SYNDROME DE LA FENTE MEDIANE A PROPOS D’UN CAS

The median cleft syndrome is a complex facial dysmorphism. it may be associated with other malformations incompatible to life. Prenatal diagnosis is of major interest to prepare parents and to accept the child's disability. Through an observation collected in pediatric ophthalmology department, we present the characteristics of this disease. This is an infant of 5 months, the sole of his family,from a non-consanguineous marriage and consults for a facial dysmorphism. The ophthalmology test note a cyst dermoid bilateral interest blade, the conjunctiva, 2 / 3 of the cornea uncomfortable viewing eyes. The CT cranio-orbital showed a hypoplastic orbits with cerebral atrophy. The heart-ultrasound showed a perished ventricular , restrictive intercomminication. The caryotype study was normal. The ophthalmological treatement consisted to a surgical cure of both dermoid cysts. We discuss the clinical et paraclinical, etiopathogenic aspects of the slots medians and we stress the importance of antenatal diagnosis of this condition.Le syndrome de la fente médiane est une dysmorphie faciale complexe pouvant rentrer dans un cadre polymalformatif incompatible avec la vie. Le diagnostic anténatal revêt un intérêt majeur pour préparer les parents et l’entourage à l’accueil de l’enfant et son handicap. À travers une observation colligée au service d’ophtalmologie pédiatrique, nous présentons les caractéristiques de cette affection. Il s’agit d’un nourrisson de 5 mois unique de sa famille, issu d’un mariage non consanguin et consultant pour une dysmorphie faciale. L’examen ophtalmologique a noté un kyste dermoïde bilatérale intéressant le limbe, la conjonctive, les 2/3 de la cornée gênant la visualisation des yeux. Le bilan malformatif a montré à la TDM cranio-orbitaire une hypoplasie des orbites avec atrophie cérébrale. L’écho-coeur a montré une communication inter ventriculaire péri membraneuse restrictive. L’étude du caryotype était normale. La prise en charge ophtalmologique a consisté en une cure chirurgicale des deux kystes dermoïdes. Nous discutons les aspects cliniques, para cliniques, étiopathogéniques des fentes médianes et nous insistons sur l’importance du diagnostic anténatale de cette affection

Manifestations ophtalmologiques du lupus chez l’enfant

Le lupus  à début pédiatrique est une maladie souvent sévère, dont la morbidité à long terme est importante. Ses manifestations ophtalmologiques sont variées et peuvent être révélatrices.  Elles sont  représentées par les nodules cotonneux avec ou sans hémorragies intra rétiniennes. Les auteurs rapportent l'observation d'un enfant  ayant un lupus érythémateux disséminé qui a présenté une vascularite  rétinienne

K+ channel openers restore verapamil-inhibited lung fluid resolution and transepithelial ion transport

<p>Abstract</p> <p>Background</p> <p>Lung epithelial Na⁺channels (ENaC) are regulated by cell Ca²⁺signal, which may contribute to calcium antagonist-induced noncardiogenic lung edema. Although K⁺channel modulators regulate ENaC activity in normal lungs, the therapeutical relevance and the underlying mechanisms have not been completely explored. We hypothesized that K⁺channel openers may restore calcium channel blocker-inhibited alveolar fluid clearance (AFC) by up-regulating both apical and basolateral ion transport.</p> <p>Methods</p> <p>Verapamil-induced depression of heterologously expressed human αβγ ENaC in <it>Xenopus </it>oocytes, apical and basolateral ion transport in monolayers of human lung epithelial cells (H441), and <it>in vivo </it>alveolar fluid clearance were measured, respectively, using the two-electrode voltage clamp, Ussing chamber, and BSA protein assays. Ca²⁺signal in H441 cells was analyzed using Fluo 4AM.</p> <p>Results</p> <p>The rate of <it>in vivo </it>AFC was reduced significantly (40.6 ± 6.3% of control, <it>P </it>< 0.05, n = 12) in mice intratracheally administrated verapamil. K_Ca3.1(1-EBIO) and K_ATP(minoxidil) channel openers significantly recovered AFC. In addition to short-circuit current (Isc) in intact H441 monolayers, both apical and basolateral Isc levels were reduced by verapamil in permeabilized monolayers. Moreover, verapamil significantly altered Ca²⁺signal evoked by ionomycin in H441 cells. Depletion of cytosolic Ca²⁺in αβγ ENaC-expressing oocytes completely abolished verapamil-induced inhibition. Intriguingly, K_V(pyrithione-Na), K _Ca3.1(1-EBIO), and K_ATP(minoxidil) channel openers almost completely restored the verapamil-induced decrease in Isc levels by diversely up-regulating apical and basolateral Na⁺and K⁺transport pathways.</p> <p>Conclusions</p> <p>Our observations demonstrate that K⁺channel openers are capable of rescuing reduced vectorial Na⁺transport across lung epithelial cells with impaired Ca²⁺signal.</p

Lipopolysaccharide modifies amiloride-sensitive Na+ transport processes across human airway cells: role of mitogen-activated protein kinases ERK 1/2 and 5

Bacterial lipopolysaccharides (LPS) are potent inducers of proinflammatory signaling pathways via the activation of nuclear factor-kappa B (NF-κB) and mitogen-activated protein kinase (MAPK), causing changes in the processes that control lung fluid homeostasis and contributing to the pathogenesis of lung disease. In human H441 airway epithelial cells, incubation of cells with 15 µg ml−1 LPS caused a significant reduction in amiloride-sensitive Isc from 15 ± 2 to 8 ± 2 µA cm−2 (p = 0.01, n = 13) and a shift in IC50 amiloride of currents from 6.8 × 10−7 to 6.4 × 10−6 M. This effect was associated with a decrease in the activity of 5 pS, highly Na+ selective, amiloride-sensitive <1 µM channels (HSC) and an increase in the activity of ∼18 pS, nonselective, amiloride-sensitive >10 µM cation channels (NSC) in the apical membrane. LPS decreased αENaC mRNA and protein abundance, inferring that LPS inhibited αENaC gene expression. This correlated with the decrease in HSC activity, indicating that these channels, but not NSCs, were comprised of at least αENaC protein. LPS increased NF-κB DNA binding activity and phosphorylation of extracellular signal-related kinase (ERK)1/2, but decreased phosphorylation of ERK5 in H441 cells. Pretreatment of monolayers with PD98059 (20 µM) inhibited ERK1/2 phosphorylation, promoted phosphorylation of ERK5, increased αENaC protein abundance, and reversed the effect of LPS on Isc and the shift in amiloride sensitivity. Inhibitors of NF-κB activation were without effect. Taken together, our data indicate that LPS acts via ERK signaling pathways to decrease αENaC transcription, reducing HSC/ENaC channel abundance, activity, and transepithelial Na+ transport in H441 airway epithelial cells

Combining farmers' decision rules and landscape stochastic regularities for landscape modelling

International audienceLandscape spatial organization (LSO) strongly impacts many environmental issues. Modelling agricultural landscapes and describing meaningful landscape patterns are thus regarded as key-issues for designing sustainable landscapes. Agricultural landscapes are mostly designed by farmers. Their decisions dealing with crop choices and crop allocation to land can be generic and result in landscape regularities, which determine LSO. This paper comes within the emerging discipline called "landscape agronomy", aiming at studying the organization of farming practices at the landscape scale. We here aim at articulating the farm and the landscape scales for landscape modelling. To do so, we develop an original approach consisting in the combination of two methods used separately so far: the identification of explicit farmer decision rules through on-farm surveys methods and the identification of landscape stochastic regularities through data-mining. We applied this approach to the Niort plain landscape in France. Results show that generic farmer decision rules dealing with sunflower or maize area and location within landscapes are consistent with spatiotemporal regularities identified at the landscape scale. It results in a segmentation of the landscape, based on both its spatial and temporal organization and partly explained by generic farmer decision rules. This consistency between results points out that the two modelling methods aid one another for land-use modelling at landscape scale and for understanding the driving forces of its spatial organization. Despite some remaining challenges, our study in landscape agronomy accounts for both spatial and temporal dimensions of crop allocation: it allows the drawing of new spatial patterns coherent with land-use dynamics at the landscape scale, which improves the links to the scale of ecological processes and therefore contributes to landscape ecology.L'organisation du paysage influe sur les problèmes environnementaux. Modéliser les paysages pour les décrire à l'aide de formes significatives est une étage clé. Les paysages agricoles sont principalement construits par les agriculteurs dont les décision d'assolement peuvent être génériques et déterminer des régularités dans l'organisation du paysage. Cet article contribue à l'agronomie des paysage qui est une discipline émergente. Nous cherchons à articuler les échelles du paysage et de l'exploitation agricole en développant deux méthodes : l'une consiste à identifier les décisions des agriculteurs par le bais d'enquêtes, l'autre consiste à retrouver des régularités stochastiques dans le paysage par le bais de fouille de données. Nous avons appliqué cette approche au paysage de la plaine de Niort en France. Les résultats montrent que les décisions des agriculteurs en matière de tournesol et maïs sont génériques et ont des effets sur le paysages que des méthodes de fouille de données révèlent et quantifient