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1. Characterization and performance of the Apollon Short-Focal-Area facility following its commissioning at 1 PW level

Author

Burdonov, K., Fazzini, A., Lelasseux, V., Albrecht, J., Antici, P., Ayoul, Y., Beluze, A., Cavanna, D., Ceccotti, T., Chabanis, M., Chaleil, A., Chen, S. N., Chen, Z., Consoli, F., Cuciuc, M., Davoine, X., Delaneau, J. P., d'Humières, E., Dubois, J-L., Evrard, C., Filippov, E., Freneaux, A., Forestier-Colleoni, P., Gremillet, L., Horny, V., Lancia, L., Lecherbourg, L., Lebas, N., Leblanc, A., Ma, W., Martin, L., Negoita, F., Paillard, J-L., Papadopoulos, D., Perez, F., Pikuz, S., Qi, G., Quéré, F., Ranc, L., Söderstrom, P. -A., Scisciò, M., Sun, S., Vallières, S., Wang, P., Yao, W., Mathieu, F., Audebert, P., and Fuchs, J.

Subjects
Physics - Plasma Physics
Abstract

We present the results of the first commissioning phase of the ``short focal length'' area (SFA) of the Apollon laser facility (located in Saclay, France), which was performed with the first available laser beam (F2), scaled to a nominal power of one petawatt. Under the conditions that were tested, this beam delivered on target pulses of 10 J average energy and 24 fs duration. Several diagnostics were fielded to assess the performance of the facility. The on-target focal spot, its spatial stability, the temporal intensity profile prior to the main pulse, as well as the resulting density gradient formed at the irradiated side of solid targets, have been thoroughly characterized, with the goal of helping users design future experiments. Emissions of energetic electrons, ions, and electromagnetic radiation were recorded, showing good laser-to-target coupling efficiency and an overall performance comparable with that of similar international facilities. This will be followed in 2022 by a further commissioning stage at the multi-petawatt level.

Published
2021
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2. Gain of Aggressive Histological and Molecular Patterns after Acquired Resistance to Novel Anti-EGFR Therapies in Non-Small Cell Lung Cancer

Author

Clémence Basse, Olfa Trabelsi-Grati, Julien Masliah, Céline Callens, Maud Kamal, Paul Freneaux, Jerzy Klijanienko, Ivan Bieche, and Nicolas Girard

Subjects
novel anti-EGFR, resistance, lung adenocarcinoma, SCLC-like, RB1, TP53, Biology (General), QH301-705.5, Chemistry, QD1-999
Abstract

Novel anti-EGFR therapies target resistance to standard-of-care anti-EGFR in patients with metastatic lung cancer. We describe tumors at progression versus at the initiation of novel anti-EGFR agents in patients with metastatic lung adenocarcinoma harboring EGFR mutation. This clinical case series reports the histological and genomic features and their evolution following disease progression under amivantamab or patritumab-deruxtecan in clinical trials. All patients had a biopsy at disease progression. Four patients harboring EGFR gene mutations were included. Three of them received anterior anti-EGFR treatment. Median delay to disease progression was 15 months (range: 4–24). At progression, all tumors presented a mutation in the TP53 signaling pathway associated with a loss of heterozygosis (LOH) of the allele in 75% (n = 3), and two tumors (50%) presented an RB1 mutation associated with LOH. Ki67 expression increased above 50% (range 50–90%) in all samples compared to baseline (range 10–30%), and one tumor expressed a positive neuroendocrine marker at progression. Our work reports the potential molecular mechanisms of resistance under novel anti-EGFR in patients with metastatic EGFR-mutated lung adenocarcinoma, with the transformation to a more aggressive histology with acquired TP53 mutation and/or the increase in Ki67 expression. These characteristics are usually found in aggressive Small Cell Lung Cancer.

Published
2023
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3. Characterization and performance of the Apollon short-focal-area facility following its commissioning at 1 PW level

Author

K. Burdonov, A. Fazzini, V. Lelasseux, J. Albrecht, P. Antici, Y. Ayoul, A. Beluze, D. Cavanna, T. Ceccotti, M. Chabanis, A. Chaleil, S. N. Chen, Z. Chen, F. Consoli, M. Cuciuc, X. Davoine, J. P. Delaneau, E. d’Humières, J.-L. Dubois, C. Evrard, E. Filippov, A. Freneaux, P. Forestier-Colleoni, L. Gremillet, V. Horny, L. Lancia, L. Lecherbourg, N. Lebas, A. Leblanc, W. Ma, L. Martin, F. Negoita, J.-L. Paillard, D. Papadopoulos, F. Perez, S. Pikuz, G. Qi, F. Quéré, L. Ranc, P.-A. Söderström, M. Scisciò, S. Sun, S. Vallières, P. Wang, W. Yao, F. Mathieu, P. Audebert, and J. Fuchs

Subjects
Nuclear and particle physics. Atomic energy. Radioactivity, QC770-798
Abstract

We present the results of the first commissioning phase of the short-focal-length area of the Apollon laser facility (located in Saclay, France), which was performed with the first available laser beam (F2), scaled to a nominal power of 1 PW. Under the conditions that were tested, this beam delivered on-target pulses of 10 J average energy and 24 fs duration. Several diagnostics were fielded to assess the performance of the facility. The on-target focal spot and its spatial stability, the temporal intensity profile prior to the main pulse, and the resulting density gradient formed at the irradiated side of solid targets have been thoroughly characterized, with the goal of helping users design future experiments. Emissions of energetic electrons, ions, and electromagnetic radiation were recorded, showing good laser-to-target coupling efficiency and an overall performance comparable to that of similar international facilities. This will be followed in 2022 by a further commissioning stage at the multi-petawatt level.

Published
2021
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4. A Weibull Approach for Enabling Safety-Oriented Decision-Making for Electronic Railway Signaling Systems

Author

Emanuele Pascale, Laurent Bouillaut, Thierry Freneaux, Raffaele Sista, Paolo Sannino, and Pietro Marmo

Subjects
safety modeling, maintenance modeling, reliability analysis, railway signaling systems, Weibull distribution, Industrial safety. Industrial accident prevention, T55-55.3, Medicine (General), R5-920
Abstract

This paper presents the advantages of using Weibull distributions, within the context of railway signaling systems, for enabling safety-oriented decision-making. Failure rates are used to statistically model the basic event of fault-tree analysis, and their value sizes the maximum allowable latency of failures to fulfill the safety target for which the system has been designed. Relying on field-return failure data, Weibull parameters have been calculated for an existing electronic signaling system and a comparison with existing predictive reliability data, based on exponential distribution, is provided. Results are discussed in order to drive considerations on the respect of quantitative targets and on the impact that a wrong hypothesis might have on the choice of a given architecture. Despite the huge amount of information gathered through the after-sales logbook used to build reliability distribution, several key elements for reliable estimation of failure rate values are still missing. This might affect the uncertainty of reliability parameters and the effort required to collect all the information. We then present how to intervene when operational failure rates present higher values compared to the theoretical approach: increasing the redundancies of the system or performing preventive maintenance tasks. Possible consequences of unjustified adoption of constant failure rate are presented. Some recommendations are also shared in order to build reliability-oriented logbooks and avoid data censoring phenomena by enhancing the functions of the electronic boards composing the system.

Published
2018
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5. New challenges in retinoblastoma treatment

Author

Desjardins, L., primary, Angi, M., additional, Levy, C., additional, Lumbroso Le Rouic, L., additional, Aerts, I., additional, Freneaux, P., additional, Brisse, H., additional, and Cassoux, N., additional

Published
2016
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6. ATYPICAL TERATOID RHABDOID TUMOUR

Author

Bertozzi, A. I., primary, Munzer, C., additional, Fouyssac, F., additional, Andre, N., additional, Boetto, S., additional, Leblond, P., additional, Bourdeaut, F., additional, Dufour, C., additional, Deshpande, R. K., additional, Bhat, K. G., additional, Mahalingam, S., additional, Muscat, A., additional, Cain, J., additional, Ferguson, M., additional, Popovski, D., additional, Algar, E., additional, Rossello, F. J., additional, Jayasekara, S., additional, Watkins, D. N., additional, Hodge, J., additional, Ashley, D., additional, Hishii, M., additional, Saito, M., additional, Arai, H., additional, Han, Z. Y., additional, Richer, W., additional, Lucchesi, C., additional, Freneaux, P., additional, Nicolas, A., additional, Grison, C., additional, Pierron, G., additional, Delattre, O., additional, Epari, S., additional, TS, N., additional, Gupta, T., additional, Chinnaswamy, G., additional, Sastri, J. G., additional, Shetty, P., additional, Moiyadi, A., additional, Jalali, R., additional, Fay-McClymont, T., additional, Johnston, D., additional, Janzen, L., additional, Guger, S., additional, Scheinemann, K., additional, Fleming, A., additional, Fryer, C., additional, Hukin, J., additional, Mabbott, D., additional, Huang, A., additional, Bouffet, E., additional, Lafay-Cousin, L., additional, Kawamura, A., additional, Yamamoto, K., additional, Nagashima, T., additional, Bartelheim, K., additional, Benesch, M., additional, Buchner, J., additional, Gerss, J., additional, Hasselblatt, M., additional, Kortmann, R.-D., additional, Fleischack, G., additional, Quiroga, E., additional, Reinhard, H., additional, Schneppenheim, R., additional, Seeringer, A., additional, Siebert, R., additional, Timmermann, B., additional, Warmuth-Metz, M., additional, Schmid, I., additional, Fruhwald, M. C., additional, Kerl, K., additional, Klingebiel, T., additional, Al-Kofide, A., additional, Khafaga, Y., additional, Al-Hindi, H., additional, Dababo, M., additional, Ul-Haq, A., additional, Anas, M., additional, Barria, M. G., additional, Siddiqui, K., additional, Hassounah, M., additional, Ayas, M., additional, Al-Shail, E., additional, Jeibmann, A., additional, Eikmeier, K., additional, Linge, A., additional, Johann, P., additional, Koos, B., additional, Kool, M., additional, Pfister, S. M., additional, Paulus, W., additional, Schuller, U., additional, Junckerstorff, R., additional, Rosenblum, M. K., additional, Alassiri, A. H., additional, Rossi, S., additional, Gottardo, N., additional, Toledano, H., additional, Viscardi, E., additional, Witkowski, L., additional, Nagel, I., additional, Oyen, F., additional, Foulkes, W. D., additional, Schrey, D., additional, Malietzis, G., additional, Chi, S., additional, Marshall, L., additional, Carceller, F., additional, Moreno, L., additional, Zacharoulis, S., additional, Bhardwaj, R., additional, Chakravadhanula, M., additional, Ozals, V., additional, Hampton, C., additional, Metpally, R., additional, Grillner, P., additional, Asmundsson, J., additional, Gustavsson, B., additional, Holm, S., additional, Johann, P. D., additional, Korshunov, A., additional, Ryzhova, M., additional, Milde, T., additional, Witt, O., additional, Jones, D. T. W., additional, Hovestadt, V., additional, Gajjar, A., additional, Fruhwald, M., additional, Pfister, S., additional, Finetti, M., additional, Pons, A. d. C., additional, Selby, M., additional, Smith, A., additional, Crosier, S., additional, Wood, J., additional, Skalkoyannis, B., additional, Bailey, S., additional, Clifford, S., additional, Williamson, D., additional, Rutkowski, S., additional, Kortmann, R. D., additional, Graf, N., additional, Boos, J., additional, Nysom, K., additional, Moreno, N., additional, Holsten, T., additional, Ahlfeld, J., additional, Mertins, J., additional, Hotfilder, M., additional, Schleicher, S., additional, Handgretinger, R., additional, Meisterernst, M., additional, Schmidt, C., additional, Dittmar, S., additional, Chan, G. C. F., additional, Shing, M. M. K., additional, Yuen, H. L., additional, Li, R. C. H., additional, Ling, S. L., additional, Slavc, I., additional, Peyrl, A., additional, Chocholous, M., additional, Azizi, A., additional, Czech, T., additional, Dieckmann, K., additional, Haberler, C., additional, Leiss, U., additional, Gotti, G., additional, Biassoni, V., additional, Schiavello, E., additional, Spreafico, F., additional, Pecori, E., additional, Gandola, L., additional, Massimino, M., additional, Kornelius, K., additional, Yano, H., additional, Nakayama, N., additional, Ohe, N., additional, Ozeki, M., additional, Kanda, K., additional, Kimura, T., additional, Hori, T., additional, Fukao, T., additional, Iwama, T., additional, Weil, A. G., additional, Diaz, A., additional, Gernsback, J., additional, Bhatia, S., additional, Ragheb, J., additional, Niazi, T., additional, Khatib, Z., additional, Zoghbi, A., additional, Meisterernst, a. M., additional, Birks, D., additional, Griesinger, A., additional, Amani, V., additional, Donson, A., additional, Posner, R., additional, Dunham, C., additional, Kleinschmidt-DeMasters, B. K., additional, Handler, M., additional, Vibhakar, R., additional, Foreman, N., additional, Zhou, L., additional, Catchpoole, D., additional, Kakkar, A., additional, Biswas, A., additional, Suri, V., additional, Sharma, M., additional, Kale, S., additional, Mahapatra, A., additional, Sarkar, C., additional, Torchia, J., additional, Picard, D., additional, Ho, K. C., additional, Khuong-Quang, D.-A., additional, Louterneau, L., additional, Bourgey, M., additional, Chan, T., additional, Golbourn, B., additional, Cousin, L.-L., additional, Taylor, M. D., additional, Dirks, P., additional, Rutka, J. T., additional, Hawkins, C., additional, Majewski, J., additional, Kim, S.-K., additional, Jabado, N., additional, Chang, J. H.-C., additional, Confer, M., additional, Chang, A., additional, Goldman, S., additional, Dunn, M., additional, and Hartsell, W., additional

Published
2014
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7. ATYPICAL TERATOID RHABDOID TUMOR (ATRT)

Author

Hasselblatt, M., primary, Kordes, U., additional, Wolff, J., additional, Jeibmann, A., additional, Fruhwald, M., additional, Paulus, W., additional, Hasselblatt, M., additional, Isken, S., additional, Siebert, R., additional, Schneppenheim, R., additional, Benesch, M., additional, Fleischhack, G., additional, Gruhn, B., additional, Schlegel, P.-G., additional, Witt, O., additional, Holter, W., additional, Reiter, A., additional, Urban, C., additional, Fruhwald, M. C., additional, Lafay-Cousin, L., additional, Huang, A., additional, Hawkins, C., additional, Fryer, C., additional, Bouffet, E., additional, Kruchko, C., additional, Propp, J., additional, McCarthy, B., additional, Dolecek, T., additional, Kerl, K., additional, Unland, R., additional, Jurgens, H., additional, Kieran, M. W., additional, Roberts, C. W. M., additional, Biegel, J. A., additional, MacConaill, L. E., additional, Rich, B. E., additional, Ligon, K. L., additional, Chi, S., additional, Kondo, A., additional, Shimoji, K., additional, Ogino, I., additional, Junya, F., additional, Sakaguchi, S., additional, Miyajima, M., additional, Arai, H., additional, Alimova, I., additional, Knipstein, J., additional, Harris, P., additional, Venkataraman, S., additional, Marquez, V., additional, Birks, D., additional, Foreman, N., additional, Vibhakar, R., additional, Bartelheim, K., additional, Warmuth-Metz, M., additional, Kortmann, R.-D., additional, Gerss, J., additional, Rizzo, D., additional, Freneaux, P., additional, Brisse, H., additional, Parfait, B., additional, Doz, F., additional, Dufour, C., additional, Stephan, J.-L., additional, Edan, C., additional, Ranchere-Vince, D., additional, Peuchmaur, M., additional, Delattre, O., additional, Bourdeaut, F., additional, Soh, S. Y., additional, Chan, M. Y., additional, Seow, W. T., additional, Chang, K., additional, Ng, W. H., additional, Tan, A. M., additional, Yamasaki, K., additional, Tanaka, C., additional, Okada, K., additional, Fujisaki, H., additional, Osugi, Y., additional, Hara, J., additional, Matsusaka, Y., additional, Sakamoto, H., additional, Inoue, T., additional, Batchelder, P., additional, DeMasters, B. K., additional, Handler, M., additional, Sumerauer, D., additional, Vasovcak, P., additional, Puchmajerova, A., additional, Zapotocky, M., additional, Vicha, A., additional, Kyncl, M., additional, Zamecnik, J., additional, Sedlacek, Z., additional, Kodet, R., additional, Geludkova, O., additional, Kumirova, E., additional, Korshunov, A., additional, Kushel, Y., additional, Melikyan, A., additional, Shishkina, L., additional, Ryzhova, M., additional, Ozerova, V., additional, Gorbatyh, S., additional, Popov, V., additional, Pavlova, E., additional, Scherbenko, O., additional, Borodina, I., additional, Donson, A., additional, Dunham, C., additional, Algar, E., additional, Popovski, D., additional, Muscat, A., additional, Ashley, D., additional, Modena, P., additional, Sardi, I., additional, Brenca, M., additional, Giunti, L., additional, Maestro, R., additional, Buccoliero, A. M., additional, Pollo, B., additional, Genitori, L., additional, Giangaspero, F., additional, Massimino, M., additional, Amani, V., additional, Griesinger, A., additional, Bemis, L., additional, Schittone, S., additional, Puccetti, D., additional, Wargowski, D., additional, Messiaen, L., additional, Patel, N., additional, Salamat, S., additional, Rusinak, D., additional, Iskandar, B., additional, Lun, X., additional, Jayanthan, A., additional, Forsyth, P., additional, and Narendran, A., additional

Published
2012
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8. Eye Salvage with Combination of Intravitreal and Intracameral Melphalan Injection for Recurrent Retinoblastoma with Anterior Chamber Involvement: Report of a Case

Author

Cassoux, Nathalie, Aerts, Isabelle, Lumbroso-Le Rouic, Livia, Freneaux, Paul, and Desjardins, Laurence

Abstract

Aims:Herein we report successful eye salvage following combined intravitreal and intracameral administration of melphalan for anterior chamber recurrence of retinoblastoma. Methods:A 12-month-old boy was referred with a sporadic bilateral retinoblastoma staged as group D and V in the right eye and group E and V in the left eye. Treatment initially involved 6 cycles of 3 drugs followed by focal treatments. The left eye was enucleated. Three months after the end of systemic chemotherapy, multiple recurrent lesions were noted in the ciliary body and the anterior chamber. A series of intravitreal (IVT) and intracameral (IC) injections of melphalan combined with a 7th cycle of systemic chemotherapy was performed. The therapeutic schedule alternated: week A an IVT of melphalan (30 μg/0.1 mL) for a total of 4 versus week B IC injections of melphalan (15 μg/0.05 mL) 4 IC in total. Each injection was preceded by an anterior chamber. Cryotherapy to the injection punctures was systematically performed. Results:Rapidly, iris and ciliary body nodules regressed completely based on clinical and ultrasound biomicroscopic examinations. After 3 years of follow-up, the patient has shown no evidence of recurrence. Conclusion:In our patient, combined IVT and IC administration of melphalan proved highly efficacious in eradicating the ciliary body and anterior chamber disease and salvage of his one remaining functional eye.

Published
2017
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9. DNA methylation heterogeneity defines a disease spectrum in Ewing sarcoma

Author

Sheffield, Nathan C, Pierron, Gaelle, Klughammer, Johanna, Datlinger, Paul, Schönegger, Andreas, Schuster, Michael, Hadler, Johanna, Surdez, Didier, Guillemot, Delphine, Lapouble, Eve, Freneaux, Paul, Champigneulle, Jacqueline, Bouvier, Raymonde, Walder, Diana, Ambros, Ingeborg M, Hutter, Caroline, Sorz, Eva, Amaral, Ana T, de Álava, Enrique, Schallmoser, Katharina, Strunk, Dirk, Rinner, Beate, Liegl-Atzwanger, Bernadette, Huppertz, Berthold, Leithner, Andreas, de Pinieux, Gonzague, Terrier, Philippe, Laurence, Valérie, Michon, Jean, Ladenstein, Ruth, Holter, Wolfgang, Windhager, Reinhard, Dirksen, Uta, Ambros, Peter F, Delattre, Olivier, Kovar, Heinrich, Bock, Christoph, and Tomazou, Eleni M

Abstract

Developmental tumors in children and young adults carry few genetic alterations, yet they have diverse clinical presentation. Focusing on Ewing sarcoma, we sought to establish the prevalence and characteristics of epigenetic heterogeneity in genetically homogeneous cancers. We performed genome-scale DNA methylation sequencing for a large cohort of Ewing sarcoma tumors and analyzed epigenetic heterogeneity on three levels: between cancers, between tumors, and within tumors. We observed consistent DNA hypomethylation at enhancers regulated by the disease-defining EWS-FLI1 fusion protein, thus establishing epigenomic enhancer reprogramming as a ubiquitous and characteristic feature of Ewing sarcoma. DNA methylation differences between tumors identified a continuous disease spectrum underlying Ewing sarcoma, which reflected the strength of an EWS-FLI1 regulatory signature and a continuum between mesenchymal and stem cell signatures. There was substantial epigenetic heterogeneity within tumors, particularly in patients with metastatic disease. In summary, our study provides a comprehensive assessment of epigenetic heterogeneity in Ewing sarcoma and thereby highlights the importance of considering nongenetic aspects of tumor heterogeneity in the context of cancer biology and personalized medicine.

Published
2017
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11. Low expression of bcl-2 in Brca1-associated breast cancers

Author

Freneaux, P, primary, Stoppa-Lyonnet, D, additional, Mouret, E, additional, Kambouchner, M, additional, Nicolas, A, additional, Zafrani, B, additional, Vincent-Salomon, A, additional, Fourquet, A, additional, Magdelenat, H, additional, and Sastre-Garau, X, additional

Published
2000
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12. Low expression ofbcl-2 inBrca1-associated breast cancers.

Author

Freneaux, P, Stoppa-Lyonnet, D, Mouret, E, Kambouchner, M, Nicolas, A, Zafrani, B, Vincent-Salomon, A, Fourquet, A, Magdelenat, H, and Sastre-Garau, X

Subjects
*BREAST cancer, *APOPTOSIS, *DISEASES in women
Abstract

Little data are available concerning the molecular mechanisms of action of Brca1 and Brca2 in breast oncogenesis. Recent experimental results suggest that Brca1 plays a role in the regulation of apoptosis. In order to determine whether the analysis of human tumours would provide data supporting this hypothesis, we have assessed the expression of the antiapoptotic bcl-2 and of the proapoptotic p53 genes in Brca1- and Brca2-associated breast carcinomas. The levels of expression of these genes were compared to those observed in controls and to the mitotic and the apoptotic indexes. Our series were composed of 16 cases of breast carcinoma in women with a germline Brca1 gene mutation, and of four cases with Brca2 mutation. A group of 39 patients aged under 36 years and for whom the search for Brca1 gene mutations was negative, and a group of 36 cases of sporadic cancers without data on their Brca status were used as controls. Immunohistochemistry was used to detect p53 and bcl-2 gene products. Mitotic and apoptotic indexes were higher in Brca1-associated tumours than in controls. No significant difference in p53 immunostaining was observed between the four groups of patients. In contrast, the rate of bcl-2-positive tumours was lower (31%) in Brca1-carcinomas than in carcinomas without Brca1 mutation (90%) (P < 10(-3)). A strong Bcl-2 expression was found in the four cases of Brca2-associated carcinomas. No significant correlation was observed between p53 and Bcl-2 immunostainings, either in cases or in controls. The association between Brca1 status and Bcl-2 expression remained significant after adjustment for the oestrogen receptor status. Our study shows that a low expression of bcl-2 characterises most Brca1-associated breast carcinomas, a biological trait which seems not to be shared by Brca2-associated tumours nor to be related to oestrogen receptor and/or p53 status. bcl-2 might thus be one of the target genes involved in the oncogenesis related to Brca1 and... [ABSTRACT FROM AUTHOR]

Published
2000
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13. Spectral broadening for multi-Joule pulse compression in the APOLLON Long Focal Area facility

Author

Bleotu, P.-G., Wheeler, J., Papadopoulos, D., Chabanis, M., Prudent, J., Frotin, M., Martin, L., Lebas, N., Freneaux, A., Beluze, A., Mathieu, F., Audebert, P., Ursescu, D., Fuchs, J., and Mourou, G.

Abstract

AbstractSpectral-broadening of the APOLLON PW-class laser pulses using a thin-film compression technique within the long-focal-area interaction chamber of the APOLLON laser facility is reported, demonstrating the delivery of the full energy pulse to the target interaction area. The laser pulse at 7 J passing through large aperture, thin glass wafers is spectrally broadened to a bandwidth that is compatible with a 15-fs pulse, indicating also the possibility to achieve sub-10-fs pulses using 14 J. Placing the post-compressor near the interaction makes for an economical method to produce the shortest pulses by limiting the need for high damage, broadband optics close to the final target rather than throughout the entire laser transport system.

Published
2022
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14. Concordance between abdominal scintigraphy using technetium-99m hexamethylpropylene amine oxime-labelled leucocytes and ileocolonoscopy in patients with spondyloarthropathies and without clinical evidence of inflammatory bowel disease

Author

El Maghraoui, A, Dougados, M, Freneaux, E, Chaussade, S, Amor, B, and Breban, M

Abstract

Objective.To study the concordance between abdominal scintigraphy using technetium-99m hexamethylpropylene amine oxime (99mTc-HMPAO)-labelled leucocytes (ASTLL) and ileocolonoscopy in patients with spondyloarthropathies (SpA) and without clinical evidence of inflammatory bowel disease (IBD).Patients and methods.Fifteen patients with SpA (European Spondylarthropathy Study Group 1991) criteria) without clinical evidence of IBD were studied prospectively with ASTLL and ileocolonoscopy.Results.This cohort consisted of seven men and eight women aged 31.8 ± 10.5 yr (18-47) [mean age ± S.D. (range)] and with a disease duration of 6.0 ± 4.4 yr (0.4-15). ASTLL showed abnormal uptake in four patients. Ileocolonoscopy was abnormal in five patients, showing acute inflammatory lesions in one patient with reactive arthritis, undifferentiated chronic inflammatory lesions in two cases, and features indistinguishable from those of Crohn's disease in two cases. ASTLL was negative in two cases in which ileocolonoscopy showed inflammatory lesions and was positive (terminal ileum) in one case with normal ileocolonoscopy. The concordance between the two examinations was statistically significant (k=0.53; P=0.008).Conclusion.ASTLL may be an interesting tool to detect subclinical gut inflammation in patients with SpA.Keywords:Scintigraphy, Ileocolonoscopy, Spondyloarthropathy, Inflammatory bowel disease.

Published
1999

15. ShortChain AcylCoenzyme A Dehydrogenase Activity Antigen and Biosynthesis Are Absent in the BALBcByJ Mouse

Author

AMENDT, BRAD A., FRENEAUX, ERIC, REECE, CAMERON, WOOD, PHILIP A., and RHEAD, WILLIAM J.

Abstract

BALB/cByJ (J) mice have short-chain acyl-CoA dehydrogenase (SCAD) deficiency and an organic aciduria similar to that of human SCAD deficiency. [9,10(n)-JH)- and [15,16(n)-3H)paimitale oxidations in J mouse fibroblasts were 96 and 35 of control, respectively, consistent with an isolated SCAD defect. Acyl-CoA deby-drogenase activities were assayed in muscle and fibroblast mitochondria from BALB/cBy controls (Y) and SCAD-deficient J mice. Medium-chain acyl-CoA dehydrogenase (MCAD) activities were comparable in both J and Y mice from all tissues. In the presence of MCAD antiserum, SCAD activities in J mice were undetectable in both tissues. Apparent Km and Vmaxvalues in liver mitochondria suggested a somewhat increased affinity of MCAD for butyryl-CoA in J mice, as compared with MCAD from other species. Immunoblot studies using mitochondria revealed identical apparent SCAD molecular weight in liver, muscle, and fibroblasts from Y and mice and no detectable SCAD antigen in J mice; MCAD antigen was detected in comparable amounts from both Y and J mice. Radiolabeling and immunoprecipitation studies in J mouse fibroblasts revealed no SCAD synthesis, but normal MCAD synthesis. These data argue against the existence of tissue-specific SCAD isoforms in the mouse and confirm that this mouse strain is a model for the human organic aciduria resulting from this β-oxidation defect. (Pediatr Res31: 552–556, 1992)

Published
1992

16. Short-Chain Acyl-Coenzyme A Dehydrogenase Activity, Antigen, and Biosynthesis Are Absent in the BALB/cByJ Mouse

Author

Amendt, Brad A, Freneaux, Eric, Reece, Cameron, Wood, Philip A, and Rhead, William J

Abstract

ABSTRACTS: BALB/cByJ (J) mice have short-chain acyl-CoA dehydrogenase (SCAD) deficiency and an organic aciduria similar to that of human SCAD deficiency. [9,10(n)-JH)- and [15,16(n)-3H)paimitale oxidations in J mouse fibroblasts were 96 and 35% of control, respectively, consistent with an isolated SCAD defect. Acyl-CoA deby-drogenase activities were assayed in muscle and fibroblast mitochondria from BALB/cBy controls (Y) and SCAD-deficient J mice. Medium-chain acyl-CoA dehydrogenase (MCAD) activities were comparable in both J and Y mice from all tissues. In the presence of MCAD antiserum, SCAD activities in J mice were undetectable in both tissues. Apparent Km and Vmaxvalues in liver mitochondria suggested a somewhat increased affinity of MCAD for butyryl-CoA in J mice, as compared with MCAD from other species. Immunoblot studies using mitochondria revealed identical apparent SCAD molecular weight in liver, muscle, and fibroblasts from Y and mice and no detectable SCAD antigen in J mice; MCAD antigen was detected in comparable amounts from both Y and J mice. Radiolabeling and immunoprecipitation studies in J mouse fibroblasts revealed no SCAD synthesis, but normal MCAD synthesis. These data argue against the existence of tissue-specific SCAD isoforms in the mouse and confirm that this mouse strain is a model for the human organic aciduria resulting from this ß-oxidation defect.

Published
1992
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17. Diffuse Infiltrating Retinoblastoma with Anterior Chamber Involvement: Conservative Management and Identification of RB1 Alterations in Aqueous Humor.

Author

Cassoux N, Malaise D, Lumbroso-Le-Rouic L, Le Gall J, Golmard L, Cardoen L, Freneaux P, Bouchoucha Y, Aerts I, Doz F, and Matet A

Abstract

Introduction: The aim of the study was to describe the successful conservative management of diffuse infiltrating retinoblastoma (DIR). Identification of RB1 pathogenic variant was done after cell-free DNA (cfDNA) analysis in aqueous humor., Case Presentation: Herein, we report 2 patients with unilateral, non-familial DIR with anterior and posterior involvement. Both patients underwent liquid biopsy for tumor cfDNA analysis in aqueous humor. Treatment consisted of a combination of systemic and intra-arterial chemotherapy, with consecutive intracameral and intravitreal injections of melphalan. One patient also required iodine-125 brachytherapy. In both cases, tumor cfDNA analysis revealed biallelic somatic alterations of the RB1 gene. These alterations were not found in germline DNA. Both patients retained their eyes and had a useful vision after a follow-up of 2 years., Conclusion: In selected cases, conservative management of DIR is safe and effective. Tumor cfDNA analysis in aqueous humor is an effective technique to disclose RB1 somatic alterations that guide the germline molecular explorations and improve genetic counseling after conservative treatment., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 S. Karger AG, Basel.)

Published
2023
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18. Gain of Aggressive Histological and Molecular Patterns after Acquired Resistance to Novel Anti-EGFR Therapies in Non-Small Cell Lung Cancer.

Author

Basse C, Trabelsi-Grati O, Masliah J, Callens C, Kamal M, Freneaux P, Klijanienko J, Bieche I, and Girard N

Subjects
Humans, Disease Progression, Ki-67 Antigen genetics, Mutation, Protein Kinase Inhibitors pharmacology, Adenocarcinoma of Lung drug therapy, Adenocarcinoma of Lung genetics, Carcinoma, Non-Small-Cell Lung drug therapy, Carcinoma, Non-Small-Cell Lung genetics, Drug Resistance, Neoplasm genetics, ErbB Receptors antagonists & inhibitors, ErbB Receptors genetics, Lung Neoplasms drug therapy, Lung Neoplasms genetics
Abstract

Novel anti-EGFR therapies target resistance to standard-of-care anti-EGFR in patients with metastatic lung cancer. We describe tumors at progression versus at the initiation of novel anti-EGFR agents in patients with metastatic lung adenocarcinoma harboring EGFR mutation. This clinical case series reports the histological and genomic features and their evolution following disease progression under amivantamab or patritumab-deruxtecan in clinical trials. All patients had a biopsy at disease progression. Four patients harboring EGFR gene mutations were included. Three of them received anterior anti-EGFR treatment. Median delay to disease progression was 15 months (range: 4-24). At progression, all tumors presented a mutation in the TP53 signaling pathway associated with a loss of heterozygosis (LOH) of the allele in 75% ( n = 3), and two tumors (50%) presented an RB1 mutation associated with LOH. Ki67 expression increased above 50% (range 50-90%) in all samples compared to baseline (range 10-30%), and one tumor expressed a positive neuroendocrine marker at progression. Our work reports the potential molecular mechanisms of resistance under novel anti-EGFR in patients with metastatic EGFR-mutated lung adenocarcinoma, with the transformation to a more aggressive histology with acquired TP53 mutation and/or the increase in Ki67 expression. These characteristics are usually found in aggressive Small Cell Lung Cancer.

Published
2023
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19. Embryonic signature distinguishes pediatric and adult rhabdoid tumors from other SMARCB1-deficient cancers.

Author

Richer W, Masliah-Planchon J, Clement N, Jimenez I, Maillot L, Gentien D, Albaud B, Chemlali W, Galant C, Larousserie F, Boudou-Rouquette P, Leruste A, Chauvin C, Han ZY, Coindre JM, Varlet P, Freneaux P, Ranchère-Vince D, Delattre O, and Bourdeaut F

Subjects
Adolescent, Adult, Age of Onset, Child, Child, Preschool, DNA Methylation, Diagnosis, Differential, Gene Regulatory Networks, Humans, Infant, Male, Young Adult, Gene Expression Profiling methods, Rhabdoid Tumor diagnosis, Rhabdoid Tumor genetics, SMARCB1 Protein deficiency, Exome Sequencing methods
Abstract

Extra-cranial rhabdoid tumors (RT) are highly aggressive malignancies of infancy, characterized by undifferentiated histological features and loss of SMARCB1 expression. The diagnosis is all the more challenging that other poorly differentiated cancers lose SMARCB1 expression, such as epithelioid sarcomas (ES), renal medullary carcinomas (RMC) or undifferentiated chordomas (UC). Moreover, late cases occurring in adults are now increasingly reported, raising the question of differential diagnoses and emphasizing nosological issues. To address this issue, we have analyzed the expression profiles of a training set of 32 SMARCB1-deficient tumors (SDT), with ascertained diagnosis of RT (n = 16, all < 5 years of age), ES (n = 8, all > 10 years of age), UC (n = 3) and RMC (n = 5). As compared with other SDT, RT are characterized by an embryonic signature, and up-regulation of key-actors of de novo DNA methylation processes. Using this signature, we then analysed the expression profiling of 37 SDT to infer the appropriate diagnosis. Thirteen adult onset tumors showed strong similarity with pediatric RT, in spite of older age; by exome sequencing, these tumors also showed genomic features indistinguishable from pediatric RT. In contrary, 8 tumors were reclassified within carcinoma, ES or UC categories, while the remaining could not be related to any of those entities. Our results demonstrate that embryonic signature is shared by all RT, whatever the age at diagnosis; they also illustrate that many adult-onset SDT of ambiguous histological diagnosis are clearly different from RT. Finally, our study paves the way for the routine use of expression-based signatures to give accurate diagnosis of SDT.

Published
2017
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