Does the inclusion of societal costs change the economic evaluations recommendations? A systematic review for multiple sclerosis disease.

BACKGROUND: Multiple sclerosis imposes a heavy burden on the person who suffers from it and on the relatives, due to the caregiving load involved. The objective was to analyse whether the inclusion of social costs in economic evaluations of multiple sclerosis-related interventions changed results and/or conclusions. METHODS: A systematic review was launched using Medline and the Cost-Effectiveness Analysis Registry of Tufts University (2000-2019). Included studies should: (1) be an original study published in a scientific journal, (2) be an economic evaluation of any multiple sclerosis-related intervention, (3) include productivity losses and/or informal care costs (social costs), (4) be written in English, (5) use quality-adjusted life years as outcome, and (6) separate the results according to the perspective applied. RESULTS: Twenty-nine articles were selected, resulting in 67 economic evaluation estimations. Social costs were included in 47% of the studies. Productivity losses were assessed in 90% of the estimations (the human capital approach was the most frequently used method), whereas informal care costs were included in nearly two-thirds of the estimations (applying the opportunity and the replacement-cost methods equally). The inclusion of social costs modified the figures for incremental costs in 15 estimations, leading to a change in the conclusions in 10 estimations, 6 of them changing from not recommended from the healthcare perspective to implemented from the societal perspective. The inclusion of social costs also altered the results from cost-effective to dominant in five additional estimations. CONCLUSIONS: The inclusion of social costs affected the results/conclusions in multiple sclerosis-related interventions, helping to identify the most appropriate interventions for reducing its economic burden from a broader perspective.

Economic burden and health-related quality of life in tenosynovial giant-cell tumour patients in Europe: an observational disease registry.

BACKGROUND: Tenosynovial Giant-Cell Tumour (TGCT) is a benign clonal neoplastic proliferation arising from the synovium, causing a variety of symptoms and often requiring repetitive surgery. This study aims to define the economic burden-from a societal perspective-associated with TGCT patients and their health-related quality of life (HRQOL) in six European countries. METHODS: This article analyses data from a multinational, multicentre, prospective observational registry, the TGCT Observational Platform Project (TOPP), involving hospitals and tertiary sarcoma centres from six European countries (Austria, France, Germany, Italy, the Netherlands, and Spain). It includes information on TGCT patients' health-related quality of life and healthcare and non-healthcare resources used at baseline (the 12-month period prior to the patients entering the registry) and after 12 months of follow-up. RESULTS: 146 TGCT patients enrolled for the study, of which 137 fulfilled the inclusion criteria. Their mean age was 44.5 years, and 62% were female. The annual average total costs associated with TGCT were €4866 at baseline and €5160 at the 12-month follow-up visit. The annual average healthcare costs associated with TGCT were €4620 at baseline, of which 67% and 18% corresponded to surgery and medical visits, respectively. At the 12-month follow-up, the mean healthcare costs amounted to €5094, with surgery representing 70% of total costs. Loss of productivity represented, on average, 5% of the total cost at baseline and 1.3% at follow-up. The most-affected HRQOL dimensions, measured with the EQ-5D-5L instrument, were pain or discomfort, mobility, and the performance of usual activities, both at baseline and at the follow-up visit. Regarding HRQOL, patients declared a mean index score of 0.75 at baseline and 0.76 at the 12-month follow-up. CONCLUSION: The results suggest that TGCT places a heavy burden on its sufferers, which increases after one year of follow-up, mainly due to the healthcare resources required-in particular, surgical procedures. As a result, this condition has a high economic impact on healthcare budgets, while the HRQOL of TGCT patients substantially deteriorates over time.

Evaluación neuropsicológica de la demencia avanzada: ¿son de utilidad los instrumentos psicométricos de valoración cognitiva? Una revisión sistemática / Neuropsychological evaluation of advanced dementia: are cognitive assessment psychometric instruments useful? A systematic review

Introducción: Hablamos de demencia avanzada cuando todas las funciones cognitivas muestran una generalizada e importante afectación. En el transcurso de la demencia avanzada será necesario valorar la idoneidad de la utilización o no de instrumentos psicométricos. Objetivo: Determinar la utilidad de los instrumentos psicométricos de valoración cognitiva dirigidos a la evaluación neuropsicológica de la demencia avanzada. Material y métodos. Se realizó una búsqueda de estudios en las bases de datos PubMed, PsychInfo, Medline y Cochrane. Las búsquedas combinaron términos de demencia avanzada, valoración cognitiva y pruebas psicométricas. Se incluyeron 10 estudios en la investigación. Desarrollo: Los 10 estudios seleccionados muestran cómo se pueden utilizar diferentes pruebas psicométricas para la valoración cognitiva de pacientes con demencia avanzada: Severe Impairment Battery, Short version of the Severe Impairment Battery, Ordinal Scales of Psychological Development modified, Severe Cognitive Impairment Profile, Severe Minimental State Examination, Modified version of the Test for Severe Impairment y Severe Impairment Rating Scale. Cinco de los 10 estudiaron la utilidad de esas pruebas en su versión original, y los cinco restantes comprobaron la validez y la fiabilidad de las versiones abreviadas de algunas de estas pruebas. Conclusiones: Son pocas las escalas que reúnen las características psicométricas adecuadas para poder realizar una valoración cognitiva de forma eficaz, teniendo en cuenta la dificultad que ésta supone para una persona con demencia avanzada, pero los instrumentos mencionados superan las principales limitaciones de otras herramientas al presentar ausencia de efecto techo y mínimo efecto suelo.(AU)

Introduction: We speak of advanced dementia when all the cognitive functions show a generalized and important affectation. In the course of advanced dementia it will be necessary to assess the suitability of the use or not of psychometric instruments. Objective: To determine the utility of the psychometric instruments of cognitive assessment that determines the neuropsychological evaluation of advanced dementia. Through the research of studies of the databases PubMed, PsychInfo, MedLine and Cochrane. The searches combined terms of advanced dementia, cognitive evaluation and pshycometric tests. Ten studies were included in the research. Development: The ten selected studies show how different psychometric tests can be used for the cognitive evaluation of patients with advanced dementia: SIB, SIB-s, M-OSPD, SCIP, SMMSE, mTSI and SIRS. Five of the ten studied the usefulness of these tests in their original version, and the remaining five verified the validity and reliability of the abbreviated versions of some of these tests. Conclusions: There are few scales that meet the appropriate psychometric characteristics to be able to carry out a cognitive evaluation effectively taking into account the difficulty that this poses for a person with severe dementia, but the instruments mentioned overcome the main limitations of other tools by presenting no effect ceiling and minimal floor effect.(AU)

[Neuropsychological evaluation of advanced dementia: are cognitive assessment psychometric instruments useful? A systematic review]. / Evaluación neuropsicológica de la demencia avanzada: ¿son de utilidad los instrumentos psicométricos de valoración cognitiva? Una revisión sistemática.

INTRODUCTION: We speak of advanced dementia when all the cognitive functions show a generalized and important affectation. In the course of advanced dementia it will be necessary to assess the suitability of the use or not of psychometric instruments. OBJECTIVE: To determine the utility of the psychometric instruments of cognitive assessment that determines the neuropsychological evaluation of advanced dementia. Through the research of studies of the databases PubMed, PsychInfo, MedLine and Cochrane. The searches combined terms of advanced dementia, cognitive evaluation and pshycometric tests. Ten studies were included in the research. DEVELOPMENT: The ten selected studies show how different psychometric tests can be used for the cognitive evaluation of patients with advanced dementia: SIB, SIB-s, M-OSPD, SCIP, SMMSE, mTSI and SIRS. Five of the ten studied the usefulness of these tests in their original version, and the remaining five verified the validity and reliability of the abbreviated versions of some of these tests. CONCLUSIONS: There are few scales that meet the appropriate psychometric characteristics to be able to carry out a cognitive evaluation effectively taking into account the difficulty that this poses for a person with severe dementia, but the instruments mentioned overcome the main limitations of other tools by presenting no effect ceiling and minimal floor effect.

TITLE: Evaluación neuropsicológica de la demencia avanzada: ¿son de utilidad los instrumentos psicométricos de valoración cognitiva? Una revisión sistemática.Introducción. Hablamos de demencia avanzada cuando todas las funciones cognitivas muestran una generalizada e importante afectación. En el transcurso de la demencia avanzada será necesario valorar la idoneidad de la utilización o no de instrumentos psicométricos. Objetivo. Determinar la utilidad de los instrumentos psicométricos de valoración cognitiva dirigidos a la evaluación neuropsicológica de la demencia avanzada. Material y métodos. Se realizó una búsqueda de estudios en las bases de datos PubMed, PsychInfo, Medline y Cochrane. Las búsquedas combinaron términos de demencia avanzada, valoración cognitiva y pruebas psicométricas. Se incluyeron 10 estudios en la investigación. Desarrollo. Los 10 estudios seleccionados muestran cómo se pueden utilizar diferentes pruebas psicométricas para la valoración cognitiva de pacientes con demencia avanzada: Severe Impairment Battery, Short version of the Severe Impairment Battery, Ordinal Scales of Psychological Development modified, Severe Cognitive Impairment Profile, Severe Minimental State Examination, Modified version of the Test for Severe Impairment y Severe Impairment Rating Scale. Cinco de los 10 estudiaron la utilidad de esas pruebas en su versión original, y los cinco restantes comprobaron la validez y la fiabilidad de las versiones abreviadas de algunas de estas pruebas. Conclusiones. Son pocas las escalas que reúnen las características psicométricas adecuadas para poder realizar una valoración cognitiva de forma eficaz, teniendo en cuenta la dificultad que ésta supone para una persona con demencia avanzada, pero los instrumentos mencionados superan las principales limitaciones de otras herramientas al presentar ausencia de efecto techo y mínimo efecto suelo.

Multidisciplinary expert consensus on secondary fracture prevention in Spain.

The study aimed to achieve expert consensus to optimize secondary fracture prevention in Spain. Relevant gaps in current patient management were identified. However, some aspects were considered difficult to apply. Future efforts should focus on those items with greatest divergences between importance and feasibility. PURPOSE: To establish a Spanish multidisciplinary expert consensus on secondary fracture prevention. METHODS: A two-round Delphi consensus was conducted, guided by a Scientific Committee. The 43-item study questionnaire was designed from a literature review and a subsequent multidisciplinary expert group (n = 12) discussion. The first-round questionnaire, using a 7-point Likert scale, assessed the experts' opinion of the current situation, their wish for items to happen, and their prognosis that items would be implemented within 5 years. Items for which consensus was not achieved were included in the second round. Consensus was defined as ≥ 75% agreement or ≥ 75% disagreement. A total of 102 experts from 14 scientific societies were invited to participate. RESULTS: A total of 75 (response rate 73.5%) and 69 (92.0%) experts answered the first and second Delphi rounds, respectively. Participants mean age was 51.8 years [standard deviation (SD): 10.1 years]; being 24.0% rheumatologists, 21.3% primary care physicians, 14.7% geriatricians, 8.0% internal medicine specialists, 8.0% rehabilitation physicians, and 8.0% gynecologists. Consensus was achieved for 79.1% of items (wish, 100%; prognosis, 58.1%). Effective secondary prevention strategies identified as requiring improvement included: clinical report standardization, effective hospital primary care communication (telephone/mail and case managers), health-related quality of life (HRQoL) questionnaires use, and treatment compliance monitoring (prognosis agreement 33.3%, 47.8%, 18.8%, and 55.1%, respectively). CONCLUSION: A consensus was reached by health professionals in their wish to implement strategies to optimize secondary fracture prevention; however, they considered some difficult to apply. Efforts should focus on those items with currently low application and those with greatest divergence between wish and prognosis.

[Bilingualism as a factor in the protection of Alzheimer's disease: a systematic review]. / El bilingüismo como factor de protección de la enfermedad de Alzheimer: revisión sistemática.

INTRODUCTION: Alzheimer's disease (AD) is the most prevalent type of dementia today, with an incidence estimated at 30% of the population over 85 years of age, which is why it represents a health problem in today's society. AIM: To know if bilingualism can act as a protection factor for AD, thus increasing cognitive reserve. SUBJECTS AND METHODS: We searched for studies in the PsychInfo, Pubmed, Psicodoc, Medline and PubPych databases, based on the combination of various terms related to the keywords. Finally, ten studies were included. RESULTS: Seven of the ten selected studies suggest a significantly positive relationship between bilingualism and AD, although on the contrary two of the remaining studies find a partial relationship, where there is only a relationship in very specific circumstances (in the first one there is only one positive relationship when there is a low level of education, while in the second one there is only a relationship when more than two languages are spoken); only one of the studies found no significant relationship between bilingualism and AD. CONCLUSIONS: The aforementioned studies have found a delay at the time of diagnosis or at the onset of clinically significant symptoms, between 4.5 years and 7 years; thus, bilingualism could be considered a contributing factor of the cognitive reserve and as a consequence a probable protection factor to prevent or slow the onset of AD and its subsequent progression.

TITLE: El bilingüismo como factor de protección de la enfermedad de Alzheimer: revisión sistemática.Introducción. La enfermedad de Alzheimer (EA) es el tipo de demencia más prevalente en la actualidad, con una incidencia estimada del 30% de la población mayor de 85 años, lo que representa un problema de salud en la sociedad actual. Objetivo. Conocer si el bilingüismo puede actuar como factor de protección de la EA, incrementando así la reserva cognitiva. Sujetos y métodos. Se realizó la búsqueda de estudios en las bases de datos PsychInfo, Pubmed, Psicodoc, Medline y PubPsych, a partir de la combinación de diversos términos relacionados con las palabras clave. Finalmente se incluyeron 10 estudios. Resultados. Siete de los 10 estudios seleccionados sugieren una relación significativamente positiva entre bilingüismo y EA, aunque dos de los estudios restantes encuentran una relación parcial, sólo en unas circunstancias muy concretas (en uno se da una relación positiva cuando hay un nivel bajo de educación, mientras que en el otro sólo se encuentra relación cuando se hablan más de dos lenguas). Únicamente un estudio no halla una relación significativa entre bilingüismo y EA. Conclusiones. Los citados estudios encontraron un retraso en el momento del diagnóstico o en el inicio de la sintomatología clínicamente significativa de entre 4,5 y 7 años; así pues, el bilingüismo se podría considerar un factor contribuidor de la reserva cognitiva y, en consecuencia, un probable factor de protección para prevenir o retrasar la aparición de la EA y su posterior progresión.

Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma.

BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.

Reaction strategies for the enzymatic synthesis of neopentyl glycol diheptanoate.

This work describes for the first time the green synthesis of neopentyl glycol diheptanoate in a solvent-free medium via an enzymatic pathway. The process has been carried out in an open-air reactor in order to ease water removal through evaporation and shift the chemical equilibrium towards product formation. The inhibiting effect of high concentrations of heptanoic acid has been put into evidence by a reduction of initial reaction rate when esterification was performed with stoichiometric amounts of substrates. Therefore, in this work different strategies for the stepwise addition of heptanoic acid are proposed, and best results were obtained when stoichiometric quantities of acid were divided in four equal amounts and added when previous batch was consumed. Biocatalyst Novozym® 435 concentration and temperature were optimised, giving yields of 90% in neopentyl glycol diheptanoate when 7.5% (w/w) and 70 °C were used. With a remaining 7% of heptanoic acid (probably caused by the alcohol evaporation) the addition of neopentyl glycol led to a conversion of 95%. Thus, product can be used in cosmetics without further purification and can be labelled as environmentally-friendly synthesized because of its enzymatic origin.

How relevant are social costs in economic evaluations? The case of Alzheimer's disease.

BACKGROUND: The main objective of this study was to analyse how the inclusion (exclusion) of social costs can alter the results and conclusions of economic evaluations in the field of Alzheimer's disease interventions. METHODS: We designed a systematic review that included economic evaluations in Alzheimer's disease. The search strategy was launched in 2000 and ran until November 2018. The inclusion criteria were: being an original study published in a scientific journal, being an economic evaluation of any intervention related to Alzheimer's disease, including social costs (informal care costs and/or productivity losses), being written in English, using QALYs as an outcome for the incremental cost-utility analysis, and separating the results according to the perspective applied. RESULTS: It was finally included 27 studies and 55 economic evaluations. Around 11% of economic evaluations changed their main conclusions. More precisely, three of them concluded that the new intervention became cost-effective when the societal perspective was considered, whereas when using just the health care payer perspective, the new intervention did not result in a cost-utility ratio below the threshold considered. Nevertheless, the inclusion of social cost can also influence the results, as 37% of the economic evaluations included became the dominant strategy after including social costs when they were already cost-effective in the health care perspective. CONCLUSIONS: Social costs can substantially modify the results of the economic evaluations. Therefore, taking into account social costs in diseases such as Alzheimer's can be a key element in making decisions about public financing and pricing of health interventions.

Tissue deconjugation of urolithin A glucuronide to free urolithin A in systemic inflammation.

Urolithin A (Uro-A) is an anti-inflammatory and cancer chemopreventive metabolite produced by the gut microbiota from the polyphenol ellagic acid. However, in vivo conjugation of Uro-A to Uro-A glucuronide (Uro-A glur) dramatically hampers its activity. We describe here for the first time the tissue deconjugation of Uro-A glur to Uro-A after lipopolysaccharide (LPS)-induced inflammation, which could explain the systemic in vivo activity of free Uro-A in microenvironments subjected to inflammatory stimuli.

Using a stated preference discrete choice experiment to assess societal value from the perspective of decision-makers in Europe. Does it work for rare diseases?

OBJECTIVES: To pilot the feasibility of using a discrete choice experiment (DCE) design to investigate individual preferences from the decision-maker perspective regarding the use of public funding for orphan drugs and generate prior information for future experimental designs. METHODS: A DCE was used on a convenience sample of participants from five European countries (England, France, Germany, Italy and Spain), exploring their preferences in distinct healthcare scenarios involving orphan drugs. A preliminary review of the empirical literature on distributive preferences informed the selection of attributes and their levels in the design. An online questionnaire was used to conduct the DCE survey. RESULTS: A total of 199 questionnaires were completed. The five country model showed relative preference for some attributes over others: cost of treatment, improvement in health, value for money and availability of treatment alternatives received the greatest attention. However, disease severity, beginning of life, waiting times and side effects were also shown to be important social values that should not be ignored. CONCLUSIONS: The findings presented in this study provide insight about the preferences that can influence decisions on orphan drugs in different countries. This study also provides valuable prior information that could inform future DCE designs in this area.

Buckwheat and buckwheat enriched products exert an anti-inflammatory effect on the myofibroblasts of colon CCD-18Co.

Buckwheat (BW) constitutes a good source of bioactive components that show anti-inflammatory effects in vitro and in vivo. The use of functional foods in the prevention and treatment of inflammatory bowel diseases (IBDs) has aroused increasing interest. This study investigates the effect of in vitro digested BW and BW-enriched products (BW-enriched wheat breads, roasted BW groats -fermented and non-fermented-, and BW sprouts) on colon myofibroblasts, the cells involved in the regulation of inflammatory response in the intestine. The cells were treated with different digested-BW products, alone or together with TNF-α (20 ng mL-1), and the effects on the cell migration, mitochondrial membrane potential and cell cycle, processes altered during intestinal inflammation, were investigated. A significant reduction in TNF-α-induced migration (25.5%, p < 0.05) and attenuation of the TNF-α-altered cell cycle (p < 0.05) was observed in myofibroblasts treated with BW-enriched white wheat bread. These results contribute to extend the beneficial effects derived from BW bioactive compounds, and suggest that BW consumption can exert beneficial effects on IBDs.

First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.

Two novel variants of the ABCG5 gene cause xanthelasmas and macrothrombocytopenia: a brief review of hematologic abnormalities of sitosterolemia.

Essentials Diagnosis of sitosterolemia, a rare recessive or syndromic disorder, is usually delayed. Peripheral blood smear is extremely useful for establishing the suspicion of sitosterolemia. High-throughput sequencing technology enables the molecular diagnosis of inherited thrombocytopenias. Accurate characterization of sitosterolemia helps us determine appropriate management. SUMMARY: Background Sitosterolemia (STSL) is a recessive inherited disorder caused by pathogenic variants in the ABCG5 and ABCG8 genes. Increased levels of plasma plant sterols (PSs) usually result in xanthomas and premature coronary atherosclerosis, although hematologic abnormalities may occasionally be present. This clinical picture is unfamiliar to many physicians, and patients may be at high risk of misdiagnosis. Objectives To report two novel ABCG5 variants causing STSL in a Spanish patient, and review the clinical and mutational landscape of STSL. Patient/Methods A 46-year-old female was referred to us with lifelong macrothrombocytopenia. She showed familial hypercholesterolemia-related xanthomas. Molecular analysis was performed with high-throughput sequencing. Plasma PS levels were evaluated with gas-liquid chromatography. The STSL landscape was reviewed with respect to specific online databases and all reports published since 1974. Results A blood smear revealed giant platelets and stomatocytes. Novel compound heterozygous variants were detected in exons 7 (c.914C>G) and 13 (c.1890delT) of ABCG5. The patient showed an increased plasma level of sitosterol. These findings support the diagnosis of STSL. In our review, we identified only 25 unrelated STLS patients who presented with hematologic abnormalities including macrothrombocytopenia. It remains unknown why only some patients develop hematologic abnormalities. Conclusions This is the first Spanish STSL patient to be reported and molecularly characterized. The early diagnosis of STLS is strongly supported by the presence of stomatocytes in blood smears. The definitive diagnosis of STSL by measurement of serum PS levels and molecular analyses prompted the use of ezetimibe therapy.

Calidad de vida relacionada con la salud en pacientes con ataxias espinocerebelosas / Health-related quality of life in patients with spinocerebellar ataxia

Introducción. El deterioro progresivo de los pacientes con ataxias espinocerebelosas (AEC) genera un gran impacto sobre su calidad de vida relacionada con la salud (CVRS). En este estudio se evalúa la CVRS en una muestra de pacientes diagnosticados de AEC y se pretende estimar la capacidad predictiva de un conjunto de variables sociodemográficas en las distintas dimensiones que conforman el cuestionario. Métodos. Se evaluó a un total de 80 pacientes diagnosticados de AEC, a través de un cuestionario sociodemográfico y del cuestionario genérico de salud SF-36. Se estudiaron variables sociodemográficas como sexo, edad, existencia de cuidador, situación laboral y tiempo desde el diagnóstico de la enfermedad. Resultados. Las dimensiones que componen el SF-36 muestran correlaciones positivas y significativas entre ellas. Las puntuaciones medias obtenidas en cada una de las dimensiones difieren según el sexo, siendo significativa la diferencia solo en la dimensión salud general, con media más alta en los hombres. En relación con la edad, se encuentran diferencias significativas en las dimensiones vitalidad y función social, con puntuaciones mayores en el rango de menor edad (menores de 34 años). La variable que explica la mayor parte de la varianza del cuestionario es la existencia de cuidador. Conclusiones. El Cuestionario de Salud SF-36 es un instrumento válido y fiable para medir la CVRS en pacientes con diagnóstico de AEC. Asimismo, la variable existencia de cuidador parece determinar la calidad de vida autopercibida por estos pacientes (AU)

Introduction. The progressive deterioration of patients with spinocerebellar ataxia (SCA) has a major impact on their health-related quality of life (HRQOL). This study evaluates HRQOL in a sample of patients diagnosed with SCA and aims to estimate the predictive ability of a set of sociodemographic variables for the different dimensions of the General Health Questionnaire. Methods. A total of 80 patients diagnosed with SCA were assessed using a sociodemographic questionnaire and the SF-36 General Health Questionnaire. The sociodemographic variables studied were sex, age, presence of a carer, employment status, and time elapsed from diagnosis of the disease. Results. The 8 subscales of the SF-36 show positive and significant correlations to one another. Mean scores obtained on each SF-36 subscale differ between women and men, although this difference is significant only on the general health subscale, with men scoring higher than women. We found significant age differences on the vitality and social function subscales, with higher scores among younger patients (< 34 years). The variable ‘presence of a carer’ accounts for most of the total variance of the questionnaire. Conclusions. The SF-36 is a valid and useful instrument for evaluating HRQOL in patients diagnosed with SCA. Presence of a carer seems to be a determinant of self-perceived quality of life in these patients (AU)

Health-related quality of life in patients with spinocerebellar ataxia. / Calidad de vida relacionada con la salud en pacientes con ataxias espinocerebelosas.

INTRODUCTION: The progressive deterioration of patients with spinocerebellar ataxia (SCA) has a major impact on their health-related quality of life (HRQOL). This study evaluates HRQOL in a sample of patients diagnosed with SCA and aims to estimate the predictive ability of a set of sociodemographic variables for the different dimensions of the General Health Questionnaire. METHODS: A total of 80 patients diagnosed with SCA were assessed using a sociodemographic questionnaire and the SF-36 General Health Questionnaire. The sociodemographic variables studied were sex, age, presence of a carer, employment status, and time elapsed from diagnosis of the disease. RESULTS: The 8 subscales of the SF-36 show positive and significant correlations to one another. Mean scores obtained on each SF-36 subscale differ between women and men, although this difference is significant only on the general health subscale, with men scoring higher than women. We found significant age differences on the vitality and social function subscales, with higher scores among younger patients (< 34 years). The variable 'presence of a carer' accounts for most of the total variance of the questionnaire. CONCLUSIONS: The SF-36 is a valid and useful instrument for evaluating HRQOL in patients diagnosed with SCA. Presence of a carer seems to be a determinant of self-perceived quality of life in these patients.