Illicit supply networks (ISNs) are composed of coordinated human actors that source, transit, and distribute illicitly traded goods to consumers, while also creating widespread social and ...environmental harms. Despite growing documentation of ISNs and their impacts, efforts to understand and disrupt ISNs remain insufficient due to the persistent lack of knowledge connecting a given ISN’s modus operandi and its patterns of activity in space and time. The core challenge is that the data and knowledge needed to integrate it remain fragmented and/or compartmentalized across disciplines, research groups, and agencies tasked with understanding or monitoring one or a few specific ISNs. One path forward is to conduct comparative analyses of multiple diverse ISNs. We present and apply a conceptual framework for linking ISN modus operandi to spatial-temporal dynamics and patterns of activity. We demonstrate this through a comparative analysis of three ISNs – cocaine, illegally traded wildlife, and illegally mined sand – which range from well-established to emergent, global to domestic in geographic scope, and fully illicit to de facto legal. The proposed framework revealed consistent traits related to geographic price structure, value capture at different supply chain stages, and key differences among ISN structure and operation related to commodity characteristics and their relative illicitness. Despite the diversity of commodities and ISN attributes compared, social and environmental harms inflicted by the illicit activity consistently become more widespread with increasing law enforcement disruption. Drawing on these lessons from diverse ISNs, which varied in their histories and current sophistication, possible changes in the structure and function of nascent and/or low salience ISNs may be anticipated if future conditions or law enforcement pressure change.
Type 2B von Willebrand disease (VWD2B) and platelet-type von Willebrand disease (PT-VWD) are rare bleeding disorders characterised by an increased ristocetin-induced platelet aggregation (RIPA) at ...low dose of ristocetin. It was the objective of this study to detect children with VWD2B and PT-VWD using RIPA at low dose of ristocetin (0.5 mg/ml) in the screening evaluation of bleeding disorders, and to analyse the phenotypic data along with the molecular findings. Over a 14-year period, 641 children with personal and family bleeding symptoms or bleeding from birth with previously uncharacterised haemostatic disorders were prospectively studied. Six unrelated patients (0.93%) showed RIPA at low dose of ristocetin. RIPA-based mixing studies identified that the plasma of the six probands and at least one parent from five unrelated families induced aggregation of normal platelets with the addition of low-dose ristocetin. None of the probands’ platelets showed aggregation with cryoprecipitate. Low ristocetin cofactor activity/VWF antigen ratio with absent collagen binding activity or thrombocytopenia were detected respectively in only two patients. Molecular analysis of exon 28 of the VWF gene identified mutations in only three patients. No mutation in the GP1BA gene was found. In this large prospective paediatric study, the screening approach including RIPA at low dose of ristocetin permitted the detection of patients with VWD2B that would otherwise have been missed. No patient with phenotype or genotype of PT-VWD was identified. Heterogeneity of bleeding symptoms and phenotypic parameters were found among members of the same family.
The enzyme glucose-6-phosphate dehydrogenase (G6PD) catalyses the first step in the pentose phosphate pathway, producing nicotinamide adenine dinucleotide phosphate (NADPH). NADPH plays a crucial ...role in preventing oxidative damage to proteins and other molecules in cells, mostly red blood cells. G6PD deficiency has an x-linked pattern of inheritance in which hemizygous males are deficient, while females may or may not be deficient depending on the number of affected alleles. We report two novel DNA variants in the G6PD gene detected in two male probands with chronic nonspherocytic hemolytic anemia (CNSHA), who were referred for hematological evaluation.
Probands and their relatives underwent clinical, biochemical, and molecular assessment.
Two novel DNA variants, c.995C>T and c.1226C>A, were found in this study. At the protein level, they produce the substitution of Ser332Phe and Pro409Gln, respectively. These DNA variants were analyzed in the female relatives of probands for genetic counseling.
The novel DNA variants were classified as class I based on the clinical, biochemical, and molecular evaluations performed.
•Two novel class I G6PD DNA variants (S332F; P409Q).•In silico analysis indicates likely deleterious changes to the enzyme.•The change of the amino acid S332F could affect the ingress to the active site.•The P409Q change could affect normal dimerization, decreasing enzyme activity.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
En el presente artículo se abordan las diferentes herramientas legales que existen en el Perú a fin de poder hacer frente a las diversas emergencias ambientales, especialmente, aquellas derivadas del ...aprovechamiento de recursos naturales por parte de los inversionistas. Esto incluye herramientas de tipo constitucional, administrativo, penal y civil.Igualmente, a fin de poder retratar de forma clara lo antes expuesto, se hará referencia a un caso de estudio ocurrido hace poco tiempo. Este es el caso del derrame de petróleo de Repsol en el mar peruano.
•Sand mining provides opportunities for diversifying livelihoods within the Usumacinta basin.•In the Usumacinta River basin, mining is a male-dominated activity linked with gendered ...riverscapes.•Women were fully excluded at sites managed by private companies and indigenous communities.•Women are more present at collective organizations for sand extraction, yet they are subordinated and invisible.
Globally, sand and gravel are the second most extracted natural resources by volume after water. Despite that, knowledge about social and gender implications of sand-supply systems is limited. Here we aimed to advance the understanding of practices and gender relations in sand mining at male and female spaces in the Usumacinta River basin in Mexico and Guatemala. Drawing from desk and field research carried out in 79 locations on the riverbanks of the Usumacinta River and eight tributaries, this paper examines the extractive activities, gender roles and conditions of the workers. Sand extraction is characterized by a large diversity of actors, forms of organization, and local practices. We provide empirical evidence of a prevalent gender imbalance in the sand mining sector, as well as between men and women's spaces. Women participate in sand-mining related works in 19.51% of the sites, although in half of those cases they perform administrative tasks from town offices. Overall, their roles are seen as peripheral and minor even as they sell and classify materials and contribute to organizational tasks. We argue that the low participation of women at extraction sites is due to a combination of physically demanding work conditions and deeply ingrained gender norms in rural communities.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Habitat loss and deterioration represent the main threats to wildlife species, and are closely linked to the expansion of roads and human settlements. Unfortunately, large-scale effects of these ...structures remain generally overlooked. Here, we analyzed the European transportation infrastructure network and found that 50% of the continent is within 1.5 km of transportation infrastructure. We present a method for assessing the impacts from infrastructure on wildlife, based on functional response curves describing density reductions in birds and mammals (e.g., road-effect zones), and apply it to Spain as a case study. The imprint of infrastructure extends over most of the country (55.5% in the case of birds and 97.9% for mammals), with moderate declines predicted for birds (22.6% of individuals) and severe declines predicted for mammals (46.6%). Despite certain limitations, we suggest the approach proposed is widely applicable to the evaluation of effects of planned infrastructure developments under multiple scenarios, and propose an internationally coordinated strategy to update and improve it in the future.
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BFBNIB, NMLJ, NUK, PNG, SAZU, UL, UM, UPUK
Glucose-6-phosphate dehydrogenase deficiency is an erythrocyte enzyme disorder caused by mutations in the G6PD gene, which has an X-linked inheritance. Here we analyze the clinical and laboratory ...characteristics of 24 subjects with G6PD deficiency over 25 years. Their median age at diagnosis was 10.2 years (range: 0.6-56.4). No symptoms were observed in 54.2 % of patients, whereas 25 % had chronic non-spherocytic hemolytic anemia; 12.5 %, neonatal jaundice and postinfectious hemolytic anemia; and 8.3 %, acute hemolytic anemia after ingestion of fava beans. The 24 studied patients had variants that had been previously described in the bibliography. The clinical characteristics observed here were consistent with the variants found. A total of 21 women from the maternal line of affected subjects were identified as deficiency carriers using molecular biology techniques, so they received the corresponding genetic counseling.
Para un sector de la sociedad, opuesto a los diálogos (extrema derecha), unos acuerdos de paz deben incluir la pena carcelaria para los guerrilleros y limitar su participación política sin armas. ...Otros sectores en cambio consideran que se deben establecer bases sobre las cuales se acuerde una justicia transicional para todos; el perdón y el olvido no se contemplan como opción política, o la desactivación de la confrontación. El objeto de este estudio es analizar el proceso de los diálogos de paz entre el gobierno y la guerrilla de las FARC-EP, la forma cómo cada uno concibe lo que debe ser la aplicación de justicia transicional. El artículo aborda los planteamientos de las FARC, la posición del gobierno frente a un acuerdo final y la Comisión de la Verdad como elemento fundamental para la justicia.
Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya syndrome remains undetermined. The role of the prothrombotic disorders contributing to ...its pathogenesis has not been completely elucidated. The purpose of this study was to determine the frequency of prothrombotic disorders in a pediatric population with moyamoya syndrome.
From May 1992 to April 2000, a prospective study of 10 consecutive children with moyamoya syndrome was carried out at a single center. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden, and prothrombin gene mutations. Lupus anticoagulant, anticardiolipin antibodies, and anti-beta(2)-glycoprotein I antibodies assays were also performed. The clinical characteristics, underlying diseases, family history of thrombosis, radiological findings, treatment, and outcome were also recorded.
In our series, prothrombotic disorders were detected in 4 patients (40%). Inherited protein S deficiency was found in 1 patient; lupus anticoagulant and anticardiolipin antibodies were detected in the remaining 3 patients. One presented persistent lupus anticoagulant for 2.7 years until his death. In the case of the other 2 patients, 1 has maintained lupus anticoagulant for 9 months, whereas the other has kept anticardiolipin/anti-beta(2)-glycoprotein I antibodies for 10 months.
We report the hemostatic data of the largest prospective pediatric study carried out at a single center in the western hemisphere. In 4 patients (40%), a prothrombotic disorder was detected. It is tempting to speculate that these hemostatic abnormalities may contribute to the pathogenesis of moyamoya syndrome in some of our patients.
Este artículo de revisión es resultado de la investigación referente a la política pública, la gestión educativa y la formación docente en el ámbito colombiano. Para su desarrollo, se describen ...algunos conceptos en los cuales se enmarca la política educativa y la relevancia que esta tiene en los procesos educativos llevados a cabo en el país; así mismo, se presentan los conceptos que desde diferentes ópticas se han establecido para la política pública, la gestión educativa, la formación docente, la relación que estas tienen con el ser y el deber ser y los retos de la formación docente frente a los planteamientos de las políticas educativas. A partir de ello, se concluye que las políticas educativas deben crearse sobre el principio de la transversalidad, de donde tomen cada uno de los elementos importantes para brindar una educación de calidad que aporte directamente al desarrollo y solución de las problemáticas sociales.
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