Necessary improvement of the brazilian autocompositive system: proposals for changes in the training of conciliators and mediators

This article aims to expose reflections on the autocompositional system in Brazil. Its objective is to formulate a proposal for remodeling the syllabus of the Training and Training Course for Conciliators and Mediators, because a well-structured training for the professional, which also introduces general notions about the mental universe of the human being, can guarantee self-composing sessions in a more complete way. Using the deductive method, based on descriptive research, it is concluded that such reformulation needs to be carried out, as a way of giving effectiveness to the Brazilian autocompositional system.Este artículo tiene como objetivo exponer reflexiones sobre el sistema autocompositivo en Brasil. Tiene como objetivo formular una propuesta de remodelación curricular del Curso de Formación de Conciliadores y Mediadores, como una formación bien estructurada para el profesional, que además introduzca nociones generales sobre el universo psíquico del ser humano, pueda garantizar el autocuidado a componer las sesiones de una forma más completa. Utilizando el método deductivo, basado en una investigación descriptiva, se concluye que tal reformulación necesita ser realizada, como una forma de dar efectividad al sistema de autocomposición brasileño

RALI: Increasing Reliability in LoRaWAN through Repetition and Iteration

International audienc

Study of LoRaWAN Networks Reliability

International audienceThe Internet of Things (IoT) is a rapidly evolving field that incorporates a wide range of technologies and applications, enabling the seamless integration of everyday objects into the digital world. The effective integration of IoT into various systems requires the implementation of lightweight solutions to overcome the challenges posed by highly dense networks and constrained resources, including computational power, memory capacity, and battery life. The present research is dedicated to investigating a specific context of the Internet of Things (Io’T), namely LoRaWAN, in which devices communicate with the access network using ALOHA-type access and spread spectrum technology. LoRaWAN advocates simplicity in order to reduce drastically the battery consumption, which severely degrades reliability. In this paper we introduce blind repetition in LoRaWAN: a packet is retransmitted a fixed number of times regardless of its good reception. Leveraging on existing data link layer functionalities, we compare this redundant mode to two existing modes, namely the unacknowledged mode and acknowledged mode. We run extensive simulations that consider the capture effect in LoRaWAN, in addition to the non-uniform distribution of devices. In such a realistic scenario, we perform thorough numerical simulations to quantify the discrepancy between the three modes, and identify the traffic conditions for which a given mode has precedence over the two others

Mortality of French participants in the Tour de France (1947-2012)

International audienceAims : In the context of recent concerns regarding performance enhancing techniques and potential negative health effects of high-level physical activity, data on the long-term outcomes and causes of death in elite endurance cyclists are of particular interest. Methods and results : Characteristics and vital status of all French participants in the Tour de France were collected for the 1947–2012 period. Causes of death were obtained from 1968. Overall and disease-specific mortalities were compared with the French male population using overall and specific standardized mortality ratios (SMRs) with their 95% confidence intervals (CIs). Among the 786 French cyclists who participated at least once between 1947 and 2012, 208 (26%) died by 1 September 2012. Neoplasms and cardiovascular diseases accounted for 61% of deaths. We observed a 41% lower mortality in French cyclists (SMR: 0.59, 95% CI: 0.51– 0.68, P , 0.0001), which did not change over time (P = 0.70). It was observed for main mortality causes: for neoplasms (SMR: 0.56; 95% CI: 0.42–0.72, P , 0.0001) and for cardiovascular death (SMR: 0.67; 95% CI: 0.50– 0.88, P = 0.004), except mortality related to external causes (SMR: 1.06, 95% CI: 0.71 –1.53, P = 0.80). Conclusion : We observed a substantially and significantly lower mortality in participants in the Tour de France, compared with the general male population. However, our results do not allow us to assess in detail the balance between positive effects of high-level sports activity and selection of healthy elite athletes, vs. any potential deleterious effects of excessive physical exercise or alleged doping

Mutation analysis of NPHP6/CEP290 in patients with Joubert syndrome and Senior-Løken syndrome

BACKGROUND: Nephronophthisis (NPHP) is an autosomal recessive cystic kidney disease that constitutes the most common genetic cause of renal failure in the first three decades of life. Using positional cloning, six genes (NPHP1-6) have been identified as mutated in NPHP. In Joubert syndrome (JBTS), NPHP may be associated with cerebellar vermis aplasia/hypoplasia, retinal degeneration and mental retardation. In Senior-Løken syndrome (SLSN), NPHP is associated with retinal degeneration. Recently, mutations in NPHP6/CEP290 were identified as a new cause of JBTS. METHODS: Mutational analysis was performed on a worldwide cohort of 75 families with SLSN, 99 families with JBTS and 21 families with isolated nephronophthisis. RESULTS: Six novel and six known truncating mutations, one known missense mutation and one novel 3 bp pair in-frame deletion were identified in a total of seven families with JBTS, two families with SLSN and one family with isolated NPHP

The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4

The molecular basis of nephronophthisis, the most frequent genetic cause of renal failure in children and young adults, and its association with retinal degeneration and cerebellar vermis aplasia in Joubert syndrome are poorly understood. Using positional cloning, we here identify mutations in the gene CEP290 as causing nephronophthisis. It encodes a protein with several domains also present in CENPF, a protein involved in chromosome segregation. CEP290 (also known as NPHP6) interacts with and modulates the activity of ATF4, a transcription factor implicated in cAMP-dependent renal cyst formation. NPHP6 is found at centrosomes and in the nucleus of renal epithelial cells in a cell cycle-dependent manner and in connecting cilia of photoreceptors. Abrogation of its function in zebrafish recapitulates the renal, retinal and cerebellar phenotypes of Joubert syndrome. Our findings help establish the link between centrosome function, tissue architecture and transcriptional control in the pathogenesis of cystic kidney disease, retinal degeneration, and central nervous system development

Individuals with mutations in XPNPEP3, which encodes a mitochondrial protein, develop a nephronophthisis-like nephropathy

The autosomal recessive kidney disease nephronophthisis (NPHP) constitutes the most frequent genetic cause of terminal renal failure in the first 3 decades of life. Ten causative genes (NPHP1–NPHP9 and NPHP11), whose products localize to the primary cilia-centrosome complex, support the unifying concept that cystic kidney diseases are “ciliopathies”. Using genome-wide homozygosity mapping, we report here what we believe to be a new locus (NPHP-like 1 [NPHPL1]) for an NPHP-like nephropathy. In 2 families with an NPHP-like phenotype, we detected homozygous frameshift and splice-site mutations, respectively, in the X-prolyl aminopeptidase 3 (XPNPEP3) gene. In contrast to all known NPHP proteins, XPNPEP3 localizes to mitochondria of renal cells. However, in vivo analyses also revealed a likely cilia-related function; suppression of zebrafish xpnpep3 phenocopied the developmental phenotypes of ciliopathy morphants, and this effect was rescued by human XPNPEP3 that was devoid of a mitochondrial localization signal. Consistent with a role for XPNPEP3 in ciliary function, several ciliary cystogenic proteins were found to be XPNPEP3 substrates, for which resistance to N-terminal proline cleavage resulted in attenuated protein function in vivo in zebrafish. Our data highlight an emerging link between mitochondria and ciliary dysfunction, and suggest that further understanding the enzymatic activity and substrates of XPNPEP3 will illuminate novel cystogenic pathways