Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009

<p>Abstract</p> <p>Objective</p> <p>To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.</p> <p>Study design</p> <p>Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.</p> <p>Results</p> <p>21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications.</p> <p>Conclusion</p> <p>Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.</p

Système d'acquisition de comptages de neutrons (ou d'événements) modulaire en standard CAMAC

Le présent mémoire a pour objet la réalisation matérielle et logicielle d'un système d'acquisition de comptage de neutrons (ou d'évènements). Un faisceau de neutron envoyé sur un échantillon à étudier est diffracté dans différentes directions. L'étude de ces directions et des longueurs d'ondes donnent des information sur la constitution interne de l'échantillon. Les neutrons diffractés, localisés par un détecteur ou multidétecteur sont remis en forme par une électronique analogique d'amplification. Les signaux supérieurs à un seuil génèrent une impulsion logique d'une durée avoisinant la µs. Ces impulsion doivent être comptabilisée pendant la durée d'une acquisition. Pour avoir des informations sur l(évolution de processus physiques, il est nécessaire d'incrémenter dans différentes zones correspondant aux différents temps. L'acquisition terminée les données sont transférées au calculateur de contrôle de processus (transmission parallèle) pour stockage temporaire et prétraitement, puis transmission au calculateur central (réseau local DECNET)

Clinical predictors of radiographic abnormalities among infants with bronchiolitis in a paediatric emergency department

International audienceBackgroundAcute viral respiratory exacerbation is one of the most common conditions encountered in a paediatric emergency department (PED) during winter months. We aimed at defining clinical predictors of chest radiography prescription and radiographic abnormalities, among infants with bronchiolitis in a paediatric emergency department.MethodsWe conducted a prospective cohort study of children less than 2 years of age with clinical bronchiolitis, who presented for evaluation at the paediatric emergency department of an urban general hospital in France. Detailed information regarding historical features, examination findings, and management were collected. Clinical predictors of interest were explored in multivariate logistic regression models.ResultsAmong 410 chest radiographs blindly interpreted by two experts, 40 (9.7%) were considered as abnormal. Clinical predictors of chest radiography achievement were age (under three months), feeding difficulties, fever over 38°C, hypoxia under than 95% of oxygen saturation, respiratory distress, crackles, and bronchitis rales. Clinical predictors of radiographic abnormalities were fever and close to significance hypoxia and conjunctivitis.ConclusionOur study provides arguments for reducing chest radiographs in infants with bronchiolitis. For infants with clinical factors such as age less than three months, feeding difficulties, respiratory distress without hypoxia, isolated crackles or bronchitis rales, careful clinical follow-up should be provided instead of chest radiography

The ESRF's Extremely Brilliant Source - a 4th Generation Light Source

International audienceAfter 20 years of operation, the ESRF has embarked upon an extremely challenging project - the Extremely Brilliant Source (ESRF - EBS) . The goal of this project is to construct a 4th generation light source storage ring inside the existing 844m long tunnel. The EBS will increase the brilliance and coherence by a factor of 100 with respect to the present ESRF storage ring. A major challenge is to keep the present ring operating 24x7 while designing and pre-constructing all the elements of the new ring. This is the first time a 4th generation light source will be constructing inside an existing tunnel. This paper concentrates on the control system aspects. The control system is 100% TANGO based. The paper will list the main challenges of the new storage ring like the Hot Swap Powersupply, the new timing system, how reliable operation was maintained while modernizing the injector control system and preparing the new storage ring control system, the new historical database, and how extensive use was made of software simulators achieve this

NKX2-1 mutations leading to surfactant protein promoter dysregulation cause interstitial lung disease in "Brain-Lung-Thyroid Syndrome"

NKX2-1 (NK2 homeobox 1) is a critical regulator of transcription for the surfactant protein (SP)-B and -C genes (SFTPB and SFTPC, respectively). We identified and functionally characterized two new de novo NKX2-1 mutations c.493C<T (p.R165W) and c.786_787del2 (p.L263fs) in infants with closely similar severe interstitial lung disease (ILD), hypotonia, and congenital hypothyroidism. Functional analyses using A549 and HeLa cells revealed that NKX2-1-p.L263fs induced neither SFTPB nor SFTPC promoter activation and had a dominant negative effect on wild-type (WT) NKX2-1. In contrast,NKX2-1-p.R165W activated SFTPC, to a significantly greater extent than did WTNKX2-1, while SFTPB activation was only significantly reduced in HeLa cells. In accordance with our in vitro data, we found decreased amounts of SP-B and SP-C by western blot in bronchoalveolar lavage fluid (patient with p.L263fs) and features of altered surfactant protein metabolism on lung histology (patient with NKX2-1-p.R165W). In conclusion, ILD in patients with NKX2-1 mutations was associated with altered surfactant protein metabolism, and both gain and loss of function of the mutated NKX2-1 genes on surfactant protein promoters were associated with ILD in "Brain-Lung-Thyroid syndrome"

Effect of chest radiography on antibiotic use.

<p>Effect of chest radiography on antibiotic use.</p

Demographics of the study population (<i>n</i> = 697).

<p>Demographics of the study population (<i>n</i> = 697).</p

Age-related respiratory rate, from Liu et al. [10].

<p>Age-related respiratory rate, from Liu et al. <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0096189#pone.0096189-Liu1" target="_blank">[10]</a>.</p