Guía de práctica clínica multidisciplinar española sobre la enfermedad de Anderson-Fabry en adultos. I: Método y recomendaciones / Spanish multidisciplinary clinical practice guidelines for Anderson-Fabry Disease in Adults. I. Method and recommendations

La enfermedad de Anderson-Fabry es una afección multisistémica progresiva y grave de origen genético que afecta tanto a hombres como a mujeres y que reduce sus expectativas y calidad de vida. La gran variabilidad en su expresión clínica, las dificultades para su diagnóstico y la disponibilidad actual de varias alternativas para su tratamiento suponen un gran reto que justifica la realización de una guía de práctica clínica basada en la evidencia que pueda ayudar a los profesionales sanitarios en la toma de decisiones en el manejo de estos pacientes. Para elaborarla se ha realizado una búsqueda sistemática en las principales bases de datos bibliográficas mediante estrategias adaptadas a cada una de las 32 preguntas clínicas consideradas. Se confeccionaron fichas para la síntesis y evaluación de la calidad de las evidencias para cada una de las preguntas. La metodología empleada se basa en el Manual metodológico español para la elaboración de guías de práctica clínica e incorpora en la evaluación de la evidencia científica y en la elaboración de las recomendaciones la metodología GRADE, considerando la calidad de la evidencia, el balance entre beneficios y riesgos, valores y preferencias de los pacientes, equidad y uso de recursos. Para la elaboración definitiva de las recomendaciones se llevó a cabo un proceso de consenso estructurado basado en la metodología Delphi-RAND en 2 rondas, con un panel de expertos propuesto por diferentes sociedades científicas, centros de investigación y asociaciones de pacientes. Finalmente, se han elaborado 92 recomendaciones específicas para el manejo de la enfermedad de Fabry

Anderson-Fabry disease is a severe progressive multisystem condition of genetic origin that affects men and women, reducing their life expectancy and quality of life. The considerable variability in its clinical expression, the difficulties in diagnosing the condition and the current availability of several alternatives for its treatment represent a considerable challenge that justifies the development of evidence-based clinical practice guidelines that can help health professionals in the decision-making process for managing these patients. To develop these guidelines, we conducted a systematic search of the main reference databases using strategies adapted to each of the 32 clinical questions considered. We prepared documents to synthesise the evidence and assess its quality for each of the questions. The methodology employed is based on the Spanish methodology manual for preparing clinical practice guidelines, incorporating the GRADE methodology in the assessment of the scientific evidence and the preparation of the recommendations, considering the quality of the evidence, the risk-benefit balance, patient values and preferences, equity and use of resources. For the definitive preparation of the recommendations, we conducted a structured consensus process based on the Delphi-RAND methodology in 2 rounds, with an expert panel proposed by various scientific societies, research centres and patient associations. Ultimately, we developed 92 specific recommendations for managing Fabry disease

Spanish multidisciplinary clinical practice guidelines for Anderson-Fabry Disease in Adults. I. Method and recommendations. / Guía de práctica clínica multidisciplinar española sobre la enfermedad de Anderson-Fabry en adultos. I: Método y recomendaciones.

Anderson-Fabry disease is a severe progressive multisystem condition of genetic origin that affects men and women, reducing their life expectancy and quality of life. The considerable variability in its clinical expression, the difficulties in diagnosing the condition and the current availability of several alternatives for its treatment represent a considerable challenge that justifies the development of evidence-based clinical practice guidelines that can help health professionals in the decision-making process for managing these patients. To develop these guidelines, we conducted a systematic search of the main reference databases using strategies adapted to each of the 32 clinical questions considered. We prepared documents to synthesise the evidence and assess its quality for each of the questions. The methodology employed is based on the Spanish methodology manual for preparing clinical practice guidelines, incorporating the GRADE methodology in the assessment of the scientific evidence and the preparation of the recommendations, considering the quality of the evidence, the risk-benefit balance, patient values and preferences, equity and use of resources. For the definitive preparation of the recommendations, we conducted a structured consensus process based on the Delphi-RAND methodology in 2 rounds, with an expert panel proposed by various scientific societies, research centres and patient associations. Ultimately, we developed 92 specific recommendations for managing Fabry disease.

Consenso Delphi-RAND de la Sociedad Española de Medicina Interna sobre controversias en terapia y profilaxis anticoagulante en enfermedades médicas. Proyecto INTROMBIN (INcertidumbre en TROMBoprofilaxis en Medicina INterna) / Delphi-RAND consensus of the Spanish Society of Internal Medicine on the controversies in anticoagulant therapy and prophylaxis in medical diseases. INTROMBIN Project (Uncertainty in thromboprophylaxis in internal medicine)

El objetivo de este estudio ha sido conocer la opinión de los internistas sobre el manejo de la anticoagulación y profilaxis tromboembólica en escenarios clínicos complejos en los que el balance riesgo/beneficio de la intervención es estrecho y elaborar un documento de consenso sobre el uso de fármacos anticoagulantes en este grupo de pacientes. Para ello, se identificaron por consenso las áreas clínicas de mayor incertidumbre, se elaboró una encuesta con 20 escenarios desplegados en 40 preguntas clínicas y se realizó una revisión bibliográfica específica. La encuesta se distribuyó entre los internistas de la Sociedad Española de Medicina Interna (SEMI) y fue cumplimentada por 290 de sus miembros. El proceso de consenso se desarrolló mediante una modificación del método Delphi-RAND de adecuación en un proceso anonimizado de doble ronda que permite al panel de expertos identificar áreas de acuerdo y de incertidumbre. En nuestro caso, además, se incorporaron al panel los resultados de la encuesta, innovación metodológica que permite aportar información adicional de la práctica clínica habitual. El resultado del proceso es un conjunto de 19 recomendaciones formuladas por expertos de la SEMI que permite establecer pautas de actuación sobre el tratamiento anticoagulante en escenarios complejos (alto riesgo o hemorragia activa, corta expectativa vital, coexistencia de tratamiento antiagregante o comorbilidades como enfermedad renal, hepática, etc.), que no son infrecuentes en la práctica clínica habitual

The aim of this study was to determine the opinion of internists on the management of anticoagulation and thromboembolism prophylaxis in complex clinical scenarios in which the risk-benefit ratio of surgery is narrow and to develop a consensus document on the use of drugs anticoagulant therapy in this patient group. To this end, we identified by consensus the clinical areas of greatest uncertainty, a survey was created with 20 scenarios laid out in 40 clinical questions, and we reviewed the specific literature. The survey was distributed among the internists of the Spanish Society of Internal Medicine (SEMI) and was completed by 290 of its members. The consensus process was implemented by changing the Delphi-RAND appropriateness method in an anonymous, double-round process that enabled an expert panel to identify the areas of agreement and uncertainty. In our case, we also added the survey results to the panel, a methodological innovation that helps provide additional information on the standard clinical practice. The result of the process is a set of 19 recommendations formulated by SEMI experts, which helps establish guidelines for action on anticoagulant therapy in complex scenarios (high risk or active haemorrhage, short life expectancy, coexistence of antiplatelet therapy or comorbidities such as kidney disease and liver disease), which are not uncommon in standard clinical practice

Delphi-RAND consensus of the Spanish Society of Internal Medicine on the controversies in anticoagulant therapy and prophylaxis in medical diseases. INTROMBIN Project (Uncertainty in thromboprophylaxis in internal medicine). / Consenso Delphi-RAND de la Sociedad Española de Medicina Interna sobre controversias en terapia y profilaxis anticoagulante en enfermedades médicas. Proyecto INTROMBIN (INcertidumbre en TROMBoprofilaxis en Medicina INterna).

The aim of this study was to determine the opinion of internists on the management of anticoagulation and thromboembolism prophylaxis in complex clinical scenarios in which the risk-benefit ratio of surgery is narrow and to develop a consensus document on the use of drugs anticoagulant therapy in this patient group. To this end, we identified by consensus the clinical areas of greatest uncertainty, a survey was created with 20 scenarios laid out in 40 clinical questions, and we reviewed the specific literature. The survey was distributed among the internists of the Spanish Society of Internal Medicine (SEMI) and was completed by 290 of its members. The consensus process was implemented by changing the Delphi-RAND appropriateness method in an anonymous, double-round process that enabled an expert panel to identify the areas of agreement and uncertainty. In our case, we also added the survey results to the panel, a methodological innovation that helps provide additional information on the standard clinical practice. The result of the process is a set of 19 recommendations formulated by SEMI experts, which helps establish guidelines for action on anticoagulant therapy in complex scenarios (high risk or active haemorrhage, short life expectancy, coexistence of antiplatelet therapy or comorbidities such as kidney disease and liver disease), which are not uncommon in standard clinical practice.

[Are the guidelines guiding us reliable? Evaluation of the Spanish clinical practice guidelines]. / Las guías que nos guían son fiables? Evaluación de las guías de práctica clínica españolas.

BACKGROUND AND OBJECTIVE: There is a large proliferation of clinical practice guidelines (CPG), there being doubts on inconsistencies between the recommendations made and internal incongruencies, that affect the role of the guidelines as instruments helping the clinicians. This study aims to analyze the quality of a group of Spanish CPG and to assess the feasibility of use and consistency of the AGREE Instrument in its Spanish version, designed to evaluate the CPG. METHOD: A total of 278 CPG produced in Spain between January 1990 and December 2002 were identified. The originals of 61 guidelines were recovered from their authors and passed to the AGREE Instrument independently by four raters. Quality of the guidelines feasibility and reproducibility of AGREE were analyzed. RESULTS: For all the attributes of AGREE except in the editorial independence, more than 60% of the guidelines evaluated obtained a poor quality score, there being no domain in which more than 10% of the guidelines was excellent. The worst grading areas are those of applicability, participation and rigour of development of the guidelines. Of the 61 guidelines evaluated, the reviewers graded 25 as "very poor quality", 26 as "non recommendable" and 6 and 4 as "recommendable" or "highly recommendable", respectively. Interrater consistency was high. CONCLUSIONS: The mean quality of the Spanish guidelines evaluated is very low. The AGREE Instrument in its Spanish version is consistent for the Spanish guidelines and easy to use.

¿Las guías que nos guían son fiables? Evaluación de las guías de práctica clínica españolas / Are the guidelines guiding us reliable? Evaluation of the Spanish clinical practice guidelines

Fundamento y objetivo. Hay gran proliferación de guías de práctica clínica (GPC), existiendo dudas sobre inconsistencias entre las recomendaciones que realizan e incongruencias internas que comprometen el papel de las guías como instrumentos de ayuda a los clínicos. El estudio pretende analizar la calidad de un conjunto de GPC españolas, así como valorar la factibilidad de uso y consistencia del Instrumento AGREE en su versión en español, diseñado para la evaluación de GPC. Método. Se identificaron 278 GPC producidas en España entre enero de 1990 y diciembre del 2002. Se recuperaron, mediante solicitud a sus autores, originales de 61 guías a las que se pasó el Instrumento AGREE de forma independiente por cuatro evaluadores. Se analizó la calidad de las guías, la factibilidad y reproducibilidad del AGREE. Resultados. Para todos los atributos del AGREE, excepto en la independencia editorial, más del 60% de las guías evaluadas obtienen una puntuación de mala calidad, no habiendo ningún dominio en el que más del 10% de las guías sea excelente. Las áreas de peor calificación son las de aplicabilidad, participación y rigor en el desarrollo de las guías. De las 61 guías evaluadas, los revisores a 25 las califican de «muy poco recomendables», a 26 de «no recomendables» y tan sólo a 6 y 4 guías como «recomendables» o «muy recomendables», respectivamente. La consistencia entre evaluadores fue alta. Conclusiones. La calidad media de las guías españolas evaluadas es muy baja. El Instrumento AGREE, en su versión en español es consistente para las guías españolas y de uso fácil

Background and objective. There is a large proliferation of clinical practice guidelines (CPG), there being doubts on inconsistencies between the recommendations made and internal incongruencies, that affect the role of the guidelines as instruments helping the clinicians. This study aims to analyze the quality of a group of Spanish CPG and to assess the feasibility of use and consistence of the AGREE Instrument in its Spanish version, designed to evaluate the CPG. Method. A total of 278 CPG produced in Spain between Jannuary 1990 and December 2002 were identified. The originals of 61 guidelines were recovered from their authors and passed to the AGREE Instrument independently by four raters. Quality of the quidelines feasibility and reproducibility of AGREE were analyzed. Results. For all the attributes of AGREE except in the editorial independence, more than 60% of the guidelines evaluated obtained a poor quality score, there being no domain in which more than 10% of the guidelines was excellent. The worst grading areas are those of applicability, participation and rigour of development of the guidelines. Of the 61 guidelines evaluated, the reviewers graded 25 as «very poor quality», 26 as «non recommendable» and 6 and 4 as «recommendable» or «highly recommendable», respectively. Interrater consistency was high. Conclusions. The mean quality of the Spanish guidelines evaluated is very low. The AGREE Instrument in its Spanish version is consistent for the Spanish guidelines and easy to use

Carta al Director / Letter to the Editor

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