Search

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.

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.

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.

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.

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.

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.

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.

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.