An autosomal recessive leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa maps to chromosome 17q24.2-25.3

Background Single-gene disorders related to ischemic stroke seem to be an important cause of stroke in young patients without known risk factors. To identify new genes responsible of such diseases, we studied a consanguineous Moroccan family with three affected individuals displaying hereditary leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa that appears to segregate in autosomal recessive pattern. Methods All family members underwent neurological and radiological examinations. A genome wide search was conducted in this family using the ABI PRISM linkage mapping set version 2.5 from Applied Biosystems. Six candidate genes within the region linked to the disease were screened for mutations by direct sequencing. Results Evidence of linkage was obtained on chromosome 17q24.2-25.3. Analysis of recombination events and LOD score calculation suggests linkage of the responsible gene in a genetic interval of 11 Mb located between D17S789 and D17S1806 with a maximal multipoint LOD score of 2.90. Sequencing of seven candidate genes in this locus, ATP5H, FDXR, SLC25A19, MCT8, CYGB, KCNJ16 and GRIN2C, identified three missense mutations in the FDXR gene which were also found in a homozygous state in three healthy controls, suggesting that these variants are not disease-causing mutations in the family. Conclusion A novel locus for leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa has been mapped to chromosome 17q24.2-25.3 in a consanguineous Moroccan family

Les tumeurs péri-ampullaires.

Introduction: Le but de ce travail est d’évaluer notre expérience dans la prise en charge des patients porteurs de cancers péri-ampullaires (cancer de la tête du pancréas exclu) à propos de 28 cas et nous éclairer par celles de la littérature médicale. Matériel et méthodes : 28 cas consécutifs de cancers péri-ampullaires sont inclus dans cette étude et chez qui l’examen histopathologique définitif avait confirmé l’origine péri-ampullaire de la tumeur. Tous les patients ont été diagnostiqués et traités au service de la chirurgie « C » à l’hôpital Ibn Sina de Rabat entre 2000 et 2009. Nous avons noté les données démographiques des patients, leurs antécédents pathologiques, les symptômes, ainsi que les paramètres péri-opératoires : résécabilité, la morbidité et la mortalité opératoires. Résultats : Notre étude a inclue vingt-huit patients, l’âge moyen est de 63,4 ans (extrêmes entre 46 et 81 ans). Le sexe ratio est de H/F=2. Un traitement palliatif est indiqué chez 17 patients tandis que 11 patients ont subit une duodénopancréatectomie céphalique soit un taux de résécabilité de 40%. La mortalité et la morbidité opératoires étaient respectivement de 9% et de 28,5%. Conclusion : le taux de résécabilité était significativement diminué en comparaison avec les données publiées (entre 52 et 92%), contrairement aux taux de la morbidité et la mortalité opératoires qui restaient compatibles avec la littérature (respectivement entre 16,4 et 58% et entre 0,4 et 10%)

Un système de vérification de signature manuscrite en ligne basé sur la décomposition modale empirique

La signature manuscrite est une modalité biométrique utilisée pour l’identification d' une personne. Ce travail s'inscrit dans le cadre de l’élaboration d'un système de vérification de signatures manuscrites en ligne. Nous modélisons la signature manuscrite par une approche analytique basée sur la décomposition modale empirique (EMD). Le système proposé est muni d'un module d’apprentissage et d'une base de signatures. Le protocole d'évaluation mis en oeuvre montre l'intérêt de la méthode adoptée et permet d'obtenir un taux de reconnaissance allant jusqu'à 98%, avec un taux de fausse acceptation(FAR) de 3.33%, un taux de faux rejet (FRR) de 4,67% et un taux d'erreur égal (EER) à 1,83%.Mot clés: signatures en ligne, vérification, biométrie, approche analytique. Online handwritten signature verification system based on the empirical mode decompositionThe handwritten signature is a biometric method used to verify the identity of a person. This work deals with an online handwritten signature verification system. We model the handwritten signature by an analytical approach based on the Empirical Mode Decomposition (EMD). The organized system is provided with a training module and a base of signatures. The implemented evaluation protocol points out the interest of the adopted method and allows obtaining a recognition rate going up to 98%, with a false acceptance rate (FAR) of 3.33%, a false rejection rate of 4.67 % and an equal error rate (EER) of 1.83 %.Keywords: online signature, verification, biometrics, analytical approach

Topology Optimization and Design Guidelines of Sub-Stiffened Panels in Aerospace Applications

The present work is a proposed optimization model of grid sub-stiffened panels for aerospace applications, having a better knowledge about the stability analysis of conventional grid sub-stiffened panels against loading and different mode of failure occurring lead us thinking about the topology optimization. In a first stage, we will analyse the stability performance of such structures against specific mechanical loading and conclude some techniques to introduce a topology optimization through PSO algorithm of some design variables. The aim is the better exploitation of the sub-stiffened panel along the plan so that the area less stressed due the charging will be extruded in mass for the enhancement of the concept of looking for better performance with a structure already saved in weight

Topology Optimization and Design Guidelines of Sub-Stiffened Panels in Aerospace Applications

The present work is a proposed optimization model of grid sub-stiffened panels for aerospace applications, having a better knowledge about the stability analysis of conventional grid sub-stiffened panels against loading and different mode of failure occurring lead us thinking about the topology optimization. In a first stage, we will analyse the stability performance of such structures against specific mechanical loading and conclude some techniques to introduce a topology optimization through PSO algorithm of some design variables. The aim is the better exploitation of the sub-stiffened panel along the plan so that the area less stressed due the charging will be extruded in mass for the enhancement of the concept of looking for better performance with a structure already saved in weight

Lingual metastasis as an initial presentation of renal cell carcinoma: a case report

Abstract Background Renal cell carcinoma is the third most common tumor that metastasizes to the head and neck, after breast and lung carcinomas. Tongue metastasis as an initial presentation of renal cell carcinoma is extremely rare, and very few cases have been reported. The prognosis is poor. We present a rare case of metastatic renal cell carcinoma that initially presented as a tongue lesion. Case presentation We report the case of a 55-year-old white man who presented with a large exophytic lesion on his tongue. A biopsy was taken, and pathologic examination showed a poorly differentiated carcinoma including a sarcomatoid component. Subtotal glossectomy with neck dissection were planned, but a positron emission tomographic-computed tomography scan showed a left kidney mass. Immunohistochemical evaluation of the tongue lesion was performed, and it was compatible with metastasis from primary renal cell carcinoma. The biopsy of the renal lesion showed a high-grade unclassified renal cell carcinoma. Although our patient underwent systemic therapy, he died of hemorrhagic complications 3 months after the initiation of therapy. Conclusion Tongue lesions require a complete assessment to distinguish a metastasis from a primary cancer in order to give the appropriate treatment

Évaluation de la salinité des eaux souterraines utilisées en irrigation et risques de dégradation des sols : exemple de la plaine de Meskiana (Nord-Est Algérien)

In the plain of Meskiana (North-East of Algeria), under a semi-arid climate, the use of irrigation is inevitable for most crops. Groundwater is therefore being increasingly used.A hydrochemical study was carried out; she has focused on the major elements in the water of the aquifer. Four sampling campaigns were realized over two years (2007-2008). The 39 wells that were analyzed are distributed throughout the plain.The analyses reveal a large diversity of chemical compositions, the samples being divided between four facies: calcium bicarbonate, calcium chloride, sodium chloride and calcium sulfate. The data allowed the mapping of suitable water for irrigation. Water mineralization, which is generally high, carries a risk of soil salinization

Conception d’un SIG pour l’évaluation de l’impact des activités anthropiques sur la qualité des eaux superficielles de la ville de Meknès (Maroc)

L’utilisation des sols liée aux activités urbaines, agricoles et industrielles a contribué, au cours des dernières décennies, à augmenter le potentiel de contamination des eaux de surface et, dans certains cas, à une détérioration réelle de la qualité de cette ressource. De ce fait, une gestion rationnelle de la ressource est nécessaire en vue de la protéger. Dès lors, l’exploitation des capacités de SIG en termes d’analyse et de cartographie s’est révélée pertinente en vue d’en faire un outil d’aide à la décision pour une gestion de la qualité des ressources en eau. Une approche basée sur le couplage d’un SIG avec un système d’évaluation de la qualité des eaux (SEQ-eau) a été développée et testée au niveau des eaux superficielles de la ville de Meknès. La démarche adoptée repose sur le traitement des résultats d’analyses microbiologiques et physicochimiques effectuées au niveau de l’oued Boufekrane par le SEQ-Eau. Une représentation cartographique de la zone d’étude a été réalisée avec élaboration des cartes de qualité pour chaque altération (ensemble de paramètres ayant un même effet sur le milieu aquatique) : microorganismes; phosphore total; PH; matières organiques et oxydables; matières en suspension. Les résultats obtenus ont permis d’élaborer une carte de qualité globale et d’évaluer la pollution organique tout le long de l’oued Boufekrane avec des indicateurs de pollution comme l’oxydabilité et la demande chimique en oxygène. Ces résultats préliminaires montrent que le couplage SIG-système d’évaluation de la qualité des eaux développé avec l’ambition de servir d’outil d’aide à la décision pour une gestion intégrée de la qualité des eaux a un apport potentiel considérable pour l’étude des ressources en eau

Assessment of the Coastal Marchica Watershed through Ecotoxicological Indices of Trace Elements in Superficial Sediments

This study analyses the seasonal distribution in the superficial sediments of the Marchica watershed of trace and major elements, grain size, and total organic carbon. Samples were taken during the wet and dry seasons of 2018 in the mainstream valleys carrying the discharges to the Marchica Lagoon. Ecotoxicological indices (Enrichment Factor, Contamination Factor, Pollution-Load Index) and sedimen-quality guidelines were used to evaluate the ecotoxicological risk. The results revealed during both wet and dry seasons contamination by lead in all stream valleys. The northwest stream valley, characterized by some industrial activities, and the stream valley crossing the abandoned mine were the most polluted by zinc and copper. Minimal pollution for Cr, Co, and Ba was found during both the wet and dry seasons. Despite the restoration actions achieved around the Ramsar site of Marchica lagoon during the last decades, the watershed continues to have a negative impact on this ecosystem. Therefore, an environmental management strategy isnecessary, taking into account the rehabilitation of the abandoned mining sites, the rational use of pesticides and fertilizers in agriculture, and the environmental responsibility of industrial companies, especially those installed before the implementation of the national law on environmental impact studies