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The Liane River is a small costal river, famous for its floods, which can affect the city of Boulogne-sur-Mer. Due to the complexity of land cover and hydrologic processes, a black-box non-linear modelling was chosen using neural networks. The multilayer perceptron model, known for its property of universal approximation is thus chosen. Four models were designed, each one for one forecasting horizon using rainfall forecasts: 24h, 12h, 6h, 3h. The desired output of the model is original: it represents the maximal value of the water level respectively 24h, 12h, 6h, 3h ahead. Working with best forecasts of rain (the observed ones during the event in the past), on the major flood of the database in test set, the model provides excellent forecasts. Nash criteria calculated for the four lead times are 0.98 (3h), 0.97 (6h), 0.91 (12h), 0.89 (24h). Designed models were thus estimated as efficient enough to be implemented in a specific tool devoted to real time operational use. The software tool is described hereafter: designed in Java, it presents a friendly interface allowing applying various scenarios of future rainfalls, and a graphical visualization of the predicted maximum water levels and their associated real time observed values.

Jusqu’en 2012, le prix d’architecture de l’Équerre d’argent a mis sur le devant de la scène des œuvres à l’originalité affirmée mais dont la notoriété n’était pas avérée, notamment auprès du grand public. Rompant avec cette tradition, la précieuse Équerre a été décernée en 2013 au musée du Louvre à Lens des Japonais Kazuyo Sejima et Ryue Nishizawa (agence Sanaa). Tant qu’à célébrer une réalisation emblématique de cette année-là, le MuCem de Rudy Ricciotti à Marseille, pour la force de son concept et sa puissance à défrayer la chronique, aurait mieux convenu.

It is generally accepted that human β -melanocyte-stimulating hormone (hβ MSH) does not normally exist in humans but was merely an artifactually generated 22-amino acid peptide corresponding to a lipotropin (LPH) fragment (residues 35-56). We examined whether the shorter 18-amino acid peptide hβ MSH-(5-22) could be detected in some human tissues. Normal human pituitaries and hypothalami as well as corticotropin-secreting pituitary and nonpituitary tumors were extracted and chromatographed on Sephadex G-50, and the fractions were measured with two radioimmunoassays using either a COOH-terminal human γ LPH (hγ LPH) antiserum that recognized equally hγ LPH, hβ MSH, and hβ MSH-(5-22) or a mid-portion hγ LPH antiserum that recognized hγ LPH and hβ MSH but not hβ MSH-(5-22). Normal pituitaries and pituitary tumors contained a single immunoreactive material coeluting with hγ LPH. The hypothalami and the nonpituitary tumors all contained hγ LPH and a smaller molecular weight material that was only detected in the COOH-terminal hγ LPH radioimmunoassay; its elution volume (Ve/V, 0.75) was identical to that of hβ MSH-(5-22) but different from that of hβ MSH (Ve/V, 0.60); on reversed-phase HPLC, it coeluted with synthetic hβ MSH-(5-22) with a retention time different from that of hβ MSH. It is concluded that hβ MSH-(5-22) that corresponds to the 18-amino acid peptide hβ LPH-(39-56), flanked by two pairs of basic amino acids within the hβ LPH molecule, is a normal maturation product of proopiomelanocortin in human nonpituitary tissues.

Holt-Oram syndrome (HOS) is an autosomal dominant condition characterised by the association of congenital heart defect (CHD), with or without rhythm disturbances and radial defects, due to TBX5 variants. The diagnosis is challenged by the variability of expression and the large phenotypic overlap with other conditions, like Okihiro syndrome, TAR syndrome or Fanconi disease. We retrospectively reviewed 212 patients referred for suspicion of HOS between 2002 and 2014, who underwent TBX5 screening. A TBX5 variant has been identified in 78 patients, representing the largest molecular series ever described. In the cohort, 61 met the previously described diagnostic criteria and 17 have been considered with an uncertain HOS diagnosis. A CHD was present in 91% of the patients with a TBX5 variant, atrial septal defects being the most common (61.5%). The genotype–phenotype study highlights the importance of some critical features in HOS: the septal characteristic of the CHD, the bilateral and asymmetric characteristics of the radial defect and the presence of shoulder or elbow mobility defect. Besides, 21 patients presented with an overlapping condition. Among them, 13 had a typical HOS presentation. We discuss the strategies that could be adopted to improve the molecular delineation of the remaining typical patients.