Conséquence d’un dysfonctionnement métabolique, la stéatose hépatique ou NAFLD (Non-Alcoholic Fatty Liver Disease) peut être présente au cours de nombreuses maladies nutritionnelles ou métaboliques, au premier rang desquelles l’obésité. La stéatose peut être diagnostiquée dans le cadre d’un bilan ou du suivi d’une pathologie prédisposante ou de découverte fortuite sur une échographie abdominale. L’obésité est la cause principale de stéatose (NAFLD), retrouvée chez environ 80 % des enfants ayant une stéatose hépatique, notamment après l’âge de 10 ans. Une composante inflammatoire (NASH) peut s’accompagner d’une fibrose mais le risque d’évolution vers une cirrhose est faible chez l’enfant. Toutefois, du fait de l’augmentation croissante de la prévalence de l’obésité, ce risque ne doit pas être négligé et un diagnostic précoce est indispensable afin d’instaurer une prise en charge multidisciplinaire le plus tôt possible. Par ailleurs, il faut savoir évoquer d’autres causes de stéatose hépatique, nutritionnelles ou métaboliques, en particulier chez le jeune enfant.Due to a metabolic dysfunction, hepatic steatosis or NAFLD (Non-Alcoholic Fatty Liver Disease) may be present in numerous nutritional or metabolic diseases, obesity being the main one. Steatosis may be diagnosed by a medical work-up or follow-up of a predisposing disease, or it may be discovered incidentally during an ultrasound examination of the liver. Obesity is the main cause of NAFLD, which is present in 80% of these children, mostly after 10 years of age. The presence of inflammation (NASH) may lead to fibrosis, although cirrhosis remains very rare in childhood. Nevertheless, this risk must not be neglected in view of the increasing incidence of obesity, and early multidisciplinary care is mandatory. In young children, other causes of fatty liver must be ruled out.

Abstract Objectives and study Anastomotic stricture (AS) is a frequent complication of the surgery for œsophageal atresia (OA) during the first year of life. The primary objective of this study was to evaluate the prevalence of AS before 1 year old in infants with type A and C OA who were operated on. Secondary objectives were to determine risk factors for AS in OA, for recurrent and refractory AS, and to establish if AS is associated with antireflux surgery. Methods A prospective national multicentric study was conducted including all infants born with OA between 2008 and 2015. Patients deceased before one year old, OA types B and E, and patients for whom data about AS were missing were excluded from the study. Data were collected at birth and at 12 months of age. Anastomosis under tension was defined by the surgeon and a delayed anastomosis was defined by an anastomosis after 15 days of life. Recurrent stricture was defined by the need of ≥3 dilations and refractory stricture was defined by the need of ≥5 dilations. Univariate and multivariate statistical analyses were conducted. Results Of the 1258 eligible patients (84%), 1054 were included in the study from 38 centers. The prevalence of AS in the first year of life was 23.3% [20.7–28.9]. Anastomosis under tension (AUT) and delayed anastomosis (DA) were found to be independent risk factors for AS (respectively 2.5 [1.73–3.45] and 3.7 [1.95–7.2] (OR [CL 95%])) in the total population. Neither sex, birth weight, prematurity, intrauterine growth retardation, associated malformations, type A OA, nor the type of surgical approach was a risk factor for AS. In type C OA, DA was the only risk factor for AS (OR: 3.1 [1.65–5.86]). The group with AS had 2.5-fold more fundoplication compared to the patients without AS (P = 0.0005) in the total population and in type C OA. AUT and DA were found to be independent risk factors for recurrent stricture (OR: 2.4 [1.47–3.9] and 4.7 [2.2–10.4], respectively) and DA was the only risk factor for refractory stricture (OR: 6.23 [2.4–16.2]). Conclusion Surgical factors at the time of first repair of OA are the only risk factors for AS.

Summary Respiratory diseases are common in children with esophageal atresia (EA), leading to an increased morbidity and mortality in the first months of life. Objective Assess the prevalence of hospitalizations linked to a respiratory disease and of maintenance inhaled therapy at the age of 1 year in French children. Methods Population based-study using data from the French national EA register. We included all children born between 2010 and 2015 with data available at birth and at follow-up at one year of age. Results A total of 981 patients born with EA were included in the register, 75 of them (8%) being deceased at the age of 1 year. Data were missing for 60 patients, thus 846 children (86%) were retained for analysis. EAs were type III of Ladd classification in 89% and type I in 7%. Rate of prematurity was 37% while 51% presented associated malformations. At 1 year of age, 1297 hospitalizations were reported for 508 patients (60%), at least one hospitalization for a respiratory disease for 251 children (51%). Factors significantly associated with respiratory hospitalizations were longer median length of oxygen supplementation (P < 10−2) and noninvasive ventilation in the neonatal period (P = 0.02), gastrostomy tube (P < 10−2), esophageal anastomosis dilation (P < 10−2). At 1 year of age, 29% had an inhaled maintenance treatment, and 85% inhaled corticosteroids. Factors significantly associated with inhaled maintenance treatment were male gender (P = 0.04), advanced older median age at gastrostomy tube insertion (P < 10−3), enteral feeding at one year of age (P < 10−3). Conclusion This study shows a high rate of respiratory problems responsive of frequent hospitalizations and inhaled maintenance treatment in children born with EA in the first year of life.

Background Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM). Objective The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management. Study design The authors retrospectively reviewed cases of S-PHA related to UTM observed at the institution and searched the PubMed (R) database to review the literature. Results A total of 116 cases of S-PHA associated with UTM, including the four cases from the institution, were reviewed. One hundred six cases (92.2%) were younger than 6 months, and 95 cases (81.9%) occurred in boys. Urinary tract infection was associated in 105 cases (90.5%). All types of UTM were observed. In the absence of urinary tract infection, S-PHA was related to bilateral UTM or solitary kidney. In 89 cases (76.5%), S-PHA resolved with medical treatment only. In cases of UTM requiring immediate surgery, electrolyte imbalance related to S-PHA also resolved after surgery. Children with associated urinary tract infection and bilateral UTM are at higher risk of developing S-PHA. Discussion The pathogenesis of S-PHA has not been fully elucidated. Renal tubular immaturity may be one of the factors involved, in view of the young age of the population being affected. A high rate of bilateral UTM (or UTM on solitary kidney) was observed (50.9%), suggesting an association with S-PHA. In the absence of urinary tract infection (UTI), S-PHA appeared to occur more frequently in the presence of bilateral UTM. Although the indication for early surgery remains unclear, it may have a role in the prevention of UTI and prevention of recurrence of S-PHA. Serum electrolytes should be checked in children with UTM before urological surgery, and/or presenting urinary tract infection, before the age of 6 months. The results of this study must be interpreted cautiously because of its retrospective nature and the fact that data were derived from various articles. Few articles on S-PHA related to UTM have been published in the literature. To the best of the authors' knowledge, the study constitutes the largest series published to date. Conclusions S-PHA results in potentially severe electrolyte imbalance and affects children younger than 6 months with UTI and/or UTM. Electrolyte abnormalities related to S-PHA often resolve after administration of appropriate intravenous electrolyte solution and treatment of UTI and/or surgery. [GRAPHICS] .

Abstract Objectives and Study The aim of our study is to assess the risk factor of mortality and morbidity in a large population-based registry in a population of type III/C esophageal atresia (EA). Methods Under the umbrella of the national plan for rare disease, a population based register was set up in 2008 recording the data of all the live newborns with EA in France. Based on the registry data, survival and morbidity at 1 year were studied. Morbidity was approached by calculating the rate of full oral autonomy and the hospital length of stay during the first year. Multivariate analysis was performed via multinomial logistic regression to evaluate independent predictors of overall survival and morbidity items among all significant category variables identified on univariate analysis. Results A total of 1008 patients with type III EA were extracted from the national database born from January first 2008 till 31 December 2014. Mean birth weight was 2610 g (2060 to 3075). Right lateral thoracotomy was used in 93% of cases and primary anastomosis was possible in 95% of cases. Associated abnormalities were present in 53% of patients, VACTERL in 19%, and CHARGE syndrome in 3%. Mortality at 3 months was at 5% and at one year at 6% and was correlated to prenatal diagnosis (odd ratio (OR):2.96 (1.08 to 8.08)), low birth weight (OR: 0.52 (0.38 to 0.72)), and heart defect (OR: 6.09 (1.96 to 18.89)). Length of hospitalization was correlated to birth weight (OR: 0.83 (0.61–1.13)), the difficulty of the anastomosis (OR: 1.59 (0.71–3.55)) and associated abnormalities (OR: 1.93(0.65–5.68)), the prenatal diagnosis is correlated only to the rate of full oral autonomy and not with the length of hospitalization. Conclusions Surgical procedure or difficulties did not seem to affect survival in this group of patients. In addition to the continuous need to improve care of low weight neonates, our results clearly identify that efforts should be focused on the small group of EA with prenatal diagnosis and/or severe cardiac malformation to reduce even more mortality and morbidity in EA patients.

La bride mésentérique congénitale est une cause rare d’occlusion digestive. Nous décrivons un cas d’occlusion digestive haute sur une bride congénitale jéjunale chez un enfant âgé de 2 ans. La difficulté est d’évoquer le diagnostic chez un enfant sans aucun antécédent d’intervention chirurgicale.

Cystic lymphangiomas are usually located in the neck region. Less frequently, they can be found in the abdomen. In those cases, pre- and neonatal diagnosis is extremely difficult. We report on the case of a giant mesocolic cystic lymphangioma, diagnosed at birth, in a child who had been monitored during the prenatal period for what was believed to be a digestive dilatation. The progression was marked by excellent tolerance despite a complete lack of regression in the first 10 months of life. The authors discuss the prenatal signs that should suggest this diagnosis and an MRI, as well as management during the 1st year of life.

Isolated gallbladder agenesis is a very rare and unrecognized congenital anomaly. Patients are usually asymptomatic, but 23% present with symptoms suggestive of biliary colic. Ultrasound investigation often fails to diagnose this malformation, misinterpreted as scleroatrophic gallbladder, leading to unnecessary and potentially dangerous surgery. We report on a case of a 9-year-old child who complained of biliary colic. Ultrasound showed a possible scleroatrophic gallbladder. This diagnosis was in doubt, however, because the patient had no previous history of cholecystitis. Finally, magnetic resonance cholangiopancreatography failed to show any gallbladder. The absence of the visualization of the gallbladder in a context of right upper quadrant pain should suggest gallbladder agenesis. Pain can be explained by the so-called postcholecystectomy syndrome. (C) 2017 Elsevier Masson SAS. All rights reserved.

A congenital mesenteric band is a rare cause of intestinal obstruction. We describe a case of upper gastrointestinal obstruction on a jejunal congenital band in a 2-year-old child. The challenge is to make the diagnosis in a patient with no history of previous surgery.

Inflammatory myofibroblastic tumors (IMT) are rare benign tumors, most commonly arising in the lungs and urinary bladder. Many etiologic factors are suspected in their development, but none have been formally demonstrated. Conventional treatment for bladder IMT is complete surgical resection by partial cystectomy or transurethral resection. We report the case of an 8-year-old girl with documented bladder IMT that resolved completely after antibiotic therapy. (C) 2016 Elsevier Masson SAS. All rights reserved.

Les tumeurs myofibroblastiques inflammatoires (TMI) de vessie sont des tumeurs rares et bénignes de l’enfant. De nombreux facteurs étiologiques ont été avancés sans qu’aucun ne puisse être formellement identifié. La résection chirurgicale complète fait référence mais une surveillance ou une corticothérapie peuvent être proposées. Nous rapportons le cas d’une fillette de 8 ans avec une authentique TMI de vessie ayant régressé complètement après antibiothérapie.

We report herein the synthesis and biological evaluation of a new series of 2,4,6-trisubstituted 1,3,5-triazines as reversible inhibitors of human cysteine cathepsins. The desired products bearing morpholine and N-Boc piperidine, respectively, were obtained in three to four steps from commercially available trichlorotriazine. Seventeen hitherto unknown compounds were evaluated in vitro against various cathepsins for their inhibitory properties. Among them, compound 7c (4-(morpholin-4-yl)-6-[4-(trifluoromethoxy)anilino]-1,3,5-triazine-2-carbonitrile) was identified as the most potent and selective inhibitor of cathepsin S (Ki = 2 ± 0.3 nM). Also 7c impaired the autocatalytic maturation of procathepsin S. Molecular docking studies support that 7c bound within the active site of cathepsin S, by interacting with Gly23, Cys25 and Trp26 (S1 subsite), with Asn67, Gly69 and Phe70 (S2 subsite) and with Gln19 (S1′ pocket).

The latest developments in the domain of HPC have lead to the deployment of complex extreme-scale systems, based on diverse computing devices (CPU, GPU, accelerators) thus posing the question of scalability in the light not only of parallel efficiency, but also in terms of energy efficiency. In this paper we propose a new metrics for energy aware performance estimation based on our experience and the analysis of the existing metrics. We study the performance of computationally intensive Monte Carlo applications deployed on heterogeneous HPC systems with focus on energy efficiency and equipment costs. We compare the energy aware performance results of CPU and GPU variants of the tested algorithms with respect to the introduced measures and metrics. The results of our study demonstrate the importance of taking into account not only scalability of the HPC applications but also energy efficiency and equipment cost. They also show how to optimize the selection of CPU computing or computing with GPGPUs. The results can be used by application developers/users and also by resource providers.

La fréquence de l’hypospade est en forte augmentation depuis les vingt dernières années. L’objectif de notre étude est de déterminer les facteurs de risque d’hypospadias. Cette étude prospective multicentrique et randomisée a été réalisée de mars 2011 à décembre 2013 dans 9 maternités picardes. Les données ont été recueillies par interrogatoire et examen clinique à la naissance. Cinquante hypospades appariés à 3 témoins ont été inclus. Le terme des patients inclus était comparable (39 SA). Aucune différence n’a été observée entre les 2 groupes concernant: l’âge des parents, le délai de grossesse, la prise de poids et la consommation de tabac maternelles durant la grossesse, les fréquences des grossesses sous contraceptif, des antécédents maternels endocriniens et des fausses couches. Par rapport au groupe témoin, le groupe hypospade présentait un poids et une taille de naissance significativement inférieurs (3094g±586 vs 3297±421 g, p<0.05) (48,3±2,8 vs 49,4±4,9, p<0,05). Le taux d’hypospade était augmenté en cas de 1ère grossesse (OR(IC95%)= 2.1(1.1–4.4), p<0,05) et de poids normal de la mère. L’association entre RCIU, primiparité et hypospade fait évoquer le rôle d’une dysfonction placentaire dans la survenue de cette malformation.

Le laparoschisis est une malformation congénitale caractérisée par des troubles de la motricité intestinale sévères imposant le repos digestif prolongé. L'objectif est d'étudier l'impact du type anatomique (TA) de laparoschisis sur le chois de fermeture chirurgicale, la durée de nutrition parentérale exclusive (NPE), le délai d'alimentation entérale totale (AET), la durée de séjour en réanimation (DSR) et la durée d'hospitalisation totale (DHT). Cette étude rétrospective concerne 30 cas de laparoschisis hospitalisés en réanimation néonatale au CHU d'Amiens entre 2001 et 2011. Nous avons corrélé le choix de fermeture chirurgicale à l'aspect anatomoclinique de laparoschisis selon la classification de Lefort. Le terme de naissance des 30 enfants (15 nés par voie basse, 15 par césarienne) est de 35,7 semaines d'aménorhées (SA) (extrêmes : 32,9–38), et le poids de 2 250 g (extrême : 1300–3510). On distingue 13 types I, 13 types II et 4 types III. Il a été réalisé 17 fermetures primaires par l'orifice initial, 8 fer-meteures après laparotomie, 4 Schuster, une pose de plaque de Goretex (pression veineuse élevée lors de la fermeture) et 4 entéros-tomies (atrésie intestinale associée). La durée de NPE (3 à 33 jours) et le délai d'AET (14 à 135 jours) sont indépendants du TA. Les DSR et DHT pour les types I, II et III sont respectivement de 6 ; 10 ; 23,5 jours (p = 0,03) et de 49 ; 69 ; 78 jours (p = 0,05). Deux enfants du type II sont décédés d'une enté-rocolite à 3 mois de vie. 2/3 des enfants ont présenté une cholestase. Le TA de laparoschisis n'a pas d'impact, ni sur le choix de la technique chirurgicale, ni sur la durée de NPE ou le délai d'AET. La DSR et la DHT sont prolongées en cas de type III. La fermeture primaire quand elle est possible, reste la technique de choix pour la fermeture de laparoschisis.

Neonatal compartment syndrome is rare, and the diagnosis is often missed or delayed because other ischemic diseases can mimic clinical signs observed on the skin. A premature newborn infant presented with skin lesions during the first hours of life that were recognized as the sentinel finding in compartment syndrome of the newborn. We restored normal function by emergency surgery. The authors highlight the importance of effective collaboration between pediatricians and surgeons to improve the management of this neonatal condition.