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Background: Several studies have demonstrated the utility of methylene blue (MB) to treat vasoplegic syndrome (VS), but some have cautioned against its routine use in lung transplantation with only two cases described in prominent literature. Cystic fibrosis patients commonly have chronic infections which predispose them to a systemic inflammatory syndrome-like vasoplegic response during lung transplantation. We present 13 cystic fibrosis patients who underwent lung transplantation and received MB for vasoplegic syndrome while on cardiopulmonary bypass, with or without inhaled pulmonary vasodilator therapy. Methods: Single-center, retrospective, case series analysis of cystic fibrosis patients who underwent lung transplant and received MB for vasoplegia. We defined the primary outcome as 30-day mortality, and secondary outcomes as primary graft failure, 1-year mortality, postoperative complications, and hemodynamic response to MB. Results: MB was associated with a significant increase in mean arterial pressure (MAP) (P < 0.001) in all patients, and 84.6% (11/13) of the patients had either a decrease or no change in vasopressor requirement. No patients developed acute primary graft dysfunction and there was 100% 30-day and 1-year survival. One patient required Extracorporeal membrane oxygenation (ECMO) for hypoxemia and 69% (9/13) of the patients had evidence of postoperative right ventricular dysfunction, but no patients required a right ventricular assist device. Conclusion: This case series demonstrates the effectiveness of MB in treating vasoplegia in cystic fibrosis patients during lung transplantation, without evidence of primary graft dysfunction, 30-day or 1-year mortality. The safety of MB regarding hypoxemia and increased pulmonary vascular resistance requires further investigation.

Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4 , which encodes the farnesoid X receptor (FXR), a bile acid-activated nuclear hormone receptor that regulates bile acid metabolism. Clinical features of severe, persistent NR1H4 -related cholestasis include neonatal onset with rapid progression to end-stage liver disease, vitamin K-independent coagulopathy, low-to-normal serum gamma-glutamyl transferase activity, elevated serum alpha-fetoprotein and undetectable liver bile salt export pump ( ABCB11 ) expression. Our findings demonstrate a pivotal function for FXR in bile acid homeostasis and liver protection. Neonatal cholestasis is a result of elevated bile acid levels, and is associated with mutations in genes regulating bile acid homeostasis. Here the authors identify mutations in the bile acid sensing farnesoid X receptor in four individuals with neonatal cholestasis from two unrelated families.

This study evaluates the ability of Large Language Models (LLMs) to summarize real-world dialogues between patients and the healthcare team of an e-health company that provides digital healthcare services, primarily communicating via WhatsApp. The team needs quick access to patient information to deliver accurate and personalized responses. Summarizing past messages is the approach examined here, aiming for concise, non-redundant, and truthful summaries that capture the main dialogue characteristics despite facing (real-world) noisy and informal content in an under-represented language - Portuguese. To do so, we collected an anonymized Portuguese dataset of WhatsApp messages exchanged between patients and the healthcare team. Dialogue quality was assessed for size, readability, and correctness before generating summaries with LLaMA3 and Qwen2 using specific prompts. Volunteers evaluated these summaries on coverage, relevance, redundancy, and veracity using a 5-point Likert scale. Our qualitative and quantitative experimental results indicate that LLMs can produce effective summaries of dialogues between patients and healthcare teams, even when faced with low-quality data in an underrepresented language. This is a surprising result due to the challenging scenario. Among the tested LLMs, LLaMA3 demonstrated a slight edge over QWen2 in coverage and veracity among the evaluated methods. Our results demonstrate a potential to build real-world practical services to assist healthcare professionals in responding to patient messages with agility, clarity, and cohesion, enhancing both communication efficiency and patient satisfaction. Ultimately, the advocated approach could significantly improve the landscape of online healthcare communication, particularly in resource-constrained settings like Brazil, where access to primary care is limited.

Structural changes in the spleen have been reported in several infectious diseases. In visceral leishmaniasis (VL), a severe parasitic disease caused by Leishmania spp., the loss of white pulp accompanies a severe clinical presentation. Hamster model reproduces aspects of human VL progression. In the early stages, a transcriptomic signature of leukocyte recruitment was associated with white pulp hyperplasia. Subsequently, impaired leukocyte chemotaxis with loss of T lymphocytes in the periarteriolar lymphoid sheath occurred. This differential gene expression was subsequently corroborated by transcriptomic profiling of spleens in severe human VL. At the latest stage, spleen disorganization was associated with increasing clinical signs of VL. White pulp disruption was accompanied by decreased DLK1 expression. The expression of CXCL13, CCR5, CCL19, CCR6, CCR7 and LTA decreased, likely regulated by CDKN2A overexpression. Our findings enlighten a pathway implying cell cycle arrest and decreased gene expression involved in spleen organization.

Abstract It is repeatedly stressed the need to characterize the extant biodiversity in tropical ecosystems. However, inventory studies are still progressing slowly in dry ecosystems, leading to the underestimation of their true biodiversity and hindering conservation efforts. In this study, we present primary and secondary data, along with an updated list of amphibians and reptiles from two localities in the São Francisco-Gurguéia region in Piauí. Additionally, we compare the species composition between nine areas within the Caatinga, which were sampled using standardized methods over the past ten years, to examine broader spatial patterns of community composition. To survey reptiles and amphibians, we employed similar methods and sampling efforts in two areas within the Serra das Confusões National Park (SCNP) region. Our surveys recorded a total of 73 species of amphibians and reptiles, of which 24 are new distribution records for the SCNP region. Consequently, our findings increase the known herpetofauna in the region to 94 species. Despite their proximity, the two sites in the SCNP region exhibited only 42% similarity in species composition, and they differed significantly from other areas within the Caatinga. Furthermore, even the closer Caatinga areas presented differences in species composition, highlighting the necessity to evaluate biodiversity across the landscape and contribute to understanding biogeographic patterns. Resumo É repetidamente enfatizada a necessidade de caracterizar a biodiversidade vivente em ecossistemas tropicais. No entanto, os estudos de inventário ainda estão progredindo lentamente em ecossistemas secos, levando à subestimação de sua verdadeira biodiversidade e dificultando os esforços de conservação. Neste estudo, apresentamos dados primários e secundários, juntamente com uma lista atualizada de anfíbios e répteis de duas localidades na região de São Francisco-Gurguéia, do Piauí. Além disso, comparamos a composição de espécies entre nove áreas dentro da Caatinga, que foram amostradas usando métodos padronizados nos últimos dez anos, para examinar padrões espaciais mais amplos de composição da comunidade. Para estudar répteis e anfíbios, utilizamos métodos e esforços de amostragem semelhantes em duas áreas na região do Parque Nacional da Serra das Confusões (PNSC). Nossos levantamentos registraram um total de 73 espécies de anfíbios e répteis, das quais 24 são novos registros de distribuição para a região do PNSC. Consequentemente, nossos resultados aumentam a herpetofauna conhecida na região para 94 espécies. Apesar da proximidade, os dois locais na região do PNSC exibiram apenas 42% de similaridade na composição de espécies e diferiram significativamente de outras áreas dentro da Caatinga. Mesmo áreas mais próximas da Caatinga apresentaram diferenças na composição de espécies, destacando a necessidade de avaliar a biodiversidade em toda a paisagem e contribuir para a compreensão de padrões biogeográficos.

Giant tortoises are among the longest-lived vertebrate animals and, as such, provide an excellent model to study traits like longevity and age-related diseases. However, genomic and molecular evolutionary information on giant tortoises is scarce. Here, we describe a global analysis of the genomes of Lonesome George—the iconic last member of Chelonoidis abingdonii —and the Aldabra giant tortoise ( Aldabrachelys gigantea ). Comparison of these genomes with those of related species, using both unsupervised and supervised analyses, led us to detect lineage-specific variants affecting DNA repair genes, inflammatory mediators and genes related to cancer development. Our study also hints at specific evolutionary strategies linked to increased lifespan, and expands our understanding of the genomic determinants of ageing. These new genome sequences also provide important resources to help the efforts for restoration of giant tortoise populations. The genomes of two long-lived giant tortoises, including Lonesome George, reveal candidate genes and pathways associated with their development, gigantism and longevity.