Drug Cost Avoidance Resulting from Participation in Clinical Trials: A 10-Year Retrospective Analysis of Cancer Patients with Solid Tumors.

The objective of this single-center retrospective study was to describe the clinical characteristics of adult patients with solid tumors enrolled in cancer clinical trials over a 10-year period (2010-2019) and to assess drug cost avoidance (DCA) associated with sponsors' contributions. The sponsors' contribution to pharmaceutical expenditure was calculated according to the actual price (for each year) of pharmaceutical specialties that the Vall d'Hebron University Hospital (HUVH) would have had to bear in the absence of sponsorship. A total of 2930 clinical trials were conducted with 10,488 participants. There were 140 trials in 2010 and 459 in 2019 (228% increase). Clinical trials of high complexity phase I and basket trials accounted for 34.3% of all trials. There has been a large variation in the pattern of clinical research over the study period, whereas, in 2010, targeted therapy accounted for 79.4% of expenditure and cytotoxic drugs for 20.6%; in 2019, immunotherapy accounted for 68.4%, targeted therapy for 24.4%, and cytotoxic drugs for only 7.1%. A total of four hundred twenty-one different antineoplastic agents were used, the variability of which increased from forty-seven agents in 2010, with only seven of them accounting for 92.8% of the overall pharmaceutical expenditure) to three hundred seventeen different antineoplastic agents in 2019, with thirty-three of them accounting for 90.6% of the overall expenditure. The overall expenditure on antineoplastic drugs in clinical care patients not included in clinical trials was EUR 120,396,096. The total cost of antineoplastic drugs supplied by sponsors in a clinical trial setting was EUR 107,306,084, with a potential DCA of EUR 92,662,609. Overall, clinical trials provide not only the best context for the progress of clinical research and healthcare but also create opportunities for reducing cancer care costs.

Hostilities faced by people on the frontlines of sexual and reproductive health and rights: a scoping review.

Frontline workers for sexual and reproductive health and rights (SRHR) provide life-changing and life-saving services to millions of people every year. From accompanying the pregnant, delivering babies and caring for the newborn to supporting those subjected to sexual violence; from treating debilitating infections to expanding contraceptive choices; from enabling access to safe abortion services to countering homophobia: all over the world frontline SRHR carers and advocates make it possible for so many more to experience dignity in sex, sexuality and reproduction. Yet they are also subjected to hostility for what they do, for whom they provide care, for where they work and for the issues they address. From ostracistion and harassment in the workplace to verbal threats and physical violence, hostilities can extend even into their private lives. In other words, as SRHR workers seek to fulfil the human rights of others, their own human rights are put at risk. Yet, as grave as that is, it is a reality largely undocumented and thus also underestimated. This scoping review sets out to marshal what is known about how hostilities against frontline SRHR workers manifest, against whom, at whose hands and in which contexts. It is based on review of six sources: peer-reviewed and grey literature, news reports, sector surveys, and consultations with sector experts and, for contrast, literature issued by opposition groups. Each source contributes a partial picture only, yet taken together, they show that hostilities against frontline SRHR workers are committed the world over-in a range of countries, contexts and settings. Nevertheless, the narratives given in those sources more often treat hostilities as 'one-off', exceptional events and/or as an 'inevitable' part of daily work to be tolerated. That works in turn both to divorce such incidents from their wider historical, political and social contexts and to normalise the phenomena as if it is an expected part of a role and not a problem to be urgently addressed. Our findings confirm that the SRHR sector at large needs to step-up its response to such reprisals in ways more commensurate with their scale and gravity.

Nutritional Quality of Meat from Barren Merino Ewes in Comparison to Meat from Traditional Lambs.

In Spain, lamb consumption has decreased over the last few years. To increase farmers' income, we studied the nutritional quality of the barren ewes' meat in comparison to traditional lambs' meat with 10 barren ewes, 10 male lambs, and 10 female lambs from the Merino breed. We measured the subcutaneous fat, muscle color, and carcass tissue composition, as well as proximal composition, mineral, tocopherol, retinol, lutein, and cholesterol contents, and the TPA texture profile, fatty acid profile, and lipid oxidation of the meat. There was no effect of the animal type (males, females, and ewes) on the pH, and the differences in the subcutaneous fat color, lipid oxidation, and texture were irrelevant from a practical point of view. The tissue composition in the three groups of animals reflected a high percentage of saleable meat, with no penalty incurred for intensive fattening in any of the groups. The ewes' meat presented a higher percentage of moisture, collagen, ash, calcium, iron, α-tocopherol, and retinol than the lambs' meat. In addition, it had higher content of DHA and CLA and lower values for the ratio n6/n3, which is beneficial for health, although it also contained more fat, saturated fatty acids, and cholesterol than the lambs' meat.

Relationship between Consumers' Perceptions about Goat Kid Meat and Meat Sensory Appraisal.

The goat meat preferred by consumers in Spain comes from suckling goat kids, slaughtered at a live weight of 8-10 kg. However, consumption of this meat is very uncommon, so it is necessary to show its value. To achieve this, we planned to investigate consumers' perceptions about goat kid meat and to study whether their perceptions are related to their sensory appraisal of the meat, measured by the mean of the consumers' home tests. The experiment was conducted with 30 volunteer families (from two cities with different consumption patterns), who were surveyed regarding their sociodemographic parameters, purchasing and eating habits, and the importance of the meat's attributes. As a result, four clusters were obtained, which were labeled "negative," "idealistic," "positive," and "concerned about fat content". The parameters of the animal production system were valued differently by the clusters. Meat tenderness, taste, and amount of fat were considered very decisive factors by most respondents. When the goat kid meat was valued, tenderness was considered more important than taste among older people ("negative" cluster), whereas there was not so much difference between the appraisal of all parameters for the other three clusters. We conclude that it is necessary to improve the information received by consumers about goat production systems and meat quality parameters. There is certainly potential for creating new markets, especially targeted toward young consumers and considering specific strategies for the different groups of consumers, depending on the region and habits of consumption.

Morphostructural Differences between the Historical Genetic Lines of the Spanish Merino Sheep.

The Merino breed, which originates from Spain, is the most emblematic livestock breed in the world, since it is the first with a worldwide extension and has had an important impact on the genetic origin of several of the main current sheep populations. For this reason, it is of vital importance to typify the historical genetic lines of the original Spanish Merino breed and thereby ensure the conservation of its variability. In the present study, we used 337 purebred animals (males and females) registered in the Genealogical Book of the Native Merino Breed. All the animals were descendants of herds from six ancestral genetic lines (Maesso, Egea, Granda, López-Montenegro, Hidalgo, and Donoso). Significant differences were found in all the morphometric traits and indexes between the different genetic lines. Using discriminant analysis, 84% of the animals were classified correctly into their historical genetic lines. Furthermore, the distances between the lines, calculated by a cluster test, showed that Hidalgo, Maesso, and Donoso had the most clearly defined lines, while the Granda, López-Montenegro, and Egea lines were more similar to each other. All this demonstrates the rich genetic variability existing in the genuine gene pool of the Merino sheep breed.

PAUSA: Resultados de un programa de proximidad dirigido a personas con prácticas de chemsex en la ciudad de Madrid / PAUSA: Results of an outreach programme aimed at people with chemsex practices in the city of Madrid

Introducción: El Instituto de Adicciones de Madrid Salud (Ayuntamiento de Madrid) utiliza habitualmenteestrategias de acercamiento orientadas a diferentes grupos de población. En 2020 se impulsó un nuevoprograma de acercamiento dirigido a usuarios con prácticas de chemsex, cuyo diseño y resultadosiniciales se presentan en este trabajo. Objetivos: El programa de acercamiento PAUSA se proponeacceder a usuarios de chemsex que todavía no han demandado atención profesional, ofreciéndolesapoyo desde fases más tempranas para: reducir los riesgos de estas prácticas, programar un descansode las mismas, o facilitar un mejor acceso y enlace a los servicios públicos y comunitarios especializadosen chemsex en la ciudad de Madrid. Materiales y métodos: El diseño del programa exploró diferentesopciones de acercamiento a usuarios de chemsex en activo, incluyendo estrategias de difusión onliney a través de tarjetas y cartelería. Se ofrecieron a los usuarios dos tipos de intervenciones, breves yestructuradas. Las primeras se desarrollaron vía chat. Las segundas mediante cita programada presencialo por Skype/Zoom. Resultados: Durante el periodo de actividad analizado se realizaron un total de 739intervenciones breves por chat, beneficiando a un total de 442 usuarios. Se realizaron además un totalde 190 citas estructuradas, presenciales u online, de una hora de duración. Un total de 115 usuariosfueron derivados a centros de atención a las adicciones. La atención recibida por parte del programarecibió excelentes evaluaciones. Conclusiones: El modelo de intervención implementado en el programaPAUSA resultó efectivo para contactar con usuarios de chemsex en fases más tempranas. La captaciónonline en aplicaciones de contactos gais fue la estrategia de acercamento de mayor éxito. Los usuariosatendidos mostraron una alta aceptabilidad de este formato de intervención. (AU)

Introduction: The Institute of Addictions of Madrid Health (City Council of Madrid) routinely employsoutreach strategies aimed at different population groups. In 2020, a new outreach programme wasinitiated targeting individuals engaged in chemsex practices. This paper presents the design and initialoutcomes of this programme. Objectives: The PAUSE outreach programme aims to engage chemsexusers who have not yet sought professional help, providing them support at earlier stages to mitigatethe risks associated with these practices, introduce breaks from them, or facilitate better access andlinkage to public and community services specialised in chemsex within the city of Madrid. Materialsand Methods: The programme’s design explored various approaches to engage active chemsex users,including online dissemination strategies and through cards and posters. Users were offered twotypes of interventions: brief and structured. The former were conducted via chat, while the latterwere scheduled either for in-person meetings or through Skype/Zoom. Results: During the analysedactivity period, a total of 739 brief interventions were conducted via chat, benefiting 442 users in total.Additionally, 190 structured sessions, either in-person or online, lasting an hour each were carriedout. A total of 115 users were referred to addiction care centres. The programme’s services receivedexcellent evaluations. Conclusions: The intervention model implemented in the PAUSE programmeproved effective in reaching users engaged in chemsex during early stages. Online outreach throughgay contact apps emerged as the most successful engagement strategy. Users served demonstrated ahigh acceptability of this intervention format. (AU)

Genomic Population Structure of the Main Historical Genetic Lines of Spanish Merino Sheep.

According to historiographical documentation, the Romans first began to select Merino sheep in the Iberian Peninsula during the first century, with the aim of obtaining a breed appreciated for the quality of its wool. This process continued locally during the Middle Ages, when Spanish sheep were protected, and their export to foreign countries was banned. It was during the 16th century when individual Merino sheep were allowed to spread around the world to be used to improve the wool quality of local breeds. However, the wool crisis of the 1960s shifted the selection criteria of the Merino breed towards meat production at the expenses of wool. Consequently, individuals that display the genetic and phenotypic characteristics of those sheep originally bred in the kingdom of Spain in the Middle Ages are extremely difficult to find in commercial herds. In this study, we characterized the genetic basis of 403 individuals from the main historical Spanish Merino genetic lines (Granda, Hidalgo, Lopez-Montenegro, Maeso, Donoso and Egea), which were bred in isolation over the last 200 years, using a genomic approach based on genotyping data from the Axiom™ Ovine 50K SNP Genotyping Array. Our analysis included measuring population structure, genomic differentiation indexes, runs of homozygosity (ROH) patterns, and an analysis of molecular variance (AMOVA). The results showed large genetic differences between the historical lines, even though they belong to the same breed. In addition, ROH analysis showed differences due to increased inbreeding among the ancient generations compared with the modern Merino lines, confirming the breed's ancestral and closed origin. However, our results also showed a high variability and richness within the Spanish historical Merino lines from a genetic viewpoint. This fact, together with their great ability to produce high-quality wool, suggests that ancestral Merino lines from Spain should be considered a valuable genetic population to be maintained as a resource for the improvement of wool-producing sheep breeds all around the world.

Contingent cfDNA Screening Implementation: Increasing Diagnostic Accuracy and Reducing Invasive Testing - 6 Years' Results in a Single Center.

OBJECTIVES: Universal screening for trisomy using cell-free DNA (cfDNA) has proven to be more effective than combined test, but it is not cost efficient currently. Contingent cfDNA screening on the results of the first-trimester combined test can improve the detection rate of the combined test and reduce the number of invasive tests at a lower cost than universal screening. In 2018, a contingent screening program was implemented in the community of Castilla y Leon (Spain). This study aims to compare the results achieved in Salamanca University Hospital during the first 3 years of contingent screening (2018-2020) with those of the previous 3 years (2015-2017) to assess the changes in the trisomy detection rate and the number of invasive tests. METHODS: A total of 9,903 singleton pregnancies without malformations nor nuchal translucency >p99 were included. 5,165 patients underwent combined screening and 4,738 had contingent screening based on the combined test risk. In the combined test group, women were offered an invasive test if the risk was ≥1:270, while risks under 1:270 were considered low risks, and no further testing was offered. In the contingent screening group, invasive testing was offered if the risk was ≥1:100 (≥1:50 from 2020 onwards), while cfDNA was offered if the combined test risk was between 1:100 and 1:1,000 (1:50-1:1,000 from 2020 onwards). When risk was <1:1,000, no further testing was offered. Aneuploidies detected by cfDNA were confirmed by invasive diagnostic testing. RESULTS: There were 33 cases of trisomy 21 (T21) throughout the 6 years of study. Four cases had low/intermediate risks and were spotted by cfDNA. Risk >1:1,000 threshold for contingent test detected 100% T21. There was a false-positive result for trisomy 13. There were no false-negative results. "No-call" cfDNA results were minimized by repeating blood collection 2 weeks later, as fetal fraction (FF) was doubled. Invasive testing had a drop rate of 84% after contingent screening implementation. DISCUSSION: The implementation of population-based contingent screening significantly reduces the number of invasive tests without lowering diagnostic accuracy. To achieve the maximum efficiency of the program, it is important to know the best cut-offs according to the population where the program is to be implemented. The number of uninformative results due to low FF can be reduced by repeating the test 2 weeks after the initial extraction: this increases the FF to twice the initial one, achieving informative results and avoiding unnecessary invasive tests.

Retinol and α-Tocopherol Contents, Fat Color, and Lipid Oxidation as Traceability Tools of the Feeding System in Suckling Payoya Kids.

The effects of Payoya kid feeding systems on the fat-soluble vitamin (retinol/α-tocopherol) contents, fat content, fat color, and the oxidation index were evaluated to determine their potential for use as feeding system traceability tools. Four groups of Payoya kids (55 animals in total) fed milk exclusively were studied: a group fed a milk replacer (MR) and three groups fed natural milk from dams reared with different management systems (mountain grazing (MG), cultivated meadow (CM) and total mixed ration (TMR)). Kids were slaughtered around one month of age and 8 kg of live weight. Kids from the MG and CM groups presented lower retinol (5.56 and 3.72 µg/mL) and higher α-tocopherol plasma (11.43 and 8.85 µg/mL) concentrations than those from the TMR and MR groups (14.98 and 22.47 µg/mL of retinol; 2.49 and 0.52 µg/mL of α-tocopherol, respectively) (p < 0.001). With respect to fat, kids with a higher intramuscular fat percentage (CM and TMR groups) had lower retinol contents (16.52 and 15.99 µg/mL, respectively) than kids from the MG and MR groups (26.81 and 22.63 µg/mL, respectively) (p < 0.001). A dilution effect of vitamins on fat was shown: the higher the amount of fat, the lower the vitamin concentrations, the higher the lipid oxidation index (MDA), and the lower the SUM (absolute value of the integral of the translated spectra between 450 and 510 nm). A discriminant analysis that included all studied variables showed that 94.4% of the kids were classified correctly according to their feeding system and could allow traceability to the consumer.

Influence of the Use of Milk Replacers on Carcass Characteristics of Suckling Kids from Eight Spanish Goat Breeds.

Since goat milk has a higher value than kid meat in Europe, some farmers rear kids with milk replacers, although some studies have stated that kids raised on natural milk yield higher-quality carcasses. Our previous studies showed some interactions between breed and rearing system on carcass and meat quality. This study evaluated the influence of the use of milk replacers on several carcass characteristics of suckling kids from eight Spanish goat breeds (Florida, Cabra del Guadarrama, Majorera, Palmera, Payoya, Retinta, Tinerfeña, and Verata). A total of 246 kids fed milk replacer (MR) or natural milk (NM) were evaluated. Carcass, head, viscera, and kidney fat weights, as well as several carcass measurements (round perimeter, forelimb width, carcass length, forelimb length, and carcass compactness index), were registered. Forelimbs were dissected to study tissue composition. For all studied variables, interactions were found between rearing system and breed. In general, the MR rearing system increased the head and visceral weights, as well as the length measurements and muscle percentages. Conversely, the NM rearing system increased carcass compactness and resulted in higher fat contents, independent of the deposit. The choice of one or another rearing system should be made according to the needs of the target market.

How Management System Affects the Concentration of Retinol and α-Tocopherol in Plasma and Milk of Payoya Lactating Goats: Possible Use as Traceability Biomarkers.

The retinol and α-tocopherol concentrations were quantified (µg/mL) using high-performance liquid chromatography (HPLC) in both plasma and milk of goats from three management systems. The aim was to investigate if the compounds pass from feed to animals' fluids and to evaluate their potential use as feeding regime biomarkers. A total of 45 Payoya dams were distributed in three groups according to management system during the first month of lactation: mountain grazing (MG), cultivated meadow (CM) and total mixed ration (TMR). TMR group had higher concentrations of retinol in both plasma (25.92 ± 3.61 at 30 days postpartum) and milk (8.26 ± 0.79 at 10 days postpartum), and they were also the unique animals whose milk contained detectable concentrations of α-tocopherol (3.15 ± 0.19 at parturition). However, MG and CM goats showed higher plasma concentrations of α-tocopherol (64.26 ± 14.56 and 44.65 ± 5.75 at 30 days postpartum, respectively). These results could imply differences in the bioavailability of supplemented vitamin A and natural ß-carotene and between the natural/synthetic forms of α-tocopherol. An inverse relationship between the fluids (plasma/milk) in the contents of α-tocopherol and retinol was observed as lactation progressed. Since 80% of the animals were correctly classified using a discriminant analysis based on these vitamins, these compounds could be used as traceability biomarkers of feeding system, but further studies are necessary to know the possible passage to kid meat.

Cesarean Hysterectomy in Abnormally Invasive Placenta: The Role of Prenatal Diagnosis.

An abnormally invasive placenta (AIP) is a placenta that cannot be removed spontaneously or manually without causing severe bleeding. It is a dangerous condition associated with a high rate of maternal and perinatal morbidity and mortality due to the high rate of massive bleeding and visceral injuries. The standardized ultrasound diagnostic criteria have helped improve its early diagnosis, which is essential to plan coordinated actions to reduce associated morbimortality. We present a case report in which ultrasound diagnosis played a decisive role, enabling the coordination of a multidisciplinary team and improving the immediate care of both mother and newborn. Cesarean hysterectomy was performed with minimal blood loss and a good postsurgical recovery.

Effects of Farm Management Practices and Transport Time on Post-Mortem Changes of Longissimus lumborum Muscle Proteins in Suckling Goat Kids.

The combined effect of farm management practices, transport time, and ageing time on the electrophoretic changes of sarcoplasmic (SPP) and myofibrillar (MFP) protein fractions of goat kids was studied. A total of 64 suckling goat kids were withdrawn from two farms with "high" (GW) and "low" (DW) welfare-friendly management practices, and they were transported for 2 or 6 h immediately before slaughtering. Longissimus lumborum samples were obtained at 3, 8, and 21 days post-mortem, and muscle proteins were separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis SDS-PAGE. Both protein extracts displayed significant changes attributable to meat maturation. Managing conditions of kids in DW farms increased the post-mortem susceptibility of muscle proteins. Some MFP of Longissimus lumborum muscle, such as troponin T, as well as 26-30 and 35-37 kDa fractions were influenced significantly by deficient on-farm management, and therefore, these protein fragments might be considered as indicators of low-welfare on-farm management in goat kids.

Effect of Rearing System on the Straight and Branched Fatty Acids of Goat Milk and Meat of Suckling Kids.

Goat meat is considered healthy because it has fewer calories and fat than meat from other traditional meat species. It is also rich in branched chain fatty acids that have health advantages when consumed. We studied the effects of maternal milk and milk replacers fed to suckling kids of four breeds on the straight and branched fatty acid compositions of their muscle. In addition, the proximal and fatty acid compositions of colostrum and milk were studied. Goat colostrum had more protein and fat and less lactose than milk. Goat milk is an important source of healthy fatty acids such as C18:1 c9 and C18:2 n-6. Suckling kid meat was also an important source of C18:1c9. Dairy goat breeds had higher percentages of trans monounsaturated fatty acids (MUFAs) and most of the C18:1 isomers but lower amounts of total MUFAs than meat breeds. However, these dairy kids had meat with a lower percentage of conjugated linoleic acid (CLA) than meat kids. The meat of kids fed natural milk had higher amounts of CLA and branched chain fatty acids (BCFAs) and lower amounts of n-6 fatty acids than kids fed milk replacers. Both milk and meat are a source of linoleic, α-linolenic, docosahexaenoic, eicosapentaenoic and arachidonic fatty acids, which are essential fatty acids and healthy long-chain fatty acids.

Prevalence of iron deficiency and related factors in Spanish adolescents.

Iron deficiency anaemia continues to be the world's most important cause of years lived with disability in children and adolescents. Assessment of iron deficiency traditionally depended on laboratory parameters that may be modified by inflammation states, including obesity, which is nowadays a current condition in adolescent population of high-income countries. The present study ascertains the prevalence of iron deficiency and its related factors in adolescents, using the serum transferrin receptor and the reticulocyte haemoglobin content, in order to avoid this confusing effect of classical parameters. A cross-sectional study was conducted on a population-based representative sample for teenagers in Almería (Spain), of 405 subjects aged 12 to 16 years. Iron deficiency was present in 13.3% of adolescents, but iron deficiency anaemia only in 1.2%. Multivariate logistic regression analyses showed that being part of an immigrant family, a low iron bioavailability diet, meat consumption below four times a week and fish consumption below twice a week, were independent risk factors for iron deficiency.Conclusion: This study provides an estimate iron deficiency prevalence of 13.3% in Spanish healthy adolescents, avoiding potential confounding factors through the use of new iron status parameters, based on a wide representative sample of adolescents from the city of Almería. What is Known: • For children and adolescents, iron deficiency anaemia continues to be the world's most important cause of years lived with disability. • Assessment of iron deficiency has traditionally depended on laboratory parameters that may be modified by inflammatory states, including obesity. What is New: • Iron deficiency prevalence and their related factors were analysed in Spanish adolescents, avoiding potential confounding factors through the use of sTfR and CHr. • Being part of an immigrant family and consuming a low iron bioavailability diet are independent risk factors for iron deficiency.

Características y evolución de una cohorte de niños con síndrome PFAPA en la Comunidad de Madrid / Characteristics and disease course in a cohort of children with PFAPA syndrome in the Community of Madrid, Spain

INTRODUCCIÓN: El síndrome PFAPA es una enfermedad autoinflamatoria de diagnóstico clínico. Se han propuesto diversos tratamientos; entre ellos, la tonsilectomía podría ser un tratamiento efectivo. MATERIAL Y MÉTODOS: Estudio multicéntrico retrospectivo. Se incluyeron pacientes diagnosticados de síndrome PFAPA, según los criterios de Thomas, en 3 hospitales madrileños, entre 2009-2013. RESULTADOS: Se incluyeron 32 casos. Las medianas de edad de inicio del cuadro y al diagnóstico fueron 32 meses (RIQ 24-44) y 47,5 meses (RIQ 37-60), respectivamente. Se hallaron incrementos en las cifras de leucocitos (13.580/μl [RIQ 8.200-16.600] vs.8.300/μl [RIQ 7.130-9.650], p = 0,005), neutrófilos (9.340/μl [RIQ 5.900-11.620] vs.3.660/μl [RIQ 2.950-4.580], p = 0,002) y proteína C reactiva (11,0mg/dl [RIQ 6,6-12,7] vs.0,2mg/dl [RIQ 0,1-0,6], p = 0,003) durante los episodios febriles, respecto a los periodos libres de síntomas. El 80,8% refería remisión de los síntomas en 24h tras corticoterapia oral. Fueron tonsilectomizados 14 pacientes. En 11 cesaron los episodios febriles, mientras que en 3 se redujo su frecuencia; hubo 2 sangrados posquirúrgicos como complicación. El cuadro se había resuelto en el 56,3% de los pacientes, a una mediana de edad de 60 meses (RIQ 47-95), con una duración similar entre los pacientes que fueron tonsilectomizados y los que no. CONCLUSIONES: Se presenta una cohorte amplia de niños con síndrome de PFAPA en la que se confirma que, en nuestro medio, los niños con este síndrome presentan unas características clínicas y analíticas similares a las descritas en la literatura, con buena respuesta a corticoterapia y elevada resolución de la clínica tras la amigdalectomía

INTRODUCTION: PFAPA syndrome is an autoinflammatory disease whose diagnosis is mainly clinical. Several treatments have been proposed; among them, tonsillectomy could be an effective one. MATERIAL AND METHODS: Retrospective multicenter study. Patients included were diagnosed with PFAPA syndrome, according to the Thomas criteria, in 3 hospitals in Madrid between 2009-2013. RESULTS: Thirty-two cases were included. Median age at onset and at diagnosis were 32 months (IQR 24-44) and 47.5 months (IQR 37-60), respectively. There were increases in leukocytes (13,580/μL [IQR 8,200-16,600] vs.8,300/μL [IQR 7,130-9,650], P=.005), neutrophils (9,340/μL [IQR 5,900-11,620] vs.3,660/μL [IQR 2,950-4,580], P=.002) and C-reactive protein (11.0mg/dL [IQR 6.6-12.7] vs.0.2mg/dL [IQR 0.1-0.6], P=.003) during febrile episodes. In all, 80.8% of patients reported remission of symptoms within 24h after oral corticosteroid therapy. Fourteen patients were tonsillectomized. In 11, the febrile episodes stopped while, in 3, the frequency was reduced; there were 2 cases of postoperative bleeding. The disease was resolved in 56.3% of the patients, at a median age of 60 months (IQR 47-95), with similar duration in patients who were tonsillectomized and those who were not. CONCLUSIONS: We present a large cohort of children with PFAPA syndrome, with clinical and analytical features similar to those described in the literature, and a good response to corticosteroids and a high resolution rate of symptoms after tonsillectomy

Influence of the Use of Milk Replacers and pH on the Texture Profiles of Raw and Cooked Meat of Suckling Kids.

The aim of this work was to study the texture profile of fresh and cooked longissimus thoracis et lumborum muscle from suckling kids raised with natural milk or milk replacers. Suckling male kids from eight goat breeds (Florida, FL; Cabra del Guadarrama, GU; Majorera, MA; Palmera, PL; Payoya, PY; Retinta, RE; Tinerfeña, TI; Verata, VE), all of single parturition, were raised with milk replacers (MR) or with natural milk from the dams (NM). The meat pH, Warner-Bratzler shear force, texture profile analysis and chemical composition were determined. Kids were clustered based on their pH by k-means clustering. The effect of the rearing system on the textural profile was strongly modulated by breed. The values of Warner-Bratzler shear force and hardness found in these breeds under both rearing systems were very low. Hence, the toughness of very light suckling kids should not be a determining factor in choosing a breed or rearing system. Nevertheless, the use of milk replacers increased the presence of meat with high pH, which modified the textural parameters, decreasing the shear force but increasing cohesiveness and adhesiveness. Consequently, depending on the commercial strategy of the farm, the election of the breed and rearing system must be considered together.

Ductus Venosus Agenesis as a Marker of Pallister-Killian Syndrome.

The ductus venosus (DV) is a shunt that allows the direct flow of well-oxygenated blood from the umbilical vein (UV) to the coronary and cerebral circulation through the foramen ovale. Its agenesis has been associated with chromosomal abnormalities and rare genetic syndromes, structural defects, intrauterine growth restriction (IUGR) and even antepartum fetal demise. Pallister-Killian Syndrome (PKS) is a rare sporadic disorder with specific tissue mosaic distribution of an extra 12p isochromosome (i(12p)). Its main clinical features are moderate to severe intellectual disability/neuromotor delay, skin pigmentation abnormalities, typical facial appearance, variable association with multiple congenital malformations and epilepsy. Though prenatal findings (including congenital diaphragmatic hernia, ventriculomegaly, congenital heart disease, polyhydramnios, and rhizomelic shortening) have been described in literature, prenatal diagnosis is difficult as there are no associated identification signs no distinctive or pathognomonic signs, and some of these malformations are hard to identify prenatally. The tissue mosaicism linked to this syndrome and the decrease of the abnormal clone carrier of the i(p12) after successive trypsinizations of cultured cells makes the diagnosis even more challenging. We present the case of a 27.5 weeks pregnant woman with a fetal ductus venosus agenesis (DVA) as the main guide marker. To our knowledge this is the first case published in literature reporting a DVA as a guide sign to diagnose a complex condition as Pallister-Killian syndrome. We also underscore the key role of new genetic techniques as microarrays to avoid misdiagnosis when only a subtle sonographic sign is present in complex conditions like this.

Palbociclib and ribociclib in breast cancer: consensus workshop on the management of concomitant medication.

Drug-drug interactions are of significant concern in clinical practice in oncology, particularly in patients receiving Cyclin-dependent kinase (CDK) 4/6 inhibitors, which are typically exposed to long-term regimens. This article presents the highlights from the 'First Workshop on Pharmacology and Management of CDK4/6 Inhibitors: Consensus about Concomitant Medications'. The article is structured into two modules. The educational module includes background information regarding drug metabolism, corrected QT (QTc) interval abnormalities, management of psychotropic drugs and a comprehensive review of selected adverse effects of palbociclib and ribociclib. The collaborative module presents the conclusions of the five working groups, each of which comprised five experts from different fields. From these conclusions positive lists of drugs for treating common comorbid conditions that can be safely administered concomitantly with palbociclib and/or ribociclib were developed.