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Reducing dietary phosphorus (P) is a common approach to reduce effluent P outputs. The potential resulting P-deficiency is known to negatively impact fish bone condition and might result in vertebral deformities. To date, no large-scale study involving deep sequencing of the bone transcriptome has been conducted in salmonids and vertebral molecular changes remain poorly described. This study aims to provide the first comprehensive vertebral transcriptome for rainbow trout ( Oncorhynchus mykiss ) to allow functional and quantitative expression studies. Fish weighing 60.8 ± 1.6 g, were fed for 27 weeks using two practical diets having 0.29% (deficient) and 0.45% (sufficient) available phosphorus (P), respectively. Deep sequencing was conducted using HiSeq2000 Illumina 100 paired-end technology from pooled P-deficient and P-sufficient fish and individuals displaying vertebral deformities. Over 140 million trimmed paired-end reads were assembled de novo with Trinity and resulted in 679,869 transcripts with a mean length of 542.5 bp. From these sequences, 340,747 matched with referenced ESTs from rainbow trout. Furthermore, 141,909 and 117,564 sequences were functionally annotated against Nr and Uniprot databases, respectively. Interestingly, we observed putative homologue sequences for most of the key components involved in bone formation and turnover in mammals. [ABSTRACT FROM AUTHOR]

Summary: Aim of the study: In a double-blind, randomized, controlled, multicentric study, the study compared the efficacy of a specific adapted formula (lactose-free, high-mineral, low-osmolarity formula, containing rice and pectin fortified with Saccharomyces boulardii) in the management of infants with acute diarrhea with a standard formula used to feed healthy infants from birth. Methodology: Seventy infants with an average age of 163 days±11.7 suffering from acute diarrhea with an average of 6.6±0.4 stools per day received, after randomization, either a standard (control) formula (n =36) or an adapted lactose-free formula fortified with S. boulardii (26mg/100ml; n =34). The duration of the diarrhea was defined as the time needed until the occurrence of the first normal stool after the last liquid stool. Results: At inclusion, the age, weight, and number of stools per day were the same in the two groups. There were 15 infants with rotavirus in the treated group and 13 in the control group. The duration of the diarrhea from time of inclusion was significantly reduced in the treated group (35.4±3.7h) versus the control group (67.1±5h; p <0.001), In both groups, with or without rotavirus, the duration of diarrhea did not depend on the presence or absence of rotavirus but only on the treatment. The average daily weight gain was significantly higher in the treated group compared with the control group (74.2±26.4g versus 23.7±6.7g; p <0.05). The oral rehydration solution consumption was, however, higher in the control group than in the treated group; the consumption of formula was higher in the treated group, but these differences were not significant. The number of stools as of Day 4 remained significantly higher in the control group than in the treated group. After 6.5 days, almost no S. boulardii was found in the stools. Conclusion: In moderately dehydrated infants (<10 %), the use of a specially designed formula, containing S. boulardii (Novalac AD+/Diarinova+), significantly shortens the duration of diarrhea and allows quicker weight regain than a standard formula. [Copyright &y& Elsevier]

Summary: Gastric infection with Helicobacter heilmannii is rare but is known to be associated with chronic active gastritis, peptic ulcer, and low-grade mucosa-associated lymphoid tissue lymphoma in humans. In contrast to H. pylori, various H. heilmannii species colonize the stomachs of domestic animals, which might be a reservoir for transmission to humans. We report the case of a 12-year-old boy presenting with chronic gastritis caused by H. heilmannii. Endoscopic examination reveals H. heilmannii-like infection on biopsy samples in his two pet dogs. Sequencing of the 16S and 23S ribosomal DNA by PCR was used to compare the H. heilmannii-like bacterial samples isolated in the boy and his dogs. These DNA amplification methods suggest that the boy was infected by his pet dogs (zoonosis). Our patient was cured by treatment with proton-pump inhibitor and antibiotics. Endoscopic follow-up of the boy showed a complete cure of gastritis and eradication of the bacterium. [Copyright &y& Elsevier]

La constipation idiopathique de l''enfant est responsable de troubles fonctionnels variés, de fausses diarrhées et d''angoisses familiales importantes. Sa prise en charge fait appel au traitement médicamenteux et à un abord comportemental où l''enfant devient acteur de sa guérison en réapprenant à aller à la selle. Le traitement médical comprend dans un premier temps l''évacuation du fécalome puis le rétablissement d''un transit régulier par un traitement d''entretien. Des laxatifs osmotiques, des huiles lubrifiantes ou le polyéthylène glycol peuvent être prescrits pendant plusieurs mois jusqu''à la normalisation de la défécation. Un régime alimentaire équilibré et enrichi en fibres est important. La rééducation du réflexe d''exonération peut être effectuée par technique de suppositoires libérant du gaz carbonique ou par biofeedback dans certains troubles fonctionnels neuromoteurs révélés à la manométrie dans des cas de constipations sévères résistant au traitement médical. [Copyright 2004 Elsevier]

High-resolution X-rays imaging requires thin films of dense materials with high light yield under X-ray excitation. Polycrystalline films of europium-doped gadolinium and lutetium oxide have been prepared by sol–gel method. The scintillation performances of these compounds for X-rays imaging are presented and discussed. [Copyright &y& Elsevier]

A combined approach integrating bioenergetics and major biological activities is essential to properly understand the impact of microplastics (MP) on marine organisms. Following experimental exposure of polystyrene microbeads (micro-PS of 6 and 10 μm) at 0.25, 2.5, and 25 μg L−1, which demonstrated a dose-dependent decrease of energy balance in the pearl oyster Pinctada margaritifera, a transcriptomic study was conducted on mantle tissue. Transcriptomic data helped us to decipher the molecular mechanisms involved in P. margaritifera responses to micro-PS and search more broadly for effects on energetically expensive maintenance functions. Genes related to the detoxification process were impacted by long-term micro-PS exposure through a decrease in antioxidant response functioning, most likely leading to oxidative stress and damage, especially at higher micro-PS doses. The immune response was also found to be dose-specific, with a stress-related activity stimulated by the lowest dose present after a 2-month exposure period. This stress response was not observed following exposure to higher doses, reflecting an energy-limited capacity of pearl oysters to cope with prolonged stress and a dramatic shift to adjust to pessimum conditions, mostly limited and hampered by a lowered energetic budget. This preliminary experiment lays the foundation for exploring pathways and gene expression in P. margaritifera , and marine mollusks in general, under MP exposure. We also propose a conceptual framework to properly assess realistic MP effects on organisms and population resilience in future investigations. Image 1 • Antioxidant and detoxification processes are impacted by long-term polystyrene microbeads exposure in P. margaritifera. • Energy metabolism and immunity are dose specific response to micro-PS exposure. • Bioenergetics and transcriptomics refine the energy-limited tolerance to stress concept for emerging pollutants. [ABSTRACT FROM AUTHOR]

Nous rapportons l’observation d’un enfant dont la maladie d’Addison s’est manifestée initialement par une atteinte minéralo-corticoïde. Observation garçon de 8 ans hospitalisé pour syndrome de Kawasaki. Deuxième injection de gammaglobuline choc avec hyponatrémie (115 mmole/L), hyperkaliémie (5,5 mmole/L). Cortisolémie normale (319 nmol/L) ACTH et Renine augmentés (181pg/ml) 500mUi/LAldostérone effondrée (75 pmole/L), 18 OH corticostérone élevée (1247 pg/ml). Traitement de l’insuffisance surrénale. À plusieurs reprises cortisolèmie de 8 H est normal. Appétence pour le sel et rapport 18 OH corticostérone/Aldostérone élevé chez l’enfant et le père. Le diagnostic d’hyoaldostéronisme et traitement par fludrocortisone®. L’étude génétique retrouve une mutation non parlante du gèneCYP11B2. J25, récidive de la maladie de Kawasaki avec cholécystite acalculeuse. IRM surrénale, AGLC, tuberculose négatif. 7 mois plus tard, Cortisolémie effondré pas d’asthénie, de mélanodermie. Dosage des anticorps anti 21 hydroxylase, anti cellule pariétale, anti GAD positif, anticorps fait porter le diagnostic de poly endocrinopathie de type 2. 2 questions sont soulevées par cette observation : existe-t-il un lien entre poly endocrinopathie et vascularite, gamma globulines ont-ils été un facteur déclenchant ? [ABSTRACT FROM AUTHOR]

L’élévation de l’uricémie chez les patients atteints de mucoviscidose est classique. Elle est rapportée, soit à l’utilisation de fortes doses d’extrait pancréatique (1), soit à un turn over cellulaire important (2). La survenue d’une crise typique chez une adolescente de 18 ans, nous a incité à éntreentreprendre une étude du taux d’acide urique chez une cohorte de patients mucoviscidosique. Méthode: dosage de l’acide urique, de l’uricurie, du rapport uricurie/créatinurie, dose unitaire d’apport lipase/kg. Résultat: L’effectif comporte 27 enfants adolescents d’âge moyen 12.9 ans (5 ans-22 ans) réparti en 12 filles et 15 garçons. L’uricémie moyenne du garçon est plus élevée 310 ± 74μ Mole/l que chez la fille 288 ± 77μ Mole/l mais de façon non significative. Elle est élevée chez le sujet ayant présenté une goutte 451 μ Mole/l. L’uricurie des 24 H est en moyenne de 2.8 ± 0.9 mmole/24 H, plus élevée chez la patiente avec goutte (5.63 mmole/24 H). Les apports en extraits pancréatiques sont variables mais élevés : 10130 ± 6400 UI/kg mais la patiente avec goutte est celle qui recevait la plus faible dose 1344 UI/Kg. Conclusion: Les valeurs rapportées dans notre étude sont superposables aux données de la littérature (291 ± 12μ Mole/l). Dans notre série, il n’y a pas de relation entre les apports en extraits pancréatiques et les valeurs de l’uricémie. La crise de goutte chez les patients mucoviscidosique ne semble pas avoir jusqu’alors été rapportée (recherche Pubmed). [Copyright &y& Elsevier]

In this study, we examined the effect of near future ocean acidification (OA) on the transcriptome of a sensory organ in contact with surrounding water, the tongue in adult European sea bass (Dicentrarchus labrax) by mean of RNAseq experiment. We acquired a total of 14.1 Mb quality-trimmed reads covering 18,703 expressed genes from the tongue of fish reared from two generations at actual (pH 8.0 condition) and predicted near-future seawater pH (pH 7.6 condition). Gene ontologies analyses of expressed genes support the evidence that the tongue exhibits biological processes related to the sensory system, tooth mineralization and immune defences among others. Our data revealed only 295 OA-induced regulated genes with 114 up- and 181 down-regulated by OA. Functions over-represented encompass processes involved in organic substance metabolic process, RNA metabolism and especially RNA methylation which, combined with the regulation of some hsp genes expression, suggest a molecular response to stress which might contribute to lingual cell homeostasis under OA. The immune system process is also found enriched within OA-induced regulated genes. With the exception of one fatty acid receptor, known taste perception effectors were not impacted by OA in the tongue. However, a complementary droplet digital PCR approach dedicated to genes involved in gustatory signal transduction revealed the down regulation by OA of pyrimidinergic receptor (p2ry4) transcript expression in the gills of the fish. Combined with scanning electron microscopy analysis, our RNAseq data revealed that OA has no impact on processes related to teeth development and mineralization. Altogether, our data reveal that multigenerational exposure to OA has not a substantially effect on the tongue transcriptome but emphasis should be placed on investigating the potential physiological consequences related to the regulation of genes related to cell stress, immune system and fatty acid sensitivity to conclude on species resilience in face of OA. • Multigenerational exposure to OA has no impact on teeth mineralization in Sea bass. • Of the 18703 genes expressed in the tongue, 295 exhibited OA-induced regulation. • Genes related to cell stress, immune system and fatty acid sensitivity are regulated. • OA impacts the branchial expression of p2ry4 gene involved in gustatory system. [ABSTRACT FROM AUTHOR]

[Display omitted] • Bio-based N-acetylhomocysteine thiolactone was used in anionic copolymerization. • Well-defined alternating poly(ester- alt -thioether)s are synthesized. • Lateral acetamido groups induced large increase of glass transition temperatures. The anionic ring-opening polymerizations (AROP) of bio-based N-acetyl homocysteine thiolactone (NHTL) and different epoxides were carried out using benzyl alcohol and 2- tert -butylimino-2-diethylamino-1,3-dimethylperhydro-1,3,2-diazaphosphorine (BEMP) as initiating system. This polymerization is a rare example of AROP in the presence of an acidic moiety (acetamido group). Well-defined alternating poly(ester- alt -sulfide)s are obtained with number-average molar masses M n ranging from 1.7 to 13.0 kg mol−1 and dispersities as low as 1.14. The presence of one acetamido function in the lateral group on each repeating unit of the copolymers derived from NHTL results in very significant increases (up to 94 °C) of the glass transition temperature T g compared to similar poly(ester- alt -sulfide) derived from petro-based γ-butyrothiolactone (BTL). These functional poly(NHTL- alt -epoxide)s are valuable structures with numerous potential applications due to the presence in each repeating unit of one cleavable ester group and one redox-sensitive thioether group. [ABSTRACT FROM AUTHOR]

Abstract: We report a case of macroamylasemia in an 11-year-old boy. We compare our clinical and paraclinical data with those described in pediatric literature. Macroamylase resulted in a complex of amylase and immuglobulin. Its fortuitous detection did not reveal, up to now, any associated pathology, in particular any autoimmune disorders or celiac disease. Identification of this biochemical abnormality is essential in order to avoid invasive investigations and/or unnecessary therapies. [Copyright &y& Elsevier]

Abstract: Climatotherapy has been shown to have beneficial effects in asthma. Recent studies confirm the improvement of obstructive pulmonary disease, regression of bronchial hyperreactivity, and diminution of markers of inflammation during stays at high altitude. Reduction in the level of allergen is considered to be one of the most significant consequences of climatotherapy. In addition to its direct therapeutic benefits, climatotherapy incorporates comprehensive care including patient education and physical rehabilitation for asthmatic patients. [Copyright &y& Elsevier]

Nosocomial infections are a preoccupation in a pediatric hospital mainly during the winter with bronchiolitis and gastroenteritis epidemics. We have examined the risk factors of nosocomial infections.Material and methods. – A prospective study was conducted between November, 1999 and March, 2000 in the infants units of the Le Havre hospital. We systematically listed the admissions and contacted the family after their discharge by phone. A geographic information system was implemented to display the epidemiological data; this software is able to illustrate the sectors at risk.Results. – During the study, 687 infants were hospitalized of whom 458 for bronchiolitis and community-acquired gastroenteritis. Mean age was 5.4 months old. No nosocomial bronchiolitis occured. Prevalence of nosocomial gastroenteritis was 10% (68 cases including nine after discharge). Infants with nosocomial infection were younger than those with community-acquired infection (6.6 months vs. 11.2 months, P < 0.01). The mean length of stay was longer in nosocomial infection (7.7 vs. 4.1 days, P < 0.05). Among the infants with bronchiolitis, 16% have developed nosocomial intestinal infections (RR = 2.65, IC: 1.59–4.4; P < 0.01). The geographic analysis pointed the area with nosocomial risk (bedroom without water, nearness of nurse office and games room).Conclusion. – Geographic information system is a part of the quality control system and may have some interaction effect on final decision making. Incidence of nosocomial infections showed the need for a prevention strategy in a pediatric hospital. [Copyright &y& Elsevier]

Hyperuricemia is a well-known consequence of glucose-6-phosphatase (G6Pase) deficiency, the enzymatic abnormality that characterizes glycogen storage disease (GSD) Type Ia. However, acute gout as the presenting manifestation of GSD Type Ia has been reported in only a few patients. We report a new case in a 17-year-old male evaluated for acute gouty tendinitis in the right Achilles tendon. Blood tests showed chronic acidosis with high levels of uric acid, lactic acid, and cholesterol. A liver enzyme study confirmed the diagnosis of GSD Type Ia. A genetic study showed that the index patient and his sister were composite heterozygotes for the known mutation R83C and the previously unreported mutation M5R. Acute gout in an adolescent with liver enlargement and high blood levels of uric acid and cholesterol should suggest GSD. Demonstration by molecular biology techniques of a mutation in both alleles of the G6Pase gene establishes the diagnosis of GSD Type Ia, obviating the need for a liver biopsy. [Copyright &y& Elsevier]

Introduction. – Hyperuricemia is a known consequence of glucose-6-phosphatase deficiency. Very few cases of tophaceous gout revealing a glycogen storage disease type 1a have been described.Case report. – A 17 years-old adolescent presents with tophaceous gout of Achilles’ right heel. The diagnosis of glycogen storage disease type 1a is suggested by blood chemical studies ( hyperlactacidemia, hypercholesterolemia) and confirmed by hepatic enzyme dosage. Mutation analysis reveals a novel mutation. Both the index case and his sister are compound heterozygotes for the mutations R83C/M5R.Conclusion. – When a mutation in the glucose-6-phosphatase gene is discovered, be it a homozygous or heterozygous mutation and when associated with evocative symptoms, then the diagnosis of glycogen storage disease type 1a can be made without hepatic biopsies. [Copyright &y& Elsevier]

To investigate and describe the variation in enamel daily secretion rates (DSRs) of naturally exfoliated deciduous molars (n = 345) from five modern-day populations (Aotearoa New Zealand, Britain, Canada, France, and Sweden). Each tooth was thin sectioned and examined using a high-powered Olympus BX51 microscope and DP25 digital microscope camera. Mean DSRs were recorded for the inner, mid, and outer regions of cuspal and lateral enamel, excluding enamel nearest the enamel-dentin junction and at the outermost crown surface. Mean DSRs did not vary significantly between populations, or by sex. Cuspal enamel grew slightly faster than lateral enamel (mean difference 0.16 µm per day; p < 0.001). The trajectory of DSRs remained relatively constant from inner to outer cuspal enamel and increased slightly in lateral enamel (p = 0.003). The DSRs of deciduous molars from modern-day children are remarkably consistent when compared among populations. While growth rates are faster in cuspal than lateral enamel, the trajectory of enamel formation changes only slightly from inner to outer regions. The trajectory of DSRs for deciduous molars differs to that of permanent molar enamel, which typically display a steep increase in matrix deposition from inner to outer enamel. • Daily secretion rates (DSR) reported for deciduous molars from global sample. • Deciduous molar enamel DSRs show limited variation between populations. • Deciduous molars show minor increase in DSRs from inner to outer enamel. [ABSTRACT FROM AUTHOR]

Nous rapportons un cas de pancréatite chronique chez une fillette de 12 ans présentant une mutation des gènes PRSS1 (pR116C) et SPINK1 (pN34S). Observation: A l’âge de 10 ans, une fille sans antécédents particuliers ni familiaux est hospitalisée pour une pancréatite (amylasémie 848 UI/ml, lipasémie 687 UI/ml). Le bilan étiologique y compris malformatif est négatif. Les suites sont marquées par l’apparition d’un faux kyste du pancréas résolutif sous repos digestif et alimentation parentérale. A l’âge de 12 ans survient une récidive de la pancréatite (amylasémie 1200 UI/ml, lipasémie 2175 UI/ml). La radiographie d’abdomen sans préparation met en évidence des calcifications pancréatiques. Le bilan para clinique (cholestérol, triglycéride, PTH) de même que le biliscan est normal. Une enquête génétique est réalisée et porte sur les gènes CFTR, PRSS1, SPINK1). Elle retrouve une mutation hétérozygote pR116C du géne PRSS1, une mutation pN34S du gène SPINK1. Aucune mutation du géne CFTR n’est retrouvée Discussion: Cette double mutation n’a jamais été rapportée dans les cas de pancréatite récidivante. La mutation hétérozygote pR116C exprimée par cette patiente est un variant rare, entraînant la substitution d’une arginine par une cystéine, mise en évidence dans des cas de PC sporadiques. La mutation N34S, entraîne une perte de fonction de la protéine et diminue ainsi l’inactivation du trypsinogène par SPINK1. L’étude des gènes impliqués dans les pancréatites d’origine génétique présente un grand intérêt : la mise en évidence de mutation permet en effet d’expliquer un grand nombre des pancréatites considérées jusqu’à présent comme idiopathiques et de délivrer au patient des recommandations hygiéno-diététiques, afin de limiter les facteurs de risque associés. [Copyright &y& Elsevier]