40 results on '"Ghorab K"'
Search Results
2. Neuropathie des petites fibres au cours du syndrome de Sjögren primitif
- Author
-
Fauchais, A.-L., Richard, L., Gondran, G., Ghorab, K., Palat, S., Bezanahary, H., Loustaud-Ratti, V., Ly, K., Jauberteau, M.-O., Vallat, J.-M., Vidal, E., and Magy, L.
- Published
- 2011
- Full Text
- View/download PDF
3. Long-term benefit of enzyme replacement therapy with alglucosidase alfa in adults with Pompe disease: prospective analysis from the French Pompe Registry
- Author
-
Semplicini, C., de Antonio, M., Taouagh, N., Béhin, A., Bouhour, F., Echaniz-Laguna, A., Magot, A., Nadaj-Pakleza, A., Orlikowski, D., Sacconi, S., Salort-Campana, E., Solé, G., Tard, Celine, Zagnoli, F., Jean-Yves, H., Hamroun, D., Laforêt, P., Attarian, S., Aubé-Nathier, A. C., Arrassi, A., Bassez, G., Bedat-Millet, A. L., Bouibede, F., Boyer, F. C., Caillaud, C., Canal, A., Carlier, R. Y., Chanson, J. B., Chapon, F., Cintas, P., Deibener-Kaminsky, J., Demurger, F., Desnuelle, C., Durieu, I., Eymard, B., Feasson, L., Fournier, M., Froissart, R., Furby, A., Garcia, P. Y., Germain, D. P., Ghorab, K., Morales, R. J., Krim, E., Labauge, P., Lacour, A., Lagrange, E., Lefeuvre, C., Leguy-Seguin, V., Leonard-Louis, S., Magy, L., Masseau, A., Michaud, M., Minot-Myhié, M. C., Nicolas, G., Nollet, S., Not, A., Noury, J. B., Ollivier, G., Péréon, Y., Perez, Thierry, Perniconi, B., Piraud, M., Petiot, P., Pouget, J., Praline, J., Prigent, H., Renard, D., Spinazzi, M., Stojkovic, T., Taithe, F., Tiffreau, Vincent, Vincent, D., Lille Neurosciences & Cognition - U 1172 (LilNCog), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488 (URePSSS), and Université d'Artois (UA)-Université du Littoral Côte d'Opale (ULCO)-Université de Lille
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Registry ,alglucosidase alfa ,Adolescent ,[SDV]Life Sciences [q-bio] ,Walk Test ,Disease ,Sitting ,03 medical and health sciences ,FEV1/FVC ratio ,Young Adult ,Glycogen storage disease type II ,Genetics ,medicine ,Humans ,Respiratory function ,Prospective Studies ,Registries ,Child ,Alglucosidase alfa ,Genetics (clinical) ,030304 developmental biology ,Aged ,0303 health sciences ,late onset Pompe disease ,business.industry ,030305 genetics & heredity ,alpha-Glucosidases ,Enzyme replacement therapy ,Middle Aged ,medicine.disease ,Respiratory Function Tests ,Treatment Outcome ,Ceiling effect ,Female ,France ,business ,medicine.drug ,enzyme replacement therapy - Abstract
Despite a wide clinical spectrum, the adult form of Pompe disease is the most common one, and represents more than 90% of diagnosed patients in France. Since the marketing of enzyme replacement therapy (alglucosidase alfa, Myozyme), all reports to date in adults demonstrated an improvement of the walking distance, and a trend toward stabilization of respiratory function, but the majority of these studies were less than 5 years of duration. We report here the findings from 158 treated patients included in the French Pompe Registry, who underwent regular clinical assessments based on commonly used standardized tests (6-minute walking test, MFM scale, sitting vital capacity, MIP and MEP). For longitudinal analyses, the linear mixed effects models were used to assess trends in primary endpoints over time under ERT. A two-phase model better described the changes in distance traveled in the 6-minute walk test and MFM. 6MWT showed an initial significant increase (1.4% ± 0.5/year) followed by a progressive decline (-2.3%/year), with a cut-off point at 2.2 years. A similar pattern was observed in total MFM score (6.6% ± 2.3/year followed by a - 1.1%/year decline after 0.5 years). A single-phase decline with a slope of -0.9 ± 0.1%/year (P < .001) was observed for FVC, and MEP remained stable over the all duration of follow-up. This study provides further evidence that ERT improves walking abilities and likely stabilizes respiratory function in adult patients with Pompe disease, with a ceiling effect for the 6MWT in the first 3 years of treatment.
- Published
- 2020
- Full Text
- View/download PDF
4. SUBCUTANEOUS INJECTIONS OF POLYVALENT IMMUNOGLOBULINS AS A MAINTENANCE THERAPY FOR INTRAVENOUS IMMUNOGLOBULIN-RESPONSIVE PATIENTS WITH CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY (CIDP)
- Author
-
Magy, L, Ghorab, K, Calvo, J, and Vallat, J-M
- Published
- 2008
5. Self-report questionnaire vs. clinical evaluation form in the French National Registry on facioscapulohumeral dystrophy: a statistical comparison
- Author
-
Benoit, S., Stalens, C., Villa, L., Guien, Celine, Rabarimeriarijaona, S., Bernard, R., Cintas, P., Sole, G., Tiffreau, V., Echaniz-Laguna, A., Magot, A., Morales, R. J., Bombart, V., Jacquin Piques, Agnès, Nadaj-Pakleza, A., Bouhour, F., Chapon, F., Eymard, B., Ghorab, K., Beroud, Christophe, Sacconi, S., French Fshd Registry, ., COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Association française contre les myopathies (AFM-Téléthon), Centre Hospitalier Universitaire de Nice (CHU Nice), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital de la Timone [CHU - APHM] (TIMONE), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Lille (CHU de Lille), Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Hôtel-Dieu [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Centre Hospitalier Universitaire de Reims (CHU Reims), Centre des Sciences du Goût et de l'Alimentation [Dijon] (CSGA), Institut National de la Recherche Agronomique (INRA)-Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université Bourgogne Franche-Comté [COMUE] (UBFC), Centre Hospitalier Universitaire de Strasbourg (CHU de Strasbourg ), Hospices Civils de Lyon (HCL), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Mobilités : Vieillissement, Pathologie, Santé (COMETE), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Limoges], CHU Limoges, Département de génétique médicale [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Biologie Valrose (IBV), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), AFM-Telethon, AFM Téléthon, Partenaires INRAE, Aix Marseille Université (AMU), CHU Toulouse [Toulouse], Centre Hospitalier Universitaire Gui de Chauliac (CHU Gui de Chauliac), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Centre de Mathématiques et de Leurs Applications (CMLA), École normale supérieure - Cachan (ENS Cachan)-Centre National de la Recherche Scientifique (CNRS), Direction des Applications Militaires (DAM), Commissariat à l'énergie atomique et aux énergies alternatives (CEA), CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU), ProdInra, Migration, and Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM)
- Subjects
[SDV.GEN]Life Sciences [q-bio]/Genetics ,[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology ,[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,[SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,ComputingMilieux_MISCELLANEOUS ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology - Abstract
5th Congress of the European-Academy-of-Neurology (EAN), Oslo, NORWAY, JUN 29-JUL 02, 2019; International audience
- Published
- 2019
- Full Text
- View/download PDF
6. 36th International Symposium on Intensive Care and Emergency Medicine
- Author
-
Bateman, R. M., Sharpe, M. D., Jagger, J. E., Ellis, C. G., Solé-Violán, J., López-Rodríguez, M., Herrera-Ramos, E., Ruíz-Hernández, J., Borderías, L., Horcajada, J., González-Quevedo, N., Rajas, O., Briones, M., Rodríguez de Castro, F., Rodríguez Gallego, C., Esen, F., Orhun, G., Ergin Ozcan, P., Senturk, E., Ugur Yilmaz, C., Orhan, N., Arican, N., Kaya, M., Kucukerden, M., Giris, M., Akcan, U., Bilgic Gazioglu, S., Tuzun, E., Riff, R., Naamani, O., Douvdevani, A., Takegawa, R., Yoshida, H., Hirose, T., Yamamoto, N., Hagiya, H., Ojima, M., Akeda, Y., Tasaki, O., Tomono, K., Shimazu, T., Ono, S., Kubo, T., Suda, S., Ueno, T., Ikeda, T., Ogura, H., Takahashi, H., Kang, J., Nakamura, Y., Kojima, T., Izutani, Y., Taniguchi, T., O, M., Dinter, C., Lotz, J., Eilers, B., Wissmann, C., Lott, R., Meili, M. M., Schuetz, P. S., Hawa, H., Sharshir, M., Aburageila, M., Salahuddin, N., Chantziara, V., Georgiou, S., Tsimogianni, A., Alexandropoulos, P., Vassi, A., Lagiou, F., Valta, M., Micha, G., Chinou, E., Michaloudis, G., Kodaira, A., Imaizumi, H., De la Torre-Prados, M. V., Garcia-De la Torre, A., Enguix-Armada, A., Puerto-Morlan, A., Perez-Valero, V., Garcia-Alcantara, A., Bolton, N., Dudziak, J., Bonney, S., Tridente, A., Nee, P., Nicolaes, G., Wiewel, M., Schultz, M., Wildhagen, K., Horn, J., Schrijver, R., Van der Poll, T., Reutelingsperger, C., Pillai, S., Davies, G., Mills, G., Aubrey, R., Morris, K., Williams, P., Evans, P., Gayat, E. G., Struck, J., Cariou, A., Deye, N., Guidet, B., Jabert, S., Launay, J., Legrand, M., Léone, M., Resche-Rigon, M., Vicaut, E., Vieillard-Baron, A., Mebazaa, A., Arnold, R., Capan, M., Linder, A., Akesson, P., Popescu, M., Tomescu, D., Sprung, C. L., Calderon Morales, R., Munteanu, G., Orenbuch-Harroch, E., Levin, P., Kasdan, H., Reiter, A., Volker, T., Himmel, Y., Cohen, Y., Meissonnier, J., Girard, L., Rebeaud, F., Herrmann, I., Delwarde, B., Peronnet, E., Cerrato, E., Venet, F., Lepape, A., Rimmelé, T., Monneret, G., Textoris, J., Beloborodova, N., Moroz, V., Osipov, A., Bedova, A., Sarshor, Y., Pautova, A., Sergeev, A., Chernevskaya, E., Odermatt, J., Bolliger, R., Hersberger, L., Ottiger, M., Christ-Crain, M., Mueller, B., Schuetz, P., Sharma, N. K., Tashima, A. K., Brunialti, M. K., Machado, F. R., Assuncao, M., Rigato, O., Salomao, R., Cajander, S. C., Rasmussen, G., Tina, E., Söderquist, B., Källman, J., Strålin, K., Lange, A. L., Sundén-Cullberg, J. S., Magnuson, A. M., Hultgren, O. H., Van der Geest, P., Mohseni, M., Linssen, J., De Jonge, R., Duran, S., Groeneveld, J., Miller, R., Lopansri, B. K., McHugh, L. C., Seldon, A., Burke, J. P., Johnston, J., Reece-Anthony, R., Bond, A., Molokhia, A., Mcgrath, C., Nsutebu, E., Bank Pedersen, P., Pilsgaard Henriksen, D., Mikkelsen, S., Touborg Lassen, A., Tincu, R., Cobilinschi, C., Ghiorghiu, Z., Macovei, R., Wiewel, M. A., Harmon, M. B., Van Vught, L. A., Scicluna, B. P., Hoogendijk, A. J., Zwinderman, A. H., Cremer, O. L., Bonten, M. J., Schultz, M. J., Juffermans, N. P., Wiersinga, W. J., Eren, G., Tekdos, Y., Dogan, M., Acicbe, O., Kaya, E., Hergunsel, O., Alsolamy, S., Ghamdi, G., Alswaidan, L., Alharbi, S., Alenezi, F., Arabi, Y., Heaton, J., Boyce, A., Nolan, L., Dukoff-Gordon, A., Dean, A., Mann Ben Yehudah, T., Fleischmann, C., Thomas-Rueddel, D., Haas, C., Dennler, U., Reinhart, K., Suntornlohanakul, O., Khwannimit, B., Breckenridge, F., Puxty, A., Szturz, P., Folwarzcny, P., Svancara, J., Kula, R., Sevcik, P., Caneva, L., Casazza, A., Bellazzi, E., Marra, S., Pagani, L., Vetere, M., Vanzino, R., Ciprandi, D., Preda, R., Boschi, R., Carnevale, L., Lopez, V., Aguilar Arzapalo, M., Barradas, L., Escalante, A., Gongora, J., Cetina, M., Adamik, B, Jakubczyk, D, Kübler, A, Radford, A., Lee, T., Singer, J., Boyd, J., Fineberg, D., Williams, M., Russell, J., Scarlatescu, E., Droc, G., Arama, S., Müller, M., Straat, M., Zeerleder, S. S., Fuchs, C. F., Scheer, C. S., Wauschkuhn, S. W., Vollmer, M. V., Meissner, K. M., Kuhn, S. K., Hahnenkamp, K. H., Rehberg, S. R., Gründling, M. G., Hamaguchi, S., Gómez-Sánchez, E., Heredia-Rodríguez, M., Álvarez-Fuente, E., Lorenzo-López, M., Gómez-Pesquera, E., Aragón-Camino, M., Liu-Zhu, P., Sánchez-López, A., Hernández-Lozano, A., Peláez-Jareño, M. T., Tamayo, E., Thomas-Rüddel, D. O., Adora, V., Kar, A., Chakraborty, A., Roy, S., Bandyopadhyay, A., Das, M., BenYehudah, G., Salim, M., Kumar, N., Arabi, L., Burger, T., Lephart, P., Toth-martin, E., Valencia, C., Hammami, N., Blot, S., Vincent, J. L., Lambert, M. L., Brunke, J., Riemann, T., Roschke, I., Nimitvilai, S., Jintanapramote, K., Jarupongprapa, S., Adukauskiene, D., Valanciene, D., Bose, G., Lostarakos, V., Carr, B., Khedher, S., Maaoui, A., Ezzamouri, A., Salem, M., Chen, J., Cranendonk, D. R., Day, M., Penrice, G., Roy, K., Robertson, P., Godbole, G., Jones, B., Booth, M., Donaldson, L., Kawano, Y., Ishikura, H., Al-Dorzi, H., Almutairi, M., Alhamadi, B., Crizaldo Toledo, A., Khan, R., Al Raiy, B., Talaie, H., Van Oers, J. A., Harts, A., Nieuwkoop, E., Vos, P., Boussarsar, Y., Boutouta, F., Kamoun, S., Mezghani, I., Koubaji, S., Ben Souissi, A., Riahi, A., Mebazaa, M. S., Giamarellos-Bourboulis, E., Tziolos, N., Routsi, C., Katsenos, C., Tsangaris, I., Pneumatikos, I., Vlachogiannis, G., Theodorou, V., Prekates, A., Antypa, E., Koulouras, V., Kapravelos, N., Gogos, C., Antoniadou, E., Mandragos, K., Armaganidis, A., Robles Caballero, A. R., Civantos, B., Figueira, J. C., López, J., Silva-Pinto, A., Ceia, F., Sarmento, A., Santos, L., Almekhlafi, G., Sakr, Y., Baharoon, S., Aldawood, A., Matroud, A., Alchin, J., Al Johani, S., Balkhy, H., Yousif, S. Y., Alotabi, B. O., Alsaawi, A. S., Ang, J., Curran, M. D., Enoch, D., Navapurkar, V., Morris, A., Sharvill, R., Astin, J., Patel, J., Kruger, C., O’Neal, J., Rhodes, H., Jancik, J., François, B., Laterre, P. F., Eggimann, P., Torres, A., Sánchez, M., Dequin, P. F., Bassi, G. L., Chastre, J., Jafri, H. S., Ben Romdhane, M., Douira, Z., Bousselmi, M., Vakalos, A., Avramidis, V., Craven, T. H., Wojcik, G., Kefala, K., McCoubrey, J., Reilly, J., Paterson, R., Inverarity, D., Laurenson, I., Walsh, T. S., Mongodi, S., Bouhemad, B., Orlando, A., Stella, A., Via, G., Iotti, G., Braschi, A., Mojoli, F., Haliloglu, M., Bilgili, B., Kasapoglu, U., Sayan, I., Süzer Aslan, M., Yalcın, A., Cinel, I., Ellis, H. E., Bauchmuller, K., Miller, D., Temple, A., Luyt, C. E., Singer, M., Nassar, Y., Ayad, M. S., Trifi, A., Abdellatif, S., Daly, F., Nasri, R., Ben Lakhal, S., Gul, F., Kuzovlev, A., Shabanov, A., Polovnikov, S., Kadrichu, N., Dang, T., Corkery, K., Challoner, P., Bassi, G. Li, Aguilera, E., Chiurazzi, C., Travierso, C., Motos, A., Fernandez, L., Amaro, R., Senussi, T., Idone, F., Bobi, J., Rigol, M., Hodiamont, C. J., Janssen, J. M., Bouman, C. S., Mathôt, R. A., De Jong, M. D., Van Hest, R. M., Payne, L., Fraser, G. L., Tudor, B., Lahner, M., Roth, G., Krenn, C., Jault, P., Gabard, J., Leclerc, T., Jennes, S., Que, Y., Rousseau, A., Ravat, F., Eissa, A., Al-Harbi, S., Aldabbagh, T., Abdellatif., S., Paramba, F., Purayil, N., Naushad, V., Mohammad, O., Negi, V., Chandra, P., Kleinsasser, A., Witrz, M. R., Buchner-Doeven, J. F., Tuip-de Boer, A. M., Goslings, J. C., Van Hezel, M., Boing, A, Van Bruggen, R, Juffermans, N, Markopoulou, D., Venetsanou, K., Kaldis, V., Koutete, D., Chroni, D., Alamanos, I., Koch, L., Walter, E., Maekawa, K., Hayakawa, M., Kushimoto, S., Shiraishi, A., Kato, H., Sasaki, J., Matauoka, T., Uejima, T., Morimura, N., Hagiwara, A., Takeda, M., Tarabrin, O., Shcherbakow, S., Gavrychenko, D., Mazurenko, G., Ivanova, V., Chystikov, O., Plourde, C., Lessard, J., Chauny, J., Daoust, R., Kropman, L., In het Panhuis, L., Konings, J., Huskens, D., Schurgers, E., Roest, M., De Laat, B., Lance, M., Durila, M., Lukas, P., Astraverkhava, M., Jonas, J., Budnik, I., Shenkman, B., Hayami, H., Koide, Y., Goto, T., Iqbal, R., Alhamdi, Y., Venugopal, N., Abrams, S., Downey, C., Toh, C. H., Welters, I. D., Bombay, V. B., Chauny, J. M., Daoust, R. D., Lessard, J. L., Marquis, M. M., Paquet, J. P., Siemens, K., Sangaran, D., Hunt, B. J., Durward, A., Nyman, A., Murdoch, I. A., Tibby, S. M., Ampatzidou, F., Moisidou, D., Dalampini, E., Nastou, M., Vasilarou, E., Kalaizi, V., Chatzikostenoglou, H., Drossos, G., Spadaro, S., Fogagnolo, A., Fiore, T., Schiavi, A., Fontana, V., Taccone, F., Volta, C., Chochliourou, E., Volakli, E., Violaki, A., Samkinidou, E., Evlavis, G., Panagiotidou, V., Sdougka, M., Mothukuri, R., Battle, C., Guy, K., Wijesuriya, J., Keogh, S., Docherty, A., O’Donnell, R., Brunskill, S., Trivella, M., Doree, C., Holst, L., Parker, M., Gregersen, M., Almeida, J., Walsh, T., Stanworth, S., Moravcova, S., Mansell, J., Rogers, A., Smith, R. A., Hamilton-Davies, C., Omar, A., Allam, M., Bilala, O., Kindawi, A., Ewila, H., Malamas, A., Ferreira, G., Caldas, J., Fukushima, J., Osawa, E. A., Arita, E., Camara, L., Zeferino, S., Jardim, J., Gaioto, F., Dallan, L., Jatene, F. B., Kalil Filho, R., Galas, F., Hajjar, L. A., Mitaka, C., Ohnuma, T., Murayama, T., Kunimoto, F., Nagashima, M., Takei, T., Tomita, M., Mahmoud, K., Hanoura, S., Sudarsanan, S., Sivadasan, P., Othamn, H., Shouman, Y., Singh, R., Al Khulaifi, A., Mandel, I., Mikheev, S., Suhodolo, I., Kiselev, V., Svirko, Y., Podoksenov, Y., Jenkins, S. A., Griffin, R., Tovar Doncel, M. S., Lima, A., Aldecoa, C., Ince, C., Taha, A., Shafie, A., Mostafa, M., Syed, N., Hon, H., Righetti, F., Colombaroli, E., Castellano, G., Hravnak, M., Chen, L. C., Dubrawski, A. D., Clermont, G. C., Pinsky, M. R., Gonzalez, S., Macias, D., Acosta, J., Jimenez, P., Loza, A., Lesmes, A., Lucena, F., Leon, C., Bastide, M., Richecoeur, J., Frenoy, E., Lemaire, C., Sauneuf, B., Tamion, F., Nseir, S., Du Cheyron, D., Dupont, H., Maizel, J., Shaban, M., Kolko, R., AbuRageila, M., AlHussain, A., Mercado, P., Kontar, L., Titeca, D., Brazier, F., Riviere, A., Joris, M., Soupison, T., De Cagny, B., Slama, M., Wagner, J., Körner, A., Kubik, M., Kluge, S., Reuter, D., Saugel, B., Tran, T., De Bels, D., Cudia, A., Strachinaru, M., Ghottignies, P., Devriendt, J., Pierrakos, C., Martínez González, Ó., Blancas, R., Luján, J., Ballesteros, D., Martínez Díaz, C., Núñez, A., Martín Parra, C., López Matamala, B., Alonso Fernández, M., Chana, M., Huber, W., Eckmann, M., Elkmann, F., Gruber, A., Klein, I., Schmid, R. M., Lahmer, T., Moller, P. W., Sondergaard, S., Jakob, S. M., Takala, J., Berger, D., Bastoni, D., Aya, H., Toscani, L., Pigozzi, L., Rhodes, A., Cecconi, M., Ostrowska, C., Abbas, A., Mellinghoff, J., Ryan, C., Dawson, D., Cronhjort, M., Wall, O., Nyberg, E., Zeng, R., Svensen, C., Mårtensson, J., Joelsson-Alm, E., Parenti, N., Palazzi, C., Amidei, L. A., Borrelli, F. B., Campanale, S. C., Tagliazucchi, F. T., Sedoni, G. S., Lucchesi, D. L., Carella, E. C., Luciani, A. L, Mackovic, M., Maric, N., Bakula, M., Grounds, R. M., Fletcher, N., Avard, B., Zhang, P., Mezidi, M., Charbit, J., Ould-Chikh, M., Deras, P., Maury, C., Martinez, O., Capdevila, X., Hou, P., Linde-Zwirble, W. Z., Douglas, I. D., Shapiro, N. S., Ben Aicha, Y., Laribi, B., Jeribi, B., Pereira, C., Marinho, R., Antunes, R., Marinho, A., Crivits, M., Raes, M., Decruyenaere, J., Hoste, E., Bagin, V., Rudnov, V., Savitsky, A., Astafyeva, M., Korobko, I., Vein, V., Kampmeier, T., Arnemann, P., Hessler, M., Wald, A., Bockbreder, K., Morelli, A., Van Aken, H., Rehberg, S., Ertmer, C., Reddy, S., Bailey, M., Beasley, R., Bellomo, R., Mackle, D., Psirides, A., Young, P., Venkatesh, H., Ramachandran, S., Basu, A., Nair, H., Egan, S., Bates, J., Oliveira, S., Rangel Neto, N. R., Reis, F. Q., Lee, C. P., Lin, X. L., Choong, C., Eu, K. M., Sim, W. Y., Tee, K. S., Pau, J., Abisheganaden, J., Maas, K., De Geus, H., Lafuente, E., Moura, J., Doris, T. E., Monkhouse, D., Shipley, T., Kardasz, S., Gonzalez, I, Stads, S., Groeneveld, A. J., Elsayed, I., Ward, N., Raithatha, A., Steuber, A., Pelletier, C., Schroeder, S., Michael, E., Slowinski, T., Kindgen-Milles, D., Ghabina, S., Turani, F., Belli, A., Busatti, S., Barettin, G., Candidi, F., Gargano, F., Barchetta, R., Falco, M., Demirkiran, O., Kosuk, M., Bozbay, S., Weber, V., Hartmann, J., Harm, S., Linsberger, I., Eichhorn, T., Valicek, G., Miestinger, G., Hoermann, C., Faenza, S., Ricci, D., Mancini, E., Gemelli, C., Cuoghi, A., Magnani, S., Atti, M., Laddomada, T., Doronzio, A., Balicco, B., Gruda, M. C., O’Sullivan, P., Dan, V. P., Guliashvili, T., Scheirer, A., Golobish, T. D., Capponi, V. J., Chan, P. P., Kogelmann, K., Drüner, M., Jarczak, D., Belli, A. B., Martni, S. M., Cotticelli, V. C., Mounajergi, F., Morimoto, S., Hussain, I., Nadeem, A., Ghorab, K., Maghrabi, K., Kloesel, S. K., Goldfuss, C., Stieglitz, A., Stieglitz, A. S., Krstevska, L., Albuszies, G., Jimmy, G., Izawa, J., Iwami, T., Uchino, S., Takinami, M., Kitamura, T., Kawamura, T., Powell-Tuck, J. G., Crichton, S., Raimundo, M., Camporota, L., Wyncoll, D., Ostermann, M., Hana, A., De Geus, H. R., Aydogdu, M., Boyaci, N., Yuksel, S., Gursel, G., Cayci Sivri, A. B., Meza-Márquez, J., Nava-López, J., Carrillo-Esper, R., Dardashti, A., Grubb, A., Wetzstein, M., Peters, E., Njimi, H., Pickkers, P., Waraich, M., Doyle, J., Samuels, T., Forni, L., Desai, N., Baumber, R., Gunning, P., Sell, A., Lin, S., Torrence, H., O’Dwyer, M., Kirwan, C., Prowle, J., Kim, T., O’Connor, M. E., Hewson, R. W., Kirwan, C. J., Pearse, R. M., Maksoud, M., Uzundere, O., Memis, D., Ýnal, M., Gultekin, A., Turan, N., Aydin, M. A., Basar, H., Sencan, I., Kapuagasi, A., Ozturk, M., Uzundurukan, Z., Gokmen, D., Ozcan, A., Kaymak, C., Artemenko, V. A., Budnyuk, A., Pugh, R., Bhandari, S., Mauri, T., Turrini, C., Langer, T., Taccone, P., Volta, C. A., Marenghi, C., Gattinoni, L., Pesenti, A., Sweeney, L., O’Sullivan, A., Kelly, P., Mukeria, E., MacLoughlin, R., Pfeffer, M., Thomas, J. T., Bregman, G. B., Karp, G. K., Kishinevsky, E. K., Stavi, D. S., Adi, N. A., Poropat, T., Knafelj, R., Llopart, E., Batlle, M., De Haro, C., Mesquida, J., Artigas, A., Pavlovic, D., Lewerentz, L., Spassov, A., Schneider, R., De Smet, S., De Raedt, S., Derom, E., Depuydt, P, Oeyen, S., Benoit, D., Gobatto, A., Besen, B., Tierno, P., Melro, L., Mendes, P., Cadamuro, F., Park, M., Malbouisson, L. M., Civantos, B. C., Lopez, J. L., Robles, A., Figueira, J., Yus, S., Garcia, A., Oglinda, A., Ciobanu, G., Oglinda, C., Schirca, L., Sertinean, T., Lupu, V., Wolny, M., Pagano, A., Numis, F., Visone, G., Saldamarco, L., Russo, T., Porta, G., Paladino, F., Bell, C., Liu, J., Debacker, J., Lee, C., Tamberg, E., Campbell, V., Mehta, S., Kara, Ý., Yýldýrým, F., Zerman, A., Güllü, Z., Boyacý, N., Basarýk Aydogan, B., Gaygýsýz, Ü., Gönderen, K., Arýk, G., Turkoglu, M., Aygencel, G., Ülger, Z., Isýkdogan, Z., Özdedeoglu, Ö., Badoglu, M., Gaygýsýz, U., Kongpolprom, N., Sittipunt, C., Eden, A., Kokhanovsky, Y., Bursztein – De Myttenaere, S., Pizov, R., Neilans, L., MacIntyre, N., Radosevich, M., Wanta, B., Meyer, T., Smischney, N., Brown, D., Diedrich, D., Fuller, A., McLindon, P., Sim, K., Shoaeir, M., Noeam, K., Mahrous, A., Matsa, R., Ali, A., Dridi, C., Haddad, F., Pérez-Calatayud, A., Zepeda-Mendoza, A., Diaz-Carrillo, M., Arch-Tirado, E., Carbognin, S., Pelacani, L., Zannoni, F., Agnoli, A., Gagliardi, G., Cho, R., Adams, A., Lunos, S., Ambur, S., Shapiro, R., Prekker, M., Thijssen, M., Janssen, L., Foudraine, N., Voscopoulos, C. J., Freeman, J., George, E., Eversole, D., Muttini, S., Bigi, R., Villani, G., Patroniti, N., Williams, G., George, E, Waldmann, A., Böhm, S., Windisch, W., Strassmann, S., Karagiannidis, C., Karagiannidis, C. K., Waldmann, A. W., Böhm, S. B., Windisch, W. W., Persson, P., Lundin, S., Stenqvist, O., Serra, C. S., Pagano, A. P., Masarone, M. M., Rinaldi, L. R., Amelia, A. A., Fascione, M. F., Adinolfi, L. A., Ruggiero, E. R., Asota, F., O’Rourke, K., Ranjan, S., Morgan, P., DeBacker, J. W., O’Neill, L., Munshi, L., Burry, L., Fan, E., Poo, S., Mahendran, K., Fowles, J., Gerrard, C., Vuylsteke, A., Loveridge, R., Chaddock, C., Patel, S., Kakar, V., Willars, C., Hurst, T., Park, C., Best, T., Vercueil, A., Auzinger, G., Borgman, A., Proudfoot, A. G., Grins, E., Emiley, K. E., Schuitema, J., Fitch, S. J., Marco, G., Sturgill, J., Dickinson, M. G., Strueber, M., Khaghani, A., Wilton, P., Jovinge, S. M., Sampson, C., Harris-Fox, S., Cove, M. E., Vu, L. H., Sen, A., Federspiel, W. J., Kellum, J. A., Mazo Torre, C., Riera, J., Ramirez, S., Borgatta, B., Lagunes, L., Rello, J., Kuzovlev, A. K., Goloubev, A., Nenchuk, S., Karavana, V., Glynos, C., Asimakos, A., Pappas, K., Vrettou, C., Magkou, M., Ischaki, E., Stathopoulos, G., Zakynthinos, S., Kozhevnikova, I., Dalla Corte, F., Grasso, S., Casolari, P., Caramori, G., Andrianjafiarinoa, T., Randriamandrato, T., Rajaonera, T., El-Dash, S., Costa, E. L. V., Tucci, M. R., Leleu, F, Kontar, L, Bacari-Risal, G., Amato, M., El Dash, S., Remmington, Fischer, A., Squire, S., Boichat, M., Honzawa, H., Yasuda, H., Adati, T., Suzaki, S., Horibe, M., Sasaki, M., Sanui, M., Daniel, J., Miranda, H., Milinis, K., Cooper, M., Williams, G. R., McCarron, E., Simants, S., Patanwala, I., Welters, I., Su, Y., Fernández Villanueva, J., Fernández Garda, R., López Lago, A., Rodríguez Ruíz, E., Hernández Vaquero, R., Tomé Martínez de Rituerto, S., Varo Pérez, E., Lefel, N., Schaap, F., Bergmans, D., Olde Damink, S., Van de Poll, M., Tizard, K., Lister, C., Poole, L., Ringaitiene, D., Gineityte, D., Vicka, V., Norkiene, I., Sipylaite, J., O’Loughlin, A., Maraj, V., Dowling, J., Velasco, M. B., Dalcomune, D. M., Dias, E. B., Fernandes, S. L., Oshima, T., Graf, S., Heidegger, C., Genton, L., Karsegard, V., Dupertuis, Y., Pichard, C., Friedli, N., Stanga, Z., Vandersteen, L., Stessel, B., Evers, S., Van Assche, A., Jamaer, L., Dubois, J., Castro, H., Valente, J., Martins, P., Casteloes, P., Magalhaes, C., Cabral, S., Santos, M., Oliveira, B., Salgueiro, A., Duarte, S., Castro, S., Melo, M., Gray, S., Maipang, K., Bhurayanontachai, R., Grädel, L. G., Schütz, P., Langlois, P., Manzanares, W., Lemieux, M., Elke, G., Bloos, F., Heyland, D., Aramendi, I., Babo, N., Hoshino, M., Haraguchi, Y., Kajiwara, S., Mitsuhashi, T., Tsubata, T., Aida, M., Rattanapraphat, T., Kongkamol, C., Xavier, B., Koutsogiannidis, C., Moschopoulou, M., Taskin, G., Çakir, M., Güler, AK, Taskin, A., Öcal, N., Özer, S., Yamanel, L., Wong, J. M., Fitton, C., Anwar, S., Stacey, S., Aggou, M., Fyntanidou, B., Patsatzakis, S., Oloktsidou, E., Lolakos, K., Papapostolou, E., Grosomanidis, V., Gaudry, S., Desailly, V., Pasquier, P., Brun, PB, Tesnieres, AT, Ricard, JD, Dreyfuss, D., Mignon, A., White, J. C, Stilwell, A., Friedlaender, G., Peters, M., Stipulante, S., Delfosse, A., Donneau, AF, Ghuysen, A., Feldmann, C., Freitag, D., Dersch, W., Irqsusi, M., Eschbach, D., Steinfeldt, T., Wulf, H., Wiesmann, T., Cholkraisuwat, J., Beitland, S., Nakstad, E., Stær-Jensen, H., Drægni, T., Andersen, G., Jacobsen, D., Brunborg, C., Waldum-Grevbo, B., Sunde, K., Hoyland, K., Pandit, D., Hayakawa, K., Kotzampassi, K., Loukipoudi, L., Doumaki, E., Admiraal, M. M., Van Assen, M., Van Putten, M. J., Tjepkema-Cloostermans, M., Van Rootselaar, A. F., Ragusa, F., Marudi, A., Baroni, S., Gaspari, A., Bertellini, E., Abdullah, T., Abdel Monem, S., Alcorn, S., McNeill, S., Russell, S., Eertmans, W., Genbrugge, C., Meex, I., Dens, J., Jans, F., De Deyne, C., Avard, B, Burns, R, Patarchi, A., Spina, T., Tanaka, H., Otani, N., Ode, S., Ishimatsu, S., Cho, J., Moon, J. B., Park, C. W., Ohk, T. G., Shin, M. C., Won, M. H., Dakova, S., Ramsheva, Z., Ramshev, K., Marudi, A, Baroni, S, Gaspari, A, Bertellini, E, Ozcan, P. E., Sencer, S., Ulusoy, C., Fallenius, M., Skrifvars, M. B., Reinikainen, M., Bendel, S., Raj, R., Abu-Habsa, M., Hymers, C., Borowska, A., Sivadhas, H., Sahiba, S., Perkins, S., Rubio, J., Rubio, J. A., Sierra, R., English, S., Chasse, M., Turgeon, A., Lauzier, F., Griesdale, D., Garland, A., Fergusson, D., Zarychanski, R., Tinmouth, A., Van Walraven, C., Montroy, K., Ziegler, J., Dupont Chouinard, R., Carignan, R., Dhaliwal, A., Lum, C., Sinclair, J., Pagliarello, G., McIntyre, L., Groza, T., Moreau, N., Castanares-Zapatero, D., Hantson, P., Carbonara, M., Ortolano, F., Zoerle, T., Magnoni, S., Pifferi, S., Conte, V., Stocchetti, N., Carteron, L., Suys, T., Patet, C., Quintard, H., Oddo, M., Spatenkova, V., Pokorna, E., Suchomel, P., Ebert, N., Bylinski, T., Hawthorne, C., Shaw, M., Piper, I., Kinsella, J., Kink, A. K., Rätsep, I. R., Boutin, A., Moore, L., Lacroix, J., Lessard-Bonaventure, P., Turgeon, A. F., Green, R., Erdogan, M., Butler, M., Desjardins, P., Fergusson, D. A., Goncalves, B., Vidal, B., Valdez, C., Rodrigues, A. C., Miguez, L., Moralez, G., Hong, T., Kutz, A., Hausfater, P., Amin, D., Struja, T., Haubitz, S., Huber, A., Brown, T., Collinson, J., Pritchett, C., Slade, T., Le Guen, M., Hellings, S., Ramsaran, R., Alsheikhly, A., Abe, T., Kanapeckaite, L., Bahl, R., Russell, M. Q., Real, K. J., Lyon, R. M., Oveland, N. P., Penketh, J., Mcdonald, M., Kelly, F., Alfafi, M., Almutairi, W., Alotaibi, B., Van den Berg, A. E, Schriel, Y., Dawson, L., Meynaar, I. A., Silva, D., Fernandes, S., Gouveia, J., Santos Silva, J., Foley, J., Kaskovagheorgescu, A., Evoy, D., Cronin, J., Ryan, J., Huck, M., Hoffmann, C., Renner, J., Laitselart, P., Donat, N., Cirodde, A., Schaal, J. V., Masson, Y., Nau, A., Howarth, O., Davenport, K., Jeanrenaud, P., Raftery, S., MacTavish, P., Devine, H., McPeake, J., Daniel, M., Quasim, T., Alrabiee, S., Alrashid, A., Gundogan, O., Bor, C., Akýn Korhan, E., Demirag, K., Uyar, M., Frame, F., Ashton, C., Bergstrom Niska, L., Dilokpattanamongkol, P., Suansanae, T., Suthisisang, C., Morakul, S., Karnjanarachata, C., Tangsujaritvijit, V., Mahmood, S., Al Thani, H., Almenyar, A., Morton, S. E., Chiew, Y. S., Pretty, C., Chase, J. G., Shaw, G. M., Kordis, P., Grover, V., Kuchyn, I., Bielka, K., Aidoni, Z., Stavrou, G., Skourtis, C., Lee, S. D., Williams, K., Weltes, I. D., Berhane, S., Arrowsmith, C., Peters, C., Robert, S., Panerai, R. B., Robinson, T. G., Borg-Seng-Shu, E., De Lima Oliveira, M., Mian, N. C., Nogueira, R., Zeferino, S. P., Jacobsen Teixeira, M., Killeen, P., McPhail, M., Bernal, W., Maggs, J., Wendon, J., Hughes, T., Taniguchi, L. U., Siqueira, E. M., Vieira Jr, J. M., Azevedo, L. C., Ahmad, A. N., Helme, E., Hadfield, S., Shak, J., Senver, C., Howard-Griffin, R., Wacharasint, P., Fuengfoo, P., Sukcharoen, N., Rangsin, R., Sbiti-Rohr, D., Na, H., Song, S., Lee, S., Jeong, E., Lee, K., Zoumpelouli, E., Volakli, E. A, Chrysohoidou, V., Charisopoulou, K., Kotzapanagiotou, E., Manavidou, K., Stathi, Z., AlGhamdi, B., Marashly, Q., Zaza, K., Khurshid, M., Ali, Z., Malgapo, M., Jamil, M., Shafquat, A., Shoukri, M., Hijazi, M., Rocha, F. A., Ebecken, K., Rabello, L. S., Lima, M. F., Hatum, R., De Marco, F. V., Alves, A., Pinto, J. E., Godoy, M., Brasil, P. E., Bozza, F. A., Salluh, J. I., Soares, M., Krinsley, J., Kang, G., Perry, J., Hines, H., Wilkinson, K. M., Tordoff, C., Sloan, B., Bellamy, M. C., Moreira, E., Verga, F., Barbato, M., Burghi, G., Soares, M, Silva, U. V., Torelly, A. P., Kahn, J. M., Angus, D. C., Knibel, M. F., Marshall, R., Gilpin, T., Mota, D., Loureiro, B., Dias, J., Afonso, O., Coelho, F., Martins, A., Faria, F., Al Orainni, H., AlEid, F., Tlaygeh, H., Itani, A., Hejazi, A., Messika, J., Ricard, J. D., Guillo, S., Pasquet, B., Dubief, E., Tubach, F., James, K., Temblett, P., Davies, L., Lynch, C., Pereira, S., Cavaco, S., Fernandes, J., Moreira, I., Almeida, E., Seabra Pereira, F., Malheiro, M., Cardoso, F., Aragão, I., Cardoso, T., Fister, M., Muraray Govind, P., Brahmananda Reddy, N., Pratheema, R., Arul, E. D., Devachandran, J., Chin-Yee, N., D’Egidio, G., Thavorn, K., Kyeremanteng, K., Murchison, A. G., Swalwell, K., Mandeville, J., Stott, D., Guerreiro, I., Goossens, C., Marques, M. B., Derde, S., Vander Perre, S., Dufour, T., Thiessen, S. E., Güiza, F., Janssens, T., Hermans, G., Vanhorebeek, I., De Bock, K., Van den Berghe, G., Langouche, L., Miles, B., Madden, S., Weiler, M., Marques, P., Rodrigues, C., Boeira, M., Brenner, K., Leães, C., Machado, A., Townsend, R., Andrade, J., Kishore, R., Fenlon, C., Fiks, T., Ruijter, A., Te Raa, M., Spronk, P., Docherty, P., Dickson, J., Moltchanova, E., Scarrot, C., Hall, T., Ngu, W. C., Jack, J. M., Pavli, A., Gee, X., Akin Korhan, E., Shirazy, M., Fayed, A., Gupta, S., Kaushal, A., Dewan, S., Varma, A., Ghosh, E., Yang, L., Eshelman, L., Lord, B., Carlson, E., Broderick, R., Ramos, J., Forte, D., Yang, F., Feeney, J., Wilkinson, K., Shuker, K., Faulds, M., Bryden, D., England, L., Shuker, K, Tridente, A, Faulds, M, Matheson, A, Gaynor, J., Bryden, D, Peroni, B., Daglius-Dias, R., Miranda, L., Cohen, C., Carvalho, C., Velasco, I., Kelly, J. M., Neill, A., Rubenfeld, G., Masson, N., Min, A., Boezeman, E., Hofhuis, J., Hovingh, A., De Vries, R., Cabral-Campello, G., Van Mol, M., Nijkamp, M., Kompanje, E., Ostrowski, P., Kiss, K., Köves, B., Csernus, V., Molnár, Z., Hoydonckx, Y., Vanwing, S., Medo, V., Galvez, R., Miranda, J. P., Stone, C., Wigmore, T., Arunan, Y., Wheeler, A., Wong, Y., Poi, C., Gu, C., Molmy, P., Van Grunderbeeck, N., Nigeon, O., Lemyze, M., Thevenin, D., Mallat, J., Correa, M., Carvalho, R. T., Fernandez, A., McBride, C., Koonthalloor, E., Walsh, C., Webber, A., Ashe, M., Smith, K., Volakli, E. A., Dimitriadou, M., Mantzafleri, P., Vrani, O., Arbouti, A., Varsami, T., Bollen, J. A., Van Smaalen, T. C., De Jongh, W. C., Ten Hoopen, M. M., Ysebaert, D., Van Heurn, L. W., Van Mook, W. N., Roze des Ordons, A., Couillard, P., Doig, C., Van Keer, R. V., Deschepper, R. D., Francke, A. F., Huyghens, L. H., Bilsen, J. B., Nyamaizi, B., Dalrymple, C., Dobru, A., Marrinan, E., Ankuli, A., Struthers, R., Crawford, R., Mactavish, P., Morelli, P., Degiovanangelo, M., Lemos, F., MArtinez, V., Cabrera, J., Rutten, A., Van Ieperen, S., De Geer, S., Van Vugt, M., Der Kinderen, E., Giannini, A., Miccinesi, G, Marchesi, T, and Prandi, E
- Subjects
Protocol (science) ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,French ,030208 emergency & critical care medicine ,Compression (physics) ,Critical Care and Intensive Care Medicine ,Meeting Abstracts ,language.human_language ,law.invention ,03 medical and health sciences ,0302 clinical medicine ,law ,Ventilation (architecture) ,Emergency medicine ,language ,Medicine ,030212 general & internal medicine ,Cardiopulmonary resuscitation ,business - Abstract
Table of contents P001 - Sepsis impairs the capillary response within hypoxic capillaries and decreases erythrocyte oxygen-dependent ATP efflux R. M. Bateman, M. D. Sharpe, J. E. Jagger, C. G. Ellis P002 - Lower serum immunoglobulin G2 level does not predispose to severe flu. J. Solé-Violán, M. López-Rodríguez, E. Herrera-Ramos, J. Ruíz-Hernández, L. Borderías, J. Horcajada, N. González-Quevedo, O. Rajas, M. Briones, F. Rodríguez de Castro, C. Rodríguez Gallego P003 - Brain protective effects of intravenous immunoglobulin through inhibition of complement activation and apoptosis in a rat model of sepsis F. Esen, G. Orhun, P. Ergin Ozcan, E. Senturk, C. Ugur Yilmaz, N. Orhan, N. Arican, M. Kaya, M. Kucukerden, M. Giris, U. Akcan, S. Bilgic Gazioglu, E. Tuzun P004 - Adenosine a1 receptor dysfunction is associated with leukopenia: A possible mechanism for sepsis-induced leukopenia R. Riff, O. Naamani, A. Douvdevani P005 - Analysis of neutrophil by hyper spectral imaging - A preliminary report R. Takegawa, H. Yoshida, T. Hirose, N. Yamamoto, H. Hagiya, M. Ojima, Y. Akeda, O. Tasaki, K. Tomono, T. Shimazu P006 - Chemiluminescent intensity assessed by eaa predicts the incidence of postoperative infectious complications following gastrointestinal surgery S. Ono, T. Kubo, S. Suda, T. Ueno, T. Ikeda P007 - Serial change of c1 inhibitor in patients with sepsis – A prospective observational study T. Hirose, H. Ogura, H. Takahashi, M. Ojima, J. Kang, Y. Nakamura, T. Kojima, T. Shimazu P008 - Comparison of bacteremia and sepsis on sepsis related biomarkers T. Ikeda, S. Suda, Y. Izutani, T. Ueno, S. Ono P009 - The changes of procalcitonin levels in critical patients with abdominal septic shock during blood purification T. Taniguchi, M. O P010 - Validation of a new sensitive point of care device for rapid measurement of procalcitonin C. Dinter, J. Lotz, B. Eilers, C. Wissmann, R. Lott P011 - Infection biomarkers in primary care patients with acute respiratory tract infections – Comparison of procalcitonin and C-reactive protein M. M. Meili, P. S. Schuetz P012 - Do we need a lower procalcitonin cut off? H. Hawa, M. Sharshir, M. Aburageila, N. Salahuddin P013 - The predictive role of C-reactive protein and procalcitonin biomarkers in central nervous system infections with extensively drug resistant bacteria V. Chantziara, S. Georgiou, A. Tsimogianni, P. Alexandropoulos, A. Vassi, F. Lagiou, M. Valta, G. Micha, E. Chinou, G. Michaloudis P014 - Changes in endotoxin activity assay and procalcitonin levels after direct hemoperfusion with polymyxin-b immobilized fiber A. Kodaira, T. Ikeda, S. Ono, T. Ueno, S. Suda, Y. Izutani, H. Imaizumi P015 - Diagnostic usefullness of combination biomarkers on ICU admission M. V. De la Torre-Prados, A. Garcia-De la Torre, A. Enguix-Armada, A. Puerto-Morlan, V. Perez-Valero, A. Garcia-Alcantara P016 - Platelet function analysis utilising the PFA-100 does not predict infection, bacteraemia, sepsis or outcome in critically ill patients N. Bolton, J. Dudziak, S. Bonney, A. Tridente, P. Nee P017 - Extracellular histone H3 levels are inversely correlated with antithrombin levels and platelet counts and are associated with mortality in sepsis patients G. Nicolaes, M. Wiewel, M. Schultz, K. Wildhagen, J. Horn, R. Schrijver, T. Van der Poll, C. Reutelingsperger P018 - Il-8: is this a more reliable biomarker for sepsis severity than CRP, Procalcitonin, E-selectin, IL-6 and TNF-[alpha] S. Pillai, G. Davies, G. Mills, R. Aubrey, K. Morris, P. Williams, P. Evans P019 - Relation between adrenomedullin and short-term outcome in ICU patients: Results from the frog ICU study E. G. Gayat, J. Struck, A. Cariou, N. Deye, B. Guidet, S. Jabert, J. Launay, M. Legrand, M. Léone, M. Resche-Rigon, E. Vicaut, A. Vieillard-Baron, A. Mebazaa P020 - Impact of disease severity assessment on performance of heparin-binding protein for the prediction of septic shock R. Arnold, M. Capan, A. Linder, P. Akesson P021 - Kinetics and prognostic value of presepsin (sCD14) in septic patients. A pilot study M. Popescu, D. Tomescu P022 - Comparison of CD64 levels performed by the facs and accellix systems C. L. Sprung, R. Calderon Morales, G. Munteanu, E. Orenbuch-Harroch, P. Levin, H. Kasdan, A. Reiter, T. Volker, Y. Himmel, Y. Cohen, J. Meissonnier P023 - Diagnosing sepsis in 5 minutes: Nanofluidic technology study with pancreatic-stone protein (PSP/ reg) L. Girard, F. Rebeaud P024 - How nanotechnology-based approaches could contribute to sepsis prevention, diagnosis and treatment I. Herrmann P025 - Il7r transcriptional expression analysis during septic shock B. Delwarde, E. Peronnet, E. Cerrato, F. Venet, A. Lepape, T. Rimmelé, G. Monneret, J. Textoris P026 - Disbalance of microbial metabolites of aromatic acids affects the severity in critically ill patients N. Beloborodova, V. Moroz, A. Osipov, A. Bedova, Y. Sarshor, A. Pautova, A. Sergeev, E. Chernevskaya P027 - Copeptin predicts 10-year all-cause mortality in community patients J. Odermatt, R. Bolliger, L. Hersberger, M. Ottiger, M. Christ-Crain, B. Mueller, P. Schuetz P028 - Identification of differential proteomic response in septic patients secondary to community and hospital acquired pneumonia N. K. Sharma, A. K. Tashima, M. K. Brunialti, F. R. Machado, M. Assuncao, O. Rigato, R. Salomao P029 - Monocyte HLA-DR expression in community-acquired bacteremic sepsis - dynamics associated to aetiology and prediction of secondary sepsis S. C. Cajander, G. Rasmussen, E. Tina, B. Söderquist, J. Källman, K. Strålin P030 - Soluble B- and T-lymphocyte attenuator: A possible prognostic marker in sepsis A. L. Lange, J. S. Sundén-Cullberg, A. M. Magnuson, O. H. Hultgren P031 - Fractal dimension: A new biomarker for quantifying clot microstructure in patients across the sepsis spectrum G. Davies, S. Pillai, G. Mills, R. Aubrey, K. Morris, P. Williams, P. Evans P032 - Comparison between the new biomarker for coagulation, clot microstructure (Df) with rotational thromboelastometry (ROTEM) in patients across the sepsis spectrum S. Pillai, G. Davies, G. Mills, R. Aubrey, K. Morris, P. Williams, P. Evans P033 - Changes in fibrinolysis across the sepsis spectrum: The use of rotational thromboelastometry (ROTEM) lysis index (LI60) and D-Dimer concentration S. Pillai, G. Davies, G. Mills, R. Aubrey, K. Morris, P. Williams, P. Evans P034 - The intensive care infection score – a promising marker for the prediction of infection and its severity. P. Van der Geest, M. Mohseni, J. Linssen, R. De Jonge, S. Duran, J. Groeneveld P035 - Challenges in the clinical diagnosis of sepsis R. Miller III, B. K. Lopansri, L. C. McHugh, A. Seldon, J. P. Burke P036 - Does zero heat flux thermometry more accurately identify sepsis on intensive care? J. Johnston, R. Reece-Anthony, A. Bond, A. Molokhia P037 - Advancing quality (AQ) sepsis programme: Improving early identification & treatment of sepsis in North West England. C. Mcgrath, E. Nsutebu P038 - Prehospital transport of acute septic patients P. Bank Pedersen, D. Pilsgaard Henriksen, S. Mikkelsen, A. Touborg Lassen P039 - Vasodilatory plant extracts gel as an alternative treatment for fever in critically ill patients R. Tincu, C. Cobilinschi, D. Tomescu, Z. Ghiorghiu, R. Macovei P040 - Host response and outcome of hypothermic sepsis M. A. Wiewel, M. B. Harmon, L. A. Van Vught, B. P. Scicluna, A. J. Hoogendijk, J. Horn, A. H. Zwinderman, O. L. Cremer, M. J. Bonten, M. J. Schultz, T. Van der Poll, N. P. Juffermans, W. J. Wiersinga P041 - Septic shock alert over SIRS criteria has an impact on outcome but needs to be revised G. Eren, Y Tekdos, M. Dogan, O. Acicbe, E. Kaya, O. Hergunsel P042 - Association between previous prescription of βblockers and mortality rate among septic patients: A retrospective observational study S. Alsolamy, G. Ghamdi, L. Alswaidan, S. Alharbi, F. Alenezi, Y. Arabi P043 - Recognition and treatment of sepsis on labour ward– teaching & information resources can improve knowledge J. Heaton, A. Boyce, L. Nolan, J. Johnston, A. Dukoff-Gordon, A. Dean, A. Molokhia P044 - Culture negative sepsis in the ICU – what is unique to this patient population? T. Mann Ben Yehudah P045 - Organ dysfunction in severe sepsis patients identified in administrative data in Germany, 2007-2013 C. Fleischmann, D. Thomas-Rueddel, C. Haas, U. Dennler, K. Reinhart P046 - A comparison of residents’ knowledge regarding; the Surviving Sepsis Campaign 2012 guideline O. Suntornlohanakul, B. Khwannimit P047 - Effectiveness of a septic shock bundle to improve outcomes in the ICU F. Breckenridge, A. Puxty P048 - Dose of norepinephrine in the first 24 hours as a parameter evaluating the effectiveness of treatment in patients with severe sepsis and septic shock P. Szturz, P. Folwarzcny, J. Svancara, R. Kula, P. Sevcik P049 - Norepinephrine or vasopressin + norepinephrine in septic shock. A retrospective series of 39 patients L. Caneva, A. Casazza, E. Bellazzi, S. Marra, L. Pagani, M. Vetere, R. Vanzino, D. Ciprandi, R. Preda, R. Boschi, L. Carnevale P050 - Methylene blue effectiveness as contributory treatment in patients with septic shock V. Lopez, M. Aguilar Arzapalo, L. Barradas, A. Escalante, J. Gongora, M. Cetina P051 - Coagulation disorders in patients with severe sepsis and DIC evaluated with thromboelastometry. B Adamik, D Jakubczyk, A Kübler P052 - Frequency and outcome of early sepsis-associated coagulopathy A. Radford, T. Lee, J. Singer, J. Boyd, D. Fineberg, M. Williams, J. Russell P053 - Assessment of coagulopathy in cancer patients with severe sepsis or septic shock. A case-control pilot study E. Scarlatescu, D. Tomescu, G. Droc, S. Arama P054 - Thromboelastometry in critically ill patients with disseminated intravascular coagulation M. Müller, M. Straat, S. S. Zeerleder, N. P. Juffermans P055 - Cessation of a preexisting chronic antiplatelet therapy is associated with increased mortality rates in severe sepsis and septic shock C. F. Fuchs, C. S. Scheer, S. W. Wauschkuhn, M. V. Vollmer, K. M. Meissner, S. K. Kuhn, K. H. Hahnenkamp, S. R. Rehberg, M. G. Gründling P056 - Neutrophil Extracellular Traps (NETs) production under hypoxic condition N. Yamamoto, M. Ojima, S. Hamaguchi, T. Hirose, Y. Akeda, R. Takegawa, O. Tasaki, T. Shimazu, K. Tomono P057 - Impact of ultraviolet air sterilizer in intensive care unit room, and clinical outcomes of patients E. Gómez-Sánchez, M. Heredia-Rodríguez, E. Álvarez-Fuente, M. Lorenzo-López, E. Gómez-Pesquera, M. Aragón-Camino, P. Liu-Zhu, A. Sánchez-López, A. Hernández-Lozano, M. T. Peláez-Jareño, E. Tamayo P058 - Focus of infection in severe sepsis - comparison of administrative data and prospective cohorts from Germany D. O. Thomas-Rüddel, C. Fleischmann, C. Haas, U. Dennler, K. Reinhart P059 - “Zero CLABSI” – can we get there? Obstacles on the 4 year journey and our strategies to overcome them – experience from an Indian ICU V. Adora, A. Kar, A. Chakraborty, S. Roy, A. Bandyopadhyay, M. Das P060 - Novel molecular techniques to identify central venous catheter (CVC) associated blood stream infections (BSIs) T. Mann Ben Yehudah, G. Ben Yehudah, M. Salim, N. Kumar, L. Arabi, T. Burger, P. Lephart, E. Toth-martin P061 - Zero clabsi” – can we get there? Obstacles on the 4 year journey and our strategies to overcome them – experience from an Indian ICU R. Rao, A. Kar, A. Chakraborty P062 - Prevention of central line-associated bloodstream infections in intensive care units: An international online survey C. Valencia, N. Hammami, S. Blot, J. L. Vincent, M. L. Lambert P063 - 30 days antimicrobial efficacy of non-leaching central venous catheters J. Brunke, T. Riemann, I. Roschke P064 - Efficacy of noble metal alloy-coated catheter in prevention of bacteriuria R. Tincu, C. Cobilinschi, D. Tomescu, Z. Ghiorghiu, R. Macovei P065 - Predicting bacteremic urinary tract infection in community setting: A prospective observational study S. Nimitvilai, K. Jintanapramote, S. Jarupongprapa P066 - Eight-year analysis of acinetobacter spp. monobacteremia in surgical and medical intensive care units at university hospital in Lithuania D. Adukauskiene, D. Valanciene P067 - Group A and group B streptococcal infections in intensive care unit – our experience in a tertiary centre G. Bose, V. Lostarakos, B. Carr P068 - Improved detection of spontaneous bacterial peritonitis by uritop + tm strip test and inoculation of blood culture bottles with ascitic fluid S. Khedher, A. Maaoui, A. Ezzamouri, M. Salem P069 - Increased risk of cellulitis in patients with congestive heart failure: a population based cohort study J. Chen P070 - Outcomes of severe cellulitis and necrotizing fasciitis in the critically ill D. R. Cranendonk, L. A. Van Vught, M. A. Wiewel, O. L. Cremer, J. Horn, M. J. Bonten, M. J. Schultz, T. Van der Poll, W. J. Wiersinga P071 - Botulism outbreak associated with people who inject drugs (PWIDs) in Scotland. M. Day, G. Penrice, K. Roy, P. Robertson, G. Godbole, B. Jones, M. Booth, L. Donaldson P072 - Surveillance of ESBL-producing enterobacteriaceae fecal carriers in the ICU Y. Kawano, H. Ishikura P073 - Prevalence of ESBL and carbapenemase producing uropathogens in a newly opened hospital in south India S. Sreevidya, N. Brahmananda Reddy, P. Muraray Govind, R. Pratheema, J. Devachandran Apollo Speciality Hospital - OMR, Chennai, India P074 - Prevalence, risk factors and outcomes of methicillin-resistant staphylococcus aureus nasal colonization in critically ill patients H. Al-Dorzi, M. Almutairi, B. Alhamadi, A. Crizaldo Toledo, R. Khan, B. Al Raiy, Y. Arabi P075 - Multidrug-resistant Acinetobacter baumannii infection in intensive care unit patients in a hospital with building construction: Is there an association? H. Talaie P076 - Multidrug-resistant organisms in a Dutch ICU J. A. Van Oers, A. Harts, E. Nieuwkoop, P. Vos P077 - Epidemiology and risk factors of ICU acquired infections caused by multidrug-resistant gram negative bacilli Y. Boussarsar, F. Boutouta, S. Kamoun, I. Mezghani, S. Koubaji, A. Ben Souissi, A. Riahi, M. S. Mebazaa P078 - Improving outcomes of severe infections by multidrug-resistant pathogens with polyclonal IgM-enriched immunoglobulins E. Giamarellos-Bourboulis, N. Tziolos, C. Routsi, C. Katsenos, I. Tsangaris, I. Pneumatikos, G. Vlachogiannis, V. Theodorou, A. Prekates, E. Antypa, V. Koulouras, N. Kapravelos, C. Gogos, E. Antoniadou, K. Mandragos, A. Armaganidis P079 - Must change the medical practice in ICU? A. R. Robles Caballero, B. Civantos, J. C. Figueira, J. López P080 - Mediterranean spotted fever in an infectious diseases intensive care unit A. Silva-Pinto, F. Ceia, A. Sarmento, L. Santos P081 - Clinical features and outcomes of patients with Middle East respiratory syndrome requiring admission to a saudi intensive care unit: A retrospective analysis of 31 cases G. Almekhlafi, Y. Sakr P082 - The ICU response to a hospital outbreak of Middle East respiratory syndrome coronavirus infection H. Al-Dorzi, R. Khan, S. Baharoon, A. Aldawood, A. Matroud, J. Alchin, S. Al Johani, H. Balkhy, Y. Arabi P083 - Middle East respiratory syndrome: Surveillance data analysis S. Alsolamy, S. Y. Yousif, B. O. Alotabi, A. S. Alsaawi P085 - Use of Taqman array card molecular diagnostics in severe pneumonia: A case series J. Ang, MD Curran, D. Enoch, V. Navapurkar, A. Conway Morris P086 - ‘BUNS’: An investigation protocol improves the ICU management of pneumonia R. Sharvill, J. Astin P087 - Pneumonia in patients following secondary peritonitis: epidemiological features and impact on mortality M. Heredia-Rodríguez, E. Gómez-Sánchez, M. T. Peláez-Jareño, E. Gómez-Pesquera, M. Lorenzo-López, P. Liu-Zhu, M. Aragón-Camino, A. Hernández-Lozano, A. Sánchez-López, E. Álvarez-Fuente, E. Tamayo P088 - The use of the “CURB-65 score” by emergency room clinicians in a large teaching hospital J. Patel, C. Kruger P089 - Incidence of community acquired pneumonia with viral infection in mechanically ventilated patients in the medical intensive care unit J. O’Neal, H. Rhodes, J. Jancik P090 - The SAATELLITE Study: Prevention of S aureus Nosocomial Pneumonia (NP) with MEDI4893, a Human Monoclonal Antibody (mAb) Against S aureus B. François, P. F. Laterre, P. Eggimann, A. Torres, M. Sánchez, P. F. Dequin, G. L. Bassi, J. Chastre, H. S. Jafri P091 - Risk factors and microbiological profile for nosocomial infections in trauma patients M. Ben Romdhane, Z. Douira, S. Kamoun, M. Bousselmi, A. Ben Souissi, Y. Boussarsar, A. Riahi, M.S. Mebazaa P092 - Correlation between percentages of ventilated patients developed vap and use of antimicrobial agents in ICU patients. A. Vakalos, V. Avramidis P093 - A comparison of two ventilator associated pneumonia surveillance techniques T. H. Craven, G. Wojcik, K. Kefala, J. McCoubrey, J. Reilly, R. Paterson, D. Inverarity, I. Laurenson, T. S. Walsh P094 - Lung ultrasound before and after fiberbronchoscopy - modifications may improve ventilator-associated pneumonia diagnosis S. Mongodi, B. Bouhemad, A. Orlando, A. Stella, G. Via, G. Iotti, A. Braschi, F. Mojoli P095 - Comparing the accuracy of predictors of mortality in ventilator-associated pneumonia M. Haliloglu, B. Bilgili, U. Kasapoglu, I. Sayan, M. Süzer Aslan, A. Yalcın, I. Cinel P096 - Impact of pRBCs transfusion on percentage of ventilated patients developed VAP in ICU patients A. Vakalos, V. Avramidis P097 - The impact of a series of interventions on the rate of ventilator associated pneumonia in a large teaching hospital H. E. Ellis, K. Bauchmuller, D. Miller, A Temple P098 - The EVADE study: Prevention of Nosocomial Pneumonia (NP) caused by P aeruginosa with MEDI3902, a Novel Bispecific Monoclonal Antibody, against P aeruginosa virulence factors J. Chastre, B. François, A. Torres, C. E. Luyt, M. Sánchez, M. Singer, H. S. Jafri P099 - Short-term inhaled colistin adjunctive therapy for ventilator-associated pneumonia Y. Nassar, M. S. Ayad P100 - Effect of aerosolised colistin on weaning from mechanical ventilation A. Trifi, S. Abdellatif, F. Daly, R. Nasri, S. Ben Lakhal P101 - Septic shock is an independent risk factor for colistin-induced severe acute kidney injury: a retrospective cohort study B. Bilgili, M. Haliloglu, F. Gul, I. Cinel P102 - Nosocomial pneumonia - emphasis on inhaled tobramycin A. Kuzovlev, A. Shabanov, S. Polovnikov, V. Moroz P103 - In vitro evaluation of amikacin inhale and commercial nebulizers in a mechanical ventilator N. Kadrichu, T. Dang, K. Corkery, P. Challoner P104 - The effects of nebulized amikacin/fosfomycin and systemic meropenem on severe amikacin-resistant meropenem-susceptible P.aeruginosa pneumonia G. Li Bassi, E. Aguilera, C. Chiurazzi, C. Travierso, A. Motos, L. Fernandez, R. Amaro, T. Senussi, F. Idone, J. Bobi, M. Rigol, A. Torres P105 - Optimization of gentamicin peak concentrations in critically ill patients C. J. Hodiamont, N. P. Juffermans, J. M. Janssen, C. S. Bouman, R. A. Mathôt, M. D. De Jong, R. M. Van Hest P106 - Systematic review of cefepime induced neurotoxicity L. Payne, G. L. Fraser P107 - Unasyn® causes QT prolongation during treatment of intensive care patients B. Tudor, M. Lahner, G. Roth, C. Krenn P108 - Comparative study between teicoplanin and vancomycin in methicillin-resistant staphylococcus aureus (mrsa) infectious of toxicological intensive care unit (ticu) patients – Tehran, Iran H. Talaie P109 - Phage therapy against antimicrobial resistance, design of the first clinical study phagoburn P. Jault, J. Gabard, T. Leclerc, S. Jennes, Y. Que, A. Rousseau, F. Ravat P110 - Antibiotic dosing errors in critically ill patients with severe sepsis or septic shock H. Al-Dorzi, A. Eissa, S. Al-Harbi, T. Aldabbagh, R. Khan, Y. Arabi P111 - Does empiric antifungal therapy improve survival in septic critically ill patients? (immunocompromised excluded) A. Trifi, S. Abdellatif, F. Daly, R. Nasri, S. Ben Lakhal P112 - Neurocysticercosis-Qatar experience F. Paramba, N. Purayil, V. Naushad, O. Mohammad, V. Negi, P. Chandra P113 - Early indicators in acute haemorrhagic shock A. Kleinsasser P114 - Filtering of red blood cells reduces the inflammatory response of pulmonary cells in an in vitro model of mechanical ventilation M. R. Witrz, J. F. Buchner-Doeven, A. M. Tuip-de Boer, J. C. Goslings, N. P. Juffermans P115 - Microparticles from red blood cell transfusion induce a pro-coagulant and pro-inflammatory endothelial cell response M. Van Hezel, M. Straat, A Boing, R Van Bruggen, N Juffermans P116 - The contribution of cytokines on thrombosis development during hospitalization in ICU D. Markopoulou, K. Venetsanou, V. Kaldis, D. Koutete, D. Chroni, I. Alamanos P117 - Prophylactic enoxaparin dosing and adjustment through anti-xa monitoring in an inpatient burn unit L. Koch, J. Jancik, H. Rhodes, E. Walter P118 - Determination of optimal cut-off values of haemoglobin, platelet count and fibrinogen at 24 hours after injury associated with mortality in trauma patients K. Maekawa, M. Hayakawa, S. Kushimoto, A. Shiraishi, H. Kato, J. Sasaki, H. Ogura, T. Matauoka, T. Uejima, N. Morimura, H. Ishikura, A. Hagiwara, M. Takeda P119 - Trauma-induced coagulopathy - prothrombin complex concentrate vs fresh frozen plasma O. Tarabrin, S. Shcherbakow, D. Gavrychenko, G. Mazurenko, V. Ivanova, O. Chystikov P120 - First study to prove the superiority of prothrombin complex concentrates on mortality rate over fresh frozen plasma in patients with acute bleeding C. Plourde, J. Lessard, J. Chauny, R. Daoust P121 - Prothrombin complex concentrate vs fresh frozen plasma in obstetric massive bleeding S. Shcherbakow, O. Tarabrin, D. Gavrychenko, G. Mazurenko, O. Chystikov P122 - Impact of FFP transfusion on VAP in ICU patients A. Vakalos, V. Avramidis P123 - Preoperative platelet function test and the thrombin generation assay are predictive for blood loss after cardiac surgery L. Kropman, L. In het Panhuis, J. Konings, D. Huskens, E. Schurgers, M. Roest, B. De Laat, M. Lance P124 - Rotational thromboelastometry versus standard coagulation tests before surgical interventions M. Durila, P. Lukas, M. Astraverkhava, J. Jonas P125 - Correction of impaired clot quality and stability by fibrinogen and activated prothrombin complex concentrate in a model of severe thrombocytopenia I. Budnik, B. Shenkman P126 - Assessment of point-of-care prothrombin time analyzer as a monitor after cardiopulmonary bypass H. Hayami, Y. Koide, T. Goto P127 - Disseminated intravascular coagulation (dic) is underdiagnosed in critically ill patients: do we need d-dimer measurements? R. Iqbal, Y. Alhamdi, N. Venugopal, S. Abrams, C. Downey, C. H. Toh, I. D. Welters P128 - Validity of the age-adjusted d-dimer cutoff in patients with COPD B. Bombay, J. M. Chauny, R. D. Daoust, J. L. Lessard, M. M. Marquis, J. P. Paquet P129 - A scoping review of strategies for prevention and management of bleeding following paediatric cardiopulmonary bypass surgery K. Siemens, D. Sangaran, B. J. Hunt, A. Durward, A. Nyman, I. A. Murdoch, S. M. Tibby P130 - Nadir hemoglobulin during cardiopulmonary bypass: impact on postoperative morbidity and mortality F. Ampatzidou, D. Moisidou, E. Dalampini, M. Nastou, E. Vasilarou, V. Kalaizi, H. Chatzikostenoglou, G. Drossos P131 - Red blood cell transfusion do not influence the prognostic value of RDW in critically ill patients S. Spadaro, A. Fogagnolo, T. Fiore, A. Schiavi, V. Fontana, F. Taccone, C. Volta P132 - Reasons for admission in the paediatric intensive care unit and the need for blood and blood products transfusions E. Chochliourou, E. Volakli, A. Violaki, E. Samkinidou, G. Evlavis, V. Panagiotidou, M. Sdougka P133 - The implementation of a massive haemorrhage protocol (mhp) for the management of major trauma: a ten year, single-centre study R. Mothukuri, C. Battle, K. Guy, G. Mills, P. Evans P134 - An integrated major haemorrhage protocol for pre-hospital and retrieval medical teams J. Wijesuriya, S. Keogh P135 - The impact of transfusion thresholds on mortality and cardiovascular events in patients with cardiovascular disease (non-cardiac surgery): a systematic review and meta-analysis A. Docherty, R. O’Donnell, S. Brunskill, M. Trivella, C. Doree, L. Holst, M. Parker, M. Gregersen, J. Almeida, T. Walsh, S. Stanworth P136 - The relationship between poor pre-operative immune status and outcome from cardiac surgery is specific to the peri-operative antigenic threat S. Moravcova, J. Mansell, A. Rogers, R. A. Smith, C. Hamilton-Davies P137 - Impact of simple clinical practice guidelines for reducing post-operative atrial fibrillation after cardiac surgery. A. Omar, M. Allam, O. Bilala, A. Kindawi, H. Ewila P138 - Dexamethasone administration during cardiopulmonary bypass has no beneficial effects on elective postoperative cardiac surgery patients F. Ampatzidou, D. Moisidou, M. Nastou, E. Dalampini, A. Malamas, E. Vasilarou, G. Drossos P139 - Intra-aortic balloon counterpulsation in patients undergoing cardiac surgery (IABCS): preliminary results G. Ferreira, J. Caldas, J. Fukushima, E. A. Osawa, E. Arita, L. Camara, S. Zeferino, J. Jardim, F. Gaioto, L. Dallan, F. B. Jatene, R. Kalil Filho, .F Galas, L. A. Hajjar P140 - Effects of low-dose atrial natriuretic peptide infusion on cardiac surgery-associated acute kidney injury C. Mitaka, T. Ohnuma, T. Murayama, F. Kunimoto, M. Nagashima, T. Takei, M. Tomita P141 - Acute kidney injury influence on high sensitive troponin measurements after cardiac surgery A. Omar, K. Mahmoud, S. Hanoura, S. Sudarsanan, P. Sivadasan, H. Othamn, Y. Shouman, R. Singh, A. Al Khulaifi P142 - Complex evaluation of endothelial dysfunction markers for prognosis of outcomes in patients undergoing cardiac surgery I. Mandel, S. Mikheev, I. Suhodolo, V. Kiselev, Y. Svirko, Y. Podoksenov P143 - New-onset atrial fibrillation in intensive care: incidence, management and outcome S. A. Jenkins, R. Griffin P144 - One single spot measurement of the sublingual microcirculation during acute pulmonary hypertension in a pig model of shock M. S. Tovar Doncel, A. Lima, C. Aldecoa, C. Ince P145 - Assessment of levosimendan as a therapeutic option to recruit the microcirculation in cardiogenic shock – initial experience in cardiac ICU A. Taha, A. Shafie, M. Mostafa, N. Syed, H. Hon P146 - Terlipressin vs. norepinephrine in the Potential Multiorgan Donor(PMD) F. Righetti, E. Colombaroli, G. Castellano P147 - Echocardiography in the potential heart donor exposed to substitution hormonotherapy F. Righetti, E. Colombaroli P148 - Machine learning can reduce rate of monitor alarms M. Hravnak, L. C. Chen, A. D. Dubrawski, G. C. Clermont, M. R. Pinsky P149 - Peripherally inserted central catheters placed in the ICU S. Gonzalez, D. Macias, J. Acosta, P. Jimenez, A. Loza, A. Lesmes, F. Lucena, C. Leon P150 - Recordings of abnormal central venous pressure waveform morphology during an episode of pulmonary hypertension in a porcine shock model M. S. Tovar Doncel, C. Ince, C. Aldecoa, A. Lima P151 - Ultrasound guided central venous access technique among French intensivists M. Bastide, J. Richecoeur, E. Frenoy, C. Lemaire, B. Sauneuf, F. Tamion, S. Nseir, D. Du Cheyron, H. Dupont, J. Maizel P152 - Predictive ability of the Pv-aCO2 gap in patients with shock M. Shaban, R. Kolko, N. Salahuddin, M. Sharshir, M. AbuRageila, A. AlHussain P153 - Comparison of echocardiography and pulmonary artery catheter measurements of hemodynamic parameters in critical ill patients P. Mercado, J. Maizel, L. Kontar, D. Titeca, F. Brazier, A. Riviere, M. Joris, T. Soupison, B. De Cagny, M. Slama P154 - The volume clamp method for noninvasive cardiac output measurement in postoperative cardiothoracic surgery patients: a comparison with intermittent pulmonary artery thermodilution J. Wagner, A. Körner, M. Kubik, S. Kluge, D. Reuter, B. Saugel P155 - Hemodynamic monitoring in patients with septic shock (SS) – CPCCO (continuous pulse contour cardiac output) vs. TEE (transesophageal echocardiography) E. Colombaroli, F. Righetti, G. Castellano P156 - Cardiac output measurement with transthoracic echocardiography in critically ill patients: a pragmatic clinical study T. Tran, D. De Bels, A. Cudia, M. Strachinaru, P. Ghottignies, J. Devriendt, C. Pierrakos P157 - Left ventricular outflow tract velocity time integral correlates with stroke volume index in mechanically ventilated patients Ó. Martínez González, R. Blancas, J. Luján, D. Ballesteros, C. Martínez Díaz, A. Núñez, C. Martín Parra, B. López Matamala, M. Alonso Fernández, M. Chana P158 - Transpulmonary thermodilution (TPTD) derived from femoral vs. jugular central venous catheter: validation of a previously published correction formula and a proprietary correction formula for global end-diastolic volume index (GEDVI) W. Huber, M. Eckmann, F. Elkmann, A. Gruber, I. Klein, R. M. Schmid, T. Lahmer P160 - Dynamic arterial elastance calculated with lidcoplus monitor does not predict changes in arterial pressure after a fluid challenge in postsurgical patients D. Bastoni, H. Aya, L. Toscani, L. Pigozzi, A. Rhodes, M. Cecconi P159 - Venous return driving pressure and resistance in acute blood volume changes P. W. Moller, S. Sondergaard, S. M. Jakob, J. Takala, D. Berger P160 - Dynamic arterial elastance calculated with lidcoplus monitor does not predict changes in arterial pressure after a fluid challenge in postsurgical patients D. Bastoni, H. Aya, L. Toscani, L. Pigozzi, A. Rhodes, M. Cecconi P161 - Analysis of duration of post-operative goal-directed therapy protocol C. Ostrowska, H. Aya, A. Abbas, J. Mellinghoff, C. Ryan, D. Dawson, A. Rhodes, M. Cecconi P162 - Hemodynamic optimization – back to square one? M. Cronhjort, O. Wall, E. Nyberg, R. Zeng, C. Svensen, J. Mårtensson, E. Joelsson-Alm P163 - Effectiveness of fluid thoracic content measurement by bioimpedance guiding intravascular volume optimization in patients with septic shock M. Aguilar Arzapalo, L. Barradas, V. Lopez, M. Cetina P164 - A systematic review on the role of internal jugular vein ultrasound measurements in assessment of volume status in critical shock patients N. Parenti, C. Palazzi, L. A. Amidei, F. B. Borrelli, S. C. Campanale, F. T. Tagliazucchi, G. S. Sedoni, D. L. Lucchesi, E. C. Carella, A. L Luciani P165 - Importance of recognizing dehydration in medical Intensive Care Unit M. Mackovic, N. Maric, M. Bakula P166 - Effect of volume for a fluid challenge in septic patients H. Aya, A. Rhodes, R. M. Grounds, N. Fletcher, M. Cecconi P167 - Fluid bolus practices in a large Australian intensive care unit B. Avard, P. Zhang P168 - Liberal late fluid management is associated with longer ventilation duration and worst outcome in severe trauma patients: a retrospective cohort of 294 patients M. Mezidi, J. Charbit, M. Ould-Chikh, P. Deras, C. Maury, O. Martinez, X. Capdevila P169 - Association of fluids and outcomes in emergency department patients hospitalized with community-acquired pneumonia P. Hou, W. Z. Linde-Zwirble, I. D. Douglas, N. S. Shapiro P170 - Association of positive fluid balance with poor outcome in medicosurgical ICU patients A. Ben Souissi, I. Mezghani, Y. Ben Aicha, S. Kamoun, B. Laribi, B. Jeribi, A. Riahi, M. S. Mebazaa P171 - Impact of fluid balance to organ dysfunction in critically ill patients C. Pereira, R. Marinho, R. Antunes, A. Marinho P172 - Volume bolus in ICU patients: do we need to balance our crystalloids? M. Crivits, M. Raes, J. Decruyenaere, E. Hoste P173 - The use of 6 % HES solution do not reduce total fluid requirement in the therapy of patients with burn shock V. Bagin, V. Rudnov, A. Savitsky, M. Astafyeva, I. Korobko, V. Vein P174 - Electron microscopic assessment of acute kidney injury in septic sheep resuscitated with crystalloids or different colloids T. Kampmeier , P. Arnemann, M. Hessler, A. Wald, K. Bockbreder, A. Morelli, H. Van Aken, S. Rehberg, C. Ertmer P175 - Alterations of conjunctival microcirculation in a sheep model of haemorrhagic shock and resuscitation with 0.9 % saline or balanced tetrastarch P. Arnemann, M. Hessler, T. Kampmeier, S. Rehberg, H. Van Aken, C. Ince, C. Ertmer P176 - A single centre nested pilot study investigating the effect of using 0.9 % saline or Plasma-Lyte 148 ® as crystalloid fluid therapy on gastrointestinal feeding intolerance in mechanically ventilated patients receiving nasogastric enteral nutrition S. Reddy, M. Bailey, R. Beasley, R. Bellomo, D. Mackle, A. Psirides, P. Young P177 - A single centre nested pilot study investigating the effect on post-operative bleeding of using 0.9 % saline or Plasma-Lyte® 148 as crystalloid fluid therapy in adults in ICU after heart surgery S. Reddy, M. Bailey, R. Beasley, R. Bellomo, D. Mackle, P. Young P178 - Extreme hypernatremia and sepsis in a patient with Huntington’s dementia: a conundrum in fluid management H. Venkatesh, S. Ramachandran, A. Basu, H. Nair P179 - Diagnosis and management of severe hypernatraemia in the critical care setting S. Egan, J. Bates P180 - Correlation between arterial blood gas and electrolyte disturbances during hospitalization and outcome in critically ill patients S. Oliveira, N. R. Rangel Neto, F. Q. Reis P181 - Missing the “I” in MUDPILES – a rare cause of high anion gap metabolic acidosis (HAGMA) C. P. Lee, X. L. Lin, C. Choong , K. M. Eu, W. Y. Sim , K. S. Tee, J. Pau , J. Abisheganaden P182 - Plasma NGAL and urinary output: potential parameters for early initiation of renal replacement therapy K. Maas, H. De Geus P183 - Renal replacement therapy for critically ill patients: an intermittent continuity E. Lafuente, R. Marinho, J. Moura, R. Antunes, A. Marinho P184 - A survey of practices related to renal replacement therapy in critically ill patients in the north of England. T. E. Doris, D. Monkhouse, T. Shipley, S. Kardasz, I Gonzalez P185 - High initiation creatinine associated with lower 28-day mortality in critically ill patients necessitating continuous renal replacement therapy S. Stads, A. J. Groeneveld P186 - The impact of Karnofsky performance scale on outcomes in acute kidney injury patients receiving renal replacement therapy on the intensive care unit I. Elsayed, N. Ward, A. Tridente, A. Raithatha P187 - Severe hypophosphatemia during citrate-anticoagulated CRRT A. Steuber, C. Pelletier, S. Schroeder, E. Michael, T. Slowinski, D. Kindgen-Milles P188 - Citrate regional anticoagulation for post dilution continuous renal replacement therapy S. Ghabina P189 - Citrate 18 mmol/l improves anticoagulation during RRT with adsorbing filters F. Turani, A. Belli, S. Busatti, G. Barettin, F. Candidi, F. Gargano, R. Barchetta, M. Falco P190 - Calcium gluconate instead of calcium chloride in citrate-anticoagulated CVVHD O. Demirkiran, M. Kosuk, S. Bozbay P191 - Enhanced clearance of interleukin-6 with continuous veno-venous haemodialysis (CVVHD) using Ultraflux EMiC2 vs. Ultraflux AV1000S V. Weber, J. Hartmann, S. Harm, I. Linsberger, T. Eichhorn, G. Valicek, G. Miestinger, C. Hoermann P192 - Removal of bilirubin with a new adsorbent system: in vitro kinetics S. Faenza, D. Ricci, E. Mancini, C. Gemelli, A. Cuoghi, S. Magnani, M. Atti P193 - Case series of patients with severe sepsis and septic shock treated with a new extracorporeal sorbent T. Laddomada, A. Doronzio, B. Balicco P194 - In vitro adsorption of a broad spectrum of inflammatory mediators with CytoSorb® hemoadsorbent polymer beads M. C. Gruda, P. O’Sullivan, V. P. Dan, T. Guliashvili, A. Scheirer, T. D. Golobish, V. J. Capponi, P. P. Chan P195 - Observations in early vs. late use of cytosorb therapy in critically ill patients K. Kogelmann, M. Drüner, D. Jarczak P196 - Oxiris membrane decreases endotoxin during rrt in septic patients with basal EAA > 0,6 F. Turani, A. B. Belli, S. M. Martni, V. C. Cotticelli, F. Mounajergi, R. Barchetta P197 - An observational prospective study on the onset of augmented renal clearance: the first report S. Morimoto, H. Ishikura P198 - An ultrasound- guided algorithm for the management of oliguria in severe sepsis I. Hussain, N. Salahuddin, A. Nadeem, K. Ghorab, K. Maghrabi P199 - Ultrasound in acute kidney injury (aki). First findings of farius, an education-programme in structural ultrasonography S. K. Kloesel, C. Goldfuss, A. Stieglitz, A. S. Stieglitz, L. Krstevska, G. Albuszies P200 - Effectiveness of renal angina index score predicting acute kidney injury on critically ill patients M. Aguilar Arzapalo, L. Barradas, V. Lopez, A. Escalante, G. Jimmy, M. Cetina P201 - Time length below blood pressure thresholds and progression of acute kidney injury in critically ill patients with or without sepsis: a retrospective, exploratory cohort study J. Izawa, T. Iwami, S. Uchino, M. Takinami, T. Kitamura, T. Kawamura P202 - Anaemia does not affect renal recovery in acute kidney injury J. G. Powell-Tuck, S. Crichton, M. Raimundo, L. Camporota, D. Wyncoll, M. Ostermann P203 - Estimated glomerular filtration rate based on serum creatinine: actual practice in Dutch ICU’s A. Hana, H. R. De Geus P204 - Comparison of estimated glomerular filtration rate calculated by mdrd, ckd-epi-serum-creatinine and ckd-epi-cystatin-c in adult critically ill patients H. R. De Geus, A. Hana P205 - Early diagnosis of septic acute kidney injury in medical critical care patients with a urine cell cycle arrest marker: insulin like growth factor binding protein-7 (IGFBP-7) M. Aydogdu, N. Boyaci, S. Yuksel, G. Gursel, A. B. Cayci Sivri P206 - Urinary neutrophil gelatinase-associated lipocalin as early biomarker of severe acute kidney injury in intensive care J. Meza-Márquez, J. Nava-López, R. Carrillo-Esper P207 - Shrunken pore syndrome is associated with a sharp rise in mortality in patients undergoing elective coronary artery bypass grafting A. Dardashti, A. Grubb P208 - The biomarker nephrocheck™ can discriminate the septic shock patients with an akin 1 or 2 acute renal failure who will not progress toward the akin 3 level J. Maizel, M. Wetzstein, D. Titeca, L. Kontar, F. Brazier, B. De Cagny, A. Riviere, T. Soupison, M. Joris, M. Slama P209 - A worldwide multicentre evaluation of acute kidney injury in septic and non-septic critically ill patients: the intensive care over nations (icon) audit E. Peters, H. Njimi, P. Pickkers, J. L. Vincent P210 - Does enhanced recovery after surgery reduce the incidence of acute kidney injury in those undergoing major gynae-oncological surgery? M. Waraich , J. Doyle, T. Samuels, L. Forni P211 - Identification of risk factors for the development of acute kidney injury after lower limb arthroplasty N. Desai, R. Baumber, P. Gunning, A. Sell P212 - Incidences and associations of acute kidney injury after major trauma S. Lin, H. Torrence, M. O’Dwyer, C. Kirwan, J. Prowle P213 - Acute kidney injury of major trauma patients T Kim P214 - Trajectory of serum creatinine after major surgery and the diagnosis of acute kidney injury M. E. O’Connor, R. W. Hewson, C. J. Kirwan, R. M. Pearse, J. Prowle P215 - Epidemiology of acute kidney injury after cardiac surgery. A single center retrospective study S. Hanoura , A. Omar, H. Othamn, S. Sudarsanan , M. Allam, M. Maksoud, R. Singh, A. Al Khulaifi P216 - Post-operative acute kidney injury after major non-cardiac surgery and its association with death in the following year M. E. O’Connor, R. W. Hewson, C. J. Kirwan, R. M. Pearse, J. Prowle P217 - Factors affecting acute renal failure in intensive care unit and effect of these factors on mortality O. Uzundere, D. Memis , M. Ýnal, A. , N. Turan P218 - Results of the live kidney transplantations according to national data of turkish organ and tissue information system M. A. Aydin, H. Basar, I. Sencan, A. Kapuagasi, M. Ozturk, Z. Uzundurukan, D. Gokmen, A. Ozcan, C. Kaymak P219 - Anaesthesia procedure and intensive therapy in patients with neck phlegmon V. A. Artemenko, A. Budnyuk P220 - Nasal high flow oygen for acute respiratory failure: a systematic review R. Pugh , S. Bhandari P221 - Setting optimal flow rate during high flow nasal cannula support: preliminary results T. Mauri, C. Turrini, T. Langer, P. Taccone, C. A. Volta, C. Marenghi, L. Gattinoni, A. Pesenti P222 - Dose to dose consistency across two different gas flow rates using cystic fibrosis and normal adult breathing profiles during nasal high flow oxygen therapy L. Sweeney, A . O’ Sullivan, P. Kelly, E. Mukeria, R. MacLoughlin P223 - Final results of an evaluation of airway medix closed suction system compared to a standard closed suction system M. Pfeffer, J. T. Thomas, G. B. Bregman, G. K. Karp, E. K. Kishinevsky, D. S. Stavi, N. A. Adi P224 - Different cuff materials and different leak tests - one size does not fit all T. Poropat, R. Knafelj P225 - Observational study on the value of the cuff-leak test and the onset of upper airway obstruction after extubation E. Llopart, M. Batlle, C. De Haro, J. Mesquida, A. Artigas P226 - A device for emergency transtracheal lung ventilation D. Pavlovic, L. Lewerentz, A. Spassov, R. Schneider P227 - Long-term outcome and health-related quality of life in patients discharged from the intensive care unit with a tracheostomy and with or without prolonged mechanical ventilation S. De Smet, S. De Raedt, E. Derom, P Depuydt, S. Oeyen, D. Benoit, J. Decruyenaere P228 - Ultrasound-guided percutaneous dilational tracheostomy versus bronchoscopy-guided percutaneous dilational tracheostomy in critically ill patients (trachus): a randomized clinical trial A. Gobatto, B. Bese, P. Tierno, L. Melro, P. Mendes, F. Cadamuro, M. Park, L. M. Malbouisson P229 - Is it safe to discharge patients with tracheostomy from the ICU to the ward? B. C. Civanto, J. L. Lopez, A. Robles, J. Figueira, S. Yus, A. Garcia P230 - The application of tracheostomy in children in ICU A. Oglinda, G. Ciobanu, C. Oglinda, L. Schirca, T. Sertinean, V. Lupu P231 - The impact of passive humidifiers on aerosol drug delivery during mechanical ventilation P. Kelly, A. O’Sullivan, L. Sweeney, R. MacLoughlin P232 - Evaluation of vibrating mesh and jet nebuliser performance at two different attachment setups in line with a humidifier nebuliser system A. O’Sullivan, P. Kelly, L. Sweeney, E. Mukeria, M. Wolny , R. MacLoughlin P233 - Psv-niv versus cpap in the treatment of acute cardiogenic pulmonary edema A. Pagano, F. Numis, G. Vison, L. Saldamarco, T. Russo, G. Porta, F. Paladino P234 - Noninvasive ventilation in patients with haematologic malignancy: a retrospective review C. Bell, J. Liu, J. Debacker, C. Lee, E. Tamberg, V. Campbell, S. Mehta P235 - Use of non-invasive ventilation in infectious diseases besides classical indications A. Silva-Pinto, A. Sarmento, L. Santos P236 - The impact of fragility on noninvasive mechanical ventilation application and results in the ICU Ý. Kara, F. Yýldýrým, A. Zerman, Z. Güllü, N. Boyacý, B. Basarýk Aydogan, Ü. Gaygýsýz, K. Gönderen, G. Arýk, M. Turkoglu, M. Aydogdu, G. Aygencel, Z. Ülger, G. Gursel P237 - Effects of metabolic alkalosis on noninvasive ventilation success and ICU outcome in patients with hypercapnic respiratory failure N. Boyacý, Z. Isýkdogan, Ö. Özdedeoglu, Z. Güllü, M. Badoglu, U. Gaygýsýz, M. Aydogdu, G. Gursel P238 - Asynchrony index and breathing patterns of acute exacerbation copd patients assisted with noninvasive pressure support ventilation and neurally adjusted ventilatory assist N. Kongpolprom, C. Sittipunt P239 - High frequency jet ventilation for severe acute hypoxemia A. Eden, Y. Kokhanovsky, S. Bursztein – De Myttenaere, R. Pizov P240 - HFOV revisited: a 7 year retrospective analysis of patients receiving HFOV who met oscillate trial entry criteria L. Neilans, N. MacIntyre P241 - Implementation of a goal-directed mechanical ventilation order set driven by respiratory therapists can improve compliance with best practices for mechanical ventilation M. Radosevich, B. Wanta, V. Weber, T. Meyer, N. Smischney, D. Brown, D. Diedrich P242 - A reduction in tidal volumes for ventilated patients on ICU calculated from IBW. can it minimise mortality in comparison to traditional strategies? A . Fuller, P. McLindon, K. Sim P243 - Predictive value of lung aeration scoring using lung ultrasound in weaning failure M. Shoaeir, K. Noeam, A. Mahrous, R. Matsa, A. Ali P244 - Conventional versus automated weaning from mechanical ventilation using SmartCare™ C. Dridi, S. Koubaji, S. Kamoun, F. Haddad, A. Ben Souissi, B. Laribi, A. Riahi, M. S. Mebazaa P245 - Ultrasonographic evaluation protocol for weaning from mechanichal ventilation A. Pérez-Calatayud, R. Carrillo-Esper, A. Zepeda-Mendoza, M. Diaz-Carrillo, E. Arch-Tirado P246 - Diaphragm ultrasonography: a method for weaning patients from mechanical ventilation S. Carbognin, L. Pelacani, F. Zannoni, A. Agnoli, G. Gagliardi P247 - Dorsal diaphragmatic excursion tracks transpulmonary pressure in ventilated ARDS patients: a potential non-invasive indicator of lung recruitment? R. Cho, A. Adams , S. Lunos, S. Ambur, R. Shapiro, M. Prekker P248 - Pulse oximetry in the icu patient: is the perfusion index of any value? M. Thijssen, L. Janssen, N. Foudraine P249 - Ventilation is a better assessment of respiratory status than EtCO2 C. J. Voscopoulos, J. Freeman P250 - Evaluation of the relationship between non-invasive minute ventilation and end-tidal CO2 in patients undergoing general vs spinal anesthesia C. J. Voscopoulos, J. Freeman, E. George P251 - Respiratory volume monitoring provides early warning of respiratory depression and can be used to reduce false alarms in non-intubated patients C. J. Voscopoulos, D. Eversole, J. Freeman, E. George P252 - P/i index: a predictive edi-derived weaning index during nava S. Muttini, R. Bigi, G. Villani, N. Patroniti P253 - Adequacy of ventilation in patients receiving opioids in the post anesthesia care unit: minute ventilation versus respiratory rate G. Williams, C. J. Voscopoulos, J. Freeman, E. George P254 - Comparison of regional and global expiratory time constants measured by electrical impedance tomography (EIT) A. Waldmann, S. Böhm, W. Windisch, S. Strassmann, C. Karagiannidis P255 - Electrical impedance tomography: robustness of a new pixel wise regional expiratory time constant calculation A. Waldmann, S. Böhm, W. Windisch, S. Strassmann, C. Karagiannidis P256 - Validation of regional and global expiratory time constant measurement by electrical impedance tomography in ards and obstructive pulmonary diseases C. K. Karagiannidis, A. W. Waldmann, S. B. Böhm, S. Strassmann, W. W. Windisch P257 - Transpulmonary pressure in a model with elastic recoiling lung and expanding chest wall P. Persson, S. Lundin, O. Stenqvist P258 - Lactate in pleural and abdominal effusion G. Porta, F. Numis, C. S. Serra, A. P. Pagano, M. M. Masarone, L. R. Rinaldi, A. A. Amelia, M. F. Fascione, L. A. Adinolfi, E. R. Ruggiero P259 - Outcome of patients admitted to the intensive care with pulmonary fibrosis F. Asota, K. O’Rourke, S. Ranjan, P. Morgan P260 - Sedation and analgesia practice in extra-corporeal membrane oxygenation (ECMO)-treated patients with acute respiratory distress syndrome (ARDS): a retrospective study J. W. DeBacker, E. Tamberg, L. O’Neill, L. Munshi, L. Burry, E. Fan, S. Mehta P261 - Characteristics and outcomes of patients deemed not eligible when referred for veno-venous extracorporeal membrane oxygenation (vv-ECMO) S. Poo, K. Mahendran, J. Fowles, C. Gerrard, A. Vuylsteke P262 - The SAVE SMR for veno-arterial ECMO R. Loveridge, C. Chaddock, S. Patel, V. Kakar, C. Willars, T. Hurst, C. Park, T. Best, A. Vercueil, G. Auzinger P263 - A simplified score to predict early (48 h) mortality in patients being considered for VA-ECMO A. Borgman, A. G. Proudfoot, E. Grins, K. E. Emiley, J. Schuitema, S. J. Fitch, G. Marco, J. Sturgill, M. G. Dickinson, M. Strueber, A. Khaghani, P. Wilton, S. M. Jovinge P264 - Lung function six months post extra corporeal membrane oxygenation (ECMO) for severe acute respiratory failure in adult survivors C. Sampson, S. Harris-Fox P265 - Bicarbonate dialysis removes carbon dioxide in hypoventilated rodents. M. E. Cove, L. H. Vu, A. Sen, W. J. Federspiel, J. A. Kellum P266 - Procalcitonin as predictor of primary graft dysfunction and mortality in post-lung transplantation C. Mazo Torre, J. Riera, S. Ramirez, B. Borgatta, L. Lagunes, J. Rello P267 - New molecular biomarkers of acute respiratory distress syndrome in abdominal sepsis A. K. Kuzovlev, V. Moroz, A. Goloubev, S. Polovnikov, S. Nenchuk P268 - Tight junction’s proteins claudin -5 and regulation by tnf in experimental murine lung injury model of ali/ards V. Karavana, C. Glynos, A. Asimakos, K. Pappas, C. Vrettou, M. Magkou, E. Ischaki, G. Stathopoulos, S. Zakynthinos P269 - Cell counts in endobronchial aspirate to assess airway inflammation in ARDS patients: a pilot study S. Spadaro, I. Kozhevnikova, F. Dalla Corte, S. Grasso, P. Casolari, G. Caramori, C. Volta P270 - Epidemiological and clinical profile of patients with acute respiratory distress syndrome in the surgical intensive care unit surgical, hospital JRA, Antananarivo T. Andrianjafiarinoa, T. Randriamandrato, T. Rajaonera P271 - Effect of high PEEP after recruitment maneuver on right ventricular function in ARDS. Is it good for the lung and for the heart? S. El-Dash, ELV Costa, MR Tucci, F Leleu, L Kontar, B. De Cagny, F. Brazier, D. Titeca, G. Bacari-Risal, J. Maizel, M. Amato, M. Slama P272 - Effect of recruitment maneuver on left ventricular systolic strain P. Mercado, J. Maizel, L. Kontar, D. Titeca, F. Brazier, A. Riviere, M. Joris, T. Soupison, B. De Cagny, S. El Dash, M. Slama P273 - Inhaled nitric oxide – is switching supplier cost effective? Remmington, A. Fischer, S. Squire, M. Boichat P274 - Epidemiological study of severe acute pancreatitis in Japan, comparison of the etiology and the patient outcomes on 1159 patients. H. Honzawa, H. Yasuda, T. Adati, S. Suzaki, M. Horibe, M. Sasaki, M. Sanui P275 - Extracorporeal liver support therapy. Experience in an intensive care unit R. Marinho, J. Daniel, H. Miranda, A. Marinho P276 - Accuracy of mortality prediction models in acute versus acute-on-chronic liver failure in the intensive care setting K. Milinis, M. Cooper, G. R. Williams, E. McCarron, S. Simants, I. Patanwala, I. Welters P277 - Risk of coronary artery disease in patients with chronic liver disease: a population based cohort study Y. Su P278 - 20 years of liver transplantation in Santiago de Compostela (Spain). Experience review J. Fernández Villanueva, R. Fernández Garda, A. López Lago, E. Rodríguez Ruíz, R. Hernández Vaquero, S. Tomé Martínez de Rituerto, E. Varo Pérez P279 - Diarrhea is a risk factor for liver injury and may lead to intestinal failure associated liver disease in critical illness N. Lefel, F. Schaap, D. Bergmans, S. Olde Damink, M. Van de Poll P280 - Bowel care on the intensive care unit: constipation guideline compliance and complications K. Tizard, C. Lister, L. Poole P281 - Malnutrition assessed by phase angle determines outcomes in low risk cardiac surgery patients D. Ringaitiene, D. Gineityte, V. Vicka, I. Norkiene, J. Sipylaite P282 - Preoperative fasting times in an irish hospital A. O’Loughlin, V. Maraj, J. Dowling P283 - Costs and final outcome of early x delayed feeding in a private Brazil ICU M. B. Velasco, D. M. Dalcomune, E. B. Dias, S. L. Fernandes P284 - Can ventilator derived energy expenditure measurements replace indirect calorimetry? T. Oshima, S. Graf, C. Heidegger, L. Genton, V. Karsegard, Y. Dupertuis, C. Pichard P285 - Revisiting the refeeding syndrome: results of a systematic review N. Friedli, Z. Stanga, B. Mueller, P. Schuetz P286 - Compliance with the new protocol for parenteral nutrition in our ICU L. Vandersteen, B. Stessel, S. Evers, A. Van Assche, L. Jamaer, J. Dubois P287 - Nutrition may be another treatment in the intensive care unit where less is more? R. Marinho, H. Castro, J. Moura, J. Valente, P. Martins, P. Casteloes, C. Magalhaes, S. Cabral, M. Santos, B. Oliveira, A. Salgueiro, A. Marinho P288 - Should we provide more protein to critically ill patients? R. Marinho, M. Santos, E. Lafuente, H. Castro, S. Cabral, J. Moura, P. Martins, B. Oliveira, A. Salgueiro, S. Duarte, S. Castro, M. Melo, P. Casteloes, A. Marinho P289 Protein provision in an adult intensive care unit S. Gray P290 - Prevalence and clinical outcomes of vitamin d deficiency in the medical critically ill patients in Songklanagarind hospital K. Maipang, R. Bhurayanontachai P291 - Vitamin d deficiency strongly predicts adverse medical outcome across different medical inpatient populations: results from a prospective study L. G. Grädel, P. Schütz P292 - Omega-3 fatty acids in patients undergoing cardiac surgery: a systematic review and meta-analysis P. Langlois, W. Manzanares P293 - Can 5-hydroxytriptophan prevent post-traumatic stress disorder in critically ill patients? R. Tincu, C. Cobilinschi, D. Tomescu, Z. Ghiorghiu, R. Macovei P294 - Parenteral selenium in the critically ill: an updated systematic review and meta-analysis W. Manzanares, P. Langlois, M. Lemieux, G. Elke, F. Bloos, K. Reinhart, D. Heyland P295 - Probiotics in the critically ill: an updated systematic review and meta-analysis P. Langlois, M. Lemieux, I. Aramendi, D. Heyland, W. Manzanares P296 - Diabetes with hyperglycemic crisis episodes may be associated with higher risk of pancreatic cancer: a population-based cohort study Y. Su P297 - Incidence of hypoglycemia in an intensive care unit depending on insulin protocol R. Marinho, N. Babo, A. Marinho P298 - Severity of the diseases is two-dimensionally correlated to blood glucose, including blood glucose variability, especially in moderately to severely ill patients with glucose intolerance. M. Hoshino, Y. Haraguchi, S. Kajiwara, T. Mitsuhashi, T. Tsubata, M. Aida P299 - A study of glycemic control by subcutaneous glargine injection transition from continuous regular insulin infusion in critically ill patients T. Rattanapraphat, R. Bhurayanontachai, C. Kongkamol, B. Khwannimit P300 - Glycemic control in Portuguese intensive care unit R. Marinho, M. Santos, H. Castro, E. Lafuente, A. Salgueiro, S. Cabral, P. Martins, J. Moura, B. Oliveira, M. Melo, B. Xavier, J. Valente, C. Magalhaes, P. Casteloes, A. Marinho P301 - Impact of hyperglycemia duration on the day of operation on short-term outcome of cardiac surgery patients D. Moisidou, F. Ampatzidou, C. Koutsogiannidis, M. Moschopoulou, G. Drossos P302 - Lactate levels in diabetic ketoacidosis patients at ICU admissions G. Taskin, M. Çakir, AK Güler, A. Taskin, N. Öcal, S. Özer, L. Yamanel P303 - Intensive care implications of merging heart attack centre units in London J. M. Wong, C. Fitton, S. Anwar, S. Stacey P304 - Special characteristics of in-hospital cardiac arrests M. Aggou, B. Fyntanidou, S. Patsatzakis, E. Oloktsidou, K. Lolakos, E. Papapostolou, V. Grosomanidis P305 - Clinical evaluation of ICU-admitted patients who were resuscitated in the general medicine ward S. Suda , T. Ikeda, S. Ono, T. Ueno, Y. Izutani P306 - Serious game evaluation of a one-hour training basic life support session for secondary school students: new tools for future bystanders S. Gaudry, V. Desailly, P. Pasquier, PB Brun, AT Tesnieres, JD Ricard, D. Dreyfuss, A. Mignon P307 - Public and clinical staff perceptions and knowledge of CPR compared to local and national data J. C White, A. Molokhia, A. Dean, A. Stilwell, G. Friedlaender P308 Dispatcher-assisted telephone cardiopulmonary resuscitation using a French-language compression-ventilation pediatric protocol M. Peters, S. Stipulante, A. Delfosse, AF Donneau, A. Ghuysen P309 Dantrolene versus amiodarone for resuscitation – an experimental study C. Feldmann, D. Freitag, W. Dersch, M. Irqsusi, D. Eschbach, T. Steinfeldt, H. Wulf, T. Wiesmann P310 Long term survival and functional neurological outcome in comatose survivors undergoing therapeutic hypothermia N. Kongpolprom, J. Cholkraisuwat P311 Impact of kidney disease on mortality and neurological outcome in out-of-hospital cardiac arrest: a prospective observational study S. Beitland , E. Nakstad, H. Stær-Jensen , T. Drægni , G. Andersen , D. Jacobsen , C. Brunborg, B. Waldum-Grevbo , K. Sunde P312 ICU dependency of patients admitted after primary percutaneous coronary intervention (PPCI) following out of the hospital cardiac arrest K. Hoyland, D. Pandit P313 Prognostic indicators and outcome prediction model for patients with return of spontaneous circulation from cardiopulmonary arrest: comprehensive registry of in-hospital intensive care on OHCA survival (critical) study in Osaka, Japan K. Hayakawa P314 Cerebral oxygen saturation during resuscitation in a porcine model of cardiac arrest E. Oloktsidou, K. Kotzampassi, B. Fyntanidou, S. Patsatzakis, L. Loukipoudi, E. Doumaki, V. Grosomanidis P315 Presumption of cardiopulmonary resuscitation for sustaining cerebral oxidation using regional cerebral saturation of oxygen: observational cohort study (press study) H. Yasuda P316 EEG reactivity in patients after cardiac arrest: a close look at stimuli MM Admiraal, M. Van Assen, MJ Van Putten, M. Tjepkema-Cloostermans, AF Van Rootselaar, J. Horn P317 Prognostic value of neuron-specific enolase after cardiac arrest F. Ragusa, A. Marudi , S. Baroni, A. Gaspari, E. Bertellini P318 Correlation between electroencephalographic findings and serum neuron specific enolase with outcome of post cardiac arrest patients A. Taha, T. Abdullah, S. Abdel Monem P319 Introduction of a targeted temperature management strategy following cardiac arrest in a district general hospital intensive care unit. S. Alcorn, S. McNeill, S. Russell P320 The evolution of cerebral oxygen saturation in post-cardiac arrest patients treated with therapeutic hypothermia W. Eertmans, C. Genbrugge, I. Meex, J. Dens, F. Jans, C. De Deyne P321 Prognostic factors and neurological outcomes of therapeutic hypothermia in comatose survivors from cardiac arrest: 8-year single center experience J. Cholkraisuwat, N. Kongpolprom P322 Adherence to targeted temperature management after out of hospital cardiac arrest B. Avard, R. Burns P323 Implementation of a therapeutic hypothermia protocol for comatose survivors of out-of-hospital cardiac arrest. A. Patarchi, T. Spina P324 Factors associated with ventilator weaning after targeted temperature management for cardiac arrest patients in japan H. Tanaka, N. Otani, S. Ode, S. Ishimatsu P325 Differential activation of c-fos in paraventricular nuclei of the hypothalamus and thalamus of the rat following myocardial infarction J. Cho, J. B. Moon, C. W. Park, T. G. Ohk, M. C. Shin, M. H. Won P326 Monitoring of cTroponin I in patients with acute ischemic stroke - predictor of inhospital mortality S. Dakova, Z. Ramsheva, K. Ramshev P327 Hyperthermic preconditioning severely accelerates neuronal damage in the gerbil ischemic hippocampal dentate gyrus via decreasing sods expressions J. Cho, J. B. Moon, C. W. Park, T. G. Ohk, M. C. Shin P328 Failure in neuroprotection of remote limb ischemic post conditioning in the hippocampus of a gerbil model of transient cerebral ischemia J. Cho, J. B. Moon, C. W. Park, T. G. Ohk, M. C. Shin P329 Brain death and admission diagnosis in neurologic intensive care unit, a correlation? A Marudi, S Baroni, A Gaspari, E Bertellini P330 Brain magnetic resonance imaging findings in patients with septic shock G. Orhun, E. Senturk, P. E. Ozcan, S. Sencer, C. Ulusoy, E. Tuzun, F . Esen P331 Benefits of L-carnitine in valproic acid induced encephalopathy R. Tincu, C. Cobilinschi, D. Tomescu, Z. Ghiorghiu, R. Macovei P332Automatic analysis of EEG reactivity in comatose patients M. Van Assen, M. M. Admiraal, M. J. Van Putten, M. Tjepkema-Cloostermans, A. F. Van Rootselaar, J. Horn P333 Usefulness of common ICU severity scoring systems in predicting outcome after spontaneous intracerebral hemorrhage M. Fallenius, M. B. Skrifvars, M. Reinikainen, S. Bendel, R. Raj P334 Evalution of patients with suspected subarachnoid haemorrhage and negative ct imaging M. Abu-Habsa, C. Hymers, A. Borowska, H. Sivadhas, S. Sahiba, S. Perkins P335 Timing of endovascular and surgical treatment for aneurysmal subarachnoid haemorrhage: early but not so fast. J. Rubio, J. A. Rubio, R. Sierra P336 Red blood cell transfusion in aneurysmal subarachnoid hemorrhage – the Sahara cohort study S. English, M. Chasse, A. Turgeon, F. Lauzier, D. Griesdale, A. Garland, D. Fergusson, R. Zarychanski, A. Tinmouth, C. Van Walraven, K. Montroy, J. Ziegler, R. Dupont Chouinard, R. Carignan, A. Dhaliwal, C. Lum, J. Sinclair, G. Pagliarello, L. McIntyre P337 - Aneurysmal subarachnoid hemorrhage and anemia: a canadian multi-centre retrospective cohort study S. English, M. Chasse, A. Turgeon, F. Lauzier, D. Griesdale, A. Garland, D. Fergusson, R. Zarychanski, A. Tinmouth, C. Van Walraven, K. Montroy, J. Ziegler, R. Dupont Chouinard, R. Carignan, A. Dhaliwal, C. Lum, J. Sinclair, G. Pagliarello, L. McIntyre P338 - Does the neutrophil-to-lymphocyte (NLR) ratio predict symptomatic vasospasm or delayed cerebral ischemia (DCI) after aneurysmal subarachnoid haemorrhage (SAH)? T. Groza, N. Moreau, D. Castanares-Zapatero, P. Hantson P339 - ICU-acquired infections in aneurysmal subarachnoid hemorrhage patients: impact on ICU and hospital length of stay M. Carbonara , F. Ortolano, T. Zoerle, S. Magnoni, S. Pifferi, V. Conte, N. Stocchetti P340 - Cerebral metabolic effects of normobaric hyperoxia during the acute phase of aneurysmal subarachnoid hemorrhage L. Carteron, T. Suys, C. Patet, H. Quintard, M. Oddo P341 - Postoperative care for elective craniotomy: where is best done? J. A. Rubio, J. Rubio, R. Sierra P342 - 5-year follow-up of patients after transplantation of organs from donors from neurocritical care V. Spatenkova, E. Pokorna, P. Suchomel P343 - Evaluation of levetiracetam pharmacokinetics after severe traumatic brain injury in neurocritical care patients at a level one trauma center N. Ebert, J. Jancik, H. Rhodes P344 - Model based time series cluster analysis to determine unique patient states in traumatic brain injury T. Bylinski, C. Hawthorne, M. Shaw, I. Piper, J. Kinsella P345 - Brain compartment monitoring capabilities from ICP to BI (bioimpedance) during HS (hypertonic saline) administration. State of art simulation outcome depending on brain swelling type A. K. Kink , I. R. Rätsep P346 - Transfusion of red blood cells in patients with traumatic brain injury admitted to Canadian trauma health centers: a multicenter cohort study A. Boutin, L. Moore, M. Chasse, R. Zarychanski, F. Lauzier, S. English, L. McIntyre, J. Lacroix, D. Griesdale, P. Lessard-Bonaventure, A. F. Turgeon P347 - Hemoglobin thresholds and red blood cell transfusions in adult patients with moderate or severe traumatic brain injury: a retrospective cohort study A. Boutin, L. Moore, R. Green, P. Lessard-Bonaventure, M. Erdogan, M. Butler, F. Lauzier, M. Chasse, S. English, L. McIntyre, R. Zarychanski, J. Lacroix, D. Griesdale, P. Desjardins, D. A. Fergusson, A. F. Turgeon P348 - Characteristics of patients with gunshot wounds to the head - an observational Brazilian study B. Goncalves, B. Vidal, C. Valdez, A. C. Rodrigues, L. Miguez, G. Moralez P349 - Base excess as predictor for ICU admission and the injury severity in blunt trauma patients T. Hong P350 - Enhancement of usual emergency department care with proadrenomedullin to improve outcome prediction - Results from the multi-national, prospective, observational TRIAGE study A. Kutz, P. Hausfater, D. Amin, T. Struja, S. Haubitz, A. Huber, B. Mueller, P. Schuetz P351 - Developing an innovative emergency medicine point-of-care simulation programme T. Brown, J. Collinson, C. Pritchett, T. Slade P352 - The InSim program: an in situ simulation program for junior trainees in intensive care M. Le Guen, S. Hellings, R. Ramsaran P353 - Impact of excessive and inappropriate troponin testing in the emergency setting how good are we A. Alsheikhly P354 - The development of time tracking monitor at emergency department T. Abe P355 - Role of focussed echocardiography in emergency assessment of syncope L. Kanapeckaite, M. Abu-Habsa, R. Bahl P356 - Insertion of an open-ended 14-gauge catheter through the chest wall causes a significant pneumothorax in a self-ventilating swine model M. Q Russell, K. J. Real, M. Abu-Habsa , R. M. Lyon, N. P. Oveland P357 - Ez-io® intraosseous access teaching in the workplace using a mobile ‘tea trolley’ training method J. Penketh, M. Mcdonald, F. Kelly P358 - Black widow envenomation in Saudi Arabia: a prospective observational case series M. Alfafi, S. Alsolamy, W. Almutairi, B. Alotaibi P359 - Mechanical ventilation in patients with overdose not yet intubated on icu admission A. E. Van den Berg, Y. Schriel, L. Dawson, I. A. Meynaar P360 - Central nervous system depressants poisoning and ventilator associated pneumonia: an underrated risk factor in toxicological intensive care unit H. Talaie P361 - Acute barium intoxication treated with hemodiafiltration D. Silva, S. Fernandes, J. Gouveia, J. Santos Silva P362 - Major trauma presenting to the emergency department. the spectrum of cycling injuries in Ireland J. Foley, A. Kaskovagheorgescu, D. Evoy, J. Cronin, J. Ryan P363 - Burns from French military operations: a 14-year retrospective observational analysis. M. Huck, C. Hoffmann, J. Renner, P. Laitselart, N. Donat, A. Cirodde, J. V. Schaal, Y. Masson, A. Nau, T. Leclerc P364 - A comparison of mortality scores in burns patients on the intensive care unit. O. Howarth, K. Davenport, P. Jeanrenaud, S. Raftery P365 - Clasification of pain and its treatment and an intensive care rehabiliation clinic P. MacTavish, H. Devine, J. McPeake, M. Daniel, J. Kinsella, T. Quasim P366 - Pain management adequacy in critical care areas ,the process and the barriers perceived by critical care nurses S. Alrabiee, A. Alrashid , S. Alsolamy P367 - Pain assessment in critically ill adult patients: validation of the Turkish version of the critical-care pain observation tool O. Gundogan, C. Bor, E. Akýn Korhan, K. Demirag , M. Uyar P368 - An audit of pain and sedation assessments in the intensive care unit: recommendations for clinical practice F. Frame, C. Ashton, L. Bergstrom Niska P369 - Impact of pharmaceutical care on treatment of pain and agitation in medical intensive care unit P. Dilokpattanamongkol, T. Suansanae, C. Suthisisang, S. Morakul, C. Karnjanarachata, V. Tangsujaritvijit P370 - Agitation in trauma ICU, prevention and outcome S. Mahmood, H. Al Thani, A. Almenyar P371 Correlation between percentages of ventilated patients developed vap and use of sedative agents in icu patients. A. Vakalos , V. Avramidis P372 - Improving recording of sedation events in the Emergency Department: The implementation of the SIVA International Taskforce adverse event reporting tool for procedural sedation R. Sharvill, J. Penketh P373 - Impact of sedative drug use on the length of mechanical ventilation S. E. Morton, Y. S. Chiew, C. Pretty, J. G. Chase, G. M. Shaw P374 - Co-administration of nitric oxide and sevoflurane using anaconda R. Knafelj, P. Kordis P375 - A retrospective study of the use of Dexmedetomidine in an oncological critical care setting S. Patel, V. Grover P376 - Dexmedetomidine and posttraumatic stress disorder incidence in alcohol withdrawal icu patients I. Kuchyn, K. Bielka P377 - Hemodynamic effects of dexmedetomidine in a porcine model of septic shock Z. Aidoni, V. Grosomanidis, K. Kotzampassi, G. Stavrou, B. Fyntanidou, S. Patsatzakis, C. Skourtis P378 - Ketamine for analgosedation in severe hypoxic respiratory failure S. D. Lee, K. Williams, I. D. Weltes P379 - Madness from the moon? lunar cycle and the incidence of delirium on the intensive care unit S. Berhane, C. Arrowsmith, C. Peters, S. Robert P380 - Impaired dynamic cerebral autoregulation after coronary artery bypass grafting and association with postoperative delirium J. Caldas, R. B. Panerai, T. G. Robinson, L. Camara, G. Ferreira, E. Borg-Seng-Shu, M. De Lima Oliveira, N. C. Mian, L. Santos, R. Nogueira, S. P. Zeferino, M. Jacobsen Teixeira, F. Galas, L. A. Hajjar P381 - Risk factors predicting prolonged intensive care unit length of stay after major elective surgery. P. Killeen, M. McPhail, W. Bernal, J. Maggs, J. Wendon, T. Hughes P382 - Systemic inflammatory response syndrome criteria and hospital mortality prediction in a brazilian cohort of critically ill patients L. U. Taniguchi, E. M. Siqueira, J. M. Vieira Jr, L. C. Azevedo P383 - Evaluating the efficacy of a risk predictor panel in identifying patients at elevated risk of morbidity following emergency admission A. N. Ahmad, M. Abu-Habsa, R. Bahl, E. Helme, S. Hadfield, R. Loveridge P384 - A retrospective comparison of outcomes for elective surgical patients admitted post-operatively to the critical care unit or general ward J. Shak, C. Senver, R. Howard-Griffin P385 - Effect of obesity on mortality in surgical critically ill patients. P. Wacharasint, P. Fuengfoo, N. Sukcharoen, R. Rangsin P386 - The national early warning score (news) reliably improves adverse clinical outcome prediction in community-acquired pneumonia - results from a 6 year follow-up D. Sbiti-Rohr, P. Schuetz P387 - Clinical usefulness of the charlson¡¯s weighted index of comorbidities _as prognostic factor in patients with prolonged acute mechanical ventilation H. Na, S. Song, S. Lee, E. Jeong, K. Lee P388 - Comparison of mortality prediction scoring systems in patients with cirrhosis admitted to general intensive care unit M. Cooper, K. Milinis, G. Williams, E. McCarron, S. Simants, I. Patanwala, I. D. Welters P389 - Impact of admission source and time of admission on outcome of pediatric intensive care patients: retrospective 15 years study E. Zoumpelouli, EA Volakli, V. Chrysohoidou, S. Georgiou, K. Charisopoulou, E. Kotzapanagiotou, V. Panagiotidou, K. Manavidou, Z. Stathi, M. Sdougka P390 - Heart rate variability and outcomes prediction in critical illness N. Salahuddin, B. AlGhamdi, Q. Marashly, K. Zaza, M. Sharshir, M. Khurshid, Z. Ali, M. Malgapo, M. Jamil, A. Shafquat, M. Shoukri, M. Hijazi P391 - The incidence and outcome of hyperlactatemia in the post anaesthesia care unit T. Abe, S. Uchino, M. Takinami P392 - Correlation between arterial blood gas disturbances and arterial lactate levels during hospitalization and outcome in critically septic patients N. R. Rangel Neto, S. Oliveira, F. Q. Reis, F. A. Rocha P393 - External validation of saps 3 and mpm iii scores in 48,816 patients from 72 brazilian icus G. Moralez, K. Ebecken, L. S. Rabello, M. F. Lima, R. Hatum, F. V. De Marco, A. Alves, J. E. Pinto, M. Godoy, P. E. Brasil, F. A. Bozza, J. I. Salluh, M. Soares P394 - The frailty penalty: pre-admission functional status confounds mortality prediction models in critically ill patients J. Krinsley, G. Kang P395 - ‘sooner rather than later”: how delayed discharge from critical care leads to increased out of hours discharges and subsequent increase in in-hospital mortality. J. Perry, H. Hines P396 - Identifying poor outcome patient groups in a resource-constrained critical care unit K. M. Wilkinson, C. Tordoff, B. Sloan, M. C. Bellamy P397 - Effects of icu weekend admission and discharge on mortality. E. Moreira, F. Verga, M. Barbato, G. Burghi P398 - Organizational factors, outcomes and resource use in 9,946 cancer patients admitted to 70 ICUs M Soares, U. V. Silva, L. C. Azevedo, A. P. Torelly, J. M. Kahn, D. C. Angus, M. F. Knibel, P. E. Brasil, F. A. Bozza, J. I. Salluh P399 - Evaluation of oncological critically ill patients, severity score and outcome compared to not oncological in a particular hospital cti. M. B. Velasco, D. M. Dalcomune P400 - Outcomes of patients admitted to a large uk critical care department with palliative oncological diagnoses R. Marshall, T. Gilpin, A. Tridente, A. Raithatha P401 - Predictors of mortality in febrile neutropenic patients with haematological malignancies admitted to an intensive care unit of a cancer center D. Mota, B. Loureiro, J. Dias, O. Afonso, F. Coelho, A. Martins, F. Faria P402 - Patients with hematologic malignancies requiring invasive mechanical ventilation: characteristics and predictors of mortality H. Al-Dorzi, H. Al Orainni , F. AlEid, H. Tlaygeh, A. Itani, A. Hejazi, Y. Arabi P403 - Patient-important outcomes in randomized controlled trials in critically ill patients: a systematic review S. Gaudry, J. Messika, J. D. Ricard, S. Guillo, B. Pasquet, E. Dubief, D. Dreyfuss, F. Tubach P404 - Alopecia in survivors of critical illness: a qualitative study C . Battle, K. James, P. Temblett P405 - The impact of mental health on icu admission L. Davies, C. Battle, C. Lynch P406 - Cognitive impairment 5 years after ICU discharge S. Pereira, S. Cavaco, J. Fernandes, I. Moreira, E. Almeida, F. Seabra Pereira, M. Malheiro, F. Cardoso, I. Aragão, T. Cardoso P407 - Apache ii versus apache iv for octagenerians in medical icu M. Fister, R. Knafelj P408 - Outcomes of octagenarians in an indian icu P. Muraray Govind, N. Brahmananda Reddy, R. Pratheema, E. D. Arul, J. Devachandran P409 - Mortality and outcomes in elderly patients 80 years of age or older admitted to the icu M. B. Velasco , D. M. Dalcomune P410 - Octagenerians in medical icu - adding days to life or life to days? R. Knafelj, M. Fister P411 - The very elderly admitted to intensive care unit: outcomes and economic evaluation N. Chin-Yee, G. D’Egidio, K. Thavorn, D. Heyland, K. Kyeremanteng P412 - The very elderly in intensive care: relationship between acuity of illness and long-term mortality A. G. Murchison, K. Swalwell, J. Mandeville, D. Stott P413 - Acquired weakness in an oncological intensive care unit I. Guerreiro P414 - Musculoskeletal problems in intensive care unit (ICU) patients post-discharge H. Devine, P. MacTavish, J. McPeake, T. Quasim, J. Kinsella, M. Daniel P415 - Premorbid obesity, but not nutrition, prevents critical illness-induced muscle wasting and weakness C. Goossens M. B. Marques, S. Derde, S. Vander Perre, T. Dufour, S. E. Thiessen, F. Güiza, T. Janssens, G. Hermans, I. Vanhorebeek, K. De Bock, G. Van den Berghe, L. Langouche P416 - Physical outcome measures for critical care patients following intensive care unit (icu) discharge H. Devine, P. MacTavish, T. Quasim, J. Kinsella, M. Daniel, J. McPeake P417 - Improving active mobilisation in a general intensive care unit B. Miles , S. Madden, H. Devine P418 - Mobilization in patients on vasoactive drugs use – a pilot study. M. Weiler, P. Marques, C. Rodrigues, M. Boeira, K. Brenner, C. Leães, A. Machado, R. Townsend, J. Andrade P419 - Pharmacy intervention at an intensive care rehabilitation clinic P. MacTavish, J. McPeake, H. Devine, J. Kinsella, M. Daniel, R. Kishore, C. Fenlon, T. Quasim P420 - Interactive gaming is feasible and potentially increases icu patients’ motivation to be engaged in rehabilitation programs T. Fiks, A. Ruijter, M. Te Raa, P. Spronk P421 - Simulation-based design of a robust stopping rule to ensure patient safety Y. S. Chiew, P. Docherty, J. Dickson, E. Moltchanova, C. Scarrot, C. Pretty, G. M. Shaw, J. G. Chase P422 - Are daily blood tests on the intensive care unit necessary? T. Hall, W. C. Ngu, J. M. Jack, P. Morgan P423 - Measuring urine output in ward patients: is it helpful? B. Avard, A. Pavli, X. Gee P424 - The incidence of pressure ulcers in an adult mixed intensive care unit in turkey C . Bor, E. Akin Korhan, K. Demirag, M. Uyar P425 - Intensivist/patient ratios in closed ICUs in Alexandria, Egypt; an overview M. Shirazy, A. Fayed P426 - Eicu (electronic intensive care unit): impact on ALOS (average length of stay) in a developing country like India S. Gupta, A. Kaushal, S. Dewan, A. Varma P427 - Predicting deterioration in general ward using early deterioration indicator E. Ghosh, L. Yang, L. Eshelman, B. Lord, E. Carlson P428 - High impact enhanced critical care outreach - the imobile service: making a difference E. Helme, R. Broderick, S. Hadfield, R. Loveridge P429 - Impact of bed availability and cognitive load on intensive care unit (ICU) bed allocation: a vignette-based trial J. Ramos, D. Forte P430 - Characteristics of critically ill patients admitted through the emergency department F. Yang, P. Hou P431 - Admission to critical care: the quantification of functional reserve J. Dudziak, J. Feeney, K. Wilkinson, K. Bauchmuller, K. Shuker, M. Faulds, A. Raithatha, D. Bryden, L. England, N. Bolton, A. Tridente P432 - Admission to critical care: the importance of frailty K. Bauchmuller, K Shuker, A Tridente, M Faulds, A Matheson, J. Gaynor, D Bryden, S South Yorkshire Hospitals Research Collaboration P433 - Development of an instrument to aid triage decisions for intensive care unit admission J. Ramos, B. Peroni, R. Daglius-Dias, L. Miranda, C. Cohen, C. Carvalho, I . Velasco, D. Forte P434 - Using selective serotonin re-uptake inhibitors and serotonin-norepinephrine re-uptake inhibitors in critical care: a systematic review of the evidence for benefit or harm J. M. Kelly, A. Neill, G. Rubenfeld, N. Masson, A. Min P435 - Measuring adaptive coping of hospitalized patients with a severe medical condition:the sickness insight in coping questionnaire (sicq) E. Boezeman, J. Hofhuis , A. Hovingh, R. De Vries, P. Spronk P436 - Results of a national survey regarding intensive care medicine training G. Cabral-Campello, I. Aragão, T. Cardoso P437 - Work engagement among healthcare professionals in the intensive care unit M. Van Mol, M. Nijkamp, E . Kompanje P438 - Empowering the intensive care practitioners. is it a burnout ameliorating intervention? P. Ostrowski, A. Omar P439 - Icu patients suffer from circadian rhythm desynchronisation K. Kiss , B. Köves, V. Csernus, Z. Molnár P440 - Noise reduction in the ICU: feasible ? Y. Hoydonckx, S. Vanwing, B. Stessel, A. Van Assche, L. Jamaer, J. Dubois P441 - Accidental removal of invasive devices in the critical patient into the bed-washing. does the presence of professional nurse modify his incidence? V. Medo, R. Galvez, J. P. Miranda P442 - Deprivation of liberty safeguards (dols): audit of compliance in a of a 16-bed specialist cancer critical care unit. C. Stone, T. Wigmore P443 - Use of a modified cristal score to predict futility of critical care in the elderly Y. Arunan, A. Wheeler, K. Bauchmuller, D. Bryden P444 - Improvement of Referral Rate to Palliative Care for Patients with Poor Prognosis in Neurosurgical Intensive Care Unit Y. Wong, C. Poi, C. Gu P445 - Factors associated with limitation of life supporting care (lsc) in a medico-surgical intermediate care unit, and outcome of patients with lsc limitation: a monocentric, six-month study. P. Molmy, N. Van Grunderbeeck, O. Nigeon, M. Lemyze, D. Thevenin, J. Mallat P446 - Palliative care consultation and intensive care unit admission request: a cohort study J. Ramos, M. Correa, R. T. Carvalho, D. Forte P447 - Nursing and medicine together in postsurgical intensive care unit: situations of prognostic conflict at the end of life. our critical care nurses suffer with our medical activism? A. Fernandez, C. McBride P448 - End of life who may decide E. Koonthalloor, C. Walsh P449 - Correctly diagnosing death A. Webber, M. Ashe, K. Smith, P. Jeanrenaud P450 - Skin procurement performed by intensive care physicians: yes, we can. A. Marudi , S. Baroni, F. Ragusa, E. Bertellini P451 - Death analysis in pediatric intensive care patients E. A. Volakli , E. Chochliourou, M. Dimitriadou, A. Violaki, P. Mantzafleri, E. Samkinidou, O. Vrani, A. Arbouti, T. Varsami, M. Sdougka P452 - The potential impact of euthanasia on organ donation: analysis of data from belgium J. A. Bollen, T. C. Van Smaalen, W. C. De Jongh, M. M. Ten Hoopen, D. Ysebaert, L. W. Van Heurn, W. N. Van Mook P453 - Communication within an intensive care setting K. Sim, A. Fuller P454 - Development and implementation of a longitudinal communication curriculum for critical care medicine fellows A. Roze des Ordons, P. Couillard, C. Doig P455 - Staff-family conflict in a multi-ethnic intensive care unit R. V. Van Keer, R. D. Deschepper, A. F. Francke, L. H. Huyghens, J. B. Bilsen P456 - Does the source of admission to critical care affect family satisfaction? B. Nyamaizi, C. Dalrymple, A. Molokhia, A. Dobru P457 - A simple alternative to the family satisfaction survey (fs-icu) E. Marrinan, A. Ankuli, A. Molokhia P458 - A study to explore the experiences of patient and family volunteers in a critical care environment: a phenomenological analysis J. McPeake, R. Struthers, R. Crawford , H. Devine , P. Mactavish , T. Quasim P459 - Prevalence and risk factors of anxiety and depression in relatives of burn patients. P. Morelli, M. Degiovanangelo, F. Lemos, V. MArtinez, F. Verga, J. Cabrera, G. Burghi P460 - Guidance of visiting children at an adult intensive care unit (icu) A. Rutten , S. Van Ieperen, S. De Geer, M. Van Vugt, E. Der Kinderen P461 - Visiting policies in Italian pediatric ICUs: an update A. Giannini, G Miccinesi, T Marchesi, E Prandi
- Published
- 2016
7. An exploratory randomised double-blind and placebo-controlled phase 2 study of a combination of baclofen, naltrexone and sorbitol (PXT3003) in patients with Charcot-Marie-Tooth disease type 1A (vol 9, 199, 2014)
- Author
-
Attarian, S, Vallat, J-M, Magy, L, Funalot, B, Gonnaud, P-M, Lacour, A, Pereon, Y, Dubourg, O, Pouget, J, Micallef, J, Franques, J, Lefebvre, M-N, Ghorab, K, Al-Moussawi, M, Tiffreau, V, Preudhomme, M, Magot, A, Leclair-Visonneau, L, Stojkovic, T, Bossi, L, Lehert, P, Gilbert, W, Bertrand, V, Mandel, J, Milet, A, Hajj, R, Boudiaf, L, Scart-Gres, C, Nabirotchkin, S, Guedj, M, Chumakov, I, Cohen, D, Attarian, S, Vallat, J-M, Magy, L, Funalot, B, Gonnaud, P-M, Lacour, A, Pereon, Y, Dubourg, O, Pouget, J, Micallef, J, Franques, J, Lefebvre, M-N, Ghorab, K, Al-Moussawi, M, Tiffreau, V, Preudhomme, M, Magot, A, Leclair-Visonneau, L, Stojkovic, T, Bossi, L, Lehert, P, Gilbert, W, Bertrand, V, Mandel, J, Milet, A, Hajj, R, Boudiaf, L, Scart-Gres, C, Nabirotchkin, S, Guedj, M, Chumakov, I, and Cohen, D
- Published
- 2016
8. An exploratory randomised double-blind and placebo-controlled phase 2 study of a combination of baclofen, naltrexone and sorbitol (PXT3003) in patients with Charcot-Marie-Tooth disease type 1A
- Author
-
Attarian, S, Vallat, J-M, Magy, L, Funalot, B, Gonnaud, P-M, Lacour, A, Pereon, Y, Dubourg, O, Pouget, J, Micallef, J, Franques, J, Lefebvre, M-N, Ghorab, K, Al-Moussawi, M, Tiffreau, V, Preudhomme, M, Magot, A, Leclair-Visonneau, L, Stojkovic, T, Bossi, L, Lehert, P, Gilbert, W, Bertrand, V, Mandel, J, Milet, A, Hajj, R, Boudiaf, L, Scart-Gres, C, Nabirotchkin, S, Guedj, M, Chumakov, I, Cohen, D, Attarian, S, Vallat, J-M, Magy, L, Funalot, B, Gonnaud, P-M, Lacour, A, Pereon, Y, Dubourg, O, Pouget, J, Micallef, J, Franques, J, Lefebvre, M-N, Ghorab, K, Al-Moussawi, M, Tiffreau, V, Preudhomme, M, Magot, A, Leclair-Visonneau, L, Stojkovic, T, Bossi, L, Lehert, P, Gilbert, W, Bertrand, V, Mandel, J, Milet, A, Hajj, R, Boudiaf, L, Scart-Gres, C, Nabirotchkin, S, Guedj, M, Chumakov, I, and Cohen, D
- Abstract
BACKGROUND: Charcot-Marie-Tooth type 1A disease (CMT1A) is a rare orphan inherited neuropathy caused by an autosomal dominant duplication of a gene encoding for the structural myelin protein PMP22, which induces abnormal Schwann cell differentiation and dysmyelination, eventually leading to axonal suffering then loss and muscle wasting. We favour the idea that diseases can be more efficiently treated when targeting multiple disease-relevant pathways. In CMT1A patients, we therefore tested the potential of PXT3003, a low-dose combination of three already approved compounds (baclofen, naltrexone and sorbitol). Our study conceptually builds on preclinical experiments highlighting a pleiotropic mechanism of action that includes downregulation of PMP22. The primary objective was to assess safety and tolerability of PXT3003. The secondary objective aimed at an exploratory analysis of efficacy of PXT3003 in CMT1A, to be used for designing next clinical development stages (Phase 2b/3). METHODS: 80 adult patients with mild-to-moderate CMT1A received in double-blind for 1 year Placebo or one of the three increasing doses of PXT3003 tested, in four equal groups. Safety and tolerability were assessed with the incidence of related adverse events. Efficacy was assessed using the Charcot-Marie-Tooth Neuropathy Score (CMTNS) and the Overall Neuropathy Limitations Scale (ONLS) as main endpoints, as well as various clinical and electrophysiological outcomes. RESULTS: This trial confirmed the safety and tolerability of PXT3003. The highest dose (HD) showed consistent evidence of improvement beyond stabilization. CMTNS and ONLS, with a significant improvement of respectively of 8% (0.4% - 16.2%) and 12.1% (2% - 23.2%) in the HD group versus the pool of all other groups, appear to be the most sensitive clinical endpoints to treatment despite their quasi-stability over one year under Placebo. Patients who did not deteriorate over one year were significantly more frequent in the HD group.
- Published
- 2014
9. [Hereditary neuropathies]
- Author
-
Vallat, Jean-Michel, Calvo, J., Ghorab, K., Tazir, Meriem, Service de Neurologie [CHU Limoges], CHU Limoges, Biomolécules Thérapies anti-tumorales (EA4021), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Service de Neurologie, Hopital Mustapha, and Sturtz, Franck
- Subjects
[SDV.GEN]Life Sciences [q-bio]/Genetics ,[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology ,[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,[SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,[SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology ,[SDV.GEN] Life Sciences [q-bio]/Genetics ,[SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human genetics ,[SDV.BBM.BM] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology ,[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics ,[SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN] ,[SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology ,[SDV.BBM.GTP] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN] ,[SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology ,[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,[SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,ComputingMilieux_MISCELLANEOUS ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology - Abstract
International audience
- Published
- 2008
10. G.P.44
- Author
-
Eymard, B., primary, Ghorab, K., additional, Laforet, P., additional, Chevessier, F., additional, Vallat, J., additional, Hantai, D., additional, Romero, N., additional, Böhm, J., additional, and Laporte, J., additional
- Published
- 2014
- Full Text
- View/download PDF
11. Douleurs neuropathiques au cours du Sjögren primitif : penser aux petites fibres !
- Author
-
Fauchais, A.L., primary, Ghorab, K., additional, Gondran, G., additional, Richard, L., additional, Bézanahary, H., additional, Loustaud-Ratti, V., additional, Ly, K.H., additional, Vallat, J., additional, Vidal-Cathala, E., additional, and Magy, L., additional
- Published
- 2009
- Full Text
- View/download PDF
12. G.P.44: Clinical features of eight French patients with STIM 1 gene mutations
- Author
-
Eymard, B., Ghorab, K., Laforet, P., Chevessier, F., Vallat, J., Hantai, D., Romero, N., Böhm, J., and Laporte, J.
- Published
- 2014
- Full Text
- View/download PDF
13. The DM-scope registry: a rare disease innovative framework bridging the gap between research and medical care
- Author
-
Antonio, M., Dogan, C., Eymard, B., Puymirat, J., Mathieu, J., Gagnon, C., Attarian, S., Ac Aube-Nathier, Audic, F., Bach, N., Barnerias, C., Al Bedat-Millet, Behin, A., Bellance, R., Rabah BEN YAOU, Bombard, V., Bouhour, F., Boutte, C., Boyer, F., Cances, C., Chabrol, B., Jb Chanson, Chapon, F., Chasseriau, R., Cintas, P., Am Cobo, Colombert, V., Mc Cruz, Jm Cuisset, Deschamps, R., Desguerre, I., Durigneux, J., Duval, F., Espil, C., Fafin, C., Feasson, L., Fradin, M., Furby, A., Goldenberg, A., Grotto, S., Ghorab, K., Guyant-Marechal, L., Heron, D., Isapof, A., Jacquin-Piques, A., Journel, H., Laforet, P., Lagrue, E., Laroche-Raynaud, C., Laugel, V., Lebeau, F., Magot, A., Manel, V., Mayer, M., Mercier, S., Menard, D., Michaud, M., Mc Minot, Rj Morales, Nadaj-Pakleza, A., Jb Noury, Pasquier, L., Pellieux, S., Pereon, Y., Perrier, J., Peudenier, S., Preudhomme, M., Pouget, J., Quijano-Roy, S., Ragot-Mandry, S., Richelme, C., Rivier, F., Sabouraud, P., Sacconi, S., Salort-Campana, E., Sarret, C., Schaeffer, S., Sole, G., Stojkovic, T., Taithe, F., Testard, H., Tiffereau, V., Urtizberea, A., Vanhulle, C., Vial, C., Walther-Louvier, U., Zagnoli, F., Hamroun, D., Bassez, G., CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Institut de biologie et chimie des protéines [Lyon] (IBCP), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Centre de Recherches du Service de Santé des Armées (CRSSA), Service de Santé des Armées, Energy Storage and Conversion, Research Institute of Hydro-Québec, Energy Storage and Conversion, Hôpital de la Timone [CHU - APHM] (TIMONE), Department of Medicine, Icahn School of Medicine at Mount Sinai [New York] (MSSM), INVENTAIRE FORESTIER NATIONAL CAEN, Partenaires IRSTEA, Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA)-Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA), Centre de référence Caribéen pour les maladies neuromusculaires (CeRCa), Hôpital Pierre Zobda-Quitman [CHU de la Martinique], CHU de la Martinique [Fort de France]-CHU de la Martinique [Fort de France], Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Hospices Civils de Lyon (HCL), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Neurologie Pédiatrique, Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre de compétences pathologies neuromusculaires [CHU Caen], Département Neurologie [CHU Toulouse], Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université Paris Descartes - Paris 5 (UPD5), Université d'Angers (UA), Institut de Recherche en Systèmes Electroniques Embarqués (IRSEEM), Université de Rouen Normandie (UNIROUEN), and Normandie Université (NU)-Normandie Université (NU)-École Supérieure d’Ingénieurs en Génie Électrique (ESIGELEC)
- Subjects
[SDV]Life Sciences [q-bio] - Abstract
International audience; Background: The relevance of registries as a key component for developing clinical research for rare diseases (RD) and improving patient care has been acknowledged by most stakeholders. As recent studies pointed to several limitations of RD registries our challenge was (1) to improve standardization and data comparability; (2) to facilitate interoperability between existing RD registries; (3) to limit the amount of incomplete data; (4) to improve data quality. This report describes the innovative concept of the DM-Scope Registry that was developed to achieve these objectives for Myotonic Dystrophy (DM), a prototypical example of highly heterogeneous RD. By the setting up of an integrated platform attractive for practitioners use, we aimed to promote DM epidemiology, clinical research and patients care management simultaneously.Results: The DM-Scope Registry is a result of the collaboration within the French excellence network established by the National plan for RDs. Inclusion criteria is all genetically confirmed DM individuals, independently of disease age of onset. The dataset includes social-demographic data, clinical features, genotype, and biomaterial data, and is adjustable for clinical trial data collection. To date, the registry has a nationwide coverage, composed of 55 neuromuscular centres, encompassing the whole disease clinical and genetic spectrum. This widely used platform gathers almost 3000 DM patients (DM1 n = 2828, DM2 n = 142), both children (n = 322) and adults (n = 2648), which accounts for > 20% of overall registered DM patients internationally. The registry supported 10 research studies of various type i.e. observational, basic science studies and patient recruitment for clinical trials.Conclusion: The DM-Scope registry represents the largest collection of standardized data for the DM population. Our concept improved collaboration among health care professionals by providing annual follow-up of quality longitudinal data collection. The combination of clinical features and biomolecular materials provides a comprehensive view of the disease in a given population. DM-Scope registry proves to be a powerful device for promoting both research and medical care that is suitable to other countries. In the context of emerging therapies, such integrated platform contributes to the standardisation of international DM research and for the design of multicentre clinical trials. Finally, this valuable model is applicable to other RDs.
14. Erratum to: 36th International Symposium on Intensive Care and Emergency Medicine
- Author
-
Author et al, Bateman, R. M., Sharpe, M. D., Jagger, J. E., Ellis, C. G., Solé-Violán, J., López-Rodríguez, M., Herrera-Ramos, E., Ruíz-Hernández, J., Borderías, L., Horcajada, J., González-Quevedo, N., Rajas, O., Briones, M., Rodríguez de Castro, F., Rodríguez Gallego, C., Esen, F., Orhun, G., Ergin Ozcan, P., Senturk, E., Ugur Yilmaz, C., Orhan, N., Arican, N., Kaya, M., Kucukerden, M., Giris, M., Akcan, U., Bilgic Gazioglu, S., Tuzun, E., Riff, R., Naamani, O., Douvdevani, A., Takegawa, R., Yoshida, H., Hirose, T., Yamamoto, N., Hagiya, H., Ojima, M., Akeda, Y., Tasaki, O., Tomono, K., Shimazu, T., Ono, S., Kubo, T., Suda, S., Ueno, T., Ikeda, T., Ogura, H., Takahashi, H., Kang, J., Nakamura, Y., Kojima, T., Izutani, Y., Taniguchi, T., O, M., Dinter, C., Lotz, J., Eilers, B., Wissmann, C., Lott, R., Meili, M. M., Schuetz, P. S., Hawa, H., Sharshir, M., Aburageila, M., Salahuddin, N., Chantziara, V., Georgiou, S., Tsimogianni, A., Alexandropoulos, P., Vassi, A., Lagiou, F., Valta, M., Micha, G., Chinou, E., Michaloudis, G., Kodaira, A., Imaizumi, H., De la Torre-Prados, M. V., Garcia-De la Torre, A., Enguix-Armada, A., Puerto-Morlan, A., Perez-Valero, V., Garcia-Alcantara, A., Bolton, N., Dudziak, J., Bonney, S., Tridente, A., Nee, P., Nicolaes, G., Wiewel, M., Schultz, M., Wildhagen, K., Horn, J., Schrijver, R., Van der Poll, T., Reutelingsperger, C., Pillai, S., Davies, G., Mills, G., Aubrey, R., Morris, K., Williams, P., Evans, P., Gayat, E. G., Struck, J., Cariou, A., Deye, N., Guidet, B., Jabert, S., Launay, J., Legrand, M., Léone, M., Resche-Rigon, M., Vicaut, E., Vieillard-Baron, A., Mebazaa, A., Arnold, R., Capan, M., Linder, A., Akesson, P., Popescu, M., Tomescu, D., Sprung, C. L., Calderon Morales, R., Munteanu, G., Orenbuch-Harroch, E., Levin, P., Kasdan, H., Reiter, A., Volker, T., Himmel, Y., Cohen, Y., Meissonnier, J., Girard, L., Rebeaud, F., Herrmann, I., Delwarde, B., Peronnet, E., Cerrato, E., Venet, F., Lepape, A., Rimmelé, T., Monneret, G., Textoris, J., Beloborodova, N., Moroz, V., Osipov, A., Bedova, A., Sarshor, Y., Pautova, A., Sergeev, A., Chernevskaya, E., Odermatt, J., Bolliger, R., Hersberger, L., Ottiger, M., Christ-Crain, M., Mueller, B., Schuetz, P., Sharma, N. K., Tashima, A. K., Brunialti, M. K., Machado, F. R., Assuncao, M., Rigato, O., Salomao, R., Cajander, S. C., Rasmussen, G., Tina, E., Söderquist, B., Källman, J., Strålin, K., Lange, A. L., Sundén-Cullberg, J. S., Magnuson, A. M., Hultgren, O. H., Van der Geest, P., Mohseni, M., Linssen, J., De Jonge, R., Duran, S., Groeneveld, J., Miller, R., Lopansri, B. K., McHugh, L. C., Seldon, A., Burke, J. P., Johnston, J., Reece-Anthony, R., Bond, A., Molokhia, A., Mcgrath, C., Nsutebu, E., Bank Pedersen, P., Pilsgaard Henriksen, D., Mikkelsen, S., Touborg Lassen, A., Tincu, R., Cobilinschi, C., Ghiorghiu, Z., Macovei, R., Wiewel, M. A., Harmon, M. B., Van Vught, L. A., Scicluna, B. P., Hoogendijk, A. J., Zwinderman, A. H., Cremer, O. L., Bonten, M. J., Schultz, M. J., Juffermans, N. P., Wiersinga, W. J., Eren, G., Tekdos, Y., Dogan, M., Acicbe, O., Kaya, E., Hergunsel, O., Alsolamy, S., Ghamdi, G., Alswaidan, L., Alharbi, S., Alenezi, F., Arabi, Y., Heaton, J., Boyce, A., Nolan, L., Dukoff-Gordon, A., Dean, A., Mann Ben Yehudah, T., Fleischmann, C., Thomas-Rueddel, D., Haas, C., Dennler, U., Reinhart, K., Suntornlohanakul, O., Khwannimit, B., Breckenridge, F., Puxty, A., Szturz, P., Folwarzcny, P., Svancara, J., Kula, R., Sevcik, P., Caneva, L., Casazza, A., Bellazzi, E., Marra, S., Pagani, L., Vetere, M., Vanzino, R., Ciprandi, D., Preda, R., Boschi, R., Carnevale, L., Lopez, V., Aguilar Arzapalo, M., Barradas, L., Escalante, A., Gongora, J., Cetina, M., Adamik, B, Jakubczyk, D, Kübler, A, Radford, A., Lee, T., Singer, J., Boyd, J., Fineberg, D., Williams, M., Russell, J., Scarlatescu, E., Droc, G., Arama, S., Müller, M., Straat, M., Zeerleder, S. S., Fuchs, C. F., Scheer, C. S., Wauschkuhn, S. W., Vollmer, M. V., Meissner, K. M., Kuhn, S. K., Hahnenkamp, K. H., Rehberg, S. R., Gründling, M. G., Hamaguchi, S., Gómez-Sánchez, E., Heredia-Rodríguez, M., Álvarez-Fuente, E., Lorenzo-López, M., Gómez-Pesquera, E., Aragón-Camino, M., Liu-Zhu, P., Sánchez-López, A., Hernández-Lozano, A., Peláez-Jareño, M. T., Tamayo, E., Thomas-Rüddel, D. O., Adora, V., Kar, A., Chakraborty, A., Roy, S., Bandyopadhyay, A., Das, M., BenYehudah, G., Salim, M., Kumar, N., Arabi, L., Burger, T., Lephart, P., Toth-martin, E., Valencia, C., Hammami, N., Blot, S., Vincent, J. L., Lambert, M. L., Brunke, J., Riemann, T., Roschke, I., Nimitvilai, S., Jintanapramote, K., Jarupongprapa, S., Adukauskiene, D., Valanciene, D., Bose, G., Lostarakos, V., Carr, B., Khedher, S., Maaoui, A., Ezzamouri, A., Salem, M., Chen, J., Cranendonk, D. R., Day, M., Penrice, G., Roy, K., Robertson, P., Godbole, G., Jones, B., Booth, M., Donaldson, L., Kawano, Y., Ishikura, H., Al-Dorzi, H., Almutairi, M., Alhamadi, B., Crizaldo Toledo, A., Khan, R., Al Raiy, B., Talaie, H., Van Oers, J. A., Harts, A., Nieuwkoop, E., Vos, P., Boussarsar, Y., Boutouta, F., Kamoun, S., Mezghani, I., Koubaji, S., Ben Souissi, A., Riahi, A., Mebazaa, M. S., Giamarellos-Bourboulis, E., Tziolos, N., Routsi, C., Katsenos, C., Tsangaris, I., Pneumatikos, I., Vlachogiannis, G., Theodorou, V., Prekates, A., Antypa, E., Koulouras, V., Kapravelos, N., Gogos, C., Antoniadou, E., Mandragos, K., Armaganidis, A., Robles Caballero, A. R., Civantos, B., Figueira, J. C., López, J., Silva-Pinto, A., Ceia, F., Sarmento, A., Santos, L., Almekhlafi, G., Sakr, Y., Baharoon, S., Aldawood, A., Matroud, A., Alchin, J., Al Johani, S., Balkhy, H., Yousif, S. Y., Alotabi, B. O., Alsaawi, A. S., Ang, J., Curran, M. D., Enoch, D., Navapurkar, V., Morris, A., Sharvill, R., Astin, J., Patel, J., Kruger, C., O’Neal, J., Rhodes, H., Jancik, J., François, B., Laterre, P. F., Eggimann, P., Torres, A., Sánchez, M., Dequin, P. F., Bassi, G. L., Chastre, J., Jafri, H. S., Ben Romdhane, M., Douira, Z., Bousselmi, M., Vakalos, A., Avramidis, V., Craven, T. H., Wojcik, G., Kefala, K., McCoubrey, J., Reilly, J., Paterson, R., Inverarity, D., Laurenson, I., Walsh, T. S., Mongodi, S., Bouhemad, B., Orlando, A., Stella, A., Via, G., Iotti, G., Braschi, A., Mojoli, F., Haliloglu, M., Bilgili, B., Kasapoglu, U., Sayan, I., Süzer Aslan, M., Yalcin, A., Cinel, I., Ellis, H. E., Bauchmuller, K., Miller, D., Temple, A., Luyt, C. E., Singer, M., Nassar, Y., Ayad, M. S., Trifi, A., Abdellatif, S., Daly, F., Nasri, R., Ben Lakhal, S., Gul, F., Kuzovlev, A., Shabanov, A., Polovnikov, S., Kadrichu, N., Dang, T., Corkery, K., Challoner, P., Bassi, G. Li, Aguilera, E., Chiurazzi, C., Travierso, C., Motos, A., Fernandez, L., Amaro, R., Senussi, T., Idone, F., Bobi, J., Rigol, M., Hodiamont, C. J., Janssen, J. M., Bouman, C. S., Mathôt, R. A., De Jong, M. D., Van Hest, R. M., Payne, L., Fraser, G. L., Tudor, B., Lahner, M., Roth, G., Krenn, C., Jault, P., Gabard, J., Leclerc, T., Jennes, S., Que, Y., Rousseau, A., Ravat, F., Eissa, A., Al-Harbi, S., Aldabbagh, T., Abdellatif., S., Paramba, F., Purayil, N., Naushad, V., Mohammad, O., Negi, V., Chandra, P., Kleinsasser, A., Witrz, M. R., Buchner-Doeven, J. F., Tuip-de Boer, A. M., Goslings, J. C., Van Hezel, M., Boing, A, Van Bruggen, R, Juffermans, N, Markopoulou, D., Venetsanou, K., Kaldis, V., Koutete, D., Chroni, D., Alamanos, I., Koch, L., Walter, E., Maekawa, K., Hayakawa, M., Kushimoto, S., Shiraishi, A., Kato, H., Sasaki, J., Matauoka, T., Uejima, T., Morimura, N., Hagiwara, A., Takeda, M., Tarabrin, O., Shcherbakow, S., Gavrychenko, D., Mazurenko, G., Ivanova, V., Chystikov, O., Plourde, C., Lessard, J., Chauny, J., Daoust, R., Kropman, L., In het Panhuis, L., Konings, J., Huskens, D., Schurgers, E., Roest, M., De Laat, B., Lance, M., Durila, M., Lukas, P., Astraverkhava, M., Jonas, J., Budnik, I., Shenkman, B., Hayami, H., Koide, Y., Goto, T., Iqbal, R., Alhamdi, Y., Venugopal, N., Abrams, S., Downey, C., Toh, C. H., Welters, I. D., Bombay, V. B., Chauny, J. M., Daoust, R. D., Lessard, J. L., Marquis, M. M., Paquet, J. P., Siemens, K., Sangaran, D., Hunt, B. J., Durward, A., Nyman, A., Murdoch, I. A., Tibby, S. M., Ampatzidou, F., Moisidou, D., Dalampini, E., Nastou, M., Vasilarou, E., Kalaizi, V., Chatzikostenoglou, H., Drossos, G., Spadaro, S., Fogagnolo, A., Fiore, T., Schiavi, A., Fontana, V., Taccone, F., Volta, C., Chochliourou, E., Volakli, E., Violaki, A., Samkinidou, E., Evlavis, G., Panagiotidou, V., Sdougka, M., Mothukuri, R., Battle, C., Guy, K., Wijesuriya, J., Keogh, S., Docherty, A., O’Donnell, R., Brunskill, S., Trivella, M., Doree, C., Holst, L., Parker, M., Gregersen, M., Almeida, J., Walsh, T., Stanworth, S., Moravcova, S., Mansell, J., Rogers, A., Smith, R. A., Hamilton-Davies, C., Omar, A., Allam, M., Bilala, O., Kindawi, A., Ewila, H., Malamas, A., Ferreira, G., Caldas, J., Fukushima, J., Osawa, E. A., Arita, E., Camara, L., Zeferino, S., Jardim, J., Gaioto, F., Dallan, L., Jatene, F. B., Kalil Filho, R., Galas, F., Hajjar, L. A., Mitaka, C., Ohnuma, T., Murayama, T., Kunimoto, F., Nagashima, M., Takei, T., Tomita, M., Mahmoud, K., Hanoura, S., Sudarsanan, S., Sivadasan, P., Othamn, H., Shouman, Y., Singh, R., Al Khulaifi, A., Mandel, I., Mikheev, S., Suhodolo, I., Kiselev, V., Svirko, Y., Podoksenov, Y., Jenkins, S. A., Griffin, R., Tovar Doncel, M. S., Lima, A., Aldecoa, C., Ince, C., Taha, A., Shafie, A., Mostafa, M., Syed, N., Hon, H., Righetti, F., Colombaroli, E., Castellano, G., Hravnak, M., Chen, L. C., Dubrawski, A. D., Clermont, G. C., Pinsky, M. R., Gonzalez, S., Macias, D., Acosta, J., Jimenez, P., Loza, A., Lesmes, A., Lucena, F., Leon, C., Bastide, M., Richecoeur, J., Frenoy, E., Lemaire, C., Sauneuf, B., Tamion, F., Nseir, S., Du Cheyron, D., Dupont, H., Maizel, J., Shaban, M., Kolko, R., AbuRageila, M., AlHussain, A., Mercado, P., Kontar, L., Titeca, D., Brazier, F., Riviere, A., Joris, M., Soupison, T., De Cagny, B., Slama, M., Wagner, J., Körner, A., Kubik, M., Kluge, S., Reuter, D., Saugel, B., Tran, T., De Bels, D., Cudia, A., Strachinaru, M., Ghottignies, P., Devriendt, J., Pierrakos, C., Martínez González, Ó., Blancas, R., Luján, J., Ballesteros, D., Martínez Díaz, C., Núñez, A., Martín Parra, C., López Matamala, B., Alonso Fernández, M., Chana, M., Huber, W., Eckmann, M., Elkmann, F., Gruber, A., Klein, I., Schmid, R. M., Lahmer, T., Moller, P. W., Sondergaard, S., Jakob, S. M., Takala, J., Berger, D., Bastoni, D., Aya, H., Toscani, L., Pigozzi, L., Rhodes, A., Cecconi, M., Ostrowska, C., Abbas, A., Mellinghoff, J., Ryan, C., Dawson, D., Cronhjort, M., Wall, O., Nyberg, E., Zeng, R., Svensen, C., Mårtensson, J., Joelsson-Alm, E., Parenti, N., Palazzi, C., Amidei, L. A., Borrelli, F. B., Campanale, S. C., Tagliazucchi, F. T., Sedoni, G. S., Lucchesi, D. L., Carella, E. C., Luciani, A. L, Mackovic, M., Maric, N., Bakula, M., Grounds, R. M., Fletcher, N., Avard, B., Zhang, P., Mezidi, M., Charbit, J., Ould-Chikh, M., Deras, P., Maury, C., Martinez, O., Capdevila, X., Hou, P., Linde-Zwirble, W. Z., Douglas, I. D., Shapiro, N. S., Ben Aicha, Y., Laribi, B., Jeribi, B., Pereira, C., Marinho, R., Antunes, R., Marinho, A., Crivits, M., Raes, M., Decruyenaere, J., Hoste, E., Bagin, V., Rudnov, V., Savitsky, A., Astafyeva, M., Korobko, I., Vein, V., Kampmeier, T., Arnemann, P., Hessler, M., Wald, A., Bockbreder, K., Morelli, A., Van Aken, H., Rehberg, S., Ertmer, C., Reddy, S., Bailey, M., Beasley, R., Bellomo, R., Mackle, D., Psirides, A., Young, P., Venkatesh, H., Ramachandran, S., Basu, A., Nair, H., Egan, S., Bates, J., Oliveira, S., Rangel Neto, N. R., Reis, F. Q., Lee, C. P., Lin, X. L., Choong, C., Eu, K. M., Sim, W. Y., Tee, K. S., Pau, J., Abisheganaden, J., Maas, K., De Geus, H., Lafuente, E., Moura, J., Doris, T. E., Monkhouse, D., Shipley, T., Kardasz, S., Gonzalez, I, Stads, S., Groeneveld, A. J., Elsayed, I., Ward, N., Raithatha, A., Steuber, A., Pelletier, C., Schroeder, S., Michael, E., Slowinski, T., Kindgen-Milles, D., Ghabina, S., Turani, F., Belli, A., Busatti, S., Barettin, G., Candidi, F., Gargano, F., Barchetta, R., Falco, M., Demirkiran, O., Kosuk, M., Bozbay, S., Weber, V., Hartmann, J., Harm, S., Linsberger, I., Eichhorn, T., Valicek, G., Miestinger, G., Hoermann, C., Faenza, S., Ricci, D., Mancini, E., Gemelli, C., Cuoghi, A., Magnani, S., Atti, M., Laddomada, T., Doronzio, A., Balicco, B., Gruda, M. C., O’Sullivan, P., Dan, V. P., Guliashvili, T., Scheirer, A., Golobish, T. D., Capponi, V. J., Chan, P. P., Kogelmann, K., Drüner, M., Jarczak, D., Belli, A. B., Martni, S. M., Cotticelli, V. C., Mounajergi, F., Morimoto, S., Hussain, I., Nadeem, A., Ghorab, K., Maghrabi, K., Kloesel, S. K., Goldfuss, C., Stieglitz, A., Stieglitz, A. S., Krstevska, L., Albuszies, G., Jimmy, G., Izawa, J., Iwami, T., Uchino, S., Takinami, M., Kitamura, T., Kawamura, T., Powell-Tuck, J. G., Crichton, S., Raimundo, M., Camporota, L., Wyncoll, D., Ostermann, M., Hana, A., De Geus, H. R., Aydogdu, M., Boyaci, N., Yuksel, S., Gursel, G., Cayci Sivri, A. B., Meza-Márquez, J., Nava-López, J., Carrillo-Esper, R., Dardashti, A., Grubb, A., Wetzstein, M., Peters, E., Njimi, H., Pickkers, P., Waraich, M., Doyle, J., Samuels, T., Forni, L., Desai, N., Baumber, R., Gunning, P., Sell, A., Lin, S., Torrence, H., O’Dwyer, M., Kirwan, C., Prowle, J., Kim, T., O’Connor, M. E., Hewson, R. W., Kirwan, C. J., Pearse, R. M., Maksoud, M., Uzundere, O., Memis, D., Ýnal, M., Gultekin, A., Turan, N., Aydin, M. A., Basar, H., Sencan, I., Kapuagasi, A., Ozturk, M., Uzundurukan, Z., Gokmen, D., Ozcan, A., Kaymak, C., Artemenko, V. A., Budnyuk, A., Pugh, R., Bhandari, S., Mauri, T., Turrini, C., Langer, T., Taccone, P., Volta, C. A., Marenghi, C., Gattinoni, L., Pesenti, A., Sweeney, L., O’Sullivan, A., Kelly, P., Mukeria, E., MacLoughlin, R., Pfeffer, M., Thomas, J. T., Bregman, G. B., Karp, G. K., Kishinevsky, E. K., Stavi, D. S., Adi, N. A., Poropat, T., Knafelj, R., Llopart, E., Batlle, M., De Haro, C., Mesquida, J., Artigas, A., Pavlovic, D., Lewerentz, L., Spassov, A., Schneider, R., De Smet, S., De Raedt, S., Derom, E., Depuydt, P, Oeyen, S., Benoit, D., Gobatto, A., Besen, B., Tierno, P., Melro, L., Mendes, P., Cadamuro, F., Park, M., Malbouisson, L. M., Civantos, B. C., Lopez, J. L., Robles, A., Figueira, J., Yus, S., Garcia, A., Oglinda, A., Ciobanu, G., Oglinda, C., Schirca, L., Sertinean, T., Lupu, V., Wolny, M., Pagano, A., Numis, F., Visone, G., Saldamarco, L., Russo, T., Porta, G., Paladino, F., Bell, C., Liu, J., Debacker, J., Lee, C., Tamberg, E., Campbell, V., Mehta, S., Kara, Ý., Yýldýrým, F., Zerman, A., Güllü, Z., Boyacý, N., Basarýk Aydogan, B., Gaygýsýz, Ü., Gönderen, K., Arýk, G., Turkoglu, M., Aygencel, G., Ülger, Z., Isýkdogan, Z., Özdedeoglu, Ö., Badoglu, M., Gaygýsýz, U., Kongpolprom, N., Sittipunt, C., Eden, A., Kokhanovsky, Y., Bursztein – De Myttenaere, S., Pizov, R., Neilans, L., MacIntyre, N., Radosevich, M., Wanta, B., Meyer, T., Smischney, N., Brown, D., Diedrich, D., Fuller, A., McLindon, P., Sim, K., Shoaeir, M., Noeam, K., Mahrous, A., Matsa, R., Ali, A., Dridi, C., Haddad, F., Pérez-Calatayud, A., Zepeda-Mendoza, A., Diaz-Carrillo, M., Arch-Tirado, E., Carbognin, S., Pelacani, L., Zannoni, F., Agnoli, A., Gagliardi, G., Cho, R., Adams, A., Lunos, S., Ambur, S., Shapiro, R., Prekker, M., Thijssen, M., Janssen, L., Foudraine, N., Voscopoulos, C. J., Freeman, J., George, E., Eversole, D., Muttini, S., Bigi, R., Villani, G., Patroniti, N., Williams, G., George, E, Waldmann, A., Böhm, S., Windisch, W., Strassmann, S., Karagiannidis, C., Karagiannidis, C. K., Waldmann, A. W., Böhm, S. B., Windisch, W. W., Persson, P., Lundin, S., Stenqvist, O., Serra, C. S., Pagano, A. P., Masarone, M. M., Rinaldi, L. R., Amelia, A. A., Fascione, M. F., Adinolfi, L. A., Ruggiero, E. R., Asota, F., O’Rourke, K., Ranjan, S., Morgan, P., DeBacker, J. W., O’Neill, L., Munshi, L., Burry, L., Fan, E., Poo, S., Mahendran, K., Fowles, J., Gerrard, C., Vuylsteke, A., Loveridge, R., Chaddock, C., Patel, S., Kakar, V., Willars, C., Hurst, T., Park, C., Best, T., Vercueil, A., Auzinger, G., Borgman, A., Proudfoot, A. G., Grins, E., Emiley, K. E., Schuitema, J., Fitch, S. J., Marco, G., Sturgill, J., Dickinson, M. G., Strueber, M., Khaghani, A., Wilton, P., Jovinge, S. M., Sampson, C., Harris-Fox, S., Cove, M. E., Vu, L. H., Sen, A., Federspiel, W. J., Kellum, J. A., Mazo Torre, C., Riera, J., Ramirez, S., Borgatta, B., Lagunes, L., Rello, J., Kuzovlev, A. K., Goloubev, A., Nenchuk, S., Karavana, V., Glynos, C., Asimakos, A., Pappas, K., Vrettou, C., Magkou, M., Ischaki, E., Stathopoulos, G., Zakynthinos, S., Kozhevnikova, I., Dalla Corte, F., Grasso, S., Casolari, P., Caramori, G., Andrianjafiarinoa, T., Randriamandrato, T., Rajaonera, T., El-Dash, S., Costa, E. L. V., Tucci, M. R., Leleu, F, Kontar, L, Bacari-Risal, G., Amato, M., El Dash, S., Remmington, Fischer, A., Squire, S., Boichat, M., Honzawa, H., Yasuda, H., Adati, T., Suzaki, S., Horibe, M., Sasaki, M., Sanui, M., Daniel, J., Miranda, H., Milinis, K., Cooper, M., Williams, G. R., McCarron, E., Simants, S., Patanwala, I., Welters, I., Su, Y., Fernández Villanueva, J., Fernández Garda, R., López Lago, A., Rodríguez Ruíz, E., Hernández Vaquero, R., Tomé Martínez de Rituerto, S., Varo Pérez, E., Lefel, N., Schaap, F., Bergmans, D., Olde Damink, S., Van de Poll, M., Tizard, K., Lister, C., Poole, L., Ringaitiene, D., Gineityte, D., Vicka, V., Norkiene, I., Sipylaite, J., O’Loughlin, A., Maraj, V., Dowling, J., Velasco, M. B., Dalcomune, D. M., Dias, E. B., Fernandes, S. L., Oshima, T., Graf, S., Heidegger, C., Genton, L., Karsegard, V., Dupertuis, Y., Pichard, C., Friedli, N., Stanga, Z., Vandersteen, L., Stessel, B., Evers, S., Van Assche, A., Jamaer, L., Dubois, J., Castro, H., Valente, J., Martins, P., Casteloes, P., Magalhaes, C., Cabral, S., Santos, M., Oliveira, B., Salgueiro, A., Duarte, S., Castro, S., Melo, M., Gray, S., Maipang, K., Bhurayanontachai, R., Grädel, L. G., Schütz, P., Langlois, P., Manzanares, W., Lemieux, M., Elke, G., Bloos, F., Heyland, D., Aramendi, I., Babo, N., Hoshino, M., Haraguchi, Y., Kajiwara, S., Mitsuhashi, T., Tsubata, T., Aida, M., Rattanapraphat, T., Kongkamol, C., Xavier, B., Koutsogiannidis, C., Moschopoulou, M., Taskin, G., Çakir, M., Güler, AK, Taskin, A., Öcal, N., Özer, S., Yamanel, L., Wong, J. M., Fitton, C., Anwar, S., Stacey, S., Aggou, M., Fyntanidou, B., Patsatzakis, S., Oloktsidou, E., Lolakos, K., Papapostolou, E., Grosomanidis, V., Gaudry, S., Desailly, V., Pasquier, P., Brun, PB, Tesnieres, AT, Ricard, JD, Dreyfuss, D., Mignon, A., White, J. C, Stilwell, A., Friedlaender, G., Peters, M., Stipulante, S., Delfosse, A., Donneau, AF, Ghuysen, A., Feldmann, C., Freitag, D., Dersch, W., Irqsusi, M., Eschbach, D., Steinfeldt, T., Wulf, H., Wiesmann, T., Cholkraisuwat, J., Beitland, S., Nakstad, E., Stær-Jensen, H., Drægni, T., Andersen, G., Jacobsen, D., Brunborg, C., Waldum-Grevbo, B., Sunde, K., Hoyland, K., Pandit, D., Hayakawa, K., Kotzampassi, K., Loukipoudi, L., Doumaki, E., Admiraal, M. M., Van Assen, M., Van Putten, M. J., Tjepkema-Cloostermans, M., Van Rootselaar, A. F., Ragusa, F., Marudi, A., Baroni, S., Gaspari, A., Bertellini, E., Abdullah, T., Abdel Monem, S., Alcorn, S., McNeill, S., Russell, S., Eertmans, W., Genbrugge, C., Meex, I., Dens, J., Jans, F., De Deyne, C., Avard, B, Burns, R, Patarchi, A., Spina, T., Tanaka, H., Otani, N., Ode, S., Ishimatsu, S., Cho, J., Moon, J. B., Park, C. W., Ohk, T. G., Shin, M. C., Won, M. H., Dakova, S., Ramsheva, Z., Ramshev, K., Marudi, A, Baroni, S, Gaspari, A, Bertellini, E, Ozcan, P. E., Sencer, S., Ulusoy, C., Fallenius, M., Skrifvars, M. B., Reinikainen, M., Bendel, S., Raj, R., Abu-Habsa, M., Hymers, C., Borowska, A., Sivadhas, H., Sahiba, S., Perkins, S., Rubio, J., Rubio, J. A., Sierra, R., English, S., Chasse, M., Turgeon, A., Lauzier, F., Griesdale, D., Garland, A., Fergusson, D., Zarychanski, R., Tinmouth, A., Van Walraven, C., Montroy, K., Ziegler, J., Dupont Chouinard, R., Carignan, R., Dhaliwal, A., Lum, C., Sinclair, J., Pagliarello, G., McIntyre, L., Groza, T., Moreau, N., Castanares-Zapatero, D., Hantson, P., Carbonara, M., Ortolano, F., Zoerle, T., Magnoni, S., Pifferi, S., Conte, V., Stocchetti, N., Carteron, L., Suys, T., Patet, C., Quintard, H., Oddo, M., Spatenkova, V., Pokorna, E., Suchomel, P., Ebert, N., Bylinski, T., Hawthorne, C., Shaw, M., Piper, I., Kinsella, J., Kink, A. K., Rätsep, I. R., Boutin, A., Moore, L., Lacroix, J., Lessard-Bonaventure, P., Turgeon, A. F., Green, R., Erdogan, M., Butler, M., Desjardins, P., Fergusson, D. A., Goncalves, B., Vidal, B., Valdez, C., Rodrigues, A. C., Miguez, L., Moralez, G., Hong, T., Kutz, A., Hausfater, P., Amin, D., Struja, T., Haubitz, S., Huber, A., Brown, T., Collinson, J., Pritchett, C., Slade, T., Le Guen, M., Hellings, S., Ramsaran, R., Alsheikhly, A., Abe, T., Kanapeckaite, L., Bahl, R., Russell, M. Q., Real, K. J., Lyon, R. M., Oveland, N. P., Penketh, J., Mcdonald, M., Kelly, F., Alfafi, M., Almutairi, W., Alotaibi, B., Van den Berg, A. E, Schriel, Y., Dawson, L., Meynaar, I. A., Silva, D., Fernandes, S., Gouveia, J., Santos Silva, J., Foley, J., Kaskovagheorgescu, A., Evoy, D., Cronin, J., Ryan, J., Huck, M., Hoffmann, C., Renner, J., Laitselart, P., Donat, N., Cirodde, A., Schaal, J. V., Masson, Y., Nau, A., Howarth, O., Davenport, K., Jeanrenaud, P., Raftery, S., MacTavish, P., Devine, H., McPeake, J., Daniel, M., Quasim, T., Alrabiee, S., Alrashid, A., Gundogan, O., Bor, C., Akýn Korhan, E., Demirag, K., Uyar, M., Frame, F., Ashton, C., Bergstrom Niska, L., Dilokpattanamongkol, P., Suansanae, T., Suthisisang, C., Morakul, S., Karnjanarachata, C., Tangsujaritvijit, V., Mahmood, S., Al Thani, H., Almenyar, A., Morton, S. E., Chiew, Y. S., Pretty, C., Chase, J. G., Shaw, G. M., Kordis, P., Grover, V., Kuchyn, I., Bielka, K., Aidoni, Z., Stavrou, G., Skourtis, C., Lee, S. D., Williams, K., Weltes, I. D., Berhane, S., Arrowsmith, C., Peters, C., Robert, S., Panerai, R. B., Robinson, T. G., Borg-Seng-Shu, E., De Lima Oliveira, M., Mian, N. C., Nogueira, R., Zeferino, S. P., Jacobsen Teixeira, M., Killeen, P., McPhail, M., Bernal, W., Maggs, J., Wendon, J., Hughes, T., Taniguchi, L. U., Siqueira, E. M., Vieira Jr, J. M., Azevedo, L. C., Ahmad, A. N., Helme, E., Hadfield, S., Shak, J., Senver, C., Howard-Griffin, R., Wacharasint, P., Fuengfoo, P., Sukcharoen, N., Rangsin, R., Sbiti-Rohr, D., Na, H., Song, S., Lee, S., Jeong, E., Lee, K., Zoumpelouli, E., Volakli, E. A, Chrysohoidou, V., Charisopoulou, K., Kotzapanagiotou, E., Manavidou, K., Stathi, Z., AlGhamdi, B., Marashly, Q., Zaza, K., Khurshid, M., Ali, Z., Malgapo, M., Jamil, M., Shafquat, A., Shoukri, M., Hijazi, M., Rocha, F. A., Ebecken, K., Rabello, L. S., Lima, M. F., Hatum, R., De Marco, F. V., Alves, A., Pinto, J. E., Godoy, M., Brasil, P. E., Bozza, F. A., Salluh, J. I., Soares, M., Krinsley, J., Kang, G., Perry, J., Hines, H., Wilkinson, K. M., Tordoff, C., Sloan, B., Bellamy, M. C., Moreira, E., Verga, F., Barbato, M., Burghi, G., Soares, M, Silva, U. V., Torelly, A. P., Kahn, J. M., Angus, D. C., Knibel, M. F., Marshall, R., Gilpin, T., Mota, D., Loureiro, B., Dias, J., Afonso, O., Coelho, F., Martins, A., Faria, F., Al Orainni, H., AlEid, F., Tlaygeh, H., Itani, A., Hejazi, A., Messika, J., Ricard, J. D., Guillo, S., Pasquet, B., Dubief, E., Tubach, F., James, K., Temblett, P., Davies, L., Lynch, C., Pereira, S., Cavaco, S., Fernandes, J., Moreira, I., Almeida, E., Seabra Pereira, F., Malheiro, M., Cardoso, F., Aragão, I., Cardoso, T., Fister, M., Muraray Govind, P., Brahmananda Reddy, N., Pratheema, R., Arul, E. D., Devachandran, J., Chin-Yee, N., D’Egidio, G., Thavorn, K., Kyeremanteng, K., Murchison, A. G., Swalwell, K., Mandeville, J., Stott, D., Guerreiro, I., Goossens, C., Marques, M. B., Derde, S., Vander Perre, S., Dufour, T., Thiessen, S. E., Güiza, F., Janssens, T., Hermans, G., Vanhorebeek, I., De Bock, K., Van den Berghe, G., Langouche, L., Miles, B., Madden, S., Weiler, M., Marques, P., Rodrigues, C., Boeira, M., Brenner, K., Leães, C., Machado, A., Townsend, R., Andrade, J., Kishore, R., Fenlon, C., Fiks, T., Ruijter, A., Te Raa, M., Spronk, P., Docherty, P., Dickson, J., Moltchanova, E., Scarrot, C., Hall, T., Ngu, W. C., Jack, J. M., Pavli, A., Gee, X., Akin Korhan, E., Shirazy, M., Fayed, A., Gupta, S., Kaushal, A., Dewan, S., Varma, A., Ghosh, E., Yang, L., Eshelman, L., Lord, B., Carlson, E., Broderick, R., Ramos, J., Forte, D., Yang, F., Feeney, J., Wilkinson, K., Shuker, K., Faulds, M., Bryden, D., England, L., Shuker, K, Tridente, A, Faulds, M, Matheson, A, Gaynor, J., Bryden, D, ᅟ, S South Yorkshire Hospitals Research Collaboration, Peroni, B., Daglius-Dias, R., Miranda, L., Cohen, C., Carvalho, C., Velasco, I., Kelly, J. M., Neill, A., Rubenfeld, G., Masson, N., Min, A., Boezeman, E., Hofhuis, J., Hovingh, A., De Vries, R., Cabral-Campello, G., Van Mol, M., Nijkamp, M., Kompanje, E., Ostrowski, P., Kiss, K., Köves, B., Csernus, V., Molnár, Z., Hoydonckx, Y., Vanwing, S., Medo, V., Galvez, R., Miranda, J. P., Stone, C., Wigmore, T., Arunan, Y., Wheeler, A., Wong, Y., Poi, C., Gu, C., Molmy, P., Van Grunderbeeck, N., Nigeon, O., Lemyze, M., Thevenin, D., Mallat, J., Correa, M., Carvalho, R. T., Fernandez, A., McBride, C., Koonthalloor, E., Walsh, C., Webber, A., Ashe, M., Smith, K., Volakli, E. A., Dimitriadou, M., Mantzafleri, P., Vrani, O., Arbouti, A., Varsami, T., Bollen, J. A., Van Smaalen, T. C., De Jongh, W. C., Ten Hoopen, M. M., Ysebaert, D., Van Heurn, L. W., Van Mook, W. N., Roze des Ordons, A., Couillard, P., Doig, C., Van Keer, R. V., Deschepper, R. D., Francke, A. F., Huyghens, L. H., Bilsen, J. B., Nyamaizi, B., Dalrymple, C., Dobru, A., Marrinan, E., Ankuli, A., Struthers, R., Crawford, R., Mactavish, P., Morelli, P., Degiovanangelo, M., Lemos, F., MArtinez, V., Cabrera, J., Rutten, A., Van Ieperen, S., De Geer, S., Van Vugt, M., Der Kinderen, E., Giannini, A., Miccinesi, G, Marchesi, T, and Prandi, E
- Subjects
health care facilities, manpower, and services ,education ,Erratum ,Critical Care and Intensive Care Medicine ,reproductive and urinary physiology ,humanities ,health care economics and organizations - Full Text
- View/download PDF
15. 262P A global analysis of the CMT1A locus: implications for the origin and susceptibility to Charcot-Marie-Tooth disease type 1A across populations.
- Author
-
Cruz, P. Rodriguez, Alitsiou, A., Diagne, R., Lia-Baldini, A., Ghorab, K., Diop, A. Gallo, Ndiaye, M., Beltran, S., and Lao, O.
- Subjects
- *
HOMOLOGOUS recombination , *WHOLE genome sequencing , *GENETIC recombination , *GENETIC variation , *CHARCOT-Marie-Tooth disease , *DISEASE susceptibility - Abstract
Charcot-Marie-Tooth type 1A (CMT1A) is an inherited neuropathy with an estimated prevalence of 1/5000 in the general population. CMT1A results from abnormal dosage of the PMP22 gene, arising from non-allelic homologous recombination (NAHR) events between paralogous sequences called CMT1A-REPs, flanking the gene. While extensively documented in Caucasian individuals, CMT1A is under-reported in individuals of African genetic ancestry. We hypothesize that this is due to differences in the CMT1A-REPs between individuals of African and non-African genetic background. Our aim is to study the variability of CMT1A-REPs between populations using available genetic data and in silico analyses. Here, we have built a combined dataset from previous whole genome sequencing efforts (1KGP, HGDP and other available datasets at EGA) with a focus on capturing African genetic variation. Leveraging this data, we explore haplotypes based on both common and rare SNPs, structural variants in CMT1A-REP regions, recombination-related elements, and their potential interactions, as well as their distribution across different populations. We find that individuals of African genetic ancestry harbour distinct CMT1A-REP haplotypes with lower recombination rates and reduced sequence similarity compared to other ethnicities. This suggests potential genetic variation protective against NAHR-mediated events causing CMT1A. Additionally, recombination regulators like PRDM9 might differ between African and non-African ethnicities, hinting at distinct mechanisms at play. In conclusion, our study shows genetic differences in the CMT1A locus between African and non-African ancestries, which may account to the under-reporting of CMT1A in individuals of African genetic origin. However, further research is needed to better understand the epidemiology of inherited neuropathies and CMT1A in the African continent and how CMT1A-REP variations influence disease risk. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
16. The First Large Deletion of ATL3 Identified in a Patient Presenting with a Sensory Polyneuropathy.
- Author
-
Pyromali I, Richard L, Derouault P, Vallat JM, Ghorab K, Magdelaine C, Sturtz F, Favreau F, and Lia AS
- Abstract
Hereditary sensory neuropathies (HSN) are a heterogenous group of sensory neuropathies. Mutations in ATL3 have been described in patients presenting with hereditary sensory neuropathy IF (HSN1F), a subtype of HSN. Herein, by analyzing targeted-NGS data of a patient presenting with sensory neuropathy symptoms using the CovCopCan bioinformatic tool, we discovered the presence of a deletion of around 3kb in ATL3 from Chr11:63,401,422 to Chr11:63,398,182. This deletion affects ATL3 exons 11 and 12 and could lead to the mutation c.(1036-861_1539+329del), p.(Ala346_Gln513del). In addition, an analysis of the breakpoints' sequences revealed the presence of Alu transposable elements at the position of the breakpoints, which pointed to a possible erroneous recombination event following a non-allelic-homologous-recombination mechanism in this area. Moreover, electronic microscopy analysis of the patient's nerve biopsy revealed a severe rarefaction of the myelinated fibers, a demyelinating-remyelinating process, and an abnormal aspect of the endoplasmic reticulum. These findings suggest that this structural variation could potentially be responsible for the HSN symptoms of the patient. Research of structural variations in ATL3 in numerous other patients presenting similar symptoms should be broadly investigated in order to improve patients' diagnoses.
- Published
- 2023
- Full Text
- View/download PDF
17. GM2 gangliosidosis AB variant: first case of late onset and review of the literature.
- Author
-
Ganne B, Dauriat B, Richard L, Lamari F, Ghorab K, Magy L, Benkirane M, Perani A, Marquet V, Calvas P, Yardin C, and Bourthoumieu S
- Subjects
- Humans, Male, Young Adult, G(M2) Activator Protein genetics, G(M2) Ganglioside metabolism, Gangliosides, Mutation genetics, Gangliosidoses, GM2 genetics
- Abstract
AB variant is the rarest form of GM2 gangliosidosis, neurodegenerative diseases caused by lysosomal accumulation of GM2 gangliosides. Less than thirty cases are referenced in the literature, and to date, no late-onset form has been described. Our proband is a 22-year-old male with spinocerebellar ataxia and lower limbs motor deficiency. His symptoms started at the age of 10. A genetic analysis revealed two mutations in the GM2A gene encoding the GM2 activator protein (GM2-AP), an essential co-factor of hexosaminidase A. Both mutations, GM2A:c.79A > T:p.Lys27* and GM2A:c.415C > T:p.Pro139Ser, were inherited respectively from his father and his mother. The nonsense mutation was predicted to be likely pathogenic, but the missense mutation was of unknown significance. To establish the pathogenicity of this variant, we studied GM2 accumulation and GM2A gene expression. Electron microscopy and immunofluorescence performed on patient's fibroblasts did not reveal any lysosomal accumulation of GM2. There was also no difference in GM2A gene expression using RT-qPCR, and both mutations were found on cDNA Sanger sequencing. Measurement of plasma gangliosides by liquid-phase chromatography-tandem mass spectrometry showed an accumulation of GM2 in our patient's plasma at 83.5 nmol/L, and a GM2/GM3 ratio at 0.066 (median of negative control at 30.2 nmol/L [19.7-46.8] and 0.019 respectively). Therefore, the association of both p.Lys27* and p.Pro169Ser mutations leads to a GM2-AP functional deficiency. Whereas the first mutation is more likely to be linked with infantile form of GM2 gangliosidosis, the hypomorphic p.Pro169Ser variant may be the first associated with a late-onset form of AB variant., (© 2022. Fondazione Società Italiana di Neurologia.)
- Published
- 2022
- Full Text
- View/download PDF
18. CIDP and hemopathies, an underestimated association.
- Author
-
Deschamps N, Mathis S, Duchesne M, Ghorab K, Gallouedec G, Richard L, Boulesteix JM, Corcia P, Magy L, and Vallat JM
- Subjects
- Biopsy, Humans, Peripheral Nerves, Peripheral Nervous System, Retrospective Studies, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating diagnosis, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating epidemiology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating therapy
- Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated and treatable disease that may be associated with various systemic conditions. Our objective is to describe the clinical, electrophysiological and pathological data of a series of patients with both CIDP and hemopathy. In this retrospective study, we analyzed 21 patients with CIDP and various hemopathies (malignant or not), consecutively observed for almost five years. In this particular context (with a risk of neurological complications of the hemopathy), a nerve biopsy was taken from each patient (after written consent). All the patients fulfilled the EAN/PNS electrodiagnostic criteria (2021) of CIDP: 16 with 'CIDP' and 2 with 'possible CIDP' (no data for 3 patients). For each patient, pathological analysis of nerve biopsy was compatible with the diagnosis of CIDP, and there was no evidence for hematological complication of the peripheral nervous system. In cases of peripheral neuropathy and malignant hemopathy, the possibility that the peripheral neuropathy is CIDP should not be overlooked because CIDP is clearly accessible to appropriate therapies, with high potential for a positive clinical response. If the diagnosis of CIDP is usually suspected clinically and electrophysiologically, it should be confirmed by pathological study (nerve biopsy) in certain cases. The management of such patients benefits from the collaboration of neurologists, hematologists and oncologists., (Copyright © 2021 Elsevier B.V. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
19. The Wide Spectrum of Pathophysiologic Mechanisms of Paraproteinemic Neuropathy.
- Author
-
Vallat JM, Duchesne M, Corcia P, Richard L, Ghorab K, Magy L, and Mathis S
- Subjects
- Anemia, Hemolytic, Autoimmune diagnosis, Anemia, Hemolytic, Autoimmune etiology, Anemia, Hemolytic, Autoimmune pathology, Anemia, Hemolytic, Autoimmune physiopathology, Ataxia diagnosis, Ataxia etiology, Ataxia pathology, Ataxia physiopathology, Autoantibodies immunology, Biopsy, Decision Trees, Electrodiagnosis, Humans, Immunoglobulin A, Immunoglobulin G, Immunoglobulin Light-chain Amyloidosis complications, Immunoglobulin Light-chain Amyloidosis diagnosis, Immunoglobulin Light-chain Amyloidosis physiopathology, Immunoglobulin M, Monoclonal Gammopathy of Undetermined Significance, Myelin-Associated Glycoprotein immunology, Neural Conduction physiology, Ophthalmoplegia diagnosis, Ophthalmoplegia etiology, Ophthalmoplegia pathology, Ophthalmoplegia physiopathology, POEMS Syndrome diagnosis, POEMS Syndrome etiology, POEMS Syndrome pathology, POEMS Syndrome physiopathology, Paraproteinemias complications, Paraproteinemias diagnosis, Peripheral Nerves pathology, Peripheral Nerves ultrastructure, Peripheral Nervous System Diseases diagnosis, Peripheral Nervous System Diseases etiology, Peripheral Nervous System Diseases pathology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating diagnosis, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating etiology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating pathology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating physiopathology, Primary Dysautonomias diagnosis, Primary Dysautonomias etiology, Primary Dysautonomias pathology, Primary Dysautonomias physiopathology, Small Fiber Neuropathy diagnosis, Small Fiber Neuropathy etiology, Small Fiber Neuropathy pathology, Small Fiber Neuropathy physiopathology, Waldenstrom Macroglobulinemia, Paraproteinemias physiopathology, Peripheral Nervous System Diseases physiopathology
- Abstract
Monoclonal gammopathy is encountered quite frequently in the general population. This type of hematologic abnormality may be mild, referred to as monoclonal gammopathy of undetermined significance or related to different types of hematologic malignancies. The association of a peripheral neuropathy with monoclonal gammopathy is also fairly common, and hemopathy may be discovered in an investigation of peripheral neuropathy. In such a situation, it is essential to determine the exact nature of the hematologic process in order not to miss a malignant disease and thus initiate the appropriate treatment (in conjunction with hematologists and oncologists). In this respect, nerve biopsy (discussed on a case-by-case basis) is of great value in the management of such patients. We therefore propose to present the objectives and main interests of nerve biopsy in this situation., (© 2020 American Academy of Neurology.)
- Published
- 2021
- Full Text
- View/download PDF
20. A novel pathogenic variant in DYNC1H1 causes various upper and lower motor neuron anomalies.
- Author
-
Viollet LM, Swoboda KJ, Mao R, Best H, Ha Y, Toutain A, Guyant-Marechal L, Laroche-Raynaud C, Ghorab K, Barthez MA, Pedespan JM, Hernandorena X, Lia AS, Deleuze JF, Masson C, Nelson I, Nectoux J, and Si Y
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Female, Heterozygote, Humans, Lower Extremity physiopathology, Male, Middle Aged, Motor Neurons physiology, Muscle, Skeletal physiopathology, Muscular Atrophy, Spinal pathology, Pedigree, Phenotype, Reflex, Upper Extremity physiopathology, Cytoplasmic Dyneins genetics, Muscular Atrophy, Spinal genetics, Mutation, Missense
- Abstract
Objective: To perform genotype-phenotype, clinical and molecular analysis in a large 3-generation family with autosomal dominant congenital spinal muscular atrophy., Methods: Using a combined genetic approach including whole genome scanning, next generation sequencing-based multigene panel, whole genome sequencing, and targeted variant Sanger sequencing, we studied the proband and multiple affected individuals of this family who presented bilateral proximal lower limb muscle weakness and atrophy., Results: We identified a novel heterozygous variant, c.1826T > C; p.Ile609Thr, in the DYNC1H1 gene localized within the common haplotype in the 14q32.3 chromosomal region which cosegregated with disease in this large family. Within the family, affected individuals were found to have a wide array of clinical variability. Although some individuals presented the typical lower motor neuron phenotype with areflexia and denervation, others presented with muscle weakness and atrophy, hyperreflexia, and absence of denervation suggesting a predominant upper motor neuron disease. In addition, some affected individuals presented with an intermediate phenotype characterized by hyperreflexia and denervation, expressing a combination of lower and upper motor neuron defects., Conclusion: Our study demonstrates the wide clinical variability associated with a single disease causing variant in DYNC1H1 gene and this variant demonstrated a high penetrance within this large family., (Copyright © 2020. Published by Elsevier Masson SAS.)
- Published
- 2020
- Full Text
- View/download PDF
21. Hearing loss in inherited peripheral neuropathies: Molecular diagnosis by NGS in a French series.
- Author
-
Lerat J, Magdelaine C, Roux AF, Darnaud L, Beauvais-Dzugan H, Naud S, Richard L, Derouault P, Ghorab K, Magy L, Vallat JM, Cintas P, Bieth E, Arne-Bes MC, Goizet C, Espil-Taris C, Journel H, Toutain A, Urtizberea JA, Boespflug-Tanguy O, Laffargue F, Corcia P, Pasquier L, Fradin M, Napuri S, Ciron J, Boulesteix JM, Sturtz F, and Lia AS
- Subjects
- Adult, Age of Onset, Aged, Aged, 80 and over, Alleles, Computational Biology, Female, France epidemiology, Genetic Testing, Genotype, Hearing Loss epidemiology, High-Throughput Nucleotide Sequencing, Humans, Inheritance Patterns, Male, Middle Aged, Mutation, Pedigree, Peripheral Nervous System Diseases epidemiology, Phenotype, Genetic Association Studies methods, Genetic Predisposition to Disease, Hearing Loss diagnosis, Hearing Loss genetics, Peripheral Nervous System Diseases diagnosis, Peripheral Nervous System Diseases genetics
- Abstract
Background: The most common inherited peripheral neuropathy is Charcot-Marie-Tooth disease (CMT), with a prevalence of 1/2500. Other symptoms can be associated to the condition, such as hearing loss. Currently, no global hearing impairment assessment has been determined, and the physiopathology is not well known., Methods: The aim of the study was to analyze among a French series of 3,412 patients with inherited peripheral neuropathy (IPN), the ones who also suffer from hearing loss, to establish phenotype-genotype correlations. An NGS strategy for IPN one side and nonsyndromic hearing loss (NSHL) on the other side, were performed., Results: Hearing loss (HL) was present in only 44 patients (1.30%). The clinical data of 27 patients were usable. Demyelinating neuropathy was diagnosed in 15 cases and axonal neuropathy in 12 cases. HL varied from mild to profound. Five cases of auditory neuropathy were noticed. Diagnosis was made for 60% of these patients. Seven novel pathogenic variants were discovered in five different genes: PRPS1; MPZ; SH3TC2; NEFL; and ABHD12. Two patients with PMP22 variant, had also an additional variant in COCH and MYH14 respectively. No pathogenic variant was found at the DFNB1 locus. Genotype-phenotype correlations do exist, especially with SH3TC2, PRPS1, ABHD12, NEFL, and TRPV4., Conclusion: Involvement of PMP22 is not enough to explain hearing loss in patients suffering from IPN. HL can be due to cochlear impairment and/or auditory nerve dysfunction. HL is certainly underdiagnosed, and should be evaluated in every patient suffering from IPN., (© 2019 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)
- Published
- 2019
- Full Text
- View/download PDF
22. A novel pathogenic variant of NEFL responsible for deafness associated with peripheral neuropathy discovered through next-generation sequencing and review of the literature.
- Author
-
Lerat J, Magdelaine C, Beauvais-Dzugan H, Espil C, Ghorab K, Latour P, Derouault P, Sturtz F, and Lia AS
- Subjects
- Aged, Female, High-Throughput Nucleotide Sequencing, Humans, Review Literature as Topic, Charcot-Marie-Tooth Disease genetics, Hearing Loss, Sensorineural genetics, Neurofilament Proteins genetics
- Abstract
Neurofilaments are neuron-specific intermediate filaments essential for the radial growth of axons during development and the maintenance of axonal diameter. Pathogenic variants of Neurofilament Light (NEFL) are associated with CMT1F, CMT2E, and CMTDIG and have been observed in less than 1% of Charcot-Marie-Tooth (CMT) cases, resulting in the reporting of 35 variants in 173 CMT patients to date. However, only six variants have been reported in 17 patients with impaired hearing. No genotype-phenotype correlations have yet been established. Here, we report an additional case: a 69-year-old female, who originally presented with axonal sensory and motor neuropathy at the age of 45, associated with moderate sensorineural hearing loss, with a slight slope at high frequencies. Next-generation sequencing identified a novel pathogenic variant: c.269A > G, p.(Glu90Gly). Hearing impairment is often linked to CMT due to pathogenic variants of NEFL, especially p.(Glu90Lys) and p.(Asn98Ser), and in our case p.(Glu90Gly). These pathogenic variants are all located at hot spots, in the head domain and the two ends of the rod domain of the protein., (© 2019 Peripheral Nerve Society.)
- Published
- 2019
- Full Text
- View/download PDF
23. Some new proposals for the classification of inherited myopathies.
- Author
-
Mathis S, Tazir M, Solé G, Magy L, Le Masson G, Couratier P, Ghorab K, Duval F, Lacoste I, Goizet C, and Vallat JM
- Subjects
- Humans, Muscular Diseases classification, Muscular Diseases genetics
- Published
- 2018
- Full Text
- View/download PDF
24. Value of nerve biopsy in the management of peripheral neuropathies.
- Author
-
Mathis S, Magy L, Le Masson G, Richard L, Soulages A, Solé G, Duval F, Ghorab K, Vallat JM, and Duchesne M
- Subjects
- Humans, Peripheral Nervous System Diseases complications, Peripheral Nervous System Diseases diagnosis, Vasculitis complications, Biopsy, Peripheral Nervous System Diseases etiology, Peripheral Nervous System Diseases pathology
- Abstract
Introduction: Peripheral neuropathy is a common symptom throughout the population, with numerous possible etiologies. The diagnosis of peripheral neuropathies (and their causes) is mainly based on clinical, electrophysiological, biological, and imaging features. Areas covered: This paper reviews the main causes of neuropathy and discusses the usefulness of nerve biopsy (NB) in such cases. Expert commentary: In most cases, NB is not mandatory in the diagnostic work-up of a peripheral neuropathy. However, NB is clearly an indication in cases of vasculitis. It is also valuable in peripheral neuropathies with severe and rapid worsening (without clear cause) in order to uncover a pathological hallmark (amyloid deposits). Although NB is considered an invasive method, it may be useful in the management of peripheral neuropathy, especially to guide treatment in certain cases. In summary, although NB is not a systematic procedure, it is a useful tool that should be discussed on a case-by-case basis within the clinical context.
- Published
- 2018
- Full Text
- View/download PDF
25. History and current difficulties in classifying inherited myopathies and muscular dystrophies.
- Author
-
Mathis S, Tazir M, Magy L, Duval F, Le Masson G, Duchesne M, Couratier P, Ghorab K, Solé G, Lacoste I, Goizet C, and Vallat JM
- Subjects
- History, 19th Century, History, 20th Century, History, 21st Century, Humans, Muscular Diseases genetics, Muscular Diseases history, Muscular Diseases physiopathology, Muscular Diseases classification
- Abstract
The wide spectrum of hereditary muscular disorders leads to unavoidable difficulties in their classification, even for specialists. For this reason, new proposals are required that would ultimately replace our current rather complex classifications by a simpler structure. Our proposal will be limited to dystrophic and non-dystrophic myopathies (excluding metabolic disorders, mitochondriopathies, and channelopathies) for which similar proposals would also be relevant. Various genes (encoding structural proteins associated with the sarcolemma, nuclear membrane proteins, and proteins involved in myofiber metabolism have now been sequenced and mutations ascribed to specific forms of inherited muscular disorders. Based on our observations and our recent proposals in other neurogenetic conditions and informal discussions with specialists of neuromuscular disorders, the prerequisite for a simple and sound classification for inherited muscular disorders should encompass the clinical and pathological phenotypes (described in a simple and clear manner), the mode of inheritance, and the mutated gene. We think that the denomination of the different subtypes could be simplified considerably, although any new proposal of classification of muscular disorders will need to be discussed in the neurological and genetic communities., (Copyright © 2017 Elsevier B.V. All rights reserved.)
- Published
- 2018
- Full Text
- View/download PDF
26. Erratum to: An exploratory randomised double-blind and placebo-controlled phase 2 study of a combination of baclofen, naltrexone and sorbitol (PXT3003) in patients with Charcot-Marie-Tooth disease type 1A.
- Author
-
Attarian S, Vallat JM, Magy L, Funalot B, Gonnaud PM, Lacour A, Péréon Y, Dubourg O, Pouget J, Micallef J, Franques J, Lefebvre MN, Ghorab K, Al-Moussawi M, Tiffreau V, Preudhomme M, Magot A, Leclair-Visonneau L, Stojkovic T, Bossi L, Lehert P, Gilbert W, Bertrand V, Mandel J, Milet A, Hajj R, Boudiaf L, Scart-Grès C, Nabirotchkin S, Guedj M, Chumakov I, and Cohen D
- Published
- 2016
- Full Text
- View/download PDF
27. Variations in multiple sclerosis practice within Europe - Is it time for a new treatment guideline?
- Author
-
Marziniak M, Ghorab K, Kozubski W, Pfleger C, Sousa L, Vernon K, Zaffaroni M, and Meuth SG
- Subjects
- Europe, Humans, Multiple Sclerosis diagnosis, Practice Patterns, Physicians', Healthcare Disparities, Multiple Sclerosis therapy, Practice Guidelines as Topic
- Abstract
In the past 5 years, the combination of developments in diagnostic strategy and approval of new disease-modifying therapies has provided an opportunity to achieve dramatic improvements in patient outcomes in multiple sclerosis (MS). However, across Europe there are several factors that may prevent patients from receiving the best therapy at the appropriate time, and there is variation among countries in terms of which of these factors are most relevant. Here, we review current MS clinical practices in a number of countries in the European Union to identify differences regarding initiation of treatment in patients with clinically isolated syndrome or relapsing-remitting MS, and differences in the timing of treatment switch or escalation. While recognizing that policy is not static in any country, we believe that patients' interests would be better served if a European treatment guideline was developed. Such a guideline could both inform and be informed by national policies, facilitating the dissemination of best clinical practice internationally., (Copyright © 2016 Elsevier B.V. All rights reserved.)
- Published
- 2016
- Full Text
- View/download PDF
28. Gender as a Modifying Factor Influencing Myotonic Dystrophy Type 1 Phenotype Severity and Mortality: A Nationwide Multiple Databases Cross-Sectional Observational Study.
- Author
-
Dogan C, De Antonio M, Hamroun D, Varet H, Fabbro M, Rougier F, Amarof K, Arne Bes MC, Bedat-Millet AL, Behin A, Bellance R, Bouhour F, Boutte C, Boyer F, Campana-Salort E, Chapon F, Cintas P, Desnuelle C, Deschamps R, Drouin-Garraud V, Ferrer X, Gervais-Bernard H, Ghorab K, Laforet P, Magot A, Magy L, Menard D, Minot MC, Nadaj-Pakleza A, Pellieux S, Pereon Y, Preudhomme M, Pouget J, Sacconi S, Sole G, Stojkovich T, Tiffreau V, Urtizberea A, Vial C, Zagnoli F, Caranhac G, Bourlier C, Riviere G, Geille A, Gherardi RK, Eymard B, Puymirat J, Katsahian S, and Bassez G
- Subjects
- Adult, Cross-Sectional Studies, Female, Humans, Male, Myotonic Dystrophy mortality, Sex Distribution, Socioeconomic Factors, Databases, Factual, Myotonic Dystrophy epidemiology, Phenotype
- Abstract
Background: Myotonic Dystrophy type 1 (DM1) is one of the most heterogeneous hereditary disease in terms of age of onset, clinical manifestations, and severity, challenging both medical management and clinical trials. The CTG expansion size is the main factor determining the age of onset although no factor can finely predict phenotype and prognosis. Differences between males and females have not been specifically reported. Our aim is to study gender impact on DM1 phenotype and severity., Methods: We first performed cross-sectional analysis of main multiorgan clinical parameters in 1409 adult DM1 patients (>18 y) from the DM-Scope nationwide registry and observed different patterns in males and females. Then, we assessed gender impact on social and economic domains using the AFM-Téléthon DM1 survey (n = 970), and morbidity and mortality using the French National Health Service Database (n = 3301)., Results: Men more frequently had (1) severe muscular disability with marked myotonia, muscle weakness, cardiac, and respiratory involvement; (2) developmental abnormalities with facial dysmorphism and cognitive impairment inferred from low educational levels and work in specialized environments; and (3) lonely life. Alternatively, women more frequently had cataracts, dysphagia, digestive tract dysfunction, incontinence, thyroid disorder and obesity. Most differences were out of proportion to those observed in the general population. Compared to women, males were more affected in their social and economic life. In addition, they were more frequently hospitalized for cardiac problems, and had a higher mortality rate., Conclusion: Gender is a previously unrecognized factor influencing DM1 clinical profile and severity of the disease, with worse socio-economic consequences of the disease and higher morbidity and mortality in males. Gender should be considered in the design of both stratified medical management and clinical trials.
- Published
- 2016
- Full Text
- View/download PDF
29. Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome.
- Author
-
Mathis S, Magy L, Corcia P, Ghorab K, Richard L, Ciron J, Duchesne M, and Vallat JM
- Abstract
Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies). For each patient, we carried out a pathological analysis (nerve and muscle) and an electrophysiological study (and follow-up). To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders., Competing Interests: The authors declare that they have no competing interests.
- Published
- 2016
- Full Text
- View/download PDF
30. Natalizumab as a Disease-Modifying Therapy in Chronic Inflammatory Demyelinating Polyneuropathy - A Report of Three Cases.
- Author
-
Vallat JM, Mathis S, Ghorab K, Milor MA, Richard L, and Magy L
- Subjects
- Adult, Aged, Female, Humans, Male, Middle Aged, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating pathology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating physiopathology, Immunologic Factors therapeutic use, Natalizumab therapeutic use, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating drug therapy
- Abstract
Background: Several treatments are available to treat the immune-mediated chronic inflammatory demyelinating polyneuropathy (CIDP). Among these treatments, intravenous immunoglobulins, corticosteroids and plasma exchanges are validated and widely used. A few immunosuppressive drugs have been tried, but they had little efficiency., Methods: We describe three CIDP patients treated by Natalizumab (acting against cellular adhesion and T-cell migration) after a failure of the validated treatments., Results: We observed a long-term improvement in one patient, a dramatic improvement over a significant duration in another patient and stabilization in the last one., Conclusion: This open label study provides evidence for the value of Natalizumab as second-line treatment for individual patients with a high dependency on waning efficacy of first-line therapies. CIDP is characterized by heterogeneity of clinical phenotypes, electrophysiological and pathological features, and various variable courses types of evolution. The different responses to drugs of our patients are consistent with some reported cases and may reflect the spectrum of lesional mechanisms and the molecular dysfunctions in CIDP., (© 2015 S. Karger AG, Basel.)
- Published
- 2015
- Full Text
- View/download PDF
31. Value of nerve biopsy in patients with latent malignant hemopathy and peripheral neuropathy: a case series.
- Author
-
Duchesne M, Mathis S, Corcia P, Richard L, Ghorab K, Jaccard A, Magy L, and Vallat JM
- Subjects
- Aged, Biopsy, Female, Hematologic Neoplasms pathology, Humans, Leukemia, Lymphocytic, Chronic, B-Cell diagnosis, Leukemia, Lymphocytic, Chronic, B-Cell pathology, Leukemia, Lymphocytic, Chronic, B-Cell therapy, Lymphoma, B-Cell diagnosis, Lymphoma, B-Cell pathology, Lymphoma, B-Cell therapy, Lymphoma, Non-Hodgkin diagnosis, Lymphoma, Non-Hodgkin pathology, Lymphoma, Non-Hodgkin therapy, Male, Middle Aged, Peripheral Nervous System Diseases pathology, Disease Management, Hematologic Neoplasms diagnosis, Hematologic Neoplasms therapy, Peripheral Nerves pathology, Peripheral Nervous System Diseases diagnosis
- Abstract
Hematological malignancies include several diseases that may affect the peripheral nervous system (PNS) through various mechanisms. A common and challenging situation is represented by the occurrence of an active peripheral neuropathy in a patient with a supposed inactive hematological disorder.We report clinical, electrophysiological, biological, and pathological data of 8 patients with latent malignant hemopathies (most were considered in remission): B-cell chronic lymphocytic leukemia in 3 patients, B-cell lymphoma in 1 patient, low-grade non-Hodgkin's lymphoma in 1 patient, Waldenström's macroglobulinemia in 1 patient, smoldering multiple myeloma in 1 patient, and monoclonal gammopathy of undetermined significance in 1 patient.In all these cases, the nerve biopsy (NB) helped to diagnose the hematological relapse or detect a pathological mechanism linked to the hematological disorder: epineurial lymphocytic infiltration in 5 patients (including one with antimyelin-associated glycoprotein antibodies), cryoglobulin deposits in 1 patient, chronic inflammatory demyelinating polyneuropathy in 1 patient, and necrotizing vasculitis in 1 patient. In each case, pathological findings were crucial to select the adequate treatment, leading to an improvement in the neurological and biological manifestations.These observations illustrate the value of NB and the need for active collaboration between neurologists and hematologists in such cases.
- Published
- 2015
- Full Text
- View/download PDF
32. Heterogeneity of Polyneuropathy Associated with Anti-MAG Antibodies.
- Author
-
Magy L, Kaboré R, Mathis S, Lebeau P, Ghorab K, Caudie C, and Vallat JM
- Subjects
- Aged, Aged, 80 and over, Female, Humans, Immunoglobulin M immunology, Male, Middle Aged, Myelin Sheath immunology, Peripheral Nerves immunology, Retrospective Studies, Antibodies, Monoclonal immunology, Autoantibodies immunology, Myelin-Associated Glycoprotein immunology, Polyneuropathies immunology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating immunology
- Abstract
Polyneuropathy associated with IgM monoclonal gammopathy and anti-myelin associated glycoprotein (MAG) antibodies is an immune-mediated demyelinating neuropathy. The pathophysiology of this condition is likely to involve anti-MAG antibody deposition on myelin sheaths of the peripheral nerves and it is supposed to be distinct from chronic inflammatory demyelinating neuropathy (CIDP), another immune-mediated demyelinating peripheral neuropathy. In this series, we have retrospectively reviewed clinical and laboratory findings from 60 patients with polyneuropathy, IgM gammopathy, and anti-MAG antibodies. We found that the clinical picture in these patients is highly variable suggesting a direct link between the monoclonal gammopathy and the neuropathy. Conversely, one-third of patients had a CIDP-like phenotype on electrodiagnostic testing and this was correlated with a low titer of anti-MAG antibodies and the absence of widening of myelin lamellae. Our data suggest that polyneuropathy associated with anti-MAG antibodies is less homogeneous than previously said and that the pathophysiology of the condition is likely to be heterogeneous as well with the self-antigen being MAG in most of the patients but possibly being another component of myelin in the others.
- Published
- 2015
- Full Text
- View/download PDF
33. An exploratory randomised double-blind and placebo-controlled phase 2 study of a combination of baclofen, naltrexone and sorbitol (PXT3003) in patients with Charcot-Marie-Tooth disease type 1A.
- Author
-
Attarian S, Vallat JM, Magy L, Funalot B, Gonnaud PM, Lacour A, Péréon Y, Dubourg O, Pouget J, Micallef J, Franques J, Lefebvre MN, Ghorab K, Al-Moussawi M, Tiffreau V, Preudhomme M, Magot A, Leclair-Visonneau L, Stojkovic T, Bossi L, Lehert P, Gilbert W, Bertrand V, Mandel J, Milet A, Hajj R, Boudiaf L, Scart-Grès C, Nabirotchkin S, Guedj M, Chumakov I, and Cohen D
- Subjects
- Adult, Double-Blind Method, Drug Therapy, Combination, Female, Humans, Male, Middle Aged, Baclofen administration & dosage, Charcot-Marie-Tooth Disease diagnosis, Charcot-Marie-Tooth Disease drug therapy, Naltrexone administration & dosage, Sorbitol administration & dosage
- Abstract
Background: Charcot-Marie-Tooth type 1A disease (CMT1A) is a rare orphan inherited neuropathy caused by an autosomal dominant duplication of a gene encoding for the structural myelin protein PMP22, which induces abnormal Schwann cell differentiation and dysmyelination, eventually leading to axonal suffering then loss and muscle wasting. We favour the idea that diseases can be more efficiently treated when targeting multiple disease-relevant pathways. In CMT1A patients, we therefore tested the potential of PXT3003, a low-dose combination of three already approved compounds (baclofen, naltrexone and sorbitol). Our study conceptually builds on preclinical experiments highlighting a pleiotropic mechanism of action that includes downregulation of PMP22. The primary objective was to assess safety and tolerability of PXT3003. The secondary objective aimed at an exploratory analysis of efficacy of PXT3003 in CMT1A, to be used for designing next clinical development stages (Phase 2b/3)., Methods: 80 adult patients with mild-to-moderate CMT1A received in double-blind for 1 year Placebo or one of the three increasing doses of PXT3003 tested, in four equal groups. Safety and tolerability were assessed with the incidence of related adverse events. Efficacy was assessed using the Charcot-Marie-Tooth Neuropathy Score (CMTNS) and the Overall Neuropathy Limitations Scale (ONLS) as main endpoints, as well as various clinical and electrophysiological outcomes., Results: This trial confirmed the safety and tolerability of PXT3003. The highest dose (HD) showed consistent evidence of improvement beyond stabilization. CMTNS and ONLS, with a significant improvement of respectively of 8% (0.4% - 16.2%) and 12.1% (2% - 23.2%) in the HD group versus the pool of all other groups, appear to be the most sensitive clinical endpoints to treatment despite their quasi-stability over one year under Placebo. Patients who did not deteriorate over one year were significantly more frequent in the HD group., Conclusions: These results confirm that PXT3003 deserves further investigation in adults and could greatly benefit CMT1A-diagnosed children, usually less affected than adults., Trial Registration: EudraCT Number: 2010-023097-40. ClinicalTrials.gov Identifier: NCT01401257. The Committee for Orphan Medicinal Products issued in February 2014 a positive opinion on the application for orphan designation for PXT3003 (EMA/OD/193/13).
- Published
- 2014
- Full Text
- View/download PDF
34. Constitutive activation of the calcium sensor STIM1 causes tubular-aggregate myopathy.
- Author
-
Böhm J, Chevessier F, Maues De Paula A, Koch C, Attarian S, Feger C, Hantaï D, Laforêt P, Ghorab K, Vallat JM, Fardeau M, Figarella-Branger D, Pouget J, Romero NB, Koch M, Ebel C, Levy N, Krahn M, Eymard B, Bartoli M, and Laporte J
- Subjects
- Adolescent, Adult, Aged, Amino Acid Sequence, Animals, Base Sequence, Cell Line, Child, Female, Homeostasis, Humans, Male, Membrane Proteins chemistry, Membrane Proteins genetics, Mice, Middle Aged, Molecular Sequence Data, Muscles pathology, Muscles ultrastructure, Mutation genetics, Myoblasts metabolism, Myoblasts pathology, Myopathies, Structural, Congenital genetics, Neoplasm Proteins chemistry, Neoplasm Proteins genetics, Pedigree, Phenotype, Stromal Interaction Molecule 1, Young Adult, Calcium metabolism, Membrane Proteins metabolism, Myopathies, Structural, Congenital pathology, Neoplasm Proteins metabolism
- Abstract
Tubular aggregates are regular arrays of membrane tubules accumulating in muscle with age. They are found as secondary features in several muscle disorders, including alcohol- and drug-induced myopathies, exercise-induced cramps, and inherited myasthenia, but also exist as a pure genetic form characterized by slowly progressive muscle weakness. We identified dominant STIM1 mutations as a genetic cause of tubular-aggregate myopathy (TAM). Stromal interaction molecule 1 (STIM1) is the main Ca(2+) sensor in the endoplasmic reticulum, and all mutations were found in the highly conserved intraluminal Ca(2+)-binding EF hands. Ca(2+) stores are refilled through a process called store-operated Ca(2+) entry (SOCE). Upon Ca(2+)-store depletion, wild-type STIM1 oligomerizes and thereby triggers extracellular Ca(2+) entry. In contrast, the missense mutations found in our four TAM-affected families induced constitutive STIM1 clustering, indicating that Ca(2+) sensing was impaired. By monitoring the calcium response of TAM myoblasts to SOCE, we found a significantly higher basal Ca(2+) level in TAM cells and a dysregulation of intracellular Ca(2+) homeostasis. Because recessive STIM1 loss-of-function mutations were associated with immunodeficiency, we conclude that the tissue-specific impact of STIM1 loss or constitutive activation is different and that a tight regulation of STIM1-dependent SOCE is fundamental for normal skeletal-muscle structure and function., (Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)
- Published
- 2013
- Full Text
- View/download PDF
35. Impact of 6-hour sepsis resuscitation bundle compliance on hospital mortality in a saudi hospital.
- Author
-
Memon JI, Rehmani RS, Alaithan AM, El Gammal A, Lone TM, Ghorab K, and Abdulbasir A
- Abstract
Purpose. To assess the effect of improved compliance with 6-hour sepsis resuscitation bundle on mortality in patients with severe sepsis and septic shock. Materials and Methods. A quasi-experimental prospective study was conducted at a 10-bedded combined medical and surgical intensive care unit. The historical group included all consecutive patients with severe sepsis and septic shock admitted from January 2008 to March 2009. Intervention included evidence-based written sepsis pathway, antibiotic recommendations, and an educational program.The post-intervention group included all consecutive patients admitted from July 2009 to June 2011. The primary outcome measures were the overall compliance to seven 6-hour sepsis resuscitation bundle elements and 30-day hospital mortality. There were 99 patients in the historical group and 199 in the post-intervention group. Results. The baseline patients' characteristics were similar. Overall compliance to all seven sepsis resuscitation bundle elements in historical group was 5.1% [95% confidence interval (CI), 2.1-11.3] which improved after intervention to 23.6% (95% CI, 17.9-30.1); P < 0.001. The overall compliance to 6-hour sepsis resuscitation bundle elements was associated with improved survival [odds ratio (OR), 5.8 (95% CI, 2.2-15.1; P < 0.001)]. 30-day hospital mortality reduced from 31.3% in the historical group to 21.1% in the intervention group; P = 0.05. Conclusion. Improvement in compliance to 6-hour sepsis resuscitation bundle was associated with a reduction in 30-day hospital mortality.
- Published
- 2012
- Full Text
- View/download PDF
36. [Small fibre neuropathy in primary Sjögren syndrome].
- Author
-
Fauchais AL, Richard L, Gondran G, Ghorab K, Palat S, Bezanahary H, Loustaud-Ratti V, Ly K, Jauberteau MO, Vallat JM, Vidal E, and Magy L
- Subjects
- Chronic Disease, Female, Humans, Middle Aged, Neuralgia etiology, Neuralgia pathology, Sjogren's Syndrome complications
- Abstract
Purpose: About forty percent of the patients with primary Sjögren's syndrome (pSS) experience chronic neuropathic pain with normal electrodiagnostic studies. Two previous studies suggest that chronic neuropathic pain in pSS is due to small fiber neuropathy (SFN). Quantification of epidermal nerve fiber density after skin biopsy has been validated to diagnose small fiber neuropathy., Methods: Skin biopsy was performed in 14 consecutive pSS patients (satisfying the american-european classification criteria) with chronic neuropathic pain and normal electrodiagnostic studies suggesting SFN., Results: Fourteen female pSS patients exhibited chronic neuropathic pain [burning sensation (n=14), prickling (n=4), dysesthesia (n=8)] with paroxystic exacerbations (n=10) and allodynia (n=13), for a mean period of 18.4±12.4 months. Neuropathic pain involved mostly hands and feet (n=13), with a distal (n=9) and leg (n=4) predominant distribution. Neurological examination disclosed normal deep tendon responses and absence of motor weakness (n=14). Small fiber neuropathy was confirmed by skin biopsy in 13 cases. Epidermal nerve fiber density was decreased in distal [(n=12), mean 3.5±1.7 fibers/mm (N>6.9)] and proximal site of biopsy [(n=9), mean 7.04±2.63 fibers/mm (N>9.3)]., Conclusion: Small fiber neuropathy is commonly responsible of chronic neuropathic pain in pSS. Prevalence, physiopathology and neurological evolution of such neuropathies still remain unknown., (Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)
- Published
- 2011
- Full Text
- View/download PDF
37. Genotype-phenotype correlations in Charcot-Marie-Tooth disease type 2 caused by mitofusin 2 mutations.
- Author
-
Calvo J, Funalot B, Ouvrier RA, Lazaro L, Toutain A, De Mas P, Bouche P, Gilbert-Dussardier B, Arne-Bes MC, Carrière JP, Journel H, Minot-Myhie MC, Guillou C, Ghorab K, Magy L, Sturtz F, Vallat JM, and Magdelaine C
- Subjects
- Adolescent, Adult, Aged, Charcot-Marie-Tooth Disease classification, Child, Child, Preschool, Female, GTP Phosphohydrolases, Genes, Dominant, Genes, Recessive, Genetic Markers genetics, Genotype, Humans, Male, Middle Aged, Severity of Illness Index, Young Adult, Charcot-Marie-Tooth Disease genetics, Charcot-Marie-Tooth Disease pathology, Membrane Proteins genetics, Mitochondrial Proteins genetics, Mutation, Missense genetics, Phenotype
- Abstract
Background: Mutations in the gene encoding mitofusin 2 (MFN2) cause Charcot-Marie-Tooth disease type 2 (CMT2), with heterogeneity concerning severity and associated clinical features., Objective: To describe MFN2 mutations and associated phenotypes in patients with hereditary motor and sensory neuropathy (HMSN)., Design: Direct sequencing of the MFN2 gene and clinical investigations of patients with MFN2 mutations., Setting: Molecular genetics laboratory of a university hospital and the Limoges National Referral Center for Rare Peripheral Neuropathies., Patients: One hundred fifty index patients with HMSN and a median motor nerve conduction velocity of 25 m/s or greater and without mutations in the genes encoding connexin 32 and myelin protein zero., Main Outcome Measures: Results of genetic analyses and phenotypic observations., Results: Twenty different missense mutations were identified in 20 index patients. Mutation frequency was 19 of 107 (17.8%) in patients with CMT2 and 1 of 43 (2.3%) in patients with a median motor nerve conduction velocity less than 38 m/s. Four patients had proven de novo mutations, 8 families had autosomal dominant inheritance, and 3 had autosomal recessive inheritance. The remaining 5 patients were sporadic cases with heterozygous mutations. Phenotypes varied from mild forms to early-onset severe forms. Additional features were encountered in 8 patients (32%). Six patients underwent sural nerve biopsy: electronic microscopy showed prominent mitochondrial abnormalities on longitudinal sections., Conclusions: MFN2 mutations are a frequent cause of CMT2, with variable severity and either dominant or recessive inheritance. MFN2 gene testing must be a first-line analysis in axonal HMSN irrespective of the mode of inheritance or the severity of the peripheral neuropathy.
- Published
- 2009
- Full Text
- View/download PDF
38. [Hereditary neuropathies].
- Author
-
Vallat JM, Calvo J, Ghorab K, and Tazir M
- Subjects
- Amyloid Neuropathies, Familial diagnosis, Axons physiology, Charcot-Marie-Tooth Disease classification, Charcot-Marie-Tooth Disease diagnosis, Chromosomes, Human, X genetics, Demyelinating Diseases genetics, Fabry Disease diagnosis, Genes, Dominant genetics, Genes, Recessive genetics, Humans, Porphyrias diagnosis, Hereditary Sensory and Motor Neuropathy diagnosis
- Abstract
Although there are many human hereditary neuropathies, most of them with the exception of Charcot-Marie-Tooth disease or hereditary sensorimotor neuropathy, are rare. Irrespective of their type, the mode of transmission may be autosomal dominant or recessive, or X-linked. The most difficult to diagnose, however, are the sporadic forms. It is customary to distinguish the cases in which the neuropathy is the sole clinical expression from multisystemic diseases where neuropathy is one component of multi-organ involvement. The complexity and the multiplicity of genes involved and the lack of understanding of their exact functions hinder logical presentation of these hereditary neuropathies. For understandable technical reasons, the stage of specific treatment, namely the repair of the mutated gene, has yet to be attained.
- Published
- 2008
39. Intranervous immunoglobulin deposits: an underestimated mechanism of neuropathy.
- Author
-
Vallat JM, Magy L, Richard L, Piaser M, Sindou P, Calvo J, Ghorab K, and Cros D
- Subjects
- Adult, Aged, Biopsy, Cell Count, Cryoglobulins metabolism, Electrodiagnosis, Electrophysiology, Female, Fluorescent Antibody Technique, Humans, Male, Microscopy, Immunoelectron, Middle Aged, Motor Neurons metabolism, Motor Neurons pathology, Nerve Fibers, Myelinated pathology, Neural Conduction physiology, Neurons, Afferent metabolism, Neurons, Afferent pathology, Paraproteinemias metabolism, Paraproteinemias pathology, Immunoglobulins metabolism, Nerve Tissue metabolism, Nerve Tissue pathology, Polyneuropathies metabolism, Polyneuropathies pathology
- Abstract
There are several pathogenic mechanisms of peripheral nerve involvement in patients with monoclonal dysglobulinemia. Intranervous proliferation of malignant cells, immunoglobulin, or amyloid deposits in the endoneurial space can only be determined by examination of nerve biopsy specimens. We present clinical, electrophysiological, and histological data from seven patients whose polyneuropathy was induced by immunoglobulin deposits in the endoneurial space. As these lesions cannot be demonstrated on clinical and electrophysiological grounds, the indication for nerve biopsy derives from careful analysis of each patient presenting with a polyneuropathy and a monoclonal dysglobulinemia. To visualize and clearly characterize these deposits, electron microscopic examination is indispensable. Immunocytochemical methods using both light and electron microscopy for ultrastructural analysis are of great value. Demonstration of endoneurial immunoglobulin deposits may have major therapeutic consequences. Indeed, identification of these deposits prompted the use of aggressive treatment, which was quite effective in five of our seven patients., ((c) 2008 Wiley Periodicals, Inc.)
- Published
- 2008
- Full Text
- View/download PDF
40. Carotid angioplasty stenting revisited: clinical and radiological (MRI) outcome.
- Author
-
Ghorab K, Macian F, Adoukounou T, Magy L, Chapot R, and Vallat JM
- Subjects
- Aged, Aged, 80 and over, Carotid Stenosis diagnosis, Carotid Stenosis etiology, Cohort Studies, Contraindications, Diffusion Magnetic Resonance Imaging, Endarterectomy, Carotid, Female, Humans, Male, Middle Aged, Risk Factors, Treatment Outcome, Angioplasty, Carotid Stenosis therapy, Stents
- Abstract
Background: Although carotid angioplasty and stenting (CAS) is widely used to treat carotid stenosis, recent studies point to the inferiority of the procedure compared with carotid endarterectomy., Methods: We present 50 consecutive cases of CAS treated in our unit. Endarterectomy was contraindicated in these patients due to high operative risk. All the patients underwent a diffusion-weighted MRI (DWI) before and after the procedure and had a neurological assessment in a stroke unit., Results: No deaths were recorded until 30 days after the procedure. Six patients [12%, confidence interval at 95% (CI(95)) = 3.7-20.2] had a positive DWI MRI after the procedure but only 2 (4%, CI(95) = 0-9.43) had a worse neurological status., Conclusion: This study shows that CAS is feasible with a low morbid-mortality rate in patients with a high surgical risk. DWI is highly sensitive to detect neurological complications after the procedure., ((c) 2007 S. Karger AG, Basel.)
- Published
- 2008
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.