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Purpose: Testotoxicosis is an autosomal dominant form of limited gonadotropin-independent precocious puberty in boys. It is caused by a heterozygous constitutively activating mutation of the LHCGR gene encoding the luteinizing/hormone receptor (LHR). Some twenty mutations of the LHCGR gene have been reported. Most of them are constitutive mutations isolated from blood leukocyte DNA, although others are somatic, found only in testicular tumoural tissue. In all the previously reported cases of these somatic mutations, the tumour, whether a nodular Leydig cell adenoma or hyperplasia, was easily visible on testicular ultrasonography. The aim of this study was to describe an unusual presentation of a patient with the clinical and hormonal characteristics of testotoxicosis but no well-circumscribed lesion at testicular ultrasonography. Materials and Methods: Molecular analysis of the LHCGR gene was performed by direct sequencing of DNA extracted from peripheral leucocytes and testicular biopsy. Results: Molecular analysis didn't find any LHR mutation in blood, whereas it revealed for the first time a somatic D578H mutation in testicular tissue despite no evidence of a nodular aspect at testis ultrasonography. Conclusions: This observation underlines the need to look for a somatic LHCGR gene mutation from the testicular biopsies of all boys with testotoxicosis with no constitutive LHCGR gene mutation identified from blood DNA, even in the absence of circumscribed testicular lesion at ultrasonography. In addition, based on the known link between LHR mutations and testicular tumourigenesis, yearly ultrasound monitoring of the testes should be considered for these patients. [ABSTRACT FROM AUTHOR]

We have studied the statistical properties of the observed magnitudes of seismic vibration data in discrete time in an attempt to understand the underlying complex dynamical processes. The observed magnitude data are taken from six different geographical locations. All possible magnitudes are considered in the analysis including catastrophic vibrations, foreshocks, aftershocks and commonplace daily vibrations. The probability distribution functions of these data sets obey scaling law and display a certain universality characteristic. To investigate the universality features in the observed data generated by a complex process, we applied Random Matrix Theory (RMT) in the framework of Gaussian Orthogonal Ensemble (GOE). For all these six places the observed data show a close fit with the predictions of RMT. This reinforces the idea of universality in the dynamical processes generating seismic vibrations. [ABSTRACT FROM AUTHOR]

Summary: Type 1 diabetes, whose incidence is increasing in the youngest children, could have a long-term neuropsychological impact. Mood disorders are more frequent and some elements of cognitive performance, although within normal ranges, could decrease. In this review, we detail the contribution of animal models to current physiopathological knowledge. We summarize the main clinical studies regarding mood and cognitive performance in diabetic children. Finally, the advantages of imaging in this domain as related to brain development in children are discussed. [Copyright &y& Elsevier]

Abstract: Ultrasonic signals received by pulse echo technique from plane parallel Zircaloy 2 samples of fixed thickness and of three different microstructures, were subjected to signal analysis, as conventional parameters like velocity and attenuation could not reliably discriminate them. The signals, obtained from these samples, were first sampled and digitized. Modified Karhunen Loeve Transform was used to reduce their dimensionality. A multilayered feed forward Artificial Neural Network was trained using a few signals in their reduced domain from the three different microstructures. The rest of the signals from the three samples with different microstructures were classified satisfactorily using this network. [Copyright &y& Elsevier]

The complexity of the Portevin-Le Chatelier effect in Al-2.5%Mg polycrystalline samples subjected to uniaxial tensile tests is quantified. Multiscale entropy analysis is carried out on the stress time series data observed during jerky flow to quantify the complexity of the distinct spatiotemporal dynamical regimes. It is shown that for the static type C band, the entropy is very low for all the scales compared to the hopping type B and the propagating type A bands. The results are interpreted considering the time and length scales relevant to the effect. [ABSTRACT FROM AUTHOR]

The heart beat data recorded from samples before and during meditation are analyzed using two different scaling analysis methods. These analyses revealed that meditation severely affects the long range correlation of heart beat of a normal heart. Moreover, it is found that meditation induces periodic behavior in the heart beat. The complexity of the heart rate variability is quantified using multiscale entropy analysis and recurrence analysis. The heart beat during meditation is found to be more complex. [ABSTRACT FROM AUTHOR]

Molecular dynamics simulation study based on the EAM potential is carried out to investigate the effect of pressure on the rapid solidification of Aluminum. The radial distribution function is used to characterize the structure of the Al solidified under different pressures. It is indicated that a high pressure leads to strong crystallization tendency during cooling. [ABSTRACT FROM AUTHOR]

Summary: Obesity is increasing worldwide. Abdominal obesity, which is due to the development of visceral adipose tissue, leads to metabolic disorders. Because abdominal obesity is associated with Cushing syndrome, many studies have been performed to find out how the hypothalamopituitary adrenal axis is involved in this disorder. Here, we propose to review these data before giving our experience on changes in hypothalamopituitary adrenal axis activity regarding fat mass distribution in prepubertal children. [Copyright &y& Elsevier]

Abstract: We present a continuous time random walk model for the Portevin–Le Chatelier (PLC) effect. From our result it is shown that the dynamics of the PLC band can be explained in terms of the Levy walk. [Copyright &y& Elsevier]

The use of graphite as a moderator in a low temperature thermal nuclear reactor is restricted due to accumulation of energy caused by displacement of atoms by neutrons and high energetic particles. Thermal transients may lead to a release of stored energy that may raise the temperature of the fuel clad above the design limit. Disordered carbon is thought to be an alternative choice for this purpose. Two types of disordered carbon composites, namely, CB (made up of 15 wt. % carbon black dispersed in carbonized phenolic resin) and PAN (made up of 20 vol. % chopped polyacrylonitrile carbon fibre dispersed in carbonized phenolic resin matrix) have been irradiated with 145 MeV Ne6+ ions at three fluence levels of 1.0×1013, 5.0×1013 and 1.5×1014 Ne6+/cm2, respectively. The XRD patterns revealed that both the samples remained disordered even after irradiation. The maximum release of stored energy for CB was 212 J/g and that of PAN was 906 J/g. For CB, the release of stored energy was a first order reaction with activation energy of 2.79 eV and a frequency factor of 3.72×1028 per second. 13% of the defects got annealed by heating up to 700 °C. PAN showed a third-order release rate with activation energy of 1.69 eV and a frequency factor of 1.77×1014 per second. 56% of the total defects got annealed by heating it up to 700 °C. CB seems to be the better choice than PAN as it showed less energy release with a slower rate. [ABSTRACT FROM AUTHOR]

The time series data of the monthly rainfall records (for the time period 1871–2002) in All India and different regions of India are analyzed. It is found that the distributions of the rainfall intensity exhibit perfect power law behavior. The scaling analysis revealed two distinct scaling regions in the rainfall time series. [ABSTRACT FROM AUTHOR]

The plastic deformation of dilute alloys is often accompanied by plastic instabilities due to dynamic strain aging and dislocation interaction. The repeated breakaway of dislocations from and their recapture by solute atoms leads to stress serrations and localized strain in the strain controlled tensile tests, known as the Portevin-Le Chatelier (PLC) effect. In this present work, we analyze the stress time series data of the observed PLC effect in the constant strain rate tensile tests on Al-2.5%Mg alloy for a wide range of strain rates at room temperature. The scaling behavior of the PLC effect was studied using two complementary scaling analysis methods: the finite variance scaling method and the diffusion entropy analysis. From these analyses we could establish that in the entire span of strain rates, PLC effect showed Levy walk property. Moreover, the multiscale entropy analysis is carried out on the stress time series data observed during the PLC effect to quantify the complexity of the distinct spatiotemporal dynamical regimes. It is shown that for the static type C band, the entropy is very low for all the scales compared to the hopping type B and the propagating type A bands. The results are interpreted considering the time and length scales relevant to the effect. [ABSTRACT FROM AUTHOR]

Abstract: In this paper, we investigate the scaling behavior of the average daily exchange rate returns of the Indian Rupee against four foreign currencies: namely, US Dollar, Euro, Great Britain Pound and Japanese Yen. The average daily exchange rate return of the Indian Rupee against US Dollar is found to exhibit a persistent scaling behavior and follow Levy stable distribution. On the contrary, the average daily exchange rate returns of the other three foreign currencies do not show persistency or antipersistency and follow Gaussian distribution. [Copyright &y& Elsevier]

We have characterised the microstructural parameters like domain size, microstrain within the domain, dislocation density and stacking fault probabilities in Zr–1.0%Nb–1.0%Sn–0.1%Fe alloy, irradiated with 116 MeV O5+ ion at different doses, by X-ray diffraction line profile analysis using modified Rietveld technique. The analysis revealed that there is a significant decrease in domain size but increase in microstrain and dislocation density with dose of irradiation. These microstructural parameters have been compared with our earlier observations on the same alloy irradiated at different doses with 15 MeV proton. We find significant changes of these parameters in oxygen-irradiated samples as compared to the proton-irradiated samples. The variations of microstructures in these samples have been explained in the light of the mechanism of defect evolution for light and heavy ion irradiation. [Copyright &y& Elsevier]

Plastic flow behaviour of low carbon steel has been studied at room temperature during tensile deformation by varying the initial strain rate of 3.3 x 10-4 s-1 to a final strain rate ranging from 1.33 x 10-3 s-1 to 2 x 10-3 s-1 at a fixed engineering strain of 12%. Haasen plot revealed that the mobile dislocation density remained almost invariant at the juncture where there was a sudden increase in stress with a change in strain rate and the plastic flow was solely dependent on the velocity of mobile dislocations. In that critical regime, the variation of stress with time was fitted with a Boltzmann type Sigmoid function. The increase in stress was found to increase with final strain rate and the time elapsed in attaining these stress values showed a decreasing trend. Both of these parameters saturated asymptotically at a higher final strain rate. [ABSTRACT FROM AUTHOR]

Unlabelled: We report the cases of two sibs of North African origin with AAAS gene mutation characterized by the heterogeneity of their phenotype. While an 8-y-old boy presented with acute adrenal insufficiency and mental retardation, the diagnosis was suggested by the clinical history of his 6-y-old sister who had symptomatic achalasia and chronic adrenal failure.Conclusion: Our observations corroborate the phenotypic heterogeneity reported in triple A syndrome, and underline the possibility of a variable intra-familial expression. [ABSTRACT FROM AUTHOR]

The Authors report two cases of APECED syndrome (Auto-immune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy) revealed by hyponatremia due to isolated mineralocorticoids deficiency. Such cases illustrate the variable endocrine features revealing the syndrome. The mechanisms underlying the auto-immune destruction of the adrenals are discussed. [Copyright &y& Elsevier]

Pinning of dislocations at nanosized obstacles like precipitates, voids, and bubbles is a crucial mechanism in the context of phenomena like hardening and creep. The interaction between such an obstacle and a dislocation is often studied at fundamental level by means of analytical tools, atomistic simulations, and finite element methods. Nevertheless, the information extracted from such studies cannot be utilized to its maximum extent on account of insufficient information about the underlying statistics of this process comprising a large number of dislocations and obstacles in a system. Here, we propose a new statistical approach, where the statistics of pinning of dislocations by idealized spherical obstacles is explored by taking into account the generalized size-distribution of the obstacles along with the dislocation density within a three-dimensional framework. Starting with a minimal set of material parameters, the framework employs the method of geometrical statistics with a few simple assumptions compatible with the real physical scenario. The application of this approach, in combination with the knowledge of fundamental dislocationobstacle interactions, has successfully been demonstrated for dislocation pinning at nanovoids in neutron irradiated type 316-stainless steel in regard to the non-conservative motion of dislocations. An interesting phenomenon of transition from rare pinning to multiple pinning regimes with increasing irradiation temperature is revealed [ABSTRACT FROM AUTHOR]

Microcephalic primordial dwarfisms are a group of rare Mendelian disorders characterized by severe growth retardation and microcephaly. The molecular basis is heterogeneous, with disease‐causing genes implicated in different cellular functions. Recently, 2 patients were reported with the same homozygous variant in the WDR4 gene, coding for an enzyme responsible for the m7G46 post transcriptional modification of tRNA. We report here 2 sisters harboring compound heterozygous variants of WDR4. Their phenotype differs from that of the first 2 described patients: they both have a severe microcephaly but only one of the 2 sisters had a head circumference at birth below −2 SD, their intellectual deficiency is less severe, and they have a growth hormone deficiency and a partial hypogonadotropic hypogonadotropism. One of the 2 variants is a frameshift mutation, and the other one is a missense occurring in the same nucleotide affected by the first reported pathogenic variant, which could therefore be a mutational hot spot. The description of these 2 sisters allow us to confirm that biallelic variants in the WDR4 gene can lead to a specific phenotype, characterized by severe growth retardation and microcephaly. [ABSTRACT FROM AUTHOR]

Résumé Le diabète sucré de l’enfant peut correspondre à différentes entités physiopathologiques mais le diabète de type 1 reste de loin la forme de diabète la plus fréquente chez l’enfant. Son incidence n’a cessé de progresser au cours des 2 dernières décennies. Cette progression est particulièrement forte chez les plus jeunes enfants, conduisant à un rajeunissement de l’âge de découverte du diabète et une augmentation du nombre de patients à prendre en charge par les pédiatres. Ainsi, environ 25 % des diagnostics de diabète de type 1 se font chez des enfants de moins de 5 ans. En France, le diabète de type 2 reste rare chez l’enfant malgré la progression de l’obésité. Il est à rechercher chez l’adolescent obèse aux antécédents familiaux de diabète de type 2. Les diabètes monogéniques sont de diagnostic plus fréquent que les diabètes de type 2 de l’enfant. Leur recherche dépend de l’analyse de l’anamnèse familiale et peut déboucher sur une attitude thérapeutique spécifi que. Summary Diabetes mellitus in childhood may correspond to different pathophysiological entities but type 1 diabetes is by far the most common form of diabetes in children. Its incidence has been increasing steadily over the past two decades. This trend is particularly important among younger children, leading to a youngest median age at the discovery of diabetes. Thus, approximately 25% of diagnoses of type 1 diabetes are in children under 5 years. In France, the type 2 diabetes in children is rare despite the rise in obesity. Investigations for the diagnosis are recommended in obese adolescents with a family history of type 2 diabetes. Monogenic diabetes are more common than type 2 diabetes in Europe. Their research depends on the analysis of family history and may lead to a specifi c therapeutic approach. [ABSTRACT FROM AUTHOR]

We aimed to evaluate catch-up growth in children with severe Hashimoto's hypothyroidism (HH) after thyroid hormone replacement therapy (HRT). A multicenter retrospective study was conducted including children referred for growth slowdown that led to the diagnosis of HH between 1998 and 2017. A total of 29 patients were included, with a median age of 9.7 years (13–172 months). Median height at diagnosis was -2.7 [-4.6; -0.1] standard deviation score (SDS), with a height loss of 2.5 [0.7; 5.4] SDS compared to height before growth deflection (p <0.0001). At diagnosis, the median TSH level was 819.5 mIU/L [100; 1844], the median FT4 level was 0 pmol/L [undetectable; 5.4], and the median anti-thyroperoxidase antibody level was 1601 UI/L [47; 25,500]. In the 20 patients treated only with HRT, there were significant differences between height at diagnosis and height at 1 year (n = 19, p <0.0001), 2 years (n = 13, p = 0.0005), 3 years (n = 9, p = 0.0039), 4 years (n = 10, p = 0.0078), and 5 years (n = 10, p = 0.0018) of treatment but not in the case of final height (n = 6, p = 0.0625). Median final height was -1.4 [-2.7; 1,5] SDS (n = 6), with a significant difference between height loss at diagnosis and total catch-up growth (p = 0.003). The other nine patients were also given growth hormone (GH). They were smaller at diagnosis (p = 0.01); however, there was no difference in final height between those two groups (p = 0.68). Severe HH can lead to a major height deficit, and catch-up growth seems to be insufficient after treatment with HRT alone. In the most severe cases, administration of GH may enhance this catch-up. [ABSTRACT FROM AUTHOR]

Neonatal screening for congenital hypothyroidism (CH) is based on the measurement of thyroid-stimulating hormone (TSH) in whole dried blood samples on filter paper in all newborns. The objective of screening for CH is to prevent mental retardation, which is irreversible in the event of a late diagnosis, by setting up prompt treatment (before day 15) with levothyroxine. The threshold value of TSH on filter paper on day 3 is 17 mIU/L in France in the GSP method (GSP, Genetic Screening Processor, Perkin Elmer): It is one of the highest thresholds used in the world. In many countries, the TSH threshold is between 6 and 12 mIU/L. Studies have found that a threshold of > 17 mIU/L may miss as much as 30% of cases of CH, with 30–80% of these being permanent CH. Recent studies suggest that mild CH (currently missed by the French TSH threshold) is associated with cognitive consequences if left untreated. An inverse relationship between TSH at screening (below the current threshold) and cognitive development at preschool or school age has been shown. These studies advocate for the evaluation of a lowering of the threshold of TSH on filter paper in France: (a) to determine the number of CH diagnoses with the new threshold and whether these "new cases" would be transitory or permanent; and (b) to analyze the cost-effectiveness of the strategy. [ABSTRACT FROM AUTHOR]

Dunnigan syndrome, or Familial Partial Lipodystrophy type 2 (FPLD2; ORPHA 2348), is a rare autosomal dominant disorder due to pathogenic variants of the LMNA gene. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins), is to provide health professionals with a guide to optimal management and care of patients with FPLD2, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), is available on the French Health Authority website (in French). Dunnigan syndrome is characterized by a partial atrophy of the subcutaneous adipose tissue and by an insulin resistance syndrome, associated with a risk of metabolic, cardiovascular and muscular complications. Its prevalence, assessed at 1/100.000 in Europe, is probably considerably underestimated. Thorough clinical examination is key to diagnosis. Biochemical testing frequently shows hyperinsulinemia, abnormal glucose tolerance and hypertriglyceridemia. Elevated hepatic transaminases (hepatic steatosis) and creatine phosphokinase, and hyperandrogenism in women, are common. Molecular analysis of the LMNA gene confirms diagnosis and allows for family investigations. Regular screening and multidisciplinary monitoring of the associated complications are necessary. Diabetes frequently develops from puberty onwards. Hypertriglyceridemia may lead to acute pancreatitis. Early atherosclerosis and cardiomyopathy should be monitored. In women, polycystic ovary syndrome is common. Overall, the management of patients with Dunnigan syndrome requires the collaboration of several health care providers. The attending physician, in conjunction with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are described to provide such a support. [ABSTRACT FROM AUTHOR]

Abstract: The Portevin-Le Chatelier (PLC) effect has been investigated by deforming Al–2.5%Mg alloy in the strain rate regime where simultaneously two types (type B and type A) of serrations appear in the stress–strain curve. Our analysis of the time series data suggests that in this strain rate regime the entire PLC dynamics is governed by a single band which changes its character during the deformation. [Copyright &y& Elsevier]

Determination of lattice misfit and microstructural parameters of the coherent precipitates in Ni based alloy Inconel-625 is a challenging problem as the diffraction peaks of the precipitate and the matrix are completely overlapping. X-ray Diffraction Line Profile Analysis (XRDLPA) has been used to characterize the bulk samples of Inconel 625 at different heat-treated conditions to determine the lattice parameters of the parent phase and also the coherent precipitates by the separation of the overlapping peaks. The lattice misfits of the coherent precipitates with the matrix and their microstructural parameters like size and strain have also been determined. [ABSTRACT FROM AUTHOR]

Abstract: The diffraction peaks of Zircaloy-2 and Zr–2.5% Nb alloys at various deformations are found to be asymmetric in nature. In order to characterize the microstructure from these asymmetric peaks of these deformed alloys, X-ray diffraction line profile analysis like Williamson–Hall technique, Variance method based on second- and fourth-order restricted moments and Stephens model based on anisotropic strain distribution have been adopted. The domain size and dislocation density have been evaluated as a function of deformation for both these alloys. These techniques are useful where the dislocation structure is highly inhomogeneous inside the matrix causing asymmetry in the line profile, particularly for deformed polycrystalline materials. [Copyright &y& Elsevier]

Two sets of amorphous carbon materials prepared at different routes are irradiated with swift (145 MeV) heavy ion (Ne6+). The structural parameters like the extent of local ordering along the c and a axis i.e., Lc and La, the average spacing of the crystallographic planes (002) i.e., d002 and the fraction of the amorphous phase of the unirradiated and the irradiated samples are estimated by X-ray diffraction technique. The fraction of the amorphous phase is generally found to increase with the irradiation dose for both sets of the samples. The estimated d002 values are found to be almost unaffected by irradiation but Lc and La are decreased slightly with irradiation. The estimated values of Lc and La corroborate with the increase of disorder in both sets of the samples with the increasing dose of irradiation. [ABSTRACT FROM AUTHOR]

Abstract: Tensile tests were carried out by deforming polycrystalline samples of Al-2.5%Mg alloy at room temperature in a wide range of strain rates where the Portevin–Le Chatelier (PLC) effect was observed. The experimental stress-time series data have been analyzed using the recurrence analysis technique based on the Recurrence Plot (RP) and the Recurrence Quantification Analysis (RQA) to study the change in the dynamical behavior of the PLC effect with the imposed strain rate. Our study revealed that the RQA is able to detect the unique crossover phenomenon in the PLC dynamics. [Copyright &y& Elsevier]

Abstract: Various X-ray diffraction line profile analysis (XRDLPA) techniques have been used to assess the microstructure of an heavy irradiated Zr–1.0%Nb–1.0%Sn–0.1%Fe alloy. The domain size, microstrain, density of dislocation and stacking fault probabilities of the irradiated alloy have been estimated as a function of dose by the Williamson–Hall Technique, a Modified Rietveld Analysis and by the Double Voigt Method. A clear signature for an increase in the density of dislocation with the dose of irradiation was revealed. The analysis also estimated the average density of dislocation in the major slip planes after irradiation. [Copyright &y& Elsevier]

Abstract: Tensile tests were carried out by deforming polycrystalline samples of an interstitial alloy, low carbon steel at room temperature in a wide range of strain rates where the Portevin-Le Chatelier (PLC) effect was observed. The observed stress time series data were analyzed using the non-linear dynamical methods. From the analyses, we could establish the presence of marginal deterministic chaos in the PLC effect of the low carbon steel. Moreover, we made a comparative study of the PLC effect of this interstitial alloy with the substitutional Al–Mg alloy, which shows that the dynamics of the PLC effect in the interstitial alloy is more complex compared to that of the substitutional alloy. [Copyright &y& Elsevier]

Abstract: We have carried out irradiation with 116 MeV O5+ ions on Zr–1Sn–1Nb–0.1Fe (ZIRLO) alloy at different doses and the microstructural parameters of the irradiated samples have been characterised by X-ray Diffraction Line Profile Analysis (XRDLPA). The average volume-weighted and surface-weighted domain size, microstrain and dislocation density have been estimated as a function of dose. There was a drastic decrease in domain size from unirradiated sample to the sample at a dose of 1×1017 O5+/m2, but these values saturated with increasing dose of irradiation. The values of microstrain were found to increase with dose. The dislocation density increased almost by an order of magnitude for the samples irradiated with 1×1018 O5+/m2 and 5×1018 O5+/m2 as compared to the unirradiated samples. [Copyright &y& Elsevier]

Abstract: Different techniques of the X-ray diffraction line profile analysis (XRDLPA) have been used to study the microstructural parameters of the heavily deformed solid polycrystalline Zircaloy-2 and Zr–2.5%Nb alloys. The domain size, microstrain within the domain, dislocation density and the stacking fault probabilities have been estimated by the Simplified Breadth Method, Williamson–Hall technique and the Modified Rietveld Method. A clear signature of the anisotropy in the domain shape was revealed. The domain size (De) was quantified along the different crystallographic directions. In these deformed alloys, the dislocation density (ρe) was of the order of 1015 m−2 but the stacking fault probabilities were almost negligible. For the first time, we could establish an empirical relationship between De and for these hexagonal closed packed alloys. [Copyright &y& Elsevier]

Abstract Purpose Binge eating disorder (BED) is associated with higher psychopathology, including emotional and personality disorders, in the adult population, whether or not they are obese; although few data are available on adolescents, particularly among obese adolescents. Objective To explore the association of both emotional disorders and personality dimensions with BED in obese adolescents. Methods The sample consisted of 115 French adolescents enrolled at a clinical unit for the multidisciplinary care of their overweight or obesity. BED was defined using the Binge Eating Scale (BES). Emotional disorders and personality dimensions were assessed using the following tools: Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Junior Temperament and Character Inventory (JTCI); impulsivity was determined by the Barratt Impulsiveness Scale (BIS) and the Toronto Alexithymia Scale (TAS). Associations between emotional disorders (BDI/BAI) and personality dimensions (TAS/BIS/JTCI), considered first separately and then jointly with BED were determined with multivariate analysis. Results More severe depression (β=0.27, CI [0.06; 0.48], P = 0.011), a higher level of excess weight (β=1.91, CI [0.22; 3.59], P = 0.027), older age (β=1.28, CI [0.43; 2.14], P = 0.003), and greater cooperativeness (β=0.36, CI [0.07; 0.66], P = 0.017) were independently significantly associated with the presence of BED. Conclusions This cross-sectional study underlines the co-occurrence of emotional and personality disorders with BED. This points out the importance of a multidisciplinary approach and the relevance of a joint diagnosis of binge eating, emotional disorders, and personality dimensions in obese adolescents, for better prevention and treatment of pediatric obesity. [ABSTRACT FROM AUTHOR]

Abstract Background The impact of 7-day real-time continuous glucose monitoring (RT-CGM) on type 1 diabetes (T1D) management remains unknown in youths with suboptimal control by multiple daily injections (MDI). The DIACCOR Study aimed to describe treatment decisions and glucose outcomes after a short-term RT-CGM sequence in real-life conditions. Methods This French multicenter longitudinal observational study included T1D youths with HbA1c > 7.5% or a history of severe hypoglycemia (SH) or recurrent documented hypoglycemia. A sensor was inserted at the study-inclusion visit, and one of three predefined treatment changes was proposed by the investigator within 7–15 days: INT = MDI intensification, CSII = switch to continuous insulin infusion, or ER = educational reinforcement with no change in insulin regimen and a 4-month follow-up visit (M4) was scheduled. Results A total of 229 children (12.2 ± 3.5 years old) were recruited by 74 pediatricians; 12.8% had a history of SH, 22.2% had recurrent hypoglycemia. Baseline HbA1c was 8.7 ± 1.5% (> 7.5% in 82.8%). Overall, 139 (79.4%), 19 (10.9%), and 17 patients (9.7%) were, respectively, included in the INT, CSII, and ER subgroups. At M4, the global incidence of SH and recurrent hypoglycemia dropped (3.4% vs. 12.8% and 6.0% vs. 22.2%, respectively) as well as the incidence of ketoacidosis (2.1% vs. 8.1%) or ketosis (6.9% vs. 11.4%). The HbA1c decrease was significant overall and in the INT subgroup (adjusted difference −0.29%, P = 0.009). The satisfaction rate was ≥ 93.0% among children. Conclusion In a real-life setting, a 1-week RT-CGM can promote treatment optimization in youths with uncontrolled T1D resulting mostly in less acute events. CGM acceptance may improve with new-generation sensors. [ABSTRACT FROM AUTHOR]

Presents a surface analysis of bismuth-based cuprates superconductors through secondary ion mass spectrometry and X-ray photoelectron spectroscopy. Properties of cuprates superconductors; Discussion on kinetic energy distributions for secondary negative ions; Explanation for the emission of small clusters in bismuth-based cuprates.

Presents a study which examined the superconducting compound YBa[sub2]Cu[sub3]O[sub7-x] and oxides of copper by secondary-ion-mass spectrometry (SIMS). Experimental procedures; Results; Conclusions.

An attempt is made to understand the pattern of behaviour of the BSE Sensex by analysing the tick-by-tick Sensex data for the years 2006 to 2012 on yearly as well as cumulative basis using Principal Component Analysis (PCA) and its nonlinear variant Kernel Principal Component Analysis (KPCA). The latter technique ensures that the nonlinear character of the interactions present in the system gets captured in the analysis. The analysis is carried out by constructing vector spaces of varying dimensions. The size of the data set ranges from a minimum of 360,000 for one year to a maximum of 2,520,000 for seven years. In all cases the prices appear to be highly correlated and restricted to a very low dimensional subspace of the original vector space. An external perturbation is added to the system in the form of noise. It is observed that while standard PCA is unable to distinguish the behaviour of the noise-mixed data from that of the original, KPCA clearly identifies the effect of the noise. The exercise is extended in case of daily data of other stock markets and similar results are obtained. [ABSTRACT FROM AUTHOR]

Aim: The RePPOP Aquitaine network, which was established in south-west France to prevent and treat paediatric obesity, has developed a multidisciplinary approach based on multicomponent lifestyle interventions and family-based actions. This study assessed the impact of its care management programme and investigated the factors associated with better outcomes.Methods: The impact of the RePPOP care management programme was assessed by changes in the body mass index (BMI) Z score, between baseline and the end-of-care management. We focused on 982 overweight or obese children (59.9% girls) with a mean age of 10.64 years and a range of 2.4-17.9 years. A multivariate analysis examined the independent factors associated with better outcomes.Results: At the end-of-care management programme, 75.5% of children had decreased their BMI Z score. Initial characteristics significantly associated with better outcomes were as follows: the age at baseline between five years and 15 years of age, playing sport at a club, being followed up by RePPOP for longer than 10 months, no parental obesity and no academic difficulties.Conclusion: This study confirmed that multidisciplinary treatment had a significant positive effect on paediatric obesity and that social and individual factors affected the efficiency of the care management. [ABSTRACT FROM AUTHOR]

Résumé L’objectif de cette étude était d’évaluer, au cours de la première année suivant une campagne nationale d’information, l’effet sur la fréquence et la sévérité de l’acidocétose au moment du diagnostic de diabète de type 1 (DT1) chez l’enfant et l’adolescent. Les données suivantes ont été colligées pendant deux années consécutives chez les jeunes de moins de 15 ans ayant un DT1 débutant dans 146 services de pédiatrie : âge, sexe, durée des symptômes, parcours du patient, signes cliniques et biologiques, antécédents familiaux de DT1. L’acidocétose était définie par un pH < 7,30 ou une réserve alcaline (RA) < 15 mmol/L ; l’acidocétose sévère par un pH < 7,10 ou une RA < 5 mmol/L. Pendant la deuxième année, une campagne ayant pour but d’informer les professionnels de santé et les familles a débuté pour éviter les retards au diagnostic. Les données de la première année de campagne (année 1) ont été comparées à celles de l’année avant la campagne (année 0). Le nombre de nouveaux cas de DT1 a été de 1299 pour l’année 0 et 1247 pour l’année 1. Entre l’année 0 et l’année 1, la fréquence de l’acidocétose a diminué, de 43,9 % à 40,5 % ( p = 0,08), exclusivement du fait d’une diminution des acidocétoses sévères, de 14,8 à 11,4 % ( p = 0,01). Dans les groupes d’âge 0–5 ans et 5–10 ans, la baisse relative de la fréquence de l’acidocétose a été respectivement de 13 % et 15 %, et celle des formes sévères de 23 % et 41 %. Chez les enfants adressés à l’hôpital par un pédiatre ou venus à l’initiative de la famille, elle a été respectivement de 34 et 7 %, et celle des formes sévères de 39 et 32 %. Aucun changement n’a été observé pour les jeunes de 10–15 ans et pour ceux qui ont été adressés par un médecin généraliste. En analyse multivariée, une fréquence plus élevée de l’acidocétose était associée avec le jeune âge de l’enfant (< 5 ans), le fait d’être venu à l’hôpital à l’initiative de la famille plutôt qu’adressé par un médecin, et l’absence d’antécédent familial de DT1. Une fréquence plus élevée d’acidocétose sévère était associée à ces deux derniers facteurs mais pas à l’âge de l’enfant. La fréquence de l’acidocétose sévère au moment du diagnostic de DT1 chez l’enfant et l’adolescent reste élevée, mais la campagne nationale d’information a permis de la diminuer dès la première année. Les résultats ont aussi permis de préciser la stratégie et les cibles de la campagne qui se poursuit, afin de réduire de manière de plus en plus efficace la morbi-mortalité au moment du diagnostic du DT1 chez l’enfant en France. Summary The aim of the study was to evaluate, after the first year of a national information campaign, the effect on the frequency and severity of diabetic ketoacidosis (DKA) at diagnosis of type 1 diabetes (T1D) in children and adolescents in France. The following data were collected during a 2-year period in people younger than 15 years of age at diagnosis of T1D, in 146 pediatric centers: age, sex, duration of symptoms, patient's previous care, clinical and biological signs, and family history of T1D. DKA was defined as pH < 7.30 or bicarbonate < 15 mmol/L, severe DKA as pH < 7.10 or bicarbonate < 5 mmol/L. During the 2nd year, an information campaign targeting health professionals and families was launched with the objective of reducing the time to diagnosis. Data were compared between the year before the campaign (year 0) and the first year of the campaign (year 1). The number of new cases of T1D was 1299 for year 0 and 1247 for year 1. Between year 0 and year 1, the rate of DKA decreased from 43.9% to 40.5% ( P = 0.08), exclusively due to the decrease of severe DKA from 14.8 to 11.4% ( P = 0.01). In the 0- to 5-year-old and 5- to 10-year-old age groups, the relative decrease in the rate of DKA was 13% and 15%, and 23% and 41% for severe DKA, respectively. In patients referred to the hospital by a pediatrician or who came at the family's initiative, the decrease was 34% and 7%, and 39% and 32% for severe DKA, respectively. No change was observed in the 10- to 15-year-old group or in those children who were referred by a general practitioner. In multivariate analyses, a higher DKA rate was associated with the young age of the child (< 5 years), being hospitalized at the parents’ initiative rather than being referred by a doctor, and the absence of a family history of T1D. A higher rate of severe DKA was associated with these last two factors but not with the child's age. The frequency of DKA at diagnosis of type 1 diabetes remains high in children and adolescents, but the first year of an information campaign decreased it. The results have also helped better define the strategy and targets of the continuing prevention campaign, to more efficiently reduce the morbidity and mortality of T1D at diagnosis in children and adolescents in France. [ABSTRACT FROM AUTHOR]

L’éducation thérapeutique du patient (ETP) permet au patient atteint de maladie chronique d’acquérir des connaissances et compétences, d’adapter ses comportements afin améliorer certains indicateurs de santé, son autonomie, sa qualité de vie et diminuer d’éventuelles complications médicales. Le diabète de type 1 est l’une des maladies chroniques les plus fréquentes chez l’enfant et l’adolescent, l’ETP fait partie de sa prise en charge. L’objectif de ce travail était d’identifier s’il existait des différences entre les enfants diabétiques de type 1 suivis au CHU de Bordeaux pendant l’année 2010 participant à l’ETP et ceux n’y participant pas. Pour cela, nous avons comparé les caractéristiques sociales et médicales des patients des deux groupes. Nous n’avons pas retrouvé de différence de caractéristiques sociales. Concernant les données médicales, nous n’avons pas retrouvé de différence pour le taux et le nombre de dosage d’HbA1c, le nombre de consultations et d’hospitalisations de jour. Par contre, nous avons retrouvé une différence significative concernant l’ancienneté du diabète, le schéma thérapeutique et le médecin référent. Ce type de travail permet d’analyser les caractéristiques des patients bénéficiant ou non du programme d’ETP afin d’en améliorer l’offre de soins. [ABSTRACT FROM AUTHOR]

La néoplasie endocrinienne multiple de type 2 (NEM2) est une atteinte multiglandulaire à transmission autosomique dominante responsable de 100% de survenue de cancer médullaire de la thyroïde (CMT). Une thyroïdectomie préventive est proposée à ces patients. Objectif Décrire les complications post-thyroïdectomie préventive chez les enfants porteurs de NEM2 opérés avant 15 ans. Méthode Etude descriptive, multicentrique. Inclusion de 37 patients porteurs d’une NEM2 ayant eu une thyroïdectomie préventive avant 15 ans dans les CHU de Bordeaux, Toulouse et Angers. Résultats Les patients ont bénéficié à un âge moyen de 5,7 ans (±3,2) d’une thyroïdectomie totale, avec curage ganglionnaire pour 40%. Complications post-opératoire : hypoparathyroïdie (8%), paralysie récurentielle (2%), plaie trachéale (2%). L’âge moyen de la chirurgie chez les enfants ayant présenté des complications est de 3,8 ans (± 2,3 ans), 2 sur 5 avaient eu un curage ganglionnaire. Conclusion Les complications semblent plus fréquentes lors des gestes très précoces, mais cette précocité est garante de l’absence de dissémination du CMT et permet d’éviter le curage ganglionnaire, lui-même à l’origine de complications. Les recommandations actuelles préconisent une thyroïdectomie précoce sans curage ganglionnaire. [ABSTRACT FROM AUTHOR]

A simplified instrumentation with nominal circuitry has been developed to measure in- and out-of-phase complex magnetic susceptibility of bulk magnetic samples. A unique shape of the absolute coil is implemented to improve the magnetic field homogeneity inside the coil. Complex susceptibility of nano-composite is measured by an innovative phase detection circuit. In this paper, we also discuss in detail the principle, design, and the performance of the instrumentation. This susceptometer is also suitable for liquid samples, viz., ferro-fluids, blood samples, etc. [ABSTRACT FROM PUBLISHER]

In this study, samples of zircaloy-2 were irradiated with oxygen ion beam (O5+ ions with 116 MeV energy) to fluence varying from 1013 to 1015 ion/cm2. The irradiated samples were observed under a transmission electron microscope to identify the irradiation-induced defects and phase transformations. Microstructural investigations on these samples showed the presence of interstitial defects and ZrO2 precipitates in the α (hcp) matrix. The alignment of defects appeared to facilitate the formation of the ZrO2 phase in the irradiated samples. Orientation relationship between the oxide and the matrix phase was determined and a possible mechanism for the formation of the ZrO2 phase based on the entrapment of the oxygen ions has been proposed. [ABSTRACT FROM AUTHOR]

Abstract: Techniques of atomistic simulation have been employed to determine the mechanism of the newly observed process of climb of a dislocation line during depinning at a nanosized void. The analyses prove that, in contrast to the conventional notion of dislocation climb, this unique process is not a diffusion controlled phenomenon. Instead, the gradient of structural energy of the system alone suffices to produce the climb motion. The energetics of this process has been investigated through molecular statics and dynamics computations and the causes of discrepancies are discussed. In order to further explore these concepts in a quantitative manner we have developed a robust simulation strategy to accurately measure the reduction in energy of the nanovoid during dislocation climb. The results explain the lowering of the critical depinning load and the effect of thermal facilitation on void-induced climb. Moreover, we highlight the key role played by the curvature of the dislocation line in mediating this mechanism. [Copyright &y& Elsevier]