Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis

Background: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. Methods: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. Results: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. Conclusions: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. Type of Study: Clinical consensus statement. Level of Evidence: 3a.</p

Financial and relational impact of having a boy with posterior urethral valves

IntroductionChildhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care.Patients and methodWe gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the “impact on family scale” (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score.ResultsWe retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15–51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards.ConclusionIn conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Abstract Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

Caractérisation du système nerveux entérique et de la barrière épithéliale intestinale dans la maladie de Hirschsprung : source de biomarqueurs prédictifs de complications ?

La maladie de Hirschsprung (MH) est une maladie congénitale rare avec une incidence estimée à 1/5000 naissances vivantes. Elle est secondaire à une anomalie développementale du système nerveux entérique (SNE) et est caractérisée par une absence de cellules ganglionnaires (neurones et cellules gliales entériques) dans les plexus myentérique et sous-muqueux prédominant au niveau de la partie distale du tube digestif (Langer, 2013). C’est une neurocristopathie qui provient d’un défaut de colonisation, migration et différenciation des cellules progénitrices de la crête neurale (CPCN) qui a lieu chez l’homme entre le 5ème et la 12ème semaine de gestation (Obermayr et al, 2012; Lake and Heuckeroth, 2013). Cette anomalie est responsable d’une contraction permanente de l’intestin atteint entrainant une perte du péristaltisme intestinal et une occlusion digestive fonctionnelle se révélant majoritairement en période néonatale. Le traitement est chirurgical et consiste en la résection des zones aganglionnaire et de transition avec rétablissement de la continuité digestive en zone ganglionnaire dite « saine ».Absen

A Wzx-Wzy-like Polysaccharide Biosynthetic Pathway Contributes to Virulence in Brucella abortus 2308 through an Unknown Mechanism

Brucella abortus is an intracellular pathogen that causes spontaneous abortion in cattle and undulant fever in humans. As a member of the a-proteobacteria, B. abortus is closely related to the bacterial species Caulobacter crescentus and Agrobacterium tumefaciens, which have been shown to produce a holdfast and a unipolar polysaccharide (UPP), respectively, at one pole of the bacterial cell. Although little is currently known about exopolysaccharide production and function in Brucella species, recent evidence suggests that these bacteria are capable of producing exopolysaccharides, and with the exception of a flippase gene, B. abortus 2308 carries the genes for a complete UPP biosynthetic pathway. In A. tumefaciens, UPP can be detected by crystal violet, Congo red, or lectin staining; however, UPP production was not detected in B. abortus 2308 using these assays. UPP production can also be increased in A. tumefaciens by overexpressing the diguanylate cyclase PleD, which regulates intracellular cyclic di-GMP levels, but we were unable to demonstrate that UPP is regulated by cyclic di-GMP in B. abortus 2308. Nonetheless, upp homologs in B. abortus 2308 are able to restore biofilm production in A. tumefaciens. In A. tumefaciens, knockout mutants of the outer membrane transporter uppC and the polyisoprenylphosphate hexose-1-phosphate transferase uppE result in strong biofilm phenotypes, wherein the ability of A. tumefaciens to produce a biofilm is nearly abrogated. B. abortus 2308 uppC and uppE homologs restore biofilm production in the respective Agrobacterium upp mutants, indicating that uppC and uppE from B. abortus 2308 produce functional proteins. A uppCE mutant in B. abortus 2308 also exhibits significant attenuation in mice and an altered intracellular replication profile in cultured murine macrophages. Accordingly, uppCE appears to be required for virulence in B. abortus 2308, although by a currently unknown mechanism. Additional studies will be needed to determine whether B. abortus 2308 produces an authentic UPP as well as define the mechanisms by which the putative upp genes contribute to virulence

A Wzx-Wzy-like Polysaccharide Biosynthetic Pathway Contributes to Virulence in Brucella abortus 2308 through an Unknown Mechanism

Brucella abortus is an intracellular pathogen that causes spontaneous abortion in cattle and undulant fever in humans. As a member of the a-proteobacteria, B. abortus is closely related to the bacterial species Caulobacter crescentus and Agrobacterium tumefaciens, which have been shown to produce a holdfast and a unipolar polysaccharide (UPP), respectively, at one pole of the bacterial cell. Although little is currently known about exopolysaccharide production and function in Brucella species, recent evidence suggests that these bacteria are capable of producing exopolysaccharides, and with the exception of a flippase gene, B. abortus 2308 carries the genes for a complete UPP biosynthetic pathway. In A. tumefaciens, UPP can be detected by crystal violet, Congo red, or lectin staining; however, UPP production was not detected in B. abortus 2308 using these assays. UPP production can also be increased in A. tumefaciens by overexpressing the diguanylate cyclase PleD, which regulates intracellular cyclic di-GMP levels, but we were unable to demonstrate that UPP is regulated by cyclic di-GMP in B. abortus 2308. Nonetheless, upp homologs in B. abortus 2308 are able to restore biofilm production in A. tumefaciens. In A. tumefaciens, knockout mutants of the outer membrane transporter uppC and the polyisoprenylphosphate hexose-1-phosphate transferase uppE result in strong biofilm phenotypes, wherein the ability of A. tumefaciens to produce a biofilm is nearly abrogated. B. abortus 2308 uppC and uppE homologs restore biofilm production in the respective Agrobacterium upp mutants, indicating that uppC and uppE from B. abortus 2308 produce functional proteins. A uppCE mutant in B. abortus 2308 also exhibits significant attenuation in mice and an altered intracellular replication profile in cultured murine macrophages. Accordingly, uppCE appears to be required for virulence in B. abortus 2308, although by a currently unknown mechanism. Additional studies will be needed to determine whether B. abortus 2308 produces an authentic UPP as well as define the mechanisms by which the putative upp genes contribute to virulence

Analgésie postopératoire après chirurgie modérément douloureuse (paracétamol plus kétoprofène versus parécoxib)

LIMOGES-BU Médecine pharmacie (870852108) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

A Wzx-Wzy-like Polysaccharide Biosynthetic Pathway Contributes to Virulence in Brucella abortus 2308 through an Unknown Mechanism

Brucella abortus is an intracellular pathogen that causes spontaneous abortion in cattle and undulant fever in humans. As a member of the a-proteobacteria, B. abortus is closely related to the bacterial species Caulobacter crescentus and Agrobacterium tumefaciens, which have been shown to produce a holdfast and a unipolar polysaccharide (UPP), respectively, at one pole of the bacterial cell. Although little is currently known about exopolysaccharide production and function in Brucella species, recent evidence suggests that these bacteria are capable of producing exopolysaccharides, and with the exception of a flippase gene, B. abortus 2308 carries the genes for a complete UPP biosynthetic pathway. \nIn A. tumefaciens, UPP can be detected by crystal violet, Congo red, or lectin staining\; however, UPP production was not detected in B. abortus 2308 using these assays. UPP production can also be increased in A. tumefaciens by overexpressing the diguanylate cyclase PleD, which regulates intracellular cyclic di-GMP levels, but we were unable to demonstrate that UPP is regulated by cyclic di-GMP in B. abortus 2308. \nNonetheless, upp homologs in B. abortus 2308 are able to restore biofilm production in A. tumefaciens. In A. tumefaciens, knockout mutants of the outer membrane transporter uppC \nand the polyisoprenylphosphate hexose-1-phosphate transferase uppE result in strong biofilm phenotypes, wherein the ability of A. tumefaciens to produce a biofilm is nearly abrogated. B. abortus 2308 uppC and uppE homologs restore biofilm production in the respective Agrobacterium upp mutants, indicating that uppC and uppE from B. abortus 2308 produce functional proteins.\nA uppCE mutant in B. abortus 2308 also exhibits significant attenuation in mice and an altered intracellular replication profile in cultured murine macrophages. Accordingly, uppCE appears to be required for virulence in B. abortus 2308, although by a currently unknown mechanism. Additional studies will be needed to determine whether B. abortus 2308 produces an authentic UPP as well as define the mechanisms by which the putative upp genes contribute to virulence

A Wzx-Wzy-like Polysaccharide Biosynthetic Pathway Contributes to Virulence in Brucella abortus 2308 through an Unknown Mechanism

Brucella abortus is an intracellular pathogen that causes spontaneous abortion in cattle and undulant fever in humans. As a member of the a-proteobacteria, B. abortus is closely related to the bacterial species Caulobacter crescentus and Agrobacterium tumefaciens, which have been shown to produce a holdfast and a unipolar polysaccharide (UPP), respectively, at one pole of the bacterial cell. Although little is currently known about exopolysaccharide production and function in Brucella species, recent evidence suggests that these bacteria are capable of producing exopolysaccharides, and with the exception of a flippase gene, B. abortus 2308 carries the genes for a complete UPP biosynthetic pathway. \nIn A. tumefaciens, UPP can be detected by crystal violet, Congo red, or lectin staining\; however, UPP production was not detected in B. abortus 2308 using these assays. UPP production can also be increased in A. tumefaciens by overexpressing the diguanylate cyclase PleD, which regulates intracellular cyclic di-GMP levels, but we were unable to demonstrate that UPP is regulated by cyclic di-GMP in B. abortus 2308. \nNonetheless, upp homologs in B. abortus 2308 are able to restore biofilm production in A. tumefaciens. In A. tumefaciens, knockout mutants of the outer membrane transporter uppC \nand the polyisoprenylphosphate hexose-1-phosphate transferase uppE result in strong biofilm phenotypes, wherein the ability of A. tumefaciens to produce a biofilm is nearly abrogated. B. abortus 2308 uppC and uppE homologs restore biofilm production in the respective Agrobacterium upp mutants, indicating that uppC and uppE from B. abortus 2308 produce functional proteins.\nA uppCE mutant in B. abortus 2308 also exhibits significant attenuation in mice and an altered intracellular replication profile in cultured murine macrophages. Accordingly, uppCE appears to be required for virulence in B. abortus 2308, although by a currently unknown mechanism. Additional studies will be needed to determine whether B. abortus 2308 produces an authentic UPP as well as define the mechanisms by which the putative upp genes contribute to virulence

: [French-style bundled payments ? Feedback on an experiment in episode-based payment in orthopaedics and oncology to improve coordination]

International audienceThis article presents the initial results of a national experiment aimed at introducing episode-based bundled payments in the French healthcare system. Launched in 2019, restricted for the moment to three pathologies (hip and knee replacement and colectomy for cancer) and to around 40 hospitals, this experiment is presented as a step to move away from a much criticized activity-based payment system and to promote value-based payment. Episodes of care’s financing aims to pay healthcare organizations on the basis of a flat rate payment for a group of acts and services associated with a pathology upstream, during and downstream of in hospital surgery. Coupled with quality objectives, bundled payments are expected to support both quality and efficiency gains by incentivizing hospitals to optimize patients’ pathways and care coordination between acute care hospitals and primary care providers. Based on a qualitative empirical study, this article analyzes the extent to which the incentives associated with the introduction of this payment method participate to transform care practices at the hospital level. Building on a multidisciplinary approach (health policy and management and health sociology) and reflecting on the literature on management tools, policy instruments and algorithmic regulation, we show that the financing framework used to calculate the reimbursement package does not yet produce the expected incentives in terms of care coordination.Cet article rend compte des premiers résultats d’une expérimentation, lancée en France en 2019, portant sur l’introduction d’un mode de financement des soins à l’épisode de soins (EDS). Le principe de ce mode de financement est de rémunérer les organisations de soin sur la base d’un forfait couvrant un ensemble d’actes et de prestations associés à une pathologie (l’épisode de soins) en amont, pendant et en aval des soins délivrés à l’hôpital. Censé permettre à la fois des gains de qualité et des gains d’efficience, il vise à répondre aux critiques portées à la tarification à l’activité, notamment en termes de coordination des soins entre ville et hôpital. Appuyé sur une enquête qualitative menée auprès des établissements participants, cet article propose d’analyser le déploiement du paiement à l’épisode de soins en France en posant la question de la congruence entre les objectifs de coordination ville-hôpital, les incitatifs tels que élaborés par les équipes techniques nationales et les réceptions locales par les établissements participants. Après avoir présenté la singularité de ce mode de financement en mobilisant la littérature sur les outils de gestion, les instruments d’action publique et la sociologie de la quantification, nous montrons qu’EDS produit une série d’incitatifs répondant, à l’heure actuelle, seulement partiellement aux objectifs de coordination ville-hôpital