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High-resolution observations have demonstrated the presence of strong time-mean near-surface wind convergences (NSWC) anchored across oceanic frontal zones, such as the western boundary currents. Initial analyses appeared to show a close association between this time-mean NSWC and time-mean properties of the underlying sea-surface temperature (SST), such as the gradients and second derivatives (e.g., Laplacian of SST), acting through pressure adjustment and vertical mixing mechanisms. However, a series of recent papers have revealed the instantaneous NSWC to be dominated by atmospheric fronts, and suggested the importance of air-sea processes occurring instead on shorter, synoptic timescales. In this paper, using the ERA-5 reanalysis dataset in the Gulf Stream region, we aim to reconcile these viewpoints by investigating the spatial and temporal dependence of NSWC and its relationship to SST. It is revealed that while atmospheric frontal processes govern the day-to-day variability of NSWC, the relatively weak but persistent pressure adjustment and vertical mixing mechanisms provide lower frequency modulations in conditions both with and without atmospheric fronts. In addition to their temporal characteristics, each mechanism is shown through spectral analysis to dominate on specific spatial scales. In light of recent work that has tied remote atmospheric responses to NSWC anomalies in western boundary current regions, these results emphasize the importance of oceanic frontal zones for atmospheric variability on all spatiotemporal scales.

With the increasing life expectancy for patients with esophageal atresia (EA), and a known predisposition to certain cancers, cumulative radiation exposure from imaging is of increasing significance. This study describes the cumulative effective radiation dose (CED), during the first 6 months of life, estimated from radiologic procedures when dose reduction methods are used. Medical records of newborns with EA followed in our pediatric hospital in 2019–2020 were reviewed for demographics, EA subtype and hospitalization length of stay. Number and type of imaging studies, and radiation exposure were recorded in order to estimate CED in mSv. Twenty-one children were included. Each child underwent a median of 16 (IQR 13–39) imaging studies, which were primarily chest radiography (median of 12 (IQR 10–35)). The overall median CED per patient was 0.4 mSv (IQR 0.08–1.28), with the majority of radiation exposure resulting from plain radiography (median of 0.3 mSv (0.08–0.52)). Careful consideration should be given to the use of imaging in EA to minimize morbidity in these vulnerable infants. A low dose of radiation can be achieved by following these recommendations: proscription of systematic superfluous examinations, standardization and optimization of the imaging protocols, dosimetry monitoring and proper training of operators.

Objective: Bowel obstructions beyond the duodenum represent a heterogeneous group of congenital anomalies with a highly variable prognosis, the main issue being postnatal short bowel syndrome (SBS). The objective of our study was to evaluate the contributions of fetal MRI in cases of bowel obstruction. Materials and Methods: A retrospective analysis of all newborns, for whom both ante-natal ultrasound and fetal MRI were available, referred to our center for suspected bowel obstruction was performed. Examinations were reviewed blinded to the postnatal outcome. Key outcome measures included exact diagnosis and the existence of postoperative SBS. We evaluated the contribution of MRI in determining precise location and etiology of the bowel obstruction, dilatation of the proximal bowel loops, and assessment of the quality of the remaining distal bowel loops. Results: Twenty-five newborns were included. There were 19 single obstructions and 6 complex forms (4 apple peel syndromes and 2 multiple atresias). MRI correctly identified the affected segment of the small bowel in 59.1% of the cases. MRI identified the mechanism of obstruction in 72% of cases. MRI reliably predicted an abnormal appearance of the bowel distal to the obstruction in 100% of the severe cases (3/3) and in 66.7% of complex forms (4/6). Conclusion: Our study suggests that fetal MRI, when done in addition to prenatal ultrasound, is contributory in the management of fetuses with suspected bowel obstruction. MRI may be particularly useful in determining the location and origin of the bowel obstruction and in assessing the quality of the bowel distal to the obstruction, which might assist in the prediction of SBS and more detailed prenatal counseling.

Resume Objectif Le pronostic des nouveau-nes porteurs d’une omphalocele depend de nombreux facteurs, en particulier de l’existence d’anomalies associees. Les syndromes de Wiedemann–Beckwith sont reputes presenter de petites omphaloceles. Cependant, aucun critere echographique ne permet de predire les autres anomalies associees. L’objectif de ce travail etait donc de decrire les issues globales des omphaloceles de diagnostic prenatal, et de rechercher une correlation eventuelle entre le contenu de l’omphalocele precocement evaluee et les anomalies associees constatees en postnatal. Methode Etude retrospective realisee a l’hopital Necker-Enfants Malades entre 2008 et 2018. Les issues de grossesses et le diagnostic post natal ont ete recueillis, et analyses en fonction du contenu de l’omphalocele au premier trimestre. Resultats Cent quatre-vingt-onze femmes avec diagnostic antenatal d’omphalocele ont ete incluses. Vingt-huit pour cent des cas etaient isoles a la naissance, 32 % presentaient un syndrome polymalformatif associe a une anomalie chromosomique, 13 % de syndrome polymalformatif sans cause genetique retrouvee, 9 % de syndrome de Wiedemann Beckwith, 7 % d’association a une cardiopathie, 6 % de sequence du cordon court, 3 % de sequence OEIS et une pentalogie de Cantrell. La presence du foie au 1er trimestre a ete un facteur predictif de cardiopathie (85,7 % vs 48,6 % p = 0,01). La presence des anses digestives au premier trimestre a ete un facteur predictif d’anomalies chromosomiques (69,6 % vs 37,2 % p Conclusion L’analyse en echographie au premier trimestre du contenu exhaustif de l’omphalocele est une aide precieuse pour l’evaluation du risque d’anomalies associees et donc le conseil prenatal.

To interpret in vivo experiments designed to understand brain function, high-resolution whole-brain microscopy provides a means for post hoc determination of the location of implanted devices and recorded cells in three dimensional brain space that is a critical step for data interrogation. Here we have developed Python-based tools (brainreg and brainreg-segment) to accurately map, in a common coordinate space, the position of dye-labelled probe tracks and two-photon imaged cell populations expressing fluorescent protein. The precise location of probes and cells were validated using physiological recordings and human raters that indicate accuracy levels to less than 70µm. These flexible, open-source methodologies are expected to further evolve with need and to deliver the anatomical precision that is necessary for understanding the functional architecture of the brain.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P

Background/Objective: Thoracoscopic repair of esophageal atresia (EA) is gaining popularity, but it is a highly technically demanding procedure. The aim of our study is to evaluate our outcomes in ...

To identify prenatal and neonatal predictors of short bowel syndrome-related intestinal failure (SBS-IF) in gastroschisis.This retrospective study included all patients with gastroschisis born between 2000 and 2017 who were enrolled in our home parenteral nutrition program, and all patients with gastroschisis born in our institution who survived 2 weeks, during the same time period. Prenatal ultrasound features, neonatal status, anatomic features, oral feeding, and parenteral nutrition dependency were analyzed.Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6 months (oral feeding group). The mean follow-up was 7.9 years (IQR, 1.6-17.5 years) and 5.0 years (IQR, 0.1-18.2 years), respectively. Both bowel matting (OR, 14.23; 1.07-16.7; P = .039) and secondarily diagnosed atresia or stenosis (OR, 17.78; 3.13-100.98; P = .001) were independent postnatal predictors of SBS-IF. Eighteen children (51% of the SBS-IF group) were still dependent on artificial nutrition at the last follow-up. patients with SBS-IF who achieved full oral feeding had a median residual small-bowel length of 74 cm (IQR, 51-160 cm) vs 44 cm (IQR, 10-105 cm) for those still dependent on artificial nutrition (P = .02). An initial residual small bowel length of more than 50 cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%.Bowel matting, complex gastroschisis, and secondary intestinal obstruction were associated with SBS-IF in gastroschisis. For patients with SBS-IF, a small bowel length of more than 50 cm was predictive of secondary nutritional autonomy.

Giant omphalocele often represents a major surgical challenge and is reported with high mortality and morbidity rates. The aim of this study was to assess the outcome of neonates with giant omphalocele managed with early operative surgical treatment, and subsequently to identify possible factors that could alter the prognosis.We reviewed the medical records of 29 consecutive newborns with prenatally diagnosed giant omphalocele. In these cases one of two procedures had been performed: either staged closure after silo, or immediate closure with a synthetic patch. The cases were separated into 2 groups: Isolated giant omphalocele (IO group) and giant omphalocele associated with malformation (NIO group).Infants in the IO group had a lower size of the omphalocele (p0,001), a shorter hospital stay (95 days [45-915] vs. 41.5 days [10-110] p= 0, 02), and a shorter median ventilation length (10 days [1-33] vs. 27, 5 [6-65] p = 0, 05). In the NIO group, 5 cases displayed a significantly more difficult course than the others. They were compared to the remaining cases for prenatal and anatomic features. Four factors associated with greater morbidity were identified: CONCLUSIONS: Isolated omphalocele, even containing the whole liver, has a very good prognosis with early surgical treatment. Without associated anomalies, 95% of giant omphaloceles can be discharged with a median of 41.5 days in hospital. However, associated anomalies (especially cardiopathies) may burden the prognosis and should be both carefully assessed during pregnancy and taken into account in parental information.Retrospective Study LEVEL OF EVIDENCE: Level I.

OBJECTIVE Evaluate the neonatal management and outcomes of neonates with prenatal diagnosis of esophageal atresia (EA) type A. METHODS This population-based study was conducted using data from the French National Register for infants with EA born from 2008 to 2014, including all cases of EA type A. We compared prenatal and neonatal characteristics and outcomes in children with prenatal diagnosis of EA type A with those with a postnatal diagnosis until the age of 1. RESULTS A total of 1118 live births with EA were recorded among which 88 (7.9%) were EA type A. Prenatal diagnoses were performed in 75 cases (85.2%), and counselling with a prenatal specialist was conducted in 84.8% of the prenatal group. Still within that group, the gestational age at delivery was significantly higher than in the postnatal group (36 [35-38] versus 34 [32-36] weeks; P = .048). Inborn births were more frequent in the prenatal group (86.1% vs 7.7%, P

Introduction En l’absence de traitement antiviral le pronostic de la leucoencephalopathie multifocale progressive (LEMP) repose sur la restauration des reponses immunitaires antivirales de l’hote. L’utilisation d’interleukine 7 recombinante humaine (IL-7rh), sensee promouvoir la restauration quantitative et qualitative des reponses immunitaires cellulaires, a montre de maniere anecdotique un benefice dans la prise en charge de LEMP. Notre objectif est de rapporter l’issue a 1 an d’une cohorte de patients traites par rhIL-7 pour une LEMP. Materiels et methodes Inclusion de tous les patients consecutifs ayant recu au minimum 1 injection d’IL-7rh fournie par Cytheris/Revimmune pour une LEMP prouvee entre 2007 et 2020. Description retrospective des caracteristiques initiales, et des donnees de suivi. Resultats 85 patients ont recu un traitement par IL-7rh. Nous avons exclu 21 patients principalement en raison de donnees manquantes. Parmi les 64 patients inclus avec une LEMP prouvee par PCR et/ou histologie, 27 presentaient une infection par le VIH (PvVIH, 42,2 %), 16 une hemopathie maligne (HM, 25 %), 4 une pathologie auto-immune (PAI, 6,3 %), 13 un deficit immunitaire primitif (DIP, 20,3 %), et 4 etaient transplantes d’organe solide (TOS, 6,3 %). Le traitement etait utilise principalement a la dose de 10 μg/kg (79,7 %), par voie intramusculaire (73,4 %), et a raison de 3 ou 4 injections (85,9 %). Le delai median entre le diagnostic de LEMP et l’initiation de l’IL-7rh etait de 42 jours (IQR 22-61). A 1 an de l’initiation de l’IL-7rh 35 patients etaient vivants (54,7 %), 1 patient etait perdu de vue, et 28 patients etaient decedes (43,8 %) dans un delai median de 42,5 jours (IQR 23,8-60,8). Les patients decedes a 1 an representent 44,4 % des PvVIH, 50 % des HM, 25 % des PAI, 46,2 % des DIP et 25 % des TOS. Tous les patients sont decedes de complications de la LEMP, avec pour 2 patients l’imputabilite surajoutee de leur pathologie initiale. 5 patients (7,8 %) ont presente un IRIS-LEMP dans un delai median de 22 jours apres la 1ere injection d’IL-7rh (IQR 17-25). 19 patients ont presente des reactions au point d’injection ou des syndromes pseudo-grippaux (29,6 %). L’analyse multivariee des caracteristiques initiales montre un meilleur pronostic chez les femmes (p = 0,019), et en cas d’atteinte a l’IRM peu etendue (≤ 2 regions, p = 0,033). L’analyse univariee des donnees de suivi montre un meilleur pronostic en cas de diminution ≥ 1 log de la charge virale JCV dans le LCR au 1er controle (p = 0,019) et en cas d’augmentation ≥ 50% du taux de CD4 dans le 1er mois post-IL-7rh (p = 0,008). Conclusion Bien que l’IL-7rh puisse avoir ameliore le pronostic de la LEMP chez certains patients en comparaison avec les donnees de la litterature, cette strategie n’a pas ete benefique pour tous les patients. Le profil de tolerance a moyen terme semble satisfaisant. L’interet des marqueurs predictifs de reponse tels que l’evolution de la charge virale JCV dans le LCR et le taux de CD4 merite d’etre precise.

Background Subjective cognitive complaint (SCC) is a criterion recommended by the Movement Disorder Society (MDS) task force for the diagnosis of mild cognitive impairment (MCI). Until now there were few specific tools for detecting SCC in PD. We sought to develop a new tool to assess SCC specifically dedicated for PD. Materials and Methods We set a group of experts in movements disorders and neurocognition to develop an easy-to-use tool based on a visual analogue scale (VAS) for five cognitive domains: memory, executive functions, spatial orientation, attention, and language. We use it to assess SCC twice (at a one-month interval) in PD patients with disease duration of less than 5 years. Comprehensibility of the VAS was assessed. Controls were assessed with the same VAS. Patients with PD also underwent neuropsychological testing. Results VAS was easily understandable by the 70 patients with PD. We found significant SCC for the patients with PD vs controls in three cognitive domains: executive functions (1.7 ± 1.9 vs 0.8 ± 1.1; P

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 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.

An inhibitor causes neuronal excitation Glycine is thought to be primarily an inhibitory neurotransmitter. However, it also acts as a coagonist on excitatory N -methyl-D-aspartate (NMDA) receptors. Otsu et al. examined the function of the NMDA receptor subunit combination GluN1/GluN3A in the medial habenula (MHb) of adult mice. This NMDA receptor subunit combination in MHb neurons is activated by glycine released from astrocytes. Activation of GluN1/GluN3A NMDA receptors causes depolarization and increased spiking of MHb neurons. Reducing GluN3A receptor subunit levels in the MHb blocks conditioned place aversion. Science , this issue p. 250

Objectif L’objectif de ce travail est d’evaluer la consommation medicamenteuse des salaries et son eventuel lien avec le travail. Il sera alors decrit la consommation medicamenteuse par classes ATC la semaine precedente selon les categories socioprofessionnelles des travailleurs et le lien eventuel avec la satisfaction au travail et la penibilite physique. Methodes Cette etude est observationnelle et monocentrique. Les informations ont ete recueillies par auto-questionnaires lors d’une visite dans un service de sante au travail. Resultats La population etudiee comprenait 3160 salaries, d’un âge moyen de 37 ans, comprenant 36,8 % de cols blancs, 25,1 % de techniciens/superviseurs, 22,6 % d’executifs et 15 % de cols bleus. Cette population etait composee de 60 % de femmes et 40 % d’hommes. Sur une echelle EVA validee, une satisfaction au travail cotee a au moins 5/10 etait retrouvee chez 82 % des salariee. La penibilite physique d’au moins 5/10 etait retrouvee chez 33,5 % des travailleurs et une pression temporelle de 5 et plus/10 dans 55,4 % des cas. La moitie des salaries declarent avoir consomme au moins 1 classe medicamenteuse durant la semaine precedant le remplissage du questionnaire et 33,8 % des consommations medicamenteuses sont declarees en lien avec le travail par les salaries. Les 3 categories medicamenteuses declarees les plus consommees sont les analgesiques, les traitements « musculo-squelettiques » et les psychotropes. Les salaries consommateurs de medicaments sont plus nombreux a declarer une satisfaction au travail faible que les salaries non consommateurs (p-value Conclusion Cette etude a permis d’evaluer la consommation medicamenteuse d’une population generale de travailleurs Une satisfaction au travail faible est associee statistiquement avec une consommation attribuee au travail pour les medicaments antalgiques, psychotropes, antidepresseurs, musculo-squelettiques, anxiolytiques, hypnotiques/sedatifs et homeopathiques. Quant a la penibilite physique elevee au travail, elle semble associee significativement avec la consommation attribuee au travail pour les medicaments antalgiques, musculo-squelettiques et anxiolytiques. Ainsi, nous avons mis en avant les associations etablies entre consommation medicamenteuse declaree en lien avec le travail et facteurs professionnels.

Objective The aim of this study was to assess the diagnostic agreement between the prenatal diagnosis of intra-abdominal cystic lesions made by ultrasound examination and the postnatal diagnosis. Methods We reviewed all consecutive cases referred for an anechoic abdominal cyst from 2009 to 2013. Prenatal ultrasound diagnosis was compared with postnatal diagnosis. Prenatal diagnosis was defined as ‘correct’ if a specific prenatal diagnosis or one of the possible diagnoses was confirmed postnatally, as ‘not confirmed’ if the postnatal examination revealed no abnormalities and as ‘incorrect’ if the postnatal diagnosis was different from those suggested prenatally. Results Seventy-three cases were included, and prenatal diagnoses were made at a median gestational age of 27 weeks (range: 13–36). Correct diagnoses were made in 66 cases (90.4%), including four in which the lesion resolved spontaneously in utero; two diagnoses were ‘not confirmed’ postnatally, and one was incorrect (a prenatal diagnosis of intestinal duplication was in fact an anorectal malformation). Postnatal diagnosis was not achieved in four cases: None of them required surgery, and clinical follow-up was favorable. The abdominal cysts were isolated in 52 cases (71%) and associated with other anomalies in 21 cases (29%). Aneuploidies were diagnosed in three cases (all trisomy 21). Eight cases underwent termination of pregnancy; there were no fetal deaths and one neonatal death. Postnatal surgery was performed in 30 out of 65 liveborn infants (46.1%). Conclusion Overall diagnostic agreement between prenatal and postnatal diagnosis of fetal intra-abdominal cystic lesions is high. © 2015 John Wiley & Sons, Ltd.

OBJECTIVES Few studies have evaluated long-term diaphragmatic function in congenital diaphragmatic hernia (CDH). The aim of our cross-sectional study was to assess diaphragmatic function in infants and young children with CDH after surgical repair. METHODS All the patients with CDH repair followed in our centre between February 2014 and January 2016 were enrolled. Patients with a postnatal diagnosis after 1 month of life were excluded. Breathing pattern and diaphragmatic function were assessed using esophageal and gastric (Pgas) pressure recording after surgery, or at 1 or 5 years of age. RESULTS Twenty-eight patients (24 left-sided CDH, 6 with diaphragmatic patch) were included. Twelve patients were assessed before hospital discharge (Y0), 6 around the age of 1 year (Y1) and 10 around the age of 5 years (Y5). Mean antenatal estimated pulmonary volume (VLA) was 42 ± 10% (n = 23). Diaphragmatic strength, assessed by transdiaphragmatic pressure during crying/sniff, was low at Y0 (47 ± 18 cmH2O, n = 12) and within normality at Y5 (81 ± 15 cmH2O, n = 7). Diaphragmatic dysfunction, assessed by Pgas during crying/sniff, was present at Y0 (-58 ± 22 cmH2O, n = 12) and Y1 (-53 ± 36 cmH2O, n = 5) and still present at Y5 (3 ± 9 cmH2O, n = 7) but to a lesser extent. The diaphragmatic tension time index (TTdi), which estimates diaphragmatic endurance, was high at Y0 (0.10 ± 0.04, n = 11) and within normality at Y5 (0.03 ± 0.01, n = 6). VLA correlated with neonatal TTdi (r = -0.961, P

Objective To compare the accuracy of Magnetic Resonance Imaging (MRI) and ultrasound (US) to diagnose and characterize congenital obstructive genital abnormalities. Method Retrospective cohort of 20 fetuses who underwent a fetal MRI following an ultrasound diagnosis of obstructive urogenital malformation. We compared MRI and US findings and their correlation with the definitive diagnosis. Result The correct diagnosis was obtained in 6/20 (30%) cases and 19/20 cases (95%) with US and MRI respectively. MRI revealed additional information to US in 15/20 cases (75%) and modified the prenatal management in 14 fetuses (70%). The identification rates of the most important anatomical landmarks for the diagnosis, using US and MRI, were compared. Bladder: US 17/20 (85%) vs MRI 20/20 (100%) p=0.23; vagina: US 6/19 (31.5%) vs MRI 19/19 (100%) p

SummarySensory computations performed in the neocortex involve layer six (L6) cortico-cortical (CC) and cortico-thalamic (CT) signaling pathways. Developing an understanding of the physiological role of these circuits requires dissection of the functional specificity and connectivity of the underlying individual projection neurons. By combining whole-cell recording from identified L6 principal cells in the mouse primary visual cortex (V1) with modified rabies virus-based input mapping, we have determined the sensory response properties and upstream monosynaptic connectivity of cells mediating the CC or CT pathway. We show that CC-projecting cells encompass a broad spectrum of selectivity to stimulus orientation and are predominantly innervated by deep layer V1 neurons. In contrast, CT-projecting cells are ultrasparse firing, exquisitely tuned to orientation and direction information, and receive long-range input from higher cortical areas. This segregation in function and connectivity indicates that L6 microcircuits route specific contextual and stimulus-related information within and outside the cortical network.

Resume Objectifs de l’etude La dosimetrie in vivo a pour objectif de detecter et de corriger les ecarts de traitements significatifs en radiotherapie. L’utilisation des imageurs portals permet de rendre ces controles rapides et riches en informations. De nombreux travaux ont ete publies sur ce sujet, mais aucune recommandation n’a ete etablie pour definir les niveaux de tolerance pour ces controles. Nous proposons une methodologie simple pour evaluer le niveau de tolerance global des resultats in vivo obtenus avec ces detecteurs plans. Materiels et methodes Dans un premier temps, les incertitudes liees a la methode de reconstruction ont ete evaluees. Elles ont ete determinees sur un ensemble de geometries, representative des situations cliniques, pour lesquelles 45 points de mesure de dose ont ete definis. Dans un deuxieme temps, il a ete realise une etude des variations induites par le cadre de la mesure in vivo en presence du patient afin de definir des niveaux d’actions realistes et adaptes a la routine clinique. Pour finir, une methodologie est proposee pour determiner si la methode, associee a ces niveaux de tolerance, permet de detecter des ecarts significatifs de traitements, superieures a 5 % a la dose prescrite. Resultats – conclusion En appliquant cette methodologie, nous avons conclu qu’un niveau de tolerance de 6,5 % (k = 2) pouvait etre associe a notre methode de dosimetrie in vivo. Il a ete montre que cette valeur permettait dans de nombreux cas de detecter des differences de 5 % de la dose prescrite. La dosimetrie in vivo de transmission ne peut pas pretendre detecter tous les ecarts de traitements, mais elle permet d’augmenter la securite des patients.

Resume Les malformations ano-rectales (MAR) sont la consequence d’anomalies du developpement de la partie terminale du tube digestif interessant l’anus et/ou le rectum qui surviennent precocement entre la 6e et la 10e semaines de developpement embryonnaire. Elles realisent un spectre malformatif dont la severite est fonction du niveau d’interruption du conduit ano-rectal et des malformations caudales associees (sacrum et cone terminal de la moelle). Les MAR s’associent dans plus de la moitie des cas a d’autres malformations pouvant s’integrer dans des syndromes connus. Si le traitement chirurgical permettant de restaurer une anatomie aussi proche que possible de la normale est essentiel, la prise en charge postoperatoire est tout aussi fondamentale pour obtenir chez ces patients, dont les mecanismes defecatoires sont toujours alteres, sinon une continence, au moins une proprete socialement acceptable.

Pain associated with vaso-occlusive crisis is the main cause of hospitalization in children with sickle cell disease. Recent studies have suggested that pain might have a neuropathic component. Lidocaine patches are commonly prescribed as a topical analgesic in adult neuropathic pain. This study reports the efficacy and safety of such treatment in 6 patients with sickle cell disease, aged 6-18 years, who had been hospitalized for vaso-occlusive crisis after failure of the standard analgesic treatment. These data have led to setting up a confirmatory phase II trial, which is currently underway.

Background Complete intestinal volvulus is mainly related to congenital anomalies of the so-called intestinal malrotation, whereas segmental volvulus appears as a distinct entity, mostly observed during the perinatal period. Because these two situations are still lumped together, the aim of this study was to describe the particular condition of neonatal segmental volvulus. Study design We analyzed the circumstances of diagnosis and management of 17 consecutives neonates operated for segmental volvulus more than a 10-year period in a single institution. During the same period, 19 cases of neonatal complete midgut volvulus were operated. Results Prenatal US exam anomalies were observed in 16/17 (94%) of segmental volvulus, significantly more frequently than in complete volvulus ( p =0.003). Intestinal malposition was described peroperatively in all cases of complete volvulus, but also in 4/17 segmental volvulus (23%). Intestinal resection was performed in 88% of segmental volvulus when only one extensive intestinal necrosis was observed in complete volvulus. Parenteral nutrition was required in all patients with segmental volvulus with a median duration of 50days (range 5–251). Conclusion Segmental volvulus occurs mainly prenatally and leads to fetal ultrasound anomalies. This situation, despite a limited length of intestinal loss, is associated to significant postnatal morbidity. Type of the study: Treatment study. Level of evidence Level IV.

Resume Neisseria meningitidis peut etre a l’origine d’infections invasives severes, notamment des septicemies et des meningites. Les atteintes articulaires sont rares et les atteintes osseuses semblent exceptionnelles. Si les myosites inflammatoires associees aux meningococcemies sont bien connues, la possibilite de myosite infectieuse a meningocoque n’a jamais ete rapportee a notre connaissance. Nous decrivons le cas d’un garcon de 7 ans ayant presente une osteoarthrite de hanche droite associee a une myosite multifocale bilaterale dont la seule documentation bacteriologique avait ete la mise en evidence d’un meningocoque B par reaction de polymerisation en chaine (PCR) dans le liquide articulaire. La premiere particularite de cette observation a ete l’evolution longue et initialement defavorable. Malgre une antibiotherapie probabiliste associant cefamandol et gentamycine des le 2e j, l’evolution clinique n’avait ete favorable qu’au 10e j, apres remplacement de l’antibiotherapie par amoxicilline-acide clavulanique et clindamycine et une arthrotomie-lavage. La seconde particularite a ete la coexistence d’une myosite multifocale dont un des foyers etait controlateral a la lesion osteoarticulaire. Le diagnostic d’arthrite meningococcique avait ete pose grâce a la PCR 16S realisee sur le liquide articulaire. Cette observation confirme l’importance d’une prise en charge medico-chirurgicale dans les arthrites des grosses articulations, ainsi que l’interet diagnostique de la PCR universelle sur le liquide articulaire. Elle montre egalement la possibilite de myosite bacterienne a meningocoque B, l’atteinte musculaire controlaterale etant atypique pour une cause inflammatoire.

The cerebellar cortex coordinates movements and maintains balance by modifying motor commands as a function of sensory-motor context, which is encoded by mossy fiber (MF) activity. MFs exhibit a wide range of activity, from brief precisely timed high-frequency bursts, which encode discrete variables such as whisker stimulation, to low-frequency sustained rate-coded modulation, which encodes continuous variables such as head velocity. While high-frequency MF inputs have been shown to activate granule cells (GCs) effectively, much less is known about sustained low-frequency signaling through the GC layer, which is impeded by a hyperpolarized resting potential and strong GABAA-mediated tonic inhibition of GCs. Here we have exploited the intrinsic MF network of unipolar brush cells to activate GCs with sustained low-frequency asynchronous MF inputs in rat cerebellar slices. We find that low-frequency MF input modulates the intrinsic firing of Purkinje cells, and that this signal transmission through the GC layer requires synaptic activation of Mg2+-block-resistant NMDA receptors (NMDARs) that are likely to contain the GluN2C subunit. Slow NMDAR conductances sum temporally to contribute approximately half the MF-GC synaptic charge at hyperpolarized potentials. Simulations of synaptic integration in GCs show that the NMDAR and slow spillover-activated AMPA receptor (AMPAR) components depolarize GCs to a similar extent. Moreover, their combined depolarizing effect enables the fast quantal AMPAR component to trigger action potentials at low MF input frequencies. Our results suggest that the weak Mg2+block of GluN2C-containing NMDARs enables transmission of low-frequency MF signals through the input layer of the cerebellar cortex.

Objective A decrease in the volume of congenital pulmonary malformations (CPM) can be observed on prenatal ultrasonography, but the underlying mechanism for this phenomenon is unknown. Our objective was to identify factors associated with the prenatal reduction in size of cystic and/or hyperechoic lung lesions. Methods This was a retrospective study of cases with a prenatal diagnosis of hyperechoic and/or cystic lung lesion. The extent of reduction in lesion size was calculated from ultrasound measurements. Clinical, ultrasound, radiological and histological data were tested for their relationship with prenatal CPM reduction. Results In a 4-year period, 36 patients were referred with a cystic and/or hyperechoic fetal lung lesion diagnosed at a mean gestational age of 23.4 weeks. The lesions were cystic in 16 cases (44%), hyperechoic in 12 (33%) and both in eight (22%). The malformation was no longer visible before birth (apparent disappearance) in nine cases (25%), shrank by 18–90% in 15 (42%) and did not reduce in 12 (33%). Findings on postnatal computed tomography were always abnormal. Isolated hyperechoic lesions were significantly more likely to shrink in utero. The mean reductions were 79%, 35% and 19%, for isolated hyperechoic, cystic and mixed lesions, respectively (P = 0.001). Only 8% of hyperechoic lesions demonstrated no volume reduction, as compared to 50% and 42% of cystic and mixed lesions, respectively (P = 0.03). Greater gestational age at birth was also associated with a decrease in the incidence of malformations (P = 0.02). In cases that underwent surgery, hyperechoic lesions were linked to a variety of pathological diagnoses, whereas cystic lesions were all described histologically as congenital cystic adenomatoid malformations. Conclusions Prenatal size reduction of fetal lung malformations is associated with isolated hyperechogenicity and greater gestational age at birth. This might result from the resumption of normal lung development after local disruption of lung growth. Copyright  2011 ISUOG. Published by John Wiley & Sons, Ltd.

The prenatal diagnosis of esophageal atresia is challenging. The length of the defect of the esophageal atretic portion is one of the parameters affecting outcome and prenatal evaluation of this length has not, to our knowledge, been described previously. We report on seven fetuses assessed prospectively which were suspected to have esophageal atresia. Targeted ultrasound examination of both fetal cervical and thoracic structures was performed in each case in order to assess prenatally the atretic portion. The length of the defect was assessed both directly, by visualizing the interruption of the hyperechoic lines representing the walls of the esophagus in a mid-sagittal view (n = 4), and indirectly, by means of the 'tracheal print' (n = 5). Both methods were used in three cases. Prenatal results were compared with postnatal or postmortem findings. The prenatal diagnosis of esophageal atresia was made correctly in six of the seven cases and in all of these there was concordance between prenatal and postnatal estimates of the esophageal defect lengths. Direct or indirect sonographic assessment of the esophagus in cases of suspected prenatal esophageal atresia improves the specificity of its diagnosis and aids prenatal evaluation.

Objectives The objectives of the study were to provide a review of patients with giant omphalocele managed in a single institution (2001-2006), focusing on medical management in the neonatal period, and to evaluate short-term outcomes. Methods Data from 14 neonates with giant ompholocele (abdominal wall defect >5 cm and/or containing liver) and the absence of malformation and chromosomal anomalies during fetal screening were retrospectively reviewed. All were intubated and sedated before surgical treatment. Initial management consisted of progressive reduction of the herniated organs by gentle compression. After sequential reduction, abdominal wall closure was attempted at the skin and fascia level and, when necessary, with a Gore-Tex patch. Results Median gestational age was 39 weeks (38-40), and median birth weight was 3100 g (2470-3700). Median age at closure was 6 days (0-20). A central Gore-Tex patch was inserted in 10 cases. Median ventilation length was 26 days (2-78). Full enteral diet was achieved after an average of 33 days (8-82), and median time until discharge from the intensive care unit was 24.5 days (11-85). Nine patients developed sepsis in the postoperative course. In 10 patients, at least 1 associated malformation was diagnosed in the postnatal course, among which cardiac and diaphragmatic defects were the most common. Survival rate was 85.7%. Conclusion Mortality rate of giant omphalocele without chromosomal anomaly or major malformations is low when treated by gradual reduction of the contents. Parents should be informed of the long hospitalization in the intensive care unit at birth, the potential nonthreatening associated malformations to be diagnosed after birth, and the high risk of sepsis.

Summary To interpret visual-motion events, the underlying computation must involve internal reference to the motion status of the observer’s head. We show here that layer 6 (L6) principal neurons in mouse primary visual cortex (V1) receive a diffuse, vestibular-mediated synaptic input that signals the angular velocity of horizontal rotation. Behavioral and theoretical experiments indicate that these inputs, distributed over a network of 100 L6 neurons, provide both a reliable estimate and, therefore, physiological separation of head-velocity signals. During head rotation in the presence of visual stimuli, L6 neurons exhibit postsynaptic responses that approximate the arithmetic sum of the vestibular and visual-motion response. Functional input mapping reveals that these internal motion signals arrive into L6 via a direct projection from the retrosplenial cortex. We therefore propose that visual-motion processing in V1 L6 is multisensory and contextually dependent on the motion status of the animal’s head., Highlights • Inputs onto V1 L6 neurons convey head-motion information • These L6 signals are widespread and provide a reliable estimate of angular velocity • In L6 neurons, vestibular and visual inputs sum during sensory processing • At least in part, V1 L6 head-motion signals are conveyed via an RSP-V1 pathway, V1 layer 6 neurons receive a widespread head-motion signal that is integrated with visual input during visual-motion processing. These V1 head-motion inputs project from the retrosplenial cortex, a multisensory area involved in spatial navigation and contextual processing.

Contains fulltext : 81905.pdf (Publisher’s version ) (Closed access) OBJECTIVES: To investigate the value of the observed to expected fetal lung area to head circumference ratio (o/e LHR) and liver position in the prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia (CDH). METHODS: Neonatal morbidity was recorded in 100 consecutive cases with isolated CDH diagnosed in fetal medicine units, which were expectantly managed in the prenatal period, were delivered after 30 weeks and survived until discharge from hospital. Regression analysis was used to identify the significant predictors of morbidity, including prenatal and immediate neonatal findings. RESULTS: The o/e LHR provided significant prediction of the need for prosthetic patch repair, duration of assisted ventilation, need for supplemental oxygen at 28 days, and incidence of feeding problems. An additional independent prenatal predictor of the need for patch repair was the presence of fetal liver in the chest. CONCLUSIONS: In isolated CDH the prenatally assessed size of the contralateral lung is a significant predictor of the need for prosthetic patch repair, the functional consequences of impaired lung development and occurrence of feeding problems.

The cerebellum controls complex, coordinated, and rapid movements, a function requiring precise timing abilities. However, the network mechanisms that underlie the temporal organization of activity in the cerebellum are largely unexplored, because in vivo recordings have usually targeted single units. Here, we use tetrode and multisite recordings to demonstrate that Purkinje cell activity is synchronized by a high-frequency (approximately 200 Hz) population oscillation. We combine pharmacological experiments and modeling to show how the recurrent inhibitory connections between Purkinje cells are sufficient to generate these oscillations. A key feature of these oscillations is a fixed population frequency that is independent of the firing rates of the individual cells. Convergence in the deep cerebellar nuclei of Purkinje cell activity, synchronized by these oscillations, likely organizes temporally the cerebellar output.

Les traumatismes abdominaux de l’enfant resultent de deux mecanismes differents. Chocs directs dans les accidents de la vie courante, et mecanismes complexes d’acceleration-deceleration, plus ou moins associes a des chocs directs, dans les accidents de la voie publique (AVP), et les chutes de grande hauteur, sont essentiellement responsables de lesions hepatospleniques, renales, ou plus rarement pancreatiques. Si l’orientation clinique, sur des lesions, le plus souvent isolees, et a faible retentissement general initial, est en regle facile dans le premier cas, des lesions souvent multiples, engageant le pronostic vital, et integrees dans le cadre d’un polytraumatisme, doivent etre recherchees systematiquement au decours des AVP. L’hypovolemie est le risque principal de ces lesions et doit etre compensee rapidement avant meme d’entreprendre des examens complementaires. L’echographie, facile a mettre en œuvre au lit du malade, est un excellent moyen de diagnostic des lesions hepatiques, spleniques, et renales, et permet d’apprecier l’importance d’un epanchement. Le scanner, examen de reference, en particulier chez le polytraumatise, permet de faire un bilan precis des lesions. Le diagnostic des lesions de perforation d’organes creux reste cependant difficile en urgence, et est le plus souvent retarde devant l’apparition de signes de peritonite. Le traitement conservateur est la regle. Une compensation rigoureuse de l’hypovolemie initiale, une strategie transfusionnelle adaptee, et le recours, dans les cas les plus graves, aux accelerateurs de l’hemostase, ont permis de diminuer de facon drastique les indications chirurgicales en urgence, mais aussi les besoins transfusionnels. Les echecs du traitement conservateur sont rares, et sont essentiellement le fait de lesions de perforation digestive. Les rares indications restantes du traitement chirurgical en urgence sont les plaies penetrantes avec lesions intraperitoneales averees, les lesions renales necessitant un geste de revascularisation, et certaines lesions de la voie excretrice. Dans tous les cas, une laparotomie d’hemostase en extreme urgence est un geste a tres haut risque, greve d’une tres lourde mortalite, et souvent illusoire chez un polytraumatise.

Modifications of water state were analyzed during ethylnitrosourea-induction of brain tumor in rats. Four different steps were identified in the cancerization process according to NMR and histological findings. Two analogies were observed in the pattern of ‘bound’ water at decreasing temperatures: first the pattern was similar in tumor area and white matter, second the pattern was similar in the same area of normal brain tissue and cortical gray matter. This phenomenon, which corroborates previous reports on liver cancerization, points out that pathological proliferation of glial cells, and their progressive organization into multiple layers, is accompanied by a transformation of water properties at the cellular level.

Thin films of carbon nitride CNx were prepared on a non-heated substrate in the pressure range 100–1000 Pa. Carbon nitride films were deposited by ablation of carbon molecular fragments from a graphite or pyrocarbon target onto a silicon substrate after a few pulses of a localized transversely excited atmospheric pressure (TEA) CO2 laser. The deposition medium was either a gaseous flow of pure nitrogen or nitrogen—helium mixtures or cold remote plasma of nitrogen (CRNP) or of nitrogen—hydrogen mixtures (CRNPH). Fourier transform IR (FTIR) spectra of the deposited films show a very efficient nitrogen fixation with C-N bands characteristic of tetrahedral carbon. These results are confirmed by Raman spectroscopy. X-ray photoelectron spectroscopy of samples typically shows two peaks in the N 1s core level spectrum (centred at 399.4 and 400.8 eV). The effect of the distance of the deposition zone from the discharge is also discussed. The plasma created around the target is studied by UV—visible optical emission spectroscopy (OES). Gas phase results are correlated with the characteristics of the deposits.

The aim of our work is to explore the use of IFN-beta for gene therapy in the HIV-infection. Transduction of various HIV target cells with a retroviral vector that carries the Hu-IFN-beta coding sequence under constitutive low expression control, confers resistance to HIV without affecting cell replication or function. After transduction, lymphocytes from HIV-infected patients develop resistance to the endogenous virus, provided the cells are derived from individuals with a CD4 cell count higher than 200 per mm3.

Intestinal morphogenesis and differentiation are dependent on heterotypic cell interactions between embryonic epithelial cells (endoderm) and stromal cells (mesenchyme). Extracellular matrix molecules represent attractive candidates for regulators of these interactions. The structural and functional diversity of the extracellular matrix as intestinal development proceeds is demonstrated by 1) spatio-temporal specific expression of the classically described constituents, 2) the finding of laminin and collagen IV variants, 3) changes in the ratio of individual constituent chains, and 4) a stage-specific regulation of basement membrane molecule production, in particular by glucocorticoids. The orientation/assembly of these extracellular matrix molecules could direct precise cellular functions through interactions via integrin molecules. The involvement of extracellular matrix, and in particular basement membrane molecules in heterotypic cell interactions leading to epithelial cell differentiation, has been highlighted by the use of experimental models such as cocultures, hybrid intestines and antisense approaches. These models allowed us to conclude that a correct elaboration and assembly of the basement membrane, following close contacts between epithelial and fibroblastic cells, is necessary for the expression of differentiation markers such as digestive enzymes.

In this paper we develop applications of the spectral moments method to the study of the propagation of sound waves and electromagnetic waves in heterogeneous systems with or without energy dissipation. In these systems the matrices are often asymmetric. We show that the moments can be very useful for determining the Green functions of such systems. For a matrix with real eigenvalues the method requires only the introduction of a 1D auxiliary density function while for a matrix with complex eigenvalues it needs the introduction of a 2D density function. These functions can be calculated by the moments technique and lead to computation of the Green functions. For systems involving slightly asymmetric matrices, we found that the method is particularly stable while, for strongly asymmetric matrices, it is better to calculate directly a response function which is a bilinear combination of Green functions.

Objective The aim of this study was to investigate psychological stress in parents of children with esophageal atresia and to explore factors associated with the development of Posttraumatic Stress disorder (PTSD). Design Self-report questionnaires were administered to parents of children with EA. Domains included: (1) sociodemographic data, current personal difficulties, assessment scales for the quality of life and for the global health status of the child (2) French-validated versions of the Perinatal Posttraumatic Stress disorder Questionnaire and of the State-Trait Anxiety Inventory. Associations between PTSD and severity of the neonatal course, presence of severe sequelae at 2 years of age, and quality of life and global health status of children according to their parents’ perception were studied. Setting A Tertiary care University Hospital Results Among 64 eligible families, 54 parents of 38 children (59%) participated to the study. PTSD was present in 32 (59%) parents; mothers were more frequently affected than fathers (69 vs 46%, p = 0.03). Four mothers (8%) had severe anxiety. PTSD was neither associated with neonatal severity nor with severe sequelae at 2 years. Parents with PTSD rated their child’s quality of life and global health status significantly lower (7.5 vs 8.6; p = 0.01 and 7.4 vs 8.3; p = 0.02 respectively). Conclusions PTSD is frequent in parents of children with esophageal atresia, independently of neonatal severity and presence of severe sequelae at 2 years of age. Our results highlight the need for a long-term psychological support of families.

This trial compares Casodex (ICI 176,334) monotherapy with the combination of castration (medical or surgical) plus nilutamide. The trial is now closed to entry, 270 patients having been recruited from 32 French centres. As it is too early to present efficacy data, only patient characteristics and interim tolerability data appear in this paper. In the combined treatment group, interstitial pneumonitis (4.5%) was observed, leading to withdrawal from the trial. Other adverse events leading to withdrawal included dyspnoea and ocular problems. There was also 1 case of hepatitis in this treatment group. In the Casodex treatment group, only 6 patients (as compared with 13 in the combined treatment group) withdrew from the trial because of adverse events. As expected with this group, the adverse events were mainly pharmacological effects of an anti-androgen as monotherapy. In the majority of patients, the effects of gynaecomastia and breast tenderness did not result in withdrawal

Interband tunneling in InAs/AlSb/GaSb double-barrier heterostructures was calculated. A realistic ten-band tight-binding model was used to include the effect of mixing between the bands. The model was solved utilising a technique for many-layer structures which eliminates numerical instabilities. We present current density versus voltage curves for different well and barrier widths and show the effects of including the dependence of the transmission on the parallel component of the incident wave vector (K ∥)