Executive summary on the optimization of the multidisciplinary and integrated approach to polymyalgia rheumatica and giant cell arteritis in Madrid region.

Polymyalgia rheumatica and giant cell arteritis can be a medical emergency in which a delay in correct diagnosis and therapeutic management can cause serious complications. With the aim of improving the care of patients with these pathologies in the Community of Madrid, a study was designed to identify the causes and possible solutions to address the problems related to the diagnosis of these pathologies. After the analysis, 11 areas of improvement related to four different aspects of the care process were identified: coordination and protocols, equipment, training and awareness of pathologies, and patient experience. Of all the areas identified, it was considered a priority to resolve those related to the generation of protocols for the comprehensive management of the pathologies, which include all the specialties and levels of care involved. Another crucial aspect is the increase in the degree of clinical suspicion of these pathologies.

Techno-economic analysis of torrefied fuel pellet production from agricultural residue via integrated torrefaction and pelletization process.

Torrefied pellets have gained more commercial importance due to their excellent performance in combustion, co-firing and gasification. The present investigation provides a conceptual design for torrefied fuel pellets production via combined torrefaction and pelletization technologies with and without additives. The entire design contains torrefaction unit, grinding, preparation of pellet formulation, pelletizing, and finally cooling of pellets. Two scenarios, scenario 1 (pelletization of torrefied biomass with additives) and scenario 2 (pelletization of torrefied biomass without any external additives) were tested and compared. The economic analysis suggests that both scenarios are profitable. Both scenarios were simulated using Aspen plus™, and economic feasibility was estimated using a complete cash flow analysis for a base case plant with 40,080 tonne/y capacity. For both cases, a discounted cash flow is a useful tool for estimating the minimal selling price for torrefied pellets as well as the capital investment, production cost and operating costs. The cost of the reactor used for torrefaction was found to be the most important component of combined torrefaction and pelletization system. The lowest selling price of generated torrefied pellets was found to be $103.4 and $105.1 per tonne at the plant gate for scenarios 1 and 2, respectively. Sensitivity analysis shows that, among all variable costs, labor cost has the highest influence on both net present value (NPV) and minimum selling price (MSP) in making pellets for both the scenarios. Furthermore, the internal rate of return was found to be25% and 22% at 10% discounted cash flow rate for scenarios 1 and 2, respectively. The framework that was created was found to lessen over-dependence on wood or fossil fuels and facilitate the promotion of bioenergy in rural areas.

Vertebral fractures are increased in rheumatoid arthritis despite recent therapeutic advances: a case-control study.

Prevalence and risk factors of vertebral fractures in postmenopausal RA women were assessed in 323 patients and compared with 660 age-matched women. Of patients, 24.15% had at least one vertebral fracture vs.16.06% of controls. Age, glucocorticoids and falls were the main fracture risks. Vertebral fractures were associated with disease severity. INTRODUCTION: There is little quality data on the updated prevalence of fractures in rheumatoid arthritis (RA) that may have changed due to advances in the therapeutic strategy in recent years. This study was aimed at analysing the prevalence and risk factors of vertebral fractures in postmenopausal women with RA and comparing it with that of the general population. METHODS: We included 323 postmenopausal women diagnosed with RA from 19 Spanish Rheumatology Departments, randomly selected and recruited in 2018. Lateral radiographs of the thoracic and lumbar spine were obtained to evaluate morphometric vertebral fractures and the spinal deformity index. We analysed subject characteristics, factors related to RA, and fracture risk factors. The control group consisted of 660 age-matched Spanish postmenopausal women from the population-based Camargo cohort. RESULTS: Seventy-eight (24.15%) RA patients had at least one vertebral fracture. RA patients had increased fracture risk compared with controls (106 of 660, 16.06%) (p = 0.02). Logistic regression analysis showed that age (OR 2.17; 95% CI 1.27-4.00), glucocorticoids (OR 3.83; 95% CI 1.32-14.09) and falls (OR 3.57; 95% CI 1.91-6.86) were the independent predictors of vertebral fractures in RA patients. The subgroup with vertebral fractures had higher disease activity (DAS28: 3.15 vs. 2.78, p = 0.038) and disability (HAQ: 0.96 vs. 0.63, p = 0.049), as compared with those without vertebral fractures. CONCLUSION: The risk of vertebral fracture in RA is still high in recent years, when compared with the general population. The key determinants of fracture risk are age, glucocorticoids and falls. Patients with vertebral fractures have a more severe RA.

[Medullar thoracic compression by tophaceous gout: presentation of a case and review of the literature]. / Compresion medular dorsal por tofos gotosos: presentacion de un caso y revision de la bibliografia.

INTRODUCTION: Spine involvement in gout is an extremely uncommon complication. Dorsalgia and quadriplegia are some manifestations that may occur, although these symptoms are seen more frequently in other more prevalent pathologies, such as spinal tumors. CASE REPORT: We present an unusual case of thoracic spinal cord compression at T10-T11 level caused by the extradural deposit of tophaceous material in a 52-year-old woman with uncontrolled chronic tophaceous gout. In addition to intensive medical treatment, the patient required surgery (hemilaminectomy and spinal decompression) and subsequent rehabilitation. Overall and neurological evolution were satisfactory.

TITLE: Compresion medular dorsal por tofos gotosos: presentacion de un caso y revision de la bibliografia.Introduccion. La afectacion de la columna vertebral en la gota es una complicacion extremadamente infrecuente. La dorsalgia y la cuadriplejia son algunas manifestaciones que se pueden presentar, aunque estos sintomas se ven con mas frecuencia en otras patologias mas prevalentes, como los tumores medulares. Caso clinico. Se presenta un caso inusual de compresion medular dorsal en D10-D11 causado por el deposito extradural de material tofaceo en una paciente de 52 años con gota tofacea cronica incontrolada. Ademas de un tratamiento medico intensivo, la paciente requirio cirugia (hemilaminectomia y descompresion medular) y rehabilitacion posterior. La evolucion general y neurologica fue satisfactoria.

Estrategia de salud bucal para niños con discapacidad visual / Oral health strategy for visually impaired children

El propósito de este trabajo es presentar una propuesta de la Guía de Salud Bucal para niños con discapacidad visual ya que es una preocupación del Programa de Maestría en Odontopediatría de la UCSUR la inclusión de personas en este caso con discapacidades visuales, al acceso de información sobre la importancia de la salud bucal y no habiendo encontrado en nuestro medio este material educativo, es que esta Guía fue desarrollada dentro de uno de los proyectos de innovación y producción académica; esto es un inicio, que con seguridad servirá como base para desarrollar futuros trabajos. (AU)

The aim of this paper is to present an approach of an Oral Health Guide for children with visual disabilities. One of the concern of the Master Program in Pediatric Dentistry of UCSUR is the inclusion of people with disabilities. Nowadays this kind of educative material is missing. This guide was developed into one of the innovation projects and academic production of the Pediatric Dentistry Master Program; this is a start, which certainly will serve as basis for developing future works. (AU)

Painful legs and moving toes syndrome responsive to pregabalin.

Report three cases of painful legs and moving toes (PLMT) syndrome responsive to pregabalin along with a review of its literature. Three patients with PLMT syndrome improved with pregabalin. The first and third patient reported improvement in pain scores, quality of life, and quality of sleep sustained over time. The second and third patient had near complete remission of toe movements, but pregabalin was discontinued in the second patient due to aggravation of leg edema. PLMT is a rare and debilitating disorder characterized by lower limb pain and involuntary toes or feet movements. Its pathophysiology remains unknown and its therapy refractory to most drugs, except for pregabalin, as shown in this case series. PLMT is a rare and incapacitating syndrome due to the lack of an effective pain therapy. We report three patients with PLMT who favorable responded to pregabalin. We propose pregabalin be considered in the management of PLMT.

[Video-assisted sigmoidectomy as an option for Sigmoid volvulus management in the pediatric population]. / Sigmoidectomía videoasistida como opción de manejo del vólvulo del sigmoides en la población pediátrica.

INTRODUCTION: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children. Sigmoid volvulus is one of the three leading causes of acute obstruction of the colon and is between 50 and 90% of all large bowel volvulus. In the pediatric population only 3 to 5% of bowel obstructions are caused by volvulus and there are less than 100 cases reported in the literature. The presence of a redundant sigmoid with a narrow mesentery (dolicosigmoide) is a prerequisite for the volvulus formation. The etiology in the pediatric population is considered secondary to the presence of a broad meso with a narrow base associated with abnormal fixation colon. Other factors include history of anorectal malformation, Prune Belly syndrome, intestinal malrotation and Hirschsprung disease. Initial management followed by endoscopic minimally invasive sigmoidectomy has proven safe and effective. MATERIALS AND METHODS: This paper presents the experience of 4 patients between 9 and 14 managed in our department in 2013, with a diagnosis of volvulus of the sigmoid, which were initially taken to a first surgical endoscopic decompression of volvulus and a second half were carried sigmoid which took place in a video-assisted. In this series, no intraoperative complications were documented and monitoring more than six months only one patient has required new interventions, in a special case because the patient has associated myopathy; inflammatory leiomioscitis, which predisposes to episodes of intestinal obstruction. CONCLUSION: We believe that endoscopic detorsion followed by an early video-assisted sigmoid is the ideal technique for the management of these patients.

INTRODUCCION: El objetivo del estudio es evaluar la presentación y el manejo del vólvulo del Colon Sigmoides (VS) en pacientes pediátricos. El (VS) es una de las tres principales causas de obstrucción aguda del colon y constituye entre el 50 y 90% de todos los vólvulos del intestino grueso. En la población pediátrica, solo del 3 al 5% de las obstrucciones intestinales son causadas por vólvulo, y son menos de 100 los casos publicados en la literatura médica. La etiología en la población pediátrica se considera secundaria a la presencia de un meso amplio con una base estrecha asociado a una fijación anormal del colon. Otros factores incluyen historia de malformación anorectal, síndrome de Prune Belly, malrotacion intestinal y enfermedad de Hirschsprung. El manejo inicial por vía endoscópica, seguido de la sigmoidectomía mínimamente invasiva, han demostrado ser seguros y efectivos. MATERIAL Y METODOS: El presente trabajo recoge la experiencia de 4 pacientes entre los 9 y 14 años manejados en nuestro servicio, en el año 2013, con diagnóstico de vólvulo del sigmoides, los cuales inicialmente fueron llevados a un primer tiempo quirúrgico para descompresión endoscópica del vólvulo y en un segundo tiempo fueron llevados a sigmoidectomía, la cual se llevó a cabo de forma videoasistida. En esta serie no se documentaron complicaciones intraoperatorias y, en el seguimiento a más de 6 meses, solo uno de los pacientes ha requerido nuevas intervenciones, en un caso especial, dado que el paciente presenta asociada una miopatía (leiomioscitis inflamatoria), la cual lo predispone a episodios de obstrucción intestinal. CONCLUSION: Consideramos que la devolvulación endoscópica seguida de una pronta sigmoidectomía videoasistida es la técnica ideal para el manejo de estos pacientes.

Self-reported and accelerometer-measured physical activity by body mass index in US Hispanic/Latino adults: HCHS/SOL.

The association between obesity and physical activity has not been widely examined in an ethnically diverse sample of Hispanic/Latino adults in the US. A cross-sectional analysis of 16,094 Hispanic/Latino adults 18-74 years was conducted from the multi-site Hispanic Community Health Study/Study of Latinos (HCHS/SOL). Body mass index (BMI) was measured and categorized into normal, overweight, and obese; underweight participants were excluded from analyses. Physical activity was measured using the 16-item Global Physical Activity Questionnaire and by an Actical accelerometer. Minutes/day of physical activity and prevalence of engaging in ≥ 150 moderate-vigorous physical activity (MVPA) minutes/week were estimated by BMI group and sex adjusting for covariates. No adjusted differences were observed in self-reported moderate (MPA), vigorous (VPA), or MVPA across BMI groups. Accelerometry-measured MPA, VPA, and MVPA were significantly higher for the normal weight (females: 18.9, 3.8, 22.6 min/day; males: 28.2, 6.1, 34.3 min/day, respectively) compared to the obese group (females: 15.3, 1.5, 16.8 min/day; males: 23.5, 3.6, 27.1 min/day, respectively). The prevalence of engaging in ≥ 150 MVPA minutes/week using accelerometers was lower compared to the self-reported measures. Efforts are needed to reach the Hispanic/Latino population to increase opportunities for an active lifestyle that could reduce obesity in this population at high risk for metabolic disorders.

Development of an autoimmune syndrome affecting the skin and internal organs in P-selectin glycoprotein ligand 1 leukocyte receptor-deficient mice.

OBJECTIVE: To define and characterize the progression of the spontaneous autoimmune disease that develops in mice in the absence of the leukocyte adhesion receptor P-selectin glycoprotein ligand 1 (PSGL-1). METHODS: Skin-resident immune cells from PSGL-1-deficient mice and C57BL/6 control mice of different ages were isolated and analyzed by flow cytometry. Biochemical parameters were analyzed in mouse serum and urine, and the presence of serum autoantibodies was investigated. Skin and internal organs were extracted, and their structure was analyzed histologically. RESULTS: Skin-resident innate and adaptive immune cells from PSGL-1(-/-) mice had a proinflammatory phenotype with an imbalanced T effector cell:Treg cell ratio. Sera from PSGL-1(-/-) mice had circulating autoantibodies commonly detected in connective tissue-related human autoimmune diseases. Biochemical and histologic analysis of skin and internal organs revealed skin fibrosis and structural and functional abnormalities in the lungs and kidneys. Furthermore, PSGL-1(-/-) mice exhibited vascular alterations, showing loss of dermal vessels, small vessel medial layer remodeling in the lungs and kidneys, and ischemic processes in the kidney that promote renal infarcts. CONCLUSION: Our study demonstrates that immune system overactivation due to PSGL-1 deficiency triggers an autoimmune syndrome with characteristics similar to systemic sclerosis, including skin fibrosis, vascular alterations, and systemic organ involvement. These results suggest that PSGL-1 expression contributes to the maintenance of the homeostasis of the immune system and could act as a barrier for autoimmunity in mice.

Influence of the IL17A locus in giant cell arteritis susceptibility.

OBJECTIVE: Different lines of evidence have highlighted the role of IL-17A in the inflammatory process occurring in giant cell arteritis (GCA). The aim of the present study was to assess whether the IL17A locus influences GCA susceptibility and its clinical subphenotypes. METHODS: We carried out a large meta-analysis including a total of 1266 biopsy-proven GCA patients and 3779 healthy controls from four European populations (Spain, Italy, Germany and Norway). Five IL17A polymorphisms (rs4711998, rs8193036, rs3819024, rs2275913 and rs7747909) were selected by tagging and genotyped using TaqMan assays. Allelic combination and dependency tests were also performed. RESULTS: In the pooled analysis, two of the five analysed polymorphisms showed evidence of association with GCA (rs2275913: PMH=1.85E-03, OR=1.17 (1.06-1.29); rs7747909: PMH=8.49E-03, OR=1.15 (1.04-1.27)). A clear trend of association was also found for the rs4711998 variant (PMH=0.059, OR=1.11 (1.00-1.23)). An independent effect of rs2275913 and rs4711998 was evident by conditional regression analysis. In addition, the haplotype harbouring the risk alleles better explained the observed association than the polymorphisms independently (likelihood p value <10(-05)). CONCLUSIONS: Polymorphisms within the IL17A locus show a novel association with GCA. This finding supports the relevant role of the Th17 cells in this vasculitis pathophysiology.

[Assessment of nutritional risk among in-school adolescents from Cantabria]. / Evaluación del riesgo nutricional de los adolescentes escolarizados en Cantabria.

OBJECTIVE: To analyse nutritional risk, by age and sex, among primary and secondary education adolescents from Cantabria. METHODOLOGY: a cross-sectional study was carried out, analysing a sample of 1101 adolescents: 568 (51.6%) were men and 533 (48.4%) were women, aged 12 to 17, attending 16 different primary and secondary education centres in Cantabria, by means of a Krece Plus questionnaire. RESULTS: A high percentage of adolescents with a high nutritional risk (35%) can be observed. Men show a high nutritional risk slightly higher than women (37.8% male vs 32.1% female). Moreover, the high nutritional risk experiences a notable increase as young people get older. Significant statistical differences can be seen both in male and female groups, and as a global group. In all three cases, the nutritional risk distribution in the youngest group is very similar (35.2-35.8% in male, 27.9-29.7% in female, 31.7-32.7% in the global group); whereas in elder adolescents, those values are practically doubled (57.1% in male, 69.0% in female, y 62.2% in the global group). CONCLUSIONS: Results are alarming mainly given the high percentage of adolescents with a high nutritional risk. Men and older adolescents are the groups in which high nutritional risk is more evident.

Objetivo: Evaluar el riesgo nutricional, por edad y sexo, que presentan los adolescentes escolarizados en la Comunidad Autónoma de Cantabria. Sujetos: Se realizó un estudio transversal, analizando una muestra de 1101 adolescentes, de los que 51,6% eran varones y 48,4% fueron mujeres de edades comprendidas entre los 10 y los 17 años, escolarizados en centros de enseñanza pública, mediante el cuestionario Krece Plus. Resultados: Se observa un elevado porcentaje de adolescentes que presentan un riesgo nutricional elevado (35%). Los varones presentan un riesgo nutricional alto en un porcentaje ligeramente superior a las mujeres (37,8 % vs 32,1%). Además, el riesgo nutricional alto sufre un notable incremento a medida que la edad de los jóvenes aumenta. Se aprecian diferencias estadísticamente significativas tanto en los grupos de edad de los varones (p = 0,024), de las mujeres (p < 0,001) como en el grupo global (p = 0,001). En los tres casos, la distribución del riego nutricional en los grupos de menor edad es muy similar (entre 35,2 y 35,8% en los h, entre 27,9 y 29,7% en las m, y entre 31,7 y 32,7% en el grupo total). Mientras que en el grupo de mayor edad estos valores prácticamente se duplican (57,1% en los h, 69,0% en las m, y 62,2 % en el grupo total). Conclusión: Los resultados obtenidos muestran una realidad preocupante debido, principalmente, al elevado porcentaje de adolescentes que presentan un riesgo nutricional elevado. Siendo los varones y los adolescentes de mayor edad los sectores en los que este riesgo nutricional elevado es superior.

The 11q23.3 genomic region-rs964184-is associated with cardiovascular disease in patients with rheumatoid arthritis.

Rheumatoid arthritis (RA) is an inflammatory disease associated with high risk of cardiovascular (CV) events. Recently, the rs964184 polymorphism has been associated with coronary artery disease in nonrheumatic Caucasian individuals. 2160 Spanish RA patients were genotyped for the rs964184 polymorphism. Sex, age at diagnosis and traditional CV risk factors (diabetes mellitus, dyslipidemia and smoking habit) were associated with increased risk of CV events. Interestingly, RA patients carrying the rs964184 GG genotype had significantly higher risk of CV events than those with CC genotype [hazard ratio (HR) = 2.91, 95% confidence interval (CI): 1.36-6.26, P = 0.006] after adjusting the results for sex, age at diagnosis and traditional CV risk factors. Our results indicate that rs964184 polymorphism is associated with CV disease in RA.

Identification of the PTPN22 functional variant R620W as susceptibility genetic factor for giant cell arteritis.

OBJECTIVE: To analyse the role of the PTPN22 and CSK genes, previously associated with autoimmunity, in the predisposition and clinical phenotypes of giant cell arteritis (GCA). METHODS: Our study population was composed of 911 patients diagnosed with biopsy-proven GCA and 8136 unaffected controls from a Spanish discovery cohort and three additional independent replication cohorts from Germany, Norway and the UK. Two functional PTPN22 polymorphisms (rs2476601/R620W and rs33996649/R263Q) and two variants of the CSK gene (rs1378942 and rs34933034) were genotyped using predesigned TaqMan assays. RESULTS: The analysis of the discovery cohort provided evidence of association of PTPN22 rs2476601/R620W with GCA (PFDR=1.06E-04, OR=1.62, CI 95% 1.29 to 2.04). The association did not appear to follow a specific GCA subphenotype. No statistically significant differences between allele frequencies for the other PTPN22 and CSK genetic variants were evident either in the case/control or in stratified case analysis. To confirm the detected PTPN22 association, three replication cohorts were genotyped, and a consistent association between the PTPN22 rs2476601/R620W variant and GCA was evident in the overall meta-analysis (PMH=2.00E-06, OR=1.51, CI 95% 1.28 to 1.79). CONCLUSIONS: Our results suggest that the PTPN22 polymorphism rs2476601/R620W plays an important role in the genetic risk to GCA.

Therapeutic update in large vessel vasculitides.

The term large vessel vasculitidis includes two distinct clinical entities: Takayasu's arteritis and giant cell arteritis. Takayasu's arteritis mainly affects children and women under 40 years, affecting the aorta and its major branches. Its incidence in Southern Europe is low. Giant cell arteritis is the most common vasculitis in those 60. Its incidence in the European population is greater than that of Takayasu's arteritis. It mainly affects the extracranial vessels, especially those derived from the carotid artery. Both conditions are characterized by inflammation of the vessel wall, this causing structural damage and the expression of different clinical manifestations. The treatment of choice of both conditions is based on high-dose glucocorticoids associated, in some cases, to immunosuppressants. Biologic agents have been reserved for cases refractory to conventional therapies.

A case-control study suggests that the CCR6 locus is not involved in the susceptibility to giant cell arteritis.

OBJECTIVES: Polymorphisms of the CC chemokine receptor 6 (CCR6) gene have been recently reported to be associated with a number of autoimmune diseases. We aimed to investigate the possible influence of CCR6 rs3093024 gene variant in the susceptibility to and clinical expression of GCA. METHODS: The CCR6 polymorphism rs3093024 was genotyped in a total of 463 Spanish patients diagnosed with biopsy-proven GCA and 920 healthy controls using a TaqMan® allelic discrimination assay. PLINK software was used for the statistical analyses. RESULTS: No significant association between this CCR6 variant and GCA was observed (p=0.42, OR=0.94, CI95% 0.79-1.10). Similarly, when patients were stratified according to the specific clinical features of GCA such as polymyalgia rheumatica, visual ischaemic manifestations or irreversible occlusive disease, no statistical significant difference was detected either between the case subgroups and the control set or between GCA patients with and without the specific features of the disease. CONCLUSIONS: Our results suggest that the CCR6 rs3093024 polymorphism may not play a relevant role in the GCA pathophysiology.

Clinical practice guideline for an integrated approach to comorbidity in patients with psoriasis.

BACKGROUND: The relationship between psoriasis and associated diseases has drawn particular interest in recent years. To provide appropriate management of psoriasis from an early stage, it is necessary to include prompt diagnosis of concomitant disease and to prevent and treat any comorbidity found. Such an integrated approach also serves to ensure that the drugs used to treat associated diseases do not interfere with the management of psoriasis, and vice versa. OBJECTIVE: To provide the dermatologist a guide focuses specifically on the diagnosis and management of the diseases most often found in patients with psoriasis. METHODS: The selection of the diseases, and corresponding supporting research, to be included was based on a systematic review of the literature. The recommendations on diagnostic criteria are based on the main clinical practice guidelines for each of the diseases discussed as well as on the recommendations of a clinical expert advisory group. The information regarding the repercussions of psoriasis treatments on associated comorbid diseases was obtained from the summary of product characteristics of each drug. In turn, the statements concerning the impact of the associated diseases, and their treatment, on psoriasis are based on the review of the literature. RESULTS: This guide is a precise, easy-to-use tool for systematizing the diagnosis of comorbidity in patients with psoriasis and facilitate decision making regarding referral and treatment of patients diagnosed with an associated disease. CONCLUSION: The application of this guide not only will benefit psoriasis patients' health and quality of life but it will also optimize available resources.

Single-nucleotide polymorphisms at the 9p21.3 genomic region not associated with the risk of cardiovascular disease in patients with rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic polygenic inflammatory disease associated with accelerated atherosclerosis and high risk of cardiovascular disease (CVD). In this study, we evaluated the potential association of 9p21.3 single-nucleotide polymorphisms (SNPs) - previously linked to coronary artery disease - and CVD risk in 2001 Spanish RA patients genotyped for 9p21.3 SNPs using TaqMan™ assays. Carotid intima media thickness (cIMT) and presence of carotid plaques were also analyzed. Cox regression model did not disclose significant differences between patients who experienced CVD and those who did not. Neither association was found between cIMT or carotid plaques and SNPs allele distribution. In conclusion, results do not support a role of rs10116277 or rs1537375 SNPs in CVD risk in Spanish RA patients.