La maladie de Fanconi: à propos d’une nouvelle observation

La maladie de Fanconi ou l'anémie de Fanconi (AF) est une maladie  génétique rare à transmission autosomique récessive. Elle est marquée parune hétérogénéité phénotypique. Certains symptômes et notamment la triade classique faite d'une petite taille, d'un syndrome malformatif varié et parfois discret et d'une insuffisance médullaire d'apparition précoce, doivent faire évoquer le diagnostic. Nous rapportons le cas d'un enfant âgé de sept ans, suivi et traité pour une luxation congénitale des hanches, qui présentait une pancytopénie avec à l'examen clinique on note un faciès dysmorphique triangulaire, une duplication du pouce droit, une surélévation de l'épaule gauche et un retard staturo-pondéral

Analyse de la qualité physico-chimique des eaux souterraines de la communauté des Mzamza, au voisinage des eaux usées

Analyze of the physicochemical quality of Mzamza’s communityof ground waters, around waste waters In the Mzamza’s community, characterized by an important agricultural potentiality especially in the perimeters irrigated by domestic and industrial water, ground waters are prone to several possibilities of contamination. However in this zone, the ground waters are considered as an important resource used to drink water and irrigation. The objective of this study is, on the one hand, to try to evaluate the impact of the wastes water’s re-use on the physicochemical quality of wells’water in the zone of study and, on the other hand, to determine its quality refering to the standards of potability or irrigation. The results obtained showed that the concentrations of the elements considered (principal pollutants and indicators of pollution) are high in waste waters and ground waters.The ratio of adsorption of sodium (SAR) enabled us to qualifie groundwaters intended to the irrigation, and the causes of salinisation of the soil when using it in this domain.Several determining factors were highlighted in the contamination of ground waters by these pollutants, namely: the contribution of nitrogenized fertilizers, the nature of soil, the lithology, the permeability of the aquifer and the outdistances well compared to the sources of pollution which are waste waters

Grande tache pigmentée pileuse révélant une forme familiale de la maladie de Von Recklinghausen

La neurofibromatose de type 1 (NF1) ou maladie de Von Recklinghausen appartient au groupe de maladies appelées phacomatoses. C'est une affection autosomique dominante relativement rare. La NF1 est caractérisée par une extrême variabilité clinique que l'on retrouve également au sein d'une même famille. Le tableau clinique de la NF1 associe, le plus souvent, de multiples taches café au lait, des lentigines axillaires ou inguinales, des neurofibromes cutanés et des nodules de Lisch. Les difficultés d'apprentissage sont fréquentes et peuvent être graves dans certaines formes cliniques. Il est important de détecter précocement les neurofibromes plexiformes, les gliomes intracérébraux, les tumeurs des gaines nerveuses, les anomalies vasculaires et les dysplasies osseuses. L'évolution est imprévisible ce qui rend le pronostic incertain par une éventuelle survenue dedégénérescence malignes. Nous rapportons ici l'observation d'une grande tache cutanée pigmentée pilleuse de découverte fortuite qui nous a révélé deux cas familiaux de neurofibromatose 1 d'expression différente

Integrated Models of Ultrasonic Examination for NDT Expertise

For several years, the French Atomic Energy Commission (CEA) has developed a system calledCIVA for multiple-technique NDE data acquisition and processing [1]. Modeling tools for ultrasonic non-destructive testing have been developed and implemented within this system allowing direct comparison between measured and predicted results. These models are not only devoted to laboratory uses but also must be usable by ultrasonic operators without special training in simulation techniques. Therefore, emphasis has been on finding the best compromise between as accurate as possible quantitative predictions and ease, simplicity and speed, crucial requirements in the industrial context

Traitement Expérimental Par Coagulation Floculation Des Effluents Brutes D’une Industrie Aéronautique À Casablanca (Maroc)

Les effluents de l’industrie de traitement de surface présentent souvent une forte charge en produits chimiques très toxiques qui doivent être traités conformément à une réglementation de plus en plus stricte. Dans ce travail, nous avons effectué une caractérisation des effluents liquides d’une unité de traitement de surface au Maroc qui a révélé une forte pollution colloïdale (Turbidité=131 NTU), une charge en matière oxydable élevée (DCO = 960 mg/l) et en éléments métalliques (Fer, cuivre, zinc, nickel, aluminium, chrome et cyanure). L’objectif de cette étude expérimentale consiste à appliquer un traitement physico-chique par coagulation-floculation à ces eaux uses industrielles toxiques en vue de les conformer aux normes de rejet dans le réseau d’assainissement local. En effet, ce traitement, précédé d’un ajustement du pH, une oxydation des cyanures et d’une réduction du chrome hexavalent, a abouti à une réduction très satisfaisante de la toxicité de ces effluents avec des taux d’abattement de 94% pour la DCO et jusqu'à 99 % pour la turbidité et charge métallique. Effluents from the surface treatment industry often have a high load o f highly toxic chemicals that need to be treated in accordance with increasing ly stringent regulations. In this work, we performed a characterization of liqu id effluents from a surface treatment unit in Morocco that revealed high collo idal pollution (Turbidity=131 NTU), high stainless material (COD=960 mg/l ) and metal elements (Iron, copper, zinc, nickel, aluminum, chromium and cy anide). The objective of this experimental study is to apply a physico-chemical treatment by coagulation-flocculation to these toxic industrial wastewater in order to comply with the discharge standards in the local sewage system. This treatment, preceded by pH adjustment, cyanide oxidation and reduction of hexavalent chromium, resulted in a very satisfactory reduction in toxicity of these effluents with abatement rates of 94% for COD and up to 99% for turbidity and metal load

Toxicological evaluation of Thymelaea hirsuta and protective effect against CCl4-induced hepatic injury in rats

The aerial part of Thymelaea hirsuta (TH) is used as decoction in the treatment of different pathologies in folk medicine in Morocco. The present investigation was carried out to evaluate the potential of toxicity of an aqueous extract of TH and its hepatoprotective activity against CCl4-induced hepatic injury in rats. In the acute study, there were no signs of toxicity observed after oral administration of single dose of the extract (5g/kg body weight). In the sub-chronic dose study, (given by gavages (4 ml/kg) to Wistar rats daily at: 0.0 g/kg, 0.5g/kg, 1 g/kg or 2 g/kg body weight for 4 weeks) no variation on biochemical parameters; serum glucose, creatinine and urea levels was observed compared to the control group. However, the serum levels of alanine aminotransferase (ALAT) and aspartate aminotransferase (ASAT) were markedly decreased (P < 0.05 and P<0.001 respectively) as compared to the controls. There were no significant differences in red blood cells count (p<0.05), in Hemoglobin and Hematocrit serum levels but a significant increase in white blood cells count (p<0.001) and in platelets (p< 0.05) for all doses tested. Histopathological examination of the liver, spleen and kidneys tissues at the end of the study showed normal architecture. The elevated serum enzymatic activities of ALAT and ASAT, due to carbon tetrachloride treatment (1 ml/kg CCl4 every 72 h during 15 days) were restored towards normalization by the plant extracts. The biochemical observations were supplemented with histopathological examination of liver sections. The results indicate that this plant possesses no toxicity effects but hepatoprotective properties.Keywords: Thymelaea hirsuta, Wistar rats, Toxicity, Histopathology, hepatoprotective action, Folk medicin

Expression and activation of the oxytocin receptor in airway smooth muscle cells: Regulation by TNFα and IL-13

<p>Abstract</p> <p>Background</p> <p>During pregnancy asthma may remain stable, improve or worsen. The factors underlying the deleterious effect of pregnancy on asthma remain unknown. Oxytocin is a neurohypophyseal protein that regulates a number of central and peripheral responses such as uterine contractions and milk ejection. Additional evidence suggests that oxytocin regulates inflammatory processes in other tissues given the ubiquitous expression of the oxytocin receptor. The purpose of this study was to define the role of oxytocin in modulating human airway smooth muscle (HASMCs) function in the presence and absence of IL-13 and TNFα, cytokines known to be important in asthma.</p> <p>Method</p> <p>Expression of oxytocin receptor in cultured HASMCs was performed by real time PCR and flow cytomery assays. Responses to oxytocin was assessed by fluorimetry to detect calcium signals while isolated tracheal rings and precision cut lung slices (PCLS) were used to measure contractile responses. Finally, ELISA was used to compare oxytocin levels in the bronchoalveloar lavage (BAL) samples from healthy subjects and those with asthma.</p> <p>Results</p> <p>PCR analysis demonstrates that OXTR is expressed in HASMCs under basal conditions and that both interleukin (IL)-13 and tumor necrosis factor (TNFα) stimulate a time-dependent increase in OXTR expression at 6 and 18 hr. Additionally, oxytocin increases cytosolic calcium levels in fura-2-loaded HASMCs that were enhanced in cells treated for 24 hr with IL-13. Interestingly, TNFα had little effect on oxytocin-induced calcium response despite increasing receptor expression. Using isolated murine tracheal rings and PCLS, oxytocin also promoted force generation and airway narrowing. Further, oxytocin levels are detectable in bronchoalveolar lavage (BAL) fluid derived from healthy subjects as well as from those with asthma.</p> <p>Conclusion</p> <p>Taken together, we show that cytokines modulate the expression of functional oxytocin receptors in HASMCs suggesting a potential role for inflammation-induced changes in oxytocin receptor signaling in the regulation of airway hyper-responsiveness in asthma.</p

Non-genomic Effects of Glucocorticoids: An Updated View

Glucocorticoid (GC) anti-inflammatory effects generally require a prolonged onset of action and involve genomic processes. Because of the rapidity of some of the GC effects, however, the concept that non-genomic actions may contribute to GC mechanisms of action has arisen. While the mechanisms have not been completely elucidated, the non-genomic effects may play a role in the management of inflammatory diseases. For instance, we recently reported that GCs ‘rapidly’ enhanced the effects of bronchodilators, agents used in the treatment of allergic asthma. In this review article, we discuss (i) the non-genomic effects of GCs on pathways relevant to the pathogenesis of inflammatory diseases and (ii) the putative role of the membrane GC receptor. Since GC side effects are often considered to be generated through its genomic actions, understanding GC non-genomic effects will help design GCs with a better therapeutic index