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Previous reports have demonstrated that tumor necrosis factor alpha (TNF-alpha) plays an important role in the pathogenesis of fulminant hepatic necrosis. The purpose of this experimental study was to measure TNF-alpha blood activity in paracetamol-induced liver necrosis and in coronavirus (MHV3)-induced fulminant hepatitis in mice. No elevation of TNF-alpha activity was found in hepatic failure complicating paracetamol poisoning. In contrast, TNF-alpha activity significantly increased in response to MHV3, reaching 16.3 +/- 5.5 U/ml from 24 h post infection (P less than 0.01). This augmentation was observed even though the virus was not detectable in the liver. Serum alanine aminotransferase levels were low and no histological lesion was observed. In conclusion, our study further supports the implication of TNF-alpha in virus-induced hepatitis failure and confirms that paracetamol poisoning does not cause increased TNF-alpha activity in the circulation.

A paediatric intensive care unit is a complex environment with many hazards for potential medical errors. Due to the high acuity of illness and the complexity of medical care with multiple disciplines and individuals involved, medical errors occur due to breakdowns in communication and teamwork. Medication errors are the most frequent errors in paediatrics. This chapter proposes some strategies to prevent medical errors in paediatric intensive care units.

Resume Les unites de reanimation pediatriques representent un environnement complexe favorable a la survenue de nombreux evenements indesirables. Du fait du caractere aigu des pathologies rencontrees, du nombre de disciplines en cause et du nombre de personnel medical ou paramedical impliques, les evenements indesirables surviennent surtout du fait de problemes de communication. Les evenements indesirables les plus frequents concernent les erreurs de traitement. Ce chapitre propose quelques strategies pour prevenir les evenements indesirables en reanimation pediatrique.

ResumeObjectif L'objectif de ce travail non controle etait d'evaluer la place de la ventilation non invasive (VNI) en mode « nasal continuous positive airway pressure » (NCPAP) dans la prise en charge des formes severes de bronchiolites saisonnieres. Population et methode Sur 2 periodes epidemiques successives (2003–2004, 2004–2005), 145 nourrissons admis en reanimation pediatrique ont ete inclus dont 121 ont necessite en premiere intention une assistance ventilatoire en mode VNI (n = 53) ou par voie endotracheale (VI) (n = 68). Les donnees ont ete recueillies de facon prospective. Resultats Les caracteristiques generales des patients etaient similaires entre les 2 periodes a l'exception de l'âge moyen au moment de l'admission, plus faible dans le groupe VNI (p = 0,003). Le pourcentage d'echec de la VNI, defini par la necessite d'une intubation tracheale, a ete stable sur les 2 periodes (25 %) alors que le pourcentage des enfants ventiles en mode non invasif a double. Les facteurs predictifs identifies d'echec etaient un score de PRISM a h24 plus eleve (9,2 ± 4,1 vs 5,1 ± 3,2, p = 0,02) et une reduction plus faible de la PaCO2 a H2 de NCPAP (p = 0,003). Quelle que soit l'evolution en VNI, une reduction significative de la frequence respiratoire (60 ± 16 vs 47,5 ± 13,7 cycles/minute, p

Summary Objectives The authors describe 28 pediatric cases of foreign body inhalation requiring treatment in intensive care units between 1987 and 1999. The purpose of this study was: (1) to analyze the circumstances, diagnostic difficulties and initial treatment of serious foreign bodies and (2) to compare our series with other literature descriptions and define principles for optimal prevention and initial treatment. Methods Twenty-one children presented a penetration syndrome, which was responsible in 13 cases for asphyxia with cardiorespiratory arrest. All these children died, regardless of the initial treatment. Seven children were hospitalized for apparent asthmatic symptoms that did not respond to traditional treatment. Results The inefficiency of external extraction methods like the Heimlich maneuver and the mean delay between clinical signs and initial treatment lead us to propose a new strategy for the emergency treatment of foreign bodies with asphyxia. Conclusions We recommend that emergency teams promote the use of a laryngoscope and Magill forceps. Flexible endoscopy is still recommended as the appropriate diagnostic tool to eliminate doubt in the case of a first severe asthma attack.

Resume La bronchiolite virale du nourrisson demeure plus que jamais un probleme de sante publique. Le devenir respiratoire des nourrissons qui necessitent un support ventilatoire est peu etudie. Methodes. – Etude retrospective, sur une periode de quatre ans (1994–1998), de tous les dossiers d'enfants ventiles dans l'unite de reanimation pediatrique de l'hopital Bicetre pour bronchiolite. Les renseignements sur le devenir respiratoire ont ete obtenus par contact avec les familles et le medecin traitant. Resultats. – Cent trente-cinq nourrissons ont ete inclus sur la periode d'etude. L'âge median au moment de l'admission etait de 45 jours (extremes 8–396), 33,3 % etant nes avant 37 SA (10 % avant 32nSA). La mortalite etait de 2,9 % (4/135), tous les deces concernant d'anciens prematures ayant ete ventiles en periode neonatale. En analyse univariee, les facteurs associes a la mortalite etaient la prematurite (p = 0,056) et le syndrome de detresse respiratoire aigue (p = 0,017). A deux ans, l'incidence d'asthme du nourrisson etait de 40,4 %. Aucun facteur etudie n'etait associe a l'evolution vers un asthme du nourrisson. Conclusion. – Les bronchiolites admises en unite de reanimation pediatrique concernent essentiellement le petit nourrisson. Les anciens prematures representent une population a risque. L'incidence d'asthme du nourrisson au decours ne semble pas etre augmentee chez les enfants ventiles. Le benefice des techniques de ventilation non invasive meriterait d'etre evalue.

Resume Le syndrome de Reye idiopathique associe classiquement et de facon inexpliquee une encephalopathie non inflammatoire et une steatose hepatique microvesiculaire. Un certain nombre d’etudes cliniques ou epidemiologiques, principalement en langue anglaise, tendent a nier la realite meme de ce syndrome car de nombreuses maladies metaboliques, dont certaines ne sont pas encore identifiees, peuvent comporter une telle association. L’objectif de ce travail etait d’apprecier actuellement la realite des formes graves de syndrome de Reye, diagnostic positif retenu au terme d’un bilan metabolique exhaustif. Methodes. – Etude retrospective, sur une periode de 11 ans (1991–2001) des dossiers d’enfants hospitalises dans une unite de reanimation medicochirurgicale pour un tableau clinique repondant aux criteres de syndrome de Reye probable (Center for Diseases and Control). Tous nos patients ont beneficie, pendant et au decours de l’episode neurologique aigu, d’une investigation metabolique extensive. Resultats. – Quatorze dossiers (l’âge median a l’admission etait de 52 mois) ont ete retenus. Tous les enfants presentaient initialement un tableau febrile justifiant un traitement par acide acetylsalicylique et paracetamol chez respectivement 12 et sept d’entre eux. A l’entree, le score de Glasgow median etait de 7. L’ammoniemie etait en moyenne de 320 μmol/L et le pic d’alanine-aminotransferase (ASAT) de 1 475 ± 1 387 UI/L. Dix enfants ont ete ventiles et six places en hemofiltration veino-veineuse continue. Trois enfants sont decedes precocement en situation d’hypertension intracrânienne et 11 ont gueri sans sequelles ni recidive avec un recul moyen de cinq ans. Les explorations metaboliques standardisees, effectuees dans deux centres de reference, se sont revelees negatives a l’exception d’un enfant chez lequel le diagnostic d’intolerance au fructose a ete pose. Conclusion. – Les formes graves de syndrome de Reye sans etiologie metabolique identifiee n’ont pas disparu dans notre pays, contrairement a une opinion repandue. Elles restent grevees d’une importante mortalite et relevent d’une prise en charge agressive et precoce, plus particulierement centree sur la lutte contre l’œdeme cerebral. Si le lien de causalite entre consommation de salicyles et survenue d’un syndrome de Reye n’a pas ete etabli en France, la prudence impose neanmoins de respecter les precautions d’emploi figurant dans l’AMM de l’aspirine en pediatrie.

Resume Observation – Nous rapportons le cas d’un enfant de deux mois hospitalise en urgence en etat de choc cardiogenique associe a une hypertension arterielle. L’echographie abdominale mettait en evidence une masse developpee dans la loge surrenalienne gauche. L’examen tomodensitometrique la mesurait a 7 × 6 × 8 cm. Cette masse refoulait le rein gauche et l’aorte. Un examen histologique a fait porter le diagnostic de neuroblastome, sans amplification de l’oncogene N-myc. La prise en charge en reanimation a permis une stabilisation hemodynamique. Une chimiotherapie antitumorale alors realisee a entraine une diminution de la tumeur, et l’a rendue operable. L’enfant restait en remission de sa maladie trois ans apres le diagnostic. Conclusion. – Les catecholamines secretees par la tumeur pourraient provoquer une augmentation de la post-charge du ventricule gauche avec une augmentation du travail myocardique. Ces mecanismes pourraient etre responsables du choc cardiogenique. Ce mode de decouverte d’un neuroblastome est rare.

The natural history of the rare association "multicystic encephalopathy-arthrogryposis" was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks.

Resumen El equilibrio acidobasico del feto depende estrechamente de los intercambios gaseosos transplacentarios. En menor medida y mas tardiamente, el rinon fetal cumple la funcion de modulador. En el periodo postnatal inmediato, los grandes desordenes acidobasicos estan dominados por la acidosis mixta, expresion biologica habitual de las anoxias perinatales mas graves. Las acidosis respiratorias, las acidosis metabolicas de origen lactico y la inmadurez tubular del prematuro son los desordenes acidobasicos hallados cotidianamente en medicina neonatal. Si bien las consecuencias negativas de la acidosis extracelular, en particular miocardica o hemodinamica, se han colocado en primer termino durante mucho tiempo, argumentos experimentales y clinicos recientes proponen modular estos conceptos y ponen el acento en el papel potencialmente protector de la acidosis intracelular en una celula disoxica. Una rutina clinica y bioquimica sencilla pero rigurosa permite, en la gran mayoria de casos, guiar el diagnostico fisiopatologico. Los esquemas terapeuticos han evolucionado notablemente desde hace una quincena de anos. Datos clinicos o teoricos han revelado un cierto numero de efectos deletereos, atribuibles a la utilizacion del bicarbonato sodico (acidosis intracelular denominada «paradojica», por ejemplo), que contribuyen a restringir la utilizacion de las sustancias amortiguadoras unicamente alas acidosis minerales por perdidas digestivas o renales de iones bicarbonato. En el recien nacido, la instauracion conjunta del tratamiento etiologico y del restablecimiento de una hemodinamica y una ventilacion alveolar eficaces (depuracion del dioxido de carbono), recurriendo si es preciso a la ventilacion artificial, constituyen siempre la accion prioritaria.

Resume L'electroencephalogramme (EEG) d'urgence aide a apprecier l'etat de la vigilance aussi bien chez l'enfant que chez l'adulte. Afin de determiner la valeur diagnostique et pronostique de l'EEG d'urgence en cas de confusion mentale, de troubles du comportement ou de troubles de la vigilance chez l'enfant, nous avons fait une etude retrospective chez 397 enfants âges de 2 mois a 16 ans. Les EEG ont ete pratiques moins de 24 heures apres une consultation d'urgence pour confusion ou trouble aigu du comportement (n = 106) ou apres admission pour coma en unite de soins intensifs (n = 291). L'EEG a apporte des indications diagnostiques principalement en cas de pathologie convulsivante ou d'encephalopathie hepatique. Chez les enfants comateux, nous avons etabli une classification en 4 stades EEG de severite croissante. Cette classification a ete comparee aux echelles EEG deja publiees dans la litterature, elle est apparue tres proche de celle de Pampiglione et Harden [18] etablie dans une population de 150 enfants apres arret cardiaque. La valeur pronostique pejorative de l'EEG apparait surtout en cas de trace postanoxique dit ≪burst-suppression≫ ou dans les traces isoelectriques de mort cerebrale. Nous concluons que, en dehors de ces situations, la valeur diagnostique et pronostique d'une classification de l'EEG d'urgence en pediatrie est modulee par une surveillance clinique et EEG repetees.

We report two patients with severe haemophilia A, factor VIII < 2 u dL-1, diagnosed at birth, through isolated bleeding in the adrenal gland (case 1) and through a haematoma in the liver (case 2). In these two clinical cases, the vital emergency, with haemorrhagic shock, required early diagnosis of the hereditary coagulation defect. Generally at birth, any unexplained bleeding should prompt screening for haemophilia. In the newborn period, the PTT is inadequate for many reasons and the laboratory evaluation must include factor VIII and IX levels.

Background. — Agranulocytosis associated with the use of silver sulfadiazine has been described in burn patients. This agranulocytosis may be due to either an allergic reaction or a bone marrow toxicity by silver sulfadiazine. Case report. — A 1-month old baby was hospitalized in pediatric intensive care unit after a stage one repair of a tracheosophegeal fistula. Gastroesophageal reflux and tracheomalacia appeared in the post operative period. When she was 2-months old, she developed a perineal erythema. A topical therapy was begun, with daily applications of silver sulfadiazine and ketoconazole. Five days later, agranulocytosis (granulocytes = 0.21 × 109 L−1) occurred. Silver sulfadiazine therapy was immediately stopped. Granulocyte count returned to normal over a few days. No other etiology than silver sulfadiazine could be found to explain agranulocytosis. The small surface of administration, the chronology of the events and the rapid correction of the disorders after silver sulfadiazine interruption argued for an immuno-allergic reaction to this drug. Medical problems were easily controlled and the patient recovered well. Conclusion. — This event occurred when sulfadiazine was applied in a small cutaneous area in a young baby. This may be an argument for an allergic reaction; alternatively, it could be explained by an increased cutaneous absorption of a cytotoxic drug in younger people.

Among the main causes of acute liver failure (ALF) in children, metabolic diseases (especially in infants), viral and toxin or drug induced hepatitis are the most frequent. The cause remains, however, undetermined in about 30% of the cases. Management must be conducted in a pediatric hepatology unit or intensive care unit in relation with a pediatric transplant team in order: 1) to perform urgent etiological diagnosis; 2) to initiate specific therapy and symptomatic treatment; 3) to evaluate the severity and prognosis of liver disease for selection of children for emergency liver transplantation; 4) to evaluate contraindications to liver transplantation. The overall survival of post-emergency liver transplantation for ALF in children averages 65%.

Le syndrome de detresse respiratoire aigue (SDRA) de l'enfant est un motif d'admission frequent dans les unites de soins intensifs pediatriques. La mortalite de ces 20 dernieres annees demeure elevee en depit des progres de la ventilation mecanique conventionnelle et des traitements etiologiques. Plusieurs etudes chez l'animal ont demontre les effets deleteres d'une ventilation a haut volume, et des travaux cliniques ont clairement montre l'heterogeneite de la ventilation pulmonaire et la surdistension des zones saines (theorie du baby lung) dans le SDRA conduisant a une agression pulmonaire secondaire. La place des supports ventilatoires extracorporels reste neanmoins debattue. D'autres strategies ont ete developpees visant a reduire le traumatisme ventilatoire et a ameliorer l'oxygenation systemique, mais seules des etudes multicentriques et prospectives conforteront le bien-fonde des differentes approches.

Resume Plusieurs mesures ont ete recemment prises pour restructurer les urgences des hopitaux. Elles ont essentiellement concerne les urgences d'adultes. Les pediatres, de leur cote, ont fait plusieurs propositions pour amelioner l'accueil des enfants aux urgences. Ils ont voulu que ces propositions reposent sur des donnees chiffrees. C'est pourquoi, differentes enquetes ont ete realisees ces dernieres annees. Population et methodes. — Les auteurs rapportent les resultats de deux enquetes menees en janvier 1994 et janvier 1995. Il s'agit d'enquetes nationales ⪡ un jour donne ⪢ interrogeant tous les etablissements hospitaliers universitaires et non universitaires de France metropolitaine. Leur but etait de connaitre les modalites d'accueil des enfants aux urgences. Resultats. — Les resultats montrent que l'urgence n'est consideree comme prioritaire que dans 10 % des cas, les autres motifs de consultations etant des urgences ressenties ou de simples consultations. Les urgences medicales et la pathologie accidentelle representent respectivement 60 % et 30 % des motifs de consultation. Plus de la moitie des enfants ont moins de 5 ans. Dans la majorite des CHU, un pediatre en titre est present quelle que soit l'hemmealors que dans les CHG, les pediatres sont en nombre insuffisant, notamment la nuit, pour assurer la continuied d'un accueil specialise. Conclusions. — Ces enquetes, de par leur methodologie, ne donnent qu'une vision instantanee des urgences pediatriques. Elles en montrent neanmoins l'importance quantitative. Elles soulignent par ailleurs la diversite des modes d'accueil des enfants. Elles temoignent des efforts qu'il reste a faire pour que l'enfant soit accucilli par un personnel qualifie dans des locaux adaptes a la pediatrie.

Chez l’enfant comme l’adulte, la classification de Hambourg distingue les peritonites primitives (encore appelees bacteriennes spontanees) et les formes secondaires, qui sont communautaires (PC) ou nosocomiales (PN) mais le plus souvent postoperatoires. Les auteurs anglosaxons identifient par ailleurs les peritonites tertiaires (PT), qui correspondent a des peritonites persistantes ou recidivantes en depit d’un traitement chirurgical et d’une antibiotherapie initiale adequate. Leur evolution est volontiers emaillee d’abces ou de fistules digestives necessitant des reprises iteratives [1–2]. De facon pragmatique, ce sont les peritonites compliquees, communautaires ou nosocomiales, qui continuent de poser aux reanimateurs des problemes de prise en charge et conditionnent le pronostic dans une population d’enfants souvent indemnes de comorbidite.

L'etat de mal epileptique (EDME) est une situation d'urgence tres frequente puisque, tous âges confondus, du nouveau-ne au vieillard, sa frequence est estimee a 25 000 a 30 000 par an en France; la moitie sont des formes convulsivantes; un cinquieme survient chez le nouveau-ne. Sa prise en charge etait, jusqu'a present, tres differente d'une equipe a l'autre. La Societe de reanimation de langue francaise a organise, en juin 1995, une conference de consensus pour proposer une conduite rationnelle. Les membres du jury, en se fondant sur une analyse quasi exhaustive de la litterature disponible et apres avoir ecoute plusieurs experts reconnus sur le sujet, ont suggere une definition pratique de l'EDME et ont propose un protocole de prise en charge. Nous en rapportons les principales conclusions pratiques.

Diaphragmatic paralysis, a difficult diagnosis in the pediatric age group, has classically been made by fluoroscopy or B-mode ultrasound. We report our experience with TM-mode exploration. Twenty-seven patients suspected to have diaphragmatic paralysis were examined by means of inspiratory and expiratory chest radiography, fluoroscopy and B-mode ultrasound. The diaphragmatic echo was recorded on TM-tracing during spontaneous breathing using coronal oblique scans. Direction, excursion and the pattern of the transition between inspiration and expiration were analysed. In 7 patients examination was normal and TM mode demonstrated movement of normal direction and excursion with a sharp aspect of the transition zone. Diaphragmatic paralysis was present in 11 patients: unilateral in 9 and bilateral in 2 cases. TM mode demonstrated paradoxical movement, reduced excursion and a smooth transition zone. In 9 patients with diaphragmatic dysfunction TM mode demonstrated movement in the normal direction but with reduced excursion and a smooth transition zone. Compared to other imaging modalities, TM-mode records diaphragmatic movements more objectively. It can identify direction of the movement even if they are fast and of weak amplitude and in the case of bilateral paralysis. TM can differentiate paralysis from dysfunction. Moreover, this low-cost, non-irradiating made of imaging can be performed at the bedside and is available on all basic devices.

Resume Une enquete menee sur 2 annees au sein du Groupe Francophone de Reanimation Pediatrique a permis de recenser 459 cas de bronchiolites graves hospitalisees en reanimation :2/3 de ces enfants ont moins de 3 mois et 50 % des nourrrissons admis ont au moins un des classiques facteurs de risque (antecedents de prematurite, de bronchodysplasie, cardiopathie congenitale, immunodepression). Plus de 60 % de ces malades requierent une ventilation mecanique d'une duree moyenne de 8 jours. Les complications classiques (barotraumatisme, SIADH et convulsions) touchent moins de 5 % des enfants. Le taux de mortalite globale est de 2,2 %.et il n'y a pas de surmortalite dans les groupes a risque. L'utilisation de la ribavirine a nettement augmente d'une annee a l'autre. L'evaluation de son efficacite sera delicate en raison de la difficulte a trouver des criteres d'evaluation.

Les determinants de la decision medicale sont multiples. Certains sont cliniques et bases sur l’analyse medico-scientifique de la situation, analyse qui est de nature essentiellement probabiliste. Il s’agit de prendre en compte l’incertitude du diagnostic, des effets du traitement et du pronostic par exemple. Mais la decision medicale ne saurait se resumer a une analyse medicoscientifique d’une situation. D’autres facteurs plus contingents interviennent : ils sont d’ordre psychologique, inconscient, social, economique, religieux, culturel par exemple. Tous ces determinants sont relies entre eux par le contexte tres particulier de la reanimation qui confronte directement les acteurs de la decision aux mysteres de la vie et de la mort. On saisit alors tout le tragique de cette situation et l’importance de l’analyse des determinants, scientifiques ou non, qui ont conduit a prendre telle ou telle decision. On comprend egalement la necessite d’encadrer la decision medicale par les recomman dations des societes savantes, par la societe ou encore par le legislateur tant les enjeux sont d’importance.

We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.

A paediatric intensive care unit is a complex environment with many hazards for potential medical errors. Due to the high acuity of illness and the complexity of medical care with multiple disciplines and individuals involved, medical errors occur due to breakdowns in communication and teamwork. Medication errors are the most frequent errors in paediatrics. This chapter proposes some strategies to prevent medical errors in paediatric intensive care units.

Resume Les insuffisances hepato-cellulaires aigues graves (IHCAG) de l'enfant entrainent 60 a 90% de mortalite, suivant les etudes, sans influence de l'âge. Vingt-deux enfants atteints de cette pathologie ont eu des EEG quotidiens; 15 sont decedes, sept ont survecu. L'etude neurologique electroclinique a mis en evidence une evolution stereotypee des anomalies EEG sans rapport avec l'âge, correlees aux stades cliniques de coma et a des troubles severes de la coagulation; 5/22 enfants ayant eu des manifestations convulsives cliniques et/ou EEG sont morts. Chez un seul enfant decede d'intoxication par le paracetamol, on a observe des ondes lentes triphasiques. Une etude prospective sur une population plus importante permettrait de reconnaitre des facteurs de decision pour une transplantation hepatique.

Among a series of 90 pediatric liver transplantations, 9 cases of hepatic artery thrombosis (HAT) in 8 patients are reported. All cases were diagnosed in the first two weeks and confirmed angiographically and surgically. Clinical presentation was often unreliable with only 2 cases showing the typical pattern of massive hepatic necrosis. Five cases presented with biliary complications and in 2 cases, HAT was discovered fortuitously on duplex sonography. The role of imaging modalities is emphasized. Duplex sonography is the best non-invasive screening method and we recommend a routine daily examination in the first 2 weeks. CT is the most useful method to assess the extension of liver infarction. PTC remains necessary to evaluate biliary complications and to plant the best therapeutic approach in this devastating event. Two patients died, 2 had to be retransplanted (one patient twice) and are doing well, 2 are on a waiting list for re-transplantation and one underwent an emergency surgical arterial desobstruction and is completely asymptomatic.

Usefulness of nasal continuous positive airway pressure (NCPAP) in severe acute bronchiolitis has been checked. The objective of this descriptive study was to evaluate the feasibility, safety and risk factors of NCPAP failure.One hundred and forty-five infants were hospitalised in our intensive care unit during the 2 last epidemics (2003-2004, 2004-2005). Among them, 121 needed a respiratory support, either invasive ventilation (N=68) or NCPAP (N=53).General characteristics were similar during the 2 periods. Percentage of NCPAP failure, defined by tracheal intubation requirement during the stay in paediatric intensive care unit, was quite similar during the 2 periods (25%), but number of NCPAP increased twofold. Whatever the evolution was in the NCPAP group, we observed a significant decrease in respiratory rate (60+/-16 vs 47.5+/-13.7 cycle/min., P0.001) and PaCO2 (64.3+/-13.8 vs 52.6+/-11.7 mmHg, P=0.001) during NCPAP. Only PRISM calculated at day 1 and initial reduction of PaCO2 were predictive of NCPAP failure. Percentage of ventilator associated pneumonia was similar (22%) between the invasive ventilation group and infants who where intubated because of failure of NCPAP. Duration of respiratory support and stay were reduced in the NCPAP group (P0.002).NCPAP appears to be a safe alternative to immediate intubation in infants with severe bronchiolitis.

Viral bronchiolitis is usually associated with favorable outcome as regard to mortality. Only few studies reported severe bronchiolitis requiring mechanical ventilation, and respiratory outcome is not well described.Therefore, we conducted a retrospective study in a series of 135 children admitted in a single Pediatric Intensive Care Unit (PICU) over a four year period (1994-1998). All of them were admitted for viral bronchiolitis requiring mechanical ventilation.At admission, 83% of them were less than three months old. Prematurity at birth was present in 33,3%. Mortality was observed in four cases (2,9%), all premature babies with mechanical ventilation at birth. Univariate analysis showed as main factors associated to mortality: prematurity (P =0,056) and acute respiratory distress syndrome (P =0,017). Childhood asthma was observed in 40,4% of children without any associated factor wether at birth or in PICU related to such outcome.Bronchiolitis associated with mechanical ventilation is particularly observed in very young babies and prematurity is the main factor associated to mortality. Mechanical ventilation seems not to be associated with unfavorable respiratory outcome. Considering physiology and population, non invasive ventilation could be an effective alternative of mechanical ventilation.

A two-month-old male child presented a severe heart failure associated with a malignant hypertension. Abdominal ultrasound revealed a mass connected to the left adrenal gland. CT scan showed a tumor of 7 x 6 x 8 cm, forcing back both the left kidney and the aorta. A biopsy allowed the diagnosis of neuroblastoma without MYCN oncogene amplification. Intensive care stabilized the hemodynamic situation. Under chemotherapy the tumor volume decreased significantly and complete surgical excision became possible. Three years after diagnosis, the patient remained in complete remission.Clinical presentation of this neuroblastoma was extremely uncommon. The catecholamines produced by the tumoral cells could induce an increase of the myocardiac work following the left ventricule post charge increase. Theses mechanisms could be synergistic for a myocardial exhaustion.

Idiopathic Reye syndrome is a rare disease revealed by unexplained encephalopathy and microvesicular liver steatosis. Some clinical and epidemiological studies mainly performed in English speaking countries questioned the reality of Reye syndrome because numerous know inherited metabolic diseases, and some of them unrecognized, could mimick this disorder. We focused in our study on severe forms of Reye syndrome admitted to a pediatric intensive care unit.Retrospective study over the last eleven years (1991-2001) included all the pediatric patients admitted to our tertiary referral center with the classical American Reye syndrome criteria (e.g. CDC). Extensive metabolic screening was performed in all cases, except for the ultimately dead patients.Fourteen patients (mean age 52 months) were included. Fever always occurred before their admission and aspirin (n = 12) or acetaminophen (n = 7) was prescribed. Median Glasgow scale was 7 on admission. Mean amoniac plasma level was 320 mumol/L and alanine-aminotransferase peak plasma level 1475 +/- 1387 IU/L. Mechanical ventilation was started in ten children and six of them underwent continuous venovenous hemofiltration. Three patients ultimately died and 11 survived with a mean five years follow-up without relapses or neurological impairment. Any of them demonstrated inherited metabolic disease except for one infant with hereditary fructose intolerance.Unlike widespread opinion, severe Reye syndrome without identified metabolic disorders seems to not disappear in our country. Reye syndrome remains a potentially life threatening disease and raises for aggressive treatment of brain edema. If aspirin and Reye syndrome association are not formally documented in France, cautiousness must be kept in mind and all the aspirin adverse effects notifications should be addressed to the public drugs survey network.

Liver involvement in mitochondrial respiratory chain disorders (MRCD) frequently ends in liver failure and death. Because of the high risk of extrahepatic, particularly neuromuscular, manifestations of the disease, the indication of orthotopic liver transplantation (OLT) in these patients remains controversial. We report on 5 such children in whom OLT was carried out, in an attempt to help clarify the matter.Patients 1 and 2 presented with fulminant liver failure at ages 7 and 6 months respectively. Emergency liver transplantation was performed before etiological investigations were completed. Retrospective examination of the explanted livers showed defects in complexes I, III and IV. In patient 1, severe neurological deterioration occurred 2 months after OLT with fatal outcome 9 months later. Patient 2 is alive 22 months after OLT with moderate motor impairment. Patients 3, 4 and 5 presented with progressive liver failure before 6 months of age. Surgical liver biopsies displayed a 50% defect in complex IV (patient 3), a defect in complexes I, IV (patient 4) and in complexes I, III, IV (patient 5). Because there was no clinical extrahepatic involvement on investigations, OLT was carried out in these patients. Patient 3 died of multiple organ failure soon after OLT, patients 4 and 5 are alive respectively 21 months and 12 months after OLT with normal neurological examination.OLT may be a valid therapeutic option in infants with delayed liver cell failure due to MRCD, only after performing in emergency a thorough inves tigation to exclude clinically significant extrahepatic, especially neuromuscular, involvement.

A diagnosis of pulmonary embolism is uncommon in the infant and the child, and chronic cor pulmonale secondary to pulmonary embolism is an even rarer occurrence.In this study, a case of pulmonary embolism in a 6-month-old male infant has been reported. His past history included preterm birth, and severe bronchopulmonary dysplasia, with prolonged oxygen dependency. The positive diagnosis was based on cardiac ultrasound examination, with the direct imaging of a right pulmonary arterial thrombus. Surgical thromboendarterectomy was performed, with a long-term favorable outcome.After excluding from the diagnosis those hemostatic disorders known to be thrombogenic, the most likely hypothesis was retained, i.e., that it was catheter-related. A central venous catheter had been inserted during the neonatal period, and was probably responsible for the embolism. The clinical characteristics and the diagnostic and therapeutic aspects of chronic postembolic cor pulmonale have been discussed in the light of the present findings.

To compare literature data with results obtained with organs procured from donors who died from cardiac arrest and to make proposals for this mode of organ procurement in France.Over the last 10 years, 10 organ donors (2%) among a series of 486 donors in a state of brain death, had died of cardiac arrest. The arrest were perfused with double-balloon catheters. The outcome of the subsequent kidney grafts was compared with data in the literature.Fifteen of the 18 kidneys from cardiac arrest donors were functioning 1 month after implantation compared with 17 of the 20 kidneys from braindeath donors with beating hearts. The rate of acute tubular necrosis was 55% in the cardiac arrest kidneys and 40% in the beating-heart kidneys. Serum creatinine at 1 yeart was 145 +/- 69 mumol/l 17 +/- 29 mumol/l respectively.These results and those reported in the literature demonstrate that kidney procurement from cardiac arrest donors is feasible. If intensive care and surgery units are well organized, this type of organ procurement could provide a larger number of organs for transplantation. Emergency teams must be available for preparing and transferring the organs.

Recording of electroencephalogram (EEG) is of value to estimate vigilance states in children as in adults. In order to determine the diagnostic and prognostic value of emergency EEG in case of mental confusion, behavioral disorders and vigilance disorders in childhood, we conducted a retrospective study in 397 children (aged 2 months to 16 years). EEG was recorded less than 24 hours after an emergency consultation for acute confusion or acute behavioral disorder (n = 106) or after admission to the intensive care unit for comatose stage (n = 291). EEG gave diagnostic indications mainly in case of convulsive pathology or hepatic encephalopathy. In comatose children, we established a 4-stage EEG scale of increasing severity. This classification was compared to EEG scales already published in the literature and appeared very similar to that from Pampiglione and Harden, established in 150 children after cardiac arrest. A highly poor prognostic value was associated with burst-suppression post-anoxic patterns and with isoelectric records signaling brain death. Our classification of emergency EEG patterns is mainly helpful in these two situations, but does not exclude strict and repeated clinical and EEG follow-up in other cases, as a relatively preserved initial EEG may later deteriorate.

ORGAN procurement network needs the implication of transplant team outside of the transplant center. A transplant surgery team seems most appropriate for organ procurement because of their training in the field of organ procurement and transplantation. The 5-year kidney graft survival rate decreases to 8% when a nontransplant-surgeon makes the organ procurement. Moreover, the rate of acute tubular necrosis increases to 54% (vs 35%). The outcome of renal transplantation depends on surgical training of the organ procurement team. The purpose of this retrospective work was to compare the results of organ procurements made by our team (inside and outside our center) to those by other organ procurement teams.