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102. Clinical phenotype, fibrinogen supplementation, and health-related quality of life in patients with afibrinogenemia

Author

Casini, Alessandro, von Mackensen, Sylvia, Santoro, Cristina, Djambas Khayat, Claudia, Belhani, Meriem, Ross, Cecil, Dorgalaleh, Akbar, Naz, Arshi, Ünal, Ekrem, Abdelwahab, Magy, Dupuis Lozeron, Elise, Trillot, Nathalie, Susen, Sophie, Peyvandi, Flora, De Moerloose, Philippe, and QualyAfib Study Group

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Multivariate analysis, Settore MED/09 - Medicina Interna, Adolescent, Health-related quality of life, Immunology, Hemorrhage, 030204 cardiovascular system & hematology, Fibrinogen, Biochemistry, 03 medical and health sciences, 0302 clinical medicine, Afibrinogenemia, Age Factors, Cerebral Hemorrhage, Child, Child, Preschool, Factor VIII, Female, Humans, Middle Aged, Risk Factors, Thrombosis, Quality of Life, Quality of life, Medicine, Thrombus, Concentrate dosage form, Preschool, ddc:616, ddc:618, business.industry, Cell Biology, Hematology, Bleed, medicine.disease, Institutional repository, Phenotype, business, 030215 immunology, medicine.drug
Abstract

Due to the low prevalence of afibrinogenemia, epidemiologic data on afibrinogenemia are limited, and no data are available on health-related quality of life (HRQoL). We conducted a cross-sectional international study to characterize the clinical features, the fibrinogen supplementation modalities, and their impact on HRQoL in patients with afibrinogenemia. A total of 204 patients (119 adults and 85 children) from 25 countries were included. The bleeding phenotype was severe: 68 (33.3%) patients having at least one bleed per month and 48 (23%) a history of cerebral bleeding. About 35% (n = 72) of patients were treated with fibrinogen concentrates or cryoprecipitates as prophylaxis, 18.1% (n = 37) received ≥1 injection per week, and 16.6% (n = 34) were on home treatment. A thrombotic event was reported in venous and/or arterial territories by 37 (18.1%) patients. Thrombosis occurred even in young patients, and recurrence was frequent (7.4%). The total HRQoL was lower in children than in adults. Discomfort linked to treatment and limitations to sports and leisure were the main concerns. Women and children were particularly affected in family relationships. In multivariate analyses, younger age, residence in Asia or Africa, and a previous thrombotic event were statistically correlated with a worse HRQoL. In summary, our study underlines the severe bleeding and thrombotic phenotype and their impact on HRQoL in afibrinogenemia. The optimal strategy for fibrinogen supplementation needs to be determined. This trial was registered at www.clinicaltrials.gov as #NCT03484065.

Published
2021

103. Is low dose Tranexamic acid less effective than a standard dose at reducing blood loss and inhibiting hyperfibrinolysis in hemorrhagic caesarean section? Multicenter double–blind placebo-controlled dose-ranging (TRACES) trial.

Author

Ducloy-Bouthors, Anne-Sophie, primary, Gilliot, Sixtine, additional, Kyheng, Maeva, additional, Faraoni, David, additional, Turbelin, Alexandre, additional, Keita, Hawa, additional, Rigouzzo, Agnes, additional, Moyanotidou, Gabirela, additional, Constant, Benjamin, additional, Broisin, Francoise, additional, Le-Gouez, Agnes, additional, Favier, Remi, additional, Peynaud, Edith, additional, Ghesquiere, louise, additional, Lebuffe, Gilles, additional, Duhamel, Alain, additional, Allorge, Delphine, additional, Susen, Sophie, additional, Hennart, Benjamin, additional, Jeanpierre, Emmanuelle, additional, and Odou, Pascal, additional

Published
2021
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104. Comparison of Fibrin Monomers and D-dimers to predict thrombotic events in critically ill patients with COVID-19 pneumonia: A retrospective study

Author

Godon, Alexandre, primary, Durand, Zoé, additional, Agier, Lydiane, additional, Lecompte, Thomas, additional, Mullier, François, additional, Marlu, Raphaël, additional, de Maistre, Emmanuel, additional, Tacquard, Charles, additional, Levy, Jerrold H., additional, Godier, Anne, additional, Susen, Sophie, additional, and Albaladejo, Pierre, additional

Published
2021
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105. Pharmacokinetics of enoxaparin in COVID-19 critically ill patients

Author

Zufferey, Paul Jacques, primary, Dupont, Annabelle, additional, Lanoiselée, Julien, additional, Bauters, Anne, additional, Poissy, Julien, additional, Goutay, Julien, additional, Jean, Laurent, additional, Caplan, Morgan, additional, Levy, Lionel, additional, Susen, Sophie, additional, and Delavenne, Xavier, additional

Published
2021
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106. Optogenetic and chemogenetic insights into the neurocircuitry of depression‐like behaviour: A systematic review

Author

Tom Biselli, Johannes Steffen, Andreas Walther, Susen Sophie Lange, and Lynn Sablottny

Subjects
Depressive Disorder, Major, 0303 health sciences, Depression, General Neuroscience, Hippocampus, Chemogenetics, Optogenetics, Nucleus accumbens, Amygdala, Nucleus Accumbens, Ventral tegmental area, 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, Reward, medicine, Animals, Prefrontal cortex, Psychology, Neuroscience, 030217 neurology & neurosurgery, Anterior cingulate cortex, 030304 developmental biology
Abstract

Major depressive disorder (MDD) and its treatment are challenges for global health. Optogenetics and chemogenetics are driving MDD research forward by unveiling causal relations between cell-type-specific control of neurons and depressive-like behaviour in rodents. Using a systematic search process, in this review, a set of 43 original studies applying optogenetic or chemogenetic techniques in rodent models of depression was identified. Our aim was to provide an examination of all available studies elucidating central neuronal mechanisms leading to depressive-like behaviour in rodents and thereby unveiling the most promising routes for future research. A complex interacting network of relevant structures, in which central circuitries causally related to depressive-like behaviour are implicated, has been identified. As most relevant structures emerge: medial prefrontal cortex, anterior cingulate cortex, amygdala, nucleus accumbens, ventral tegmental area, hippocampus and raphe nuclei. Further evidence, though examined by only few studies, emerges for structures like the lateral habenula, or medial dorsal thalamus. Most of the identified brain areas have previously been associated with MDD neuropathology, but now evidence can be provided for causal pathological mechanisms within a complex cortico-limbic reward circuitry. However, the studies also show conflicting results concerning the mechanisms underlying the causal involvement of specific circuitries. Comparability of studies is partly limited since even small deviations in methodological approaches lead to different outcomes. Factors influencing study outcomes were identified and need to be considered in future studies (e.g. frequency used for stimulation, time and duration of stimulation, limitations of applied animal models of MDD).

Published
2019

107. Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

Author

Rauch, Antoine, Paris, Camille, Repesse, Yohann, Branche, Julien, D’Oiron, Roseline, Harroche, Annie, Ternisien, Catherine, Castet, Sabine‐Marie, Lebreton, Aurélien, Pan‐Petesch, Brigitte, Volot, Fabienne, Claeyssens, Segolene, Chamouni, Pierre, Gay, Valérie, Berger, Claire, Desprez, Dominique, Falaise, Céline, Biron Andreani, Christine, Marichez, Catherine, Pradines, Benedicte, Zawadzki, Christophe, Itzhar Baikian, Nathalie, Borel‐Derlon, Annie, Goudemand, Jenny, Gerard, Romain, and Susen, Sophie

Published
2021
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111. Prevention of venous thromboembolism and haemostasis monitoring in patients with COVID-19: Updated proposals (April 2021)

Author

Godon, Alexandre, primary, Tacquard, Charles Ambroise, additional, Mansour, Alexandre, additional, Garrigue, Delphine, additional, Nguyen, Philippe, additional, Lasne, Dominique, additional, Testa, Sophie, additional, Levy, Jerrold H., additional, Albaladejo, Pierre, additional, Gruel, Yves, additional, Susen, Sophie, additional, Godier, Anne, additional, Albaladejo, P., additional, Blais, N., additional, Bonhomme, F., additional, Borel-Derlon, A., additional, Cohen, A., additional, Collet, J.-P., additional, de Maistre, E., additional, Fontana, P., additional, Garrigue Huet, D., additional, Godier, A., additional, Gruel, Y., additional, Godon, A., additional, Ickx, B., additional, Laporte, S., additional, Lasne, D., additional, Llau, J., additional, Le Gal, G., additional, Lecompte, T., additional, Lessire, S., additional, Levy, J.H., additional, Longrois, D., additional, Madi-Jebara, S., additional, Mansour, A., additional, Mazighi, M., additional, Mismetti, P., additional, Morange, P.E., additional, Motte, S., additional, Mullier, F., additional, Nathan, N., additional, Nguyen, P., additional, Pernod, G., additional, Rosencher, N., additional, Roullet, S., additional, Roy, P.M., additional, Schlumberger, S., additional, Sié, P., additional, Steib, A., additional, Susen, S., additional, Tacquard, C.A., additional, Testa, S., additional, Vincentelli, A., additional, Zufferey, P., additional, Boissier, E, additional, Dumont, B, additional, James, C, additional, and Siguret, V., additional

Published
2021
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112. Response

Author

Tacquard, Charles, primary, Godon, Alexandre, additional, Mansour, Alexandre, additional, Gruel, Yves, additional, Susen, Sophie, additional, and Godier, Anne, additional

Published
2021
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113. Hypotheses behind the very rare cases of thrombosis with thrombocytopenia syndrome after SARS-CoV-2 vaccination

Author

Douxfils, Jonathan, primary, Favresse, Julien, additional, Dogné, Jean-Michel, additional, Lecompte, Thomas, additional, Susen, Sophie, additional, Cordonnier, Charlotte, additional, Lebreton, Aurélien, additional, Gosselin, Robert, additional, Sié, Pierre, additional, Pernod, Gilles, additional, Gruel, Yves, additional, Nguyen, Philippe, additional, Vayne, Caroline, additional, and Mullier, François, additional

Published
2021
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114. Impact of High-Dose Prophylactic Anticoagulation in Critically Ill Patients With COVID-19 Pneumonia

Author

Tacquard, Charles, primary, Mansour, Alexandre, additional, Godon, Alexandre, additional, Godet, Julien, additional, Poissy, Julien, additional, Garrigue, Delphine, additional, Kipnis, Eric, additional, Rym Hamada, Sophie, additional, Mertes, Paul Michel, additional, Steib, Annick, additional, Ulliel-Roche, Mathilde, additional, Bouhemad, Bélaïd, additional, Nguyen, Maxime, additional, Reizine, Florian, additional, Gouin-Thibault, Isabelle, additional, Besse, Marie Charlotte, additional, Collercandy, Nived, additional, Mankikian, Stefan, additional, Levy, Jerrold H., additional, Gruel, Yves, additional, Albaladejo, Pierre, additional, Susen, Sophie, additional, Godier, Anne, additional, Albaladejo, P., additional, Blais, N., additional, Bonhomme, F., additional, Borel-Derlon, A., additional, Cohen, A., additional, Collet, J.-P., additional, de Maistre, E., additional, Fontana, P., additional, Garrigue Huet, D., additional, Godier, A., additional, Gruel, Y., additional, Godon, A., additional, Ickx, B., additional, Laporte, S., additional, Lasne, D., additional, Llau, J., additional, Le Gal, G., additional, Lecompte, T., additional, Lessire, S., additional, Levy, J.H., additional, Longrois, D., additional, Madi-Jebara, S., additional, Mansour, A., additional, Mazighi, M., additional, Mismetti, P., additional, Morange, P.E., additional, Motte, S., additional, Mullier, F., additional, Nathan, N., additional, Nguyen, P., additional, Pernod, G., additional, Rosencher, N., additional, Roullet, S., additional, Roy, P.M., additional, Schlumberger, S., additional, Sié, P., additional, Steib, A., additional, Susen, S., additional, Tacquard, C.A., additional, Testa, S., additional, Vincentelli, A., additional, and Zufferey, P., additional

Published
2021
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115. Autoantibodies neutralizing type I IFNs are present in similar to 4% of uninfected individuals over 70 years old and account for similar to 20% of COVID-19 deaths

Author

Bastard, Paul, Gervais, Adrian, Le Voyer, Tom, Rosain, Jeremie, Philippot, Quentin, Manry, Jeremy, Michailidis, Eleftherios, Hoffmann, Hans-Heinrich, Eto, Shohei, Garcia-Prat, Marina, Bizien, Lucy, Parra-Martinez, Alba, Yang, Rui, Haljasmagi, Liis, Migaud, Melanie, Sarekannu, Karita, Maslovskaja, Julia, de Prost, Nicolas, Tandjaoui-Lambiotte, Yacine, Luyt, Charles-Edouard, Amador-Borrero, Blanca, Gaudet, Alexandre, Poissy, Julien, Morel, Pascal, Richard, Pascale, Cognasse, Fabrice, Troya, Jesus, Trouillet-Assant, Sophie, Belot, Alexandre, Saker, Kahina, Garcon, Pierre, Riviere, Jacques G., Lagier, Jean-Christophe, Gentile, Stephanie, Rosen, Lindsey B., Shaw, Elana, Morio, Tomohiro, Tanaka, Junko, Dalmau, David, Tharaux, Pierre-Louis, Sene, Damien, Stepanian, Alain, Megarbane, Bruno, Triantafyllia, Vasiliki, Fekkar, Arnaud, Heath, James R., Franco, Jose Luis, Anaya, Juan-Manuel, Sole-Violan, Jordi, Imberti, Luisa, Biondi, Andrea, Bonfanti, Paolo, Castagnoli, Riccardo, Delmonte, Ottavia M., Zhang, Yu, Snow, Andrew L., Holland, Steven M., Biggs, Catherine M., Moncada-Velez, Marcela, Arias, Andres Augusto, Lorenzo, Lazaro, Boucherit, Soraya, Coulibaly, Boubacar, Anglicheau, Dany, Planas, Anna M., Haerynck, Filomeen, Duvlis, Sotirija, Nussbaum, Robert L., Ozcelik, Tayfun, Keles, Sevgi, Bousfiha, Ahmed A., El Bakkouri, Jalila, Ramirez-Santana, Carolina, Paul, Stephane, Pan-Hammarstrom, Qiang, Hammarstrom, Lennart, Dupont, Annabelle, Kurolap, Alina, Metz, Christine N., Aiuti, Alessandro, Casari, Giorgio, Lampasona, Vito, Ciceri, Fabio, Barreiros, Lucila A., Dominguez-Garrido, Elena, Vidigal, Mateus, Zatz, Mayana, van de Beek, Diederik, Sahanic, Sabina, Tancevski, Ivan, Stepanovskyy, Yurii, Boyarchuk, Oksana, Nukui, Yoko, Tsumura, Miyuki, Vidaur, Loreto, Tangye, Stuart G., Burrel, Sonia, Duffy, Darragh, Quintana-Murci, Lluis, Klocperk, Adam, Kann, Nelli Y., Shcherbina, Anna, Lau, Yu-Lung, Leung, Daniel, Coulongeat, Matthieu, Marlet, Julien, Koning, Rutger, Reyes, Luis Felipe, Chauvineau-Grenier, Angelique, Venet, Fabienne, Monneret, Guillaume, Nussenzweig, Michel C., Arrestier, Romain, Boudhabhay, Idris, Baris-Feldman, Hagit, Hagin, David, Wauters, Joost, Meyts, Isabelle, Dyer, Adam H., Kennelly, Sean P., Bourke, Nollaig M., Halwani, Rabih, Sharif-Askari, Narjes Saheb, Dorgham, Karim, Sallette, Jerome, Sedkaoui, Souad Mehlal, AlKhater, Suzan, Rigo-Bonnin, Raul, Morandeira, Francisco, Roussel, Lucie, Vinh, Donald C., Ostrowski, Sisse Rye, Condino-Neto, Antonio, Prando, Carolina, Bondarenko, Anastasiia, Spaan, Andras N., Gilardin, Laurent, Fellay, Jacques, Lyonnet, Stanislas, Bilguvar, Kaya, Lifton, Richard P., Mane, Shrikant, Anderson, Mark S., Boisson, Bertrand, Beziat, Vivien, Zhang, Shen-Ying, Andreakos, Evangelos, Hermine, Olivier, Pujol, Aurora, Peterson, Part, Mogensen, Trine H., Rowen, Lee, Mond, James, Debette, Stephanie, de Lamballerie, Xavier, Duval, Xavier, Mentre, France, Zins, Marie, Soler-Palacin, Pere, Colobran, Roger, Gorochov, Guy, Solanich, Xavier, Susen, Sophie, Martinez-Picado, Javier, Raoult, Didier, Vasse, Marc, Gregersen, Peter K., Piemonti, Lorenzo, Rodriguez-Gallego, Carlos, Notarangelo, Luigi D., Su, Helen C., Kisand, Kai, Okada, Satoshi, Puel, Anne, Jouanguy, Emmanuelle, Rice, Charles M., Tiberghien, Pierre, Zhang, Qian, Cobat, Aurelie, Abel, Laurent, Casanova, Jean-Laurent, and Gunst, Jan

Subjects
INTERFERON, alpha, Science & Technology, ANTINUCLEAR, myasthenia-gravis patients, AUTOIMMUNITY, autoimmunity, Immunology, interferon, IMMUNITY, immunity, DISTINCT FUNCTIONS, PATIENT, ALPHA, ANTIBODIES, antibodies, patient, distinct functions, Life Sciences & Biomedicine, MYASTHENIA-GRAVIS PATIENTS, antinuclear
Abstract

Circulating autoantibodies (auto-Abs) neutralizing high concentrations (10 ng/mL, in plasma diluted 1 to 10) of IFN-α and/or -ω are found in about 10% of patients with critical COVID-19 pneumonia, but not in subjects with asymptomatic infections. We detect auto-Abs neutralizing 100-fold lower, more physiological, concentrations of IFN-α and/or -ω (100 pg/mL, in 1/10 dilutions of plasma) in 13.6% of 3,595 patients with critical COVID-19, including 21% of 374 patients > 80 years, and 6.5% of 522 patients with severe COVID-19. These antibodies are also detected in 18% of the 1,124 deceased patients (aged 20 days-99 years; mean: 70 years). Moreover, another 1.3% of patients with critical COVID-19 and 0.9% of the deceased patients have auto-Abs neutralizing high concentrations of IFN-β. We also show, in a sample of 34,159 uninfected subjects from the general population, that auto-Abs neutralizing high concentrations of IFN-α and/or -ω are present in 0.18% of individuals between 18 and 69 years, 1.1% between 70 and 79 years, and 3.4% >80 years. Moreover, the proportion of subjects carrying auto-Abs neutralizing lower concentrations is greater in a subsample of 10,778 uninfected individuals: 1% of individuals 80 years. By contrast, auto-Abs neutralizing IFN-β do not become more frequent with age. Auto-Abs neutralizing type I IFNs predate SARS-CoV-2 infection and sharply increase in prevalence after the age of 70 years. They account for about 20% of both critical COVID-19 cases in the over-80s, and total fatal COVID-19 cases. ispartof: SCIENCE IMMUNOLOGY vol:6 issue:62 ispartof: location:United States status: published

Published
2021

117. Characteristics and Outcomes of Patients with Cerebral Venous Sinus Thrombosis in SARS-CoV-2 Vaccine-Induced Immune Thrombotic Thrombocytopenia

Author

Sánchez Van Kammen, Mayte, Aguiar De Sousa, Diana, Poli, Sven, Cordonnier, Charlotte, Heldner, Mirjam R., Van De Munckhof, Anita, Krzywicka, Katarzyna, Van Haaps, Thijs, Ciccone, Alfonso, Middeldorp, Saskia, Levi, Marcel M., Kremer Hovinga, Johanna A., Silvis, Suzanne, Hiltunen, Sini, Mansour, Maryam, Arauz, Antonio, Barboza, Miguel A., Field, Thalia S., Tsivgoulis, Georgios, Nagel, Simon, Lindgren, Erik, Tatlisumak, Turgut, Jood, Katarina, Putaala, Jukka, Ferro, Jose M., Arnold, Marcel, Coutinho, Jonathan M., Sharma, Aarti R., Elkady, Ahmed, Negro, Alberto, Günther, Albrecht, Gutschalk, Alexander, Schönenberger, Silvia, Buture, Alina, Murphy, Sean, Paiva Nunes, Ana, Tiede, Andreas, Puthuppallil Philip, Anemon, Mengel, Annerose, Medina, Antonio, Hellström Vogel, Åslög, Tawa, Audrey, Aujayeb, Avinash, Casolla, Barbara, Buck, Brian, Zanferrari, Carla, Garcia-Esperon, Carlos, Vayne, Caroline, Legault, Catherine, Pfrepper, Christian, Tracol, Clement, Soriano, Cristina, Guisado-Alonso, Daniel, Bougon, David, Zimatore, Domenico S., Michalski, Dominik, Blacquiere, Dylan, Johansson, Elias, Cuadrado-Godia, Elisa, De Maistre, Emmanuel, Carrera, Emmanuel, Vuillier, Fabrice, Bonneville, Fabrice, Giammello, Fabrizio, Bode, Felix J., Zimmerman, Julian, D'Onofrio, Florindo, Grillo, Francesco, Cotton, Francois, Caparros, François, Puy, Laurent, Maier, Frank, Gulli, Giosue, Frisullo, Giovanni, Polkinghorne, Gregory, Franchineau, Guillaume, Cangür, Hakan, Katzberg, Hans, Sibon, Igor, Baharoglu, Irem, Brar, Jaskiran, Payen, Jean-François, Burrow, Jim, Fernandes, João, Schouten, Judith, Althaus, Katharina, Garambois, Katia, Derex, Laurent, Humbertjean, Lisa, Lebrato Hernandez, Lucia, Kellermair, Lukas, Morin Martin, Mar, Petruzzellis, Marco, Cotelli, Maria, Dubois, Marie-Cécile, Carvalho, Marta, Wittstock, Matthias, Miranda, Miguel, Skjelland, Mona, Bandettini Di Poggio, Monica, Scholz, Moritz J., Raposo, Nicolas, Kahnis, Robert, Kruyt, Nyika, Huet, Olivier, Sharma, Pankaj, Candelaresi, Paolo, Reiner, Peggy, Vieira, Ricardo, Acampora, Roberto, Kern, Rolf, Leker, Ronen, Coutts, Shelagh, Bal, Simerpreet, Sharma, Shyam S., Susen, Sophie, Cox, Thomas, Geeraerts, Thomas, Gattringer, Thomas, Bartsch, Thorsten, Kleinig, Timothy J., Dizonno, Vanessa, Arslan, Yildiz, Sánchez Van Kammen, Mayte, Aguiar De Sousa, Diana, Poli, Sven, Cordonnier, Charlotte, Heldner, Mirjam R., Van De Munckhof, Anita, Krzywicka, Katarzyna, Van Haaps, Thijs, Ciccone, Alfonso, Middeldorp, Saskia, Levi, Marcel M., Kremer Hovinga, Johanna A., Silvis, Suzanne, Hiltunen, Sini, Mansour, Maryam, Arauz, Antonio, Barboza, Miguel A., Field, Thalia S., Tsivgoulis, Georgios, Nagel, Simon, Lindgren, Erik, Tatlisumak, Turgut, Jood, Katarina, Putaala, Jukka, Ferro, Jose M., Arnold, Marcel, Coutinho, Jonathan M., Sharma, Aarti R., Elkady, Ahmed, Negro, Alberto, Günther, Albrecht, Gutschalk, Alexander, Schönenberger, Silvia, Buture, Alina, Murphy, Sean, Paiva Nunes, Ana, Tiede, Andreas, Puthuppallil Philip, Anemon, Mengel, Annerose, Medina, Antonio, Hellström Vogel, Åslög, Tawa, Audrey, Aujayeb, Avinash, Casolla, Barbara, Buck, Brian, Zanferrari, Carla, Garcia-Esperon, Carlos, Vayne, Caroline, Legault, Catherine, Pfrepper, Christian, Tracol, Clement, Soriano, Cristina, Guisado-Alonso, Daniel, Bougon, David, Zimatore, Domenico S., Michalski, Dominik, Blacquiere, Dylan, Johansson, Elias, Cuadrado-Godia, Elisa, De Maistre, Emmanuel, Carrera, Emmanuel, Vuillier, Fabrice, Bonneville, Fabrice, Giammello, Fabrizio, Bode, Felix J., Zimmerman, Julian, D'Onofrio, Florindo, Grillo, Francesco, Cotton, Francois, Caparros, François, Puy, Laurent, Maier, Frank, Gulli, Giosue, Frisullo, Giovanni, Polkinghorne, Gregory, Franchineau, Guillaume, Cangür, Hakan, Katzberg, Hans, Sibon, Igor, Baharoglu, Irem, Brar, Jaskiran, Payen, Jean-François, Burrow, Jim, Fernandes, João, Schouten, Judith, Althaus, Katharina, Garambois, Katia, Derex, Laurent, Humbertjean, Lisa, Lebrato Hernandez, Lucia, Kellermair, Lukas, Morin Martin, Mar, Petruzzellis, Marco, Cotelli, Maria, Dubois, Marie-Cécile, Carvalho, Marta, Wittstock, Matthias, Miranda, Miguel, Skjelland, Mona, Bandettini Di Poggio, Monica, Scholz, Moritz J., Raposo, Nicolas, Kahnis, Robert, Kruyt, Nyika, Huet, Olivier, Sharma, Pankaj, Candelaresi, Paolo, Reiner, Peggy, Vieira, Ricardo, Acampora, Roberto, Kern, Rolf, Leker, Ronen, Coutts, Shelagh, Bal, Simerpreet, Sharma, Shyam S., Susen, Sophie, Cox, Thomas, Geeraerts, Thomas, Gattringer, Thomas, Bartsch, Thorsten, Kleinig, Timothy J., Dizonno, Vanessa, and Arslan, Yildiz

Abstract

Importance: Thrombosis with thrombocytopenia syndrome (TTS) has been reported after vaccination with the SARS-CoV-2 vaccines ChAdOx1 nCov-19 (Oxford-AstraZeneca) and Ad26.COV2.S (Janssen/Johnson & Johnson). Objective: To describe the clinical characteristics and outcome of patients with cerebral venous sinus thrombosis (CVST) after SARS-CoV-2 vaccination with and without TTS. Design, Setting, and Participants: This cohort study used data from an international registry of consecutive patients with CVST within 28 days of SARS-CoV-2 vaccination included between March 29 and June 18, 2021, from 81 hospitals in 19 countries. For reference, data from patients with CVST between 2015 and 2018 were derived from an existing international registry. Clinical characteristics and mortality rate were described for adults with (1) CVST in the setting of SARS-CoV-2 vaccine-induced immune thrombotic thrombocytopenia, (2) CVST after SARS-CoV-2 vaccination not fulling criteria for TTS, and (3) CVST unrelated to SARS-CoV-2 vaccination. Exposures: Patients were classified as having TTS if they had new-onset thrombocytopenia without recent exposure to heparin, in accordance with the Brighton Collaboration interim criteria. Main Outcomes and Measures: Clinical characteristics and mortality rate. Results: Of 116 patients with postvaccination CVST, 78 (67.2%) had TTS, of whom 76 had been vaccinated with ChAdOx1 nCov-19; 38 (32.8%) had no indication of TTS. The control group included 207 patients with CVST before the COVID-19 pandemic. A total of 63 of 78 (81%), 30 of 38 (79%), and 145 of 207 (70.0%) patients, respectively, were female, and the mean (SD) age was 45 (14), 55 (20), and 42 (16) years, respectively. Concomitant thromboembolism occurred in 25 of 70 patients (36%) in the TTS group, 2 of 35 (6%) in the no TTS group, and 10 of 206 (4.9%) in the control group, and in-hospital mortality rates were 47% (36 of 76; 95% CI, 37-58), 5% (2 of 37; 95% CI, 1-18), and 3.9% (8 of 207; 95% CI

Published
2021
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118. Viscoelastometric Testing to Assess Hemostasis of COVID-19: A Systematic Review.

Author

UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Laboratoire de biologie clinique, UCL - (MGD) Service d'anesthésiologie, Bareille, Marion, Hardy, Michaël, Douxfils, Jonathan, Roullet, Stéphanie, Lasne, Dominique, Levy, Jerrold H, Stépanian, Alain, Susen, Sophie, Frère, Corinne, Lecompte, Thomas, Mullier, François, UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Laboratoire de biologie clinique, UCL - (MGD) Service d'anesthésiologie, Bareille, Marion, Hardy, Michaël, Douxfils, Jonathan, Roullet, Stéphanie, Lasne, Dominique, Levy, Jerrold H, Stépanian, Alain, Susen, Sophie, Frère, Corinne, Lecompte, Thomas, and Mullier, François

Abstract

Infection by SARS-CoV-2 is associated with a high risk of thrombosis. The laboratory documentation of hypercoagulability and impaired fibrinolysis remains a challenge. Our aim was to assess the potential usefulness of viscoelastometric testing (VET) to predict thrombotic events in COVID-19 patients according to the literature. We also (i) analyzed the impact of anticoagulation and the methods used to neutralize heparin, (ii) analyzed whether maximal clot mechanical strength brings more information than Clauss fibrinogen, and (iii) critically scrutinized the diagnosis of hypofibrinolysis. We performed a systematic search in PubMed and Scopus databases until 31st December 2020. VET methods and parameters, and patients' features and outcomes were extracted. VET was performed for 1063 patients (893 intensive care unit (ICU) and 170 non-ICU, 44 studies). There was extensive heterogeneity concerning study design, VET device used (ROTEM, TEG, Quantra and ClotPro) and reagents (with non-systematic use of heparin neutralization), timing of assay, and definition of hypercoagulable state. Notably, only 4 out of 25 studies using ROTEM reported data with heparinase (HEPTEM). The common findings were increased clot mechanical strength mainly due to excessive fibrinogen component and impaired to absent fibrinolysis, more conspicuous in the presence of an added plasminogen activator. Only 4 studies out of the 16 that addressed the point found an association of VETs with thrombotic events. So-called functional fibrinogen assessed by VETs showed a variable correlation with Clauss fibrinogen. Abnormal VET pattern, often evidenced despite standard prophylactic anticoagulation, tended to normalize after increased dosing. VET studies reported heterogeneity, and small sample sizes do not support an association between the poorly defined prothrombotic phenotype of COVID-19 and thrombotic events.

Published
2021

119. Hypotheses behind the very rare cases of thrombosis with thrombocytopenia syndrome after SARS-CoV-2 vaccination.

Author

UCL - (MGD) Laboratoire de biologie clinique, UCL - SSS/IREC/MONT - Pôle Mont Godinne, Douxfils, Jonathan, Favresse, Julien, Dogné, Jean-Michel, Lecompte, Thomas, Susen, Sophie, Cordonnier, Charlotte, Lebreton, Aurélien, Gosselin, Robert, Sié, Pierre, Pernod, Gilles, Gruel, Yves, Nguyen, Philippe, Vayne, Caroline, Mullier, François, UCL - (MGD) Laboratoire de biologie clinique, UCL - SSS/IREC/MONT - Pôle Mont Godinne, Douxfils, Jonathan, Favresse, Julien, Dogné, Jean-Michel, Lecompte, Thomas, Susen, Sophie, Cordonnier, Charlotte, Lebreton, Aurélien, Gosselin, Robert, Sié, Pierre, Pernod, Gilles, Gruel, Yves, Nguyen, Philippe, Vayne, Caroline, and Mullier, François

Abstract

As of 4 April 2021, a total of 169 cases of cerebral venous sinus thrombosis (CVST) and 53 cases of splanchnic vein thrombosis were reported to EudraVigilance among around 34 million people vaccinated in the European Economic Area and United Kingdom with COVID-19 Vaccine AstraZeneca, a chimpanzee adenoviral vector (ChAdOx1) encoding the spike protein antigen of the SARS-CoV-2 virus. The first report of the European Medicines Agency gathering data on 20 million people vaccinated with Vaxzevria® in the UK and the EEA concluded that the number of post-vaccination cases with thromboembolic events as a whole reported to EudraVigilance in relation to the number of people vaccinated was lower than the estimated rate of such events in the general population. However, the EMA's Pharmacovigilance Risk Assessment Committee concluded that unusual thromboses with low blood platelets should be listed as very rare side effects of Vaxzevria®, pointing to a possible link. The same issue was identified with the COVID-19 Vaccine Janssen (Ad26.COV2.S). Currently, there is still a sharp contrast between the clinical or experimental data reported in the literature on COVID-19 and the scarcity of data on the unusual thrombotic events observed after the vaccination with these vaccines. Different hypotheses might support these observations and should trigger further in vitro and ex vivo investigations. Specialized studies were needed to fully understand the potential relationship between vaccination and possible risk factors in order to implement risk minimization strategies.

Published
2021

120. Comparison of Fibrin Monomers and D-dimers to predict thrombotic events in critically ill patients with COVID-19 pneumonia: A retrospective study.

Author

UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Laboratoire de biologie clinique, Godon, Alexandre, Durand, Zoé, Agier, Lydiane, Lecompte, Thomas, Mullier, François, Marlu, Raphaël, de Maistre, Emmanuel, Tacquard, Charles, Levy, Jerrold H, Godier, Anne, Susen, Sophie, Albaladejo, Pierre, UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Laboratoire de biologie clinique, Godon, Alexandre, Durand, Zoé, Agier, Lydiane, Lecompte, Thomas, Mullier, François, Marlu, Raphaël, de Maistre, Emmanuel, Tacquard, Charles, Levy, Jerrold H, Godier, Anne, Susen, Sophie, and Albaladejo, Pierre

Published
2021

124. ADAMTS13inhibition to treat acquired von Willebrand syndrome during mechanical circulatory support device implantation

Author

Deconinck, Shannen J., Nix, Christoph, Barth, Svenja, Bennek‐Schöpping, Eveline, Rauch, Antoine, Schelpe, An‐Sofie, Roose, Elien, Feys, Hendrik B., Pareyn, Inge, Vandenbulcke, Aline, Muia, Joshua, Vandenbriele, Christophe, Susen, Sophie, Meyns, Bart, Tersteeg, Claudia, Jacobs, Steven, De Meyer, Simon F., and Vanhoorelbeke, Karen

Abstract

Acquired von Willebrand syndrome (aVWS) is common in patients with mechanical circulatory support (MCS) devices. In these patients, the high shear stress in the device leads to increased shear‐induced proteolysis of von Willebrand factor (VWF) by A Disintegrin And Metalloprotease with Thrombospondin type 1 repeats, number 13 (ADAMTS13). As a result, the high molecular weight (HMW) VWF multimers are lost, leading to a decreased VWF function and impaired hemostasis that could explain the bleeding complications that are frequently observed in these patients. To counteract this abnormal VWF degradation by ADAMTS13, we developed a novel targeted therapy, using an anti‐ADAMTS13 monoclonal antibody (mAb) that inhibits the shear‐induced proteolysis of VWF by ADAMTS13. Human or bovine blood was circulated through in vitroMCS device systems with either inhibitory anti‐ADAMTS13 mAb 3H9 or 17C7 (20 μg/ml) or control anti‐ADAMTS13 mAb 5C11 or phosphate buffered saline (PBS). VWF multimers and function (collagen binding activity) were determined at different time points. Next, Impella pumps were implanted in calves and the calves were injected with PBS and subsequently treated with mAb 17C7. VWF, ADAMTS13, and blood parameters were determined. We demonstrated that blocking ADAMTS13 could prevent the loss of HMW VWF multimers in in vitroMCS device systems. Importantly, our antibody could reverse aVWS in a preclinical Impella‐induced aVWS calf model. Hence, inhibition of ADAMTS13 could become a novel therapeutic strategy to manage aVWS in MCS device patients.

Published
2022
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125. PF4 Immunoassays in Vaccine-Induced Thrombotic Thrombocytopenia

Author

Vayne, Caroline, primary, Rollin, Jérôme, additional, Gruel, Yves, additional, Pouplard, Claire, additional, Galinat, Hubert, additional, Huet, Olivier, additional, Mémier, Vincent, additional, Geeraerts, Thomas, additional, Marlu, Raphael, additional, Pernod, Gilles, additional, Mourey, Guillaume, additional, Fournel, Alexandra, additional, Cordonnier, Charlotte, additional, and Susen, Sophie, additional

Published
2021
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126. Evaluation of Anti-Activated Factor X Activity and Activated Partial Thromboplastin Time Relations and Their Association with Bleeding and Thrombosis during Veno-Arterial ECMO Support: A Retrospective Study

Author

Moussa, Mouhamed Djahoum, primary, Soquet, Jérôme, additional, Lamer, Antoine, additional, Labreuche, Julien, additional, Gantois, Guillaume, additional, Dupont, Annabelle, additional, Abou-Arab, Osama, additional, Rousse, Natacha, additional, Liu, Vincent, additional, Brandt, Caroline, additional, Foulon, Valentin, additional, Leroy, Guillaume, additional, Schurtz, Guillaume, additional, Jeanpierre, Emmanuel, additional, Duhamel, Alain, additional, Susen, Sophie, additional, Vincentelli, André, additional, and Robin, Emmanuel, additional

Published
2021
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130. Management of von Willebrand disease with a factor VIII‐poor von Willebrand factor concentrate: Results from a prospective observational post‐marketing study

Author

Itzhar‐baïkian, Nathalie, Borel‐derlon, Annie, Goudemand, Jenny, Bridey, Françoise, Claeyssens, Segolene, Itzhar-Baikian, Nathalie, Harroche, Annie, Desprez, Dominique, Negrier, Claude, Chamouni, Pierre, Chambost, Hervé, Henriet, Céline, Susen, Sophie, Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Effilux, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service d'hématologie biologique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service d'immuno-hématologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Département d'Oncologie et Hématologie [Strasbourg], Les Hôpitaux Universitaires de Strasbourg (HUS), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Laboratoire d'hématologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Assistance Publique - Hôpitaux de Marseille (APHM), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), CHU Toulouse [Toulouse], and Lucas, Nelly

Subjects
[SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology, medicine.medical_specialty, 030204 cardiovascular system & hematology, von Willebrand factor, 03 medical and health sciences, 0302 clinical medicine, Von Willebrand factor, post‐marketing, Internal medicine, Breakthrough bleeding, hemic and lymphatic diseases, Von Willebrand disease, Humans, Medicine, Childbirth, Prospective Studies, post-marketing, Marketing, Thrombotic risk, biology, business.industry, HAEMOSTASIS, clinical trial, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, Original Articles, medicine.disease, 3. Good health, Clinical trial, von Willebrand Diseases, Tolerability, factor VIII, biology.protein, Female, Observational study, Original Article, France, medicine.symptom, business, von Willebrand disease
Abstract

International audience; Background A triple-secured plasma-derived von Willebrand factor (pdVWF) almost devoid of factor VIII (FVIII):WILFACTIN(R), was approved in France in 2003, and then in other countries for the treatment of patients with von Willebrand disease (VWD). Objective To investigate long-term safety and efficacy of the product in real-life over the first 5 post-approval years. Patients/Methods This prospective, observational, national post-marketing study (PMS) enrolled patients of all ages and VWD types. Patients were observed for up to 3 years and treated for one or more occasions. Efficacy was assessed for each major event. Breakthrough bleeding rate 3 days post-infusion and annualized bleeding rate (ABR) were also evaluated for long-term prophylaxis. Results Overall, 155 of 174 patients enrolled from 31 centers were eligible for efficacy assessment. Most patients (76.8%) were severely affected (VWF:RCo = 12 months. Excellent tolerability was confirmed with no safety concerns. No thrombotic events were observed. Conclusions Results from this PMS increase the clinical experience of a FVIII-poor pdVWF in patients of all ages and VWD types including those with thrombotic risk factors and emphasize that giving FVIII is not always mandatory to effectively treat patients with severe VWD.

Published
2020

137. Pulmonary Embolism and Deep Vein Thrombosis in COVID-19: A Systematic Review and Meta-Analysis

Author

Suh, Young Joo, primary, Hong, Hyunsook, additional, Ohana, Mickaël, additional, Bompard, Florian, additional, Revel, Marie-Pierre, additional, Valle, Clarissa, additional, Gervaise, Alban, additional, Poissy, Julien, additional, Susen, Sophie, additional, Hékimian, Guillaume, additional, Artifoni, Mathieu, additional, Periard, Daniel, additional, Contou, Damien, additional, Delaloye, Julie, additional, Sanchez, Bienvenido, additional, Fang, Cheng, additional, Garzillo, Giorgio, additional, Robbie, Hasti, additional, and Yoon, Soon Ho, additional

Published
2021
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138. The homozygous variant p.Gln1311* in exon 28 of VWF is associated with the development of alloantibodies in 3 unrelated patients with type 3 VWD

Author

Lassalle, Fanny, primary, Zawadzki, Christophe, additional, Harroche, Annie, additional, Biron‐Andréani, Christine, additional, Falaise, Céline, additional, Boisseau, Pierre, additional, Duployez, Nicolas, additional, Jeanpierre, Emmanuelle, additional, Rauch, Antoine, additional, Paris, Camille, additional, Susen, Sophie, additional, and Goudemand, Jenny, additional

Published
2021
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140. Acquired Hemophilia A in IgG4-Related Disease: Case Report, Immunopathogenic Study, and Review of the Literature

Author

Sanges, Sébastien, primary, Jeanpierre, Emmanuelle, additional, Lopez, Benjamin, additional, Russick, Jules, additional, Delignat, Sandrine, additional, Carpentier, Benjamin, additional, Dubois, Romain, additional, Dubucquoi, Sylvain, additional, Guerrier, Thomas, additional, Hachulla, Éric, additional, Hatron, Pierre-Yves, additional, Paris, Camille, additional, Susen, Sophie, additional, Launay, David, additional, Lacroix-Desmazes, Sébastien, additional, and Terriou, Louis, additional

Published
2020
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142. Acquired von Willebrand syndrome in aortic stenosis

Author

Vincentelli, Andre, Susen, Sophie, Le Tourneau, Thierry, Six, Isabelle, Fabre, Olivier, Juthier, Francis, Bauters, Anne, Decoene, Christophe, Goudemand, Jenny, Prat, Alain, and Jude, Brigitte

Subjects
Hemorrhage -- Causes of, Aortic valve stenosis -- Complications, Von Willebrand's disease -- Causes of
Abstract

Aortic stenosis can cause excessive bleeding by damaging proteins involved in blood clotting, according to a study of 50 patients. In patients with aortic valve stenosis, the aortic valve in the heart is narrower than normal. This damages a large blood clotting protein called von Willebrand factor as it passes through the valve and this in turn causes excessive bleeding. This explains why blood clotting returns to normal in many patients who have the valve replaced with an artificial valve.

Published
2003

145. Aldosterone, mortality, and acute ischaemic events in coronary artery disease patients outside the setting of acute myocardial infarction or heart failure

Author

Ivanes, Fabrice, Susen, Sophie, Mouquet, Frédéric, Pigny, Pascal, Cuilleret, François, Sautière, Karine, Collet, Jean-Philippe, Beygui, Farzin, Hennache, Bernadette, Ennezat, Pierre Vladimir, Juthier, Françis, Richard, Florence, Dallongeville, Jean, Hillaert, Marieke A., Doevendans, Pieter A., Jude, Brigitte, Bertrand, Michel, Montalescot, Gilles, and Van Belle, Eric

Published
2012
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147. Intracoronary autologous mononucleated bone marrow cell infusion for acute myocardial infarction: results of the randomized multicenter BONAMI trial

Author

Roncalli, Jérôme, Mouquet, Frédéric, Piot, Christophe, Trochu, Jean-Noel, Le Corvoisier, Philippe, Neuder, Yannick, Le Tourneau, Thierry, Agostini, Denis, Gaxotte, Virginia, Sportouch, Catherine, Galinier, Michel, Crochet, Dominique, Teiger, Emmanuel, Richard, Marie-Jeanne, Polge, Anne-Sophie, Beregi, Jean-Paul, Manrique, Alain, Carrie, Didier, Susen, Sophie, Klein, Bernard, Parini, Angelo, Lamirault, Guillaume, Croisille, Pierre, Rouard, Hélène, Bourin, Philippe, Nguyen, Jean-Michel, Delasalle, Béatrice, Vanzetto, Gérald, Van Belle, Eric, and Lemarchand, Patricia

Published
2011
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150. Paradoxical Increase of Stroke in Patients with Defect of High Molecular Weight Multimers of the von Willebrand Factors following Transcatheter Aortic Valve Replacement

Author

Matsushita, Kensuke, additional, Marchandot, Benjamin, additional, Trimaille, Antonin, additional, Kibler, Marion, additional, Heger, Joe, additional, Peillex, Marilou, additional, Hess, Sebastien, additional, Grunebaum, Lelia, additional, Reydel, Antje, additional, Kindo, Michel, additional, Hoang, Minh Tam, additional, Sato, Chisato, additional, De Poli, Fabien, additional, Leddet, Pierre, additional, Ohana, Mickael, additional, Jesel, Laurence, additional, Ohlmann, Patrick, additional, Susen, Sophie, additional, and Morel, Olivier, additional

Published
2020
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