the process, and their level of understanding of the study itself. METHOD: This study looked into the development, adaptation and validation of a self-administered questionnaire intended to evaluate the informed consent process on the basis of information provided by respondents. The steps followed included: literature review, generation of an items pool, drawing up of the questionnaire, expert review, piloting, and reading ease optimization and analysis. A commonly-used English-language questionnaire was evaluated, translated into Spanish and adapted so as to determine the extent to which subjects understood the information conveyed to them. RESULTS: In its final version, the questionnaire came to comprise four sections intended to evaluate: 1) socio-demographic data; 2) practical aspects related with the development of the informed consent process; 3) patients' perception of the process (satisfaction, expectations and motivations); and 4) their level of understanding. Understanding was gaged using the QuIC questionnaire, translated by three bilingual translators. Additional questions were included to evaluate the understanding of concepts related with blinding and therapeutic misconception. The validity of the contents was evaluated by consulting with an expert panel. The reading ease analysis yielded an IFSZ score of 64.34, equivalent to an "average difficulty" grade on the Inflesz scale. In the pilot study, interviews were held with 32 patients, who did not appear to have any difficulties in understanding the questions asked of them or in using Likert-type scales to respond. Mean completion time was 16.6 minutes. CONCLUSIONS: The tool developed as part of this study has shown itself capable of providing an understanding and an assessment of the informed consent process from the perspective of a patient who is invited to participate in a clinical trial. Implementation of the questionnaire could help investigators ascertain that the process has been correctly executed and identify specific aspects that may require to be changed or optimized

Informed consent process in clinical trials: development of a patient-reported questionnaire.

OBJECTIVE: To develop a Spanish-language questionnaire aimed at  evaluating patients' perception of the way they are briefed and their  consent is obtained prior to participating in clinical trials. The tool was  conceived to evaluate the following aspects: patients' personal experience,  the way the informed consent process was implemented in practice,  patients' level of satisfaction with the process, and their level of  understanding of the study itself. METHOD: This study looked into the development, adaptation and  validation of a self-administered questionnaire intended to evaluate the  informed consent process on the basis of information provided by  respondents. The steps followed included: literature review, generation of  an items pool, drawing up of the questionnaire, expert review, piloting, and reading ease optimization and analysis. A commonly-used English- language questionnaire was evaluated, translated into Spanish and  adapted so as to determine the extent to which subjects understood the  information conveyed to them. RESULTS: In its final version, the questionnaire came to comprise four sections intended to evaluate: 1) socio-demographic data; 2) practical aspects related with the development of the informed consent  process; 3) patients' perception of the process (satisfaction, expectations  and motivations); and 4) their level of understanding. Understanding was gaged using the QuIC questionnaire, translated by three bilingual  translators. Additional questions were included to evaluate the  understanding of concepts related with blinding and therapeutic  misconception. The validity of the contents was evaluated by consulting  with an expert panel. The reading ease analysis yielded an IFSZ score of  64.34, equivalent to an "average difficulty" grade on the Inflesz scale. In  the pilot study, interviews were held with 32 patients, who did not appear to have any difficulties in understanding the questions asked of  them or in using Likert-type scales to respond. Mean completion time was 16.6 minutes. CONCLUSIONS: The tool developed as part of this study has shown itself capable of providing an understanding and an assessment of the  informed consent process from the perspective of a patient who is invited  to participate in a clinical trial. Implementation of the questionnaire could help investigators ascertain that the process has been correctly  executed and identify specific aspects that may require to be changed or  optimized.

Objetivo: Desarrollar un cuestionario en español dirigido a evaluar el proceso de información y obtención del consentimiento informado en  investigación clínica desde la perspectiva del paciente. Con esta  herramienta se pretende analizar en los pacientes que participan en un  ensayo clínico los siguientes aspectos: la experiencia y desarrollo práctico  del proceso de consentimiento informado, su nivel de satisfacción con  dicho proceso y su nivel de comprensión del estudio.Método: Estudio de desarrollo, adaptación y validación de un cuestionario autocumplimentable para evaluar el proceso de  consentimiento informado a través de la información obtenida de los  pacientes. Los pasos seguidos fueron: revisión bibliográfica, generación de  un pool de ítems, redacción del cuestionario, revisión por expertos,  pilotaje, optimización y análisis de legibilidad. También se realizó una  evaluación, selección, traducción y adaptación al español de una  herramienta disponible en lengua inglesa que permitiese valorar la  comprensión del paciente de la información.Resultados: El cuestionario quedó conformado por cuatro apartados que  permiten evaluar: 1) datos sociodemográficos, 2) aspectos prácticos relacionados con el desarrollo del proceso de consentimiento  informado, 3) valoración del paciente del proceso (satisfacción,  expectativas y motivaciones), 4) grado de comprensión. Para valorar la  comprensión se seleccionó el cuestionario Quality of Informed Consent  questionnaire, que fue traducido por tres traductores bilingües. Se  incluyeron tres preguntas adicionales para evaluar la comprensión de  conceptos relacionados con el equívoco terapéutico y el enmascaramiento  de los tratamientos. La validez de contenido fue evaluada mediante  consulta con un panel de expertos. En el análisis de legibilidad se obtuvo  un valor de Índice de Flesch-Szigriszt de 64,34 equivalente a un grado de  dificultad "normal" en la escala Inflesz. En el estudio piloto se entrevistó a  32 pacientes que mostraron no tener dificultades para comprender las  preguntas ni problemas a la hora de utilizar las escalas de respuesta. El  tiempo medio de cumplimentación del cuestionario fue de 16,6 minutos.Conclusiones: La herramienta desarrollada es útil a la hora de conocer y  valorar el proceso de consentimiento informado desde la perspectiva del  paciente al que se le invita a participar en un estudio. Su aplicación podría  resultar de ayuda a los investigadores para verificar que se ha seguido un  adecuado proceso y para identificar aspectos concretos que son  susceptibles de ser modificados y optimizados.

Defibrotide for the treatment of severe hepatic sinusoidal obstruction syndrome: a single-centre experience.

OBJECTIVES: Determine the effectiveness and cost of defibrotide in patients with severe hepatic sinusoidal obstruction syndrome following haematopoietic stem cell transplantation in a tertiary hospital. METHODS: A retrospective observational study. Adults or children treated with defibrotide at a mean dose of 6.8 mg/kg every 6 hours, until the total bilirubin levels are normalised, were included. Main endpoint was complete response, defined as normalised total serum bilirubin levels and resolution of multiple organ failure. Secondary endpoints were survival by 100 days post-transplant, influence of risks factors in effectiveness and cost of treatment. RESULTS: 51 patients (36 adults and 15 children) received defibrotide; median dose of defibrotide administered was 25.19 mg/kg/day (10.0-100.3). Complete response was achieved in 19 (37.3%) patients. By 100 days post-transplant complete response was achieved in 18 (35.3%) patients and the survival rate was 29 (56.9%) patients. There were no significant differences in effectiveness between adults versus paediatrics and between patients who presented risk factors. The mean cost of treatment per patient was €32 916, mean costs in adults was €104 292 and €17 394 in children. CONCLUSION: Regarding the results of our study, it is convenient to identify the patients who will be able to benefit from this treatment according to clinical conditions, medical history and prognosis. Given the great economic impact of defibrotide, we consider that more cost-effectiveness studies are required. TRIAL REGISTRATION NUMBER: EPA-OD, number LBM-DEF-2016-01.

Analysis of an electronic medication reconciliation and information at discharge programme for frail elderly patients.

Background During care transitions, discrepancies and medication errors often occur, putting patients at risk, especially older patients with polypharmacy. Objective To assess the results of a medication reconciliation and information programme for discharge of geriatric patients conducted through hospital information systems. Setting A 1300-bed university hospital in Madrid, Spain. Method A prospective observational study. Geriatricians selected candidates for medication reconciliation at discharge, and sent an electronic inter-consultation request to the pharmacy department. Pharmacists reviewed the medication list, comparing it with electronic prescriptions, medication previously prescribed by primary care physicians and other medical records, and resolved any discrepancies. An individualized and tailored drug information at discharge sheet was sent to geriatricians and made available to primary care physicians. Main outcome measure The number and type of discrepancies, the number, type and severity of errors, and the main pharmacological groups involved. Results Medication reconciliation was performed for 118 patients with a mean age of 87 years (SD 5.9), involving a total of 2054 medications, or 17.4 per patient. Discrepancies were found in 723 (35 %) drugs, 105 of which were considered medication errors (15 %); 66 patients (56 %) had at least one error. This gave 0.9 reconciliation errors per patient reviewed and 1.6 per patient with errors. Of the 105 errors, 14 (13 %) were considered serious. The most frequent errors were incomplete prescriptions (40 %) and omissions (35 %). Conclusion An electronic medication reconciliation programme helps pharmacists detect serious medication errors in frail elderly patients and provides complete and up-to-date written information to prevent additional errors at home.

Study of the True Clinical Progression of Autosomal Dominant Alport Syndrome in a European Population.

BACKGROUND/AIMS: Autosomal dominant Alport syndrome represents 5% of all Alport syndrome cases. This entity presents a different clinical expression from the recessive inheritance pattern and the X chromosome-linked pattern, because it is mild and it shows a late onset, which in many cases even goes unnoticed. METHODS: We carried out a descriptive observational and retrospective clinical study on 19 patients from 5 families with a clinical diagnosis of autosomal dominant Alport Syndrome, and we analyzed the expression of the symptoms in the different families, comparing the results with what has been described in the literature. RESULTS: Renal involvement appeared at a young age, with a progression towards end-stage chronic kidney disease at a median age of 31 years (20.5-36.5). Hearing involvement also appeared in early stages, at a median age of 28.5 years (7.5-62.5). Also, we observed ocular lenticonus-like injuries, which until now have only been described in other inheritance patterns. CONCLUSIONS: Our results suggest that dominant patterns are accompanied by a severe clinical expression that can be superimposed to the recessive and X chromosome-linked patterns, contrary to what has been classically stated. The high phenotypic variability observed in the families lead to the fact that many cases go unnoticed and the severest cases are erroneously diagnosed as recessive, which means that the real prevalence of dominant forms is probably higher than the current 5%.

Emopamil binding protein mutation in conradi-hünermann-happle syndrome representing plaque-type psoriasis.

Conradi-Hünermann-Happle syndrome (CDPX2, OMIM 302960) is an inherited X-linked dominant variant of chondrodysplasia punctata which primarily affects the skin, bones, and eyes. CDPX2 patients display skin defects, including ichthyotic lesions, follicular atrophoderma, cicatricial alopecia, and less frequently ichthyosiform erythroderma, cataracts, and skeletal abnormalities consisting of short stature, asymmetric shortening of the limbs, epiphyseal stippling, and craniofacial defects. CDPX2 results from mutations in emopamil binding protein (EBP) gene. The aim of our study is to identify EBP mutation in a unique case of Conradi-Hünermann-Happle syndrome with rare psoriasiform lesions.

Novel compound heterozygous Thyroglobulin mutations c.745+1G>A/c.7036+2T>A associated with congenital goiter and hypothyroidism in a Vietnamese family. Identification of a new cryptic 5' splice site in the exon 6.

Several patients were identified with dyshormonogenesis caused by mutations in the thyroglobulin (TG) gene. These defects are inherited in an autosomal recessive manner and affected individuals are either homozygous or compound heterozygous for the mutations. The aim of the present study was to identify new TG mutations in a patient of Vietnamese origin affected by congenital hypothyroidism, goiter and low levels of serum TG. DNA sequencing identified the presence of compound heterozygous mutations in the TG gene: the maternal mutation consists of a novel c.745+1G>A (g.IVS6 + 1G>A), whereas the hypothetical paternal mutation consists of a novel c.7036+2T>A (g.IVS40 + 2T>A). The father was not available for segregation analysis. Ex-vivo splicing assays and subsequent RT-PCR analyses were performed on mRNA isolated from the eukaryotic-cells transfected with normal and mutant expression vectors. Minigene analysis of the c.745+1G>A mutant showed that the exon 6 is skipped during pre-mRNA splicing or partially included by use of a cryptic 5' splice site located to 55 nucleotides upstream of the authentic exon 6/intron 6 junction site. The functional analysis of c.7036+2T>A mutation showed a complete skipping of exon 40. The theoretical consequences of splice site mutations, predicted with the bioinformatics tool NNSplice, Fsplice, SPL, SPLM and MaxEntScan programs were investigated and evaluated in relation with the experimental evidence. These analyses predicted that both mutant alleles would result in the abolition of the authentic splice donor sites. The c.745+1G>A mutation originates two putative truncated proteins of 200 and 1142 amino acids, whereas c.7036+2T>A mutation results in a putative truncated protein of 2277 amino acids. In conclusion, we show that the c.745+1G>A mutation promotes the activation of a new cryptic donor splice site in the exon 6 of the TG gene. The functional consequences of these mutations could be structural changes in the protein molecule that alter the biosynthesis of thyroid hormones.

A new mutation in the COL4A3 gene responsible for autosomal dominant Alport syndrome, which only generates hearing loss in some carriers.

Bilateral sensorineural hearing loss is a characteristic feature of Alport syndrome, which is always linked to renal manifestations so they have a parallel evolution and prognosis, and deafness helps to identify the renal disease. We report a family that suffers an autosomal dominant Alport syndrome caused by a previously undescribed mutation in the COL4A3 gene, in which several members have hearing impairment as the only clinical manifestation, suggesting that in this family deafness can occur independent of renal disease. This mutation is also present in a patient with anterior lenticonus, an observation only found in families with recessive and sex-linked Alport disease.