Sickle cell maculopathy : identification of systemic risk factors, and microstructural analysis of individual retinal layers of the macula

PURPOSE:To identify systemic risk factors for sickle cell maculopathy, and to analyze the microstructure of the macula of Sickle Cell Disease (SCD) patients by using automated segmentation of individual retinal layers. METHODS: Thirty consecutive patients with SCD and 30 matched controls underwent spectral-domain optical coherence tomography (SD-OCT) and automated thickness measurement for each retinal layer; thicknesses for SCD patients were then compared to normal controls. Demographic data, systemic data, and lab results were collected for each SCD patient; multivariate logistic regression analysis was used to identify potential risk factors for sickle cell maculopathy. RESULTS: Ongoing chelation treatment (p = 0.0187) was the most predictive factor for the presence of sickle cell maculopathy; the odds were 94.2% lower when chelation was present. HbF level tended to influence sickle cell maculopathy (p = 0.0775); the odds decreased by 12.9% when HbF increased by 1%. Sickle cell maculopathy was detected in 43% of SCD patients as patchy areas of retinal thinning on SD-OCT thickness map, mostly located temporally to the macula, especially in eyes with more advanced forms of sickle cell retinopathy (p = 0.003). In comparison to controls, SCD patients had a subtle thinning of the overall macula and temporal retina compared to controls (most p<0.0001), involving inner and outer retinal layers. Thickening of the retinal pigment epithelium was also detected in SCD eyes (p<0.0001). CONCLUSIONS: Chronic chelation therapy and, potentially, high levels of HbF are possible protective factors for the presence of sickle cell maculopathy, especially for patients with more advanced forms of sickle cell retinopathy. A subtle thinning of the overall macula occurs in SCD patients and involves multiple retinal layers, suggesting that ischemic vasculopathy may happen in both superficial and deep capillary plexi. Thinning of the outer retinal layers suggests that an ischemic insult of the choriocapillaris may also occur in SCD patients

No difference in the outcome of metastatic thyroid cancer patients when using recombinant or endogenous TSH

Objective: At the present, recombinant TSH cannot be used for the treatment of metastatic differentiated thyroid cancer patients. Aim of this study was to evaluate if the type of TSH stimulation, recombinant or endogenous, had an impact on the outcome of these patients. Design and methods: We compared the outcome of two propensity score-matched groups of metastatic patients, stimulated by either only recombinant TSH (n=43) or only endogenous TSH (n=34). Results: As expected from the matching procedure, the clinical-pathological features and the cumulative 131I-activities administered of the two groups were very similar. After 4 years of follow-up 4% of patients were cured, 3% had biochemical disease and 93% had structural disease. However, 91% of patients obtained a clinical benefit from this therapy in terms of stabilization of the disease or complete remission or partial response. When considering the two groups separately we did not find any difference in their outcome. When considering the response to 131-I therapy of the single type of metastases, 8% of lymph node metastases and 8% of lung metastases disappeared but none of bone metastases. The response to 131-I therapy of the single type of metastases was similar when we looked at the two groups separately. Conclusions: this study shows a) an overall clinical benefit of the 131-I therapy since the majority of patients remained affected but with a stable disease b) the preparation with either recombinant or endogenous TSH has no impact on the 131-I therapy efficacy and the outcome of our two groups of patients

Multifunctionality of the [C2mim][Ln(fod)4] series (Ln = Nd-Tm except Pm):magnetic, luminescent and thermochemical studies

A series of nine tetrakis lanthanide β-diketonate complexes of the type [C2mim][Ln(fod)4] (C2mim = 1-ethyl-3-methylimidazolium, fod = 6,6,7,7,8,8,8-heptafluoro-2,2-dimethyl-3,5-octanedionate) were prepared, with yields above 80%, and their thermochemical, photophysical and magnetic susceptibilities were evaluated. Thermochemical studies presented a rare and reversible conversion between two solid phases (polymorphism), characteristic of the [Ln(fod)4]− anion. Photophysical and magnetic studies revealed that Dy and Er presented the multifunctionality of being simultaneously SMMs and visible (Dy) or near infra-red (Er) emitters. The Nd, Ho and Tm analogues present characteristic emission bands in the NIR region (800–1200 nm), while Sm, Eu, Tb and Dy present emissions in the visible range. Magnetic susceptibility of Tb, Dy, Ho, Er and Tm salts were measured in the temperature range of 2–300 K, showing paramagnetic behaviour, although with different regimes, with AC susceptibility measurements, at different frequencies in the range of 10–10 000 Hz, providing evidence of slow magnetic relaxation processes for Gd, Dy and Er analogues with SMM behavior.publishe

Registros

Compreender as mutações do ecossistema midiático tem sido uma prática na academia, especialmente no campo das ciências sociais aplicadas. As transformações têm sido constantemente observadas e podem ser vistas em publicações de diversas formas midiáticas. Entretanto, pela liquidez desse cenário, os estudos não se esgotam e tornam-se fundamentais para que a relação sociedade e meios exista de maneira harmônica e eficaz. Mas essa preocupação não é algo de hoje. No início da década de 1970, na cidade de Nova Iorque, surgia pelas mãos de Neil Postman e Marshall McLuhan o programa de doutorado em Ecologia dos Meios, impulsionado pela preocupação em desenvolver as teorias relacionadas a essa corrente e, em seguida sustentar estudos futuros. No escopo, encontravam-se olhares direcionados à rádio, à fotografia, ao cinema, aos jornais, às revistas, aos livros, à televisão e a todos os ambientes e processos aos quais esses canais ou meios estavam relacionados. Processos que compunham uma galáxia composta por uma, ou algumas, aldeias globais. Uma aldeia onde o meio era a mensagem. Porém, nos dias atuais, os estudos sobre ecologia dos meios tornam-se ainda mais relevantes. As estruturas comunicacionais são construídas a partir de novas tramas de atores midiáticos. A potencialização dos processos comunicacionais cresceu exponencialmente. O poder midiático, por sua vez, ganhou outro status. Com isso tudo, novas linguagens comunicacionais passaram a ser adotadas pela sociedade.http://www.riaeditorial.com/index.php/registros/Compreender as mutações do ecossistema midiático tem sido uma prática na academia, especialmente no campo das ciências sociais aplicadas. As transformações têm sido constantemente observadas e podem ser vistas em publicações de diversas formas midiáticas. Entretanto, pela liquidez desse cenário, os estudos não se esgotam e tornam-se fundamentais para que a relação sociedade e meios exista de maneira harmônica e eficaz. Mas essa preocupação não é algo de hoje. No início da década de 1970, na cidade de Nova Iorque, surgia pelas mãos de Neil Postman e Marshall McLuhan o programa de doutorado em Ecologia dos Meios, impulsionado pela preocupação em desenvolver as teorias relacionadas a essa corrente e, em seguida sustentar estudos futuros. No escopo, encontravam-se olhares direcionados à rádio, à fotografia, ao cinema, aos jornais, às revistas, aos livros, à televisão e a todos os ambientes e processos aos quais esses canais ou meios estavam relacionados. Processos que compunham uma galáxia composta por uma, ou algumas, aldeias globais. Uma aldeia onde o meio era a mensagem. Porém, nos dias atuais, os estudos sobre ecologia dos meios tornam-se ainda mais relevantes. As estruturas comunicacionais são construídas a partir de novas tramas de atores midiáticos. A potencialização dos processos comunicacionais cresceu exponencialmente. O poder midiático, por sua vez, ganhou outro status. Com isso tudo, novas linguagens comunicacionais passaram a ser adotadas pela sociedade

Prevalence and geographical distribution of bovine sexually transmitted diseases in the province of Formosa, Argentina

La campilobacteriosis genital bovina (CGB) y la tricomonosis bovina (TB) son enfermedades de transmisión sexual (ETS) que afectan a los rodeos de cría bovina y disminuyen su eficiencia reproductiva. El objetivo de este trabajo fue estimar la prevalencia de estas enfermedades y su distribución témporo-espacial en la provincia de Formosa, Argentina. El estudio fue transversal, se desarrolló durante 2018-2021 e incluyó 15.571 toros. Se encontró una prevalencia de CGB y TB inter-rodeo de 29,62 y 17,23%, respectivamente. La prevalencia de animales positivos fue de 2,05% para CGB y de 0,43% para TB. El análisis témporo-espacial de la CGB mostró dos agrupaciones espaciales, una de bajo riesgo (RR = 0,13; p < 0,001; 2018-2021) y otra de alto riesgo (RR = 2,84; p < 0,001; 2020-2021) de contraer la enfermedad. La TB presentó una agrupación de alto riesgo de contraer la enfermedad (RR = 35,24; p < 0,001; 2019). Este estudio muestra que las ETS son endémicas en la región y aporta información actualizada y de interés como herramienta para el manejo sanitario.Bovine genital campylobacteriosis (BGC) and bovine trichomonosis (BT) are sexually transmitted diseases (STDs) that affect bovine breeding herds, decreasing their reproductive efficiency. The objective of this work was to estimate the prevalence of these diseases and their temporal-spatial distribution in the province of Formosa, Argentina. The cross-sectional study conducted between 2018 and 2021 included a total of 15,571 bulls, inter-herd prevalence being 29.62% and 17.23% for BGC and BT, respectively. The prevalence of positive animals was 2.05% for BGC and 0.43% for BT. The temporal-spatial analysis of BGC showed two distinct spatial groupings, one group had a low risk of contracting the disease (RR = 0.13; p < 0.001; 2018–2021) while the other group had a high risk (RR = 2.84; p < 0.001; 2020–2021). BT had a high-risk group for the disease (RR = 35.24; p < 0.001; 2019). This study shows that STDs are endemic in the region, providing updated and valuable information as a tool for the health management of these diseases.Fil: Viola, María Nair. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro de Investigaciones y Transferencia de Formosa. Provincia de Formosa. Centro de Investigaciones y Transferencia de Formosa. Universidad Nacional de Formosa. Centro de Investigaciones y Transferencia de Formosa; ArgentinaFil: Elías, Iris Carolina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro de Investigaciones y Transferencia de Formosa. Provincia de Formosa. Centro de Investigaciones y Transferencia de Formosa. Universidad Nacional de Formosa. Centro de Investigaciones y Transferencia de Formosa; ArgentinaFil: Signorini Porchietto, Marcelo Lisandro. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Instituto Nacional de Tecnología Agropecuaria. Centro Regional Santa Fe. Estación Experimental Agropecuaria Rafaela; ArgentinaFil: Molineri, Ana Inés. Instituto Nacional de Tecnología Agropecuaria. Centro Regional Santa Fe. Estación Experimental Agropecuaria Rafaela; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Russo, Ana Maria. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro de Investigaciones y Transferencia de Formosa. Provincia de Formosa. Centro de Investigaciones y Transferencia de Formosa. Universidad Nacional de Formosa. Centro de Investigaciones y Transferencia de Formosa; ArgentinaFil: Zimmer, Patricia Andrea. Instituto Nacional de Tecnologia Agropecuaria. Centro Regional Chaco-formosa. Estacion Experimental Agropecuaria El Colorado. Agencia de Extension Rural Formosa.; ArgentinaFil: Lozina, Laura Analía. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro de Investigaciones y Transferencia de Formosa. Provincia de Formosa. Centro de Investigaciones y Transferencia de Formosa. Universidad Nacional de Formosa. Centro de Investigaciones y Transferencia de Formosa; ArgentinaFil: Giménez, Juana Noemí. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro de Investigaciones y Transferencia de Formosa. Provincia de Formosa. Centro de Investigaciones y Transferencia de Formosa. Universidad Nacional de Formosa. Centro de Investigaciones y Transferencia de Formosa; Argentin

OFICINA DE EXTRAÇÃO DE PIGMENTOS VEGETAIS E PINTURA EM TELA

A oficina de extração de pigmentos vegetais foi desenvolvida com asturmas de 7º ano do Colégio UNESC, dentro do conteúdo do Reino das Plantase teve como objetivo desenvolver conceitos científicos sobre as cores e seuspigmentos na disciplina de ciências. A atividade consiste em coletar diversostipos de elementos naturais e macerá-los com álcool a fim de obter o pigmentoque será utilizado na pintura das telas. Com o uso desta oficina, pode-setrabalhar de forma mais clara alguns conceitos científicos de ciências no Reinodas Plantas, além de remeter a história. Com a utilização desta metodologia oprocesso de aprendizagem tornou-se mais prazeroso despertando acuriosidade, uma vez que, durante todo o desenvolvimento, os envolvidosbuscaram responder as suas dúvidas, muitas vezes sem ter a exata noção doprocesso, que permitia, contudo, avançar, pelo aguçamento da curiosidade

SNUPN deficiency causes a recessive muscular dystrophy due to RNA mis-splicing and ECM dysregulation

SNURPORTIN-1, encoded by SNUPN, plays a central role in the nuclear import of spliceosomal small nuclear ribonucleoproteins. However, its physiological function remains unexplored. In this study, we investigate 18 children from 15 unrelated families who present with atypical muscular dystrophy and neurological defects. Nine hypomorphic SNUPN biallelic variants, predominantly clustered in the last coding exon, are ascertained to segregate with the disease. We demonstrate that mutant SPN1 failed to oligomerize leading to cytoplasmic aggregation in patients’ primary fibroblasts and CRISPR/Cas9-mediated mutant cell lines. Additionally, mutant nuclei exhibit defective spliceosomal maturation and breakdown of Cajal bodies. Transcriptome analyses reveal splicing and mRNA expression dysregulation, particularly in sarcolemmal components, causing disruption of cytoskeletal organization in mutant cells and patient muscle tissues. Our findings establish SNUPN deficiency as the genetic etiology of a previously unrecognized subtype of muscular dystrophy and provide robust evidence of the role of SPN1 for muscle homeostasis

Monitoring and predicting the risk of violence in residential facilities. No difference between patients with history or with no history of violence

none34noopende Girolamo, Giovanni; Buizza, Chiara; Sisti, Davide; Ferrari, Clarissa; Bulgari, Viola; Iozzino, Laura; Boero, Maria Elena; Cristiano, Giuseppe; De Francesco, Alessandra; Giobbio, Gian Marco; Maggi, Paolo; Rossi, Giuseppe; Segalini, Beatrice; Candini, Valentina; Andreose, Suor; Basso, Pasquale; Beneduce, Rossella; Bertolotti, Pietro; Braida, Vanda; Bonelli, Marina; Bongiorno, Fanny; Bussi, Riccardo; Castagno, Elisa; Dominicis, Fabio; Ghersi, Loredana; Greppo, Stefania; Sodano, Alessandro Jaretti; Leporatti, Massimo; Presti, Eleonora Lo; Milone, Valeria; Panigada, Fausto; Pasquadibisceglie, Livia; Rigamonti, Danilo; Rillosi, Lucianade Girolamo, Giovanni; Buizza, Chiara; Sisti, Davide; Ferrari, Clarissa; Bulgari, Viola; Iozzino, Laura; Boero, Maria Elena; Cristiano, Giuseppe; De Francesco, Alessandra; Giobbio, Gian Marco; Maggi, Paolo; Rossi, Giuseppe; Segalini, Beatrice; Candini, Valentina; Andreose, Suor; Basso, Pasquale; Beneduce, Rossella; Bertolotti, Pietro; Braida, Vanda; Bonelli, Marina; Bongiorno, Fanny; Bussi, Riccardo; Castagno, Elisa; Dominicis, Fabio; Ghersi, Loredana; Greppo, Stefania; Sodano, Alessandro Jaretti; Leporatti, Massimo; Presti, Eleonora Lo; Milone, Valeria; Panigada, Fausto; Pasquadibisceglie, Livia; Rigamonti, Danilo; Rillosi, Lucian

A first-in-class Wiskott-Aldrich syndrome protein activator with anti-tumor activity in hematologic cancers

Hematological cancers are among the most common cancers in adults and children. Despite significant improvements in therapies, many patients still succumb to the disease. Therefore, novel therapies are needed. The Wiskott-Aldrich syndrome protein (WASp) family regulates actin assembly in conjunction with the Arp2/3 complex, a ubiquitous nucleation factor. WASp is expressed exclusively in hematopoietic cells and exists in two allosteric conformations: autoinhibited or activated. Here, we describe the development of EG-011, a first-in-class small molecule activator of the WASp auto-inhibited form. EG-011 possesses in vitro and in vivo anti-tumor activity as a single agent in lymphoma, leukemia, and multiple myeloma, including models of secondary resistance to PI3K, BTK, and proteasome inhibitors. The in vitro activity was confirmed in a lymphoma xenograft. Actin polymerization and WASp binding was demonstrated using multiple techniques. Transcriptome analysis highlighted homology with drugs-inducing actin polymerization