Supervivencia de la ciclosporina en el tratamiento de la dermatitis atópica moderada-grave: Registro Español de Dermatitis Atópica (BIOBADATOP) / Drug Survival in Cyclosporine Treatment for Moderate to Severe Atopic Dermatitis: Analysis of the Spanish Atopic Dermatitis Registry (BIOBADATOP)

Antecedentes: Debido a la eclosión en el último quinquenio de nuevas alternativas terapéuticas para la dermatitis atópica (DA), nos planteamos estudiar la supervivencia actual de la ciclosporina (CsA) en esta patología. La CsA, como paso necesario solicitado por el Sistema Nacional de Salud de España para la autorización de otros tratamientos sistémicos, podría presentar una supervivencia menor que en otras enfermedades. Material y método: Estudio multicéntrico, observacional, de cohortes prospectivo para el que se recogieron pacientes incluidos en el Registro Español de Dermatitis Atópica (BIOBADATOP). Como cohorte de comparación se emplearon los datos del Registro Español de tratamientos sistémicos en Psoriasis (BIOBADADERM). Resultados: Se incluyeron 130 pacientes diagnosticados de DA que habían recibido CsA (mediana de supervivencia de CsA: 1 año). En el grupo comparador se incluyeron 150 pacientes psoriásicos que habían recibido CsA (mediana de supervivencia: 0,37 años). Observamos una mayor supervivencia de la CsA en los pacientes con DA en comparación con los pacientes psoriásicos (p<0,001). Conclusión: La supervivencia de la CsA en BIOBADATOP es similar a la descrita en otras series de pacientes con DA, y superior a la observada en los pacientes con psoriasis en el registro BIOBADADERM.(AU)

Background: The past 5 years have seen a proliferation of new treatments for atopic dermatitis (AD). We analyzed recent drug survival data for cyclosporine in this setting. Because the Spanish National Healthcare system requires patients with AD to be treated with cyclosporine before they can be prescribed other systemic treatments, drug survival for cyclosporine may be shorter than in other diseases. Material and method: Multicenter, observational, prospective cohort study using data from the Spanish Atopic Dermatitis Registry (BIOBADATOP). Data from the Spanish Registry of Systemic Treatments in Psoriasis (BIOBADADERM) were used to create a comparison cohort. Results: We analyzed data for 130 patients with AD treated with cyclosporine (median drug survival, 1 year). Median cyclosporine survival in the psoriasis comparison group (150 patients) was 0.37 years. Drug survival was significantly longer in AD than in psoriasis (P<.001). Conclusion: Drug survival of cyclosporine in the BIOBADATOP registry is similar to that described in other series of patients with AD and longer than that observed in the BIOBADADERM psoriasis registry.(AU)

[Translated article] Drug Survival in Cyclosporine Treatment for Moderate to Severe Atopic Dermatitis: Analysis of the Spanish Atopic Dermatitis Registry (BIOBADATOP) / Supervivencia de la ciclosporina en el tratamiento de la dermatitis atópica moderada-grave: Registro Español de Dermatitis Atópica (BIOBADATOP)

Antecedentes: Debido a la eclosión en el último quinquenio de nuevas alternativas terapéuticas para la dermatitis atópica (DA), nos planteamos estudiar la supervivencia actual de la ciclosporina (CsA) en esta patología. La CsA, como paso necesario solicitado por el Sistema Nacional de Salud de España para la autorización de otros tratamientos sistémicos, podría presentar una supervivencia menor que en otras enfermedades. Material y método: Estudio multicéntrico, observacional, de cohortes prospectivo para el que se recogieron pacientes incluidos en el Registro Español de Dermatitis Atópica (BIOBADATOP). Como cohorte de comparación se emplearon los datos del Registro Español de tratamientos sistémicos en Psoriasis (BIOBADADERM). Resultados: Se incluyeron 130 pacientes diagnosticados de DA que habían recibido CsA (mediana de supervivencia de CsA: 1 año). En el grupo comparador se incluyeron 150 pacientes psoriásicos que habían recibido CsA (mediana de supervivencia: 0,37 años). Observamos una mayor supervivencia de la CsA en los pacientes con DA en comparación con los pacientes psoriásicos (p<0,001). Conclusión: La supervivencia de la CsA en BIOBADATOP es similar a la descrita en otras series de pacientes con DA, y superior a la observada en los pacientes con psoriasis en el registro BIOBADADERM.(AU)

Background: The past 5 years have seen a proliferation of new treatments for atopic dermatitis (AD). We analyzed recent drug survival data for cyclosporine in this setting. Because the Spanish National Healthcare system requires patients with AD to be treated with cyclosporine before they can be prescribed other systemic treatments, drug survival for cyclosporine may be shorter than in other diseases. Material and method: Multicenter, observational, prospective cohort study using data from the Spanish Atopic Dermatitis Registry (BIOBADATOP). Data from the Spanish Registry of Systemic Treatments in Psoriasis (BIOBADADERM) were used to create a comparison cohort. Results: We analyzed data for 130 patients with AD treated with cyclosporine (median drug survival, 1 year). Median cyclosporine survival in the psoriasis comparison group (150 patients) was 0.37 years. Drug survival was significantly longer in AD than in psoriasis (P<.001). Conclusion: Drug survival of cyclosporine in the BIOBADATOP registry is similar to that described in other series of patients with AD and longer than that observed in the BIOBADADERM psoriasis registry.(AU)

[Effects of a smoke-free policy on healthcare staff attitudes and aggression in psychiatry]. / Effecten van een rookvrijbeleid op attitudes zorgpersoneel en agressie in de psychiatrie.

Background The prevalence of smoking among patients with psychiatric disorders is 3-4 times higher than the general population. However, smoking is still permitted in many psychiatric clinics. The National Prevention Agreement (2018) mandates that all psychiatric wards be smoke-free by 2025. The UMC Utrecht clinics have been smoke-free since November 2020. Aim To examine healthcare workers’ attitudes before and after implementing the smoke-free policy. Method In an observational study with quantitative data analysis, data were collected in one center from healthcare workers in psychiatry departments with surveys. We collected demographic information, smoking status, attitudes towards the smoke-free policy, and its impact on patients and care. Incidents of aggression were prospectively recorded and reported in the MAP (aggression incidents in patient care). Results Out of 172 healthcare workers invited to participate, 30% (n = 52) completed the pre-implementation survey, and 20% (n = 34) completed the post-implementation survey. Prior to implementation, 62% (n = 32/52) of healthcare workers had a positive attitude towards the smoke-free policy, which increased to 77% (n = 26/34) post-implementation. Expectations of increased aggression incidents were reported by 62% (n = 32/52) during the pre-implementation phase. The number of aggression incidents was 46 in the one-year period before implementation (November 2019 – February 2020) and 45 incidents after implementation (November 2020 – February 2021). Conclusion This study supports the implementation of a smoke-free policy in psychiatric clinics due to the lack of a significant increase in aggression incidents. Healthcare workers perceived this outcome and observed quicker granting of ‘green’ freedoms.

[Translated article] Drug Survival in Cyclosporine Treatment for Moderate to Severe Atopic Dermatitis: Analysis of the Spanish Atopic Dermatitis Registry (BIOBADATOP).

BACKGROUND: The past 5 years have seen a proliferation of new treatments for atopic dermatitis (AD). We analyzed recent drug survival data for cyclosporine in this setting. Because the Spanish National Healthcare system requires patients with AD to be treated with cyclosporine before they can be prescribed other systemic treatments, drug survival for cyclosporine may be shorter than in other diseases. MATERIAL AND METHOD: Multicenter, observational, prospective cohort study using data from the Spanish Atopic Dermatitis Registry (BIOBADATOP). Data from the Spanish Registry of Systemic Treatments in Psoriasis (BIOBADADERM) were used to create a comparison cohort. RESULTS: We analyzed data for 130 patients with AD treated with cyclosporine (median drug survival, 1 year). Median cyclosporine survival in the psoriasis comparison group (150 patients) was 0.37 years. Drug survival was significantly longer in AD than in psoriasis (P<.001). CONCLUSION: Drug survival of cyclosporine in the BIOBADATOP registry is similar to that described in other series of patients with AD and longer than that observed in the BIOBADADERM psoriasis registry.

Drug Survival in Cyclosporine Treatment for Moderate to Severe Atopic Dermatitis: Analysis of the Spanish Atopic Dermatitis Registry (BIOBADATOP). / Supervivencia de la ciclosporina en el tratamiento de la dermatitis atópica moderada-grave: Registro Español de Dermatitis Atópica (BIOBADATOP).

BACKGROUND: The past 5 years have seen a proliferation of new treatments for atopic dermatitis (AD). We analyzed recent drug survival data for cyclosporine in this setting. Because the Spanish National Healthcare system requires patients with AD to be treated with cyclosporine before they can be prescribed other systemic treatments, drug survival for cyclosporine may be shorter than in other diseases. MATERIAL AND METHOD: Multicenter, observational, prospective cohort study using data from the Spanish Atopic Dermatitis Registry (BIOBADATOP). Data from the Spanish Registry of Systemic Treatments in Psoriasis (BIOBADADERM) were used to create a comparison cohort. RESULTS: We analyzed data for 130 patients with AD treated with cyclosporine (median drug survival, 1 year). Median cyclosporine survival in the psoriasis comparison group (150 patients) was 0.37 years. Drug survival was significantly longer in AD than in psoriasis (P<.001). CONCLUSION: Drug survival of cyclosporine in the BIOBADATOP registry is similar to that described in other series of patients with AD and longer than that observed in the BIOBADADERM psoriasis registry.

Acné fulminans: revisión narrativa de la literatura / Acne Fulminans: A Narrative Review

El acné fulminans (AF) es una forma rara y grave de acné inflamatorio. La intensidad de las lesiones cutáneas y las cicatrices secundarias impactan negativamente en la calidad de vida. Presentamos una revisión narrativa de casos de AF publicados en Medline. Se incluyeron artículos escritos en lengua castellana o inglesa, que reportasen casos aislados o series de casos de AF, de los que se recogieron variables clínico-demográficas. El objetivo principal de la revisión fue describir las características clínico-demográficas del AF. Secundariamente se pretendió determinar si la localización o la extensión lesional repercuten en la calidad de vida. Se revisaron 212 casos de AF incluidos en 91 artículos (edad media: 16,6 años, 91,94% varones). El 97,63% de los pacientes presentaron acné previo al brote de AF y el 54,90% contaba con antecedentes familiares de acné. En el 44,79% existió un factor desencadenante de AF (98,63% farmacológico: 65,28% isotretinoína). Las localizaciones más habitualmente afectas fueron cara (89,31%), tronco posterior (77,86%) y anterior (74,81%). El subtipo predominante fue el AF asociado a síntomas sistémicos (59,12%). Entre los síntomas sistémicos destacó la sintomatología general (97,06%). El tratamiento más prevalente fue la corticoterapia sistémica (81,03%). La repercusión del AF en la calidad de vida se reportó en 2 pacientes. Como conclusiones, el AF predomina en los varones adolescentes con antecedentes de acné vulgar, afectando fundamentalmente a la cara y el tronco. La forma más frecuente es el AF con síntomas sistémicos. Para su tratamiento, los corticoides sistémicos son los fármacos más frecuentemente empleados. La repercusión del AF en la calidad de vida está infrarreportada (AU)

Acne fulminans is a severe and rare form of inflammatory acne. Lesion severity and subsequent scarring has a negative impact on the patient's quality of life. We conducted a narrative review of the literature on acne fulminans based on a search for relevant English- and Spanish-language articles published in Medline. We included case reports and case series. The main aim was to describe the clinical and demographic characteristics of patients with acne fulminans. A secondary aim was to determine whether quality of life was affected by the site or extent of lesions. We reviewed 91 articles describing 212 cases of acne fulminans. The mean age of the patients (91.94% male) was 16.6 years. A personal and family history of acne vulgaris was reported for 97.63% and 54.90% of patients, respectively. A trigger was identified in 44.79% of cases. The main cause was pharmacologic (96.63%) and the main drug isotretinoin (65.28%). The sites most often affected were the face (89.31%), the posterior trunk (77.86%), and the anterior trunk (74.81%). The predominant disease subtype was acne fulminans with systemic symptoms (59.12%), which were mostly general (97.06%). Systemic corticosteroids were the most widely used treatment (81.03%). The impact of the disease on quality of life was reported for 2 patients. In conclusion, acne fulminans mainly affects the face and trunk of male adolescents with a history of acne vulgaris. The main subtype was acne fulminans with systemic symptoms, and most patients were treated with systemic corticosteroids. The effect of acne fulminans on quality of life is underreported (AU)

[Translated article] Acne Fulminans: A Narrative Review / Acné fulminans: revisión narrativa de la literatura

Acne fulminans is a severe and rare form of inflammatory acne. Lesion severity and subsequent scarring has a negative impact on the patient's quality of life. We conducted a narrative review of the literature on acne fulminans based on a search for relevant English- and Spanish-language articles published in Medline. We included case reports and case series. The main aim was to describe the clinical and demographic characteristics of patients with acne fulminans. A secondary aim was to determine whether quality of life was affected by the site or extent of lesions. We reviewed 91 articles describing 212 cases of acne fulminans. The mean age of the patients (91.94% male) was 16.6 years. A personal and family history of acne vulgaris was reported for 97.63% and 54.90% of patients, respectively. A trigger was identified in 44.79% of cases. The main cause was pharmacologic (96.63%) and the main drug isotretinoin (65.28%). The sites most often affected were the face (89.31%), the posterior trunk (77.86%), and the anterior trunk (74.81%). The predominant disease subtype was acne fulminans with systemic symptoms (59.12%), which were mostly general (97.06%). Systemic corticosteroids were the most widely used treatment (81.03%). The impact of the disease on quality of life was reported for 2 patients. In conclusion, acne fulminans mainly affects the face and trunk of male adolescents with a history of acne vulgaris. The main subtype was acne fulminans with systemic symptoms, and most patients were treated with systemic corticosteroids. The effect of acne fulminans on quality of life is underreported (AU)

El acné fulminans (AF) es una forma rara y grave de acné inflamatorio. La intensidad de las lesiones cutáneas y las cicatrices secundarias impactan negativamente en la calidad de vida. Presentamos una revisión narrativa de casos de AF publicados en Medline. Se incluyeron artículos escritos en lengua castellana o inglesa, que reportasen casos aislados o series de casos de AF, de los que se recogieron variables clínico-demográficas. El objetivo principal de la revisión fue describir las características clínico-demográficas del AF. Secundariamente se pretendió determinar si la localización o la extensión lesional repercuten en la calidad de vida. Se revisaron 212 casos de AF incluidos en 91 artículos (edad media: 16,6 años, 91,94% varones). El 97,63% de los pacientes presentaron acné previo al brote de AF y el 54,90% contaba con antecedentes familiares de acné. En el 44,79% existió un factor desencadenante de AF (98,63% farmacológico: 65,28% isotretinoína). Las localizaciones más habitualmente afectas fueron cara (89,31%), tronco posterior (77,86%) y anterior (74,81%). El subtipo predominante fue el AF asociado a síntomas sistémicos (59,12%). Entre los síntomas sistémicos destacó la sintomatología general (97,06%). El tratamiento más prevalente fue la corticoterapia sistémica (81,03%). La repercusión del AF en la calidad de vida se reportó en 2 pacientes. Como conclusiones, el AF predomina en los varones adolescentes con antecedentes de acné vulgar, afectando fundamentalmente a la cara y el tronco. La forma más frecuente es el AF con síntomas sistémicos. Para su tratamiento, los corticoides sistémicos son los fármacos más frecuentemente empleados. La repercusión del AF en la calidad de vida está infrarreportada (AU)

[Translated article] Acne Fulminans: A Narrative Review.

Acne fulminans is a severe and rare form of inflammatory acne. Lesion severity and subsequent scarring has a negative impact on the patient's quality of life. We conducted a narrative review of the literature on acne fulminans based on a search for relevant English- and Spanish-language articles published in Medline. We included case reports and case series. The main aim was to describe the clinical and demographic characteristics of patients with acne fulminans. A secondary aim was to determine whether quality of life was affected by the site or extent of lesions. We reviewed 91 articles describing 212 cases of acne fulminans. The mean age of the patients (91.94% male) was 16.6 years. A personal and family history of acne vulgaris was reported for 97.63% and 54.90% of patients, respectively. A trigger was identified in 44.79% of cases. The main cause was pharmacologic (96.63%) and the main drug isotretinoin (65.28%). The sites most often affected were the face (89.31%), the posterior trunk (77.86%), and the anterior trunk (74.81%). The predominant disease subtype was acne fulminans with systemic symptoms (59.12%), which were mostly general (97.06%). Systemic corticosteroids were the most widely used treatment (81.03%). The impact of the disease on quality of life was reported for two patients. In conclusion, acne fulminans mainly affects the face and trunk of male adolescents with a history of acne vulgaris. The main subtype was acne fulminans with systemic symptoms, and most patients were treated with systemic corticosteroids. The effect of acne fulminans on quality of life is under-reported.

Acne Fulminans: A Narrative Review. / Acné fulminans: revisión narrativa de la literatura.

Acne fulminans is a severe and rare form of inflammatory acne. Lesion severity and subsequent scarring has a negative impact on the patient's quality of life. We conducted a narrative review of the literature on acne fulminans based on a search for relevant English- and Spanish-language articles published in Medline. We included case reports and case series. The main aim was to describe the clinical and demographic characteristics of patients with acne fulminans. A secondary aim was to determine whether quality of life was affected by the site or extent of lesions. We reviewed 91 articles describing 212 cases of acne fulminans. The mean age of the patients (91.94% male) was 16.6 years. A personal and family history of acne vulgaris was reported for 97.63% and 54.90% of patients, respectively. A trigger was identified in 44.79% of cases. The main cause was pharmacologic (96.63%) and the main drug isotretinoin (65.28%). The sites most often affected were the face (89.31%), the posterior trunk (77.86%), and the anterior trunk (74.81%). The predominant disease subtype was acne fulminans with systemic symptoms (59.12%), which were mostly general (97.06%). Systemic corticosteroids were the most widely used treatment (81.03%). The impact of the disease on quality of life was reported for 2 patients. In conclusion, acne fulminans mainly affects the face and trunk of male adolescents with a history of acne vulgaris. The main subtype was acne fulminans with systemic symptoms, and most patients were treated with systemic corticosteroids. The effect of acne fulminans on quality of life is underreported.

BIOBADATOP Spanish Atopic Dermatitis Registry: Description and Early Findings. / BIOBADATOP: Registro Español de Dermatitis Atópica. Descripción y primeros resultados.

BACKGROUND: In recent years, remarkable improvements in our understanding of atopic dermatitis (AD) have revolutionized treatment perspectives, but access to reliable data from clinical practice is essential. MATERIALS AND METHOD: The Spanish Atopic Dermatitis Registry, BIOBADATOP, is a prospective, multicenter database that collects information on patients of all ages with AD requiring systemic therapy with conventional or novel drugs. We analyzed the registry to describe patient characteristics, diagnoses, treatments, and adverse events (AEs). RESULTS: We studied data entries for 258 patients who had received 347 systemic treatments for AD. Treatment was discontinued in 29.4% of cases, mostly due to a lack of effectiveness (in 10.7% of cases). A total of 132 AEs were described during follow-up. Eighty-six AEs (65%) were linked to a systemic treatment, most commonly dupilumab (39AEs) and cyclosporine (38AEs). The most common AEs were conjunctivitis (11patients), headache (6), hypertrichosis (5), and nausea (4). There was 1severe AE (acute mastoiditis) associated with cyclosporine. CONCLUSIONS: Initial findings on AEs from the Spanish BIOBADATOP registry are limited by short follow-up times precluding comparisons or calculation of crude and adjusted incidence rates. At the time of our analysis, no severe AEs had been reported for novel systemic therapies. BIOBADATOP will help answer questions on the effectiveness and safety of conventional and novel systemic therapies in AD.

Longitudinal associations between alcohol use, smoking, genetic risk scoring and symptoms of depression in the general population: a prospective 6-year cohort study.

BACKGROUND: Alcohol consumption, smoking and mood disorders are leading contributors to the global burden of disease and are highly comorbid. Yet, their interrelationships have remained elusive. The aim of this study was to examine the multi-cross-sectional and longitudinal associations between (change in) smoking and alcohol use and (change in) number of depressive symptoms. METHODS: In this prospective, longitudinal study, 6646 adults from the general population were included with follow-up measurements after 3 and 6 years. Linear mixed-effects models were used to test multi-cross-sectional and longitudinal associations, with smoking behaviour, alcohol use and genetic risk scores for smoking and alcohol use as independent variables and depressive symptoms as dependent variables. RESULTS: In the multi-cross-sectional analysis, smoking status and number of cigarettes per day were positively associated with depressive symptoms (p < 0.001). Moderate drinking was associated with less symptoms of depression compared to non-use (p = 0.011). Longitudinally, decreases in the numbers of cigarettes per day and alcoholic drinks per week as well as alcohol cessation were associated with a reduction of depressive symptoms (p = 0.001-0.028). Results of genetic risk score analyses aligned with these findings. CONCLUSIONS: While cross-sectionally smoking and moderate alcohol use show opposing associations with depressive symptoms, decreases in smoking behaviour as well as alcohol consumption are associated with improvements in depressive symptoms over time. Although we cannot infer causality, these results open avenues to further investigate interventions targeting smoking and alcohol behaviours in people suffering from depressive symptoms.

Formulación magistral en pacientes con genodermatosis. Impacto en la calidad de vida: un estudio transversal / Effect of Drug Compounding on Quality of Life in Patients With Genodermatoses: A Cross-Sectional Study

Introducción El abordaje terapéutico de las manifestaciones cutáneas de las enfermedades raras es complejo. El objetivo principal de este trabajo consistió en determinar el impacto de la formulación magistral de dispensación hospitalaria en la calidad de vida de los pacientes con genodermatosis. Material y métodos Se diseñó un estudio descriptivo transversal. Se incluyeron pacientes con genodermatosis que recibieron tratamientos tópicos elaborados y dispensados por el Servicio de Farmacia Hospitalaria del Complejo Hospitalario Universitario de Pontevedra. Se recogieron datos demográficos, cuestionarios generales y específicos sobre la calidad de vida, y cuestionarios que evaluaban los tratamientos administrados y la adherencia terapéutica. Resultados Se incluyeron 9 pacientes. Se observó que, tras la terapia con fórmulas magistrales, hubo una reducción estadísticamente significativa del impacto en la calidad de vida de los pacientes. La satisfacción con los productos fue 2,8 sobre 25 (siendo 0 la mejor puntuación). La adherencia terapéutica superó el 89%. Conclusiones La formulación magistral permite el acceso a medicamentos huérfanos y no comercializados para numerosas enfermedades raras. Su impacto en la calidad de vida de los pacientes afectos de estas enfermedades ha sido escasamente estudiado. En la serie de pacientes que se presenta, la elaboración y dispensación hospitalaria de fórmulas magistrales específicas conllevó efectos positivos en su calidad de vida. Este estudio inicial ha derivado en otro trabajo multicéntrico, centrado en las ictiosis, donde previsiblemente aumentará el número de pacientes a incluir y permitirá confirmar nuestros resultados (AU)

Background Cutaneous manifestations are complicated to treat in rare diseases. The main aim of this study was to analyze the impact of compounded drugs prepared by hospital pharmacists on the quality of life of patients with genodermatoses. Material and methods We undertook a cross-sectional study of patients with genodermatoses treated with topical medications compounded and dispensed by the pharmacy at Complejo Hospitalario Universitario in Pontevedra, Spain. We collected demographic data and answers to questionnaires examining generic and disease-specific quality of life, treatment satisfaction, and treatment adherence.Results Nine patients were included. We observed a significant improvement in health-related quality of life following treatment with compounded drugs. Satisfaction with the topical medications was 2.8 on a scale of 0 (greatest satisfaction) to 25. Treatment adherence was 59%. Conclusions Drug compounding facilitates access to orphan drugs that are not available for many rare diseases. Few studies, however, have analyzed impact on quality of life in this setting. In this series of patients with genodermatoses, topical medications compounded and dispensed by a hospital pharmacy improved health-related quality of life. This preliminary study has given rise to a multicenter study of compounding for ichthyosis. We expect that analysis of a larger sample will confirm our findings (AU)

[Translated article] Effect of Drug Compounding on Quality of Life in Patients With Genodermatoses: A Cross-Sectional Study / Formulación magistral en pacientes con genodermatosis. Impacto en la calidad de vida: un estudio transversal

Background Cutaneous manifestations are complicated to treat in rare diseases. The main aim of this study was to analyze the impact of compounded drugs prepared by hospital pharmacists on the quality of life of patients with genodermatoses. Material and methods We undertook a cross-sectional study of patients with genodermatoses treated with topical medications compounded and dispensed by the pharmacy at Complejo Hospitalario Universitario in Pontevedra, Spain. We collected demographic data and answers to questionnaires examining generic and disease-specific quality of life, treatment satisfaction, and treatment adherence.Results Nine patients were included. We observed a significant improvement in health-related quality of life following treatment with compounded drugs. Satisfaction with the topical medications was 2.8 on a scale of 0 (greatest satisfaction) to 25. Treatment adherence was 59%. Conclusions Drug compounding facilitates access to orphan drugs that are not available for many rare diseases. Few studies, however, have analyzed impact on quality of life in this setting. In this series of patients with genodermatoses, topical medications compounded and dispensed by a hospital pharmacy improved health-related quality of life. This preliminary study has given rise to a multicenter study of compounding for ichthyosis. We expect that analysis of a larger sample will confirm our findings (AU)

Introducción El abordaje terapéutico de las manifestaciones cutáneas de las enfermedades raras es complejo. El objetivo principal de este trabajo consistió en determinar el impacto de la formulación magistral de dispensación hospitalaria en la calidad de vida de los pacientes con genodermatosis. Material y métodos Se diseñó un estudio descriptivo transversal. Se incluyeron pacientes con genodermatosis que recibieron tratamientos tópicos elaborados y dispensados por el Servicio de Farmacia Hospitalaria del Complejo Hospitalario Universitario de Pontevedra. Se recogieron datos demográficos, cuestionarios generales y específicos sobre la calidad de vida, y cuestionarios que evaluaban los tratamientos administrados y la adherencia terapéutica. Resultados Se incluyeron 9 pacientes. Se observó que, tras la terapia con fórmulas magistrales, hubo una reducción estadísticamente significativa del impacto en la calidad de vida de los pacientes. La satisfacción con los productos fue 2,8 sobre 25 (siendo 0 la mejor puntuación). La adherencia terapéutica superó el 89%. Conclusiones La formulación magistral permite el acceso a medicamentos huérfanos y no comercializados para numerosas enfermedades raras. Su impacto en la calidad de vida de los pacientes afectos de estas enfermedades ha sido escasamente estudiado. En la serie de pacientes que se presenta, la elaboración y dispensación hospitalaria de fórmulas magistrales específicas conllevó efectos positivos en su calidad de vida. Este estudio inicial ha derivado en otro trabajo multicéntrico, centrado en las ictiosis, donde previsiblemente aumentará el número de pacientes a incluir y permitirá confirmar nuestros resultados (AU)

Effect of Drug Compounding on Quality of Life in Patients With Genodermatoses: A Cross-Sectional Study. / Formulación magistral en pacientes con genodermatosis. Impacto en la calidad de vida: un estudio transversal.

BACKGROUND: Cutaneous manifestations are complicated to treat in rare diseases. The main aim of this study was to analyze the impact of compounded drugs prepared by hospital pharmacists on the quality of life of patients with genodermatoses. MATERIAL AND METHODS: We undertook a cross-sectional study of patients with genodermatoses treated with topical medications compounded and dispensed by the pharmacy at Complejo Hospitalario Universitario in Pontevedra, Spain. We collected demographic data and answers to questionnaires examining generic and disease-specific quality of life, treatment satisfaction, and treatment adherence. RESULTS: Nine patients were included. We observed a significant improvement in health-related quality of life following treatment with compounded drugs. Satisfaction with the topical medications was 2.8 on a scale of 0 (greatest satisfaction) to 25. Treatment adherence was 59%. CONCLUSIONS: Drug compounding facilitates access to orphan drugs that are not available for many rare diseases. Few studies, however, have analyzed impact on quality of life in this setting. In this series of patients with genodermatoses, topical medications compounded and dispensed by a hospital pharmacy improved health-related quality of life. This preliminary study has given rise to a multicenter study of compounding for ichthyosis. We expect that analysis of a larger sample will confirm our findings.

Intraoperative crystalloid utilization variability and association with postoperative outcomes: A post hoc analysis of two multicenter prospective cohort studies.

BACKGROUND: The optimal regimen for intravenous administration of intraoperative fluids remains unclear. Our goal was to analyze intraoperative crystalloid volume administration practices and their association with postoperative outcomes. METHODS: We extracted clinical data from two multicenter observational studies including adult patients undergoing colorectal surgery and total hip (THA) and knee arthroplasty (TKA). We analyzed the distribution of intraoperative fluid administration. Regression was performed using a general linear model to determine factors predictive of fluid administration. Patient outcomes and intraoperative crystalloid utilization were summarized for each surgical cohort. Regression models were developed to evaluate associations of high or low intraoperative crystalloid with the likelihood of increased postoperative complications, mainly acute kidney injury (AKI) and hospital length of stay (LOS). RESULTS: 7580 patients were included. The average adjusted intraoperative crystalloid infusion rate across all surgeries was to 7.9 (SD 4) mL/kg/h. The regression model strongly favored the type of surgery over other patient predictors. We found that high fluid volume was associated with 40% greater odds ratio (OR 1.40; 95% confidence interval 1.01-1.95, p = 0.044) of postoperative complications in patients undergoing THA, while we found no associations for the other types of surgeries, AKI and LOS CONCLUSIONS: A wide variability was observed in intraoperative crystalloid volume administration; however, this did not affect postoperative outcomes.

Postoperative radiotherapy after flap reconstructive surgery in patients with head and neck cancer: A retrospective monocentric study with flap delineation to assess toxicity and relapse.

PURPOSE: Flaps are increasingly used during reconstructive surgery of head and neck cancers to improve functional outcomes. There are no guidelines as to whether the whole flap or its anastomotic border should be included in the primary tumour target volume of postoperative radiotherapy to prevent local relapses. Relapse and toxicity rates can increase substantially if the whole flap received full dose. Our aim was to determine whether flaps were included in the primary tumour target volume and to report the patterns of relapse and toxicity. MATERIALS AND METHODS: Consecutive patients in 2014 through 2016, with or without a flap, receiving postoperative radiotherapy were selected in a retrospective monocentric control study. Flaps were homogenously delineated blind to treating radiation oncologists using a flap-specific atlas. Tumour recurrence, acute and late toxicity were evaluated using univariate and propensity score analyses. RESULTS: A hundred patients were included; 54 with a flap. Median flap volume included in the tumour volume was 80.9%. Twelve patients experienced local recurrences: six with a flap, among whom two within their flap (3.7%). Patients with flaps had larger median tumour volumes to be irradiated (25cm3 versus 58cm3, p<0.001) and higher acute/late toxicity rates (p<0.001) even after adjustment on biases (more advanced T stage, oral cavity, active smoking in patients with flaps). Locoregional recurrence and survival rates were similar between patients with/without a flap. CONCLUSION: Recurrences within a flap were rare in this series when including the whole flap body in the 60Gy-clinical target volume but inclusion of the flap in the primary tumour target volume increased toxicity. Multicentric studies are warranted.

Last-Minute Recovery of a Psychiatric Patient Requesting Physician-Assisted Death.

Physician-assisted death is becoming legal in an increasing number of jurisdictions, but psychiatric patients are often explicitly excluded. However, in some countries, including the Netherlands, physician-assisted death of psychiatric patients is allowed. This Open Forum describes a patient with schizophrenia and symptoms diagnosed as refractory musical hallucinations. The patient requested assistance in dying only to recover after a mandatory second opinion, where his complaints were recognized as intrusive thoughts and treated accordingly. This case is used to reflect on how to deal with uncertainty about physician-assisted death of psychiatric patients and to argue for implementation of a due-diligence procedure, such as the one proposed in the Dutch Psychiatric Association's recent guideline concerning this issue.

A new Monte Carlo model of a Cyberknife® system for the precise determination of out-of-field doses.

In a previous work, a PENELOPE Monte Carlo model of a Cyberknife system equipped with fixed collimator was developed and validated for in-field dose evaluation. The aim of this work is to extend it to evaluate peripheral doses and to determine the precision of the treatment planning system (TPS) Multiplan in evaluating the off-axis doses. The Cyberknife® head model was completed with surrounding components based on manufacturer drawings. The contribution of the different head parts on the out-of-field dose was studied. To model the attenuation and the modification of particle energy caused by components not modelled, the photon transport was modified in one of the added components. The model was iteratively adjusted to fit dose profiles measured with EBT3 films and an ionization chamber for several collimator sizes. Finally, dose profiles were calculated using the two Multiplan TPS algorithms and were compared to our simulations. The contributions to out-of-field dose were identified as scattered radiation from the phantom and head leakage and scatter originating at the secondary collimator level. Particle transport in the additional pieces was modified to model this radiation. The maximum differences between simulated and measured doses are of 20.4%. Regarding the detector responses away from axis, EBT3 films and the Farmer chamber give similar response (less than 20% difference). The TPS Monte Carlo algorithm underestimates the doses away from axis more importantly for the smaller field sizes (up to 98%). Besides, RayTracing simplifies peripheral dose to a constant value with no inclusion of particle transport. A Monte Carlo model of a Cyberknife system for the determination of out-of-field doses up to 14 cm off-axis was successfully developed and validated for different depths and field sizes in comparison with measurements. This study also confirms that TPS algorithms do not model peripheral dose properly.