المؤلفون: Elsea, SH, Solyom, A, Martin, K, Harmatz, P, Mitchell, J, Lampe, C, Grant, C, Selim, L, Mungan, NO, Guelbert, N, Magnusson, B, Sundberg, E, Puri, R, Kapoor, S, Arslan, N, DiRocco, M, Zaki, M, Ozen, S, Mahmoud, IG, Ehlert, K, Hahn, A, Gokcay, G, Torcoletti, M, Ferreira, CR

المصدر: Human mutation. 41(9):1469-1487

مصطلحات موضوعية: Medicin och hälsovetenskap, Medicinska och farmaceutiska grundvetenskaper, Medicinsk genetik, Klinisk medicin, Neurologi

الوصول الحر: http://kipublications.ki.se/Default.aspx?queryparsed=id:144042828Test

المؤلفون: Ozen, S., Seker, D. Z.

المصدر: eISSN: 2194-9034

الوصف: Natural gas has been used for heating and production purposes in Turkey since 1987, and every passing year, the natural gas pipeline network continues to expand with investments. The distribution of natural gas within urban areas is achieved through a complex network structure formed by the joining of Polyethylene (PE) and Steel (ST) pipes using various connection elements. This complex structure is susceptible to deformation over time due to various reasons such as corrosion in steel lines, manufacturing errors in connection elements in PE lines, and other factors. As a result, natural gas leaks occasionally occur in these pipelines. The leakage control process in natural gas pipelines is carried out using specially designed equipment and vehicles equipped for this purpose. However, while these systems may be sufficient for collecting the necessary data for leak detection, the complex structure of the distribution network and the necessary data for leak detection, the complex structure of the distribution network and the necessity of performing this process with multiple teams bring about many challenges in terms of tracking, management, and reliability of the operation. At this point, the advancement of GIS (Geographic Information System), mobile, and communication technologies has made it possible to overcome these challenges. In this study, a real-time GIS-supported leak detection system developed using these technologies for Başkent Natural Gas Distrubition Inc. (Başkentgaz), which provides natural gas distribution services in Ankara, has been described. The study discusses the benefits brought by this system to the natural gas leak detection process.

وصف الملف: application/pdf

العلاقة: https://isprs-archives.copernicus.org/articles/XLVIII-4-W9-2024/283/2024Test/

الإتاحة: https://doi.org/10.5194/isprs-archives-XLVIII-4-W9-2024-283-2024Test
https://isprs-archives.copernicus.org/articles/XLVIII-4-W9-2024/283/2024Test/

المؤلفون: Ombrello, MJ, Arthur, VL, Remmers, EF, Hinks, A, Tachmazidou, I, Grom, AA, Foell, D, Martini, A, Gattorno, M, Ozen, S, Prahalad, S, Zeft, AS, Bohnsack, JF, Ilowite, NT, Mellins, ED, Russo, R, Len, C, Hilario, MOE, Oliveira, S, Yeung, RSM, Rosenberg, AM, Wedderburn, LR, Anton, J, Haas, JP, Rosen-Wolff, A, Minden, K, Tenbrock, K, Demirkaya, E, Cobb, J, Baskin, E, Signa, S, Shuldiner, E, Duerr, RH, Achkar, JP, Kamboh, MI, Kaufman, KM, Kottyan, LC, Pinto, D, Scherer, SW, Alarcon-Riquelme, ME, Docampo, E, Estivill, X, Gul, A, Langefeld, CD, Thompson, S, Zeggini, E, Kastner, DL, Woo, P, Thomson, W

المصدر: Annals of the rheumatic diseases. 76(5):906-913

مصطلحات موضوعية: Medicin och hälsovetenskap

الوصول الحر: http://kipublications.ki.se/Default.aspx?queryparsed=id:135467661Test

المؤلفون: Ravelli, A, Minoia, F, Davi, S, Horne, A, Bovis, F, Pistorio, A, Arico, M, Avcin, T, Behrens, EM, De Benedetti, F, Filipovic, L, Grom, AA, Henter, JI, Ilowite, NT, Jordan, MB, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, DJ, Miettunen, P, Nichols, KE, Ozen, S, Schmid, JP, Ramanan, AV, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, HI, Martini, A, Ruperto, N, Cron, RQ

المصدر: Arthritis & rheumatology (Hoboken, N.J.). 68(3):566-576

مصطلحات موضوعية: Medicin och hälsovetenskap

الوصول الحر: http://kipublications.ki.se/Default.aspx?queryparsed=id:133075865Test

المؤلفون: Gaggiano C., Del Bianco A., Sota J., Gentileschi S., Ruscitti P., Giacomelli R., Piga M., Crisafulli F., Monti S., Emmi G., De Paulis A., Vitale A., Tarsia M., Caggiano V., Nuzzolese R., Parretti V., Fabiani C., Lopalco G., Maier A., Cattalini M., Rigante D., Govoni M., Li Gobbi F., Guiducci S., Parronchi P., Marino A., Ciccia F., Maggio M. C., Aragona E., Bartoloni E., Iagnocco A., Viapiana O., Sebastiani G. D., Guerriero S., Insalaco A., Del Giudice E., Conti G., Barone P., Olivieri A. N., Brucato A., Carubbi F., Triggianese P., Mauro A., Tosi G. M., Fonollosa A., Giardini H. A. M., Ragab G., Tharwat S., Hernández-Rodríguez J., Sfikakis P. P., Laskari K., Karamanakos A., Espinosa G., Shahram F., Direskeneli H., Hinojosa-Azaola A., Opris-Belinski D., AlMaghlouth I. A., Hatemi G., Eksin M. A., Önen F., Więsik-Szewczyk E., Akkoç N., Tufan A., Şahin A., Erten Ş., Ozen S., Batu E. D., Frediani B., Balistreri A., Cantarini L.

المساهمون: Gaggiano, C., Del Bianco, A., Sota, J., Gentileschi, S., Ruscitti, P., Giacomelli, R., Piga, M., Crisafulli, F., Monti, S., Emmi, G., De Paulis, A., Vitale, A., Tarsia, M., Caggiano, V., Nuzzolese, R., Parretti, V., Fabiani, C., Lopalco, G., Maier, A., Cattalini, M., Rigante, D., Govoni, M., Li Gobbi, F., Guiducci, S., Parronchi, P., Marino, A., Ciccia, F., Maggio, M. C., Aragona, E., Bartoloni, E., Iagnocco, A., Viapiana, O., Sebastiani, G. D., Guerriero, S., Insalaco, A., Del Giudice, E., Conti, G., Barone, P., Olivieri, A. N., Brucato, A., Carubbi, F., Triggianese, P., Mauro, A., Tosi, G. M., Fonollosa, A., Giardini, H. A. M., Ragab, G., Tharwat, S., Hernández-Rodríguez, J., Sfikakis, P. P., Laskari, K., Karamanakos, A., Espinosa, G., Shahram, F., Direskeneli, H., Hinojosa-Azaola, A., Opris-Belinski, D., Almaghlouth, I. A., Hatemi, G., Eksin, M. A., Önen, F., Więsik-Szewczyk, E., Akkoç, N., Tufan, A., Şahin, A., Erten, Ş., Ozen, S., Batu, E. D., Frediani, B., Balistreri, A., Cantarini, L.

مصطلحات موضوعية: Behçet’s disease, Autoinflammatory diseases: Patient involvement, Patient-driven registrie, Patient-reported outcome, Rare diseases

الوصف: IntroductionThis paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behcet's disease (BD). MethodsThe project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behcet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. ResultsRespondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behcet's Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0-30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1-50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 & PLUSMN; 1.1 (range - 1.8-4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557-1.766], p < 0.001). DiscussionPreliminary results from the AIDA for Patients BD registry were consistent ...

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/37396916; info:eu-repo/semantics/altIdentifier/wos/WOS:001016757600001; volume:10; numberofpages:10; journal:FRONTIERS IN MEDICINE; https://hdl.handle.net/11584/385983Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85164497118; https://www.frontiersin.org/articles/10.3389/fmed.2023.1188021/fullTest

الإتاحة: https://doi.org/10.3389/fmed.2023.1188021Test
https://hdl.handle.net/11584/385983Test

المؤلفون: Yilmaz, MI, Demirkaya, E, Acikel, C, Saldir, M, Akar, S, Cayci, T, Saglam, M, Unal, HU, Gok, M, Polat, A, Cetinkaya, H, Eyileten, T, Sari, S, Yildirim, AO, Sonmez, A, Oguz, Y, Vural, A, Ozen, S, Carrero, JJ

المصدر: Rheumatology (Oxford, England). 53(11):2002-2008

مصطلحات موضوعية: Medicin och hälsovetenskap

الوصول الحر: http://kipublications.ki.se/Default.aspx?queryparsed=id:130119854Test

المؤلفون: Akca, UK, Batu, ED, Jelusic, M, Calatroni, M, Bakry, R, Frkovic, M, Vinsova, N, Campos, RT, Horne, A, Caglayan, S, Vaglio, A, Moroni, G, Emmi, G, Ghiggeri, GM, Koker, O, Sinico, RA, Kim, S, Gagro, A, Matucci-Cerinic, C, Comak, E, Tekin, ZE, Aydin, EA, Heshin-Bekenstein, M, Acar, BC, Gattorno, M, Akman, S, Sozeri, B, Palmblad, K, Al-Mayouf, SM, Silva, CA, Dolezalova, P, Merkel, PA, Ozen, S

المصدر: Rheumatology (Oxford, England).

مصطلحات موضوعية: Medicin och hälsovetenskap

الوصول الحر: http://kipublications.ki.se/Default.aspx?queryparsed=id:154673342Test

المؤلفون: Karaca, N., Azarsiz, E., Karaca, E., Aksu, G., Genel, F., Gulez, N., Ozen, S.

مصطلحات موضوعية: consanguinity, BCG vaccine, IL12Rβ1 deficiency, mendelian susceptibility to mycobacterial disease, primary immunodeficiency, antibiotic agent, gamma interferon receptor, gamma interferon receptor 1, I kappa B kinase gamma, interleukin 12 receptor beta1, protein gamma interferon receptor 2, recombinant gamma interferon, STAT1 protein, tuberculostatic agent, unclassified drug, Article, cellular parameters, child, clinical article, clinical feature, early diagnosis, family history, female, follow up, gene mutation, genetic analysis, human, immune deficiency, infant, infection sensitivity

الوصف: Background: Mendelian susceptibility to mycobacterial disease (MSMD) is a rare primary immunodeficiency, caused by non-tuberculous mycobacteria or Bacillus Calmette-Guerin (BCG) vaccine and characterized by severe diseases in childhood. Objective: In this study, we examined eight years followed-up 12 Turkish children with genetically proven MSMD and we tried to evaluate the survival rate with succesfull disease management, rate of consanguinity, molecular, cellular and clinical features of patients. In addition, we wanted to emphasize the importance of early diagnosis before administration of BCG vaccine in countries where this vaccine is routinely used. Methods: Twelve patients diagnosed with molecular studies [IFNγR1 complete (n = 1), IFNγR2 partial (n = 3), IL12Rβ1 (n = 6), NEMO (n = 1), STAT1 mutation (n = 1)] were included. Results: Ten patients (83%) were born from consanguineous parents and frequency of family history for the primary immunodeficiency was 58% (n = 7). All the cases had been immunized with BCG vaccine (Mycobacterium bovis) due to lack of early diagnosis. Two patients had BCG-itis and four patients had “BCG-osis”. Survival rate was 75% after successful disease management with antibiotics, anti-tuberculous agents and recombinant IFN-γ. Conclusion: It was concluded that MSMD must be differentiated from different forms of primary immunodeficiencies, so clinicians should be aware of MSMD especially in patients with BCG vaccine complications and non-tuberculous mycobacterial infection. © 2023, Allergy and Immunology Society of Thailand. All rights reserved. ; Jeffrey Modell Foundation, JMF

العلاقة: Asian Pacific Journal of Allergy and Immunology; Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı; https://hdl.handle.net/11454/93140Test; https://doi.org/10.12932/ap-271219-0726Test; 41; 372; 378

الإتاحة: https://doi.org/10.12932/ap-271219-0726Test
https://hdl.handle.net/11454/93140Test

المؤلفون: Smith, E.M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C.M., Kamphuis, S., Lambert, L., Levy, D.M., Lewandowski, L., Maxwell, N., Morand, E., Ozen, S., Pain, C.E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., Beresford, M.W., Goilav, B., Marks, S., Oni, L.

المصدر: Clinical Immunology ; volume 250, page 109296 ; ISSN 1521-6616

مصطلحات موضوعية: Immunology, Immunology and Allergy

الإتاحة: https://doi.org/10.1016/j.clim.2023.109296Test
https://api.elsevier.com/content/article/PII:S152166162300075X?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S152166162300075X?httpAccept=text/plainTest

المؤلفون: Jouret, M., Aguiar, F., Girard, C., Vyzhga, Y., Oliveira-Ramos, F., Lanna, C. C., Guedri, R., Lefevre-Utile, A., Djohra, H., Mosquera Angarita, J. M., Ozen, S., Sahin, S., Georgin-Lavialle, S., Theodoropoulou, K.

المصدر: Scientific Abstracts

الإتاحة: https://doi.org/10.1136/annrheumdis-2024-eular.6042Test