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Preventive medicine...
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Krauss, TobiasUniv Freiburg, Med Ctr, Fac Med, Dept Radiol, Freiburg, Germany
(author)
Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors
- Article/chapterEnglish2018
Publisher, publication year, extent ...
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BIOSCIENTIFICA LTD,2018
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:uu-387867
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-387867URI
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https://doi.org/10.1530/ERC-18-0100DOI
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https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-150210URI
Supplementary language notes
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Language:English
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Summary in:English
Part of subdatabase
Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel-Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10-75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2 cm; P < 0.001) and tumor volume doubling time (TVDT) was faster (22 vs 126 months; P = 0.001). All metastatic tumors were >= 2.8 cm. Codons 161 and 167 were hotspots for VHL germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off >= 2.8 cm, 44% and 91% for TVDT cut-off of <= 24 months). In 117 of 273 patients, PanNETs > 1.5 cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs < 2.8 cm vs >= 2.8 cm (94% vs 85% by 10 years; P = 0.020; 80% vs 50% at 10 years; P = 0.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8 cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.
Subject headings and genre
Added entries (persons, corporate bodies, meetings, titles ...)
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Ferrara, Alfonso MassimilianoIRCCS, Veneto Inst Oncol IOV, Familial Canc Clin & Oncoendocrinol, Padua, Italy
(author)
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Links, Thera P.Univ Groningen, Univ Med Ctr Groningen, Dept Endocrinol, Groningen, Netherlands
(author)
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Wellner, UlrichUniv Lubeck, Dept Surg, Lubeck, Germany
(author)
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Bancoss, IrinaMayo Clin, Div Endocrinol Diabet Metab &Nutr, Rochester, MN USA
(author)
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Kvachenyuk, AndreyNAMS Ukraine, Inst Endocrinol & Metab, Kiev, Ukraine
(author)
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Gomez de las Heras, Karim VillarServ Salud Castilla La Mancha SESCAM, Cent Serv, Toledo, Spain
(author)
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Yukina, Marina Y.Endocrinol Res Ctr, Dept Surg, Moscow, Russia
(author)
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Petrov, RomanBakhrushin Bros Moscow City Hosp, Dept Surg, Moscow, Russia,Fed Univ Ceara UFC, Brazil
(author)
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Bullivant, GarrettUniv Hlth Network, Princess Margaret Canc Ctr, Toronto, ON, Canada
(author)
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von Duecker, LauraAlbert Ludwigs Univ, Fac Med, Dept Med 4, Freiburg, Germany
(author)
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Jadhav, SwatiKing Edward Mem Hosp, Dept Endocrinol, Bombay, Maharashtra, India
(author)
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Ploeckinger, UrsulaCharite Univ Med Berlin, Interdisciplinary Ctr Metab Endocrinol Diabet & M, Campus Virchow Klinikum, Berlin, Germany
(author)
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Welin, StaffanUppsala universitet,Onkologisk endokrinologi,Uppsala Univ Hosp, Sweden(Swepub:uu)stafweli
(author)
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Schalin-Jantti, CamillaUniv Helsinki, Abdominal Ctr, Endocrinol, Helsinki, Finland;Helsinki Univ Hosp, Helsinki, Finland
(author)
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Gimm, OliverLinköpings universitet,Avdelningen för Kirurgi, Ortopedi och Onkologi,Medicinska fakulteten,Region Östergötland, Kirurgiska kliniken US(Swepub:liu)oligi51
(author)
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Pfeifer, MarijaUniv Med Ctr, Dept Endocrinol, Ljubljana, Slovenia
(author)
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Ngeow, JoanneNanyang Technol Univ, Natl Canc Ctr Singapore, Canc Genet Serv, Div Med Oncol, Singapore, Singapore;Nanyang Technol Univ, Lee Kong Chian Sch Med, Singapore, Singapore
(author)
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Hasse-Lazar, KorneliaMSC Mem Inst, Ctr Oncol, Dept Endocrine Oncol & Nucl Med, Gliwice, Poland
(author)
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Sanso, GabrielaHosp Ninos Dr Ricardo Gutierrez, Ctr Invest Endocrinol Dr Cesar Bergada CEDIE, Buenos Aires, DF, Argentina
(author)
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Qi, XiaopingWenzhou Med Univ, PLA Hosp 117, Dept Oncol & Urol Surg, Hangzhou, Zhejiang, Peoples R China
(author)
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Ugurlu, M. UmitMarmara Univ, Dept Gen Surg, Breast & Endocrine Surg Unit, Sch Med, Istanbul, Turkey
(author)
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Diaz, Rene E.Hosp Salvador, Endocrine Sect, Santiago, Chile
(author)
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Wohllk, NelsonUniv Chile, Hosp Salvador, Dept Med, Endocrine Sect, Santiago, Chile
(author)
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Peczkowska, MariolaInst Cardiol, Dept Hypertens, Warsaw, Poland
(author)
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Aberle, JensUniv Med Ctr Hamburg Eppendorf, Dept Med 3, Hamburg, Germany
(author)
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Lourenco Jr, Delmar M.Univ Sao Paulo, Serv Endocrinol, Hosp Clin HCFMUSP, Sao Paulo, Brazil;Univ Sao Paulo, ICESP, Fac Med, Sao Paulo, Brazil
(author)
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Pereira, Maria A. A.Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Serv Endocrinol, Sao Paulo, Brazil
(author)
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Fragoso, Maria C. B., VUniv Sao Paulo, Serv Endocrinol, Hosp Clin HCFMUSP, Sao Paulo, Brazil;Univ Sao Paulo, ICESP, Fac Med, Sao Paulo, Brazil,Hosp Ninos Dr Ricardo Gutierrez, Argentina,Univ Freiburg, Germany
(author)
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Hoff, Ana O.Univ Sao Paulo, Serv Endocrinol, Hosp Clin HCFMUSP, Sao Paulo, Brazil;Univ Sao Paulo, ICESP, Fac Med, Sao Paulo, Brazil
(author)
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Almeida, Madson Q.Univ Sao Paulo, Serv Endocrinol, Hosp Clin HCFMUSP, Sao Paulo, Brazil;Univ Sao Paulo, ICESP, Fac Med, Sao Paulo, Brazil
(author)
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Violante, Alice H. D.Univ Fed Rio de Janeiro, Dept Internal Med Endocrinol, Fac Med, Hosp Univ Clementino Fraga Filho, Rio De Janeiro, Brazil
(author)
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Ouidute, Ana R. P.Fed Univ Ceara UFC, Fac Med, Dept Physiol & Pharmacol, Drug Res & Dev Ctr NPDM, Fortaleza, Ceara, Brazil
(author)
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Zhang, ZheweiZhejiang Univ, Sch Med, Dept Urol, Hosp 2, Hangzhou, Zhejiang, Peoples R China
(author)
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Recasens, MonicaHosp Univ Girona, Inst Catala Salut, Gerencia Terr Girona, Girona, Spain
(author)
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Robles Diaz, LuisHosp Univ 12 Octubre, Serv Oncol Med, Unidad Tumores Digest, Madrid, Spain
(author)
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Kunavisarut, TadaMahidol Univ, Siriraj Hosp, Div Endocrinol & Metab, Bangkok, Thailand
(author)
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Wannachalee, TaweesakMahidol Univ, Siriraj Hosp, Div Endocrinol & Metab, Bangkok, Thailand
(author)
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Sirinvaravong, SirinartMahidol Univ, Siriraj Hosp, Div Endocrinol & Metab, Bangkok, Thailand
(author)
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Jonasch, EricUniv Texas MD Anderson Canc Ctr, Dept Genitourinary Med Oncol, Div Canc Med, Houston, TX 77030 USA
(author)
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Grozinsky-Glasberg, SimonaHadassah Hebrew Univ, Dept Med, Endocrinol & Metab Serv, Neuroendocrine Tumor Div,Med Ctr, Jerusalem, Israel
(author)
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Fraenkel, MeravHadassah Hebrew Univ, Dept Med, Endocrinol & Metab Serv, Neuroendocrine Tumor Div,Med Ctr, Jerusalem, Israel
(author)
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Beltsevich, DmitryEndocrinol Res Ctr, Dept Surg, Moscow, Russia
(author)
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Egorov, Viacheslav, IBakhrushin Bros Moscow City Hosp, Dept Surg, Moscow, Russia
(author)
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Bausch, DirkUniv Lubeck, Dept Surg, Lubeck, Germany
(author)
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Schott, MatthiasHeinrich Heine Univ, Dept Endocrinol, Dusseldorf, Germany
(author)
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Tiling, NikolausCharite Univ Med Berlin, Interdisciplinary Ctr Metab Endocrinol Diabet & M, Campus Virchow Klinikum, Berlin, Germany
(author)
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Pennelli, GianmariaUniv Padua, Dept Med DIMED, Surg Pathol Unit, Padua, Italy
(author)
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Zschiedrich, StefanAlbert Ludwigs Univ, Fac Med, Dept Med 4, Freiburg, Germany
(author)
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Daerr, RolandAlbert Ludwigs Univ, Fac Med, Dept Med 4, Freiburg, Germany;Univ Freiburg, Heart Ctr Freiburg Univ, Fac Med, Dept Cardiol & Angiol 1, Freiburg, Germany
(author)
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Ruf, JuriAlbert Ludwigs Univ, Fac Med, Dept Nucl Med, Freiburg, Germany
(author)
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Denecke, TimmCharite Univ Med Berlin, Dept Radiol, Campus Virchow Klinikum, Berlin, Germany
(author)
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Link, Karl-HeinrichAsklepios Paulinen Klin, Dept Surg, Wiesbaden, Germany
(author)
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Zovato, StefaniaIRCCS, Veneto Inst Oncol IOV, Familial Canc Clin & Oncoendocrinol, Padua, Italy
(author)
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von Dobschuetz, ErnstAcad Teaching Hosp Univ Hamburg, Reinbek Hosp, Sect Endocrine Surg, Reinbek, Germany
(author)
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Yaremchuk, SvetlanaNAMS Ukraine, Inst Endocrinol & Metab, Kiev, Ukraine
(author)
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Amthauer, HolgerCharite Univ Med Berlin, Dept Clin Nucl Med, Berlin, Germany
(author)
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Makay, OzerDept Gen Surg, Div Endocrine Surg, Izmir, Turkey
(author)
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Patocs, AttilaSemmelweis Univ, Hungarian Acad Sci, Dept Med 2, Budapest, Hungary;Semmelweis Univ, Hungarian Acad Sci, Mol Med Res Grp, Budapest, Hungary
(author)
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Walz, Martin K.Huyssens Fdn Clin, Dept Surg, Essen, Germany
(author)
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Huber, Tobias B.Univ Med Ctr Hamburg Eppendorf, Dept Med 3, Hamburg, Germany
(author)
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Seufert, JochenUniv Freiburg, Med Ctr, Fac Med, Dept Med 2, Freiburg, Germany
(author)
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Hellman, PerUppsala universitet,Endokrinkirurgi,Experimentell kirurgi,Uppsala Univ, Sweden,Albert Ludwigs Univ, Germany(Swepub:uu)perhellm
(author)
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Ekaterina, Raymond H.Univ Toronto, Univ Hlth Network, Dept Med, Toronto, ON, Canada;Mt Sinai Hosp, Fred Litwin Family Ctr Genet Med, Toronto, ON, Canada
(author)
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Kuchinskaya, EkaterinaLinköpings universitet,Avdelningen för cellbiologi,Medicinska fakulteten,Region Östergötland, Klinisk genetik(Swepub:liu)ekaku57
(author)
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Schiavi, FrancescaIRCCS, Veneto Inst Oncol IOV, Familial Canc Clin & Oncoendocrinol, Padua, Italy
(author)
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Malinoc, AngelicaAlbert Ludwigs Univ, Fac Med, Dept Med 4, Freiburg, Germany
(author)
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Reisch, NicoleLudwigs Maximilians Univ Munich, Dept Endocrinol, Munich, Germany
(author)
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Jarzab, BarbaraMSC Mem Inst, Ctr Oncol, Dept Endocrine Oncol & Nucl Med, Gliwice, Poland
(author)
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Barontini, MartaHosp Ninos Dr Ricardo Gutierrez, Ctr Invest Endocrinol Dr Cesar Bergada CEDIE, Buenos Aires, DF, Argentina
(author)
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Januszewicz, AndrzejInst Cardiol, Dept Hypertens, Warsaw, Poland
(author)
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Shah, NaliniKing Edward Mem Hosp, Dept Endocrinol, Bombay, Maharashtra, India
(author)
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Young, William F., Jr.Mayo Clin, Div Endocrinol Diabet Metab &Nutr, Rochester, MN USA
(author)
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Opocher, GiuseppeVeneto Inst Oncol IOV IRCCS, Sci Direct, Padua, Italy
(author)
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Eng, CharisCleveland Clin, Genom Med Inst, Lerner Res Inst, Cleveland, OH 44106 USA
(author)
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Neumann, Hartmut P. H.Albert Ludwigs Univ, Fac Med, Sect Prevent Med, Freiburg, Germany
(author)
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Bausch, BirkeUniv Freiburg, Med Ctr, Fac Med, Dept Med 2, Freiburg, Germany
(author)
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Univ Freiburg, Med Ctr, Fac Med, Dept Radiol, Freiburg, GermanyIRCCS, Veneto Inst Oncol IOV, Familial Canc Clin & Oncoendocrinol, Padua, Italy
(creator_code:org_t)
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In:Endocrine-Related Cancer: BIOSCIENTIFICA LTD25:9, s. 783-7931351-00881479-6821
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Krauss, Tobias
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Ferrara, Alfonso ...
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Links, Thera P.
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Wellner, Ulrich
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Bancoss, Irina
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Kvachenyuk, Andr ...
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Yukina, Marina Y ...
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Petrov, Roman
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von Duecker, Lau ...
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Jadhav, Swati
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Ploeckinger, Urs ...
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Welin, Staffan
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Schalin-Jantti, ...
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Gimm, Oliver
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Pfeifer, Marija
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Ngeow, Joanne
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Hasse-Lazar, Kor ...
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Sanso, Gabriela
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Qi, Xiaoping
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Ugurlu, M. Umit
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Diaz, Rene E.
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Wohllk, Nelson
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Peczkowska, Mari ...
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Aberle, Jens
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Lourenco Jr, Del ...
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Pereira, Maria A ...
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Fragoso, Maria C ...
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Hoff, Ana O.
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Almeida, Madson ...
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Violante, Alice ...
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Ouidute, Ana R. ...
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Zhang, Zhewei
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Recasens, Monica
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Robles Diaz, Lui ...
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Wannachalee, Taw ...
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Sirinvaravong, S ...
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Jonasch, Eric
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Grozinsky-Glasbe ...
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Fraenkel, Merav
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Beltsevich, Dmit ...
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Egorov, Viachesl ...
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Bausch, Dirk
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Schott, Matthias
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Tiling, Nikolaus
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Pennelli, Gianma ...
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Zschiedrich, Ste ...
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Daerr, Roland
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Ruf, Juri
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Denecke, Timm
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Link, Karl-Heinr ...
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Zovato, Stefania
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von Dobschuetz, ...
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Yaremchuk, Svetl ...
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Amthauer, Holger
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Makay, Ozer
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Patocs, Attila
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Walz, Martin K.
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Huber, Tobias B.
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Seufert, Jochen
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Hellman, Per
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Ekaterina, Raymo ...
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Kuchinskaya, Eka ...
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Schiavi, Frances ...
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Malinoc, Angelic ...
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Reisch, Nicole
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Jarzab, Barbara
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Barontini, Marta
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Januszewicz, And ...
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Shah, Nalini
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Young, William F ...
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Opocher, Giusepp ...
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Eng, Charis
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Neumann, Hartmut ...
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Bausch, Birke
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