12 research outputs found

    Kyphoscolitic Type of Ehlers-Danlos Syndrome with Prenatal Stroke.

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    Place: IndiaBACKGROUND: The kyphoscoliotic type of Ehlers-Danlos syndrome (EDS type VIA) is an autosomal recessive disorder characterized by connective tissue dysplasia. CASE CHARACTERISTICS: We report two children with perinatal stroke; accompanied by neonatal joint hypermobility, hypotonia; and early development of kyphoscoliosis. OUTCOME: Molecular analysis revealed a PLOD1 gene mutation. Our definitive diagnosis was a EDS VIA. MESSAGE: Prenatal brain stroke is a rare clinical feature of EDSVIA

    Bioconversion of agricultural lignocellulosic residues into branched-chain fatty acids using Streptomyces lividans

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    Two lignocellulosic agricultural residues, sunflower stalks and rape straw, were investigated as potential low-cost, non-food substrates for the production of triacylglycerols by the oleaginous, lignocellulolytic bacteria Streptomyces lividans. Chemical analysis of each type of residue revealed similar cell wall compositions in the polysaccharides and lignins of the two feedstocks, with high lignin β-O-4 bond content compared to other angiosperms’ lignin. Growing tests of Streptomyces lividans TK 24 were performed before and after sequential water and ethanol extraction by assessing bacterial fatty acid accumulation. All extracted and non-extracted samples were found to be substrates of the bacteria with fatty acid production ranging between 19% and 44% of the production obtained with arabinose as a reference substrate. The maximum conversion rate was obtained with the less lignified, non-extracted sample. This study suggests that lignocellulosic residues from oleaginous crops could be advantageously valorized by microbial bioconversion processes for the production of lipids of interest

    Bioconversion of agricultural lignocellulosic residues into branched-chain fatty acids using

    No full text
    Two lignocellulosic agricultural residues, sunflower stalks and rape straw, were investigated as potential low-cost, non-food substrates for the production of triacylglycerols by the oleaginous, lignocellulolytic bacteria Streptomyces lividans. Chemical analysis of each type of residue revealed similar cell wall compositions in the polysaccharides and lignins of the two feedstocks, with high lignin β-O-4 bond content compared to other angiosperms’ lignin. Growing tests of Streptomyces lividans TK 24 were performed before and after sequential water and ethanol extraction by assessing bacterial fatty acid accumulation. All extracted and non-extracted samples were found to be substrates of the bacteria with fatty acid production ranging between 19% and 44% of the production obtained with arabinose as a reference substrate. The maximum conversion rate was obtained with the less lignified, non-extracted sample. This study suggests that lignocellulosic residues from oleaginous crops could be advantageously valorized by microbial bioconversion processes for the production of lipids of interest

    Etude de la production de lipides par <em>Streptomyces</em> à partir de résidus lignocellulosiques

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    Dans le contexte actuel d’épuisement des ressources fossiles et de protection de l’environnement, la valorisation énergétique des huiles issues de la biomasse et la chimie verte prennent de l’importance. En effet, ces huiles et leurs dérivés biodégradables peuvent venir en remplacement des produits d'origine fossile. Ils sont de plus en plus retrouvés dans les produits de grande consommation (savon, produits d’entretien) ou industriels (solvants, lubrifiants). Deux sources sont envisagées, celles des huiles végétales déjà bien implantées, et celle des huiles produites à partir de microorganismes, actuellement en plein essor. Parmi les microorganismes, Streptomyces lividans est une bactérie des sous-bois qui présente l’avantage d’être capable d’exploiter des matières premières ligno-cellulosiques comme source de carbone pour sa production de biomasse (Antai et al., 1981 ; Davis et al., 2010). Par ailleurs, dans certaines conditions de culture, mises au point dans l'équipe Métabolisme Energétique des Streptomyces, il a été montré que cette bactérie était capable de produire de l'huile sous forme d'inclusions cytoplasmiques, les corps lipidiques (Kaddor et al., 2009). Ce travail a reçu le soutien financier du PRES Universud pour 2010 et les premiers résultats montrent que la souche Streptomyces lividans TK24 stocke jusqu'à 25% de sa masse sèche en acides gras (majoritairement des acides gras impairs en C15 et branchés), essentiellement sous forme de triglycérides

    Etude de la production de lipides par <em>Streptomyces</em> à partir de résidus lignocellulosiques

    No full text
    Dans le contexte actuel d’épuisement des ressources fossiles et de protection de l’environnement, la valorisation énergétique des huiles issues de la biomasse et la chimie verte prennent de l’importance. En effet, ces huiles et leurs dérivés biodégradables peuvent venir en remplacement des produits d'origine fossile. Ils sont de plus en plus retrouvés dans les produits de grande consommation (savon, produits d’entretien) ou industriels (solvants, lubrifiants). Deux sources sont envisagées, celles des huiles végétales déjà bien implantées, et celle des huiles produites à partir de microorganismes, actuellement en plein essor. Parmi les microorganismes, Streptomyces lividans est une bactérie des sous-bois qui présente l’avantage d’être capable d’exploiter des matières premières ligno-cellulosiques comme source de carbone pour sa production de biomasse (Antai et al., 1981 ; Davis et al., 2010). Par ailleurs, dans certaines conditions de culture, mises au point dans l'équipe Métabolisme Energétique des Streptomyces, il a été montré que cette bactérie était capable de produire de l'huile sous forme d'inclusions cytoplasmiques, les corps lipidiques (Kaddor et al., 2009). Ce travail a reçu le soutien financier du PRES Universud pour 2010 et les premiers résultats montrent que la souche Streptomyces lividans TK24 stocke jusqu'à 25% de sa masse sèche en acides gras (majoritairement des acides gras impairs en C15 et branchés), essentiellement sous forme de triglycérides

    Predictive value of brain MRI at term-equivalent age in extremely preterm children on neurodevelopmental outcome at school-age.

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    Place: United StatesThis study's objective was to correlate the abnormalities in brain MRIs performed at corrected-term age for minor or moderate neurocognitive disorders in children school-age born extremely premature (EPT) and without serious sequelae such as autism, cerebral palsy, mental impairment. Data were issued from a cross-sectional multicenter study (GP-Qol study, number NCT01675726). Clinical examination and psychometric assessments were performed when the children were between 7 and 10 years old during a day-long evaluation. Term-equivalent age brain MRIs on EPT were analyzed with a standardized scoring system. There were 114 children included in the study. The mean age at the time of evaluation, was 8.47 years old (± 0.70). 59% of children with at least one cognitive impairment and 53% who had a dysexecutive disorder. Only ten EPT (8.7%) presented moderate to severe white and grey matter abnormalities. These moderate to severe grey matter abnormalities were associated with at least two abnormal executive functions [OR 3.08 (95% CI 1.04-8.79), p = 0.04] and language delay [OR 3.25 (95% CI 1.03-9.80), p = 0.04]. These results remained significant in the multivariate analysis. Moderate to severe ventricular dilatation abnormalities (15%, n = 17) were associated with ideomotor dyspraxia [OR 7.49 (95% CI 1.48-35.95), p = 0.02] and remained significant in multivariate analysis [OR 11.2 (95% CI 1.45-131.4), p = 0.02]. Biparietal corrected diameters were moderate abnormal in 20% of cases (n = 23) and were associated to visuo spatial integration delay [OR 4.13 (95% CI 1.23-13.63), p = 0.02]. Cerebral MRI at term-equivalent age with scoring system analysis can provide information on long-term neuropsychological outcomes at school-age in EPTs children having no severe disability

    Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition

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    In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. Subjects and Methods: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. Results: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children′s hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. Conclusion: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation

    Etude de la production de lipides par Streptomyces à partir de résidus lignocellulosiques

    No full text
    Dans le contexte actuel d’épuisement des ressources fossiles et de protection de l’environnement, la valorisation énergétique des huiles issues de la biomasse et la chimie verte prennent de l’importance. En effet, ces huiles et leurs dérivés biodégradables peuvent venir en remplacement des produits d'origine fossile. Ils sont de plus en plus retrouvés dans les produits de grande consommation (savon, produits d’entretien) ou industriels (solvants, lubrifiants). Deux sources sont envisagées, celles des huiles végétales déjà bien implantées, et celle des huiles produites à partir de microorganismes, actuellement en plein essor. Parmi les microorganismes, Streptomyces lividans est une bactérie des sous-bois qui présente l’avantage d’être capable d’exploiter des matières premières ligno-cellulosiques comme source de carbone pour sa production de biomasse (Antai et al., 1981 ; Davis et al., 2010). Par ailleurs, dans certaines conditions de culture, mises au point dans l'équipe Métabolisme Energétique des Streptomyces, il a été montré que cette bactérie était capable de produire de l'huile sous forme d'inclusions cytoplasmiques, les corps lipidiques (Kaddor et al., 2009). Ce travail a reçu le soutien financier du PRES Universud pour 2010 et les premiers résultats montrent que la souche Streptomyces lividans TK24 stocke jusqu'à 25% de sa masse sèche en acides gras (majoritairement des acides gras impairs en C15 et branchés), essentiellement sous forme de triglycérides
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