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

GPS observables in general relativity

I present a complete set of gauge invariant observables, in the context of general relativity coupled with a minimal amount of realistic matter (four particles). These observables have a straightforward and realistic physical interpretation. In fact, the technology to measure them is realized by the Global Positioning System: they are defined by the physical reference system determined by GPS readings. The components of the metric tensor in this physical reference system are gauge invariant quantities and, remarkably, their evolution equations are local.Comment: 6 pages, 1 figure, references adde

"Ethnic disparities in the prevalence of Molar-Incisor-Hypomineralisation (MIH) and caries among 6-12-year-old children in Catalonia, Spain"

AIM: To study the prevalence of MIH and caries in 6- and 12-year-old schoolchildren and their association with ethnic disparities and other relevant factors. BACKGROUND: In recent years, there has been uneven improvement in school children's oral health, highlighting inequalities in access to dental care and health outcomes, particularly among ethnic minorities. The most prevalent oral disease in childhood, caries, is preventable, as its risk factors are well known. However, MIH, a common condition affecting the enamel of permanent incisors and/or molars, has no established aetiology or preventive measures. METHODS: A cross-sectional study among schoolchildren was conducted in 725 children from Masnou (Barcelona, 2013) and in 577 children from Sant Andreu de Llavaneres (Barcelona, 2018-2020). Data collection was carried out by means of clinical examination and a selfreferenced questionnaire. Oral health outcomes included: presence of dental caries, presence of MIH, hypomineralised second primary molars (HSPM). All variables were analysed according to ethnic disparities and other variables such as socioeconomics, diet, hygiene habits, plaque and access to dental services. We performed multivariate Poisson regression models with robust variance to examine ethnic disparities in MIH and caries. CONCLUSION: This cross-sectional study based in Catalonia, Spain showed that there are ethnic disparities in caries as observed with other child's diseases; however, they do not seem to follow the same pattern for MIH. More studies are needed (i) to explore how MIH behaves among populations in terms of inequality; (ii) to study the aetiological factors of MIH; and (iii) to identify potential factors associated with MIH and caries that have not been studied and that may contribute to the observed ethnic disparities.</p

"Ethnic disparities in the prevalence of Molar-Incisor-Hypomineralisation (MIH) and caries among 6-12-year-old children in Catalonia, Spain"

AIM: To study the prevalence of MIH and caries in 6- and 12-year-old schoolchildren and their association with ethnic disparities and other relevant factors. BACKGROUND: In recent years, there has been uneven improvement in school children's oral health, highlighting inequalities in access to dental care and health outcomes, particularly among ethnic minorities. The most prevalent oral disease in childhood, caries, is preventable, as its risk factors are well known. However, MIH, a common condition affecting the enamel of permanent incisors and/or molars, has no established aetiology or preventive measures. METHODS: A cross-sectional study among schoolchildren was conducted in 725 children from Masnou (Barcelona, 2013) and in 577 children from Sant Andreu de Llavaneres (Barcelona, 2018-2020). Data collection was carried out by means of clinical examination and a selfreferenced questionnaire. Oral health outcomes included: presence of dental caries, presence of MIH, hypomineralised second primary molars (HSPM). All variables were analysed according to ethnic disparities and other variables such as socioeconomics, diet, hygiene habits, plaque and access to dental services. We performed multivariate Poisson regression models with robust variance to examine ethnic disparities in MIH and caries. CONCLUSION: This cross-sectional study based in Catalonia, Spain showed that there are ethnic disparities in caries as observed with other child's diseases; however, they do not seem to follow the same pattern for MIH. More studies are needed (i) to explore how MIH behaves among populations in terms of inequality; (ii) to study the aetiological factors of MIH; and (iii) to identify potential factors associated with MIH and caries that have not been studied and that may contribute to the observed ethnic disparities.</p

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

Structures déployables constituées de mètres rubans : modélisations et essais expérimentaux

International audienceThe study focuses on the numerical modeling and experimental characterization of the static behavior of composite tape springs. Rod models with flexible cross section have been developed and are extended in order to take into account the use of composite materials. Tape springs have been manufactured with carbon/epoxy plies to use it in an experimental setup to compare rod models to experimental results. The pure bending test highlights a complex scenario of folding that involves both bending and twisting mechanisms. The comparison between numerical and experimental results of the transverse bending of tape springs is discussed.Les travaux présentés ici portent sur la modélisation et la caractérisation expérimentale du comporte-ment en statique de rubans composites. Les modèles de poutres à section flexible précédemment développés sont étendus ici à l'utilisation de matériaux composites. Des mètres rubans ont été fabriqués à partir de plis unidirectionnels carbone/époxy pour servir d'éprouvette dans une machine d'essais conçue dans le but de confronter le modèle à des résultats expérimentaux. L'essai réalisé est la flexion transverse du ruban qui présente un scénario complexe de formation de plis faisant intervenir des phénomènes de flexion et de torsion. Une discussion est menée au sujet de la confrontation numérique/expérimental pour l'essai de flexion transverse de rubans composites

European regulatory agenices should employ full time statisticians

No abstract available

Measurement of radon-induced backgrounds in the NEXT double beta decay experiment

The measurement of the internal $^{222}$Rn activity in the NEXT-White detector during the so-called Run-II period with $^{136}$Xe-depleted xenon is discussed in detail, together with its implications for double beta decay searches in NEXT. The activity is measured through the alpha production rate induced in the fiducial volume by $^{222}$Rn and its alpha-emitting progeny. The specific activity is measured to be $(38.1\pm 2.2~\mathrm{(stat.)}\pm 5.9~\mathrm{(syst.)})$~mBq/m$^3$. Radon-induced electrons have also been characterized from the decay of the $^{214}$Bi daughter ions plating out on the cathode of the time projection chamber. From our studies, we conclude that radon-induced backgrounds are sufficiently low to enable a successful NEXT-100 physics program, as the projected rate contribution should not exceed 0.1~counts/yr in the neutrinoless double beta decay sample.Comment: 28 pages, 10 figures, 6 tables. Version accepted for publication in JHE