Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age

BACKGROUND: Hirschsprung disease is a rare congenital disease typically requiring surgical treatment during childhood. Quality of life and social condition at adult age can be impaired by disease-specific sequelae. OBJECTIVE: This study aimed to assess the quality of life and social outcome of adult patients operated on for Hirschsprung disease during childhood. DESIGN: Patients operated on for Hirschsprung disease during childhood were identified and specific questionnaires were sent to them. SETTINGS: Data from 2 referral centers were used. PATIENTS: Patients who completed the questionnaires regarding quality of life and social condition were included. MAIN OUTCOME MEASURES: The Hirschsprung\u27s Disease and Anorectal Malformations Quality of Life disease-specific questionnaire (8 dimensions explored; each scored from 0 to 100 maximum score) and a sociodemographic questionnaire were sent to identified patients. Sociodemographic data were compared with those of the French general population. RESULTS: Thirty-four patients had Hirschsprung disease (men, 76%; mean age, 32 years) were included in the study. Mean total Hirschsprung\u27s Disease and Anorectal Malformations Quality of Life score was 611 of 800 (maximum score 800). The 2 most impaired dimensions were "physical symptoms" and "diarrhea" (62.9/100 and 73.6/100). Fecal continence was only marginally affected (mean score, 89/100). Patients with Hirschsprung disease achieved better educational levels than the French general population. Parental and marital status did not differ between the 2 groups. LIMITATIONS: This study had the limitations inherent to a retrospective study. CONCLUSION: The quality of life of adult patients with Hirschsprung disease sequelae is marginally impaired in this study. Despite the consequences of this congenital abnormality, the condition eventually achieved can be considered as satisfactory. See Video Abstract at http://links.lww.com/DCR/A917

Revision of the Cretaceous shark Protoxynotus (Chondrichthyes, Squaliformes) and early evolution of somniosid sharks

Due to the peculiar combination of dental features characteristic for different squaliform families, the position of the Late Cretaceous genera Protoxynotus and Paraphorosoides within Squaliformes has long been controversial. In this study, we revise these genera based on previously known fossil teeth and new dental material. The phylogenetic placement of Protoxynotus and Paraphorosoides among other extant and extinct squaliforms is discussed based on morphological characters combined with DNA sequence data of extant species. Our results suggest that Protoxynotus and Paraphorosoides should be included in the Somniosidae and that Paraphorosoides is a junior synonym of Protoxynotus. New dental material from the Campanian of Germany and the Maastrichtian of Austria enabled the description of a new species Protoxynotus mayrmelnhofi sp. nov. In addition, the evolution and origin of the characteristic squaliform tooth morphology are discussed, indicating that the elongated lower jaw teeth with erected cusp and distinct dignathic heterodonty of Protoxynotus represents a novel functional adaptation in its cutting-clutching type dentition among early squaliform sharks. Furthermore, the depositional environment of the tooth bearing horizons allows for an interpretation of the preferred habitat of this extinct dogfish shark, which exclusively occupied shelf environments of the Boreal- and northern Tethyan realms during the Late Cretaceous.publishedVersio

Piecewise Parabolic Method on a Local Stencil for Magnetized Supersonic Turbulence Simulation

Stable, accurate, divergence-free simulation of magnetized supersonic turbulence is a severe test of numerical MHD schemes and has been surprisingly difficult to achieve due to the range of flow conditions present. Here we present a new, higher order-accurate, low dissipation numerical method which requires no additional dissipation or local "fixes" for stable execution. We describe PPML, a local stencil variant of the popular PPM algorithm for solving the equations of compressible ideal magnetohydrodynamics. The principal difference between PPML and PPM is that cell interface states are evolved rather that reconstructed at every timestep, resulting in a compact stencil. Interface states are evolved using Riemann invariants containing all transverse derivative information. The conservation laws are updated in an unsplit fashion, making the scheme fully multidimensional. Divergence-free evolution of the magnetic field is maintained using the higher order-accurate constrained transport technique of Gardiner and Stone. The accuracy and stability of the scheme is documented against a bank of standard test problems drawn from the literature. The method is applied to numerical simulation of supersonic MHD turbulence, which is important for many problems in astrophysics, including star formation in dark molecular clouds. PPML accurately reproduces in three-dimensions a transition to turbulence in highly compressible isothermal gas in a molecular cloud model. The low dissipation and wide spectral bandwidth of this method make it an ideal candidate for direct turbulence simulations.Comment: 28 pages, 18 figure

Molecular Systematics of the Deep-Sea Hydrothermal Vent Endemic Brachyuran Family Bythograeidae: A Comparison of Three Bayesian Species Tree Methods

Brachyuran crabs of the family Bythograeidae are endemic to deep-sea hydrothermal vents and represent one of the most successful groups of macroinvertebrates that have colonized this extreme environment. Occurring worldwide, the family includes six genera (Allograea, Austinograea, Bythograea, Cyanagraea, Gandalfus, and Segonzacia) and fourteen formally described species. To investigate their evolutionary relationships, we conducted Maximum Likelihood and Bayesian molecular phylogenetic analyses, based on DNA sequences from fragments of three mitochondrial genes (16S rDNA, Cytochrome oxidase I, and Cytochrome b) and three nuclear genes (28S rDNA, the sodium–potassium ATPase a-subunit ‘NaK’, and Histone H3A). We employed traditional concatenated (i.e., supermatrix) phylogenetic methods, as well as three recently developed Bayesian multilocus methods aimed at inferring species trees from potentially discordant gene trees. We found strong support for two main clades within Bythograeidae: one comprising the members of the genus Bythograea; and the other comprising the remaining genera. Relationships within each of these two clades were partially resolved. We compare our results with an earlier hypothesis on the phylogenetic relationships among bythograeid genera based on morphology. We also discuss the biogeography of the family in the light of our results. Our species tree analyses reveal differences in how each of the three methods weighs conflicting phylogenetic signal from different gene partitions and how limits on the number of outgroup taxa may affect the results

One blind and three targeted searches for (sub)millisecond pulsars

We conducted one blind and three targeted searches for millisecond and submillisecond pulsars. The blind search was conducted within 3deg of the Galactic plane and at longitudes between 20 and 110deg. It takes 22073 pointings to cover this region, and 5487 different positions in the sky. The first targeted search was aimed at Galactic globular clusters, the second one at 24 bright polarized and pointlike radiosources with steep spectra, and the third at 65 faint polarized and pointlike radiosources. The observations were conducted at the large radiotelescope of Nancay Observatory, at a frequency near 1400 MHz. Two successive backends were used, first a VLBI S2 system, second a digital acquisition board and a PC with large storage capacity sampling the signal at 50 Mb/s on one bit, over a 24-MHz band and in one polarization. The bandwidth of acquisition of the second backend was later increased to 48 MHz and the sampling rate to 100 Mb/s. The survey used the three successive setups, with respective sensitivities of 3.5, 2.2, and 1.7 mJy. The targeted-search data were obtained with the third setup and reduced with a method based on the Hough transform, yielding a sensitivity of 0.9 mJy. The processing of the data was done in slightly differed time by soft-correlation in all cases. No new short-period millisecond pulsars were discovered in the different searches. To better understand the null result of the blind survey, we estimate the probability of detecting one or more short-period pulsars among a given Galactic population of synthetic pulsars with our setup: 25% for the actual incomplete survey and 79% if we had completed the whole survey with a uniform nominal sensitivity of 1.7 mJy. The alternative of surveying a smaller, presumably more densely populated, region with a higher sensitivity would have a low return and would be impractical at a transit instrument. (abridged)Comment: accepted for publication in Astronomy & Astrophysic

On Building Maps of Web Pages with a Cellular Automaton

Abstract. We present in this paper a clustering algorithm which is based on a cellular automaton and which aims at displaying a map of web pages. We describe the main principles of methods that build such maps, and the main principles of cellular automata. We show how these principles can be applied to the problem of web pages clustering: the cells, which are organized in a 2D grid, can be either empty or may contain a page. The local transition function of cells favors the creation of groups of similar states (web pages) in neighbouring cells. We then present the visual results obtained with our method on standard data as well as on sets of documents. These documents are thus organized into a visual map which eases the browsing of these pages

TEMPO2, a new pulsar timing package. I: Overview

Contemporary pulsar timing experiments have reached a sensitivity level where systematic errors introduced by existing analysis procedures are limiting the achievable science. We have developed tempo2, a new pulsar timing package that contains propagation and other relevant effects implemented at the 1ns level of precision (a factor of ~100 more precise than previously obtainable). In contrast with earlier timing packages, tempo2 is compliant with the general relativistic framework of the IAU 1991 and 2000 resolutions and hence uses the International Celestial Reference System, Barycentric Coordinate Time and up-to-date precession, nutation and polar motion models. Tempo2 provides a generic and extensible set of tools to aid in the analysis and visualisation of pulsar timing data. We provide an overview of the timing model, its accuracy and differences relative to earlier work. We also present a new scheme for predictive use of the timing model that removes existing processing artifacts by properly modelling the frequency dependence of pulse phase.Comment: Accepted by MNRA

European regulatory agenices should employ full time statisticians

No abstract available