Design of mobile and website health application devices for drug tolerability in hereditary fructose intolerance.

BACKGROUND: Hereditary fructose intolerance (HFI) is a rare metabolic disease caused by aldolase B deficiency. The aim of our study was to analyse excipient tolerability in patients with HFI and other related diseases and to design mobile and website health applications to facilitate the search for drugs according to their tolerance. RESULTS: A total of 555 excipients listed in the Spanish Medicines Agency database (July 2023) were classified as suitable for HFI patients, suitable with considerations ((glucose and glucose syrup, intravenous sucrose, oral mannitol, polydextrose, gums and carrageenans, ethanol, sulfite caramel and vanilla), not recommended (intravenous mannitol) and contraindicated (fructose, oral sucrose, invert sugar, sorbitol, maltitol, lactitol, isomaltitol, fruit syrups, honey, sucrose esters and sorbitol esters). Glucose and glucose syrup were classified as suitable with considerations due to its possible fructose content and their potential endogenous fructose production. For other related intolerances, wheat starch was contraindicated and oatmeal was not recommended in celiac disease; oral lactose and lactose-based coprocessed excipient (Cellactose®) were not recommended in lactose intolerance; and glucose, invert sugar and oral sucrose were not recommended in diabetes mellitus. The applications were named IntoMed®. Results are listed in order of tolerability (suitable drugs appear first and contraindicated drugs at the end), and they are accompanied by a note detailing their classified excipients. If a drug contains excipients within different categories, the overall classification will be the most restrictive. The apps are also able to classify substances with the same criteria if they act as active ingredients. The tools exhibited good usability (82.07 ± 13.46 points on the System Usability Scale [range: 0-100]) on a sample of HFI patients, their families and health care professionals. CONCLUSIONS: IntoMed® is a tool for finding information about the tolerability of drugs according to excipients for patients with HFI and other related intolerances, with good usability. It is a fast and reliable system that covers the current excipient legislation and expands on it with other specific information: HFI patients should be alert for excipients such as mannitol (especially in intravenous drugs), fruit syrups, honey, sulfite caramel or vanilla. Glucose might contain or produce fructose, and special precaution is needed because of potential errors in their composition.

Reply letter to the editor concerning the article 'Safety of Sars-Cov-2 vaccines administration for adult patients with hereditary fructose intolerance'.

According to the current European medicines legislation, on the labeling is mandatory a warning contraindicating for hereditary fructose intolerance (HFI) patients medicines with oral or parenteral fructose and sorbitol, and oral sucrose, invert sugar, isomaltitol, lactitol and maltitol, but parenteral sucrose is not mentioned. Intravenous administration of sucrose does not increase blood glucose concentrations, because sucrose is poorly oxidized to CO2 and mainly excreted in the urine as a disaccharide; absence of enzimatic activity outside the gut explains why there is not a warning for parenteral sucrose presentations. For this reason, parenteral drugs with sucrose are allowed in HFI patients. Nevertheless, due to interindividual variability and the fact that not all parenterally administered sucrose is recovered in urine, HFI patients need to be closely monitored after parenteral administration of sucrose-containing drugs, especially when the amount exceeds the maximum permissible thresholds.

Systemic thrombosis in a large cohort of COVID-19 patients despite thromboprophylaxis: A retrospective study.

BACKGROUND: Incidence of thrombotic events associated to Coronavirus disease-2019 (COVID-19) is difficult to assess and reported rates differ significantly. Optimal thromboprophylaxis is unclear. OBJECTIVES: We aimed to analyze the characteristics of patients with a confirmed thrombotic complication including inflammatory and hemostatic parameters, compare patients affected by arterial vs venous events and examine differences between survivors and non-survivors. We reviewed compliance with thromboprophylaxis and explored how the implementation of a severity-adjusted protocol could have influenced outcome. METHODS: Single-cohort retrospective study of COVID-19 patients admitted, from March 3 to May 3 2020, to the Infanta Leonor University Hospital in Madrid, epicenter of the Spanish outbreak. RESULTS: Among 1127 patients, 80 thrombotic events were diagnosed in 69 patients (6.1% of the entire cohort). Forty-three patients (62%) suffered venous thromboembolism, 18 (26%) arterial episodes and 6 (9%) concurrent venous and arterial thrombosis. Most patients (90%) with a confirmed thrombotic complication where under low-molecular-weight heparin treatment. Overt disseminated intravascular coagulation (DIC) was rare. Initial ISTH DIC score and pre-event CRP were significantly higher among non-survivors. In multivariate analysis, arterial localization was an independent predictor of mortality (OR = 18, 95% CI: 2.4-142, p < .05). CONCLUSIONS: Despite quasi-universal thromboprophylaxis, COVID-19 lead to a myriad of arterial and venous thrombotic events. Considering the subgroup of patients with thrombotic episodes, arterial events appeared earlier in the course of disease and conferred very poor prognosis, and an ISTH DIC score ≥ 3 at presentation was identified as a potential predictor of mortality. Severity-adjusted thromboprophylaxis seemed to decrease the number of events and could have influenced mortality. Randomized controlled trials are eagerly awaited.

Exposición a fármacos asociados a agravamiento de síntomas en pacientes con miastenia grave / Drug exposure associated with exacerbation of symptoms in patients with myasthenia gravis

INTRODUCCIÓN: Numerosos fármacos se han relacionado con el agravamiento de síntomas en pacientes con miastenia grave, pero hasta la fecha no existen estudios sobre la exposición a fármacos en estos pacientes. OBJETIVOS: Describir el consumo de fármacos y calcular la tasa anual de episodios de exacerbación en una cohorte de pacientes con miastenia grave, y explorar posibles factores de riesgo de exacerbaciones graves. PACIENTES Y MÉTODOS: Estudio observacional longitudinal retrospectivo que incluye a pacientes adultos con miastenia grave seguidos en consulta. Cálculo de frecuencias, tasas y construcción de modelo de eventos repetidos. RESULTADOS: De 91 pacientes incluidos, el 94,51% estuvo expuesto al menos a un fármaco durante el período de estudio (siete años y un mes). De ellos, 51 tuvieron al menos una prescripción de un fármaco contraindicado en la ficha técnica (56,04%). Se contabilizaron 145 exacerbaciones en 50 pacientes. La tasa anual de incidencia fue de 0,35 exacerbaciones por paciente y año. De estas exacerbaciones, 48 fueron graves (en 18 pacientes), con una tasa anual de incidencia de 0,12. Se halló una posible asociación entre diagnóstico de miastenia grave generalizada y timectomía, con un aumento del riesgo de episodios de exacerbación graves. CONCLUSIONES: En esta cohorte se encontró una amplia exposición a fármacos, pero no asociación con el riesgo de episodios de exacerbación graves. Algo más de la mitad de pacientes tuvo al menos un episodio de exacerbación durante el período de estudio, la mayoría leves. Son necesarios estudios que corroboren estas conclusiones y puedan estudiar posibles correlaciones entre fármacos y el riesgo de episodios de exacerbación

INTRODUCTION: Numerous drugs have been related to exacerbation of myasthenia gravis. So far there are no studies examining the extent of use of drugs related to exacerbation of myasthenia gravis. AIMS: We sought to assess the extent of use of drugs related to exacerbations and the annual incidence rate of exacerbations in a cohort of myasthenia gravis patients. We explored possible risk factors of severe exacerbations. PATIENTS AND METHODS: We performed a retrospective cohort study. We included adult patients followed in neurology department. We estimated frequencies, rates and built a recurrent events model. RESULTS: We included 91 patients. 94.51% of patients had at least one prescription of a drug. 51 patients had at least one prescription of a drug contraindicated according to its drug label. 145 exacerbation episodes were reported in 50 patients. The annual incidence rate of exacerbation episodes was 0.35. 48 exacerbations were severe (in 18 patients). The annual incidence rate of severe exacerbation episodes was 0.12. Generalized myasthenia gravis and thymectomy were associated with a higher risk of severe exacerbation episodes. CONCLUSIONS: Our patients were extensive and widespread exposed to drugs during the follow-up period but we did not find and association with severe exacerbation episodes. Just over half of the patients had at least one exacerbation episode during the study period, most of them were mild. Further studies with larger sample sizes are necessary to corroborate these conclusions and to study possible correlations between the use of drugs and the risk of exacerbation episodes

Experiencia autorreferida en pacientes tratados con antivirales directos frente al virus de la hepatitis C / Self-reported experience in patients treated with Hepatitis C direct acting antivirals

Introducción y objetivo: Conocer y analizar la experiencia autorreferida del tratamiento de los pacientes VHC que iniciaron tratamiento con agentes antivirales directos (AAD), a tiempo real, de forma proactiva e integrada en el proceso asistencial de atención farmacéutica. Material y métodos: Estudio observacional y transversal desarrollado entre abril (inicio del Plan Estratégico Nacional) y diciembre de 2015 en la Consulta Externa del Servicio de Farmacia. Se utilizó como herramienta un cuestionario prospectivo cumplimentado por los pacientes donde se recogieron variables relacionadas con la calidad de vida vinculadas con la salud (CVRS), adherencia, efectos adversos (EA), satisfacción con el tratamiento, y valoración y utilidad del programa formativo implementado por el Servicio de Farmacia. Se realizó un análisis descriptivo de todas las variables incluidas en el estudio y se analizó la influencia de las diferentes variables en el grado de adherencia y CVRS. El análisis de las diferencias entre los dos grupos se realizó mediante el test de chi cuadrado y el cálculo de las OR con un modelo de regresión logística simple. Se utilizó el programa SPSS® versión 20, y se estableció una significación estadística para valores de p < 0,05. Resultados: Se recogieron155 encuestas de las 226 enviadas, tasa de respuesta del 68,6%.En referencia a la CVRS (valoración del estado físico y emocional), un 38,7% de los pacientes refieren que su estado físico y emocional es mucho mejor desde el inicio del tratamiento. La presencia de EA y una peor información global de su enfermedad se asoció con un peor estado físico y emocional (p < 0,05). La adherencia reportada fue del 84,5% y el tratamiento fue valorado como muy bueno o bueno por el 87% de los pacientes. Un 52,9% de los pacientes no tuvieron efectos adversos relacionados con la medicación y el proceso formativo realizado por el farmacéutico especialista en la primera consulta fue valorado por el 96,7% de los pacientes como muy bueno o bueno. Conclusiones: La experiencia autorreferida obtenida a través de un contacto directo y permanente con los pacientes permite obtener información sobre aspectos importantes del tratamiento. Creemos que estas herramientas deben incorporarse a los procedimientos de atención farmacéutica como una forma de mantener la continuidad. Además, inducen al paciente a una autoevaluación de diversos aspectos de su propio tratamiento, que pueden ayudar a conseguir una mayor implicación en el mismo y contribuir a conseguir el máximo resultado en salud posible de la farmacoterapia (AU)

Background and objective: To learn about and analyze the self-reported treatment experience of HCV patients who started treatment with direct acting antivirals agents (DAA), at a real-time, proactive and integrated into the pharmaceutical care healthcare process, using a prospective questionnaire completed by patients as clinical tool. Material and methods: Observational and cross-sectional study conducted between April (start of the National Strategic Plan) and December 2015 in the Outpatient Pharmacy Service. The questionnaire includes variables related to health related quality of life (HRQOL), adherence, adverse effects (AEs), satisfaction, and usefulness of the Pharmacy Service implemented training program. A descriptive analysis of all variables included in the study was conducted and the influence of different variables analyzed in the degree of adherence and HRQOL. The analysis of the differences was performed using chi-square test and simple logistic regression model for calculation of OR. We use SPSS version 20 program and statistical significance for values of p < 0.05 was considered. Results: 155 of the 226 surveys returned, with a response rate of 68.6%. Referring to the HRQOL (evaluation of physical and emotional state), 38.7% of patients reports that their physical and emotional state is much better from the start of treatment. The presence of EA and worse global information of their disease was associated with worse physical and emotional state (p < 0.05). Reported adherence was 84.5% and the treatment was evaluated as very good or good by 87% of patients. 52.9% had no adverse effects related to the medication and the training process performed by the specialist pharmacist at the first visit 96.7% of patients assessed as very good or good. Conclusions: Self-reported experience acquired through direct and constant contact with patients provides information on important aspects of treatment. We believe that these tools should be incorporated into pharmaceutical care procedures as a way to maintain continuity in patients direct contact. They also induce patients to a self-assessment of various aspects of their own treatment, which can help achieve greater involvement in it and can contribute to achieve the maximum health outcome in pharmacotherapy (AU)

[Self-reported experience in patients treated with Hepatitis C direct acting antivirals]. / Experiencia autorreferida en pacientes tratados con antivirales directos frente al virus de la hepatitis C.

BACKGROUND AND OBJECTIVE: To learn about and analyze the self-reported treatment experience of HCV patients who started treatment with direct acting antivirals agents (DAA), at a real-time, proactive and integrated into the pharmaceutical care healthcare process, using a prospective questionnaire completed by patients as clinical tool. MATERIAL AND METHODS: Observational and cross-sectional study conducted between April (start of the National Strategic Plan) and December 2015 in the Outpatient Pharmacy Service. The questionnaire includes variables related to health related quality of life (HRQOL), adherence, adverse effects (AEs), satisfaction, and usefulness of the Pharmacy Service implemented training program. A descriptive analysis of all variables included in the study was conducted and the influence of different variables analyzed in the degree of adherence and HRQOL. The analysis of the differences was performed using chi-square test and simple logistic regression model for calculation of OR. We use SPSS version 20 program and statistical significance for values of p < 0.05 was considered. RESULTS: 155 of the 226 surveys returned, with a response rate of 68.6%. Referring to the HRQOL (evaluation of physical and emotional state), 38.7% of patients reports that their physical and emotional state is much better from the start of treatment. The presence of EA and worse global information of their disease was associated with worse physical and emotional state (p < 0.05). Reported adherence was 84.5% and the treatment was evaluated as very good or good by 87% of patients. 52.9% had no adverse effects related to the medication and the training process performed by the specialist pharmacist at the first visit 96.7% of patients assessed as very good or good. CONCLUSIONS: Self-reported experience acquired through direct and constant contact with patients provides information on important aspects of treatment. We believe that these tools should be incorporated into pharmaceutical care procedures as a way to maintain continuity in patients direct contact. They also induce patients to a self-assessment of various aspects of their own treatment, which can help achieve greater involvement in it and can contribute to achieve the maximum health outcome in pharmacotherapy.

Introducción y objetivo: Conocer y analizar la experiencia autorreferida del tratamiento de los pacientes VHC que iniciaron tratamiento con agentes antivirales directos (AAD), a tiempo real, de forma proactiva e integrada en el proceso asistencial de atención farmacéutica. Material y métodos: Estudio observacional y transversal desarrollado entre abril (inicio del Plan Estratégico Nacional) y diciembre de 2015 en la Consulta Externa del Servicio de Farmacia. Se utilizó como herramienta un cuestionario prospectivo cumplimentado por los pacientes donde se recogieron variables relacionadas con la calidad de vida vinculadas con la salud (CVRS), adherencia, efectos adversos (EA), satisfacción con el tratamiento, y valoración y utilidad del programa formativo implementado por el Servicio de Farmacia. Se realizó un análisis descriptivo de todas las variables incluidas en el estudio y se analizó la influencia de las diferentes variables en el grado de adherencia y CVRS. El análisis de las diferencias entre los dos grupos se realizó mediante el test de chi cuadrado y el cálculo de las OR con un modelo de regresión logística simple. Se utilizó el programa SPSS® versión 20, y se estableció una significación estadística para valores de p < 0,05. Resultados: Se recogieron155 encuestas de las 226 enviadas, tasa de respuesta del 68,6%.En referencia a la CVRS (valoración del estado físico y emocional), un 38,7% de los pacientes refieren que su estado físico y emocional es mucho mejor desde el inicio del tratamiento. La presencia de EA y una peor información global de su enfermedad se asoció con un peor estado físico y emocional (p < 0,05). La adherencia reportada fue del 84,5% y el tratamiento fue valorado como muy bueno o bueno por el 87% de los pacientes. Un 52,9% de los pacientes no tuvieron efectos adversos relacionados con la medicación y el proceso formativo realizado por el farmacéutico especialista en la primera consulta fue valorado por el 96,7% de los pacientes como muy bueno o bueno. Conclusiones: La experiencia autorreferida obtenida a través de un contacto directo y permanente con los pacientes permite obtener información sobre aspectos importantes del tratamiento. Creemos que estas herramientas deben incorporarse a los procedimientos de atención farmacéutica como una forma de mantener la continuidad. Además, inducen al paciente a una autoevaluación de diversos aspectos de su propio tratamiento, que pueden ayudar a conseguir una mayor implicación en el mismo y contribuir a conseguir el máximo resultado en salud posible de la farmacoterapia.

Cross-cultural Adaptation and Validation of the Medication Regimen Complexity Index Adapted to Spanish.

BACKGROUND: The most widely used validated instrument to assess the complexity of medication regimens is the Medication Regimen Complexity Index (MRCI). OBJECTIVE: This study aimed to translate, adapt, and validate a reliable version of the MRCI adapted to Spanish (MRCI-E). METHODS: The cross-cultural adaptation process consisted of an independent translation by 3 clinical pharmacists and a backtranslation by 2 native English speakers. A reliability analysis was conducted on 20 elderly randomly selected patients. Two clinical pharmacists calculated the MRCI-E from discharge treatments and 2 months later. For the validity analysis, the sample was augmented to 60 patients. Convergent validity was assessed by analyzing the correlation between the number of medications; discriminant validity was stratified by gender; and predictive validity was determined by analyzing the ability to predict readmission and mortality at 3 and 6 months. RESULTS: The MRCI-E retained the original structure of 3 sections. The reliability analysis demonstrated an excellent internal consistency (Cronbach's α=0.83), and the intraclass correlation coefficient exceeded 0.9 in all cases. The correlation coefficient with the number of medications was 0.883 ( P<0.001). No significant differences were found when stratified by gender (3.6; 95%CI=-2.9 to 10.2; P=0.27). Patients who were readmitted at 3 months had a higher MRCI-E score (10.7; 95%CI=4.4 to 17.2; P=0.001). The differences remained significant in patients readmitted at 6 months, but differences in mortality were not detected. CONCLUSIONS: The MRCI-E retains the reliability and validity of the original index and provides a suitable tool to assess the complexity of medication regimens in Spanish.

[Risk prevention evaluation in the incorporation of new drugs for healthcare practices: a methodological proposal]. / Evaluación preventiva de riesgos en la incorporación de nuevos medicamentos a la práctica asistencial: una propuesta metodológica.

OBJECTIVE: Submit a preventive assessment methodology for the risk of medication errors when incorporating new drugs in clinical practice as well as a description of the types of actions arising from such action. MATERIALS AND METHODS: A Standard Operating Procedure was established which defines and plans the process of assessing the risks of new drugs purchased by the Pharmacy Services before being incorporated into the distribution and dispensation channels. The pharmacist responsible for each area fills the Risk Assessment Form, a questionnaire that facilitates the analysis of the product characteristics and provides guidance on safety measures to be taken depending on the results of that assessment. If it is confirmed that the drug requires some additional security measures to be taken, all professionals and technical staff of the Pharmacy Services shall be informed of measures to be taken. RESULTS: Between 2011 and 2013, 64 drugs were assessed. 35 of them (54.6%) required some additional security measure to be applied before being incorporated into clinical practice. CONCLUSIONS: The implementation of the method of preventive risk assessment for new drugs purchased by the Pharmacy Services can be a very useful tool when taking the measures deemed necessary to minimize the likelihood of a medication error occurring before they are incorporated into the distribution/dispensing channels put in place by the Pharmacy Services.

Evaluación preventiva de riesgos en la incorporación de nuevos medicamentos a la práctica asistencial: una propuesta metodológica / Risk prevention evaluation in the incorporation of new drugs for healthcare practices: a methodological proposal

Objetivo: Presentar una metodología de evaluación preventiva de riesgos de errores de medicación en la incorporación de nuevos medicamentos a la práctica asistencial, así como una descripción de las acciones derivadas de dicha actuación. Material y métodos: Se estableció un Procedimiento Normalizado de trabajo que define el proceso de evaluación de riesgos de los nuevos medicamentos adquiridos por el Servicio de Farmacia antes de ser incorpora la Ficha de Evaluación de Riesgos, cuestionario que facilita el análisis delas características del medicamento y orienta sobre las medidas de seguridad que se deben adoptar en función de los resultados. Resultados: Se evaluaron 64 medicamentos. En 35 (54,6%) se aplicó alguna medida preventiva de seguridad: incorporación a la Guía de Administración de medicamentos por sonda nasogástrica (3 fármacos), aplicación de la instrucción Técnica de cambio de concentración/presentación segura (7), incorporación al listado de medicamentos que contienen Látex (3), remisión de notas informativas a los profesionales sanitarios (12), adopción de medidas para almacenamiento seguro (6), incorporación de material educativo en los folletos informativos para pacientes externos (12), incorporación de interacciones farmacológicas a la base de datos (2), inicio de procedimiento de resolución de expediente (1) y comunicación a la AEMPS de errores potenciales por similitud de envases (2). Conclusiones: La implantación de esta evaluación preventiva de riesgos puede resultar una herramienta muy útil a la hora de tomar las medidas para minimizar la probabilidad de que se produzca un error de medicación, antes de que se incorpore a los circuitos de distribución/dispensación

Objective: Submit a preventive assessment methodology for the risk of medication errors when incorporating new drugs in clinical practice as well as a description of the types of actions arising from such action. Materials and methods: A Standard Operating Procedure was established which defines and plans the process of assessing the risks of new drugs purchased by the Pharmacy Services before being incorporated into the distribution and dispensation channels. The pharmacist responsible for each area fills the Risk Assessment Form, a questionnaire that facilitates the analysis of the product characteristics and provides guidance on safety measures to be taken depending on the results of that assessment. If it is confirmed that the drug requires some additional security measures to be taken, all professionals and technical staff of the Pharmacy Services shall be informed of measures to be taken. Results: Between 2011 and 2013, 64 drugs were assessed. 35of them (54.6%) required some additional security measure t be applied before being incorporated into clinical practice. Conclusions: The implementation of the method of preventive risk assessment for new drugs purchased by the Pharmacy Services before being incorporated into the distribution and dispensationchannels. The pharmacist responsible for each area fills the RiskAssessment Form, a questionnaire that facilitates the analysisof the product characteristics and provides guidance on safetymeasures to be taken depending on the results of that assessment. If it is confirmed that the drug requires some additionalsecurity measures to be taken, all professionals and technicalstaff of the Pharmacy Services shall be informed of measuresto be taken. Results: Between 2011 and 2013, 64 drugs were assessed. 35of them (54.6%) required some additional security measure tobe applied before being incorporated into clinical practice. Conclusions:The implementation of the method of preventiverisk assessment for new drugs purchased by the Pharmacy Services can be a very useful tool when taking the measures dee-med necessary to minimize the likelihood of a medication erroroccurring before they are incorporated into the distribution/dis-pensing channels put in place by the Pharmacy Services

Drug prescribing in patients with renal impairment optimized by a computer-based, semi-automated system.

BACKGROUND: According to several studies, despite of the existence of several published guidelines for dosing adjustments based on renal function, inappropriate prescribing is a common drug-related problem in inpatient care. OBJECTIVE: We developed and implemented a system for drug dosage adjustment integrated into the Hospital computer provider order entry system. This system allows pharmacists to identify patients with reduced renal function, identify medication orders that may require dosage modifications based on renal function, and generate an alert with a recommendation of specific dosage adjustment. Using the Summary of Product Characteristics and two drug databases (Micromedex 2.0® and Lexicomp®), specific dosage guidelines for drugs used in patients with renal impairment were established. SETTING: A 264-bed tertiary teaching hospital. METHODS: We performed a quasi-experimental, one-group, pretest-posttest study to assess the efficacy of this intervention program. We compared the differences between the frequency of appropriate orders pre- and post-test using the McNemar test. MAIN OUTCOME MEASURES: the frequency of appropriate orders before the recommendation (pre-test) and after the recommendation (post-test). RESULTS: Before the intervention, the frequency of appropriate prescribing based on renal function was 65 %. After the intervention, this frequency was 86 % (p < 0.001). The interventions were more frequent in the emergency department (45 %). The program required 30-45 min of pharmacist time per day. The average number of patients reviewed daily was 28. This study found that a computer-based, semi-automated drug-dosage program for renal failure patients was able to reduce the number of inappropriate orders due to renal insufficiency.