Chronic myeloproliferative syndromes are rare disorders in pediatric age, for which the knowledge of the pathology and its management is limited.
Chronic myeloproliferative syndromes in adults ...are commonly driven by JAK2 and MPL mutations, but in pediatrics there is no clear relationship.
Currently, there are guidelines and protocols for the management of adult patients, which allows risk stratification and recommended treatment. However, this would not be the case in pediatric patients.
We present a patient with essential thrombocythemia and the differential diagnoses that were studied at the Alejandro A. Posadas hospital.
Los síndromes mieloproliferativos crónicos son trastornos poco frecuentes en la edad pediátrica, por lo cual el conocimiento de la patología y su manejo es limitado.
Los síndromes mieloproliferativos crónicos en adultos son impulsados comúnmente por mutaciones del JAK2 y MPL, pero en pediatría no existe una relación clara.
Actualmente, existen guías y protocolos para el manejo de pacientes adultos, lo cual permite la estratificación del riesgo y tratamiento recomendado al mismo. Sin embargo, éste no sería el caso en pacientes pediátricos.
Presentamos un paciente con trombocitemia esencial y los diagnósticos diferenciales que se estudiaron en el hospital Alejandro A. Posadas.
•Kelp forests are key biogenic habitats declining in temperate coasts worldwide.•Sustained deforestation of kelp reefs erodes recruitment success.•Recruitment success is a convenient indicator of ...kelp forest resilience.
Kelp forests are critical marine ecosystems that provide habitat and ecological services, as well as economic benefits. However, kelp forests worldwide are facing multiple pressures, including climate change and human activities. In this study, we investigated whether recruitment success, an infrequently recorded variable in kelp monitoring studies, was affected in degraded kelp reefs where adult golden kelps (Laminaria ochroleuca) were persistently absent due to fish consumption. We conducted a three-year seasonal monitoring of kelp recruits and juveniles in both healthy and degraded kelp reefs in northwest Spain. Our findings reveal a decline in spring kelp recruitment over time on degraded reefs, while it remained stable on healthy reefs. The results indicate that continued herbivory pressure can decrease kelp recruitment, suggesting the potential exhaustion of kelp stand resilience. Altogether, our study highlights the importance of biotic interactions, such as herbivory, in understanding changes in kelp forest dynamics. Moreover, it emphasizes the significance of including recruitment in kelp forest assessments as an indicator of resilience.
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•Nine withanolides were isolated from Cuatresia Colombiana.•Several semisynthetic derivatives were obtained from withaferin A and Jaborosalactone 38.•The in vitro antibacterial ...activity of 25 natural compounds with different skeletons and 9 derivatives were evaluated.•The 1-oxo-2-ene-4β-OR-5β,6β-epoxy substitution pattern on rings A and B is critical for antibacterial activity.
Two new withanolides, (17R,20S,22R)-4β-acetoxy-5β,6β-epoxy-19,27-dihydroxy-1-oxo-witha-2,24-dienolide (withalongolide A 4-acetate (5) and (17R,20S,22R)-5β,6β-epoxy-27-hydroxy-1,4-dioxo-witha-24-enolide (9), and seven known withanolides with normal structure (1–4, 6–8) were isolated from aerial parts of Cuatresia colombiana. Several semisynthetic derivatives were prepared from the natural metabolites withaferin A and jaborosalactone 38. The compounds were fully characterized by a combination of spectroscopic methods (1D and 2D NMR and MS). The compounds isolated from C. colombiana, sixteen withanolides previously isolated from different Solanaceae species with different skeletons and semisynthetic derivatives were evaluated for their antibacterial activity against a selected panel of Gram-positive and Gram-negative bacteria. According to the bioactivity against S. aureus and E. faecalis, the compounds evaluated were divided into three groups: compounds with high activity (MIC 0.063 mM), compounds with moderate activity (0.5 mM > MIC > 0.125 mM) and non-active compounds (MIC ≥1 mM); in addition, some structure-activity relationship keys could be inferred.
This study aimed to identify determinants of adherence to lifestyle and body weight recommendations for cancer prevention among Lynch Syndrome (LS) patients. Cross-sectional baseline data of LS ...patients participating in the Lifestyle & Lynch (LiLy) study was used to assess determinants of adherence to the World Cancer Research Fund cancer prevention recommendations on body weight, physical activity, and red and processed meat intake. Adherence and potential determinants of adherence were assessed using questionnaires. Multivariable logistic regression analyses were conducted to identify determinants of adherence. Of the 211 participants, 50.2% adhered to the body weight recommendation, 78.7% adhered to the physical activity recommendation, and 33.6% adhered to the red and processed meat recommendation. Being younger and having a higher level of education were associated with adherence to the recommendation on body weight. Having knowledge about the recommendation was associated with adherence to the recommendations on physical activity and red and processed meat. Results confirm that knowledge about recommendations for cancer prevention is an important determinant for adherence and suggest that strategies to increase knowledge should be included in lifestyle promotion targeted at LS patients, along with behavior change techniques influencing other modifiable determinants.
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•Sepiolite and palygorskite have been evaluated for their potential for CO2 capture.•Sepiolite and palygorskite were modified by microwave-assisted acid treatment and impregnated with ...amines.•Raw sepiolite hashigh potential in CO2 capture at 25 °C than palygorskite due to the lower microporosity of the later.•The double functionalization of sepiolite with APTES-PEI led to the highest CO2 adsorption capacity.
Two fibrous clay minerals (sepiolite and palygorskite) have been employed as adsorbents to assess their adsorption capacity of pure CO2 in a volumetric setup. The raw sepiolite reached a CO2 uptake of 1.48 mmol g−1 due to the presence of nanocavities acting as a molecular sieve. Both sepiolite and palygorskite were modified by microwave-assisted acid treatment, which led to an increase in specific surface area and pore volume due to Mg2+ leaching, particularly in the case of sepiolite. However, the partial digestion of these fibrous structures does not improve CO2 adsorption uptake due to the progressive increase of the size of nanocavities. In a next step, both fibrous clay minerals were functionalized with amine species by different procedures (grafting with (3-Aminopropyl) triethoxysilane (APTES), impregnation with polyethyleneimine (PEI) and double functionalization by grafting with APTES and then impregnation with PEI. In all cases, it can be observed that the incorporation of amine species favors the chemical interaction between the amines species and the CO2 molecules, although it also produces obstruction of the nanochannels so the adsorption takes place mainly on the outer surface of the fibers. Finally, the incorporation of amine species by double functionalization led to the highest CO2 adsorbed concentration of 2.07 mmol g−1 at 760 mm Hg and 65 °C due to a larger proportion of available amines sites as well as the use of higher adsorption temperature, which favored the diffusion of CO2 molecules within the adsorbent.
This paper proposes a maturity model (MM) to become a smart organization considering Lean as a key enabler to drive I4.0 adoption. A systematic literature review on I4.0 and Lean concepts plus I4.0 ...adoption models was conducted through the PRISMA method based on articles from Scopus and Web of Science databases, and records from official websites (e.g., consulting firms) published between 2011 and 2022. Identifying the Lean and I4.0 relationship and comparing the models’ relevant characteristics allowed the development of the MM proposal. Although previous research refers to Lean and I4.0 collaboration, the opportunity to design a reference model for adopting both approaches was identified since their interaction enhances value creation. The comprehensive model supports structuring the types of Lean principles/methods/tools and I4.0 technologies and their action to link them and define which of them need to be implemented according to the maturity level chronologically. Additionally, the proposed MM provides an adoption roadmap that starts eliminating non-added activities in the initial stages for process improvement to integrate I4.0 enabling technologies later. The model makes it possible for practitioners to generate implementation and development processes oriented toward I4.0 adoption based on maturity levels in which Lean has the starting point at the first ones. Hence, it defines the enabling technologies to be incorporated and linked throughout the value chain, enhancing a Lean culture. This model will help organizations to become “smart” by allowing them to transition toward the best technology investment and continuously add value to their processes, people, and products. Moreover, the results will motivate researchers to study further the application of models for I4.0 adoption in which Lean is integrated to fill the gap with the I4.0 embrace caused by quickly changing industrial environments and the uncertainty and unknowledge of guidelines associated with incorporating new technologies.
La Terapia de Aceptación y Compromiso (TAC) ha demostrado ser una intervención prometedora para el estigma internalizado en personas con virus de la inmunodeficiencia humana (VIH) y trastornos por ...uso de drogas (TUD). Sin embargo, hay incertidumbre sobre si esta intervención es apropiada para latinx hispanx hablantes. Este estudio preliminar evaluamos la viabilidad y aceptabilidad de una intervención TAC culturalmente adaptada y desarrollada para reducir el estigma internalizado del VIH y TUD en una muestra de latinxs de habla hispana. Ocho pacientes de clínicas primarias con diagnóstico de VIH y TUD participaron en este estudio. Se recopilaron medidas de viabilidad y aceptabilidad, así como tasa de asistencia. Todas las personas participantes describieron la intervención como aceptable, alcanzable, fácil de usar e indicaron estar receptivas a la terapia. La TAC parece ser una intervención aceptable y viable para las personas pacientes de atención primaria de descendencia latina de habla hispana. Un estudio de control aleatorizado es requerido para explorar mejor la eficacia de la intervención.
La ciudad de Lima presenta un desarrollo muy desigual de ciclovías en todos sus distritos, donde se destaca el distrito de Miraflores como el de mayor desarrollo. El presente trabajo tuvo como ...objetivo evaluar la infraestructura para bicicletas en este distrito. La metodología aplicada combinó el levantamiento terrestre, con levantamientos virtuales y procesamiento de imágenes satelitales en un entorno GIS. Esta evaluación se realizó sobre la base de los principales aspectos relacionados con ciclo-inclusividad, seguridad, coherencia, dirección e infraestructura del ciclo. Entre los principales resultados, destacan que los niveles de seguridad son bajos, lo que permite inferir que se debe promover un mayor número de carriles bici físicamente segregados. La coherencia es muy pobre en lograr conectar varios puntos de origen y destino, con numerosos cruces. Aunque las rutas principales son bastante directas, la mala ubicación de los aparcamientos para bicicletas y la distribución desigual de la red la hacen todavía muy ineficiente. A pesar de las deficiencias que aún presenta esta infraestructura ciclista en Miraflores, se evidencia una fuerte diferenciación con el resto de la ciudad de Lima, debido, entre otras causas, a una alta informalidad en el sector del transporte, derivada de la aplicación de políticas neoliberales que han profundizado las amplias desigualdades territoriales y sociales en esta ciudad. La pandemia COVID-19 ha incorporado un nuevo tipo de ciclistas, vinculados a los sistemas de «entrega», que evidencia aún más las limitaciones de la infraestructura de bicicletas en toda la ciudad.
Abstract
Calpain-2 (CAPN2) is a processing enzyme ubiquitously expressed in mammalian tissues whose pleiotropic functions depend on the role played by its cleaved-products. Nuclear interaction ...networks, crucial for a number of molecular processes, could be modified by CAPN2 activity. However, CAPN2 functions in cell nucleus are poorly understood. To unveil CAPN2 functions in this compartment, the result of CAPN2-mediated interactions in cell nuclei was studied in breast cancer cell (BCC) lines. CAPN2 abundance was found to be determinant for its nucleolar localization during interphase. Those CAPN2-dependent components of nucleolar proteome, including the actin-severing protein cofilin-1 (CFL1), were identified by proteomic approaches. CAPN2 binding, cleavage and activation of LIM Kinase-1 (LIMK1), followed by CFL1 phosphorylation was studied. Upon CAPN2-depletion, full-length LIMK1 levels increased and CFL1/LIMK1 binding was inhibited. In addition, LIMK1 accumulated at the cell periphery and perinucleolar region and, the mitosis-specific increase of CFL1 phosphorylation and localization was altered, leading to aberrant mitosis and cell multinucleation. These findings uncover a mechanism for the role of CAPN2 during mitosis, unveil the critical role of CAPN2 in the interactions among nuclear components and, identifying LIMK1 as a new CAPN2-target, provide a novel mechanism for LIMK1 activation. CFL1 is crucial for cytoskeleton remodeling and mitosis, but also for the maintenance of nuclear structure, the movement of chromosomes and the modulation of transcription frequently altered in cancer cells. Consequently, the role of CAPN2 in the nuclear compartment might be extended to other actin-associated biological and pathological processes.
MicroRNAs (miRNAs) are noncoding RNAs that contribute to gene expression modulation by regulating important cellular pathways. In this study, we used small RNA sequencing to identify a series of ...circulating miRNAs in blood samples taken from Friedreich's ataxia patients. We were thus able to develop a miRNA biomarker signature to differentiate Friedreich's ataxia (FRDA) patients from healthy people. Most research on FDRA has focused on understanding the role of frataxin in the mitochondria, and a whole molecular view of pathological pathways underlying FRDA therefore remains to be elucidated. We found seven differentially expressed miRNAs, and we propose that these miRNAs represent key mechanisms in the modulation of several signalling pathways that regulate the physiopathology of FRDA. If this is the case, miRNAs can be used to characterize phenotypic variation in FRDA and stratify patients' risk of cardiomyopathy. In this study, we identify miR-323-3p as a candidate marker for phenotypic differentiation in FRDA patients suffering from cardiomyopathy. We propose the use of dynamic miRNAs as biomarkers for phenotypic characterization and prognosis of FRDA.