314 research outputs found

    Fusionless surgery in early-onset scoliosis

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    AbstractBackgroundSurgical treatment of early-onset scoliosis has greatly developed in recent years. Early-onset scoliosis covers a variety of etiologies (idiopathic, neurologic, dystrophic, malformative, etc.) with onset before the age of 5 years. Progression and severity threaten respiratory development and may result in respiratory failure in adulthood. Many surgical techniques have been developed in recent years, aiming to protect spinal and thoracic development.Material and methodsPresent techniques are based on one of two main principles. The first consists in posterior distraction of the spine in its concavity (single growing rod, or vertical expandable prosthetic titanium rib [VEPTR]), or on either side (dual rod); this requires iterative surgery, for lengthening, unless motorized using energy provided by a magnetic system. The second option is to use spinal growth force to lengthen the assembly; these techniques (Luque Trolley, Shilla), using a sliding assembly, are known as growth guidance.ResultsThese techniques are effective in controlling early scoliotic deformity, and to some extent restore spinal growth. However, they show a high rate of complications: infection, rod breakage, spinal fixation pull out and, above all, progressive spinal stiffness, reducing long-term efficacy. Respiratory gain is harder to assess, as thoracic expansion does not systematically improve respiratory function, particularly due to impaired compliance of the thoracic cage

    Transfusion Related Acute Lung Injury (TRALI) Caused by Red Blood Cell Transfusion Involving Residual Plasma Anti-HLA Antibodies: A report on two Cases and General Considerations

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    TRALI is considered a serious hazard among immune complications of blood transfusion and its occurrence is admitted to be globally underestimated. Each type of blood product is likely to cause TRALI. We report here on two consecutive observations of TRALI caused by red blood cell concentrates, in which anti-HLA class I and class II antibodies resulting from post-gravitational allo-immunization were evidenced in donors. HLA class I and II antigenic community between recipients and donors' husbands were found and strong reacting IgG antibodies directed at several of those common antigens were detected in the donors' serum. Both donors had more than 3 pregnancies, raising the issue of blood donor selection or of plasma reduction for cellular products

    Vector cavity solitons in broad area Vertical-Cavity Surface-Emitting lasers

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    We report the experimental observation of two-dimensional vector cavity solitons in a Vertical-Cavity Surface-Emitting Laser (VCSEL) under linearly polarized optical injection when varying optical injection linear polarization direction. The polarization of the cavity soliton is not the one of the optical injection as it acquires a distinct ellipticity. These experimental results are qualitatively reproduced by the spin-flip VCSEL model. Our findings open the road to polarization multiplexing when using cavity solitons in broad-area lasers as pixels in information technology

    Memory in Interaction: An Analysis of Repeat Calls to a Home Birth Helpline

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    Drawing on a corpus of 80 calls to a Home Birth helpline, we use conversation analysis to analyze how callers and call takers display to one another that they are talking for a second or subsequent time. We focus in particular on the role of memory in these interactions. We show how caller and call taker are oriented to remembering at the beginning of calls as displayed in what we call the recognition-solicit pre-sequence, how participants are oriented to issues of forgetting and remembering during the course of repeat calls, and how remembering and forgetting are made manifest in interaction. Our analysis shows how the human capacity to remember and propensity to forget have reverberating implications in calling for help

    In vitro activity of an aqueous allicin extract and a novel allicin topical gel formulation against Lancefield group B streptococci

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    Background: Studies have shown the efficacy of intra-partum antibiotics in preventing early-onset group B streptococcal sepsis. This approach results in a high intra-partum antibiotic use. Worryingly, the same antibiotics used in prophylaxis are also first-line treatment for neonatal sepsis, and antibiotic exposure in the peri-natal period has been shown to be a risk factor for late-onset serious bacterial infections and allergic disease. Antibiotic exposure in the peri-natal period is becoming a major public health issue; alternative strategies are needed. Garlic has been traditionally used to treat vaginal infections. Allicin is the main antibacterial agent isolated from garlic. Objectives: The aim of the study was to investigate the in vitro activity of a novel allicin extract in aqueous and gel formulation against 76 clinical isolates of Lancefield group B streptococci (GBS). Methods: MICs and MBCs of allicin were determined for 76 GBS isolates by agar dilution and microtitre plate methods. Killing kinetics were determined for a selected 16 of the 76 strains. Agar diffusion tests were compared for allicin liquid and gel (500 mg/L). Results and conclusions: MICs and MBCs of allicin liquid were 35 to 95 mg/L and 75 to 315 mg/L, respectively. Time/dose kill curves produced a 2–3 log reduction in cfu/mL within 3 h and no detectable growth at 8 and 24 h. A novel 500 mg/L allicin gel produced an average zone size of 23+-6 mm compared with 21+-6 mm for allicin in water. Aqueous allicin is bactericidal against GBS isolates and maintains activity in a novel gel formulation

    The Impact of the Physical Environment on Intrapartum Maternity Care: Identification of Eight Crucial Building Spaces.

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    OBJECTIVES, PURPOSE, OR AIM: This article investigates whether the physical environment in which childbirth occurs impacts the intrapartum intervention rates and how this might happen. The study explores the spatial physical characteristics that can support the design of spaces to promote the health and well-being of women, their supporters, and maternity care professionals. BACKGROUND: Medical interventions during childbirth have consequences for the health of women and babies in the immediate and long term. The increase in interventions is multifactorial and may be influenced by the model of care adopted, the relationships between caregivers and the organizational culture, which is made up of many factors, including the built environment. In the field of birth architecture research, there is a gap in the description of the physical characteristics of birth environments that impact users' health. METHOD: A scoping review on the topic was performed to understand the direct and indirect impacts of the physical environment on birth intervention rates. RESULTS AND DISCUSSION: The findings are organized into three tables reporting the influence that the physical characteristics of a space might have on people's behaviors, experiences, practices and birth health outcomes. Eight building spaces that require further investigation and research were highlighted: unit layout configuration, midwives' hub/desk, social room, birth philosophy vectors, configuration of the birth room, size and shape of the birth room, filter, and sensory elements. CONCLUSIONS: The findings show the importance of considering the physical environment in maternity care and that further interdisciplinary studies focused on architectural design are needed to enrich the knowledge and evidence on this topic and to develop accurate recommendations for designers

    High prevalence of PRPH2 in autosomal dominant retinitis pigmentosa in france and characterization of biochemical and clinical features.

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    PURPOSE: To assess the prevalence of PRPH2 in autosomal dominant retinitis pigmentosa (adRP), to report 6 novel mutations, to characterize the biochemical features of a recurrent novel mutation, and to study the clinical features of adRP patients. DESIGN: Retrospective clinical and molecular genetic study. METHODS: Clinical investigations included visual field testing, fundus examination, high-resolution spectral-domain optical coherence tomography (OCT), fundus autofluorescence imaging, and electroretinogram (ERG) recording. PRPH2 was screened by Sanger sequencing in a cohort of 310 French families with adRP. Peripherin-2 protein was produced in yeast and analyzed by Western blot. RESULTS: We identified 15 mutations, including 6 novel and 9 previously reported changes in 32 families, accounting for a prevalence of 10.3% in this adRP population. We showed that a new recurrent p.Leu254Gln mutation leads to protein aggregation, suggesting abnormal folding. The clinical severity of the disease in examined patients was moderate with 78% of the eyes having 1-0.5 of visual acuity and 52% of the eyes retaining more than 50% of the visual field. Some patients characteristically showed vitelliform deposits or macular involvement. In some families, pericentral RP or macular dystrophy were found in family members while widespread RP was present in other members of the same families. CONCLUSIONS: The mutations in PRPH2 account for 10.3% of adRP in the French population, which is higher than previously reported (0%-8%) This makes PRPH2 the second most frequent adRP gene after RHO in our series. PRPH2 mutations cause highly variable phenotypes and moderate forms of adRP, including mild cases, which could be underdiagnosed

    Monocyte-driven atypical cytokine storm and aberrant neutrophil activation as key mediators of COVID-19 disease severity.

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    Epidemiological and clinical reports indicate that SARS-CoV-2 virulence hinges upon the triggering of an aberrant host immune response, more so than on direct virus-induced cellular damage. To elucidate the immunopathology underlying COVID-19 severity, we perform cytokine and multiplex immune profiling in COVID-19 patients. We show that hypercytokinemia in COVID-19 differs from the interferon-gamma-driven cytokine storm in macrophage activation syndrome, and is more pronounced in critical versus mild-moderate COVID-19. Systems modelling of cytokine levels paired with deep-immune profiling shows that classical monocytes drive this hyper-inflammatory phenotype and that a reduction in T-lymphocytes correlates with disease severity, with CD8+ cells being disproportionately affected. Antigen presenting machinery expression is also reduced in critical disease. Furthermore, we report that neutrophils contribute to disease severity and local tissue damage by amplification of hypercytokinemia and the formation of neutrophil extracellular traps. Together our findings suggest a myeloid-driven immunopathology, in which hyperactivated neutrophils and an ineffective adaptive immune system act as mediators of COVID-19 disease severity

    Evolutionary history of hepatitis C virus genotype 5a in France, a multicenter ANRS study

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    The epidemic history of HCV genotype 5a is poorly documented in France, where its prevalence is very low, except in a small central area, where it accounts for 14.2% of chronic hepatitis C cases. A Bayesian coalescent phylogenetic investigation based on the E1 envelope gene and a non-structural genomic segment (NS3/4) was carried out to trace the origin of this epidemic using a large sample of genotype 5a isolates collected throughout France. The dates of documented transmissions by blood transfusion were used to calibrate five nodes in the phylogeny. The results of the E1 gene analysis showed that the best-fitting population dynamic model was the expansion growth model under a relaxed molecular clock. The rate of nucleotide substitutions and time to the most recent common ancestors (tMRCA) of genotype 5a isolates were estimated. The divergence of all the French HCV genotype 5a strains included in this study was dated to 1939 [95% HPD: 1921–1956], and the tMRCA of isolates from central France was dated to 1954 [1942–1967], which is in agreement with epidemiological data. NS3/4 analysis provided similar estimates with strongly overlapping HPD values. Phylodynamic analyses give a plausible reconstruction of the evolutionary history of HCV genotype 5a in France, suggesting the concomitant roles of transfusion, iatrogenic route and intra-familial transmission in viral diffusion

    Holoprosencephaly

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    Holoprosencephaly (HPE) is a complex brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and the 28th day of gestation and affecting both the forebrain and the face. It is estimated to occur in 1/16,000 live births and 1/250 conceptuses. Three ranges of increasing severity are described: lobar, semi-lobar and alobar HPE. Another milder subtype of HPE called middle interhemispheric variant (MIHF) or syntelencephaly is also reported. In most of the cases, facial anomalies are observed in HPE, like cyclopia, proboscis, median or bilateral cleft lip/palate in severe forms, ocular hypotelorism or solitary median maxillary central incisor in minor forms. These latter midline defects can occur without the cerebral malformations and then are called microforms. Children with HPE have many medical problems: developmental delay and feeding difficulties, epilepsy, instability of temperature, heart rate and respiration. Endocrine disorders like diabetes insipidus, adrenal hypoplasia, hypogonadism, thyroid hypoplasia and growth hormone deficiency are frequent. To date, seven genes have been positively implicated in HPE: Sonic hedgehog (SHH), ZIC2, SIX3, TGIF, PTCH, GLI2 and TDGF1. A molecular diagnosis can be performed by gene sequencing and allele quantification for the four main genes SHH, ZIC2, SIX3 and TGIF. Major rearrangements of the subtelomeres can also be identified by multiplex ligation-dependent probe amplification (MLPA). Nevertheless, in about 70% of cases, the molecular basis of the disease remains unknown, suggesting the existence of several other candidate genes or environmental factors. Consequently, a "multiple-hit hypothesis" of genetic and/or environmental factors (like maternal diabetes) has been proposed to account for the extreme clinical variability. In a practical approach, prenatal diagnosis is based on ultrasound and magnetic resonance imaging (MRI) rather than on molecular diagnosis. Treatment is symptomatic and supportive, and requires a multidisciplinary management. Child outcome depends on the HPE severity and the medical and neurological complications associated. Severely affected children have a very poor prognosis. Mildly affected children may exhibit few symptoms and may live a normal life
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