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International audience; A panel of pediatric experts met to develop recommendations on the technical requirements specific to pediatric controlled donation after planned withdrawal of life-sustaining therapies (Maastricht category III). The panel recommends following the withdrawal of life-sustaining therapies protocol usually applied in each unit, which may or may not include immediate extubation. The organ retrieval process should be halted if death does not occur within 3 h of life-support discontinuation. Circulatory arrest is defined as loss of pulsatile arterial pressure and should be followed by a 5-min no-touch observation period. Death is declared based on a list of clinical criteria assessed by two senior physicians. The no-flow time should be no longer than 30, 45, and 90 min for the liver, kidneys, and lungs, respectively. At present, the panel does not recommend pediatric heart donation after death by circulatory arrest. The mean arterial pressure cutoff that defines the start of the functional warm ischemia (FWI) phase is 45 mmHg in patients older than 5 years and/or weighing more than 20 kg. The panel recommends normothermic regional perfusion in these patients. The FWI phase should not exceed 30 and 45 min for retrieving the pancreas and liver, respectively. There is no time limit to the FWI phase for the lungs and kidneys. The panel recommends routine sharing of experience with Maastricht-III donation among all healthcare institutions involved in order to ensure optimal outcome assessment and continuous discussion on the potential difficulties, notably those related to the management of normothermic regional perfusion in small children.

Neurological involvement is frequent in inherited metabolic disease of the intoxication type. Hyperammonemic coma related to these diseases may cause severe neurological sequelae. Early optimal treatment is mandatory combining metabolite scavengers (MS) and sometimes continuous veno-venous hemodialysis (CVVHD). We aimed to describe the therapeutic management of hyperammonemia in neonates upon diagnosis of their metabolic disease and to compare neonates managed with MS alone or with both MS and CVVHD. We conducted a retrospective study including all neonates admitted for initial hyperammonemia to the pediatric intensive care unit of a Reference Center of Inherited Metabolic Diseases, between 2001 and 2012. The study included 35 neonates. Before admission, MS were initiated for 11 neonates. At admission, the median ammonia levels were 391 μmol/L and were significantly lower in neonates who received MS before admission. At admission, ammonia levels were 644 μmol/L in dialyzed and 283 μmol/L in non-dialyzed neonates. The median time to reach a 50% decrease of the initial ammonia levels was significantly shorter in dialyzed neonates; however, the normalization of ammonia levels was similar between dialyzed and non-dialyzed neonates. Hemodynamic disorders were more frequent in dialyzed neonates. Conclusion MS represent an effective treatment for hyperammonemia and should be available in all pediatric units to avoid the need for CVVHD. Although CVVHD enhances the kinetics of toxic metabolite decrease, it is associated with adverse hemodynamic effects.

International audience; The French Transplant Health Authority (Agence de la Biomédecine) has broadened its organ- and tissue-donation criteria to include pediatric patients whose death is defined by circulatory criteria and after the planned withdrawal of life-sustaining therapies (WLST) (Maastricht category III). A panel of pediatric experts convened to translate data in the international literature into recommendations for organ and tissue donation in this patient subgroup. The panel estimated that, among children aged 5 years or over with severe irreversible neurological injury (due to primary neurological injury or post-anoxic brain injury) and no progression to brain death, the number of potential donors, although small, deserves attention. The experts emphasized the importance of adhering strictly to the collegial procedure for deciding to withdraw life support. Once this decision is made, the available data should be used to evaluate whether the patient might be a potential donor, before suggesting organ donation to the parents. This suggestion should be reserved for parents who have unequivocally manifested their acceptance of WLST. The discussion with the parents should include both the pediatric intensive care unit (PICU) team under the responsibility of a senior physician and the hospital organ- and tissue-procurement team. All recommendations about family care during the end of life of a child in the PICU must be followed. The course and potential challenges of organ donation in Maastricht-III pediatric patients must be anticipated. The panel of experts recommended strict compliance with French recommendations (by the Groupe Francophone de Réanimation et Urgences Pédiatriques) about WLST and providing deep and continuous sedation until circulatory arrest. The experts identified the PICU as the best place to implement life-support discontinuation and emphasized the importance of returning the body to the PICU after organ donation. French law prohibits the transfer of these patients from one hospital to another. A description of the expert-panel recommendations regarding the organization and techniques appropriate for children who die after controlled circulatory arrest (Maastricht III) is published simultaneously in the current issue of this journal..

Resume En 2005, le Groupe francophone de reanimation et d’urgences pediatriques (GFRUP) publiait des recommandations concernant les limitations et arrets des traitements (LAT) en reanimation pediatrique. Depuis cette date, le Code de sante publique (CSP), modifie par les lois de 2005 et 2016 et leurs decrets d’application, est venu encadrer ces pratiques. Quinze ans apres leur parution, ces recommandations necessitaient d’etre actualisees pour tenir compte de l’experience acquise par les equipes, des nouvelles questions soulevees par des etudes de pratique, des recommandations parues dans l’intervalle, ainsi que des evolutions legislatives et des rares jurisprudences. Ce texte a pour objectif d’aider les equipes de reanimation pediatrique a conjuguer l’ethique de la prise en charge de l’enfant, de sa famille et le respect des dispositions reglementaires actuelles.

Une prophétie autoréalisatrice (PAR) [self-fulfilling prophecy] désigne le fait que la croyance en une situation entraîne sa réalisation, renforçant alors la croyance initiale. D’abord décrite en sociologie, la notion de PAR a également une pertinence en médecine, car la pratique clinique se fonde en grande partie sur le pronostic des patients et de leurs maladies. Ainsi, la croyance en un pronostic péjoratif peut être à l’origine d’une prise en charge médicale qui aboutira à une évolution défavorable ou ne l’empêchera pas. Ce risque est particulièrement aigu en médecine intensive-réanimation où les pratiques de limitation ou d’arrêt des thérapeutiques (LAT) conduisent fréquemment au décès du patient. Il est d’autant plus prégnant que les grandes séries de données à partir desquelles le pronostic d’un patient est évalué incluent des malades ayant fait l’objet de décisions de LAT. Cela peut aboutir à un raisonnement circulaire, difficile à interrompre. Les patients de neuroréanimation, victimes d’accidents vasculaires cérébraux, d’encéphalopathies anoxo-ischémiques postarrêt cardiaque, ou de traumatismes crâniens, sont particulièrement sujets au risque de PAR, car la perspective de séquelles neurologiques sévères motive souvent une décision de LAT. Prendre le contre-pied des PAR nécessite de trouver un compromis entre le temps d’observation indispensable pour établir un pronostic le plus précis possible et la prolongation de traitements invasifs qui s’avérerait a posteriori inutile chez des patients sans espoir d’amélioration neurologique.

International audience; In 2005, the French-speaking task force on pediatric critical and emergency care [Groupe Francophone de Réanimation et d'Urgences Pédiatriques (GFRUP)] issued recommendations on withholding and withdrawing treatments in pediatric critical care. Since then, the French Public Health Code, modified by the laws passed in 2005 and 2016 and by their enactment decrees, has established a legal framework for practice. Now, 15 years later, an update of these recommendations was needed to factor in the experience acquired by healthcare teams, new questions raised by practice surveys, the recommendations issued in the interval, the changes in legislation, and a few legal precedents. The objective of this article is to help pediatric critical care teams find the closest possible compromise between the ethical principles guiding the care offered to the child and the family and compliance with current regulations and laws.

Objectives The aim of this study was to describe severe forms of novel coronavirus disease 2019 in children, including patient characteristics, clinical, laboratory, and imaging findings, as well as the disease management and outcomes. Methods This was a retrospective, single-center, observational study conducted in a pediatric intensive and high-dependency care unit (PICU, HDU) in an urban hospital in Paris. All patients, aged from 1 month to 18 years, admitted for confirmed or highly suspected SARS-CoV-2 were included. Results We analyzed the data of 27 children. Comorbidities (n = 19, 70%) were mainly neurological (n = 7), respiratory, (n = 4), or sickle cell disease (n = 4). SARS-CoV-2 PCR results were positive in 24 children (nasopharyngeal swabs). The three remaining children had a chest CT scan consistent with COVID-19. Respiratory involvement was observed in 24 patients (89%). Supportive treatments were invasive mechanical ventilation (n = 9), catecholamine (n = 4), erythropheresis (n = 4), renal replacement therapy (n = 1), and extracorporeal membrane oxygenation (n = 1). Five children died, of whom three were without past medical history. Conclusion This study highlighted the large spectrum of clinical presentation and time course of disease progression as well as the non-negligible occurrence of pediatric life-threatening and fatal cases of COVID-19 mostly in patients with comorbidities. Additional laboratory investigations are needed to further analyze the mechanism underlying the variability of SARS-Cov-2 pathogenicity in children.

En réanimation pédiatrique, 40 % des décès surviennent à la suite d’une décision de limitation ou d’arrêt de traitement (LAT). Ces situations sont sources de questionnements éthiques complexes au sein de l’équipe soignante. La législation française et les recommandations des sociétés savantes donnent un cadre aux réanimateurs pédiatres pour les prises de décisions de LAT. Les enquêtes de pratiques nous montrent qu’ils se sont approprié certains éléments de la procédure collégiale comme la nécessité de la concertation pluriprofessionnelle, l’information et la communication avec les parents. Néanmoins, certains points tels que la présence du consultant, la réalité de la collégialité avec l’expression de toutes les personnes soignantes présentes sont encore insuffisamment appliqués. La place des parents dans les décisions doit être également réfléchie. La collaboration entre les équipes de réanimation pédiatrique et de médecine palliative est une possibilité pour améliorer sensiblement la qualité des soins et de l’accompagnement proposés. Cette collaboration élargit également les possibilités de la réflexion éthique nécessaire dans les situations de fin de vie complexes. L’intégration dans l’enseignement de la réanimation des principes de la médecine palliative est en cours de réflexion.

Pediatric intensive care units (PICUs), whose accessibility to parents raises controversy, often operate under their own rules. Patients are under critical and unstable conditions, often in a life-threatening situation. In this context, the communication with the parents and their participation in the unit may be difficult. Information is a legal, deontological, and moral duty for caregivers, confirmed by the parents' needs. But the ability to enforce them is a challenge, and there is a gap between the theory and the reality. The communication between the parents and the physicians starts at the admission of the child with a family conference. According to the Societe de reanimation de langue francaise (SRLF), the effectiveness of the communication is based on three criteria: the patients' comprehension, their satisfaction and their anxiety and depression. It has been shown that comprehension depends on multiple factors, related on the parents, the physicians, and the medical condition of the child. Regarding the parents' participation in the organization of the service, the parents' presence is becoming an important factor. In the PICU, the parents' status has evolved. They become a member of the care team, as a partner. The best interest of the child is always discussed with the parents, as the person knowing the best their child. This partnership gives them a responsibility, which is complementary to the physician's one, but does not substitute it.

Introduction La necrolyse epidermique toxique (NET) est une reaction d’hypersensibilite le plus souvent induite par des medicaments, potentiellement letale chez l’adulte, de survenue plus rare chez l’enfant. Nous en rapportons 2 observations pediatriques letales, inhabituelles par leurs atteintes extra-cutanees. Observations Deux garcons, âges de 5 (patient 1, P1) et 4 ans (patient 2, P2), etaient hospitalises en USI pour NET ( Tableau 1 ) sans prise medicamenteuse imputable. La recherche extensive d’un agent infectieux recent par PCR et serologies etait negative ( Tableau 2 ). La presentation de la NET etait classique et severe, la quasi-cicatrisation etait obtenue en 1,5 mois pour P1 et 1 mois pour P2. Cette amelioration dermatologique contrastait avec la survenue brutale, a plus d’un mois d’evolution, d’atteintes viscerales graves rapidement evolutives. Chez P1 : survenue a j35 d’un tableau d’encephalite aigue avec syndrome pyramidal et dystonie. Les explorations infectieuses et auto-immunes etaient negatives : PCR virales, synthese intrathecale d’immunoglobulines, interferon α, FAN, anticorps specifiques (anti-NMO, anti-neuronaux et NMDA). L’evolution etait rapidement fatale malgre les bolus de corticoides, immunoglobulines et plasmaphereses. Chez P2 : atteinte respiratoire obstructive severe rapidement evolutive a partir de j30, caillotage vesical, inflammation limbique et ulceres corneens bilateraux sans cicatrisation. Des depots fibrineux etaient presents dans les voies urinaires et des synechies multiples obstruaient les orifices bronchiques a l’endoscopie. Le bilan auto-immun etait negatif. Les bolus de corticoides, la ciclosporine, la greffe repetee de membranes amniotiques, les injections intravitreennes de corticoides etaient inefficaces et l’atteinte respiratoire fatale malgre l’ECMO. Discussion Nos observations dramatiques sont inhabituelles par : – un tournant evolutif a plus d’un mois, avec degradation severe et evolutive par atteinte d’organe (systeme nerveux central et poumon) alors que le processus de cicatrisation cutanee etait bien avance ; – des elements epidemiologiques similaires : âge, sexe, facteur declenchant non identifie malgre une recherche exhaustive, lieu de vie similaire (rural, 130 km entre les 2 villages) ; – une evolution fatale inhabituelle a cet âge. Elles posent la question d’un facteur declenchant commun non identifie par les explorations de routine et peut etre specifique de l’enfant : processus infectieux ? Facteur environnemental ? Predisposition genetique ? Auto-immunite comme en temoignent l’atteinte neurologique (P1) et oculaire inflammatoire (P2) ? Conclusion Ces observations temoignent d’une presentation inhabituelle de la NET de l’enfant. Le regroupement d’observations similaires et un bilan etiologique large (PCR mutiplex, HLA, etc.) sont indispensables pour mieux en comprendre les mecanismes physiopathologiques.

L’arret cardiaque en pediatrie est rare et son pronostic gravissime. Il est le plus souvent l’ultime complication d’une hypoxie ou d’une insuffisance circulatoire. Il s’agit de rythmes non defibrillables (asystolie, activite electrique sans pouls), plus rarement le fait de troubles du rythme ventriculaire (15 %). Les recommandations de prise en charge actualisees en 2015 precisent la duree (1 sec) des insufflations, l’intensite des compressions thoraciques (le tiers du diametre du thorax), renouvellent les algorithmes des rythmes choquables et non choquables d’arret, rappellent l’importance du monitorage post-arret (normothermie, normoxie / normocapnie, normoglycemie, dysfonction myocardique). L’effort doit porter sur sa prevention c’est-a-dire la reconnaissance des situations de detresse a risque de decompensation, l’evaluation hemodynamique des etats septiques et/ou febriles. La formation et l’entrainement par la simulation des equipes a des prises en charge coordonnees autour d’un leader sont des points d’amelioration potentielle tres importants.

Objective To evaluate the role of prenatal prognostic markers obtained routinely by ultrasound examination and magnetic resonance imaging (MRI) in the prediction of development of postnatal pulmonary arterial hypertension (PAH) in isolated congenital diaphragmatic hernia (CDH). Methods One hundred and ten cases of isolated CDH were referred to our fetal medicine unit between January 2004 and April 2013. Mortality and morbidity rates were reviewed for those presenting with postnatal PAH. The following prenatal markers were evaluated as potential predictive factors of PAH: liver position, side of the CDH defect, lung area to head circumference ratio (LHR) and observed/expected LHR (o/e-LHR), which were measured by ultrasound, and observed/expected total fetal lung volume (o/e-TFLV), which was measured by MRI. Univariable logistic regression was used to assess associations. Results PAH was significantly associated with perinatal mortality and morbidity (P < 0.001). The occurrence of PAH decreased significantly with an increasing LHR, o/e-LHR and o/e-TFLV and was significantly increased for cases with an intrathoracic liver, but not for those with right-sided defects. Univariable regression revealed that o/e-TFLV (odds ratio (OR), 0.9 (95% CI, 0.86–0.95); P < 0.05 for percentage unit change in o/e), LHR (OR, 0.19 (95% CI, 0.09–0.40); P < 0.05 for unit change), o/e-LHR (OR, 0.95 (95% CI, 0.93–0.98); P < 0.05 for percentage unit change in o/e) and liver position (OR, 2.82 (95% CI, 1.13–7.00); P < 0.05 for intrathoracic liver) were significant predictors of subsequent PAH. No differences were found after adjusting for gestational age at delivery. The areas under the receiver–operating characteristics curve were 0.80 and 0.75 for o/e-TFLV and o/e-LHR, respectively. Conclusion In cases of CDH, PAH is associated with high rates of mortality and morbidity. Routinely obtained prenatal markers, usually used for the assessment of pulmonary hypoplasia, are also relevant for the postnatal prediction of PAH. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.

Congenital chylothorax is a rare disease and prognostic factors are key element in properly informing parents. This study aimed at determining the prenatal factors associated with neonatal survival in a cohort of liveborn infants with congenital chylothorax. Observational monocentric cohort study including all liveborn neonates consecutively admitted for congenital chylothorax. Neonatal mortality was 32% (16/50). Prematurity (or birth weight), persistence of hydrops at birth and the absence of thoracoamniotic shunt procedure were significantly associated with mortality, whereas prenatal diagnosis of pleural effusion, side of pleural effusion, hydrops fetalis and amniodrainage were not. In case of prenatal diagnosis of hydrops fetalis, the reversal in utero of hydrops fetalis was significantly associated with survival (P=0.001). In case of thoracoamniotic shunting, the interval between thoracoamniotic shunting intervention and delivery was significantly longer for patients who survived (P=0.03). Thoracoamniotic shunting and reversal of hydrops significantly improves survival, whereas prematurity worsened outcome of liveborn infants with congenital chylothorax. Our data also suggest that the interval between thoracoamniotic shunting and birth appears to be crucial; the longer the interval, the more likely is the reversal of antenatal hydrops and neonatal survival.

Resume Introduction et objectifs Preciser la connaissance des soignants de reanimation pediatrique (RP) sur la loi Leonetti, connaitre leurs opinions vis-a-vis des decisions de limitation ou arret des therapeutiques (LAT) et leur ressenti sur la facon dont ces decisions ont ete prises et appliquees. Materiel et methode Etude multicentrique par questionnaire durant le dernier trimestre 2010. Resultats Mille cinq personnels paramedicaux et 334 medecins ont repondu. Plus de 85 % avaient une bonne connaissance des grands principes de la Loi. Plus de 80 % des soignants adheraient aux LAT lorsqu’elles concernaient une suppleance respiratoire, hemodynamique ou renale. En revanche, l’arret de la nutrition et de l’hydratation artificielle etait recuse ou generait des reticences chez la majorite des repondants. Alors que la deliberation collegiale etait estimee indispensable par l’ensemble des soignants, les personnels paramedicaux trouvaient beaucoup plus souvent que les medecins que leur implication reelle etait insuffisante. La qualite des fins de vie etait jugee tres favorablement par les soignants. Cependant, les reponses concernant les modalites d’application des LAT suggerent des interpretations tres differentes de la Loi : certains respectaient les principes des soins palliatifs tels qu’ils sont definis par le code de sante publique, alors que 40 % des medecins et 64 % des soignants jugeaient « acceptable » de provoquer un deces si la decision etait collegiale. Conclusion Cette etude est la premiere a montrer que les soignants des unites de RP francaises ont une bonne connaissance de la loi Leonetti. Il persiste cependant des divergences importantes sur les pratiques jugees legalement acceptables en fin de vie.

Objective To investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH). Methods Our database was searched to identify all consecutive cases of CDH referred to our fetal medicine unit between January 2004 and August 2011. Presence or absence of a hernia sac was assessed in liveborn cases using surgery or postnatal autopsy reports. We studied the correlation between the presence of a hernia sac and prenatal findings and perinatal morbidity and mortality. Results Over the study period, there were 70 cases with isolated CDH born alive in which either a surgery or autopsy report was available. Neonatal death, either preoperative or postoperative, occurred in 1/18 (5.6%) infants with a hernia sac and in 17/52 (32.7%) cases without a hernia sac (P = 0.03). Patients with a hernia sac had a significantly higher observed to expected pulmonary volume on prenatal magnetic resonance imaging (51.9 vs 39.3%, P = 0.01). Neonatal morbidity in surviving infants was lower in the group with a hernia sac, although not significantly. Conclusion The presence of a hernia sac is associated with a higher pulmonary volume and a better overall prognosis for CDH. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.

Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined. In this article, we will first describe the current knowledge on the BPD-associated PH and the current treatments available for this pathology. We will then present the HTP-DBP Study, carried out in Paris (France) starting in 2012. The diagnosis of PH is suspected on echocardiographic criteria, but cardiac catheterization is considered the gold standard for diagnosis and evaluation of the severity of PH. Moreover, pulmonary vasoreactivity testing is used to guide the management of patients with PH. The pathogenesis of BPD-associated PH is poorly understood and even less is known about appropriate therapy. Today, optimizing ventilation and reducing the pulmonary vascular tone with specific pulmonary vasodilatator drugs are the main goals in treating HTP-associated DBP. Animal studies and a few clinical studies suggest that medications targeting the nitric oxide (NO) signaling pathway (NO inhalation, oral sildenafil citrate) could be effective treatments for BPD-associated PH, but they have not been approved for this indication. The HTP-DBP study is a French multicenter prospective observational study. The objective is to evaluate the frequency of BPD-associated PH, to describe its physiopathology, its severity (morbidity and mortality), and the effectiveness of current treatments.

The objective of this review was to summarize the current knowledge base on the prevention of nosocomial infections in pediatric intensive care units (PICUs). Healthcare-associated infections (HAIs) are a crucial problem in PICUs because of their impact on patient outcome, length of hospital stay, and costs. Studies published between 1998 and 2011 were identified using the MEDLINE and Cochrane databases. Randomized, cohort, case-control studies, and meta-analyses concerning global strategies of prevention, general organization of the wards, general recommendations on antibiotic management, and measures for the prevention of ventilator-associated pneumonia (VAP), bloodstream infections (BSIs), urinary tract infections (UTIs), and surgical site infections (SSIs) were incorporated. Limits of age from 1 month to 18 years were used. When recommendations could not be supported by the pediatric literature, adult studies were also reviewed. This review excludes the neonate population. Specific pediatric data are often lacking so as to establish specific evidence-based pediatric recommendations. This review underlines the absolute necessity of pediatric studies and to harmonize the definitions of HAIs.

Pediatric intensive care units (PICUs), whose accessibility to parents raises controversy, often operate under their own rules. Patients are under critical and unstable conditions, often in a life-threatening situation. In this context, the communication with the parents and their participation in the unit may be difficult. Information is a legal, deontological, and moral duty for caregivers, confirmed by the parents' needs. But the ability to enforce them is a challenge, and there is a gap between the theory and the reality. The communication between the parents and the physicians starts at the admission of the child with a family conference. According to the Société de réanimation de langue française (SRLF), the effectiveness of the communication is based on three criteria: the patients' comprehension, their satisfaction and their anxiety and depression. It has been shown that comprehension depends on multiple factors, related on the parents, the physicians, and the medical condition of the child. Regarding the parents' participation in the organization of the service, the parents' presence is becoming an important factor. In the PICU, the parents' status has evolved. They become a member of the care team, as a partner. The best interest of the child is always discussed with the parents, as the person knowing the best their child. This partnership gives them a responsibility, which is complementary to the physician's one, but does not substitute it.

Resumen Menos del 10 % de los recien nacidos requieren una reanimacion activa. La rapidez y la calidad de los cuidados iniciales condicionan el pronostico del bebe y la aparicion de un sufrimiento vital neonatal es a menudo imprevisible. La reanimacion del recien nacido esta bien estandarizada y debe ser realizada por un equipo profesional calificado que disponga del material necesario en todos los partos. La apreciacion de la gravedad de la situacion del recien nacido se basa esencialmente en parametros clinicos sencillos. La aspiracion precoz de la faringe y la desobstruccion de las vias respiratorias constituyen a menudo los primeros elementos del tratamiento de las insuficiencias respiratorias del recien nacido, en particular cuando el liquido amniotico aspirado esta tenido de meconio. Los progresos de la reanimacion permiten indices importantes de supervivencia sin secuelas mayores en caso de gran prematuro o hipotrofia mayor. En este contexto suele proponerse el empleo de tecnicas menos invasivas. La existencia de malformaciones debe conducir a un tratamiento rapido por parte de un personal especializado.

To evaluate the role of prenatal prognostic markers obtained routinely by ultrasound examination and magnetic resonance imaging (MRI) in the prediction of development of postnatal pulmonary arterial hypertension (PAH) in isolated congenital diaphragmatic hernia (CDH).One hundred and ten cases of isolated CDH were referred to our fetal medicine unit between January 2004 and April 2013. Mortality and morbidity rates were reviewed for those presenting with postnatal PAH. The following prenatal markers were evaluated as potential predictive factors of PAH: liver position, side of the CDH defect, lung area to head circumference ratio (LHR) and observed/expected LHR (o/e-LHR), which were measured by ultrasound, and observed/expected total fetal lung volume (o/e-TFLV), which was measured by MRI. Univariable logistic regression was used to assess associations.PAH was significantly associated with perinatal mortality and morbidity (P0.001). The occurrence of PAH decreased significantly with an increasing LHR, o/e-LHR and o/e-TFLV and was significantly increased for cases with an intrathoracic liver, but not for those with right-sided defects. Univariable regression revealed that o/e-TFLV (odds ratio (OR), 0.9 (95% CI, 0.86-0.95); P0.05 for percentage unit change in o/e), LHR (OR, 0.19 (95% CI, 0.09-0.40); P0.05 for unit change), o/e-LHR (OR, 0.95 (95% CI, 0.93-0.98); P0.05 for percentage unit change in o/e) and liver position (OR, 2.82 (95% CI, 1.13-7.00); P0.05 for intrathoracic liver) were significant predictors of subsequent PAH. No differences were found after adjusting for gestational age at delivery. The areas under the receiver-operating characteristics curve were 0.80 and 0.75 for o/e-TFLV and o/e-LHR, respectively.In cases of CDH, PAH is associated with high rates of mortality and morbidity. Routinely obtained prenatal markers, usually used for the assessment of pulmonary hypoplasia, are also relevant for the postnatal prediction of PAH.

The authors describe a case of heparin-induced skin reaction due to two preparations of low molecular weight heparin in a pregnant woman. The main characteristics of heparin-related cutaneous allergy are reported. The use of an heparinoid, usually indicated for patients with heparin-induced thrombocytopenia, appeared to be efficient and safe for the mother and her fetus. An epidural analgesia was performed for labor analgesia, 24 hours after the last injection of danaparid of sodium.

To identify the knowledge of caregivers of pediatric intensive care units (PICUs) on the French law related to patients' rights and end of life, their views on withholding/withdrawing life-sustaining treatment (WWLST) decisions, and their feelings about how these decisions were made and implemented.A multicenter survey in 24 French PICUs during the fourth trimester 2010.One thousand three hundred and thirty-nine professional healthcare workers (1005 paramedics and 334 physicians) responded. Over 85% of caregivers had good knowledge of the WWLST decision-making processes required by law. More than 80% of caregivers accepted mechanical ventilation, hemodiafiltration, or hemodynamic support withdrawal or withholding. Nevertheless, the withdrawal of artificial nutrition and hydration generated reluctance or opposition for the majority of respondents. While paramedics' participation in the decision-making process was deemed necessary by all caregivers, paramedics found more often than physicians that they were insufficiently involved. The quality of end-of-life care was judged very positively by caregivers. The answers on how WWLST was applied suggest very different interpretations of the law. Some caregivers respect the principles of palliative care as stated in the public health code and 40% of doctors and 64% of caregivers consider it "acceptable" to hasten death if resulting from a collaborative decision-making process.This study is the first to show that caregivers of French PICUs have good knowledge of the French law concerning the end of life. Yet, there is still confusion about the limits of practice during the end-of-life period.

Nous ne traiterons dans ce chapitre que le cas des nourrissons et enfants admis en reanimation medico-chirurgicale, en excluant notamment les nouveau-nes prematures. Les mesures applicables a tout malade hospitalise independamment de son âge (par exemple, usage des solutions hydro-alcooliques, isolement par confinement des germes a potentiel epidemique) ne seront pas abordees dans leurs aspects, hors specificites pediatriques.

Dans le domaine du soin, et tout particulierement en reanimation, l’ethique est le plus souvent assimilee a l’analyse et a la resolution de tensions ou de conflits de valeurs dans une situation concrete et singuliere. On parle volontiers d’ethique clinique pour nommer la demarche collegiale qui cherche a discerner la meilleure (ou la moins mauvaise) decision, illustree par les decisions de limitation ou d’arret de traitements de suppleance vitale (LAT) visant a eviter ou a faire cesser une obstination deraisonnable.

To investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH).Our database was searched to identify all consecutive cases of CDH referred to our fetal medicine unit between January 2004 and August 2011. Presence or absence of a hernia sac was assessed in liveborn cases using surgery or postnatal autopsy reports. We studied the correlation between the presence of a hernia sac and prenatal findings and perinatal morbidity and mortality.Over the study period, there were 70 cases with isolated CDH born alive in which either a surgery or autopsy report was available. Neonatal death, either preoperative or postoperative, occurred in 1/18 (5.6%) infants with a hernia sac and in 17/52 (32.7%) cases without a hernia sac (P = 0.03). Patients with a hernia sac had a significantly higher observed to expected pulmonary volume on prenatal magnetic resonance imaging (51.9 vs 39.3%, P = 0.01). Neonatal morbidity in surviving infants was lower in the group with a hernia sac, although not significantly.The presence of a hernia sac is associated with a higher pulmonary volume and a better overall prognosis for CDH.

Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined. In this article, we will first describe the current knowledge on the BPD-associated PH and the current treatments available for this pathology. We will then present the HTP-DBP Study, carried out in Paris (France) starting in 2012. The diagnosis of PH is suspected on echocardiographic criteria, but cardiac catheterization is considered the gold standard for diagnosis and evaluation of the severity of PH. Moreover, pulmonary vasoreactivity testing is used to guide the management of patients with PH. The pathogenesis of BPD-associated PH is poorly understood and even less is known about appropriate therapy. Today, optimizing ventilation and reducing the pulmonary vascular tone with specific pulmonary vasodilatator drugs are the main goals in treating HTP-associated DBP. Animal studies and a few clinical studies suggest that medications targeting the nitric oxide (NO) signaling pathway (NO inhalation, oral sildenafil citrate) could be effective treatments for BPD-associated PH, but they have not been approved for this indication. The HTP-DBP study is a French multicenter prospective observational study. The objective is to evaluate the frequency of BPD-associated PH, to describe its physiopathology, its severity (morbidity and mortality), and the effectiveness of current treatments.

Prognosis of adult patients with cystic fibrosis (CF), admitted to intensive care unit (ICU), has improved. Few data are available for children. The aim is to describe clinical course and outcomes of CF children hospitalized in ICU. Baseline characteristics of children, reasons for admission in ICU, ventilatory support and outcomes at discharge from ICU were retrospectively analyzed. Non parametric statistical test compared data of survivors at ICU to those with poor outcome (death or lung transplantation in super emergency). From 2000 to 2013, 26 children, median age 13.4 years (1.9–17.6), were admitted to ICU (Necker-Enfants Malades, Paris). Before admission, 15 children (58%) had a non-invasive ventilation (NIV) and 23 (88%) were colonized with Pseudomonas aeruginosa . The median forced expiratory volume (FEV 1 ) was 32% (13–101). Eighteen children (69%) were admitted for respiratory exacerbation, NIV was initiated for 18 (69%) and 9 (35%) were intubated. Half of the 26 children had a poor outcome, 5 (19%) died and 8 (31%) needed urgent lung transplantation. Poor outcome-associated factors were female sex (p = 0.015), history of hemoptysis (p = 0.03), ventilation (p = 0.001) or nutritional (p = 0.017) support before ICU, chronic intravenous antibiotics use (p = 0.001), lower FEV 1 before ICU (27% (13–49) for poor outcome group and 40% (17–101) for survival group, p=0.036), admission for respiratory exacerbation (p = 0.030), hypercapnia (p = 0.010), metabolic acidosis (p = 0.007) and hypochloremia (p = 0.013). Our study highlights poor outcomes in children hospitalized in ICU and points factors associated with poor outcomes. This will help to improve indications of pediatric lung transplant.

Background: Clinical history is insufficient to detect disorders of haemostasis in infants aged less than 1 year and laboratory coagulation testing is recommended in infants before perimedullar anaesthesia. Methods: We designed a retrospective study to assess the preoperative coagulation tests [activated partial thromboplastin time (aPTT), prothrombin time, platelet count] performed in 141 former preterm infants who underwent spinal anaesthesia for elective inguinal hernia repair. All the infants had recovered from any significant medical event (necrotizing enterocolitis, intracranial haemorrhages, transfusions of blood products, sepsis, etc.) when they were scheduled for surgery. Results: Mean values for aPTT were frequently abnormal (60.4%) in infants with a postconceptional age of less than 45 weeks. No complication related to the puncture or abnormal perioperative bleeding was detected. Conclusions: Other coagulation tests should be evaluated to assess the coagulation status in infants with a postconceptional age of less than 45 weeks.

The authors describe a case of heparin-induced skin reaction due to two preparations of low molecular weight heparin in a pregnant woman. The main characteristics of heparin-related cutaneous allergy are reported. The use of an heparinoid, usually indicated for patients with heparin-induced thrombocytopenia, appeared to be efficient and safe for the mother and her fetus. An epidural analgesia was performed for labor analgesia, 24 hours after the last injection of danaparid of sodium.

Objectifs Definir les facteurs pronostiques de survie chez les nouveau-nes atteints de chylothorax congenital. Patients et Methodes Etude retrospective mono-centrique des 29 nouveau-nes hospitalises en reanimation pour chylothorax congenital entre 1999 et 2009. Recherche de facteurs de risque de deces en uni-varie par test de Fisher ou de Mann-Whitney. Resultats 11/29 enfants sont decedes (38 %), 6 d’insuffisance respiratoire, 3 d’insuffisance renale organique et 2 de cause mixte. Sur 4 autopsies, 2 ont retrouve une hypoplasie pulmonaire, 1 des lymphangiectasies pulmonaires. Les facteurs de risque de deces sont : l’importance de la prematurite (p = 0,039), l’absence de drainage antenatal (64 % de deces versus 22 % ; p = 0,048), la presence d’une anasarque postnatale (75 % de deces versus 24 % ; p = 0,028), la naissance en etat de mort apparente (p = 0,01), la necessite d’une evacuation pleurale postnatale (p = 0,019), l’association a une pathologie generale (83 % de deces versus 26 % ; p = 0,018). Conclusions La severite du chylothorax congenital est principalement liee a son association avec une insuffisance respiratoire constitutionnelle, persistant malgre un bon controle de l’epanchement pleural postnatal.

Premedication Practice for Neonatal Intubation in French Nicus and Picus: Description, Analysis and Identification of Potential Barriers to Good Practice Implementation