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538 results on '"Devauchelle P"'

52. Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome

Author

Vabres, Pierre, Sorlin, Arthur, Kholmanskikh, Stanislav S., Demeer, Bénédicte, St-Onge, Judith, Duffourd, Yannis, Kuentz, Paul, Courcet, Jean-Benoît, Carmignac, Virginie, Garret, Philippine, Bessis, Didier, Boute, Odile, Bron, Alain, Captier, Guillaume, Carmi, Esther, Devauchelle, Bernard, Geneviève, David, Gondry-Jouet, Catherine, Guibaud, Laurent, Lafon, Arnaud, Mathieu-Dramard, Michèle, Thevenon, Julien, Dobyns, William B., Bernard, Geneviève, Polubothu, Satyamaanasa, Faravelli, Francesca, Kinsler, Veronica A., Thauvin, Christel, Faivre, Laurence, Ross, M. Elizabeth, and Rivière, Jean-Baptiste

Published
2019
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53. Healthy Patients Are Not the Best Controls for Microbiome-Based Clinical Studies: Example of Sjögren’s Syndrome in a Systematic Review

Author

Elise Doaré, Geneviève Héry-Arnaud, Valérie Devauchelle-Pensec, and Guillermo Carvajal Alegria

Subjects
oral microbiota, gut microbiota, Sjögren’s syndrome, sicca, dysbiosis, Immunologic diseases. Allergy, RC581-607
Abstract

IntroductionIt has been hypothesized that gut and oral dysbiosis may contribute to the development of primary Sjögren’s syndrome (pSS). The aim of this systematic review was to assemble available data regarding the oral and gut microbiota in pSS and to compare them to data from healthy individuals and patients with dry symptoms without a diagnosis of Sjögren’s syndrome or lupus disease to identify dysbiosis and discuss the results.MethodologyUsing the PRISMA guidelines, we systematically reviewed studies that compared the oral and gut microbiota of Sjögren’s patients and controls. The PubMed database and Google Scholar were searched.ResultsTwo-hundred and eighty-nine studies were found, and 18 studies were included: 13 referred to the oral microbiota, 4 referred to the gut microbiota, and 1 referred to both anatomical sites. The most frequent controls were healthy volunteers and patients with sicca symptoms. The most common analysis method used was 16S-targeted metagenomics. The results were mostly heterogeneous, and the results regarding diversity were not always in accordance. Dysbiosis in pSS was not confirmed, and reduced salivary secretion seems to explain more microbial changes than the underlying disease.ConclusionThese heterogeneous results might be explained by the lack of a standardized methodology at each step of the process and highlight the need for guidelines. Our review provides evidence that sicca patients seem to be more relevant than healthy subjects as a control group.

Published
2021
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54. Shape and dynamics of seepage erosion in a horizontal granular bed

Author

Berhanu, Michael, Petroff, Alexander, Devauchelle, Olivier, Kudrolli, Arshad, and Rothman, Daniel H.

Subjects
Physics - Fluid Dynamics, Physics - Geophysics, 76S05
Abstract

We investigate erosion patterns observed in a horizontal granular bed resulting from seepage of water motivated by observation of beach rills and channel growth in larger scale landforms. Our experimental apparatus consists of a wide rectangular box filled with glass beads with a narrow opening in one of the side walls from which eroded grains can exit. Quantitative data on the shape of the pattern and erosion dynamics are obtained with a laser-aided topography technique. We show that the spatial distribution of the source of groundwater can significantly impact the shape of observed patterns. An elongated channel is observed to grow upstream when groundwater is injected at a boundary adjacent to a reservoir held at constant height. An amphitheater (semi-circular) shape is observed when uniform rainfall infiltrates the granular bed to maintain a water table. Bifurcations are observed as the channels grow in response to the ground water. We further find that the channels grow by discrete avalanches as the height of the granular bed is increased above the capillary rise, causing the deeper channels to have rougher fronts. The spatio-temporal distribution of avalanches increase with bed height when partial saturation of the bed leads to cohesion between grains. However, the overall shape of the channels is observed to remain unaffected indicating that seepage erosion is robust to perturbation of the erosion front., Comment: 10 pages, 9 figures

Published
2012
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55. Characterization of a Molecularly Distinct Subset of Oncocytic Pleomorphic Adenomas/Myoepitheliomas Harboring Recurrent ZBTB47-AS1::PLAG1Gene Fusion

Author

Alsugair, Ziyad, Perrot, Jimmy, Descotes, Françoise, Lopez, Jonathan, Champagnac, Anne, Pissaloux, Daniel, Castain, Claire, Onea, Mihaela, Céruse, Philippe, Philouze, Pierre, Lépine, Charles, Lanic, Marie-Delphine, Laé, Marick, Costes-Martineau, Valérie, Benzerdjeb, Nazim, Ala-Eddine, C., Aubry, K., Babin, E., Bach, C., Badoual, C., Baglin, A.C., Barry, B., Bastit, V., Baujat, B., Benezery, K., Bensadoun, R.J., Benzerdjeb, N., Bernadach, M., Bertolus, C., Biet, A., Bodmer, D., Boisselier, P., Boulagnon-Rombi, C., Bozec, L., Grayeli, A. Bozorg, Brenet, E., Brugel, L., Calais, G., Calugaru, V., Camby, S., Casiraghi, O., Cassagnau, E., Castain, C., Castelli, J., Ceruse, P., Chabolle, F., Chevalier, D., Choussy, O., Clatot, F., Constans, J.M., Coste, A., Coste, F., Costes, V., Cottier, J.P., Coutte, A., Cristofari, J.P., Cupissol, D., Delgrande, J., Delord, J.P., Devauchelle, B., Digue, L., Dolivet, G., Doré, M., Duflo, S., Dufour, X., Dupin, C., Eker, E., Even, C., Evrard, C., Fabiano, E., Faivre, S., Fakhry, N., Ferrand, F. R., Frandon, J., Franetti, D., de Gabory, L., Galy, C., Garcier, M., Garrel, R., Gauthier, H., Gilain, L., Guihard, S., Guillerm, S., Halimi, C., Hans, S., Herman, P., Houessinon, A., Hourseau, M., Huguet, F., Jadaud, E., Jankowski, R., Jeanne, C., Jegoux, F., Juliéron, M., Kaci, R., Kaminsky, M.-C., de Kermadec, H., Kolb, F., Kreps, S., Laadhari, M., Saint Guily, J. Lacau, Laccoureye, L., Lae, M., Lagarde, F., Lagrange, A., Lallemant, B., Lamuraglia, M., Lang, P., Lapeyre, M., Lapierre, A., Cardon, A. Lasne, Le Tourneau, C., Lefebvre, G, Lefevre, M., Lelonge, Y., Leroy, X., Lesnik, M., Liem, X., Linassier, C., Maingon, P., Majoufre, C., Malard, O., Malouf, G., Marchand, C., Marie, J.-P., Maurina, T., Mauvais, O., Merol, J.-C., Michel, J., Mineur, G., Mirafzal, S., Mirghani, H., Modesto, A., Molinier-Blossier, S., de Monès, E., Morinière, S., Mouawad, F., Moya-Plana, A, Muller, L., Musat, E., Nguyen, F., Noel, G., Obongo-Anga, F.R., Onea, M., Orliac, H., Page, C., Patron, V., Pestre, J., Dang, N. Pham, Philouze, P., Poissonnet, G., Pons, C., Pouliquen, C., Prades, J.-M., Prevost, A., Queiros, C., Rahmani, A., Rambeau, A., Ramin, L., Renard, S., Righini, C.A., Rolland, F., Saada, E., Sacino, F., Salas, S., Saroul, N., Schultz, P., Simonaggio, A., Sterkers, O., Strunski, V., Sudaka, A., Xu-Shan, S., Taouachi, R., Tassart, M., Testelin, S., Thariat, J., David, M. Timar, Timochenko, A., Toussaint, B., Coste, E. Uro, Valette, G., Van den Abbele, T., Varoquaux, A., Vauleon, E., Vergez, S., Verillaud, B., Villa, J., Villepelet, A., Volondat, M., Vulquin, N., Wagner, I., Wassef, M., Webert, L., and Wong, S.

Abstract

Recurrent gene fusions are common in salivary gland tumors including benign tumors, such as pleomorphic adenoma (PA) and myoepithelioma (ME). In cases where chromosomal rearrangement is identified in the pleomorphic adenoma gene 1 (PLAG1)gene, different gene partners are found. Oncocytic metaplasia, characterized by oncocytes with abundant eosinophilic granular cytoplasm and hyperchromatic nuclei, is a well-known phenomenon in salivary gland neoplasms. However, the pure oncocytic variant of PA/ME showed PLAG1gene rearrangements involving various gene partners at the molecular level, without any recurrent fusion being found. Our study includes 20 cases of PA/ME, with 11 females and 9 males. The age of patients ranged from 37 to 96 years, with a median age of 62.8 years. Most tumors originate from the parotid gland. The median size of the tumor was 26.5 mm (range: 13 to 60 mm). Among the 20 cases, 14 were a pure oncocytic variant of PA/ME, whereas 6 cases showed focal oncocytic or oncocytic-like aspects. Molecular studies on 20 cases of PA/ME were conducted. A novel recurrent ZBTB47-AS1::PLAG1fusion was identified in 6 of 12 cases with pure oncocytic metaplasia, whereas the other cases had PLAG1gene fusion with different gene partners. The transcriptomic analysis of the cases harboring ZBTB47-AS1::PLAG1fusion demonstrated that these tumors have a distinct molecular profile from conventional PA/ME. This study reveals a unique subset in the oncocytic PA/ME spectrum characterized by pure oncocytic morphology with larger oncocytic cells and recurrent ZBTB47-AS1::PLAG1fusion. It also highlights the transcriptomic distinctness of salivary gland adenomas with pure oncocytic metaplasia in the spectrum of salivary gland neoplasms. Further studies are needed to better understand the oncocytic variant of PA/ME and to determine the true nature of oncocytic cells in PA/ME.

Published
2024
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56. Identification of common predisposing loci to hematopoietic cancers in four dog breeds.

Author

Benoît Hédan, Édouard Cadieu, Maud Rimbault, Amaury Vaysse, Caroline Dufaure de Citres, Patrick Devauchelle, Nadine Botherel, Jérôme Abadie, Pascale Quignon, Thomas Derrien, and Catherine André

Subjects
Genetics, QH426-470
Abstract

Histiocytic sarcoma (HS) is a rare but aggressive cancer in both humans and dogs. The spontaneous canine model, which has clinical, epidemiological, and histological similarities with human HS and specific breed predispositions, provides a unique opportunity to unravel the genetic basis of this cancer. In this study, we aimed to identify germline risk factors associated with the development of HS in canine-predisposed breeds. We used a methodology that combined several genome-wide association studies in a multi-breed and multi-cancer approach as well as targeted next-generation sequencing, and imputation We combined several dog breeds (Bernese mountain dogs, Rottweilers, flat-coated retrievers, and golden retrievers), and three hematopoietic cancers (HS, lymphoma, and mast cell tumor). Results showed that we not only refined the previously identified HS risk CDKN2A locus, but also identified new loci on canine chromosomes 2, 5, 14, and 20. Capture and targeted sequencing of specific loci suggested the existence of regulatory variants in non-coding regions and methylation mechanisms linked to risk haplotypes, which lead to strong cancer predisposition in specific dog breeds. We also showed that these canine cancer predisposing loci appeared to be due to the additive effect of several risk haplotypes involved in other hematopoietic cancers such as lymphoma or mast cell tumors as well. This illustrates the pleiotropic nature of these canine cancer loci as observed in human oncology, thereby reinforcing the interest of predisposed dog breeds to study cancer initiation and progression.

Published
2021
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57. Geometry of Valley Growth

Author

Petroff, Alexander P., Devauchelle, Olivier, Abrams, Daniel M., Lobkovsky, Alexander E., Kudrolli, Arshad, and Rothman, Daniel H.

Subjects
Physics - Fluid Dynamics
Abstract

Although amphitheater-shaped valley heads can be cut by groundwater flows emerging from springs, recent geological evidence suggests that other processes may also produce similar features, thus confounding the interpretations of such valley heads on Earth and Mars. To better understand the origin of this topographic form we combine field observations, laboratory experiments, analysis of a high-resolution topographic map, and mathematical theory to quantitatively characterize a class of physical phenomena that produce amphitheater-shaped heads. The resulting geometric growth equation accurately predicts the shape of decimeter-wide channels in laboratory experiments, 100-meter wide valleys in Florida and Idaho, and kilometer wide valleys on Mars. We find that whenever the processes shaping a landscape favor the growth of sharply protruding features, channels develop amphitheater-shaped heads with an aspect ratio of pi.

Published
2010
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58. Forced dewetting on porous media

Author

Devauchelle, Olivier, Josserand, Christophe, and Zaleski, Stephane

Subjects
Condensed Matter - Soft Condensed Matter
Abstract

We study the dewetting of a porous plate withdrawn from a bath of fluid. The microscopic contact angle is fixed to zero and the flow is assumed to be parallel to the plate (lubrication approximation). The ordinary differential equation involving the position of the water surface is analysed in phase space by means of numerical integration. We show the existence of a critical value of the capillary number $\eta U / \gamma$, above which no stationary contact line can exist. An analytical model, based on asymptotic matching is developed, that reproduces the dependence of the critical capillary number on the angle of the plate with respect to the horizontal for large control parameters (3/2 power law).

Published
2005

59. Thrombotic Complications in Venous Malformations: Are There Differences Between Facial and Other Localizations?

Author

Simon Soudet MD, Stephanie Dakpe MD, Sandra Le Gloan MD, Esther Carmi MD, Jean Philippe Arnault MD, Sylvie Testelin MD, PhD, Marie-Christine Plancq MD, Bernard Devauchelle MD, PhD, and Marie Antoinette Sevestre MD, PhD

Subjects
Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Venous thrombosis (VT) is a frequent complication in venous malformations (VM) in relation with blood stasis and localized intravascular coagulopathy (LIC). Our aim was to describe the clinical characteristics and the treatment of patients with facial and non facial VM with VT. We implemented an observational retrospective study of patients with VM followed between 2002 and 2017. We compared features of facial and non facial VM. Descriptive and bivariate statistics were computed and the P value was set at 0.05. Fifty patients were included between 2002 and 2017. 24 of them were women (44%). The median age of the patients at diagnosis was 16,5 [8-31] years. The median follow up was 2 [2; 4] years. In non facial VM venous thrombosis occurred in 12 cases. In facial VM, 3 patients had thrombotic complication (15%). We demonstrate no difference of VT between facial VM and other localization. No patients had clinical risk factors for VT at diagnosis. Our study showed that VT is a frequent complication of VM and its proportion is not different between facial and non facial VM. Studies are needed to confirm the role of LIC in VT in VM, particularly in facial VM.

Published
2020
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62. Methotrexate effect on immunogenicity and long-term maintenance of adalimumab in axial spondyloarthritis: a multicentric randomised trial

Author

Grégoire Cormier, Benoit Le Goff, Emmanuelle Dernis, Philippe Goupille, Aleth Perdriger, Theo Rispens, David Ternant, Gilles Paintaud, Emilie Ducourau, Marine Samain, Fabienne Le Guilchard, Lucia Andras, Eric Lespessailles, Antoine Martin, Thomas Armingeat, Valérie Devauchelle-Pensec, Elisabeth Gervais, Annick de Vries, Eric Piver, Céline Desvignes, Hervé Watier, and Denis Mulleman

Subjects
Medicine
Abstract

ObjectivesAnti-drug antibodies (ADA) are responsible for decreased adalimumab efficacy in axial spondyloarthritis (SpA). We aimed to evaluate the ability of methotrexate (MTX) to decrease adalimumab immunisation.MethodsA total of 110 patients eligible to receive adalimumab 40 mg subcutaneously (s.c.) every other week were randomised (1:1 ratio) to receive, 2 weeks before adalimumab (W-2) and weekly, MTX 10 mg s.c. (MTX+) or not (MTX−). ADA detection and adalimumab serum concentration were assessed at weeks 4 (W4), 8 (W8), 12 (W12) and 26 (W26) after starting adalimumab (W0). The primary outcome was the proportion of patients with ADA at W26. Four years after the study completion, we retrospectively analysed adalimumab maintenance in relation with MTX co-treatment duration.ResultsWe analysed data for 107 patients (MTX+; n=52; MTX-; n=55). ADA were detected at W26 in 39/107 (36.4%) patients: 13/52 (25%) in the MTX+ group and 26/55 (47.3%) in the MTX− group (p=0.03). Adalimumab concentration was significantly higher in the MTX+ than MTX− group at W4, W8, W12 and W26. The two groups did not differ in adverse events or efficacy. In the follow-up study, MTX co-treatment >W26 versus no MTX or ≤W26 was significantly associated with adalimumab long-term maintenance (p=0.04).ConclusionMTX reduces the immunogenicity and ameliorate the pharmacokinetics of adalimumab in axial SpA. A prolonged co-treatment of MTX>W26 seems to increase adalimumab long-term maintenance.

Published
2020
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63. Functional Validation of a New Alginate-based Hydrogel Scaffold Combined with Mesenchymal Stem Cells in a Rat Hard Palate Cleft Model

Author

Marie Naudot, PhD, Julien Davrou, MD, Az-Eddine Djebara, MD, Anaïs Barre, MS, Nolwenn Lavagen, MD, Sandrine Lardière, PhD, Soufiane Zakaria Azdad, MD, Luciane Zabijak, BS, Stéphane Lack, PhD, Bernard Devauchelle, MD, Jean-Pierre Marolleau, MD, and Sophie Le Ricousse, PhD

Subjects
Surgery, RD1-811
Abstract

Background:. One of the major difficulties in cleft palate repair is the requirement for several surgical procedures and autologous bone grafting to form a bony bridge across the cleft defect. Engineered tissue, composed of a biomaterial scaffold and multipotent stem cells, may be a useful alternative for minimizing the non-negligible risk of donor site morbidity. The present study was designed to confirm the healing and osteogenic properties of a novel alginate-based hydrogel in palate repair. Methods:. Matrix constructs, seeded with allogeneic bone marrow–derived mesenchymal stem cells (BM-MSCs) or not, were incorporated into a surgically created, critical-sized cleft palate defect in the rat. Control with no scaffold was also tested. Bone formation was assessed using microcomputed tomography at weeks 2, 4, 8, and 12 and a histologic analysis at week 12. Results:. At 12 weeks, the proportion of bone filling associated with the use of hydrogel scaffold alone did not differ significantly from the values observed in the scaffold-free experiment (61.01% ± 5.288% versus 36.91% ± 5.132%; p = 0.1620). The addition of BM-MSCs stimulated bone formation not only at the margin of the defect but also in the center of the implant. Conclusions:. In a relevant in vivo model of cleft palate in the rat, we confirmed the alginate-based hydrogel’s biocompatibility and real advantages for tissue healing. Addition of BM-MSCs stimulated bone formation in the center of the implant, demonstrating the new biomaterial’s potential for use as a bone substitute grafting material for cleft palate repair.

Published
2020
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64. Clinical Characteristics of Pruritus in Systemic Sclerosis Vary According to the Autoimmune Subtype

Author

Greta Gourier, Chloé Théréné, Margaux Mazeas, Claire Abasq-Thomas, Emilie Brenaut, Flavien Huet, Haitham Sonbol, Elize Campillo, Julie Lemerle, Elisabeth Pasquier, Emmanuelle Le Moigne, Alain Saraux, Valérie Devauchelle-Pensec, Laurent Misery, and Yves Renaudineau

Subjects
systemicsclerosis, pruritus, itch, autoantibodies, inflammation, autoimmunity, qualityoflife, Dermatology, RL1-803
Abstract

Pruritus is a frequent symptom in systemic sclerosis (SSc), with a prevalence of 40–65%, but its pathophysiology is poorly understood. This study investigated the immunological component of pruritus. Fifty-six patients with SSc responded to a standardized questionnaire regarding both SSc disease and pruritus characteristics. Among patients with SSc, those with pruritus did not display a particular immunological profile (inflammatory, humoral, and/or cellular factors), but pruritus was, in most cases, concomitant with the development of SSc. Thus, pruritus characteristics were evaluated further, according to the detection of anti-centromere autoantibodies (ACA), into ACA+ (n = 17) and ACA– (n = 19). The ACA+ subgroup was characterized by a longer evolution of SSc and pruritus, pruritus present outside the sclerotic area, and a shorter daily duration of pruritus. In conclusion, the concomitant appearance of the 2 processes and the differences observed between ACA+ and ACA– subgroups support the presence of an immunological component in pruritus.

Published
2018
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65. Advection and dispersion of bed load tracers

Author

E. Lajeunesse, O. Devauchelle, and F. James

Subjects
Dynamic and structural geology, QE500-639.5
Abstract

We use the erosion–deposition model introduced by Charru et al. (2004) to numerically simulate the evolution of a plume of bed load tracers entrained by a steady flow. In this model, the propagation of the plume results from the stochastic exchange of particles between the bed and the bed load layer. We find a transition between two asymptotic regimes. The tracers, initially at rest, are gradually set into motion by the flow. During this entrainment regime, the plume is strongly skewed in the direction of propagation and continuously accelerates while spreading nonlinearly. With time, the skewness of the plume eventually reaches a maximum value before decreasing. This marks the transition to an advection–diffusion regime in which the plume becomes increasingly symmetrical, spreads linearly, and advances at constant velocity. We analytically derive the expressions of the position, the variance, and the skewness of the plume and investigate their asymptotic regimes. Our model assumes steady state. In the field, however, bed load transport is intermittent. We show that the asymptotic regimes become insensitive to this intermittency when expressed in terms of the distance traveled by the plume. If this finding applies to the field, it might provide an estimate for the average bed load transport rate.

Published
2018
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66. Comparison of 2002 AECG and 2016 ACR/EULAR classification criteria and added value of salivary gland ultrasonography in a patient cohort with suspected primary Sjögren’s syndrome

Author

Maëlle Le Goff, Divi Cornec, Sandrine Jousse-Joulin, Dewi Guellec, Sebastian Costa, Thierry Marhadour, Rozenn Le Berre, Steeve Genestet, Béatrice Cochener, Sylvie Boisrame-Gastrin, Yves Renaudineau, Jacques-Olivier Pers, Alain Saraux, and Valérie Devauchelle-Pensec

Subjects
Sjögren’s syndrome, Primary, Classification criteria, Diagnosis, Ultrasonography, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background The objective was to evaluate concordance between 2002 American-European Consensus Group (AECG) and 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for primary Sjögren’s syndrome (pSS) and to assess how salivary gland ultrasonography (SGUS) might improve the classification of patients. Methods Patients with suspected pSS underwent a standardised evaluation, including SGUS, at inclusion into the single-centre Brittany DIApSS cohort. Agreement between the two criteria sets was assessed using Cohen’s κ coefficient. Characteristics of discordantly categorised patients were detailed. Results We prospectively included 290 patients between 2006 and 2016, among whom 125 (43%) met ACR/EULAR criteria and 114 (39%) also met AECG criteria; thus, 11 (4%) patients fulfilled only ACR/EULAR, no patients AECG only, and 165 (57%) patients neither criteria set. Concordance was excellent (κ = 0.92). Compared to patients fulfilling both criteria sets, the 11 patients fulfilling only ACR/EULAR criteria had similar age and symptom duration but lower frequencies of xerophthalmia and xerostomia (p

Published
2017
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70. Conventional radiography in juvenile idiopathic arthritis: Joint recommendations from the French societies for rheumatology, radiology and paediatric rheumatology

Author

Marteau, Pauline, Adamsbaum, Catherine, Rossi-Semerano, Linda, De Bandt, Michel, Lemelle, Irène, Deslandre, Chantal, Tran, Tu Anh, Lohse, Anne, Solau-Gervais, Elisabeth, Sordet, Christelle, Pillet, Pascal, Bader-Meunier, Brigitte, Wipff, Julien, Gaujoux-Viala, Cécile, Breton, Sylvain, and Devauchelle-Pensec, Valérie

Published
2018
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71. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

Author

F. De Benedetti, J. Anton, M. Gattorno, H. Lachmann, I. Kone-Paut, S. Ozen, J. Frenkel, A. Simon, A. Zeft, E. Ben-Chetrit, H. M. Hoffman, Y. Joubert, K. Lheritier, A. Speziale, J. Guido, Roberta Caorsi, Federica Penco, Alice Grossi, Antonella Insalaco, Maria Alessio, Giovanni Conti, Federico Marchetti, Alberto Tommasini, Silvana Martino, Romina Gallizzi, Annalisa Salis, Francesca Schena, Francesco Caroli, Alberto Martini, Gianluca Damonte, Isabella Ceccherini, Marco Gattorno, Marie-Louise Frémond, Carolina Uggenti, Lien Van Eyck, Isabelle Melki, Darragh Duffy, Vincent Bondet, Yoann Rose, Bénédicte Neven, Yanick Crow, Mathieu P. Rodero, Yvonne Kusche, Johannes Roth, Katarzyna Barczyk-Kahlert, Giovanna Ferrara, Annalisa Chiocchetti, Silvio Polizzi, Josef Vuch, Diego Vozzi, Anna Mondino, Erica Valencic, Serena Pastore, Andrea Taddio, Flavio Faletra, Umberto Dianzani, Ugo Ramenghi, Qing Zhou, Xiaomin Yu, Erkan Demirkaya, Natalie Deuitch, Deborah Stone, Wanxia Tsai, Amanda Ombrello, Tina Romeo, Elaine F. Remmers, JaeJin Chae, Massimo Gadina, Steven Welch, Seza Ozen, Rezan Topaloglu, Mario Abinun, Daniel L. Kastner, Ivona Aksentijevich, Donatella Vairo, Rosalba Monica Ferraro, Giulia Zani, Jessica Galli, Micaela De Simone, Marco Cattalini, Elisa Fazzi, Silvia Giliani, Ebun Omoyinmi, Ariane Standing, Dorota Rowczenio, Annette Keylock, Sonia Melo Gomes, Fiona Price-Kuehne, Sira Nanthapisal, Claire Murphy, Thomas Cullup, Lucy Jenkins, Kimberly Gilmour, Despina Eleftheriou, Helen Lachmann, Philip Hawkins, Nigel Klein, Paul Brogan, Anita Dhanrajani, Mercedes Chan, Stephanie Pau, Janet Ellsworth, Jaime Guzman, Florence A. Aeschlimann, Marinka Twilt, Simon W. Eng, Shehla Sheikh, Ronald M. Laxer, Diane Hebert, Damien Noone, Christian Pagnoux, Susanne M. Benseler, Rae S. Yeung, Christoph Kessel, Katrin Lippitz, Toni Weinhage, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Niklas Grün, Dirk Föll, Pieter Van Dijkhuizen, Federica Del Chierico, Clara Malattia, Alessandra Russo, Denise Pires Marafon, Nienke M. ter Haar, Silvia Magni-Manzoni, Sebastiaan J. Vastert, Bruno Dallapiccola, Berent Prakken, Fabrizio De Benedetti, Lorenza Putignani, Berna Eren Fidanci, Kenan Barut, Serap Arıcı, Dogan Simsek, Mustafa Cakan, Ezgi D. Batu, Sezgin Şahin, Ayşenur Kısaarslan, Ebru Yilmaz, Özge Basaran, Ferhat Demir, Kubra Ozturk, Zübeyde Gunduz, Betül Sozeri, Balahan Makay, Nuray Ayaz, Onder Yavascan, Ozlem Aydog, Yelda Bilginer, Zelal Ekinci, Dilek Yıldız, Faysal Gök, Muferret Erguven, Erbil Unsal, Ozgur Kasapcopur, For the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR), Hafize E. Sönmez, Betül Sözeri, Yonatan Butbul, Seza Özen, Claudia Bracaglia, Giusi Prencipe, Manuela Pardeo, Geneviève Lapeyre, Emiliano Marasco, Walter Ferlin, Robert Nelson, Cristina de Min, N. Ruperto, H. I. Brunner, P. Quartier, T. Constantin, E. Alexeeva, K. Marzan, N. Wulffraat, R. Schneider, S. Padeh, V. Chasnyk, C. Wouters, J. B. Kuemmerle-Deschner, T. Kallinich, B. Lauwerys, E. Haddad, E. Nasonov, M. Trachana, O. Vougiouka, K. Leon, E. Vritzali, A. Martini, D. Lovell, PRINTO/PRCSG, Stefano Volpi, Claudia Pastorino, Francesca Kalli, Alessia Omenetti, Sabrina Chiesa, Arinna Bertoni, Paolo Picco, Gilberto Filaci, Elisabetta Traggiai, Marie-Louise Fremond, Naoki Kitabayashi, Olivero Sacco, Isabelle Meyts, Marie-Anne Morren, Carine Wouters, Eric Legius, Isabelle Callebaut, Christine Bodemer, Frederic Rieux-Laucat, Mathieu Rodero, Nadia Jeremiah, Alexandre Belot, Eric Jeziorski, Didier Bessis, Guilhem Cros, Gillian I. Rice, Bruno Charbit, Anne Hulin, Nihel Khoudour, Consuelo Modesto Caballero, Monique Fabre, Laureline Berteloot, Muriel Le Bourgeois, Philippe Reix, Thierry Walzer, Despina Moshous, Stéphane Blanche, Alain Fischer, Brigitte Bader-Meunier, Frédéric Rieux-Laucat, K. Annink, N. ter Haar, S. Al-Mayouf, G. Amaryan, K. Barron, S. Benseler, P. Brogan, L. Cantarini, M. Cattalini, A. Cochino, F. Dedeoglu, A. De Jesus, O. Dellacasa, E. Demirkaya, P. Dolezalova, K. Durrant, G. Fabio, R. Gallizzi, R. Goldbach-Mansky, E. Hachulla, V. Hentgen, T. Herlin, M. Hofer, H. Hoffman, A. Insalaco, A. Jansson, I. Koné-Paut, A. Kozlova, J. Kuemmerle-Deschner, R. Laxer, S. Nielsen, I. Nikishina, A. Ombrello, E. Papadopoulou-Alataki, A. Ravelli, D. Rigante, R. Russo, Y. Uziel, Nienke ter Haar, Jerold Jeyaratnam, Anna Simon, Matteo Doglio, Jordi Anton, Consuelo Modesto, Pierre Quartier, Esther Hoppenreijs, Luca Cantarini, Loredana Lepore, Inmaculada Calvo Penades, Christina Boros, Rita Consolini, Donato Rigante, Ricardo Russo, Jana Pachlopnik Schmid, Thirusha Lane, Nicolino Ruperto, Joost Frenkel, Chiara Passarelli, Elisa Pisaneschi, Virginia Messia, Antonio Novelli, Fabrizio Debenedetti, P. A. Brogan, X. Wei, Martina Finetti, Francesca Orlando, Elisabetta Cortis, Angela Miniaci, Nicola Ruperto, Charlotte Eijkelboom, Pavla Dolezalova, Isabelle Koné-Paut, Marija Jelusic-Drazic, Liliana Bezrodnik, Mari Carmen Pinedo, Valda Stanevicha, Marielle van Gijn, Silvia Federici, Hermann Girschick, Gerd Ganser, Susan Nielsen, Troels Herlin, Sulaiman Mohammed Al-Mayouf, Michael Hofer, Jasmin Kuemmerle-Deschner, Susanne Schalm, Annette Jansson, on behalf of PRINTO and Eurofever registry, Marta Marchi, Chiara Marini, Angelo Ravelli, Alberto Garaventa, Sonia Carta, Enrica Balza, Patrizia Castellani, Caterina Pellecchia, Silvia Borghini, Maria Libera Trotta, Anna Rubartelli, Andrew Henrey, Thomas Loughin, Roberta Berard, Natalie Shiff, Roman Jurencak, Susanne Benseler, Lori Tucker, on behalf of ReACCh-Out Investigators, Charalampia Papadopoulou, Ying Hong, Petra Krol, Yiannis Ioannou, Clarissa Pilkington, Hema Chaplin, Stephania Simou, Marietta Charakida, Lucy Wedderburn, Lynn R. Spiegel, Sara Ahola Kohut, Jennifer Stinson, Paula Forgeron, Miriam Kaufman, Nadia Luca, Khush Amaria, Mary Bell, J Swart, F. Boris, E. Castagnola, A. Groll, G. Giancane, G. Horneff, H. I. Huppertz, T. Wolfs, E. Alekseeva, V. Panaviene, F. Uettwiller, V. Stanevicha, L. M. Ailioaie, E. Tsitami, S. Kamphuis, G. Susic, F. Sztajnbok, B. Flato, A. Pistorio, Stephanie J. W. Shoop, Suzanne M. M. Verstappen, Janet E. McDonagh, Wendy Thomson, Kimme L. Hyrich, CAPS, Maarit Tarkiainen, Pirjo Tynjala, Pekka Lahdenne, Janne Martikainen, Acute-JIA Study Group, Meredyth Wilkinson, Christopher Piper, Georg Otto, Claire T. Deakin, Stefanie Dowle, Stefania Simou, Daniel Kelberman, Claudia Mauri, Elizabeth Jury, David Isenberg, Lucy R. Wedderburn, Kiran Nistala, I. Foeldvari, D. J. Lovell, G. Simonini, M. Bereswill, J. Kalabic, Kiem Oen, Brian M. Feldman, Brenden Dufault, Jennifer Lee, Karen Watanabe Duffy, Ciaran Duffy, ReACCh-Out Investigators, N. Tzaribachev, G. Vega-Cornejo, I. Louw, A. Berman, I. Calvo, R. Cuttica, F. Avila-Zapata, R. Cimaz, E. Solau-Gervais, R. Joos, G. Espada, X. Li, M. Nys, R. Wong, S. Banerjee, For Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology Collaborative Study Group (PRCSG), Rebecca Nicolai, Margherita Verardo, Adele D’Amico, Luisa Bracci-Laudiero, Gian Marco Moneta, Gillian Rice, Anne-Laure Mathieu, Sulliman O. Omarjee, Tracy A. Briggs, James O’Sullivan, Simon Williams, Rolando Cimaz, Eve Smith, Michael W. Beresford, Yanick J. Crow, GENIAL Investigators, UK JSLE Study Group, Madeleine Rooney, Nick Bishop, joyce davidson, Clarissa pilkington, Michael Beresford, Jacqui Clinch, Rangaraj Satyapal, Helen Foster, Janet Gardner Medwin, Janet McDonagh, Sue Wyatt, On Behalf of the British Society for Paediatric and Adolescent Rheumatology, Valentina Litta Modignani, Francesco Baldo, Stefano Lanni, Alessandro Consolaro, Giovanni Filocamo, Helen J. Lachmann, on behalf of Eurofever Registry, Gianmarco Moneta, Camilla Celani, Bilade Cherqaoui, Linda Rossi-Semerano, Perrine Dusser, Véronique Hentgen, Claire Grimwood, Linda Rossi, Isabelle Kone Paut, Veronique Hentgen, Denise Lasigliè, Denise Ferrera, Giulia Amico, Marco Di Duca, Laura Obici, Roberto Ravazzolo, Ryuta Nishikomori, Juan Arostegui, Andrea Petretto, Chiara Lavarello, Elvira Inglese, Federica Vanoni, Michaël Hofer, on behalf of EUROFEVER PROJECT, P. N. Hawkins, T. van der Poll, U. A. Walker, H. H. Tilson, Pascal N. Tyrrell, Raphaela Goldbach-Mansky, Norbert Blank, Hal M. Hoffman, Elisabeth Weissbarth-Riedel, Boris Huegle, Tilmann Kallinich, Ahmet Gul, Marlen Oswald, Fatma Dedeoglu, Aki Hanaya, Takako Miyamae, Manabu Kawamoto, Yumi Tani, Takuma Hara, Yasushi Kawaguchi, Satoru Nagata, Hisashi Yamanaka, Almira Ćosićkić, Fahrija Skokić, Belkisa Čolić, Sanimir Suljendić, Anna Kozlova, Irina Mersiyanova, Mariya Panina, Lily Hachtryan, Vasiliy Burlakov, Elena Raikina, Alexey Maschan, Anna Shcherbina, Banu Acar, Meryem Albayrak, Betul Sozeri, Sezgin Sahin, Amra Adrovic, Nese Inan, Serhan Sevgi, Caroline M. Andreasen, Anne Grethe Jurik, Mia B. Glerup, Christian Høst, Birgitte T. Mahler, Ellen-Margrethe Hauge, Cecilia Lazea, Laura Damian, Calin Lazar, Rodica Manasia, Chloe M. Stephenson, Vimal Prajapati, Paivi M. Miettunen, Dilek Yılmaz, Yavuz Tokgöz, Yasin Bulut, Harun Çakmak, Ferah Sönmez, Elif Comak, Gülşah Kaya Aksoy, Mustafa Koyun, Sema Akman, Yunus Arıkan, Ender Terzioğlu, Osman Nidai Özdeş, İbrahim Keser, Hüseyin Koçak, Ayşen Bingöl, Aygen Yılmaz, Reha Artan, X. Xu, Fatemeh F. Mehregan, Vahid Ziaee, Mohammad H. Moradinejad, Francesco La Torre, Clotilde Alizzi, Pio D’Adamo, G. Junge, J. Gregson, Hasmik Sargsyan, Hulya Zengin, Berna E. Fidanci, Cagla Kaymakamgil, Dilek Konukbay, Dilek Yildiz, Faysal Gok, Iris Stoler, Judith Freytag, Banu Orak, Christine Seib, Lars Esmann, Eva Seipelt, Faekah Gohar, Dirk Foell, Ismail Dursun, Sebahat Tulpar, Sibel Yel, Demet Kartal, Murat Borlu, Funda Bastug, Hakan Poyrazoglu, Zubeyde Gunduz, Kader Kose, Mehmet E. Yuksel, Abdullah Calıskan, Ahmet B. Cekgeloglu, Ruhan Dusunsel, Katerina Bouchalova, Jana Franova, Marcel Schuller, Marie Macku, Katerina Theodoropoulou, Raffaella Carlomagno, Annette von Scheven-Gête, Claudia Poloni, Laura O. Damian, Dan Cosma, Amanda Radulescu, Dan Vasilescu, Liliana Rogojan, Simona Rednic, Mihaela Lupse, Lien De Somer, Pierre Moens, Rocio Galindo Zavala, Laura Martín Pedraz, Esmeralda Núñez Cuadros, Gisela Díaz-Cordovés Rego, Antonio L. Urda Cardona, Ilaria Dal Forno, Sara Pieropan, Ombretta Viapiana, Davide Gatti, Gloria Dallagiacoma, Paola Caramaschi, Domenico Biasi, Daniel Windschall, Ralf Trauzeddel, Hartwig Lehmann, Rainer Berendes, Maria Haller, Manuela Krumrey-Langkammerer, Antje Nimtz-Talaska, Philipp Schoof, Ralf Felix Trauzeddel, Christine Nirschl, Estefania Quesada-Masachs, Carla Aguilar Blancafort, Sara Marsal Barril, Francisca Aguiar, Rita Fonseca, Duarte Alves, Ana Vieira, Alberto Vieira, Jorge A. Dias, Iva Brito, Gordana Susic, Vera Milic, Goran Radunovic, Ivan Boricic, Pauline Marteau, Catherine Adamsbaum, Michel De Bandt, Irène Lemelle, Chantal Deslandre, Tu Anh Tran, Anne Lohse, Elisabeth Solau-Gervais, Pascal Pillet, Julien Wipff, Cécile Gaujoux-Viala, Sylvain Breton, Valérie Devauchelle-Pensec, Sandra Gran, Olesja Fehler, Stefanie Zenker, Michael Schäfers, Thomas Vogl, Severine Guillaume Czitrom, EH Pieter Van Dijkhuizen, Silvia Magni Manzoni, Francesca Magnaguagno, Laura Tanturri de Horatio, Nienke M. Ter Haar, Annemieke S. Littooij, Vitor A. Teixeira, Raquel Campanilho-Marques, Ana F. Mourão, Filipa O. Ramos, Manuela Costa, Wafa A. Madan, Orla G. Killeen, Adriana Rodriguez Vidal, Diana Sueiro Delgado, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Aleksey Kozhevnikov, Nina Pozdeeva, Mikhail Konev, Evgeniy Melchenko, Vladimir Kenis, Gennadiy Novik, Aysenur Pac Kısaarslan, Butsabong Lerkvaleekul, Suphaneewan Jaovisidha, Witaya Sungkarat, Niyata Chitrapazt, Praman Fuangfa, Thumanoon Ruangchaijatuporn, Soamarat Vilaiyuk, Dan Ø. Pradsgaard, Arne Hørlyck, Anne H. Spannow, Carsten W. Heuck, Talia Diaz, Fernando Garcia, Lorenia De La Cruz, Nadina Rubio, Joanna Świdrowska-Jaros, Elzbieta Smolewska, Mirta Lamot, Lovro Lamot, Mandica Vidovic, Edi Paleka Bosak, Ivana Rados, Miroslav Harjacek, Nikolay Tzaribachev, Polymnia Louka, Romiesa Hagoug, Chiara Trentin, Olga Kubassova, Mark Hinton, Mikael Boesen, Olena A. Oshlianska, Illya A. Chaikovsky, G. Mjasnikov, A. Kazmirchyk, Umberto Garagiola, Irene Borzani, Paolo Cressoni, Fabrizia Corona, Eszter Dzsida, Giampietro Farronato, Antonella Petaccia, Alenka Gagro, Agneza Marija Pasini, Goran Roic, Ozren Vrdoljak, Lucija Lujic, Matija Zutelija-Fattorini, Monika M. Esser, Deepthi R. Abraham, Craig Kinnear, Glenda Durrheim, Mike Urban, Eileen Hoal, Victoria B. Nikolayenko, Kubilay Şahin, Yasar Karaaslan, Adele Civino, Giovanni Alighieri, Sergio Davì, Roberto Rondelli, Andrea Magnolato, Francesca Ricci, Alma Olivieri, Valeria Gerloni, Bianca Lattanzi, Francesca Soscia, Alessandro De Fanti, Stefania Citiso, Lorenzo Quartulli, Maria Cristina Maggio, Manuela Marsili, Maria Antonietta Pelagatti, Valentino Conter, Franca Fagioli, Andrea Pession, Marco Garrone, Mariangela Rinaldi, Jaime De Inocencio, Stella Garay, Daniel J. Lovell, Berit Flato, EPOCA Study Group, Angela Aquilani, Simona Cascioli, Ivan Caiello, Denise Pires-Marafón, Rita Carsetti, Emily Robinson, Salvatore Albani, Wilco de Jager, Sytze de Roock, Trang Duong, Justine Ellis, Kimme Hyrich, Laetitia Jervis, Daniel Lovell, Lucy Marshall, Elizabeth D. Mellins, Kirsten Minden, Jane Munro, Peter A. Nigrovic, Jason Palman, Sunil Sampath, Laura E. Schanberg, Susan D. Thompson, Richard Vesely, Chris Wallace, Chris Williams, Qiong Wu, Nico Wulffraat, Rae S. M. Yeung, M. B. Seyger, D. Arikan, J. K. Anderson, A. Lazar, D. A. Williams, C. Wang, R. Tarzynski-Potempa, J. S. Hymans, Gabriele Simonini, Erika Scoccimarro, Irene Pontikaki, Teresa Giani, Alessandro Ventura, Pier Luigi Meroni, Gaetana Minnone, Marzia Soligo, Luigi Manni, Luisa Bracci Laudiero, Noortje Groot, I. Grein, N. M. Wulffraat, R. Schepp, G. Berbers, C. C. Barbosa Sandoval de Souza, V. Paes Leme Ferriani, G. Pileggi, S. de Roock, Ingrid H. R. Grein, Silvia Scala, Elisa Patrone, Casper Schoemaker, on behalf of Dutch JIA patient organization, Wendy Costello, on behalf of ENCA, Suzanne Parsons, Jean-David Cohen, Damien Bentayou, Marc-Antoine Bernard Brunel, Sonia Trope, Jens Klotsche, Miriam Listing, Martina Niewerth, Gerd Horneff, Angelika Thon, Hans-Iko Huppertz, Kirsten Mönkemöller, Ivan Foeldvari, ICON study group, Achille Marino, Stefano Stagi, Niccolò Carli, Federico Bertini, Adriana S. Díaz-Maldonado, Sally Pino, Pilar Guarnizo, Alfonso Ragnar Torres-Jimenez, Berenice Sanchez-Jara, Eunice Solis-Vallejo, Adriana Ivonne Cespedes-Cruz, Maritza Zeferino-Cruz, Julia Veronica Ramirez-Miramontes, Ankur Kumar, Anju Gupta, Deepti Suri, Amit Rawat, Nandita Kakkar, Surjit Singh, Özge A. Gücenmez, Erbil Ünsal, Bo Magnusson, Karina Mördrup, Anna Vermé, Christina Peterson, Board of the Swedish Pediatric Rheumatology Registry, Caroline Freychet, Jean Louis Stephan, Cathryn E. Harkness, Leanne Foster, Emma Henry, Pauline Taggart, Coskun F. Ozkececi, Esra Kurt, Gokalp Basbozkurt, Daiva Gorczyca, Jacek Postępski, Aleksandra Czajkowska, Bogumiła Szponar, Mariola Paściak, Anna Gruenpeter, Iwona Lachór-Motyka, Daria Augustyniak, Edyta Olesińska, Emediong S. Asuka, Tatyana Golovko, Samuel U. Aliejim, Emilio Inarejos Clemente, Estibaliz Iglesias Jimenez, Joan Calzada Hernandez, Sergi Borlan Fernandez, Clara Gimenez Roca, David Moreno Romo, Natalia Rodriguez Nieva, Juan Manuel Mosquera Angarita, Jordi Anton Lopez, Esmeralda Nuñez-Cuadros, Gisela Diaz-Cordovés, Rocío Galindo-Zavala, Antonio Urda-Cardona, Antonio Fernández-Nebro, Daniel Álvarez de la Sierra, Marina Garcia Prat, Mónica Martínez Gallo, Ricardo Pujol Borrell, Ana M. Marín Sánchez, Etienne Merlin, Sylvie Fraitag, Jean-Louis Stephan, Federico Annoni, Giancarla Di Landro, Sofia Torreggiani, Marta Torcoletti, Georgina Tiller, Jo Buckle, Angela Cox, Peter Gowdie, Roger C. Allen, Jonathan D. Akikusa, Hayde G. Hernández-Huirache, Edel R. Rodea-Montero, William Fahy, Christelle Sordet, Karin B. Berggren, Johanna T. Kembe, Joyce Bos, Wineke Armbrust, Marco van Brussel, Jeanette Cappon, Pieter Dijkstra, Jan Geertzen, Elizabeth Legger, Marion van Rossum, Pieter Sauer, Otto Lelieveld, Levent Buluc, Gur Akansel, Bahar Muezzinoglu, Ljubov Rychkova, Tatyana Knyazeva, Anna Pogodina, Tatyana Belova, Tamara Mandzyak, Ekaterina Kulesh, Alessandro Cafarotti, Cosimo Giannini, Roberta Salvatore, Giuseppe Lapergola, Caterina Di Battista, Maria Loredana Marcovecchio, Raffaella Basilico, Piernicola Pelliccia, Francesco Chiarelli, Luciana Breda, Beverley Almeida, Sarah Tansley, Harsha Gunawardena, Neil McHugh, Juvenile Dermatomyositis Research Group (JDRG), Jessie Aouizerate, Marie De Antonio, Christine Barnerias, Guillaume Bassez, Isabelle Desguerre, Romain Gherardi, Jean-Luc Charuel, François-Jérôme Authier, Cyril Gitiaux, C. H. Spencer, Rabheh Abdul Aziz, Chack-Yung Yu, Brent Adler, Sharon Bout-Tabaku, Katherine Lintner, Melissa Moore-Clingenpeel, Liza McCann, Nicola Ambrose, Mario Cortina-Borja, Juvenile Dermatomyositis Cohort and Biomarker Study (JCDBS), Prasad T. Oommen, Fabian Speth, Johannes-Peter Haas, Working Group “Juvenile Dermatomyositis” of the German Society for Paediatric and Adolescent Rheumatology (GKJR), Claudio Lavarello, Gabriella Giancane, Angela Pistorio, Lisa Rider, Rohit Aggarwal, Sheila K. Oliveira, Ruben Cuttica, Michel Fischbach, Gary Sterba, Karine Brochard, Frank Dressler, Patrizia Barone, Ruben Burgos-Vargas, Elizabeth Candell Chalom, Marine Desjonqueres, Graciela Espada, Anders Fasth, Stella Maris Garay, Rose-Marie Herbigneaux, Claire Hoyoux, Chantal Job Deslandre, Frederick W. Miller, Jiri Vencovsky, Erdal Sag, Gulsev Kale, Haluk Topaloglu, Beril Talim, Francesco Zulian, Tadej Avcin, Roberto Marini, Anne Pagnier, Michel Rodiere, Christine Soler, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Ana V. Villarreal, Nydia Acevedo, Yuridiana Ramirez, Enrique Faugier, Rocio Maldonado, Bita Arabshahi, John H. Lee, Ian Leibowitz, Lawrence O. Okong’o, Jo Wilmshurst, Monika Esser, Christiaan Scott, Ezgi Deniz Batu, Nagehan Emiroglu, Hafize Emine Sonmez, Gokcen Dilsa Tugcu, Zehra Serap Arici, Ebru Yalcin, Deniz Dogru, Ugur Ozcelik, Mithat Haliloglu, Nural Kiper, Masato Yashiro, Mutsuko Yamada, Toshihiko Yabuuchi, Tomonobu Kikkawa, Nobuyuki Nosaka, Yosuke Fujii, Yukie Saito, Hirokazu Tsukahara, Sulaiman M. Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-shukair, Zeyad El-Habahbeh, Abdullah Alsonbul, Aleksandra Szabat, Monika Chęć, Violetta Opoka-Winiarska, Biman Saikia, Ranjana W. Minz, Christine Arango, Clara Malagon, Maria D. P. Gomez, Angela C. Mosquera, Ricardo Yepez, Tatiana Gonzalez, Camilo Vargas, GRIP study group, Marta Balzarin, Biagio Castaldi, Elena Reffo, Francesca Sperotto, Giorgia Martini, Alessandra Meneghel, Ornella Milanesi, Ozgur Kasapçopur, Maria Teresa Terreri, Ekaterina Alexeeva, Maria Katsicas, Mikhail Kostik, Thomas Lehman, W.-Alberto Sifuentes-Giraldo, Vanessa Smith, Flavio Sztajnbok, Tadey Avcin, Maria Jose Santos, Dana Nemcova, Cristina Battagliotti, Liora Harel, Mahesh Janarthanan, Kathryn Torok, Nicola Helmus, Eileen Baildem, Michael Blakley, Kim Fligelstone, Antonia Kienast, Clare Pain, Amanda Saracino, Gabriele Simoni, Lisa Weibel, Maria K. Osminina, Nathalia A. Geppe, Olga V. Niconorova, Olesya V. Karashtina, Oksana V. Abbyasova, Olga V. Shpitonkova, Sinem Durmus, Hafize Uzun, Angela Mauro, Eleonora Fanti, Fabio Voller, Franca Rusconi, Fernando Garcia-Rodriguez, Ana V. Villarreal-Treviño, Angel J. Flores-Pineda, Paola B. Lara-Herrea, Diego R. Salinas-Encinas, Talia Diaz-Prieto, Maria R. Maldonado-Velazquez, Sarbelio Moreno-Espinosa, Enrique Faugier-Fuentes, Mirella Crapanzano, Ilaria Parissenti, Man S. Parihar, Pandiarajan Vignesh, ManojKumar Rohit, Kavitha Gopalan, Savita V. Attri, Alan Salama, David Jayne, Mark Little, Yulia Kostina, Galina Lyskina, Olga Shpitonkova, Alena Torbyak, Olga Shirinsky, Maria Francesca Gicchino, Maria Cristina Smaldone, Mario Diplomatico, Alma Nunzia Olivieri, C H. Spencer, Richard McClead, Hiren Patel, Chung-Yung Yu, Dita Cebecauerová, Tomáš Dallos, Edita Kabíčková, Martin Kynčl, Daniela Chroustová, Jozef Hoza, Dana Němcová, Vladimír Tesař, Pavla Doležalová, Tuncay Hazirolan, Fatih Ozaltin, Fabiola Almeida, Isabela H. Faria de Paula, Maíra M. Sampaio, Fernando N. Arita, Andressa G. Alves, Maria Carolina Santos, Eunice M. Okuda, Silvana B. Sacchetti, Fernanda Falcini, Marini Francesca, Gemma Lepri, Marco Matucci-Cerinic, Maria Luisa Brandi, Hakan Kisaoglu, Sema Misir, Selim Demir, Yuksel Aliyazicioglu, Mukaddes Kalyoncu, Carlos Eduardo Ramalho, Fabiola D. Almeida, Joan Calzada-Hernández, Rosa Bou, Estíbaliz Iglesias, Judith Sánchez-Manubens, Fredy Hermógenes Prada Martínez, Clara Giménez Roca, Sergi Borlan Fernández, Marek Bohm, Kamran Mahmood, Valentina Leone, Mark Wood, Ken-Ichi Yamaguchi, Satoshi Fujikawa, Working Group of Behçet’s Disease, Pediatric Rheumatology Association of Japan (PRAJ), Kyu Yeun Kim, Do Young Kim, Dong Soo Kim, Maka Ioseliani, Ivane Chkhaidze, Maia Lekishvili, Nana Tskhakaia, Shorena Tvalabeishvili, Aleksandre Kajrishvili, Maiko Takakura, Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Akihiro Yachie, Giovanni Corsello, Maryam Piram, Carla Maldini, Sandra Biscardi, Nathalie Desuremain, Catherine Orzechowski, Emilie Georget, Delphine Regnard, Isabelle Kone-Paut, Alfred Mahr, Mihaela Sparchez, Zeno Sparchez, Nydia Acevedo Silva, Ana V. Villarreal Treviño, Yuridiana Ramirez Loyola, Talia Diaz Prieto, Enrique Faugier Fuentes, Maria D. R. Maldonado Velazquez, Pilar Perez, Sagar Bhattad, Ranjana Minz, Jitendra Shandilya, Pediatric Allergy and Immunology Unit, PGIMER, Chandigarh, Ana Villarreal, Yuridiana Ramírez, Zeynep Birsin Özçakar, Suat Fitoz, Fatos Yalcinkaya, Annacarin Horne, Francesca Minoia, Francesca Bovis, Sergio Davi, Priyankar Pal, Kimo Stein, Sandra Enciso, Michael Jeng, Despoina Maritsi, Randy C. Cron, Anne Thorwarth, Sae Lim von Stuckrad, Angela Rösen-Wolff, Hella Luksch, Patrick Hundsdoerfer, Peter Krawitz, Nuray Aktay Ayaz, Doğan Simsek, Şebnem Sara Kılıc, Emine Sonmez, Aysenur Pac Kisaarslan, Ozge Altug Gucenmez, Z. Serap Arıcı, Fatih Kelesoglu, Zelal Ekinci Ekinci, Maria Miranda-Garcia, Carolin Pretzer, Michael Frosch, F. Gohar, Angela McArdle, Niamh Callan, Belinda Hernandez, Miha Lavric, Oliver FitzGerald, Stephen R. Pennington, Joachim Peitz, Joern Kekow, Ariane Klein, Anna C. Schulz, Frank Weller-Heinemann, Anton Hospach, J-Peter Haas, BIKER collaborative group, Karen Put, Jessica Vandenhaute, Anneleen Avau, Annemarie van Nieuwenhuijze, Ellen Brisse, Tim Dierckx, Omer Rutgeerts, Josselyn E. Garcia-Perez, Jaan Toelen, Mark Waer, Georges Leclercq, An Goris, Johan Van Weyenbergh, Adrian Liston, Patrick Matthys, Carine H. Wouters, Yasuo Nakagishi, Michael J. Ombrello, Victoria Arthur, Anne Hinks, Patricia Woo, International Childhood Arthritis Genetics (INCHARGE) Consortium, Barbara Stanimirovic, Biljana Djurdjevic-Banjac, Olivera Ljuboja, Boris Hugle, MArgarita Onoufriou, Olga Vougiouka, Kenza Bouayed, Sanae El Hani, Imane Hafid, Nabiha Mikou, Nunu Shelia, Mari Laan, Jaanika Ilisson, and Chris Pruunsild

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
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72. Laboratory rivers: Lacey's law, threshold theory, and channel stability

Author

F. Métivier, E. Lajeunesse, and O. Devauchelle

Subjects
Dynamic and structural geology, QE500-639.5
Abstract

More than a century of experiments have demonstrated that many features of natural rivers can be reproduced in the laboratory. Here, we revisit some of these experiments to cast their results into the framework of the threshold-channel theory developed by Glover and Florey (1951). In all the experiments we analyze, the typical size of the channel conforms to this theory, regardless of the river's planform (single-thread or braiding). In that respect, laboratory rivers behave exactly like their natural counterpart. Using this finding, we reinterpret experiments by Stebbings (1963). We suggest that sediment transport widens the channel until it reaches a limit width, beyond which it destabilizes into a braided river. If confirmed, this observation would explain the remarkable scarcity of single-thread channels in laboratory experiments.

Published
2017
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74. Molecular Characterization of Monocyte Subsets Reveals Specific and Distinctive Molecular Signatures Associated With Cardiovascular Disease in Rheumatoid Arthritis

Author

Patricia Ruiz-Limon, Rafaela Ortega-Castro, Nuria Barbarroja, Carlos Perez-Sanchez, Christophe Jamin, Alejandra Maria Patiño-Trives, Maria Luque-Tevar, Alejandro Ibáñez-Costa, Laura Perez-Sanchez, Iván Arias de la Rosa, MaCarmen Abalos-Aguilera, Yolanda Jimenez-Gomez, Jerusalem Calvo-Gutierrez, Pilar Font, Alejandro Escudero-Contreras, Marta E. Alarcon-Riquelme, Eduardo Collantes-Estevez, Chary López-Pedrera, the PRECISESADS Clinical Consortium and Flow Cytometry Study Group, C Marañón, L Le Lann, N Varela, B Muchmore, A Dufour, Alvarez, C Carlo Montserrat Chizzolini, E, NB De Langhe, CL-P, V Gerl, A De Groof, J Ducreux, E Trombetta, T Li, D Alvarez-Errico, S Rao, JO Pers, L Beretta, R AguilarQuesada, MA Aguirre-Zamorano, JL Callejas Rubio, MC Castro-Villegas, R Cervera, C Chizzolini, E Collantes, D Cornec, E De Langhe, V Devauchelle-Pensec, AE-C, G Espinosa, MC Fernández Roldán, T Gomes Anjos, F Hiepe, I Jiménez Moleón, S Jousse-Joulin, B Lauwerys, A López-Berrio, R Lories, J Marovac, PL Meroni, B Miranda, H Navarro-Linares, R Ortega-Castro, N Ortego, E Ramón Garrido, E Raya, R Ríos Fernández, I Rodríguez-Pintó, and A Saraux

Subjects
rheumatoid arthritis, cardiovascular disease, monocyte subsets, microRNAs, gene profile, Immunologic diseases. Allergy, RC581-607
Abstract

Objectives: This study, developed within the Innovative Medicines Initiative Joint Undertaking project PRECISESADS framework, aimed at functionally characterize the monocyte subsets in RA patients, and analyze their involvement in the increased CV risk associated with RA.Methods: The frequencies of monocyte subpopulations in the peripheral blood of 140 RA patients and 145 healthy donors (HDs) included in the PRECISESADS study were determined by flow cytometry. A second cohort of 50 RA patients and 30 HDs was included, of which CD14+ and CD16+ monocyte subpopulations were isolated using immuno-magnetic selection. Their transcriptomic profiles (mRNA and microRNA), proinflammatory patterns and activated pathways were evaluated and related to clinical features and CV risk. Mechanistic in vitro analyses were further performed.Results: CD14++CD16+ intermediate monocytes were extended in both cohorts of RA patients. Their increased frequency was associated with the positivity for autoantibodies, disease duration, inflammation, endothelial dysfunction and the presence of atheroma plaques, as well as with the CV risk score. CD14+ and CD16+ monocyte subsets showed distinctive and specific mRNA and microRNA profiles, along with specific intracellular signaling activation, indicating different functionalities. Moreover, that specific molecular profiles were interrelated and associated to atherosclerosis development and increased CV risk in RA patients. In vitro, RA serum promoted differentiation of CD14+CD16− to CD14++CD16+ monocytes. Co-culture with RA-isolated monocyte subsets induced differential activation of endothelial cells.Conclusions: Our overall data suggest that the generation of inflammatory monocytes is associated to the autoimmune/inflammatory response that mediates RA. These monocyte subsets, -which display specific and distinctive molecular signatures- might promote endothelial dysfunction and in turn, the progression of atherosclerosis through a finely regulated process driving CVD development in RA.

Published
2019
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75. Peripheral-blood b-cell subset disturbances in inflammatory joint diseases induced by Tropheryma whipplei.

Author

Maëlle Le Goff, Divi Cornec, Dewi Guellec, Thierry Marhadour, Valérie Devauchelle-Pensec, Sandrine Jousse-Joulin, Marion Herbette, Jean Michel Cauvin, Clara Le Guillou, Yves Renaudineau, Christophe Jamin, Jacques Olivier Pers, and Alain Saraux

Subjects
Medicine, Science
Abstract

OBJECTIVE:To look for abnormalities in circulating B-cell subsets in patients with rheumatic symptoms of Whipple's disease (WD). METHOD:Consecutive patients seen between 2010 and 2016 for suspected inflammatory joint disease were identified retrospectively. Results of standardized immunological and serological tests and of peripheral-blood B-cell and T-cell subset analysis by flow cytometry were collected. Patients with criteria suggesting WD underwent PCR testing for Tropheryma whipplei, and those with diagnosis of WD (cases) were compared to those without diagnosis (controls). We used ROC curve analysis to evaluate the diagnostic value of flow cytometry findings for WD. RESULTS:Among 2917 patients seen for suspected inflammatory joint disease, 121 had suspected WD, including 9 (9/121, 7.4%) confirmed WD. Proportions of T cells and NK cells were similar between suspected and confirmed WD, whereas cases had a lower proportion of circulating memory B cells (IgD-CD38low, 18.0%±9.7% vs. 26.0%±14.2%, P = 0.041) and higher ratio of activated B cells over memory B cells (4.4±2.0 vs. 2.9±2.2, P = 0.023). Among peripheral-blood B-cells, the proportion of IgD+CD27- naive B cells was higher (66.2%±18.2% vs. 54.6%±18.4%, P = 0.047) and that of IgD-CD27+ switched memory B cells lower (13.3%±5.7% vs. 21.4%±11.9%, P = 0.023), in cases vs. controls. The criterion with the best diagnostic performance was a proportion of IgD+CD27- naive B cells above 70.5%, which had 73% sensitivity and 80% specificity. CONCLUSION:Our study provides data on peripheral-blood B-cell disturbances that may have implications for the diagnosis and pathogenetic understanding of WD.

Published
2019
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78. Geometry of meandering and braided gravel-bed threads from the Bayanbulak Grassland, Tianshan, P. R. China

Author

F. Métivier, O. Devauchelle, H. Chauvet, E. Lajeunesse, P. Meunier, K. Blanckaert, P. Ashmore, Z. Zhang, Y. Fan, Y. Liu, Z. Dong, and B. Ye

Subjects
Dynamic and structural geology, QE500-639.5
Abstract

The Bayanbulak Grassland, Tianshan, P. R. China, is located in an intramontane sedimentary basin where meandering and braided gravel-bed rivers coexist under the same climatic and geological settings. We report and compare measurements of the discharge, width, depth, slope and grain size of individual threads from these braided and meandering rivers. Both types of threads share statistically indistinguishable regime relations. Their depths and slopes compare well with the threshold theory, but they are wider than predicted by this theory. These findings are reminiscent of previous observations from similar gravel-bed rivers. Using the scaling laws of the threshold theory, we detrend our data with respect to discharge to produce a homogeneous statistical ensemble of width, depth and slope measurements. The statistical distributions of these dimensionless quantities are similar for braided and meandering threads. This suggests that a braided river is a collection of intertwined threads, which individually resemble those of meandering rivers. Given the environmental conditions in Bayanbulak, we furthermore hypothesize that bedload transport causes the threads to be wider than predicted by the threshold theory.

Published
2016
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83. Likely Pathogenic Variants in One Third of Non-Syndromic Discontinuous Cleft Lip and Palate Patients

Author

Bénédicte Demeer, Nicole Revencu, Raphael Helaers, Cica Gbaguidi, Stéphanie Dakpe, Geneviève François, Bernard Devauchelle, Bénédicte Bayet, and Miikka Vikkula

Subjects
discontinuous cleft, fgfr1, dlg1, wes (whole exome sequencing), Genetics, QH426-470
Abstract

Oral clefts are composed of cleft of the lip, cleft of the lip and palate, or cleft of the palate, and they are associated with a wide range of expression and severity. When cleft of the palate is associated with cleft of the lip with preservation of the primary palate, it defines an atypical phenotype called discontinuous cleft. Although this phenotype may represent 5% of clefts of the lip and/or palate (CLP), it is rarely specifically referred to and its pathophysiology is unknown. We conducted whole exome sequencing (WES) and apply a candidate gene approach to non-syndromic discontinuous CLP individuals in order to identify genes and deleterious variants that could underlie this phenotype. We discovered loss-of-function variants in two out of the seven individuals, implicating FGFR1 and DLG1 genes, which represents almost one third of this cohort. Whole exome sequencing of clinically well-defined subgroups of CLP, such as discontinuous cleft, is a relevant approach to study CLP etiopathogenesis. It could facilitate more accurate clinical, epidemiological and fundamental research, ultimately resulting in better diagnosis and care of CLP patients. Non-syndromic discontinuous cleft lip and palate seems to have a strong genetic basis.

Published
2019
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84. Stomach-brain synchrony reveals a novel, delayed-connectivity resting-state network in humans

Author

Ignacio Rebollo, Anne-Dominique Devauchelle, Benoît Béranger, and Catherine Tallon-Baudry

Subjects
resting-state networks, fMRI, phase synchrony, body maps, gastric rhythm, Medicine, Science, Biology (General), QH301-705.5
Abstract

Resting-state networks offer a unique window into the brain’s functional architecture, but their characterization remains limited to instantaneous connectivity thus far. Here, we describe a novel resting-state network based on the delayed connectivity between the brain and the slow electrical rhythm (0.05 Hz) generated in the stomach. The gastric network cuts across classical resting-state networks with partial overlap with autonomic regulation areas. This network is composed of regions with convergent functional properties involved in mapping bodily space through touch, action or vision, as well as mapping external space in bodily coordinates. The network is characterized by a precise temporal sequence of activations within a gastric cycle, beginning with somato-motor cortices and ending with the extrastriate body area and dorsal precuneus. Our results demonstrate that canonical resting-state networks based on instantaneous connectivity represent only one of the possible partitions of the brain into coherent networks based on temporal dynamics.

Published
2018
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85. Morphology of the Kosi megafan channels

Author

K. Gaurav, F. Métivier, O. Devauchelle, R. Sinha, H. Chauvet, M. Houssais, and H. Bouquerel

Subjects
Dynamic and structural geology, QE500-639.5
Abstract

We report on a new set of measurements on the morphology of braided and meandering threads of the Kosi megafan, North Bihar, India. All threads develop on a uniform sandy sediment and under a similar climate. The data set is composed of the width, depth, water discharge and grain size of 51 threads. Downstream slopes and sinuosity are also available. Using this data set, we show that braided and meandering threads share common hydraulic geometries. We then use the threshold theory to explain why the aspect ratio of threads is almost naturally detrended, and rescale the data according to this theory. As expected, the rescaled dimensions of braided and meandering threads are weakly correlated to water discharge. We propose that the large dispersion observed, which is common to meandering and braided threads, is the signature of sediment transport, vegetation or cohesion effects.

Published
2015
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86. Author Correction: Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome

Author

Vabres, Pierre, Sorlin, Arthur, Kholmanskikh, Stanislav S., Demeer, Bénédicte, St-Onge, Judith, Duffourd, Yannis, Kuentz, Paul, Courcet, Jean-Benoît, Carmignac, Virginie, Garret, Philippine, Bessis, Didier, Boute, Odile, Bron, Alain, Captier, Guillaume, Carmi, Esther, Devauchelle, Bernard, Geneviève, David, Gondry-Jouet, Catherine, Guibaud, Laurent, Lafon, Arnaud, Mathieu-Dramard, Michèle, Thevenon, Julien, Dobyns, William B., Bernard, Geneviève, Polubothu, Satyamaanasa, Faravelli, Francesca, Kinsler, Veronica A., Thauvin, Christel, Faivre, Laurence, Ross, M. Elizabeth, and Rivière, Jean-Baptiste

Published
2019
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89. Mucormycosis in Burn Patients

Author

Pauline Devauchelle, Mathieu Jeanne, and Emilie Fréalle

Subjects
mucormycosis, burn, cutaneous, qPCR, outbreak, molecular typing, environmental source, nosocomial, Saksenaea, Apophysomyces, Biology (General), QH301-705.5
Abstract

Patients with extensive burns are an important group at risk for cutaneous mucormycosis. This study aimed to perform a systematic review of all reported mucormycosis cases in burn patients from 1990 onward. A Medline search yielded identification of 7 case series, 3 outbreaks, and 25 individual cases reports. The prevalence reached 0.04%–0.6%. The median age was 42–48 in the case series and outbreaks, except for the studies from military centers (23.5–32.5) and in individual reports (29.5). The median total body surface area reached 42.5%–65%. Various skin lesions were described, none being pathognomonic: the diagnosis was mainly reached because of extensive necrotic lesions sometimes associated with sepsis. Most patients were treated with systemic amphotericin B or liposomal amphotericin B, and all underwent debridement and/or amputation. Mortality reached 33%–100% in the case series, 29%–62% during outbreaks, and 40% in individual cases. Most patients were diagnosed using histopathology and/or culture. Mucorales qPCR showed detection of circulating DNA 2–24 days before the standard diagnosis. Species included the main clinically relevant mucorales (i.e., Mucor, Rhizopus, Absidia/Lichtheimia, Rhizomucor) but also more uncommon mucorales such as Saksenaea or Apophysomyces. Contact with soil was reported in most individual cases. Bandages were identified as the source of contamination in two nosocomial outbreaks.

Published
2019
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90. Tracer dispersion in bedload transport

Author

E. Lajeunesse, O. Devauchelle, M. Houssais, and G. Seizilles

Subjects
Science, Geology, QE1-996.5, Dynamic and structural geology, QE500-639.5
Abstract

Bedload particles entrained by rivers tends to disperse as they move downstream. In this paper, we use the erosion-deposition model of Charru et al. (2004) to describe the velocity and the spreading of a plume of tracer particles. We restrict our analysis to steady-state transport above a flat bed of uniform sediment. The transport of tracer particles is then controlled by downstream advection and particle exchange with the immobile bed. After a transitional regime dominated by initial conditions, the evolution of a plume of markers tends asymptotically towards classical advection-diffusion: its average position grows linearly with time, whereas it spreads like the square root of time.

Published
2013
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91. Sentence Syntax and Content in the Human Temporal Lobe: An fMRI Adaptation Study in Auditory and Visual Modalities

Author

Devauchelle, Anne-Dominique, Oppenheim, Catherine, and Rizzi, Luigi

Abstract

Priming effects have been well documented in behavioral psycholinguistics experiments: The processing of a word or a sentence is typically facilitated when it shares lexico-semantic or syntactic features with a previously encountered stimulus. Here, we used fMRI priming to investigate which brain areas show adaptation to the repetition of a sentence's content or syntax. Participants read or listened to sentences organized in series which could or not share similar syntactic constructions and/or lexico-semantic content. The repetition of lexico-semantic content yielded adaptation in most of the temporal and frontal sentence processing network, both in the visual and the auditory modalities, even when the same lexico-semantic content was expressed using variable syntactic constructions. No fMRI adaptation effect was observed when the same syntactic construction was repeated. Yet behavioral priming was observed at both syntactic and semantic levels in a separate experiment where participants detected sentence endings. We discuss a number of possible explanations for the absence of syntactic priming in the fMRI experiments, including the possibility that the conglomerate of syntactic properties defining "a construction" is not an actual object assembled during parsing.

Published
2009
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93. High-Grade Salivary-Gland Involvement, Assessed by Histology or Ultrasonography, Is Associated with a Poor Response to a Single Rituximab Course in Primary Sjögren's Syndrome: Data from the TEARS Randomized Trial.

Author

Divi Cornec, Sandrine Jousse-Joulin, Sebastian Costa, Thierry Marhadour, Pascale Marcorelles, Jean-Marie Berthelot, Eric Hachulla, Pierre-Yves Hatron, Vincent Goeb, Olivier Vittecoq, Emmanuel Nowak, Jacques-Olivier Pers, Valérie Devauchelle-Pensec, and Alain Saraux

Subjects
Medicine, Science
Abstract

PURPOSE:To determine whether the severity of salivary-gland involvement, assessed using salivary gland ultrasonography [SGUS], histological focus score, or the unstimulated whole salivary flow [UWSF], was associated with the response to rituximab in patients with primary Sjögren's syndrome [pSS]. MATERIALS AND METHODS:Among the 120 patients with pSS enrolled in the randomised TEARS trial of rituximab versus placebo, 35 underwent either centralised minor salivary-gland biopsy or SGUS at inclusion. The echostructure of each parotid and submandibular gland was graded on a scale of 0 to 4. Histologic minor salivary gland involvement was assessed by the focus score. Among rituximab-treated patients with available data (n = 14), half met the Sjögren's Syndrome Responder Index [SSRI]-30 definition of a response at week 24. RESULTS:The SGUS score correlated positively to the focus score [r = 0.61] and negatively to the UWSF [r = -0.68]. The focus score was not correlated to the UWSF. The median total SGUS grade at inclusion was 9 [6-11] in responders versus 16 [11-16] in non-responders [p = 0.04]. The proportion of SSRI-30 responders was 0% among patients with SGUS grade 4 and 88% among those with SGUS grade ≤3. Low baseline SGUS scores were associated with sicca-related outcomes improvement, but not with fatigue or biological improvement. Median baseline focus score was 0.3 [0.0-1.3] in the responders versus 4.0 [2.7-5.3] in the non-responders [p = 0.02]. Baseline UWSF was not associated with the response rate. CONCLUSION:In patients with pSS, the highest SGUS grade or a high histological focus score is associated with absence of a response to a single rituximab course after 6 months. Further studies, including more patients and different treatment strategies, are required to confirm the clinical utility of these potential biomarkers in pSS.

Published
2016
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97. Premalignant and Malignant Skin Lesions in Two Recipients of Vascularized Composite Tissue Allografts (Face, Hands)

Author

Jean Kanitakis, Palmina Petruzzo, Aram Gazarian, Sylvie Testelin, Bernard Devauchelle, Lionel Badet, Jean-Michel Dubernard, and Emmanuel Morelon

Subjects
Surgery, RD1-811
Abstract

Recipients of solid organ transplants (RSOT) have a highly increased risk for developing cutaneous premalignant and malignant lesions, favored by the lifelong immunosuppression. Vascularized composite tissue allografts (VCA) have been introduced recently, and relevant data are sparse. Two patients with skin cancers (one with basal cell carcinoma and one with squamous cell carcinomas) have been so far reported in this patient group. Since 2000 we have been following 9 recipients of VCA (3 face, 6 bilateral hands) for the development of rejection and complications of the immunosuppressive treatment. Among the 9 patients, one face-grafted recipient was diagnosed with nodular-pigmented basal cell carcinoma of her own facial skin 6 years after graft, and one patient with double hand allografts developed disseminated superficial actinic porokeratosis, a potentially premalignant dermatosis, on her skin of the arm and legs. Similar to RSOT, recipients of VCA are prone to develop cutaneous premalignant and malignant lesions. Prevention should be applied through sun-protective measures, regular skin examination, and early treatment of premalignant lesions.

Published
2015
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98. Which and How Many Patients Should Be Included in Randomised Controlled Trials to Demonstrate the Efficacy of Biologics in Primary Sjögren's Syndrome?

Author

Valérie Devauchelle-Pensec, Jacques-Eric Gottenberg, Sandrine Jousse-Joulin, Jean-Marie Berthelot, Aleth Perdriger, Eric Hachulla, Pierre Yves Hatron, Xavier Puechal, Véronique Le Guern, Jean Sibilia, Laurent Chiche, Vincent Goeb, Olivier Vittecoq, Claire Larroche, Anne Laure Fauchais, Gilles Hayem, Jacques Morel, Charles Zarnitsky, Jean Jacques Dubost, Philippe Dieudé, Jacques Olivier Pers, Divi Cornec, Raphaele Seror, Xavier Mariette, Emmanuel Nowak, and Alain Saraux

Subjects
Medicine, Science
Abstract

OBJECTIVE:The goal of this study was to determine how the choice of the primary endpoint influenced sample size estimates in randomised controlled trials (RCTs) of treatments for primary Sjögren's syndrome (pSS). METHODS:We reviewed all studies evaluating biotechnological therapies in pSS to identify their inclusion criteria and primary endpoints. Then, in a large cohort (ASSESS), we determined the proportion of patients who would be included in RCTs using various inclusion criteria sets. Finally, we used the population of a large randomised therapeutic trial in pSS (TEARS) to assess the impact of various primary objectives and endpoints on estimated sample sizes. These analyses were performed only for the endpoints indicating greater efficacy of rituximab compared to the placebo. RESULTS:We identified 18 studies. The most common inclusion criteria were short disease duration; systemic involvement; high mean visual analogue scale (VAS) scores for dryness, pain, and fatigue; and biological evidence of activity. In the ASSESS cohort, 35 percent of patients had recent-onset disease (lower than 4 years), 68 percent systemic manifestations, 68 percent high scores on two of three VASs, and 52 percent biological evidence of activity. The primary endpoints associated with the smallest sample sizes (nlower than 200) were a VAS dryness score improvement higher to 20 mm by week 24 or variable improvements (10, 20, or 30 mm) in fatigue VAS by week 6 or 16. For patients with systemic manifestations, the ESSDAI change may be the most logical endpoint, as it reflects all domains of disease activity. However, the ESSDAI did not improve significantly with rituximab therapy in the TEARS study. Ultrasound score improvement produced the smallest sample size estimate in the TEARS study. CONCLUSION:This study provides valuable information for designing future RCTs on the basis of previously published studies. Previous RCTs used inclusion criteria that selected a small part of the entire pSS population. The endpoint was usually based on VASs assessing patient complaints. In contrast to VAS dryness cut-offs, VAS fatigue cut-offs did not affect estimated sample sizes. SGUS improvement produced the smallest estimated sample size. Further studies are required to validate standardised SGUS modalities and assessment criteria. Thus, researchers should strive to develop a composite primary endpoint and to determine its best cut-off and assessment time point.

Published
2015
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100. The Facial Graft

Author

Devauchelle, Bernard, Dakpe, Stephanie, and Testelin, Sylvie

Abstract

Although there have been numerous scientific papers reporting on composite allotransplantation of the face, few have been about assessment and evolution of the functional benefit for patients in terms of soft tissue mobility, movement coordination, and face expressions, even fewer on the choice of the surgical procedures to restore motricity. On some videos and interviews, it is possible to analyze significative motricity. Of course that has been voluntary motricity, not emotional motricity. The data confirmed that the result on the complexity of the expression of the face is not great, providing opportunity to reflect on the question of nerve regeneration.

Published
2023
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