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Purpose: To identify the risk factors of early occurrence of malnutrition in infants with severe congenital heart disease (CHD) during their first year of life. Methods and Results: Retrospective longitudinal multicentre study carried out from January 2014 to December 2020 in two tertiary care CHD centres. Four CHD hemodynamic groups were identified. Malnutrition was defined by a Waterlow score under 80% and/or the need for calorie-enriched formula. A total of 216 infants with a severe CHD, e.g., requiring cardiac surgery, cardiac catheterization, or hospitalization for heart failure during their first year of life were included in the study. Malnutrition was observed among 43% of the cohort, with the highest prevalence in infants with increased pulmonary blood flow (71%) compared to the others hemodynamic groups (pConclusion: In infants with a severe CHD, early occurrence of malnutrition during the first year of life affected a high proportion of subjects. CHD with increased pulmonary blood flow, low birthweight, heart failure, and repeated hospitalizations were risk factors for malnutrition. Further studies are required to identify optimal nutritional support in this population.

International audience; BackgroundAntenatal betamethasone is recommended before preterm delivery to accelerate fetal lung maturation. However, reports of growth and neurodevelopmental dose-related side-effects suggest that the current dose (12 mg plus 12 mg, 24 h apart) might be too high. We therefore investigated whether a half dose would be non-inferior to the current full dose for preventing respiratory distress syndrome.MethodsWe designed a randomised, multicentre, double-blind, placebo-controlled, non-inferiority trial in 37 level 3 referral perinatal centres in France. Eligible participants were pregnant women aged 18 years or older with a singleton fetus at risk of preterm delivery and already treated with the first injection of antenatal betamethasone (11·4 mg) before 32 weeks’ gestation. We used a computer-generated code producing permuted blocks of varying sizes to randomly assign (1:1) women to receive either a placebo (half-dose group) or a second 11·4 mg betamethasone injection (full-dose group) 24 h later. Randomisation was stratified by gestational age (before or after 28 weeks). Participants, clinicians, and study staff were masked to the treatment allocation. The primary outcome was the need for exogenous intratracheal surfactant within 48 h after birth. Non-inferiority would be shown if the higher limit of the 95% CI for the between-group difference between the half-dose and full-dose groups in the primary endpoint was less than 4 percentage points (corresponding to a maximum relative risk of 1·20). Four interim analyses monitoring the primary and the secondary safety outcomes were done during the study period, using a sequential data analysis method that provided futility and non-inferiority stopping rules and checked for type I and II errors. Interim analyses were done in the intention-to-treat population. This trial was registered with ClinicalTrials.gov, NCT02897076.FindingsBetween Jan 2, 2017, and Oct 9, 2019, 3244 women were randomly assigned to the half-dose (n=1620 [49·9%]) or the full-dose group (n=1624 [50·1%]); 48 women withdrew consent, 30 fetuses were stillborn, 16 neonates were lost to follow-up, and 9 neonates died before evaluation, so that 3141 neonates remained for analysis. In the intention-to-treat analysis, the primary outcome occurred in 313 (20·0%) of 1567 neonates in the half-dose group and 276 (17·5%) of 1574 neonates in the full-dose group (risk difference 2·4%, 95% CI –0·3 to 5·2); thus non-inferiority was not shown. The per-protocol analysis also did not show non-inferiority (risk difference 2·2%, 95% CI –0·6 to 5·1). No between-group differences appeared in the rates of neonatal death, grade 3–4 intraventricular haemorrhage, stage ≥2 necrotising enterocolitis, severe retinopathy of prematurity, or bronchopulmonary dysplasia.InterpretationBecause non-inferiority of the half-dose compared with the full-dose regimen was not shown, our results do not support practice changes towards antenatal betamethasone dose reduction.

International audience; Aim: To assess nurses’ ability to observe newborn behaviour after in situ training provided by caregivers with advanced practice certification in the Newborn Individualized Developmental Care and Assessment Program (NIDCAP).Design: Prospective observational study.Methods: Twelve nurses viewed 20-min films showing the behaviour of 10 premature newborns before, during and after the usual caregiving. The behaviour was rated on an observation sheet with 88 items distributed into six systems. The responses were compared to the reference ratings established by two professionals certified for this programme.Results: Despite less accurate observations during care and for some components, the nurses generally showed a satisfactory ability to observe newborn behaviour after training by NIDCAP expert professionals. The dissemination of observation skills among caregivers may result in an improved quality of patient care and better communication among professionals in a department of neonatology.

L’administration de sedatif ou analgesique par voie intranasale est une alternative facile, efficace et sure a l’administration intraveineuse quand celle-ci est impossible ou non souhaitee. Plusieurs etudes attestent de la faisabilite chez les nouveau-nes dans un but de sedation avec du midazolam ou de dexmedetomidine pour la realisation d’intubation ou d’exploration radiologique. Pour les douleurs procedurales, le fentanyl intranasal est le plus utilise, la ketamine est une autre option. La prudence reste de mise dans une population a risque de detresse respiratoire.

AIM To describe maternal employment and the socio-economic status of the household up to 8 years after the very preterm birth of a child, according to the presence and type of motor or cognitive impairment. METHOD A total of 1885 families from the French EPIPAGE cohort of children who were born very preterm between 1997 and 1998 were included. Motor and cognitive impairments were identified in children between the ages of 2 and 8 years in 770 families and were classified according to type. The 1115 families with children born very preterm without these impairments were considered the reference group. RESULTS Mothers of children with severe motor or cognitive impairments were less often working at 5 years after the birth than the reference mothers (21% and 30% vs 57%; p

To compare paediatric patients with cerebral sinovenous thrombosis (CSVT) with and without head/neck infection to improve management of the condition.We conducted a bicentric retrospective study of consecutive children (neonates excluded) with radiologically confirmed CSVT, comparing children with a concurrent head/neck infection and children with other causes.A total of 84 consecutive patients (46 males and 38 females) with a median age of 4 years 6 months (range 3 months-17 years 5 months) were included. Associated head/neck infection was identified in 65.4% of cases and represented the main identified CSVT aetiology. Children in the head/neck infection group displayed a milder clinical presentation and less extensive CSVT. Median time to complete recanalization was significantly shorter in this group (89 days [interquartile range 35-101] vs 112.5 days [interquartile range 83-177], p = 0.005). These findings were even more pronounced in the subgroup of patients with otogenic infection and no neurological sign.As CSVT in the setting of an otogenic infection and no neurological sign seems to represent a milder condition with a shorter course, these results suggest adapting current recommendations: consider earlier control imaging in paediatric otogenic CSVT, and shorter anticoagulant treatment if recanalization is obtained.Children with cerebral sinovenous thrombosis related to head/neck infections have a milder clinical presentation. They also have a shorter recanalization time, especially if there is otogenic infection without neurological symptoms.

Key Points Question Is early amino acid intake among very preterm infants associated with cognitive performance at age 5 years? Findings In this cohort study of 1789 infants born at less than 30 weeks’ gestation, exposure vs nonexposure to amino acid intake between 3.51 and 4.50 g/kg/d at 7 days after birth was significantly and independently associated with a higher likelihood (61% vs 54%, respectively) of surviving with a full-scale IQ score greater than −1 SD at age 5 years. Meaning The results of this study suggest that high early amino acid intake among very preterm infants is safe and significantly associated with improved cognitive outcomes at age 5 years., Importance An international expert committee recently revised its recommendations on amino acid intake for very preterm infants, suggesting that more than 3.50 g/kg/d should be administered only to preterm infants in clinical trials. However, the optimal amino acid intake during the first week after birth in these infants is unknown. Objective To evaluate the association between early amino acid intake and cognitive outcomes at age 5 years. Design, Setting, and Participants Using the EPIPAGE-2 (Epidemiologic Study on Small-for-Gestational-Age Children—Follow-up at Five and a Half Years) cohort, a nationwide prospective population-based cohort study conducted at 63 neonatal intensive care units in France, a propensity score–matched analysis was performed comparing infants born at less than 30 weeks’ gestation who had high amino acid intake (3.51-4.50 g/kg/d) at 7 days after birth with infants who did not. Participants were recruited between April 1 and December 31, 2011, and followed up from September 1, 2016, to December 31, 2017. Full-scale IQ (FSIQ) was assessed at age 5 years. A confirmatory analysis used neonatal intensive care unit preference for high early amino acid intake as an instrumental variable to account for unmeasured confounding. Statistical analysis was performed from January 15 to May 15, 2021. Exposures Amino acid intake at 7 days after birth. Main Outcomes and Measures The primary outcome was an FSIQ score greater than −1 SD (ie, ≥93 points) at age 5 years. A complementary analysis was performed to explore the association between amino acid intake at day 7 as a continuous variable and FSIQ score at age 5 years. Data from cerebral magnetic resonance imaging at term were available for a subgroup of preterm infants who participated in the EPIRMEX (Cerebral Abnormalities Detected by MRI, Realized at the Age of Term and the Emergence of Executive Functions) ancillary study. Results Among 1789 preterm infants (929 boys [51.9%]; mean [SD] gestational age, 27.17 [1.50] weeks) with data available to determine exposure to amino acid intake of 3.51 to 4.50 g/kg/d at 7 days after birth, 938 infants were exposed, and 851 infants were not; 717 infants from each group could be paired. The primary outcome was known in 396 of 646 exposed infants and 379 of 644 nonexposed infants who were alive at age 5 years and was observed more frequently among exposed vs nonexposed infants (243 infants [61.4%] vs 206 infants [54.4%], respectively; odds ratio [OR], 1.33 [95% CI, 1.00-1.71]; absolute risk increase in events [ie, the likelihood of having an FSIQ score >−1 SD at age 5 years] per 100 infants, 7.01 [95% CI, 0.06-13.87]; P = .048). In the matched cohort, correlation was found between amino acid intake per 1.00 g/kg/d at day 7 and FSIQ score at age 5 years (n = 775; β = 2.43 per 1-point increase in FSIQ; 95% CI, 0.27-4.59; P = .03), white matter area (n = 134; β = 144 per mm2; 95% CI, 3-285 per mm2; P = .045), anisotropy of the corpus callosum (n = 50; β = 0.018; 95% CI, 0.016-0.021; P, This cohort study assesses the association between high amino acid intake among very preterm infants at 7 days after birth and cognitive performance at age 5 years.

Différents facteurs sont susceptibles de fragiliser la continuité et la cohérence des prises en charge d’un nouveau-né malade. Certains tiennent à la variabilité, c’està- dire à la différence dans la politique de soins ou des conditions d’annonce d’un établissement à un autre et d’un professionnel à un autre. Habituellement présentée comme un défaut de compétence par les études debenchmarking, cherchant même à être corrigée par l’établissement de recommandations établies par les sociétés savantes, cette variabilité relève sans doute en partie d’autres origines appartenant à « l’appétence » des équipes pour tel ou tel type de pratique médicale ou de communication. Cet article souhaite aider les professionnels de toutes disciplines à prendre conscience de leur positionnement dans la manière de délivrer des soins et de communiquer avec les familles. Il s’agit de cheminer ensemble afin de comprendre pourquoi il est si difficile d’appliquer les recommandations médicales tout en prenant en compte la subjectivité de chaque situation. Limiter l’impact de ce qui peut être ressenti comme un défaut de cohérence médicale aux yeux des parents semble être un but à ne pas lâcher. Les situations de réanimation au seuil de la viabilité nous ont fait faire des progrès considérables dans le mouvement d’anticipation réalisé avec les parents en anténatal pour qu’ils puissent accepter et accompagner le séjour en réanimation de leur enfant et le suivi ultérieur dans une continuité suffisante pour ne pas favoriser de troubles de l’attachement. Il nous appartient maintenant de réfléchir à cette continuité dans des situations moins critiques mais quotidiennes.

Objective In premature neonates, bloody stools and/or abdominal distension with feeding intolerance may be inaugural signs of necrotizing enterocolitis (NEC). We assessed the ability of near-infrared spectroscopy (NIRS) to distinguish those neonates with NEC soon after the occurrence of these symptoms. Study Design We prospectively collected NIRS measurements of abdominal and cerebral regional tissue oxygen saturation (r-SO2), with values masked by an opaque cover. Two physicians, blinded to the NIRS data, determined whether the gastrointestinal symptoms were related to NEC 10 days after symptom onset. Results Forty-five neonates with mean (standard deviation [SD]) gestational, birth weight and postnatal ages of 31 (3.9) weeks, 1,486 (794) g, and 18 (14) days were enrolled over 30 months. Gastrointestinal symptoms were related to NEC in 23 patients and associated with other causes in 22. Analysis of the 48 hours of monitoring revealed comparable abdominal r-SO2 and splanchnic-cerebral oxygenation ratio (SCOR) in patients with and without NEC (r-SO2: 47.3 [20.4] vs. 50.4 [17.8], p = 0.59, SCOR: 0.64 [0.26] vs. 0.69 [0.24], p = 0.51). Results were unchanged after NIRS analysis in 6-hour periods, and restriction of the analysis to severe NEC (i.e., grade 2 and 3, 57% of the NEC cases). Conclusion In this study, NIRS monitoring was unable to individualize NEC in premature infants with acute gastrointestinal symptoms.

Less invasive surfactant administration (LISA) has become increasingly popular in neonatal intensive care units (NICUs), but there are currently no guidelines for the premedication prior to this procedure. The aim of this observational study was to compare the efficacy and tolerance of intravenous administrations of ketamine and propofol before LISA in neonates born before 30 weeks of gestational age (GA). The primary outcome was requirement of intubation within 2 h of the procedure. One hundred and fourteen infants, with respective GA and birthweight of 27.6 (26.4, 28.7) weeks and 940 (805, 1140) g, were prospectively included from January 2016 to December 2019. Drug doses were 1 (0.5, 1) mg/kg for ketamine and 1 (1, 1.9) mg/kg for propofol, providing comparable comfort during LISA (p = 0.61). Rates of intubation within 2 h were 5/52 after ketamine, and 5/62 after propofol [aOR 0.54 (0.11-2.68)]. No difference was observed for rates of intubation at 24 h and 72 h following LISA, mortality, or severe morbidity.Conclusion: Pending results from prospective trials, these findings suggest that ketamine or propofol can be used for premedication before LISA, as they show comparable efficacy and tolerance.Trial registration: This study was recorded on the National Library of Medicine registry (https:// clinicaltrials.gov / Identifier: NCT03705468). What is Known? • Less invasive surfactant administration (LISA) is increasingly used in spontaneously breathing premature infants supported with continuous positive airway pressure, but few data are available to guide adequate premedication for this procedure. What is New? • This observational study of 114 neonates, all less than 30-week gestational age and requiring surfactant without endotracheal tube in the delivery room, suggested that ketamine or propofol can be used for premedication before LISA with comparable efficacy and tolerance.

Background An increased risk for bronchopulmonary dysplasia (BPD) exists when moderate-to-large patent ductus arteriosus shunts (hsPDA) persist beyond 14 days. Goal To examine the interaction between prolonged exposures to tracheal ventilation (≥10 days) and hsPDA on the incidence of BPD in infants Study Design Predefined definitions of prolonged ventilation (≥10 days), hsPDA (≥14 days), and BPD (room air challenge test at 36 weeks) were used to analyze deidentified data from the multicenter TRIOCAPI RCT in a secondary analysis of the trial. Results Among 307 infants who survived >14 days, 41 died before 36 weeks. Among survivors, 93/266 had BPD. The association between BPD and hsPDA depended on the length of intubation. In multivariable analyses, prolonged hsPDA shunts were associated with increased BPD (odds ratio (OR) (95% confidence interval (CI)) = 3.00 (1.58–5.71)) when infants required intubation for ≥10 days. In contrast, there was no significant association between hsPDA exposure and BPD when infants were intubated n = 307): infants intubated ≥10 days: OR (95% CI) = 2.41 (1.47–3.95)); infants intubated Conclusions Moderate-to-large PDAs were associated with increased risks of BPD and BPD/death—but only when infants required intubation ≥10 days. Impact Infants with a moderate-to-large hsPDA that persist beyond 14 days are only at risk for developing BPD if they also receive prolonged tracheal ventilation for ≥10 days. Infants who receive less ventilatory support (intubation for Early PDA closure may be unnecessary in infants who require short durations of intubation since the PDA does not seem to alter the incidence of BPD in infants who require intubation for

Objective To assess the inspiratory demand in young infants with acute viral bronchiolitis to provide a physiological basis for initial flow setting for patients supported with high flow nasal cannula. Study design Prospective study in 44 infants up to 6 months old with acute viral bronchiolitis, admitted to a pediatric intensive care unit from November 2017 to March 2019. Airflow measurements were performed using spirometry. The primary endpoint was the inspiratory demand as measured by peak tidal inspiratory flow (PTIF). The secondary endpoints were the relationships determined between PTIF, patient weight, and disease severity. Results Median (Q25-Q75) age and weight of the patients were 37 (20-67) days and 4.3 (3.5-5.0) kg, respectively. Mean PTIF was 7.45 (95% CI 6.51-8.39, min-max: 2.40-16.00) L/minute. PTIF indexed to weight was 1.68 (95% CI 1.51-1.85, min-max: 0.67-3.00) L/kg/minute. PTIF was Conclusions High flow nasal cannula therapy is used commonly to support infants with acute viral bronchiolitis. The efficiency of the device is optimal if the flow setting matches the patient's inspiratory demand. According to our results, a flow rate of

Background We examined how specific cognitive behavioral impairments impacted quality of life (QoL) within a large multicenter cohort of 7-10 year olds surviving extremely preterm (EPT) without major neurodevelopmental disability. Methods Between 7 and 10 years of age, two generic, self-proxy, and parental evaluations were obtained. QoL measurement questionnaires (Kidscreen-10/VSPA (Vecu et Sante Percue de l'Enfant et de l'Adolescent)) were used and compared to a reference population. The general and specific cognitive functions, such as executive functions, behavior and anxiety, and clinical neurologic examination, were also assessed. Results We analyzed 211 school-aged EPT children. The mean gestational age was 26.2 (±0.8) weeks, birth weight was 879 g (±181) and the mean age was 8.4 years (±0.87). Children with a Full-Scale Index Quotient ≥89, who were considered as normal, had a lower QoL. Specific cognitive impairments: comprehensive language delay, visuo-spatial integration defect, and dysexecutive disorders) were the QoL correlates in the domains of school performance and body image. Conclusions School and health care professionals need to increase their focus on EPT children's lower so as to recognize the preterm behavioral/cognitive phenotype and their potential need for supportive measures. Research on preventive interventions is warranted to investigate if these long-term effects of an EPT birth can be attenuated in neonatal period and after.

International audience; Objective: To examine the effects of early echocardiography-targeted ibuprofen treatment of large patent ductus arteriosus (PDA) on survival without cerebral palsy at 24 months of corrected age.Study design: We enrolled infants born at

Le principe de la musicothérapie repose sur une approche holistique via la stimulation des aspects sensoriels, cognitifs, comportementaux et psycho-sociaux de chacun. La musicothérapie agit sur la variation des paramètres physiologiques comme la fréquence cardiaque, la fréquence respiratoire, et la tension artérielle.La musicothérapie via des logiciels spécifiques (séquence en « U ») ou des intervenants formés est de plus en plus utilisée dans les services de réanimation adulte ainsi que les services de réanimation néonatale. Cette revue présente les indications et les effets de la musicothérapie dans ces services (gestion de l’angoisse et de la douleur chez les patients intubés, ventilés ou non, impact sur le sommeil, le développement psychomoteur). L’expérience en réanimation pédiatrique est encore embryonnaire.Une étude pilote en réanimation pédiatrique incluant 21 enfants suggère que la mise en place de la musicothérapie est faisable avec une bonne intégration de cette méthode lors des soins quotidiens tel que la toilette.

BACKGROUND: Status dystonicus (SD) is a life-threatening condition. OBJECTIVE AND METHODS: In a dystonia cohort who developed status dystonicus, we analyzed demographics, background dystonia phenomenology and complexity, trajectory previous to-, via status dystonicus episodes, and evolution following them. RESULTS: Over 20 years, 40 of 328 dystonia patients who were receiving DBS developed 58 status dystonicus episodes. Dystonia was of pediatric onset (95%), frequently complex, and had additional cognitive and pyramidal impairment (62%) and MRI alterations (82.5%); 40% of episodes occured in adults. Mean disease duration preceding status dystonicus was 10.3 \textpm 8 years. Evolution time to status dystonicus varied from days to weeks; however, 37.5% of patients exhibited progressive worsening over years. Overall, DBS was efficient in resolving 90% of episodes. CONCLUSION: Status dystonicus is potentially reversible and a result of heterogeneous conditions with nonuniform underlying physiology. Recognition of the complex phenomenology, morphological alterations, and distinct patterns of evolution, before and after status dystonicus, will help our understanding of these conditions. \textcopyright 2018 International Parkinson and Movement Disorder Society.

Background Nitric oxide (NO) has a well-known efficacy in pulmonary hypertension (PH), with wide use for 20 years in many countries. The objective of this study was to describe the current use of NO in real life and the gap with the guidelines. Methods This is a multicenter, prospective, observational study on inhaled NO administered through an integrated delivery and monitoring device and indicated for PH according to the market authorizations. The characteristics of NO therapy and ventilation modes were observed. Concomitant pulmonary vasodilator treatments, safety data, and outcome were also collected. Quantitative data are expressed as median (25th, 75th percentile). Results Over 1 year, 236 patients were included from 14 equipped and trained centers: 117 adults and 81 children with PH associated with cardiac surgery and 38 neonates with persistent PH of the newborn. Inhaled NO was initiated before intensive care unit (ICU) admission in 57%, 12.7%, and 38.9% with an initial dose of 10 (10, 15) ppm, 20 (18, 20) ppm, and 17 (11, 20) ppm, and a median duration of administration of 3.9 (1.9, 6.1) days, 3.8 (1.8, 6.8) days, and 3.1 (1.0, 5.7) days, respectively, for the adult population, pediatric cardiac group, and newborns. The treatment was performed using administration synchronized to the mechanical ventilation. The dose was gradually decreased before withdrawal in 86% of the cases according to the usual procedure of each center. Adverse events included rebound effect for 3.4% (95% confidence interval [CI], 0.9%-8.5%) of adults, 1.2% (95% CI, 0.0%-6.7%) of children, and 2.6% (95% CI, 0.1%-13.8%) of neonates and methemoglobinemia exceeded 2.5% for 5 of 62 monitored patients. Other pulmonary vasodilators were associated with NO in 23% of adults, 95% of children, and 23.7% of neonates. ICU stay was respectively 10 (6, 22) days, 7.5 (5.5, 15) days, and 9 (8, 15) days and ICU mortality was 22.2%, 6.2%, and 7.9% for adults, children, and neonates, respectively. Conclusions This study confirms the safety of NO therapy in the 3 populations with a low rate of rebound effect. Gradual withdrawal of NO combined with pulmonary vasodilators are current practices in this population. The use of last-generation NO devices allowed good compliance with recommendations.

Resume La ventilation chez l’enfant a de grandes similarites avec celle de l’adulte. Il existe neanmoins quelques specificites a prendre en compte. Sur un plan physiologique, le poumon de l’enfant est son « talon d’Achille » avec des valeurs de compliance et de resistance tres differentes de celles de l’adulte. Le recours a la VNI est de plus en plus frequent, en premiere intention, pour la prise en charge des detresses respiratoires. Certaines indications sont etayees par des preuves, notamment les bronchiolites virales, avec un taux d’echec tres faible. D’autres restent associees a un taux d’echec important, comme le SDRA. La ventilation de l’enfant premature necessite d’adapter toutes les valeurs aux donnees anthropometriques de l’enfant et d’eviter un certain nombre de pieges.

A partir de 48 heures du post-partum, 78 meres ont repondu a l’autoquestionnaire MIBS (Mother-to-Infant Bonding Scale) traduit en francais ainsi qu’a 3 autres questionnaires. Vingt-quatre heures plus tard, un entretien pedopsychiatrique permettait d’evaluer la relation mere-enfant. Les puericultrices remplissaient le MIBS, en imaginant les reponses des meres.Le MIBS permettait un depistage satisfaisant des difficultes du lien mere-enfant (sensibilite = 0,9 et specificite = 0,8 pour un seuil superieur ou egal a 2). Les scores MIBS etaient independants de l’humeur maternelle (EPDS) et du style d’attachement de la mere (AAQ). Par contre, il existait un lien avec les caracteristiques comportementales du bebe (MAIB). Les scores MIBS de la mere et de la puericultrice etaient faiblement correles et les reponses rarement concordantes ; 100 % des puericultrices estimaient que le MIBS aidait a l’evaluation du lien mere-enfant et 85 % des meres trouvaient un benefice a la passation du questionnaire.

International audience; Background: The pathophysiology of necrotizing enterocolitis (NEC) remains poorly understood. Objective: We assessed the relation between feeding strategies, intestinal microbiota composition, and the development of NEC. Design: We performed a prospective nationwide population-based study, EPIPAGE 2 (Etude Epidemiologique sur les Petits Ages Ges-tationnels), including preterm infants born at,32 wk of gestation in France in 2011. From individual characteristics observed during the first week of life, we calculated a propensity score for the risk of NEC (Bell's stage 2 or 3) after day 7 of life. We analyzed the relation between neonatal intensive care unit (NICU) strategies concerning the rate of progression of enteral feeding, the direct-breastfeeding policy, and the onset of NEC using general linear mixed models to account for clustering by the NICU. An ancillary propensitymatched case-control study, EPIFLORE (Etude Epidemiologique de la flore), in 20 of the 64 NICUs, analyzed the intestinal microbiota by culture and 16S ribosomal RNA gene sequencing. Results: Among the 3161 enrolled preterm infants, 106 (3.4%; 95% CI: 2.8%, 4.0%) developed NEC. Individual characteristics were significantly associated with NEC. Slower and intermediate rates of progression of enteral feeding strategies were associated with a higher risk of NEC, with an adjusted OR of 2.3 (95% CI: 1.2, 4.5; P = 0.01) and 2.0 (95% CI: 1.1, 3.5; P = 0.02), respectively. Less favorable and intermediate direct-breastfeeding policies were associated with higher NEC risk as well, with an adjusted OR of 2.5 (95% CI: 1.1, 5.8; P = 0.03) and 2.3 (95% CI: 1.1, 4.8; P = 0.02), respectively. Microbiota analysis performed in 16 cases and 78 controls showed an association between Clostridium neonatale and Staphylococcus aureus with NEC (P = 0.001 and P = 0.002). Conclusions: A slow rate of progression of enteral feeding and a less favorable direct-breastfeeding policy are associated with an increased risk of developing NEC. For a given level of risk assessed by propensity score, colonization by C. neonatale and/or S. aureus is significantly associated with NEC. This trial (EPIFLORE study) was registered at clinicaltrials.gov as NCT01127698.

Objectif Evaluer la qualite de vie des enfants en âge scolaire nes avant 28 SA et qui n’ont aucune incapacite majeure. Population et methode Etude transversale multicentrique (QPQoL study) incluant des nouveau-nes Resultats Quarante (7,5%) enfants gravement handicapes ont ete exclus. Parmi les 471 enfants eligibles, 169 (36%) etaient perdus de vu lors du suivi et donc 302 (64%) etaient inclus dans l’etude. Le terme moyen de naissance etait de 26.2 SA (± 0.8), le poids moyen de naissance etait de 879 g (± 181) et l’âge moyen de suivi des enfants etait de 8.4 ans (± 0.8). 48% des participants presentaient des deficiences cognitives mineures ou moderees d’apres leur Indice FSIQ. La memoire de travail, l’attention et la flexibilite mentale avaient une moyenne faible par rapport au groupe de reference. Hormis les relations familiales, l’evaluation de la qualite de vie par VSPA et Kidscreen 10 a ete significativement plus faible selon le point de vue des enfants et des parents. Les enfants ont declare une qualite de vie significativement diminuee dans les domaines suivants: les relations amicales, l’estime de soi et les loisirs, tandis que parents avaient indique: le bien-etre psychologique, le travail scolaire et la vitalite. Les enfants ayant un FSIQ ≥ 89, consideres comme normaux, avaient une qualite de vie inferieure. Des deficiences cognitives/comportementales specifiques (retard de langage, dyspraxie visuo-spatiale et troubles dysexecutifs) etaient les determinants significatifs de la qualite de vie dans les domaines des performances scolaires et de l’image corporelle. Conclusion La qualite de vie d’un enfant ne tres grand premature en âge scolaire sans deficience grave etait inferieure a celle d’une population de reference d’enfants nes a terme, tant du point de vue des parents que de celui de l’enfant. Cette evaluation devrait permettre de mieux comprendre le devenir a long terme et d’offrir un soutien adapte a l’enfant et a sa famille. Ethique This study was approved by the CPP (Committee for the Protection of Persons) (18/12/2012 ref 12.018) and is registered on ClinicalTrials.gov, number NCT01675726 .

Objectives: Abdominal distention is a common indicator of feeding intolerance in premature newborns. In the absence of a precise definition, abdominal distention and its degree are highly subjective. The aim of this study was to construct references and smoothed percentiles for abdominal circumference (AC) and AC to head circumference (HC) ratio (AC/HC) in infants born between 24 weeks and 34 weeks of gestational age.Methods: ACs and HCs were collected weekly in eutrophic premature infants without congenital abdominal or cerebral malformation. AC and HC charts were modeled using the LMS method, excluding measures associated with abdominal distention at clinical examination or intracranial abnormality at cerebral ultrasounds. Changes in AC and AC/HC over time were studied by repeated-measures analysis using mixed-effects linear models.Results: A total of 1,605 measurements were made in 373 newborns with a mean gestational age of 31 [29–33] weeks and mean birth weight of 1,540 [1,160–1,968] g. Of these measurements, 1,220 were performed in normal conditions. Gestational age, postnatal age, singleton status, and respiratory support were significantly associated with AC and AC/HC. LMS curves were generated according to gestational age groups and postnatal age, with coherent profiles. AC/HC was 0.91 [0.86–0.95] in absence of abdominal distention. It was higher in cases of abdominal distention (0.95 [0.89–1.00], p < 0.001) and necrotizing enterocolitis (0.98 [0.93–1.07], p < 0.001).Conclusions: References constructed for AC and AC/HC might be used to assess feeding tolerance in premature infants. AC/HC was more relevant than AC to rationalize the diagnosis of abdominal distention.

OBJECTIVES To review 15-year trends in respiratory care of extremely preterm infants managed in a tertiary perinatal center; to identify the factors contributing to their evolution; and to determine whether these changes had an impact on infant mortality, severe morbidity, and growth. METHODS Retrospective cohort study of infants born at 23 to 26 weeks' gestation between 2003 and 2017. Changes in respiratory care were assessed in three 5-year periods. Logistic regression was used to examine the factors associated with prolonged duration (ie, greater than the median) of invasive mechanical ventilation (IMV), noninvasive ventilation (NIV), and overall respiratory support (ORS), and those associated with adequate weight and head circumference growth. RESULTS Of the 396 actively treated neonates, 268 (68%) survived to discharge. Between the first and third periods, IMV duration decreased from 22 (6-37) to 4 (1-14.0) days (P

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: 4.5–37.0 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’s 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). At 1 year post-deep brain stimulation, >50% of subjects showed BFMDRS-M and BFMDRS-D improvements of >30%. In the long-term deep brain stimulation cohort (deep brain stimulation inserted for >5 years, n = 8), improvement of >30% was maintained in 5/8 and 3/8 subjects for the BFMDRS-M and BFMDRS-D, respectively. The greatest BFMDRS-M improvements were observed for trunk (53.2%) and cervical (50.5%) dystonia, with less clinical impact on laryngeal dystonia. Improvements in gait dystonia decreased from 20.9% at 1 year to 16.2% at last assessment; no patient maintained a fully independent gait. Reduction of BFMDRS-D was maintained for swallowing (52.9%). Five patients developed mild parkinsonism following deep brain stimulation. KMT2B-related disease comprises an expanding continuum from infancy to adulthood, with early evidence of genotype-phenotype correlations. Except for laryngeal dysphonia, deep brain stimulation provides a significant improvement in quality of life and function with sustained clinical benefit depending on symptoms distribution.

Introduction: Acute bronchiolitis epidemics are known to destabilize healthcare structures and stand as a major public health issue. Our tertiary care regional university hospital designed an organizational infrastructure paediatric plan (OIPP) to adapt to bronchiolitis epidemics. This study aimed to assess the impact of the OIPP on the length of stay and quality of care of children hospitalized for bronchiolitis. Methods: This epidemiological study analyzed data from 2 epidemic seasons before and after the OIPP implementation. The OIPP used a standardized algorithm of patient orientation and a 4-level stratification of care. Results: A total of 1636 children were included in the study, with 718 children before and 918 children after the OIPP implementation. The length of stay significantly decreased after the OIPP implementation, from 5.1 ± 6.8 days to 3.9 ± 3 days (P

Background Respiratory syncytial virus (RSV) is the most frequently identified pathogen in children with acute lower respiratory tract infection. Fatal cases have mainly been reported during the first 6 months of life or in the presence of comorbidity. Case presentation A 47-month-old girl was admitted to the pediatric intensive care unit following sudden cardiopulmonary arrest occurring at home. The electrocardiogram showed cardiac asystole, which was refractory to prolonged resuscitation efforts. Postmortem analyses detected RSV by polymerase chain reaction in an abundant, exudative pericardial effusion. Histopathological examination was consistent with viral myoepicarditis, including an inflammatory process affecting cardiac nerves and ganglia. Molecular analysis of sudden unexplained death genes identified a heterozygous mutation in myosin light chain 2, which was also found in two other healthy members of the family. Additional expert interpretation of the cardiac histology confirmed the absence of arrhythmogenic right ventricular dysplasia or hypertrophic cardiomyopathy. Conclusions RSV-related sudden death in a normally developing child of this age is exceptional. This case highlights the risk of extrapulmonary manifestations associated with this infection, particularly arrhythmia induced by inflammatory phenomena affecting the cardiac autonomic nervous system. The role of the mutation in this context is uncertain, and it is therefore necessary to continue to assess how this pathogenic variant contributes to unexpected sudden death in childhood.

We examined how specific cognitive behavioral impairments impacted quality of life (QoL) within a large multicenter cohort of 7-10 year olds surviving extremely preterm (EPT) without major neurodevelopmental disability.Between 7 and 10 years of age, two generic, self-proxy, and parental evaluations were obtained. QoL measurement questionnaires (Kidscreen-10/VSPA (Vécu et Santé Perçue de l'Enfant et de l'Adolescent)) were used and compared to a reference population. The general and specific cognitive functions, such as executive functions, behavior and anxiety, and clinical neurologic examination, were also assessed.We analyzed 211 school-aged EPT children. The mean gestational age was 26.2 (±0.8) weeks, birth weight was 879 g (±181) and the mean age was 8.4 years (±0.87). Children with a Full-Scale Index Quotient ≥89, who were considered as normal, had a lower QoL. Specific cognitive impairments: comprehensive language delay, visuo-spatial integration defect, and dysexecutive disorders) were the QoL correlates in the domains of school performance and body image.School and health care professionals need to increase their focus on EPT children's lower so as to recognize the preterm behavioral/cognitive phenotype and their potential need for supportive measures. Research on preventive interventions is warranted to investigate if these long-term effects of an EPT birth can be attenuated in neonatal period and after.

International audience; In the context of a 3-month extended-spectrum beta-lactamase-producing Klebsiella pneumonia (ESBL-KP) outbreak in a neonatal care center (NCC), hygiene practices and hospital environment were investigated. ESBL-KP strains isolated from patients and environment were compared by molecular typing. The density of incidence of multi-drug-resistant bacteria (MDRB) was calculated from January 2014 to September 2016. The 3-month ESBL-KP outbreak involved 19 patients. Clinical strains from the 19 patients displayed the same molecular profile between them, and with a strain isolated from an incubator after cleaning. Furthermore, 52.4% of incubator mattresses were positive for diverse pathogens. Hygiene practices were acceptable except for external practitioners and parents. In addition to classical infection control (IC) measures, the replacement of mattresses and the improvement of incubators disinfection stopped the outbreak. The protocol of disinfection was revised and microbiological control was implemented. A significant decrease of MDRB incidence was concomitant (p value = 0.03219) but 3 months later, MDRB incidence increased again.Conclusion: This investigation highlighted incubators and mattresses as critical materials associated to infectious risk in NCC. NCC and IC teams should implement efficient protocol for incubators disinfection and monitoring. What is Known: • Environment in neonatal intensive care units is often suspected as reservoir for Enterobacteriaceae outbreaks but is scarcely investigated. • Incubators and mattresses offer wet and warm conditions suitable for pathogens multiplication, but microbiological survey is not performed routinely for assessing bacterial contamination. What is New: • Incubators and mattresses serve as reservoir for pathogens and relay in outbreak. • An infection control protocol associating efficient disinfection and microbiology analysis is proposed.

Resume Chez l’enfant, le recours a la ventilation non invasive (VNI) est de plus en plus frequent, en premiere intention, pour la prise en charge des detresses respiratoires. Sous reserve d’une instauration precoce et d’une selection pertinente du mode de VNI selon le type de detresse, ce support respiratoire peut permettre d’eviter l’epuisement respiratoire et le recours a la ventilation invasive. Des progres techniques recents sur les interfaces et les respirateurs rendent sa mise en œuvre plus aisee, notamment par une meilleure compensation des fuites et l’amelioration de l’interaction patient–ventilateur. Certaines indications sont etayees par des preuves, notamment les bronchiolites virales, avec un taux d’echec tres faible. D’autres restent associees a un taux d’echec important, comme le syndrome de detresse respiratoire aigu (SDRA), incitant a une utilisation tres prudente par des equipes expertes. Des indications potentielles, comme l’asthme aigu grave ou le relais de la ventilation invasive, necessitent des evaluations complementaires dans le cadre d’essais cliniques. La diffusion de formations theoriques et pratiques, sous l’egide de la societe europeenne de reanimation du nouveau-ne et de l’enfant (ESPNIC), devrait permettre d’uniformiser et de securiser cette pratique en plein essor.