Dermatofibrosarcoma Protuberans Of the Vulva Treated with Mohs Micrographic Surgery

Dermatofibrosarcoma protuberans (DFSP) of the vulva is extremely rare conditions. It has been published approximately 43 case reports in medical literature; most tumors arise on the labia majora. We present a 57-year-old woman with DFSP in vulva that was removed by Mohs Micrographic Surgery technique. The wound exhibited a satisfying functional and cosmetic appearance, with no evidence of deformity of the vulva

Effectiveness of an intervention for improving drug prescription in primary care patients with multimorbidity and polypharmacy:Study protocol of a cluster randomized clinical trial (Multi-PAP project)

This study was funded by the Fondo de Investigaciones Sanitarias ISCIII (Grant Numbers PI15/00276, PI15/00572, PI15/00996), REDISSEC (Project Numbers RD12/0001/0012, RD16/0001/0005), and the European Regional Development Fund ("A way to build Europe").Background: Multimorbidity is associated with negative effects both on people's health and on healthcare systems. A key problem linked to multimorbidity is polypharmacy, which in turn is associated with increased risk of partly preventable adverse effects, including mortality. The Ariadne principles describe a model of care based on a thorough assessment of diseases, treatments (and potential interactions), clinical status, context and preferences of patients with multimorbidity, with the aim of prioritizing and sharing realistic treatment goals that guide an individualized management. The aim of this study is to evaluate the effectiveness of a complex intervention that implements the Ariadne principles in a population of young-old patients with multimorbidity and polypharmacy. The intervention seeks to improve the appropriateness of prescribing in primary care (PC), as measured by the medication appropriateness index (MAI) score at 6 and 12months, as compared with usual care. Methods/Design: Design:pragmatic cluster randomized clinical trial. Unit of randomization: family physician (FP). Unit of analysis: patient. Scope: PC health centres in three autonomous communities: Aragon, Madrid, and Andalusia (Spain). Population: patients aged 65-74years with multimorbidity (≥3 chronic diseases) and polypharmacy (≥5 drugs prescribed in ≥3months). Sample size: n=400 (200 per study arm). Intervention: complex intervention based on the implementation of the Ariadne principles with two components: (1) FP training and (2) FP-patient interview. Outcomes: MAI score, health services use, quality of life (Euroqol 5D-5L), pharmacotherapy and adherence to treatment (Morisky-Green, Haynes-Sackett), and clinical and socio-demographic variables. Statistical analysis: primary outcome is the difference in MAI score between T0 and T1 and corresponding 95% confidence interval. Adjustment for confounding factors will be performed by multilevel analysis. All analyses will be carried out in accordance with the intention-to-treat principle. Discussion: It is essential to provide evidence concerning interventions on PC patients with polypharmacy and multimorbidity, conducted in the context of routine clinical practice, and involving young-old patients with significant potential for preventing negative health outcomes. Trial registration: Clinicaltrials.gov, NCT02866799Publisher PDFPeer reviewe

Enfermedad de Still del adulto: características clínicas y pronóstico de una cohorte de pacientes colombianos

Introduction: Adult Still disease (ASD) is a rare disorder that can lead to serious complications; however, risk factors associated with the development of this disease, its prognostic factors, and aspects related to relapse and steroids refractoriness in individuals with this condition are not yet clearly known. Objectives: To describe the clinical behavior of ASD and determine factors associated with steroid refractoriness, relapse, and complications in patients with this condition. Materials and methods: A retrospective cohort study was conducted in 45 patients diagnosed with ASD between January 2007 and January 2017 in 2 reference hospitals. Summary statistics are presented. In addition, two logistic regression analysis were conducted in order to identify possible factors explaining steroid refractoriness, relapses, and the development of complications in the study population. Results: Participants’ average age was 42.13 ± 15.8 years. Out of the 45 patients, 23 (51.1%) were women, 42 (93.3%) were prescribed steroids, and of these, 13 (32%) were considered as steroid refractory. Complications were observed in 12 individuals (26.7%), and their frequency was higher in patients whose maximum body temperature was higher than 39°C. Finally, 33 (73.3% patients had a follow-up greater than 1 year, of which 17 (37.8%) suffered relapses; in addition, relapses were more frequent in patients who were steroid refractory or those with splenomegaly. Conclusion: Patients who were prescribed any type of biological therapy were more steroid refractory. On the other hand, steroid refractoriness and splenomegaly were associated with a higher frequency of relapses, while having a body temperature >39°C was associated with developing complications.Introducción. La enfermedad de Still del adulto (ESA) es una condición médica poco frecuente que puede presentar complicaciones graves; sin embargo, aún no se conocen con claridad sus factores de riesgo, sus factores pronósticos, ni los aspectos asociados a las recaídas y a la refractariedad a esteroides en individuos con esta condición. Objetivos. Describir el comportamiento clínico de la ESA y determinar los factores asociados a la refractariedad a esteroides, a recaídas y a complicaciones en pacientes con esta enfermedad. Materiales y métodos. Estudio retrospectivo de cohortes que incluyó 45 pacientes diagnosticados con ESA entre enero de 2007 y enero de 2017 en 2 hospitales de referencia. Se presentan medidas de resumen. Se realizaron dos análisis de regresión logística para identificar posibles factores que expliquen la refractariedad a esteroides, las recaídas y el desarrollo de complicaciones en la población de estudio. Resultados. La edad promedio de los participantes fue 42.13±15.8 años. De los 45 pacientes incluidos, 23 (51.1%) eran mujeres, a 42 (93.3%) se le prescribieron esteroides, y, de estos, 13 (32%) se consideraron refractarios a esteroides. Se presentaron complicaciones en 12 (26.7%) individuos y su frecuencia fue mayor en pacientes con temperatura máxima >39°C. Finalmente, 33 (73.3%) pacientes tuvieron un seguimiento mayor a 1 año, de los cuales, 17 (37.8%) presentaron recaídas, las cuales fueron más frecuentes en individuos refractarios a esteroides o con esplenomegalia. Conclusión. Los pacientes que recibieron cualquier tipo de terapia biológica fueron más refractarios a esteroides; por su parte, la refractariedad a esteroides y la esplenomegalia se asociaron a un mayor número de recaídas, y la temperatura >39°C se asoció a al desarrollo de complicaciones