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102. A genome-wide association study of rheumatoid arthritis without antibodies against citrullinated peptides

Author

Bossini-Castillo, L, de Kovel, C, Kallberg, H, van ‘t Slot, R, Italiaander, A, Coenen, M, Tak, P P, Posthumus, M D, Wijmenga, C, Huizinga, T, van der Helm-van Mil, A H M, Stoeken-Rijsbergen, G, Rodriguez-Rodriguez, Luis, Balsa, Alejandro, González-Álvaro, Isidoro, González-Gay, Miguel Ángel, Gómez-Vaquero, Carmen, Franke, B, Vermeulen, S, van der Horst-Bruinsma, I E, Dijkmans, B A C, Wolbink, G J, Ophoff, R A, Maehlen, M T, van Riel, P, Merriman, M, Klareskog, L, Lie, B A, Merriman, T, Crusius, J B A, Brouwer, E, Martin, J, de Vries, N, Toes, R, Padyukov, L, and Koeleman, B P C

Published
2015
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103. A wearable patch based remote early warning score (REWS) in major abdominal cancer surgery patients.

Author

van der Stam, Jonna A., Mestrom, Eveline H.J., Nienhuijs, Simon W., de Hingh, Ignace H.J.T., Boer, Arjen-Kars, van Riel, Natal A.W., de Groot, Koen T.J., Verhaegh, Wim, Scharnhorst, Volkher, and Bouwman, R. Arthur

Subjects
EARLY warning score, ONCOLOGIC surgery, ABDOMINAL surgery, CANCER patients, CLINICAL deterioration
Abstract

The shift toward remote patient monitoring methods to detect clinical deterioration requires testing of wearable devices in real-life clinical settings. This study aimed to develop a remote early warning scoring (REWS) system based on continuous measurements using a wearable device, and compare its diagnostic performance for the detection of deterioration to the diagnostic performance of the conventional modified early warning score (MEWS). The study population of this prospective, single center trial consisted of patients who underwent major abdominal cancer surgery and were monitored using routine in-hospital spotcheck measurements of the vital parameters. Heart and respiratory rates were measured continuously using a wireless accelerometer patch (HealthDot). The prediction by MEWS of deterioration toward a complication graded Clavien-Dindo of 2 or higher was compared to the REWS derived from continuous measurements by the wearable patch. A total of 103 patients and 1909 spot-check measurements were included in the analysis. Postoperative deterioration was observed in 29 patients. For both EWS systems, the sensitivity (MEWS: 0.20 95% CI: [0.13–0.29], REWS: 0.20 95% CI: [0.13–0.29]) and specificity (MEWS: 0.96 95% CI: [0.95–0.97], REWS: 0.96 95% CI: [0.95–0.97]) were assessed. The diagnostic value of the REWS method, based on continuous measurements of the heart and respiratory rates, is comparable to that of the MEWS in patients following major abdominal cancer surgery. The wearable patch could detect the same amount of deteriorations, without requiring manual spot check measurements. [ABSTRACT FROM AUTHOR]

Published
2023
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106. Supplement to: Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis

Author

van Bon, L., Affandi, A. J., Broen, J., Christmann, R. B., Marijnissen, R. J., Stawski, L., Farina, G. A., Stifano, G., Mathes, A. L., Cossu, M., York, M., Collins, C., Wenink, M., Huijbens, R., Hesselstrand, R., Saxne, T., DiMarzio, M., Wuttge, D., Agarwal, S. K., Reveille, J. D., Assassi, S., Mayes, M., Deng, Y., Drenth, J. P.H., de Graaf, J., den Heijer, M., Kallenberg, C. G.M., Bijl, M., Loof, A., van den Berg, W. B., Joosten, L. A.B., Smith, V., de Keyser, F., Scorza, R., Lunardi, C., van Riel, P. L.C.M., Vonk, M., van Heerde, W., Meller, S., Homey, B., Beretta, L., Roest, M., Trojanowska, M., Lafyatis, R., and Radstake, T. R.D.J.

Published
2014

119. Three Instances of the Good in Proclus

Author

Oosthout, Arthur and Van Riel, Gerd

Abstract

Plato’s Philebusfamously combines a deliberation on the virtuous life as a balancing act between prudence and pleasure with a theory of the composition of mixtures from limit and limitedness. The latter aspect of the dialogue is used by the Neoplatonic philosopher Proclus as a basis for his own metaphysical analysis of the ultimate first principle, the One, and the manner in which it produces all things which exist. Multiple scholarly analyses have been provided of Proclus’ use of the Phileban theory of limit and the unlimited for his description of the first principle’s creation process. However, this paper shows that Proclus reads the Philebusnot merely for its discussion of limit and unlimited, but also for its description of the good life. After all, the Neoplatonic One is also the Good from Plato’s Republic. In Phil. 20d1–11, Socrates suggest that the good is perfect, sufficient, and desirable. Proclus’ Commentary on the Republicand Platonic Theologyreveal that these three aspects of the good provide Proclus with a framework to describe the Neoplatonic first principle quaGood. In Proclus’ view, the three Phileban concepts constitute the base elements of the good which reappear in all of its manifestations, and thus link the good in us to the transcendent and unqualified Good: the desirable element represents the good’s singularity and its position as the centre around which all beings circle; the sufficient element represents the non-reciprocal bestowal of existence and generative power by any form of the good upon its participants; lastly, the perfect element reverts all creatures to the good in which they participate, even when not all genera of beings in the Neoplatonic universe are equally capable of such participation. Furthermore, Proclus uses the description of the good life as not merely intellectual from the Philebusto fortify his arguments in disagreements with other Neoplatonic commentators regarding the transcendence of the Good over the intelligible principles, and conversely employs the Neoplatonic definition of the first principle to affirm the ethics of the Platonic dialogue. Thus, although Proclus’ primary goal in reading the Philebusis to obtain knowledge of the ultimate first principle, Socrates’ analysis of the virtuous life in the dialogue is just as important for his purposes as the theory of mixtures.

Published
2023
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121. Diabetes mellitus and cardiovascular risk management in patients with rheumatoid arthritis: An international audit

Author

Semb, A.G. Rollefstad, S. Ikdahl, E. Wibetoe, G. Sexton, J. Crowson, C. Van Riel, P. Kitas, G. Graham, I. Rantapää-Dahlqvist, S. Karpouzas, G.A. Myasoedova, E. Gonzalez-Gay, M.A. Sfikakis, P.P. Tektonidou, M.G.G. Lazarini, A. Vassilopoulos, D. Kuriya, B. Hitchon, C. Stoenoiu, M.S. Durez, P. Pascual-Ramos, V. Galarza-Delgado, D.A. Faggiano, P. Misra, D.P. Borg, A.A. Mu, R. Mirrakhimov, E.M. Gheta, D. Douglas, K. Agarwal, V. Myasoedova, S. Krougly, L. Valentinovna Popkova, T. TuchyÅ ová, A. Tomcik, M. Vrablik, M. Lastuvka, J. Horak, P. Medkova, H.K. Kerola, A.M.

Abstract

Aim The objective was to examine the prevalence of atherosclerotic cardiovascular disease (ASCVD) and its risk factors among patients with RA with diabetes mellitus (RA-DM) and patients with RA without diabetes mellitus (RAwoDM), and to evaluate lipid and blood pressure (BP) goal attainment in RA-DM and RAwoDM in primary and secondary prevention. Methods The cohort was derived from the Survey of Cardiovascular Disease Risk Factors in Patients with Rheumatoid Arthritis from 53 centres/19 countries/3 continents during 2014-2019. We evaluated the prevalence of cardiovascular disease (CVD) among RA-DM and RAwoDM. The study population was divided into those with and without ASCVD, and within these groups we compared risk factors and CVD preventive treatment between RA-DM and RAwoDM. Results The study population comprised of 10 543 patients with RA, of whom 1381 (13%) had DM. ASCVD was present in 26.7% in RA-DM compared with 11.6% RAwoDM (p

Published
2021

122. EULAR definition of difficult-to-treat rheumatoid arthritis

Author

Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Leerstoel Geenen, and Clinical Psychology (overkoepelend voor heel KP)

Subjects
musculoskeletal diseases, medicine.medical_specialty, rheumatoid, Advisory Committees, immune system diseases, Immunology, Drug Resistance, Arthritis, Biochemistry, General Biochemistry, Genetics and Molecular Biology, Healthcare improvement science Radboud Institute for Health Sciences [Radboudumc 18], Arthritis, Rheumatoid, All institutes and research themes of the Radboud University Medical Center, Quality of life, Rheumatology, Stakeholder Participation, Synovitis, Terminology as Topic, Dmard therapy, medicine, Humans, Immunology and Allergy, Treatment Failure, Intensive care medicine, skin and connective tissue diseases, Glucocorticoids, Biological Products, business.industry, Task force, Biochemistry, Genetics and Molecular Biology(all), Recommendation, medicine.disease, Clinical trial, Europe, arthritis, Rheumatoid arthritis, Antirheumatic Agents, Practice Guidelines as Topic, Disease Progression, Drug Therapy, Combination, business, synovitis, Rheumatism, Genetics and Molecular Biology(all)
Abstract

BackgroundDespite treatment according to the current management recommendations, a significant proportion of patients with rheumatoid arthritis (RA) remain symptomatic. These patients can be considered to have ‘difficult-to-treat RA’. However, uniform terminology and an appropriate definition are lacking.ObjectiveThe Task Force in charge of the “Development of EULAR recommendations for the comprehensive management of difficult-to-treat rheumatoid arthritis” aims to create recommendations for this underserved patient group. Herein, we present the definition of difficult-to-treat RA, as the first step.MethodsThe Steering Committee drafted a definition with suggested terminology based on an international survey among rheumatologists. This was discussed and amended by the Task Force, including rheumatologists, nurses, health professionals and patients, at a face-to-face meeting until sufficient agreement was reached (assessed through voting).ResultsThe following three criteria were agreed by all Task Force members as mandatory elements of the definition of difficult-to-treat RA: (1) Treatment according to European League Against Rheumatism (EULAR) recommendation and failure of ≥2 biological disease-modifying antirheumatic drugs (DMARDs)/targeted synthetic DMARDs (with different mechanisms of action) after failing conventional synthetic DMARD therapy (unless contraindicated); (2) presence of at least one of the following: at least moderate disease activity; signs and/or symptoms suggestive of active disease; inability to taper glucocorticoid treatment; rapid radiographic progression; RA symptoms that are causing a reduction in quality of life; and (3) the management of signs and/or symptoms is perceived as problematic by the rheumatologist and/or the patient.ConclusionsThe proposed EULAR definition for difficult-to-treat RA can be used in clinical practice, clinical trials and can form a basis for future research.

Published
2021

127. Novel Approach to Estimate Osteoarthritis Progression: Use of the Reliable Change Index in the Evaluation of Joint Space Loss

Author

Parsons, Camille M., Judge, Andrew, Leyland, Kirsten, Bruyere, Olivier, Dop, Florence Petit, Chapurlat, Roland, Reginster, Jean-yves, Edwards, Mark H., Dennison, Elaine M., Cooper, Cyrus, Inskip, Hazel, Reginster, J. -y., Christiansen, C., Delmas, P., Genant, H., Zacher, J., Bellamy, N., Speirs, C., Breart, G., Meyer, O., Gensburger, D., Arlot, M., Roux, J. -P., Deroisy, R., Sambrook, P., Leeb, B., Verbruggen, A., Bensen, W. G., Hala, T., Holm−bentzen, M., Valter, I., Chevalier, X., Swoboda, B., Adami, S., Kloppenburg, M., Grazuleviciute, E., Badurski, J., Branco, J., Nasonov, E., Navarro, F., Spector, T., Barnsley, L., Hall, S., Jones, G., Klestov, A., March, L., Nash, P., Romas, E., Will, R., Erlacher, L., Leeb, F. B., Resch, H., Rainer, F., Zamani, O., Appelboom, T., Devogelaer, J. P., Kvasz, A., Raeman, F., Beaulieu, A. D., Brown, J., Cividino, A. A., Morin, F., Olszynski, W. P., Raynauld, J. P., Thorne, J. C., Pavelka, K., Alexandersen, P., Hoeck, H. C., Holm-Bentzen, M., Lundqvist, P., Aim, L., Audouy, P., Beaunier, P., Benhamou, C. L., Berenbaum, F., Chabaud, E., Cohen-Solal, M., Delbecq, D., Euller-Ziegler, L., Fardellone, Patrice, Hilliquin, P., Jacquety, E., Jude, N., Lechevalier, D., Mouchet, J. C., Richette, P., Sainte Lorette, E., Schaeverbeke, T., Sebbah, A., Vignot, E., Brabant, T., Burm-Ester, G. R., Grifka, J., Mueller, P. E. M., Bianchi, G., Grassi, W., Di Matteo, L., Modena, V., Di Munno, O., Orto-Lani, S., Punzi, L., Zangari, M., Roorda, L. D., van Riel, P. L. C. M., Czerwinski, E., Gorecki, A., Tlustochowicz, W., da Silva, J. Canas, Gomes, J. A. Melo, Miranda, L. M., Radulescu, F., Alexeeva, L. I., Orlov-Morozov, A. V., Pikhlak, E. G., Pilyaev, V. G., Shostak, N. A., Shmidt, E. I., Zagorodniy, N. V., Rodriguez, L. Arboleya, Ruiz, P. Benito, Carmona, E. Chamizo, Estevez, E. Collantes, Herrero-Beaumont, G., Mola, E. Martin, Moreno, A., Hernandez, A. Naranjo, Sarabia, F. Navarro, Padrino, J. M., Palacios, C., Serna, A. Rodriguez, Ivorra, J. A. Roman, Torrijos, A., Abdulhakim, E., Arden, N., Birrell, F., Donnachie, H., Fraser, W., Keen, R., Sarmiento, R., Stone, M. D., Grp, Sekoia Study, Department of public health, Université de Liège, Service de Rhumatologie et Pathologie Osseuse [Lyon], Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Physiopathologie, diagnostic et traitements des maladies osseuses / Pathophysiology, Diagnosis & Treatments of Bone Diseases (LYOS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM), World Health Organization (WHO), World Health Organization, University of Southampton, University of Bristol [Bristol], Bradford Institute for Health Research, Bradford Teaching Hospitals NHS Foundation Trust, Bradford, UK (BIHR), Department of Public Health, Epidemiology and Health Economics, Center for Permafrost (CENPERM), Department of Geosciences and Natural Resource Management [Copenhagen] (IGN), Faculty of Science [Copenhagen], University of Copenhagen = Københavns Universitet (UCPH)-University of Copenhagen = Københavns Universitet (UCPH)-Faculty of Science [Copenhagen], University of Copenhagen = Københavns Universitet (UCPH)-University of Copenhagen = Københavns Universitet (UCPH), Queen's University [Kingston, Canada], Department of Computer Science [Auckland], University of Auckland [Auckland], Institut de Recherche sur la Fusion par confinement Magnétique (IRFM), Commissariat à l'énergie atomique et aux énergies alternatives (CEA), University of Antwerp (UA), Arkema (Arkema), Hutchinson SA, Chair of Aerodynamics and Fluid Mechanics, Technische Universität Munchen - Université Technique de Munich [Munich, Allemagne] (TUM), Leiden University Medical Center (LUMC), Universiteit Leiden, CEDOC - Department of Rheumatology, Centro de Estudos de Doenças Crónicas (CEDOC), NOVA Medical School - Faculdade de Ciências Médicas (NMS), Universidade Nova de Lisboa = NOVA University Lisbon (NOVA)-Universidade Nova de Lisboa = NOVA University Lisbon (NOVA)-NOVA Medical School - Faculdade de Ciências Médicas (NMS), Universidade Nova de Lisboa = NOVA University Lisbon (NOVA)-Universidade Nova de Lisboa = NOVA University Lisbon (NOVA), CHLO/Hospital Egas Moniz, Commissariat à l'énergie atomique et aux énergies alternatives - Laboratoire d'Electronique et de Technologie de l'Information (CEA-LETI), Direction de Recherche Technologique (CEA) (DRT (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Radio and Space Plasma Physics Group [Leicester] (RSPP), University of Leicester, School of Environmental Sciences, University of Greenwich, Johns Hopkins University (JHU), University of Queensland [Brisbane], Université Laval [Québec] (ULaval), Intergroupe Francophone de Cancérologie Thoracique [Paris] (IFCT), Intergroupe Francophone de Cancérologie thoracique, Institute of Rheumatology, University Prague, Oskar Klein Centre [Stockholm], Stockholm University, Laboratoire de Réactivité de Surface (LRS), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS), Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Biologie de l'Os et du Cartilage : Régulations et Ciblages Thérapeutiques (BIOSCAR (UMR_S_1132 / U1132)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Service de rhumatologie, Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpital l'Archet, Département de Rhumatologie, CHU Amiens-Picardie, Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517 (MP3CV), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Centre Hospitalier Sud Francilien, Laboratoire d'Electronique, d'Informatique et d'Image [EA 7508] (Le2i), Université de Technologie de Belfort-Montbeliard (UTBM)-Université de Bourgogne (UB)-École Nationale Supérieure d'Arts et Métiers (ENSAM), Arts et Métiers Sciences et Technologies, HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Arts et Métiers Sciences et Technologies, HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Centre National de la Recherche Scientifique (CNRS), Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Aquitaine’s Care and Research organisation for inflammatory and Immune-Mediated diseases [CHU Bordeaux] (FHU ACRONIM), CHU Bordeaux [Bordeaux], Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), Hospices Civils de Lyon (HCL), Department of Bone and Joint Diseases, Faculty of Medicine and Health Sciences, Uniwersytet Jagielloński w Krakowie = Jagiellonian University (UJ), Consejo Superior de Investigaciones Científicas [Madrid] (CSIC), Departamento de Construcción y Agronomía, Facultad de Ciencias Agrarias y Ambientales, University of Oxford, The University of Sydney, IUMEA, University of Las Palmas de Gran Canaria (ULPGC), the SEKOIA management team, Bradford Teaching Hospitals NHS Foundation Trust [Bradford, Royaume-Uni], University of Copenhagen = Københavns Universitet (KU)-University of Copenhagen = Københavns Universitet (KU)-Faculty of Science [Copenhagen], University of Copenhagen = Københavns Universitet (KU)-University of Copenhagen = Københavns Universitet (KU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPC), HESAM Université (HESAM)-HESAM Université (HESAM)-Arts et Métiers Sciences et Technologies, HESAM Université (HESAM)-HESAM Université (HESAM)-Centre National de la Recherche Scientifique (CNRS), University of Oxford [Oxford], APH - Aging & Later Life, and Rehabilitation medicine

Subjects
Male, medicine.medical_specialty, Knee Joint, [SDV]Life Sciences [q-bio], Radiography, Osteoarthritis, Thiophenes, Placebo, Severity of Illness Index, Article, law.invention, Healthcare improvement science Radboud Institute for Health Sciences [Radboudumc 18], Rheumatology, Randomized controlled trial, Strontium ranelate, law, Internal medicine, Severity of illness, Medicine, Humans, skin and connective tissue diseases, Aged, Bone Density Conservation Agents, business.industry, Reproducibility of Results, Middle Aged, Osteoarthritis, Knee, medicine.disease, Multicenter study, Physical therapy, Disease Progression, Female, sense organs, business, medicine.drug
Abstract

Contains fulltext : 202254.pdf (Publisher’s version ) (Closed access) OBJECTIVE: Osteoarthritis-related changes in joint space measurements over time are small and sensitive to measurement error. The Reliable Change Index (RCI) determines whether the magnitude of change observed in an individual can be attributed to true change. This study aimed to examine the RCI as a novel approach to estimating osteoarthritis progression. METHODS: Data were from 167 men and 392 women with knee osteoarthritis (diagnosed using the American College of Rheumatology criteria) randomized to the placebo arm of the 3-year Strontium Ranelate Efficacy in Knee Osteoarthritis trial (SEKOIA) and assessed annually. The RCI was used to determine whether the magnitude of change in joint space width (JSW) on radiographs between study years was likely to be true or due to measurement error. RESULTS: Between consecutive years, 57-69% of participants had an apparent decrease (change

Published
2019
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128. EULAR definition of difficult-to-treat rheumatoid arthritis

Author

Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., and van Laar, J.M.

Published
2021

140. Update of penetrance estimates in Birt-Hogg-Dubé syndrome

Author

Bruinsma, Fiona Jane, Dowty, James G, Win, Aung Ko, Goddard, Laura C, Agrawal, Prachi, Attina', Domenico, Bissada, Nabil, De Luise, Monica, Eisen, Daniel B, Furuya, Mitsuko, Gasparre, Giuseppe, Genuardi, Maurizio, Gerdes, Anne-Marie, Hansen, Thomas Van Overeem, Houweling, Arjan C, Johannesma, Paul Christiaan, Lencastre, André, Lim, Derek, Lindor, Noralane M, Luzzi, Valentina, Lynch, Maeve, Maffé, Antonella, Menko, Fred H, Michels, Guido, Pulido, Jose S, Ryu, Jay H, Sattler, Elke C, Steinlein, Ortrud K, Tomassetti, Sara, Tucker, Kathy, Turchetti, Daniela, van de Beek, Irma, van Riel, Lore, van Steensel, Maurice, Zenone, Thierry, Zompatori, Maurizo, Walsh, Jennifer, Bondavalli, Davide, Maher, Eamonn R, and Winship, Ingrid M

Abstract

BackgroundBirt-Hogg-Dubé (BHD) syndrome is a rare genetic syndrome caused by pathogenic or likely pathogenic germline variants in the FLCNgene. Patients with BHD syndrome have an increased risk of fibrofolliculomas, pulmonary cysts, pneumothorax and renal cell carcinoma. There is debate regarding whether colonic polyps should be added to the criteria. Previous risk estimates have mostly been based on small clinical case series.MethodsA comprehensive review was conducted to identify studies that had recruited families carrying pathogenic or likely pathogenic variants in FLCN. Pedigree data were requested from these studies and pooled. Segregation analysis was used to estimate the cumulative risk of each manifestation for carriers of FLCNpathogenic variants.ResultsOur final dataset contained 204 families that were informative for at least one manifestation of BHD (67 families informative for skin manifestations, 63 for lung, 88 for renal carcinoma and 29 for polyps). By age 70 years, male carriers of the FLCNvariant have an estimated 19% (95% CI 12% to 31%) risk of renal tumours, 87% (95% CI 80% to 92%) of lung involvement and 87% (95% CI 78% to 93%) of skin lesions, while female carriers had an estimated 21% (95% CI 13% to 32%) risk of renal tumours, 82% (95% CI 73% to 88%) of lung involvement and 78% (95% CI 67% to 85%) of skin lesions. The cumulative risk of colonic polyps by age 70 years old was 21% (95% CI 8% to 45%) for male carriers and 32% (95% CI 16% to 53%) for female carriers.ConclusionsThese updated penetrance estimates, based on a large number of families, are important for the genetic counselling and clinical management of BHD syndrome.

Published
2023
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142. Exploratory study of wire based ECM finishing of 316L stainless steel, implemented within a hybrid wire EDM-ECM platform.

Author

Van Riel, Thomas, Qian, Jun, and Lauwers, Bert

Abstract

Thermal processes such as wire electro discharge machining (wire EDM) have an intrinsic heat affected zone (HAZ) and recast layer due to their thermal removal mechanisms. This layer is adverse to the fatigue life characteristics of the manufactured parts, and has to be removed by finishing operations. Wire electrochemical machining (wire ECM) is proposed as a finishing operation, as it can dissolve conductive materials without damaging the machined surface, and is similar in mechanical operation as wire EDM. An important drawback of electrochemical machining is the low accuracy when removing large amounts of materials in one pass, which is addressed by only machining a shallow depth by ECM after roughing by EDM (i.e., finishing allowance of 15 – 70 µm, the HAZ thickness). This paper presents an experimental study of electrochemical finishing 316L stainless steel wire EDM'ed parts. To improve the machining efficiency and the accuracy of the finished parts by eliminating reclamping, the process is implemented on a wire EDM machine. The wire electrode used in EDM roughing is also used in ECM finishing and is retraced along the contour of the part with an offset to increase or decrease the interelectrode gap. The experiments presented in this paper aim to gain insight in the relationship between the process parameters of wire ECM and the resulting machined surface, with respect to the machining depth, material removal rate (MRR), and surface roughness. A number of process optimization strategies are investigated, considering parameters such as the feed rate and the number of passes. Experimental results show that wire based electrochemical machining is a viable process to finish wire EDM parts, achieving removal depths greater than 20 µm with a surface roughness below 1 µm Ra. [ABSTRACT FROM AUTHOR]

Published
2022
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148. 7. Tomographic Techniques

Author

van der Made, P. M., primary, van Riel, P., additional, Berkhout, A. J., additional, Bishop, T. N., additional, Bube, K. P., additional, Cutler, R. T., additional, Langan, R. T., additional, Love, P. L., additional, Resnick, J. R., additional, Shuey, R. T., additional, Spindler, D. A., additional, Wyld, H. W., additional, Lines, Larry R., additional, Treitel, Sven, additional, Sword, Charles H., additional, Etgen, John T., additional, Meyerholtz, Keith A., additional, Pavlis, Gary L., additional, Szpakowski, Sally A., additional, Toldi, John L., additional, Stork, Christof, additional, Boehm, Gualtiero, additional, Cavallini, Fabio, additional, Vesnaver, Aldo L., additional, Kosloff, Dan, additional, Sherwood, John, additional, Koren, Zvi, additional, Machet, Elana, additional, Falkovitz, Yael, additional, Liu, Zhenyue, additional, Bleistein, Norman, additional, Billette, Frédéric, additional, Lambaré, Gilles, additional, Bloor, Robert, additional, Clapp, Robert G., additional, Biondi, Biondo, additional, Hardy, P., additional, and Claerbout, Jon F., additional

Published
2008
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