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L’administration de sédatif ou analgésique par voie intranasale est une alternative facile, efficace et sûre à l’administration intraveineuse quand celle-ci est impossible ou non souhaitée. Plusieurs études attestent de la faisabilité chez les nouveau-nés dans un but de sédation avec du midazolam ou de dexmédétomidine pour la réalisation d’intubation ou d’exploration radiologique.

Purpose Nasal continuous positive airway pressure (nCPAP) is currently the gold standard for respiratory support for moderate to severe acute viral bronchiolitis (AVB). Although oxygen delivery via high flow nasal cannula (HFNC) is increasingly used, evidence of its efficacy and safety is lacking in infants. Methods A randomized controlled trial was performed in five pediatric intensive care units (PICUs) to compare 7 cmH 2 O nCPAP with 2 L/kg/min oxygen therapy administered with HFNC in infants up to 6 months old with moderate to severe AVB. The primary endpoint was the percentage of failure within 24 h of randomization using prespecified criteria. To satisfy noninferiority, the failure rate of HFNC had to lie within 15% of the failure rate of nCPAP. Secondary outcomes included success rate after crossover, intubation rate, length of stay, and serious adverse events. Results From November 2014 to March 2015, 142 infants were included and equally distributed into groups. The risk difference of −19% (95% CI −35 to −3%) did not allow the conclusion of HFNC noninferiority ( p  = 0.707). Superiority analysis suggested a relative risk of success 1.63 (95% CI 1.02–2.63) higher with nCPAP. The success rate with the alternative respiratory support, intubation rate, durations of noninvasive and invasive ventilation, skin lesions, and length of PICU stay were comparable between groups. No patient had air leak syndrome or died. Conclusion In young infants with moderate to severe AVB, initial management with HFNC did not have a failure rate similar to that of nCPAP. This clinical trial was recorded in the National Library of Medicine registry (NCT 02457013).

PurposeHigh-flow nasal cannula (HFNC) therapy is increasingly proposed as first-line respiratory support for infants with acute viral bronchiolitis (AVB). Most teams use 2 L/kg/min, but no study compared different flow rates in this setting. We hypothesized that 3 L/kg/min would be more efficient for the initial management of these patients.MethodsA randomized controlled trial was performed in 16 pediatric intensive care units (PICUs) to compare these two flow rates in infants up to 6 months old with moderate to severe AVB and treated with HFNC. The primary endpoint was the percentage of failure within 48 h of randomization, using prespecified criteria of worsening respiratory distress and discomfort.ResultsFrom November 2016 to March 2017, 142 infants were allocated to the 2-L/kg/min (2L) flow rate and 144 to the 3-L/kg/min (3L) flow rate. Failure rate was comparable between groups: 38.7% (2L) vs. 38.9% (3L; p = 0.98). Worsening respiratory distress was the most common cause of failure in both groups: 49% (2L) vs. 39% (3L; p = 0.45). In the 3L group, discomfort was more frequent (43% vs. 16%, p = 0.002) and PICU stays were longer (6.4 vs. 5.3 days, p = 0.048). The intubation rates [2.8% (2L) vs. 6.9% (3L), p = 0.17] and durations of invasive [0.2 (2L) vs. 0.5 (3L) days, p = 0.10] and noninvasive [1.4 (2L) vs. 1.6 (3L) days, p = 0.97] ventilation were comparable. No patient had air leak syndrome or died.ConclusionIn young infants with AVB supported with HFNC, 3 L/kg/min did not reduce the risk of failure compared with 2 L/kg/min. This clinical trial was recorded on the National Library of Medicine registry (NCT02824744).

BackgroundMalignant pertussis (MP) affects young infants and is characterised by respiratory distress, associated with permanent tachycardia and hyperleukocytosis up to 50 × 109L-1 leading to multiple organ failure and death in 75% of cases. Leukodepletion aims to improve prognosis.MethodThis study aimed at identifying factors associated with death and evaluating the impact of treatment on the outcome. We reviewed the records of the infants ≤3 months, hospitalised in 7 French paediatric intensive care units (PICUs) from January 1, 2008 to November 13, 2013with a diagnosis of pertussis, admitted for respiratory distress, with or without white blood cell count (WBCC) >50 × 109L-1. Treatment modalities (Leukodepletion and/or ECMO) were compared to Rowland’s proposition (Rowlands et al, paediatrics 2010).ResultsTwenty three infants were included, 17 were intubated. Nine of 23 (40%) died: they presented more frequently cardiovascular failure (100% vs 36%, p = 0.003) and pulmonary hypertension (PHT) (100% vs 29%, p = 0.002) than survivors. Fatal cases presented CRP level at emergency >20 mg/l (85% vs 14%, p = 0,003) and increased their WBCC three times faster than survivors (15 × 109L-1pd vs 5 × 109L-1pd, p = 0,013). Leukodepletion was performed in 10 cases (43%), 7 survived. For 15/23 patients, the treatment followed Rowlandsprotocol, resulting in a 73% survival rate (11/15).ConclusionA CRP level over 20 mg/l at hospital admission and an increase of WBCC >15×109L-1pd were associated with death. Early determination of CRP, early monitoring of WBCC every 12h, and determination of PHT by echo should be helpful in predicting the prognosis of MP and initiating Leukodepletion.

ObjectiveTo compare the effectiveness of sedation by intranasal administration of midazolam (nMDZ) or ketamine (nKTM) for neonatal intubation.DesignA multicentre, prospective, randomised, double-blind study.SettingDelivery rooms at four tertiary perinatal centres in France.PatientsPreterm neonates with respiratory distress requiring non-emergent endotracheal intubation for surfactant instillation.InterventionsTreatment was randomly allocated, with each neonate receiving a bolus of 0.1 mL/kg in each nostril, corresponding to 0.2 mg/kg for nMDZ and 2 mg/kg for nKTM. The drug was repeated once 7 min later at the same dose if adequate sedation was not obtained.Main outcome measuresSuccess was defined by adequate sedation before intubation and adequate comfort during the procedure. Intubation features, respiratory and cardiovascular events were recorded.ResultsSixty newborns, with mean (SD) gestational age and birth weight of 28 (3) weeks and 1100 (350) g, were included within the first 20 min of life. nMDZ was associated with a higher success rate (89% vs 58%; RR: 1.54, 95% CI 1.12 to 2.12, p<0.01) and shorter delays between the first dose and intubation (10 (6) vs 16 (8) min, p<0.01).Number of attempts, time to intubation, mean arterial blood pressure measures over the first 12 hours after birth and length of invasive ventilation were not different.ConclusionsnMDZ was more efficient than nKTM to adequately sedate neonates requiring intubation in the delivery room. The haemodynamic and respiratory effects of both drugs were comparable.Clinical trialThis clinical trial was recorded on the National Library of Medicine registry (NCT01517828).

BackgroundMalignant pertussis (MP) affects young infants and is characterised by respiratory distress, associated with permanent tachycardia and hyperleukocytosis up to 50 109L-1 leading to multiple organ failure and death in 75% of cases. Leukodepletion aims to improve prognosis.MethodThis study aimed at identifying factors associated with death and evaluating the impact of treatment on the outcome. We reviewed the records of the infants less than or equal to 3 months, hospitalised in 7 French paediatric intensive care units (PICUs) from January 1, 2008 to November 13, 2013with a diagnosis of pertussis, admitted for respiratory distress, with or without white blood cell count (WBCC) >50 109L-1. Treatment modalities (Leukodepletion and/or ECMO) were compared to Rowland's proposition (Rowlands et al, paediatrics 2010).ResultsTwenty three infants were included, 17 were intubated. Nine of 23 (40%) died: they presented more frequently cardiovascular failure (100% vs 36%, p = 0.003) and pulmonary hypertension (PHT) (100% vs 29%, p = 0.002) than survivors. Fatal cases presented CRP level at emergency >20 mg/l (85% vs 14%, p = 0,003) and increased their WBCC three times faster than survivors (15 109L-1pd vs 5 109L-1pd, p = 0,013). Leukodepletion was performed in 10 cases (43%), 7 survived. For 15/23 patients, the treatment followed Rowlandsprotocol, resulting in a 73% survival rate (11/15).ConclusionA CRP level over 20 mg/l at hospital admission and an increase of WBCC >15109L-1pd were associated with death. Early determination of CRP, early monitoring of WBCC every 12h, and determination of PHT by echo should be helpful in predicting the prognosis of MP and initiating Leukodepletion.

Heterozygous mutations in KMT2B are associated with an early-onset, progressive, and often complex dystonia (DYT28). Key characteristics of typical disease include focal motor features at disease presentation, evolving through a caudocranial pattern into generalized dystonia, with prominent oromandibular, laryngeal, and cervical involvement. Although KMT2B-related disease is emerging as one of the most common causes of early-onset genetic dystonia, much remains to be understood about the full spectrum of the disease. We describe a cohort of 53 patients with KMT2B mutations, with detailed delineation of their clinical phenotype and molecular genetic features. We report new disease presentations, including atypical patterns of dystonia evolution and a subgroup of patients with a non-dystonic neurodevelopmental phenotype. In addition to the previously reported systemic features, our study has identified co-morbidities, including the risk of status dystonicus, intrauterine growth retardation, and endocrinopathies. Analysis of this study cohort (n = 53) in tandem with published cases (n = 80) revealed that patients with chromosomal deletions and protein-truncating variants had a significantly higher burden of systemic disease (with earlier onset of dystonia) than those with missense variants. Eighteen individuals had detailed longitudinal data available after insertion of deep brain stimulation for medically refractory dystonia. Median age at deep brain stimulation was 11.5 years (range: 4.5 to 37.0 years). Follow-up after deep brain stimulation ranged from 0.25 to 22 years. Significant improvement of motor function and disability (as assessed by the Burke-Fahn-Marsden Dystonia Rating Scales, BFMDRS-M and BFMDRS-D) was evident at 6 months, 1 year, and last follow-up (motor, P = 0.001, P = 0.004, and P = 0.012; disability, P = 0.009, P = 0.002, and P = 0.012).

Ge–Sb–Se–Te chalcogenides, namely Se-substituted Ge–Sb–Te, have been developed as an alternative optical phase change material (PCM) with a high figure-of-merit. A need for the integration of such new PCMs onto a variety of photonic platforms has necessitated the development of fabrication processes compatible with diverse material compositions as well as substrates of varying material types, shapes, and sizes. This study explores the application of chemical solution deposition as a method capable of creating conformally coated layers and delves into the resulting modifications in the structural and optical properties of Ge–Sb–Se–Te PCMs. Specifically, we detail the solution-based deposition of Ge–Sb–Se–Te layers and present a comparative analysis with those deposited via thermal evaporation. We also discuss our ongoing endeavor to improve available choice of processing-material combinations and how to realize solution-derived high figure-of-merit optical PCM layers, which will enable a new era for the development of reconfigurable photonic devices.

Durup de baleine Antoine. Énergies renouvelables Autorisation d'exploiter un parc éolien situé en haute mer, Cour administrative d'appel de Nantes, 5ème chambre, 15 mai 2017, Association PROSIMAR et autres, req. n° 16NT02321- Conclusions. In: Revue Juridique de l'Environnement, n°3, 2017. pp. 549-580.