Human and organizational factors in the transport of radioactive materials

International audienceThe analysis of events related to the transport of radioactive materials shows that most of them are not only due to technical issues, but also involve human and organizational factors as a root cause. Indeed, human and organizational factors, such as competences, work environment, task characteristics and organization, have an effect on the safety of transport activities. In this regard, the analysis of the transport related events performed by the French Institute for Radioprotection and Nuclear Safety (IRSN) takes into account these factors in order to identify the causes of the most significant events occurred in France and to assess the actions put in place by consignors and carriers in order to prevent their recurrence. These analyses can focus on the organization implemented for the transport activities, including the operators training program and the means to take into account the operational feedback. In addition, on-site visits and interviews with operators and managers enable a better understanding of the organization (interfaces, roles and responsibilities), the working conditions and the possible difficulties related to the applicable procedures. In 2018, such an assessment has been performed by IRSN after the declaration of an event involving the loading operations of packages designed to transport spent fuel. The paper will describe the methodology applied for this analysis and present the main conclusions drawn from this assessment. The paper will also describe the major human and organizational factors that need to be taken into account in the safety of transport of radioactive materials. In fact, IRSN considers that the design process of packages and associated tools must include human factors engineering (co-design and validation with users addressing the future operating conditions). Furthermore, the safety functions ensured by the safety related components should be explained to the package users to improve their understanding of the different operations that should be performed at each step of the package transport, including loading and unloading

Human and organizational factors in the transport of radioactive materials

International audienceThe analysis of events related to the transport of radioactive materials shows that most of them are not only due to technical issues, but also involve human and organizational factors as a root cause. Indeed, human and organizational factors, such as competences, work environment, task characteristics and organization, have an effect on the safety of transport activities. In this regard, the analysis of the transport related events performed by the French Institute for Radioprotection and Nuclear Safety (IRSN) takes into account these factors in order to identify the causes of the most significant events occurred in France and to assess the actions put in place by consignors and carriers in order to prevent their recurrence. These analyses can focus on the organization implemented for the transport activities, including the operators training program and the means to take into account the operational feedback. In addition, on-site visits and interviews with operators and managers enable a better understanding of the organization (interfaces, roles and responsibilities), the working conditions and the possible difficulties related to the applicable procedures. In 2018, such an assessment has been performed by IRSN after the declaration of an event involving the loading operations of packages designed to transport spent fuel. The paper will describe the methodology applied for this analysis and present the main conclusions drawn from this assessment. The paper will also describe the major human and organizational factors that need to be taken into account in the safety of transport of radioactive materials. In fact, IRSN considers that the design process of packages and associated tools must include human factors engineering (co-design and validation with users addressing the future operating conditions). Furthermore, the safety functions ensured by the safety related components should be explained to the package users to improve their understanding of the different operations that should be performed at each step of the package transport, including loading and unloading

Human and organizational factors in the transport of radioactive materials

International audienceThe analysis of events related to the transport of radioactive materials shows that most of them are not only due to technical issues, but also involve human and organizational factors as a root cause. Indeed, human and organizational factors, such as competences, work environment, task characteristics and organization, have an effect on the safety of transport activities. In this regard, the analysis of the transport related events performed by the French Institute for Radioprotection and Nuclear Safety (IRSN) takes into account these factors in order to identify the causes of the most significant events occurred in France and to assess the actions put in place by consignors and carriers in order to prevent their recurrence. These analyses can focus on the organization implemented for the transport activities, including the operators training program and the means to take into account the operational feedback. In addition, on-site visits and interviews with operators and managers enable a better understanding of the organization (interfaces, roles and responsibilities), the working conditions and the possible difficulties related to the applicable procedures. In 2018, such an assessment has been performed by IRSN after the declaration of an event involving the loading operations of packages designed to transport spent fuel. The paper will describe the methodology applied for this analysis and present the main conclusions drawn from this assessment. The paper will also describe the major human and organizational factors that need to be taken into account in the safety of transport of radioactive materials. In fact, IRSN considers that the design process of packages and associated tools must include human factors engineering (co-design and validation with users addressing the future operating conditions). Furthermore, the safety functions ensured by the safety related components should be explained to the package users to improve their understanding of the different operations that should be performed at each step of the package transport, including loading and unloading

Immunological and virological profile of children with chilblain‐like lesions and SARS‐CoV‐2

International audienceThe link between SARS‐CoV‐2 and the reported cutaneous manifestations has not been established. We assessed a possible correlation between the paediatric dermatological manifestations and the biological investigations, using for the first time 3 different SARS‐CoV‐2 tests

The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed.Peer reviewe

Am J Hum Genet

While common obesity accounts for an increasing global health burden, its monogenic forms have taught us underlying mechanisms via more than 20 single-gene disorders. Among these, the most common mechanism is central nervous system dysregulation of food intake and satiety, often accompanied by neurodevelopmental delay (NDD) and autism spectrum disorder. In a family with syndromic obesity, we identified a monoallelic truncating variant in POU3F2 (alias BRN2) encoding a neural transcription factor, which has previously been suggested as a driver of obesity and NDD in individuals with the 6q16.1 deletion. In an international collaboration, we identified ultra-rare truncating and missense variants in another ten individuals sharing autism spectrum disorder, NDD, and adolescent-onset obesity. Affected individuals presented with low-to-normal birth weight and infantile feeding difficulties but developed insulin resistance and hyperphagia during childhood. Except for a variant leading to early truncation of the protein, identified variants showed adequate nuclear translocation but overall disturbed DNA-binding ability and promotor activation. In a cohort with common non-syndromic obesity, we independently observed a negative correlation of POU3F2 gene expression with BMI, suggesting a role beyond monogenic obesity. In summary, we propose deleterious intragenic variants of POU3F2 to cause transcriptional dysregulation associated with hyperphagic obesity of adolescent onset with variable NDD

Acute tubulointerstitial nephritis with or without uveitis: a novel form of post-acute COVID-19 syndrome in children

International audienceMultiorgan sequelae of coronavirus disease 2019 (COVID-19) beyond the acute phase of infection are increasingly described as clinical experience expands. In children, acute COVID-19 appears to be generally asymptomatic or mild. Yet, the multisystem inflammatory syndrome in children (MIS-C) may be a severe postinfectious complication following exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).1 During the first pandemic year, we observed a striking increase in the incidence of acute tubulointerstitial nephritis (aTIN) without or with uveitis (TINUs) among children. Causes of aTIN include drugs, infections, and systemic diseases, but often remain undetermined. The rare TINUs syndrome associating aTIN and uveitis is considered to result from a still ill-characterized immune-mediated process. The observed increased incidence of idiopathic aTIN/TINUs prompted us to examine whether SARS-CoV-2 might be the initial trigger

Performance of 30 commercial SARS-CoV-2 serology assays in testing symptomatic COVID-19 patients

International audienceWe report evaluation of 30 assays' (17 rapid tests (RDTs) and 13 automated/manual ELISA/CLIA assay (IAs)) clinical performances with 2594 sera collected from symptomatic patients with positive SARS-CoV-2 rRT-PCR on a respiratory sample, and 1996 pre-epidemic serum samples expected to be negative. Only 4 RDT and 3 IAs fitted both specificity (> 98%) and sensitivity (> 90%) criteria according to French recommendations. Serology may offer valuable information during COVID-19 pandemic, but inconsistent performances observed among the 30 commercial assays evaluated, which underlines the importance of independent evaluation before clinical implementation

FLT3L governs the development of partially overlapping hematopoietic lineages in humans and mice

International audienceFMS-related tyrosine kinase 3 ligand (FLT3L), encoded by FLT3LG, is a hematopoietic factor essential for the development of natural killer (NK) cells, B cells, and dendritic cells (DCs) in mice. We describe three humans homozygous for a loss-of-function FLT3LG variant with a history of various recurrent infections, including severe cutaneous warts. The patients' bone marrow (BM) was hypoplastic, with low levels of hematopoietic progenitors, particularly myeloid and B cell precursors. Counts of B cells, monocytes, and DCs were low in the patients' blood, whereas the other blood subsets, including NK cells, were affected only moderately, if at all. The patients had normal counts of Langerhans cells (LCs) and dermal macrophages in the skin but lacked dermal DCs. Thus, FLT3L is required for B cell and DC development in mice and humans. However, unlike its murine counterpart, human FLT3L is required for the development of monocytes but not NK cells