CALR-mutated patients with low allele burden represent a specific subtype of essential thrombocythemia: A study on behalf of FIM and GBMHM.

A low allele burden (i.e., <20%) of the CALR driver mutation is found in 10.8% of CALR-mutated MPNs, mostly in essential thrombocythemia, and correlates with a milder phenotype and a more indolent evolution compared to patients with an allele burden ≥20%.

Bird species responses to rangeland management in relation to their traits: Rio de la Plata Grasslands as a case study.

Areas used for livestock production and dominated by native grasses represent a unique opportunity to reconcile biodiversity conservation and livestock production. However, limited knowledge of individual species' responses to rangeland management restricts our capacity to design grazing practices that favor endangered species and other priority birds. In this work, we applied Hierarchical Modelling of Species Communities (HMSC) to study individual species responses, as well as the influence of traits on such responses, to variables related to rangeland management using birds of the Rio de la Plata Grasslands as a case study. Based on presence-absence data collected in 454 paddocks across 46 ranches we inferred the response of 69 species considering imperfect detection. This degree of detail fills a major gap in rangeland management, as species-level responses can be used to achieve targeted conservation goals other than maximizing richness or abundance. We found that artificial pastures had an overall negative impact on many bird species, whereas the presence of tussocks had a positive effect, including all threatened species. Grassland specialists were in general sensitive to grass height and tended to respond positively to tussocks but negatively to tree cover. Controlling grass height via adjustments in stocking rate can be a useful tool to favor grassland specialists. To favor a wide range of bird species in ranches, a mosaic of short and tall native grasslands with patches of tussocks and trees is desirable. We also found that species-specific responses were modulated by their traits: small-sized birds responded positively to tussocks and tree cover while large species responded negatively to increasing grass height. Ground foragers preferred short grass while birds that scarcely use this stratum were not affected by grass height. Results on the influence of traits on bird responses are an important novelty in relation to previous work in rangelands and potentially increase our predicting capacity and model transferability across grassland regions.

Differential Modulation of Catecholamine and Adipokine Secretion by the Short Chain Fatty Acid Receptor FFAR3 and α2-Adrenergic Receptors in PC12 Cells.

Sympathetic nervous system (SNS) hyperactivity is mediated by elevated catecholamine (CA) secretion from the adrenal medulla, as well as enhanced norepinephrine (NE) release from peripheral sympathetic nerve terminals. Adrenal CA production from chromaffin cells is tightly regulated by sympatho-inhibitory α2-adrenergic (auto)receptors (ARs), which inhibit both epinephrine (Epi) and NE secretion via coupling to Gi/o proteins. α2-AR function is, in turn, regulated by G protein-coupled receptor (GPCR)-kinases (GRKs), especially GRK2, which phosphorylate and desensitize them, i.e., uncouple them from G proteins. On the other hand, the short-chain free fatty acid (SCFA) receptor (FFAR)-3, also known as GPR41, promotes NE release from sympathetic neurons via the Gi/o-derived free Gßγ-activated phospholipase C (PLC)-ß/Ca2+ signaling pathway. However, whether it exerts a similar effect in adrenal chromaffin cells is not known at present. In the present study, we examined the interplay of the sympatho-inhibitory α2A-AR and the sympatho-stimulatory FFAR3 in the regulation of CA secretion from rat adrenal chromaffin (pheochromocytoma) PC12 cells. We show that FFAR3 promotes CA secretion, similarly to what GRK2-dependent α2A-AR desensitization does. In addition, FFAR3 activation enhances the effect of the physiologic stimulus (acetylcholine) on CA secretion. Importantly, GRK2 blockade to restore α2A-AR function or the ketone body beta-hydroxybutyrate (BHB or 3-hydroxybutyrate), via FFAR3 antagonism, partially suppress CA production, when applied individually. When combined, however, CA secretion from PC12 cells is profoundly suppressed. Finally, propionate-activated FFAR3 induces leptin and adiponectin secretion from PC12 cells, two important adipokines known to be involved in tissue inflammation, and this effect of FFAR3 is fully blocked by the ketone BHB. In conclusion, SCFAs can promote CA and adipokine secretion from adrenal chromaffin cells via FFAR3 activation, but the metabolite/ketone body BHB can effectively inhibit this action.

Recurrent reactive infectious mucocutaneous eruption (RIME) secondary to Chlamydophila pneumoniae infection in an adult.

The terminology surrounding the clinical syndrome characterized by acute mucositis with minimal skin involvement has been a subject of debate over time. In recent years, terms such as mycoplasma-induced rash and mucositis and reactive infectious mucocutaneous eruption (RIME) have been introduced to encompass milder mucocutaneous diseases associated with respiratory infections, with implications for management and prognosis. We report the first case of recurrent RIME associated with Chlamydophila pneumoniae infection in an adult patient. RIME is likely underreported due to misclassification and a lack of testing for potential pathogens. Early recognition of recurrent RIME is of particular interest from the patient's perspective to reduce the frequency and duration of hospital admissions.

Reliability of different body temperature measurement sites during normothermic cardiac surgery.

INTRODUCTION: Patients undergoing cardiac surgery can experience significant thermal changes during the perioperative period and, for that reason, it is essential to monitor temperatures with adequate accuracy and precision during cardiopulmonary bypass (CPB). The primary aim of the current study was to measure the discrepancies between temperatures at different body sites during normothermic or mild hypothermic CPB. METHODS: 48 patients undergoing cardiac surgery participated in our study. Simultaneous temperatures were measured at nasopharynx, pulmonary artery, arterial outlet, venous inlet, forehead using a heat flux sensor, and urinary bladder at 5-min intervals throughout surgery. The Bland-Altman plot for repeated measures was used to assess concordance between methods. RESULTS: The duration of surgery was 360 min (interquartile range (IQR) 300-412), while the median cross-clamp time was 135 min (IQR 101-169). During the CPB time, the average difference between arterial outlet and nasopharyngeal temperature was -0.16°C (95% limits of agreement of ±0.93). The bias between arterial outlet and the venous inflow was 0.16°C and the 95% limits of agreement were -0.63 to 0.95°C. The Bland-Altman analysis showed an average difference between oxigenator arterial outlet and bladder probe of -0.62 (95% limits of agreement of ±1.3). The average difference between arterial outlet and Tcore™ temperatures was 0.08°C (95% limits of agreement of ±1.46). 25 patients (52.08%) presented nasopharyngeal temperatures higher than 37°C in the post-CPB period, but none of them exceeded 38°C. CONCLUSIONS: Perfusionists should be cautious when using the nasopharyngeal site as the only surrogate of brain temperature, even in normothermic cardiac surgery because the precision of measurements is not entirely adequate.

Media Trust and the COVID-19 Pandemic: An Analysis of Short-Term Trust Changes, Their Ideological Drivers and Consequences in Switzerland.

We analyze short-term media trust changes during the COVID-19 pandemic, their ideological drivers and consequences based on panel data in German-speaking Switzerland. We thereby differentiate trust in political information from different types of traditional and non-traditional media. COVID-19 serves as a natural experiment, in which citizens' media trust at the outbreak of the crisis is compared with the same variables after the severe lockdown measures were lifted. Our data reveal that (1) media trust is consequential as it is associated with people's willingness to follow Covid-19 regulations; (2) media trust changes during the pandemic, with trust levels for most media decreasing, with the exception of public service broadcasting; (3) trust losses are hardly connected to ideological divides in Switzerland. Our findings highlight that public service broadcasting plays an exceptional role in the fight against a pandemic and that contrary to the US, no partisan trust divide occurs.

Distance Education for children with a disability and/or from vulnerable families.

Different reasons may hinder the right to education of children with a disability and/or from vulnerable families; for example, living in rural areas where they do not have access to public transport, restriction of opportunities to access schools, precarious economic situations, lack of minimum services that meet the needs of hygiene and personal care, among other reasons. This has made it necessary to implement changes in traditional education systems. For this reason, the objective of this study is to build a theoretical base that facilitates the understanding of the requirements of these children in terms of distance education. To do this, the authors interviewed primary education teachers who work with children with a disability and/or from vulnerable families. A qualitative research methodology was used with the focus group as a technique for collecting information. Ten primary school teachers participated in an interview made up of a set of open-ended questions that facilitated their narratives regarding education strategies used for students with a disability and/or those living in vulnerable families, which were later grouped into categories. As a result of this research, the following strategies used by the participating teachers were collected: recreating the feeling of the physical classroom in the virtual classroom, fostering friendship and companionship among students, designing engagement strategies, and considering the socio-cultural variables that influence learning. The teachers assured that distance education is a perfectly viable alternative for children with a disability and/or from vulnerable families.

Atypical presentation of immunoglobulin G4-related disease as subglottic stenosis: a case-based review.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory pathology that has been reported to affect principally the retroperitoneum, hepatobiliary system, salivary glands, orbital structures or lymph nodes. However, IgG4-RD with laryngeal involvement is a very rare entity. Our aims were to describe a case of subglottic stenosis as first and only manifestation of IgG4-RD and review the literature. A patient with IgG4-RD affecting the larynx that presented as subglottic stenosis is described. A MEDLINE database search of IgG4-RD cases with laryngopharyngeal manifestations was also conducted. A 30-year-old Caucasian woman was referred to a tertiary care hospital for dyspnea on exertion, which had been increasing for the last 4 months. Medical and surgical procedures revealed a subglottic stenosis, with a histological finding of IgG4 positive plasma cell infiltration. There was no evidence of other organ involvement. She was successfully treated with oral glucocorticoids and rituximab infusions. Glucocorticoids were rapidly tapered and the rituximab regimen was optimized, with no evidence of relapses. In the literature review, we found a total of 12 reported cases with laryngopharyngeal involvement, two of them with subglottic stenosis. IgG4-RD of the larynx is rare but should be considered after excluding more common disorders.

The Isolated Mucosa of the Rat Colon Decellularization, Microscopy and Cell Cultures.

This project presents the most important findings of the studies, which we carried out in our laboratory on the decellularization of the rat isolated colonic mucosa. We have also included some details of the experiences gathered with the muscle layer as well as the whole wall of the colon. The question of the cytocompatibility of this new substrate has been addressed with the application of primary cultures of human cells and well-established cell lines. The possible applications in experimental and medical settings will be discussed.

Changes in the profile of newly HIV-diagnosed men who have sex with men, Madrid, 2014 to 2019.

IntroductionKnowing the factors associated with HIV transmission is necessary in order to design preventive programmes tailored to the epidemiological situation in each region and population.AimOur objective was to study the sociodemographic, clinical and behavioural characteristics of men who have sex with men (MSM) who were newly diagnosed with HIV infection.MethodsWe carried out an observational, descriptive, study on all MSM newly diagnosed with HIV infection in one clinic for sexually transmitted infections (STI) and HIV clinic in Madrid between 2014 and 2019. Information on sociodemographic, clinical, and behavioural characteristics of participants per year of diagnosis was collected.ResultsWe detected a total of 1,398 people with HIV infection, 253 of whom were recent seroconverters (rSCV) with a median duration of documented seroconversion of 6 months. From the total, 97.9% infections were sexually transmitted and 2.1% involved injected drugs, i.e. slam practices. The average age was 32.9 years (range: 15.6-74.9), 51.8% were Spanish and 40% Latin American. These diagnoses decreased in Spanish people and increased in Latin Americans during the study period. Of the rSCV, 73.9% had condomless sex under the influence of drugs and 28.9% participated in chemsex sessions. Apps were used by 92.6% rSCV for sexual encounters and 70.4% of them attributed HIV transmission to their use.ConclusionsCombination of HIV prevention strategies, as pre-exposure prophylaxis, should be reinforced among young MSM, especially those born in Latin America, those who use drugs for sex, and those who use apps in search of sexual contacts.

Can network metrics predict vulnerability and species roles in bird-dispersed plant communities? Not without behaviour.

Network metrics are widely used to infer the roles of mutualistic animals in plant communities and to predict the effect of species' loss. However, their empirical validation is scarce. Here we parameterized a joint species model of frugivory and seed dispersal with bird movement and foraging data from tropical and temperate communities. With this model, we investigate the effect of frugivore loss on seed rain, and compare our predictions to those of standard coextinction models and network metrics. Topological coextinction models underestimated species loss after the removal of highly linked frugivores with unique foraging behaviours. Network metrics informed about changes in seed rain quantity after frugivore loss. However, changes in seed rain composition were only predicted by partner diversity. Nestedness, closeness, and d' specialisation could not anticipate the effects of rearrangements in plant-frugivore communities following species loss. Accounting for behavioural differences among mutualists is critical to improve predictions from network models.

Telemedicine experience between two hospitals from Angola and Spain.

OBJECTIVE: Telemedicine uses information and communication technologies to provide services in the field where the distance is a critical factor. The aim of the present study is to describe the experience of a synchronous telemedicine between two hospitals in Spain and Angola. METHODS: This is a retrospective observational study of all synchronous telemedicine sessions conducted between the Hospital Nossa Senhora da Paz in Angola and the Vall d'Hebron University Hospital in Spain from January 2011 to December 2014. RESULTS: Seventy-two cases were discussed in the telemedicine sessions. The average age of patients was 18.02 (SD 13.75) years and mostly women (54.38 percent). Reasons to discuss the cases were 46.47 percent doubts in the diagnosis and therapeutic management, 15.47 percent were purely formative cases, and only 8.45 percent treatment doubt. At the time of presentation, 29 percent of the patients were already diagnosed, 95 percent of whom with infectious disease diagnostic, and from the undiagnosed patients 36 percent presented a febrile syndrome. CONCLUSION: This study shows the viability of synchronous telemedicine between European and African countries without an excessively sophisticated technology.

Evaluation of the novel non-invasive zero-heat-flux Tcore™ thermometer in cardiac surgical patients.

Tcore™ Sensor is a novel zero-heat-flux thermometer that estimates core temperature from skin over forehead. We tested the hypothesis that this system estimates core temperature to an accuracy within 0.5 °C. 40 cardiac surgical patients were enrolled (960 measurements). Reference core temperatures were measured in nasopharynx, pulmonary artery and the arterial branch of the oxygenator of the cardiopulmonary bypass (CPB) circuit. 95% Bland-Altman limits of agreement for repeated measurement data was used to study the agreement between Tcore™ thermometer and the reference methods. The proportion of all differences that were within 0.5 °C and Lin's concordance correlation coefficient (LCCC) were estimated as well. The mean overall difference between Tcore™ and nasopharyngeal temperature was - 0.2 ± 0.5 °C (95% limits of agreement of ± 1.09). The proportion of differences within 0.5 °C was 68.80% (95% CI 65.70-71.70%) for nasopharyngeal reference. LCCC was 0.84 (95% CI 0.83-0.86). The mean bias between Tcore™ and the temperature measured in the pulmonary artery was - 0.2 ± 0.5 °C (95% limits of agreement of ± 1.16). 55.30% of measurements were ≤ 0.5 °C (95% CI 51.40-59.20%). LCCC was 0.60 (95% CI 0.56-0.64). The average difference between Tcore™ and the temperature measured at the arterial outlet during the CPB period was - 0.1 ± 0.7 °C (95% limits of agreement of ± 1.43). The proportion of differences within 0.5 °C was 54.40% (95% CI 48.80-60.00%). LCCC was 0.74 (0.69-0.79). Cutaneous forehead zero-flux temperatures were not sufficiently accurate for routine clinical use in the cardiac surgical population.

Ultrasound findings and associated factors to morbidity in Schistosoma haematobium infection in a highly endemic setting.

OBJECTIVE: To evaluate the usefulness of the WHO classification of ultrasound pathological changes and to establish risk factors for morbidity in a highly endemic setting. METHODS: One hundred and fifty-seven ultrasounds were performed on school-aged children previously diagnosed with urinary schistosomiasis in Cubal, Angola. The findings were analysed according to the WHO guidelines. Factors for morbidity were studied. RESULTS: Mean age of the children was 8.7 (SD 3.2) years. Pathological changes were found in 85.3% (84.7% in the bladder, 34.4% the ureter and 6.3% kidney lesions). The global score according to the WHO classification was 5.74. Male gender [OR 2.61 (1.04-6.58); P 0.043] and older age [OR 2.96 (1.17-7.46); P 0.023] were associated with a higher risk of developing any kind of urinary abnormality. Proteinuria was present in 61.7% of the children. Macroscopic haematuria [OR 2.48 (1.11-5.58); P = 0.02)] and a high level of proteinuria > 300 mg/dl [OR 5.70 (2.17-14.94); P 300 mg/dl)] were associated with abnormalities of the upper urinary tract and showed good positive and negative predictive values for the detection of pathology in the upper urinary tract (65.5% and 71.1%, respectively). CONCLUSIONS: Severe urinary tract pathology was found in a high percentage of the children in our setting. Microhaematuria and proteinuria were good markers of morbidity, proteinuria being more precise for severe alterations of the upper urinary tract. We suggest initial and evolutive ultrasound in children diagnosed with schistosomiasis, and close monitoring including periodic controls. As schistosomiasis control efforts are currently focused on reducing morbidity, tests that detect the presence or degree of morbidity are essential for targeting treatment and tracking the progress of control campaigns.

Cannabinoid Receptors, Mental Pain and Suicidal Behavior: a Systematic Review.

PURPOSE OF REVIEW: The current serotonin-based biological model of suicidal behavior (SB) may be too simplistic. There is emerging evidence that other biomarkers and biological systems may be involved in SB pathophysiology. The literature on the endocannabinoid (EC) systems and SB is limited. The objective of the present article is to review all available information on the relationship between cannabinoid receptors (CB1 and CB2 receptors), and SB and/or psychological pain. RECENT FINDINGS: Our review is limited by the small number and heterogeneity of studies identified: (1) an autopsy study describing elevated levels of CB1 receptor activity in the prefrontal cortex and suicide in both depression and alcoholism and (2) studies supporting the involvement of both CB1 and CB2 receptors in the regulation of neuropathic pain and stress-induced analgesia. We conclude that cannabinoid receptors, particularly CB1 receptors, may become promising targets for the development of novel therapeutic tools for the treatment of SB.

To whom is HIV pre-exposure prophylaxis proposed? / ¿A quién proponer la profilaxis preexposición al virus de la inmunodeficiencia humana?

INTRODUCTION: HIV Pre-Exposure Prophylaxis (PrEP) consists of administering antiretroviral drugs to seronegative individuals with high risk practices. The aim of the study was to describe the characteristics of recent seroconverted HIV patients in order to determine the profile of the appropriate candidates for PrEP. METHODS: A descriptive study of all patients diagnosed with HIV infection in 2014, and who had achieved a documented negative serology over the previous 12 months. A specific form was completed to determine the sociodemographic, behavioural, and clinical features, with complementary tests being performed for other sexually transmitted infections. RESULTS: Almost all (98.4%) of the 61 recent seroconverted were men who have sex with men, and aged between 20 to 39 years (88.5%). They also had a background of sexually transmitted infections (80.3%), performed multiple and unprotected sexual practices (82.7%), and under the effect of recreational drugs (87%). CONCLUSIONS: The evaluation of the risk factors for HIV infection in seronegative patients should enable the appropriate candidates for PrEP to be identified.

A Case of Persistent and Possibly Treatment Resistant Pharyngeal Gonorrhea.

An HIV-negative man with pharyngeal gonorrhea had a positive test-of-cure (nucleic acid amplification test) result 7 days after treatment with ceftriaxone/azithromycin. Neisseria gonorrhoeae Multi-Antigen Sequencing Type 1407 and mosaic pen A (XXXIV) gene were identified in the test-of-cure specimen, and culture was negative. Retreatment with ceftriaxone 500 mg intramuscularly plus azithromycin 2 g orally yielded a negative test-of-cure result.

Changes in malaria epidemiology in a rural area of Cubal, Angola.

BACKGROUND: Scarce information about malaria epidemiology in Angola has been published. The objective of this study is to describe the epidemiology of malaria at the Hospital Nossa Senhora da Paz (Cubal, Angola) and the fatality rate due to malaria (total and in children under five years) in the last five years. METHODS: A retrospective, observational study was performed at the Hospital Nossa Senhora da Paz, a 400-bed rural hospital located in Benguela Province of Angola. The study population included all patients who attended the hospital from January 2009 to December 2013. Outcome variables were calculated as follows: the percentage of malaria cases (number of positive thick blood films, divided by the total thick blood films performed); the percentage of in-patients for malaria (number of in-patients diagnosed with malaria, divided by the total number of in-patients); and, the fatality rate (number of deaths due to malaria divided by the number of positive thick blood films). RESULTS: Overall, 23,106 thick blood films were performed, of which 3,279 (14.2%) were positive for Plasmodium falciparum infection. During this five-year period, a reduction of 40% (95% CI 37-43%, p < 0.001) in the malaria-positive slides was detected. Distribution of positive-malaria slides showed a seasonal distribution with a peak from December to March (rainy season). An average annual reduction of 52% (95% CI 50-54%, p < 0.001) in the admissions due to malaria was observed. The overall fatality rate due to malaria was 8.3%, and no significant differences in the annual fatality rate were found (p = 0.553). CONCLUSIONS: A reduction in the number of malaria cases and the number of admissions due to malaria has been observed at the Hospital Nossa Senhora da Paz, during the last five years, and incidence along the study period showed a seasonal distribution. All this information could be useful when deciding which malaria control strategies have to be implemented in this area.

Utility of intracardiac echocardiography for catheter ablation of complex cardiac arrhythmias in a medium-volume training center.

AIMS AND OBJECTIVES: New electrophysiology tools like intracardiac echocardiography (ICE) might help to minimize and early detect complications during cardiac ablation procedures. The aim of the study was to assess the utility and vascular safety of ICE during catheter ablation of complex cardiac arrhythmias in a medium-volume training center. METHODS: Prospective, observational study consisted of consecutive patients who underwent catheter-based ablation of complex cardiac arrhythmias. All procedures were performed using three-dimensional electro-anatomical mapping and routine cannulation of right and left femoral veins. The ICE probe was initially positioned at the mid-level of the right atrium and properly moved to monitor different steps of the procedure and identify complications. All procedure-related vascular complications were registered. RESULTS: One hundred two patients (age 61.4 ± 13.1 years, 69 male) underwent 110 ablation procedures. Pulmonary vein isolation was the most common ablation substrate (55.4%). Ventricular tachycardia (17.2%) and left atrial flutter procedures (16.4%) were also common. The use of ICE enabled us to early initiate anticoagulation and to optimize the transseptal puncture. It also provided the capability to early detect life-threatening complications such as tamponade (3.6%), along with important information during the procedure such as exact catheter location, lesion formation, and stability during radiofrequency delivery. Such benefits were not associated with a higher number of vascular complications. CONCLUSION: The use of ICE during catheter-based ablation of complex cardiac substrates provides technical features that may decrease complications and increase accuracy while applying radiofrequency, especially in training centers where fellows start to perform complex procedures.