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3,848 results on '"Bouvier R"'

1. Mid-term results demonstrate salvage high-intensity focused ultrasound (HIFU) as an effective and acceptably morbid salvage treatment option for locally radiorecurrent prostate cancer: Murat FJ, Poissonnier L, Rabilloud M, Belot A, Bouvier R, Rouviere O, Chapelon JY, Gelet A, Urology Department, Val d'Ouest Hospital, Ecully, France; Urology and Transplantation Department, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France

Author

Smith, Joseph A.

Published
2008
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2. Neurodevelopmental deficits in Pierson (microcoria‐congenital nephrosis) syndromeHow to cite this article: Wühl E, Kogan J, Zurowska A, Matejas V, Vandevoorde RG, Aigner T, Wendler O, Lesniewska I, Bouvier R, Reis A, Weis J, Cochat P, Zenker M. 2007. Neurodevelopmental deficits in Pierson (microcoria‐congenital nephrosis) syndrome. Am J Med Genet Part A 143A:311–319.

Author

Wühl, Elke, Kogan, Jillene, Zurowska, Aleksandra, Matejas, Verena, Vandevoorde, Rene G., Aigner, Thomas, Wendler, Olaf, Lesniewska, Iga, Bouvier, Raymonde, Reis, André, Weis, Joachim, Cochat, Pierre, and Zenker, Martin

Abstract

Pierson syndrome is an autosomal recessive disorder comprising congenital nephrotic syndrome with diffuse mesangial sclerosis and distinct eye abnormalities with microcoria reported as the most prominent clinical feature. LAMB2 mutations leading to lack of laminin β2 were identified as the molecular cause underlying Pierson syndrome. Although LAMB2 is known to be expressed in the neuromuscular system, and defects of the neuromuscular junctions had been found in laminin β2‐deficient mice, no consistent neurological phenotype has been described clinically in murine or human laminin β2‐deficiency before. This is likely due to the early lethality from renal failure. Here we provide a detailed description of neurological manifestations and development in four patients affected by Pierson syndrome, who survived until the age of 1.3–4.8 years owing to renal replacement therapy. Severe muscular hypotonia, psychomotor retardation, and blindness were present in three patients harboring truncating mutations on both LAMB2 alleles. These symptoms were not attributable to complications of chronic renal failure, thus representing a primary feature of the genetic disorder. Alterations in skeletal muscle tissue from one case were compatible with a chronic denervating process. One affected girl, however, exhibited a milder course of renal disease, normal development, and preserved vision, presumably owing to some residual LAMB2 function. Our findings indicate that severe neurodevelopmental deficits have to be considered as part of Pierson syndrome, at least in the presence of biallelic functional null mutations (complete lack of laminin β2). This is an important issue in the counseling of parents of an affected newborn or infant. © 2007 Wiley‐Liss, Inc.

Published
2007
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3. SICANE: a Detector Array for the Measurement of Nuclear Recoil Quenching Factors using Monoenergetic Neutron Beam

Author

Simon, E., Berge, L., Broniatowski, A., Bouvier, R., Chambon, B., De Jesus, M., Drain, D., Dumoulin, L., Gascon, J., Hadjout, J. P., Juillard, A., Martineau, O., Pastor, C., Stern, M., and Vagneron, L.

Subjects
Astrophysics
Abstract

SICANE is a neutron scattering multidetector facility for the determination of the quenching factor (ratio of the response to nuclear recoils and to electrons) of cryogenic detectors used in direct WIMP searches. Well collimated monoenergetic neutron beams are obtained with inverse (p,n) reactions. The facility is described, and results obtained for the quenching factors of scintillation in NaI(Tl) and of heat and ionization in Ge are presented., Comment: 30 pages, Latex, 11 figures. Submitted to NIM A

Published
2002
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4. Dark Matter Search in the EDELWEISS experiment

Author

Juillard, A., Benoit, A., Berge, L., Bonnevaux, A., Bouvier, R., Broniatowski, A., Chambon, B., Chapellier, M., Chardin, G., Charvin, P., Cluzel, P., De Jesus, M., Di Stefano, P., Drain, D., Dumoulin, L., Gascon, J., Gerbier, G., Goldbach, C., Goyot, M., Gros, M., Hadjout, J. P., De Lesquen, A., Loidl, M., Loiseau, D., Mallet, J., Marnieros, S., Martineau, O., Mirabolfathi, N., Mosca, L., Navick, X-F., Nollez, G., Pari, P., Pastor, C., Simon, E., Stern, M., and Vagneron, L.

Subjects
Astrophysics
Abstract

The EDELWEISS Dark Matter Search uses low-temperature Ge detectors with heat and ionisation read-out to identify nuclear recoils induced by elastic collisions with WIMPs from the galactic halo. Preliminary results obtained with 320g bolometers are described. After a few weeks of data taking, data accumulated with one of these detectors already allow to reach the upper part of the DAMA region. Prospects for the present run and the second stage of the experiment, EDELWEISS-II, using an innovative reversed cryostat allowing data taking with 100 detectors, are briefly described., Comment: 4 pages, LaTeX, 3 figures, contribution to " Very High Energy Phenomena in the Universe ", Les Arcs, France (January 20-27, 2001)

Published
2001

5. Risk factors associated with Crimean-Congo hemorrhagic fever virus circulation among human, livestock and ticks in Mauritania through a one health retrospective study.

Author

El Ghassem A, Apolloni A, Vial L, Bouvier R, Bernard C, Khayar MS, Cheikh Ahmed M, Fausther-Bovendo H, Beyit AD, Yahya B, Ould El Mamy MB, Elbara A, Bollahi MA, Cêtre-Sossah C, and Ould Mohamed Salem Boukhary A

Subjects
Humans, Animals, Cattle, Sheep, Livestock, Retrospective Studies, Mauritania, Goats, Antibodies, Viral, RNA, Risk Factors, Seroepidemiologic Studies, Hemorrhagic Fever Virus, Crimean-Congo, Ticks, Hemorrhagic Fever, Crimean epidemiology, One Health, Ixodidae
Abstract

Background: Crimean Congo hemorrhagic fever (CCHF) is endemic in Southern Mauritania where recurrent outbreaks have been constantly observed since the 1980's. The present study is the first to assess CCHFV antibodies and RNA in humans., Methods: A retrospective study was conducted using 263 humans and 1380 domestic animals serum samples, and 282 tick specimens of Hyalomma genus collected from 54 settings in 12 provinces across Mauritania. Antibodies targeting CCHF viral nucleoprotein were detected in animal and human sera using double-antigen ELISA. CCHFV specific RNA was detected in human and animal sera as well as tick supernatants using a CCHFV real time RT-PCR kit. Individual characteristics of sampled hosts were collected at the same time and data were geo-referenced. Satellite data of several environmental and climatic factors, were downloaded from publicly available datasets, and combined with data on livestock mobility, animal and human density, road accessibility and individual characteristics to identify possible risk factors for CCHFV spatial distribution. To this end, multivariate logistic models were developed for each host category (human, small and large ruminants)., Results: The overall CCHFV antibody prevalence was 11.8% [95% CI: 8.4-16.3] in humans (17.9% in 2020 and 5.4% in 2021; p = 0.0017) and 33.1% (95% CI: 30.1-36.3) in livestock. CCHFV-specific antibodies were detected in 91 (18.1%) out of 502 sheep, 43 (9.0%) out of 477 goats, 144 (90.5%) out of 161 dromedaries and 179 (74.6%) out of 240 cattle. CCHFV RNA was detected in only 2 (0.7%) sera out of 263 animals herders samples from Hodh El Gharbi province and in 32 (11.3%) out of 282 Hyalomma ticks. In humans as well as in animals, seropositivity was not associated with sex or age groups. The multivariate analysis determined the role of different environmental, climatic and anthropic factors in the spatial distribution of the disease with animal mobility and age being identified as risk factors., Conclusion: Results of the present study demonstrate the potential risk of CCHF for human population in Mauritania primarily those living in rural areas in close vicinity with animals. Future studies should prioritize an integrative human and veterinary approach for better understanding and managing Crimean-Congo hemorrhagic fever., (© 2023. The Author(s).)

Published
2023
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7. Dark Matter Search in the EDELWEISS Experiment

Author

Juillard, A., Benoit, A., Bergé, L., Bouvier, R., Broniatowski, A., Caussignac, M., Chabert, L., Chambon, B., Chapellier, M., Chardin, G., Charvin, P., De Jésus, M., Di Stefano, P., Drain, D., Dumoulin, L., Gascon, J., Gerbier, G., Goldbach, C., Goyot, M., Gros, M., Hadjout, J. P., Hervé, S., de Lesquen, A., Loidl, M., Mallet, J., Marnieros, S., Martin, M., Martineau, O., Mirabolfathi, N., Mosca, L., Navick, X.-F., Nollez, G., Pari, P., Riccio, C., Rodenas, H., Shoeffel, L., Stern, M., Vagneron, L., Klapdor-Kleingrothaus, H. V., editor, and Viollier, R. D., editor

Published
2002
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9. Are protocol graft biopsies after pediatric liver transplantation useful? Experience in a single center over 20 years.

Author

Wischlen E, Boillot O, Rivet C, Lachaux A, Bouvier R, Hervieu V, Scoazec JY, Collardeau-Frachon S, Dumortier J, and Laverdure N

Subjects
Child, Humans, Liver pathology, Retrospective Studies, Immunosuppressive Agents therapeutic use, Graft Rejection diagnosis, Graft Rejection etiology, Graft Rejection pathology, Biopsy, Liver Transplantation adverse effects
Abstract

Background: The role of protocol liver biopsies (PLB) in the follow-up of pediatric liver transplant recipients remains questionable. This single-center retrospective study aimed to evaluate their clinical impact on the long-term management of pediatric liver transplant recipients., Methods: We described histopathological lesions and clinical consequences for patient management of PLB performed 1, 5, 10, 15, 20, and 25 years after pediatric liver transplantation (LT)., Results: A total of 351 PLB performed on 133 patients between 1992 and 2021 were reviewed. PLB found signs of rejection in 21.7% of cases (76/351), and moderate to severe fibrosis in 26.5% of cases (93/351). Overall, 264 PLB (75.2%) did not cause any changes to patient care. Immunosuppression was enhanced after 63 PLB, including 23 cases of occult rejection. The 1-year PLB triggered significantly more changes, while biopsies at 15, 20, and 25 years produced the lowest rates of subsequent modifications. PLB had a significantly higher probability of inducing therapeutic changes if the patient had abnormal biological or imaging results (odds ratio [OR] 2.82 and 2.06), or a recent history of rejection or bacterial infection (OR 2.22 and 2.03)., Conclusion: Our results suggest that, although it often does not prompt any treatment changes, PLB could be performed because of its ability to detect silent rejection requiring an increase in immunosuppression. PLB could be carried out 1, 5, and 10 years after LT and then every 10 years in patients with normal biological and imaging results and no recent complications, while other patients could be kept on a 5-year protocol., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Published
2023
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35. Dark matter search in the EDELWEISS experiment

Author

Martineau, O., Benoit, A., Bergé, L., Bouvier, R., Broniatowski, A., Caussignac, M., Chabert, L., Chambon, B., Chapellier, M., Chardin, G., Charvin, P., De Jésus, M., Di Stefano, P., Drain, D., Dumoulin, L., Gascon, J., Gerbier, G., Goldbach, C., Goyot, M., Gros, M., P. Hadjout, J., Hervé, S., Juillard, A., de Lesquen, A., Loidl, M., Mallet, J., Marnieros, S., Martin, M., Mirabolfathi, N., Mosca, L., Navick, X.-F., Nollez, G., Pari, P., Riccio, C., Rodenas, H., Shoeffel, L., Stern, M., and Vagneron, L.

Published
2002
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40. Desmoplastic small round cell tumors of the abdomen

Author

Frappaz, D., Bouffet, E., Dolbeau, D., Bouvier, R., Carrie, C., Louis, D., Pondarre, Cori, Tabone, E., Philip, T., and Brunat-Mentigny, M.

Subjects
Tumors in children -- Diagnosis, Pelvic cancer -- Diagnosis, Cancer cells -- Physiological aspects, Health
Abstract

Background Desmoplastic small round cell tumors (DSRCT) have been only recently identified. Methods. The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization. Results. These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT. Conclusions. The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted. Cancer 1994; 73:1753-6. Key words: children, desmoplastic tumors, sarcomas, small round cell tumors.

Published
1994

48. Microscopic and ultrastructural features in Wolcott-Rallison syndrome, a permanent neonatal diabetes mellitus: about two autopsy cases

Author

Collardeau-Frachon, S., Vasiljevic, A., Jouvet, A., Bouvier, R., Senee, V., Nicolino, Marc, Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL)

Subjects
[SDV]Life Sciences [q-bio]
Abstract

International audience; BACKGROUND: Wolcott-Rallison syndrome (WRS) is a rare autosomal recessive disorder characterized by the association of permanent neonatal or early-infancy insulin-dependent diabetes, multiple bone dysplasia, hepatic dysfunction, and growth retardation. All clinical manifestations result from gene mutations encoding pancreatic endoplasmic reticulum eIF2 alpha kinase (PERK), an endoplasmic reticulum transmembrane protein that plays a role in the unfolded protein response. Histological and ultrastructural lesions of bone and pancreas have been described in animal models and WRS patients. However, histological and ultrastructural findings of other organs, especially of the liver, are lacking. METHODS: Autopsy specimens from two pediatric patients with WRS were analyzed. An immunohistochemical study was performed on the pancreas. An ultrastructural study was realized from samples of liver, pancreas, kidney, and myocardium. Our findings were compared with those of the literature and correlated with the molecular data. RESULTS: Hepatocytes and pancreatic exocrine cells exhibited very peculiar features of necrosis suggestive of secondary changes because of endoplasmic reticulum overload. Steatosis occurred in renal tubular cells, hepatocytes, and myocardial fibers. Abnormal mitochondria were noted in renal and myocardial fibers. Pancreas islets were characterized by a marked reduction in the number of insulin-secreting beta cells. CONCLUSIONS: The histological and ultrastructural features that occur in WRS are directly or indirectly linked to endoplasmic reticulum (ER) dysfunction and can explain the peculiar phenotype of this syndrome.

Published
2015

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