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1. Impact of first-line use of caplacizumab on treatment outcomes in immune thrombotic thrombocytopenic purpura

Author

Özcan, Fedai, Markau, Silke, Girndt, Matthias, Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Schwenger, Vedat, Roeder, Maximilian, Radermacher, Jörg, Morgner, Anke, Herbst, Regina, von Auer, Charis, Völker, Linus A., Kaufeld, Jessica, Balduin, Gesa, Merkel, Lena, Kühne, Lucas, Eichenauer, Dennis A., Osterholt, Thomas, Hägele, Holger, Kann, Martin, Grundmann, Franziska, Kolbrink, Benedikt, Schulte, Kevin, Gäckler, Anja, Kribben, Andreas, Boss, Kristina, Potthoff, Sebastian A., Rump, Lars C., Schmidt, Tilman, Mühlfeld, Anja S., Schulmann, Karsten, Hermann, Matthias, Gaedeke, Jens, Sauerland, Kristin, Bramstedt, Jörn, Hinkel, Ulrich P., Miesbach, Wolfgang, Bauer, Frederic, Westhoff, Timm H., Bruck, Heike, Buxhofer-Ausch, Veronika, Müller, Tobias J., Wendt, Ralph, Harth, Ana, Schreiber, Adrian, Seelow, Evelyn, Tölle, Markus, Gohlisch, Christopher, Bieringer, Markus, Geuther, Gesa, Jabs, Wolfram J., Fischereder, Michael, von Bergwelt-Baildon, Anke, Schönermarck, Ulf, Knoebl, Paul, Menne, Jan, and Brinkkoetter, Paul T.

Published
2023
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2. The effects of lymphocele formation after living donor kidney transplantation on mid-term allograft function.

Author

Kuhn, Christian, Jänigen, Bernd, Matuschik, Laura, Rieger, Max J., Sidler, Daniel, and Zschiedrich, Stefan

Subjects
MEDICAL sciences, TRANSPLANTATION of organs, tissues, etc., GRAFT survival, CARDIOVASCULAR diseases risk factors, SURGICAL complications, KIDNEY transplantation
Abstract

Background: Despite advances in kidney transplant surgery and immunosuppression, lymphoceles remain a frequent complication in the early postoperative period following kidney transplantation, often requiring reintervention. While long-term outcomes such as patient and allograft survival are well studied, the impact of lymphocele formation on mid-term allograft function remains unclear. Methods: This multicentric study included 711 recipients of living donor kidney transplants to investigate the impact of lymphocele formation on mid-term graft function. Outcomes assessed included estimated glomerular filtration rate (eGFR) at 12 months, eGFR slope, and both patient and allograft survival. Results: Lymphoceles were detected in 17.4% of the recipients, with a median volume of 129 ml, and 71.8% of these patients required intervention. Patients without lymphocele formation had a significantly higher median eGFR at 12 months (52.1 ml/min/1.73 m²) compared to those with lymphoceles (48.7 ml/min/1.73 m²). Additionally, patients with lymphocele formation demonstrated a steeper median eGFR slope (-2.3 ml/min/1.73 m²/year) than those without (-0.3 ml/min/1.73 m²/year). No significant difference was observed in the composite outcome of allograft survival and patient death between the two groups. Conclusion: Lymphocele formation after living donor kidney transplantation is associated with a steeper decline in graft function. They may reflect a disturbed microvasculature and warrant closer control of cardiovascular risk factors and allograft monitoring of affected patients. Clinical trial details: Not applicable, the study is not a clinical trial. [ABSTRACT FROM AUTHOR]

Published
2025
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5. Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature

Author

Yukina, Marina, Cohen, Debbie L, Waguespack, Steven G, Pereira, Maria Adelaide Albergaria, He, Xin, Bandgar, Tushar, Donatini, Gianluca, Qi, Xiao-Ping, Cohn, Aviva, Roslyakova, Anna, Letizia, Claudio, Fishbein, Lauren, Kaur, Ravinder Jeet, Iniguez-Ariza, Nicole, Murad, Mohammad H, Gruber, Lucinda, Wachtel, Heather, Cherenko, Sergiy, Jimenez, Camilo, Else, Tobias, Ramteke-Jadhav, Swati, Fang, Xu-dong, Vaidya, Anand, Petramala, Luigi, Beltsevich, Dmitry, Walz, Martin K, Corssmit, Eleonora P.M., Wohllk, Nelson, Tufton, Nicola, Links, Thera P, Ferrara, Alfonso Massimiliano, Tsoy, Uliana, Donegan, Diane, Peczkowska, Mariola, Timmers, Henri J, Morelli, Valentina, Ebbehoj, Andreas, Kirschner, Lawrence S, Kunavisarut, Tada, Larsson, Catharina, Kudlai, Inna, Hasse-Lazar, Kornelia, Barczyński, Marcin, Deutschbein, Timo, Langton, Katharina, Rasmussen, Åse Krogh, Dvorakova, Sarka, Miller, Julie A, Liu, Longfei, Bennett, Bonita, Huang, Ya-Sheng, Yu, Zhi-xian, Kumar Jaiswal, Sanjeet, Shah, Nalini, Diaz, Rene E, Dullaart, Robin P.F, Akker, Scott A, Drake, William M, Boaretto, Francesca, Zovato, Stefania, Barbon, Giovanni, Taschin, Elisa, Schiavi, Francesca, Grineva, Elena, Rappaport, Maximilien, Skierczynski, Paul, Fassnacht, Martin, Calissendorff, Jan, Juhlin, C Christofer, Vlĕek, Petr, Li, Minghao, Jonasch, Eric, Prokop, Larry, Jovanovic, Milan, Lechan, Ronald M, Erenler, Feyza, Garla, Vishnu, Bobryk, Maryna, Kovalenko, Andrey Y, Hodson, Emma, Jenner, Bernadette, Simpson, Helen L, Casey, Ruth T, Gimm, Oliver, Ngeow Yuen Yie, Joanne, Shafigullina, Zulfiya, Martins Bugalho, Maria João, Rizzati, Silvia, Fraenkel, Merav, Sherlock, Mark, Sarma, Dipti, Kaimal Saikia, Uma, Riester, Anna, Quinkler, Marcus, Zschiedrich, Stefan, Seufert, Jochen, Bausch, Birke, Zavrashvili, Nino, Søndergaard, Esben, Mathiesen, Jes Sloth, Robaczyk, Maciej G, Poulsen, Per Løgstrup, Vasilkova, Volha, Costa-Barbosa, Flavia A, Kater, Claudio E, Yildirim Simsir, Ilgin, Ugurlu, M. Umit, Soyaltin, Utku E, Makay, Özer, Ivanov, Nikita V, Opocher, Giuseppe, Egorov, Viacheslav I, Petrov, Roman, Khudiakova, Natalia V, Bancos, Irina, Atkinson, Elizabeth, Eng, Charis, Young, William F, Jr, and Neumann, Hartmut P H

Published
2021
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7. Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura

Author

Völker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Brähler, Sebastian, Reinhardt, Martin, Kühne, Lucas, Mühlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Tölle, Markus, Jabs, Wolfram J., Özcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gäckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Jörg, Bramstedt, Jörn, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., and Menne, Jan

Published
2020
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8. ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP

Author

Völker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Brähler, Sebastian, Reinhardt, Martin, Kühne, Lucas, Mühlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Tölle, Markus, Jabs, Wolfram J., Özcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gäckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Jörg, Bramstedt, Jörn, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., and Menne, Jan

Published
2020
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10. A Multi-layered Quantitative In Vivo Expression Atlas of the Podocyte Unravels Kidney Disease Candidate Genes

Author

Rinschen, Markus M., Gödel, Markus, Grahammer, Florian, Zschiedrich, Stefan, Helmstädter, Martin, Kretz, Oliver, Zarei, Mostafa, Braun, Daniela A., Dittrich, Sebastian, Pahmeyer, Caroline, Schroder, Patricia, Teetzen, Carolin, Gee, HeonYung, Daouk, Ghaleb, Pohl, Martin, Kuhn, Elisa, Schermer, Bernhard, Küttner, Victoria, Boerries, Melanie, Busch, Hauke, Schiffer, Mario, Bergmann, Carsten, Krüger, Marcus, Hildebrandt, Friedhelm, Dengjel, Joern, Benzing, Thomas, and Huber, Tobias B.

Published
2018
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23. Characterization of endolymphatic sac tumors and von Hippel–Lindau disease in the International Endolymphatic Sac Tumor Registry

Author

Bausch, Birke, Wellner, Ulrich, Peyre, Mathieu, Boedeker, Carsten C., Hes, Frederik J., Anglani, Mariagiulia, de Campos, Jose M., Kanno, Hiroshi, Maher, Eamonn R., Krauss, Tobias, Sansó, Gabriela, Barontini, Marta, Letizia, Claudio, Hader, Claudia, Schiavi, Francesca, Zanoletti, Elisabetta, Suárez, Carlos, Offergeld, Christian, Malinoc, Angelica, Zschiedrich, Stefan, Glasker, Sven, Bobin, Serge, Sterkers, Olivier, Ba Huy, Patrice Tran, Giraud, Sophie, Links, Thera, Eng, Charis, Opocher, Giuseppe, Richard, Stephane, and Neumann, Hartmut P. H.

Published
2016
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26. AKT2 is essential to maintain podocyte viability and function during chronic kidney disease

Author

Canaud, Guillaume, Bienaime, Frank, Viau, Amandine, Treins, Caroline, Baron, William, Nguyen, Clement, Burtin, Martine, Berissi, Sophie, Giannakakis, Konstantinos, Muda, Andrea Onetti, Zschiedrich, Stefan, Huber, Tobias B., Friedlander, Gerard, Legendre, Christophe, Pontoglio, Marco, Pende, Mario, and Terzi, Fabiola

Subjects
Physiological aspects, Research, Kidney -- Physiological aspects, Glomerular filtration rate -- Research, Chronic kidney failure -- Research, Kidneys -- Physiological aspects
Abstract

In chronic kidney disease (CKD), loss of functional nephrons results in metabolic and mechanical stress in the remaining ones, resulting in further nephron loss. Here we show that Akt2 activation [...]

Published
2013
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27. Role of mTOR in podocyte function and diabetic nephropathy in humans and mice

Author

Godel, Markus, Hartleben, Bjorn, Herbach, Nadja, Liu, Shuya, Zschiedrich, Stefan, Lu, Shun, Debreczeni-Mor, Andrea, Lindenmeyer, Maja T., Rastaldi, Maria-Pia, Hartleben, Gotz, Wiech, Thorsten, Fornoni, Alessia, Nelson, Robert G., Kretzler, Matthias, Wanke, Rudiger, Pavenstadt, Hermann, Kerjaschki, Dontscho, Cohen, Clemens D., Hall, Michael N., Ruegg, Markus A., Inoki, Ken, Walz, Gerd, and Huber, Tobias B.

Subjects
Diabetic nephropathies
Abstract

Chronic glomerular diseases, associated with renal failure and cardiovascular morbidity, represent a major health issue. However, they remain poorly understood. Here we have reported that tightly controlled mTOR activity was [...]

Published
2011
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28. Association of High Pre-Transplant Caveolin-1 Serum Concentrations with Attenuated Acute Cellular Rejection in Kidney Transplantation

Author

Emmerich, Florian, Zschiedrich, Stefan, Reichenbach-Braun, Christine, Süsal, Caner, Minguet, Susana, Pauly, Marie-Christin, and Seidl, Maximilian

Subjects
allergology, cardiovascular system
Abstract

Acute and chronic transplant rejections due to alloreactivity are essential contributors to graft loss. However, the strength of alloreactivity is biased by non-immunological factors such as ischemia reperfusion injury (IRI). Accordingly, protection from IRI could be favorable in terms of limiting graft rejection. Caveolin-1 (Cav-1) is part of the cell membrane and an important regulator of intracellular signaling. Cav-1 has been demonstrated to limit IRI and to promote survival of a variety of cell types including renal cells under stress conditions. Accordingly, Cav-1 could also play a role in limiting anti-graft immune responses. Here, we evaluated a possible association between pretransplant serum concentrations of Cav-1 and the occurrence of rejection during follow up in a pilot study. Therefore, Cav-1-serum concentrations were analyzed in 91 patients at the time of kidney transplantation and compared to the incidence of acute and chronic rejection. Higher Cav-1 levels were associated with lower occurrence of acute rejection episodes. Moreover, Cav-1 could be therapeutically useful for attenuating graft rejection.

Published
2020

32. Impact of first-line use of caplacizumab on treatment outcomes in immune thrombotic thrombocytopenic purpura

Author

Völker, Linus A., Kaufeld, Jessica, Balduin, Gesa, Merkel, Lena, Kühne, Lucas, Eichenauer, Dennis A., Osterholt, Thomas, Hägele, Holger, Kann, Martin, Grundmann, Franziska, Kolbrink, Benedikt, Schulte, Kevin, Gäckler, Anja, Kribben, Andreas, Boss, Kristina, Potthoff, Sebastian A., Rump, Lars C., Schmidt, Tilman, Mühlfeld, Anja S., Schulmann, Karsten, Hermann, Matthias, Gaedeke, Jens, Sauerland, Kristin, Bramstedt, Jörn, Hinkel, Ulrich P., Miesbach, Wolfgang, Bauer, Frederic, Westhoff, Timm H., Bruck, Heike, Buxhofer-Ausch, Veronika, Müller, Tobias J., Wendt, Ralph, Harth, Ana, Schreiber, Adrian, Seelow, Evelyn, Tölle, Markus, Gohlisch, Christopher, Bieringer, Markus, Geuther, Gesa, Jabs, Wolfram J., Fischereder, Michael, von Bergwelt-Baildon, Anke, Schönermarck, Ulf, Knoebl, Paul, Menne, Jan, Brinkkoetter, Paul T., Özcan, Fedai, Markau, Silke, Girndt, Matthias, Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Schwenger, Vedat, Roeder, Maximilian, Radermacher, Jörg, Morgner, Anke, Herbst, Regina, and von Auer, Charis

Abstract

The von Willebrand factor–directed nanobody caplacizumab has greatly changed the treatment of immune thrombotic thrombocytopenic purpura (iTTP) in recent years. Data from randomized controlled trials established efficacy and safety.

Published
2023
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33. Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature

Author

Bancos, Irina, primary, Atkinson, Elizabeth, additional, Eng, Charis, additional, Young, William F, additional, Neumann, Hartmut P H, additional, Yukina, Marina, additional, Cohen, Debbie L, additional, Waguespack, Steven G, additional, Pereira, Maria Adelaide Albergaria, additional, He, Xin, additional, Bandgar, Tushar, additional, Donatini, Gianluca, additional, Qi, Xiao-Ping, additional, Cohn, Aviva, additional, Roslyakova, Anna, additional, Letizia, Claudio, additional, Fishbein, Lauren, additional, Kaur, Ravinder Jeet, additional, Iniguez-Ariza, Nicole, additional, Murad, Mohammad H, additional, Gruber, Lucinda, additional, Wachtel, Heather, additional, Cherenko, Sergiy, additional, Jimenez, Camilo, additional, Else, Tobias, additional, Ramteke-Jadhav, Swati, additional, Fang, Xu-dong, additional, Vaidya, Anand, additional, Petramala, Luigi, additional, Beltsevich, Dmitry, additional, Walz, Martin K, additional, Corssmit, Eleonora P.M., additional, Wohllk, Nelson, additional, Tufton, Nicola, additional, Links, Thera P, additional, Ferrara, Alfonso Massimiliano, additional, Tsoy, Uliana, additional, Donegan, Diane, additional, Peczkowska, Mariola, additional, Timmers, Henri J, additional, Morelli, Valentina, additional, Ebbehoj, Andreas, additional, Kirschner, Lawrence S, additional, Kunavisarut, Tada, additional, Larsson, Catharina, additional, Kudlai, Inna, additional, Hasse-Lazar, Kornelia, additional, Barczyński, Marcin, additional, Deutschbein, Timo, additional, Langton, Katharina, additional, Rasmussen, Åse Krogh, additional, Dvorakova, Sarka, additional, Miller, Julie A, additional, Liu, Longfei, additional, Bennett, Bonita, additional, Huang, Ya-Sheng, additional, Yu, Zhi-xian, additional, Kumar Jaiswal, Sanjeet, additional, Shah, Nalini, additional, Diaz, Rene E, additional, Dullaart, Robin P.F, additional, Akker, Scott A, additional, Drake, William M, additional, Boaretto, Francesca, additional, Zovato, Stefania, additional, Barbon, Giovanni, additional, Taschin, Elisa, additional, Schiavi, Francesca, additional, Grineva, Elena, additional, Rappaport, Maximilien, additional, Skierczynski, Paul, additional, Fassnacht, Martin, additional, Calissendorff, Jan, additional, Juhlin, C Christofer, additional, Vlĕek, Petr, additional, Li, Minghao, additional, Jonasch, Eric, additional, Prokop, Larry, additional, Jovanovic, Milan, additional, Lechan, Ronald M, additional, Erenler, Feyza, additional, Garla, Vishnu, additional, Bobryk, Maryna, additional, Kovalenko, Andrey Y, additional, Hodson, Emma, additional, Jenner, Bernadette, additional, Simpson, Helen L, additional, Casey, Ruth T, additional, Gimm, Oliver, additional, Ngeow Yuen Yie, Joanne, additional, Shafigullina, Zulfiya, additional, Martins Bugalho, Maria João, additional, Rizzati, Silvia, additional, Fraenkel, Merav, additional, Sherlock, Mark, additional, Sarma, Dipti, additional, Kaimal Saikia, Uma, additional, Riester, Anna, additional, Quinkler, Marcus, additional, Zschiedrich, Stefan, additional, Seufert, Jochen, additional, Bausch, Birke, additional, Zavrashvili, Nino, additional, Søndergaard,, Esben, additional, Mathiesen, Jes Sloth, additional, Robaczyk, Maciej G, additional, Poulsen, Per Løgstrup, additional, Vasilkova, Volha, additional, Costa-Barbosa, Flavia A, additional, Kater, Claudio E, additional, Yildirim Simsir, Ilgin, additional, Ugurlu, M. Umit, additional, Soyaltin, Utku E, additional, Makay, Özer, additional, Ivanov, Nikita V, additional, Opocher, Giuseppe, additional, Egorov, Viacheslav I, additional, Petrov, Roman, additional, and Khudiakova, Natalia V, additional

Published
2021
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36. Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura

Author

Voelker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Braehler, Sebastian, Reinhardt, Martin, Kuehne, Lucas, Muehlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Toelle, Markus, Jabs, Wolfram J., Oezcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gaeckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Joerg, Bramstedt, Joern, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., Menne, Jan, Voelker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Braehler, Sebastian, Reinhardt, Martin, Kuehne, Lucas, Muehlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Toelle, Markus, Jabs, Wolfram J., Oezcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gaeckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Joerg, Bramstedt, Joern, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., and Menne, Jan

Abstract

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare but life-threatening condition. In 2018, the nanobody caplacizumab was approved for the treatment of adults experiencing an acute episode of aTTP, in conjunction with plasma exchange (PEX) and immunosuppression for a minimum of 30 days after stopping daily PEX. We performed a retrospective, observational analysis on the use of caplacizumab in 60 patients from 29 medical centers in Germany during acute disease management. Caplacizumab led to a rapid normalization of the platelet count (median, 3 days; mean 3.78 days). One patient died after late treatment initiation due to aTTP-associated complications. In 2 patients with initial disease presentation and in 4 additional patients with laboratory signs of an exacerbation or relapse after the initial therapy, PEX-free treatment regimens could be established with overall favorable outcome. Caplacizumab is efficacious in the treatment of aTTP independent of timing and ancillary treatment modalities. Based on this real-world experience and published literature, we propose to administer caplacizumab immediately to all patients with an acute episode of aTTP. Treatment decisions regarding the use of PEX should be based on the severity of the clinical presentation and known risk factors. PEX might be dispensable in some patients.

Published
2020

37. ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP

Author

Voelker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Braehler, Sebastian, Reinhardt, Martin, Kuehne, Lucas, Muehlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Toelle, Markus, Jabs, Wolfram J., Ozcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gaeckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Joerg, Bramstedt, Joern, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., Menne, Jan, Voelker, Linus A., Kaufeld, Jessica, Miesbach, Wolfgang, Braehler, Sebastian, Reinhardt, Martin, Kuehne, Lucas, Muehlfeld, Anja, Schreiber, Adrian, Gaedeke, Jens, Toelle, Markus, Jabs, Wolfram J., Ozcan, Fedai, Markau, Silke, Girndt, Matthias, Bauer, Frederic, Westhoff, Timm H., Felten, Helmut, Hausberg, Martin, Brand, Marcus, Gerth, Jens, Bieringer, Markus, Bommer, Martin, Zschiedrich, Stefan, Schneider, Johanna, Elitok, Saban, Gawlik, Alexander, Gaeckler, Anja, Kribben, Andreas, Schwenger, Vedat, Schoenermarck, Ulf, Roeder, Maximilian, Radermacher, Joerg, Bramstedt, Joern, Morgner, Anke, Herbst, Regina, Harth, Ana, Potthoff, Sebastian A., von Auer, Charis, Wendt, Ralph, Christ, Hildegard, Brinkkoetter, Paul T., and Menne, Jan

Abstract

Introduction of the nanobody caplacizumab was shown to be effective in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in the acute setting. The official recommendations include plasma exchange (PEX), immunosuppression, and the use of caplacizumab for a minimum of 30 days after stopping daily PEX. This study was a retrospective, observational analysis of the use of caplacizumab in 60 patients from 29 medical centers in Germany. Immunosuppressive treatment led to a rapid normalization of ADAMTS13 activities (calculated median, 21 days). In 35 of 60 patients, ADAMTS13 activities started to normalize before day 30 after PEX; in 11 of 60 patients, the treatment was extended beyond day 30; and in 5 patients, it was extended even beyond day 58 due to persistent autoimmune activity. In 34 of 60 instances, caplacizumab was stopped before day 30 with a favorable outcome whenever ADAMTS13 activities were >10%. In contrast, 11 of 34 patients with ADAMTS13 activities <10% at the time of stopping caplacizumab treatment developed a nonfavorable outcome (disease exacerbation or relapse). In some cases, prolongation of the treatment interval to every other day was feasible and resulted in a sustained reduction of von Willebrand factor activity. ADAMTS13 activity measurements are central for a rapid diagnosis in the acute setting but also to tailor disease management. An ADAMTS13 activity-guided approach seems safe for identifying the individual time point when to stop caplacizumab to prevent overtreatment and undertreatment; this approach will result in significant cost savings without jeopardizing the well-being of patients. In addition, von Willebrand factor activity may serve as a biomarker for drug monitoring.

Published
2020

39. Kinetic Targeting of pegylated liposomal Doxorubicin: a new Approach to Reduce Toxicity during Chemotherapy (CARL-trial)

Author

Jansen Martin, Hasenburg Annette, Rautenberg Beate, Zschiedrich Stefan, Groh Ursula, Siebers Jan W, Schmah Oliver, Eckes Jürgen, Hug Martin J, Winkler Karl, and Pütz Gerhard

Subjects
Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Abstract Background The therapeutic success of chemotherapeutic agents is often limited by severe adverse effects. To reduce toxicity of these drugs, nanoscale particle-based drug delivery systems (DDS) are used. DDS accumulate to some extent in tumor tissues, but only a very small portion of a given dose reaches this target. Accumulation of DDS in tumor tissues is supposed to be much faster than in certain other tissues in which side effects occur ("Kinetic Targeting"). Once saturation in tumor tissue is achieved, most of the administered DDS still circulate in the plasma. The extracorporeal elimination of these circulating nanoparticles would probably reduce toxicity. Methods For the CARL-trial (Controlled Application and Removal of Liposomal chemotherapeutics), pegylated liposomal doxorubicin (PLD) was used as chemotherapeutic agent and double filtration plasmapheresis (DFPP) was performed for extracorporeal elimination of liposomes. PLD was given as 40 mg/m2 every 3 weeks in combination with vinorelbine 2 × 25 mg/m2 (neoadjuvant treatment of breast cancer, 12 patients), or as 40 mg/m2 every 4 weeks (recurrent ovarian cancer, 3 patients). Primary endpoints were the efficiency and safety profile of DFPP, and secondary endpoints were side effects and tumor response. Results DFPP eliminated ~62% of circulating PLD, corresponding to ~45% of the total dose (n = 57 cycles). AUC of doxorubicin was reduced by 50%. No leakage of doxorubicin was detected during elimination, and no relevant DFPP-related side effects occurred. Reduction in tumor size > 30% occurred in 10/12 (neoadjuvant) and in 1/3 patients (recurrent). Only five grade 2 events and one grade 3 event (mucositis, neutropenia or leucopenia) and a single palmar-plantar erythrodysesthesia grade 2 were reported. Conclusion Extracorporeal elimination of PLD by DFPP is safe and efficient. CARL can diminish the main dose-limiting side effects of PLD, and probably many different DDS alike. Trial registration DRKS00000163

Published
2011
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40. Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy

Author

Neumann, Hartmut P. H., Tsoy, Uliana, Bancos, Irina, Amodru, Vincent, Walz, Martin K., Tirosh, Amit, Kaur, Ravinder Jeet, McKenzie, Travis, Qi, Xiaoping, Bandgar, Tushar, Petrov, Roman, Yukina, Marina Y., Roslyakova, Anna, van der Horst-Schrivers, Anouk N. A., Berends, Annika M. A., Hoff, Ana O., Castroneves, Luciana Audi, Ferrara, Alfonso Massimiliano, Rizzati, Silvia, Mian, Caterina, Dvorakova, Sarka, Hasse-Lazar, Kornelia, Kvachenyuk, Andrey, Peczkowska, Mariola, Loli, Paola, Erenler, Feyza, Krauss, Tobias, Almeida, Madson Q., Liu, Longfei, Zhu, Feizhou, Recasens, Monica, Wohllk, Nelson, Corssmit, Eleonora P. M., Shafigullina, Zulfiya, Calissendorff, Jan, Grozinsky-Glasberg, Simona, Kunavisarut, Tada, Schalin-Jantti, Camilla, Castinetti, Frederic, Vlcek, Petr, Beltsevich, Dmitry, Egorov, Viacheslav I, Schiavi, Francesca, Links, Thera P., Lechan, Ronald M., Bausch, Birke, Young, William F. Jr., Eng, Charis, Jaiswal, Sanjeet Kumar, Zschiedrich, Stefan, Fragoso, Maria C. B. V, Pereira, Maria A. A., Li, Minghao, Biarnes Costa, Josefina, Juhlin, Carl Christofer, Gross, David, Violante, Alice H. D., Kocjan, Tomaz, Ngeow, Joanne, Yoel, Uri, Fraenkel, Merav, Simsir, Ilgin Yildirim, Ugurlu, M. Umit, Ziagaki, Athanasia, Robles Diaz, Luis, Kudlai, Inna Stepanovna, Gimm, Oliver, Scherbaum, Christina Rebecca, Abebe-Campino, Gadi, Barbon, Giovanni, Taschin, Elisa, Malinoc, Angelica, Khudiakova, Natalia Valeryevna, Ivanov, Nikita V, Pfeifer, Marija, Zovato, Stefania, Ploeckinger, Ursula, Makay, Ozer, Grineva, Elena, Jarzab, Barbara, Januszewicz, Andrzej, Shah, Nalini, Seufert, Jochen, Opocher, Giuseppe, Larsson, Catharina, Neumann, Hartmut P. H., Tsoy, Uliana, Bancos, Irina, Amodru, Vincent, Walz, Martin K., Tirosh, Amit, Kaur, Ravinder Jeet, McKenzie, Travis, Qi, Xiaoping, Bandgar, Tushar, Petrov, Roman, Yukina, Marina Y., Roslyakova, Anna, van der Horst-Schrivers, Anouk N. A., Berends, Annika M. A., Hoff, Ana O., Castroneves, Luciana Audi, Ferrara, Alfonso Massimiliano, Rizzati, Silvia, Mian, Caterina, Dvorakova, Sarka, Hasse-Lazar, Kornelia, Kvachenyuk, Andrey, Peczkowska, Mariola, Loli, Paola, Erenler, Feyza, Krauss, Tobias, Almeida, Madson Q., Liu, Longfei, Zhu, Feizhou, Recasens, Monica, Wohllk, Nelson, Corssmit, Eleonora P. M., Shafigullina, Zulfiya, Calissendorff, Jan, Grozinsky-Glasberg, Simona, Kunavisarut, Tada, Schalin-Jantti, Camilla, Castinetti, Frederic, Vlcek, Petr, Beltsevich, Dmitry, Egorov, Viacheslav I, Schiavi, Francesca, Links, Thera P., Lechan, Ronald M., Bausch, Birke, Young, William F. Jr., Eng, Charis, Jaiswal, Sanjeet Kumar, Zschiedrich, Stefan, Fragoso, Maria C. B. V, Pereira, Maria A. A., Li, Minghao, Biarnes Costa, Josefina, Juhlin, Carl Christofer, Gross, David, Violante, Alice H. D., Kocjan, Tomaz, Ngeow, Joanne, Yoel, Uri, Fraenkel, Merav, Simsir, Ilgin Yildirim, Ugurlu, M. Umit, Ziagaki, Athanasia, Robles Diaz, Luis, Kudlai, Inna Stepanovna, Gimm, Oliver, Scherbaum, Christina Rebecca, Abebe-Campino, Gadi, Barbon, Giovanni, Taschin, Elisa, Malinoc, Angelica, Khudiakova, Natalia Valeryevna, Ivanov, Nikita V, Pfeifer, Marija, Zovato, Stefania, Ploeckinger, Ursula, Makay, Ozer, Grineva, Elena, Jarzab, Barbara, Januszewicz, Andrzej, Shah, Nalini, Seufert, Jochen, Opocher, Giuseppe, and Larsson, Catharina

Abstract

IMPORTANCE Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management. OBJECTIVE To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence. DESIGN, SETTING, AND PARTICIPANTS This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019. EXPOSURES Total or cortical-sparing adrenalectomy. MAIN OUTCOMES AND MEASURES Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality. RESULTS Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutationswere detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients develope

Published
2019
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41. Lethal pulmonary hemorrhage syndrome due to Leptospira infection transmitted by pet rat

Author

Ludwig, Bernd, Zotzmann, Viviane, Bode, Christoph, Staudacher, Dawid L., and Zschiedrich, Stefan

Subjects
Case Report, Leptospira interrogans icterohaemorrhagicae, Leptospirosis, Infectious and parasitic diseases, RC109-216, Severe pulmonary hemorrhage syndrome, Hemolysis, Weil’s disease, Cytokine absorbent therapy
Abstract

Human infection with Leptospira interrogans can be life-threatening. Multiple organ involvement frequently presents with liver and kidney failure, less commonly including severe hemolysis and pulmonary hemorrhage syndrome. Here, we present a fulminant case of leptospirosis presenting with hemolysis and pulmonary hemorrhage. A formerly healthy 34 year old patient presented to a rural hospital with dyspnea and hemoptysis after a week of influenza-like symptoms. Initial assessment revealed severe sepsis, acute kidney failure and severe hemolysis. Within the next 29 h, a multi-organ failure developed, which could eventually not be reversed despite mechanical ventilation, venovenous extracorporeal membrane oxygenation, continuous renal replacement therapy, plasmapheresis and extracorporeal cytokine absorbent therapy. The diagnosis of leptospirosis was made after the patient died. The transmitting animal was a pet rat. Leptospirosis has to be considered in case of rapid multi-organ failure presenting with pulmonary hemorrhage.

Published
2017

42. Cumulative surgical morbidity in patients with multiple cerebellar and medullary hemangioblastomas

Author

Steiert, Christine, Krüger, Marie T., Jilg, Cordula Annette, Zschiedrich, Stefan, Klingler, Jan-Helge, Van Velthoven, Vera, and Gläsker, Sven

Subjects
ddc: 610, 610 Medical sciences, Medicine, urologic and male genital diseases
Abstract

Objective: Patients affected with von Hippel-Lindau disease frequently develop multiple hemangioblastomas of the central nervous system, predominantly of the cerebellum, brain stem and spinal cord. Timing of surgical intervention is difficult and depends largely on the anticipated surgical morbidity.[for full text, please go to the a.m. URL], 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS)

Published
2017

43. 101. Lipoprotein turnover and possible remnant accumulation in preeclampsia: Insights from the Freiburg preeclampsia H.E.L.P.-Apheresis Study

Author

Contini, Christine, primary, Jansen, Martin, additional, König, Brigitte, additional, Markfeld-Erol, Filiz, additional, Kunze, Mirjam, additional, Zschiedrich, Stefan, additional, Massing, Ulrich, additional, Merfort, Irmgard, additional, Prömpeler, Heinrich, additional, Pecks, Ulrich, additional, Winkler, Karl, additional, and Pütz, Gerhard, additional

Published
2018
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44. 62. Very preterm preeclampsia and treatment by heparin-mediated extracorporeal LDL-precipitation (H.E.L.P.) apheresis: The Freiburg preeclampsia H.E.L.P.-apheresis study

Author

Winkler, Karl, primary, Contini, Christine, additional, König, Brigitte, additional, Krumrey, Bärbel, additional, Pütz, Gerd, additional, Zschiedrich, Stefan, additional, Pecks, Ulrich, additional, Stavropoulou, Dimitria, additional, Prömpeler, Heinrich, additional, Kunze, Mirjam, additional, and Markfeld-Erol, Filiz, additional

Published
2018
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45. Preventive medicine of von Hippel–Lindau disease-associated pancreatic neuroendocrine tumors

Author

Krauss, Tobias, primary, Ferrara, Alfonso Massimiliano, additional, Links, Thera P, additional, Wellner, Ulrich, additional, Bancos, Irina, additional, Kvachenyuk, Andrey, additional, Villar Gómez de las Heras, Karina, additional, Yukina, Marina Y, additional, Petrov, Roman, additional, Bullivant, Garrett, additional, von Duecker, Laura, additional, Jadhav, Swati, additional, Ploeckinger, Ursula, additional, Welin, Staffan, additional, Schalin-Jäntti, Camilla, additional, Gimm, Oliver, additional, Pfeifer, Marija, additional, Ngeow, Joanne, additional, Hasse-Lazar, Kornelia, additional, Sansó, Gabriela, additional, Qi, Xiaoping, additional, Ugurlu, M Umit, additional, Diaz, Rene E, additional, Wohllk, Nelson, additional, Peczkowska, Mariola, additional, Aberle, Jens, additional, Lourenço, Delmar M, additional, Pereira, Maria A A, additional, Fragoso, Maria C B V, additional, Hoff, Ana O, additional, Almeida, Madson Q, additional, Violante, Alice H D, additional, Quidute, Ana R P, additional, Zhang, Zhewei, additional, Recasens, Mònica, additional, Díaz, Luis Robles, additional, Kunavisarut, Tada, additional, Wannachalee, Taweesak, additional, Sirinvaravong, Sirinart, additional, Jonasch, Eric, additional, Grozinsky-Glasberg, Simona, additional, Fraenkel, Merav, additional, Beltsevich, Dmitry, additional, Egorov, Viacheslav I, additional, Bausch, Dirk, additional, Schott, Matthias, additional, Tiling, Nikolaus, additional, Pennelli, Gianmaria, additional, Zschiedrich, Stefan, additional, Därr, Roland, additional, Ruf, Juri, additional, Denecke, Timm, additional, Link, Karl-Heinrich, additional, Zovato, Stefania, additional, von Dobschuetz, Ernst, additional, Yaremchuk, Svetlana, additional, Amthauer, Holger, additional, Makay, Özer, additional, Patocs, Attila, additional, Walz, Martin K, additional, Huber, Tobias B, additional, Seufert, Jochen, additional, Hellman, Per, additional, Kim, Raymond H, additional, Kuchinskaya, Ekaterina, additional, Schiavi, Francesca, additional, Malinoc, Angelica, additional, Reisch, Nicole, additional, Jarzab, Barbara, additional, Barontini, Marta, additional, Januszewicz, Andrzej, additional, Shah, Nalini, additional, Young, William F, additional, Opocher, Giuseppe, additional, Eng, Charis, additional, Neumann, Hartmut P H, additional, and Bausch, Birke, additional

Published
2018
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47. P2Y2R Signaling Is Involved in the Onset of Glomerulonephritis

Author

Rennert, Laura, primary, Zschiedrich, Stefan, additional, Sandner, Lukas, additional, Hartleben, Björn, additional, Cicko, Sanja, additional, Ayata, Cemil Korcan, additional, Meyer, Charlotte, additional, Zech, Andreas, additional, Zeiser, Robert, additional, Huber, Tobias B., additional, Idzko, Marco, additional, and Grahammer, Florian, additional

Published
2018
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48. Treatment of Very Preterm Preeclampsia via Heparin-mediated Extracorporeal LDL-precipitation Apheresis: the Freiburg Preeclampsia H.E.L.P.-Apheresis Study

Author

Winkler, Karl, primary, Contini, Christine, additional, König, Brigitte, additional, Krumrey, Bärbel, additional, Pütz, Gerd, additional, Zschiedrich, Stefan, additional, Pecks, Ulrich, additional, Stavropoulou, Dimitra, additional, Prömpeler, Heinrich, additional, Kunze, Mirjam, additional, and Markfeld-Erol, Filiz, additional

Published
2018
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49. Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors

Author

Krauss, Tobias, Ferrara, Alfonso Massimiliano, Links, Thera P., Wellner, Ulrich, Bancoss, Irina, Kvachenyuk, Andrey, Gomez de las Heras, Karim Villar, Yukina, Marina Y., Petrov, Roman, Bullivant, Garrett, von Duecker, Laura, Jadhav, Swati, Ploeckinger, Ursula, Welin, Staffan, Schalin-Jantti, Camilla, Gimm, Oliver, Pfeifer, Marija, Ngeow, Joanne, Hasse-Lazar, Kornelia, Sanso, Gabriela, Qi, Xiaoping, Ugurlu, M. Umit, Diaz, Rene E., Wohllk, Nelson, Peczkowska, Mariola, Aberle, Jens, Lourenco Jr, Delmar M., Pereira, Maria A. A., Fragoso, Maria C. B., V, Hoff, Ana O., Almeida, Madson Q., Violante, Alice H. D., Ouidute, Ana R. P., Zhang, Zhewei, Recasens, Monica, Robles Diaz, Luis, Kunavisarut, Tada, Wannachalee, Taweesak, Sirinvaravong, Sirinart, Jonasch, Eric, Grozinsky-Glasberg, Simona, Fraenkel, Merav, Beltsevich, Dmitry, Egorov, Viacheslav, I, Bausch, Dirk, Schott, Matthias, Tiling, Nikolaus, Pennelli, Gianmaria, Zschiedrich, Stefan, Daerr, Roland, Ruf, Juri, Denecke, Timm, Link, Karl-Heinrich, Zovato, Stefania, von Dobschuetz, Ernst, Yaremchuk, Svetlana, Amthauer, Holger, Makay, Ozer, Patocs, Attila, Walz, Martin K., Huber, Tobias B., Seufert, Jochen, Hellman, Per, Ekaterina, Raymond H., Kuchinskaya, Ekaterina, Schiavi, Francesca, Malinoc, Angelica, Reisch, Nicole, Jarzab, Barbara, Barontini, Marta, Januszewicz, Andrzej, Shah, Nalini, Young, William F., Jr., Opocher, Giuseppe, Eng, Charis, Neumann, Hartmut P. H., Bausch, Birke, Krauss, Tobias, Ferrara, Alfonso Massimiliano, Links, Thera P., Wellner, Ulrich, Bancoss, Irina, Kvachenyuk, Andrey, Gomez de las Heras, Karim Villar, Yukina, Marina Y., Petrov, Roman, Bullivant, Garrett, von Duecker, Laura, Jadhav, Swati, Ploeckinger, Ursula, Welin, Staffan, Schalin-Jantti, Camilla, Gimm, Oliver, Pfeifer, Marija, Ngeow, Joanne, Hasse-Lazar, Kornelia, Sanso, Gabriela, Qi, Xiaoping, Ugurlu, M. Umit, Diaz, Rene E., Wohllk, Nelson, Peczkowska, Mariola, Aberle, Jens, Lourenco Jr, Delmar M., Pereira, Maria A. A., Fragoso, Maria C. B., V, Hoff, Ana O., Almeida, Madson Q., Violante, Alice H. D., Ouidute, Ana R. P., Zhang, Zhewei, Recasens, Monica, Robles Diaz, Luis, Kunavisarut, Tada, Wannachalee, Taweesak, Sirinvaravong, Sirinart, Jonasch, Eric, Grozinsky-Glasberg, Simona, Fraenkel, Merav, Beltsevich, Dmitry, Egorov, Viacheslav, I, Bausch, Dirk, Schott, Matthias, Tiling, Nikolaus, Pennelli, Gianmaria, Zschiedrich, Stefan, Daerr, Roland, Ruf, Juri, Denecke, Timm, Link, Karl-Heinrich, Zovato, Stefania, von Dobschuetz, Ernst, Yaremchuk, Svetlana, Amthauer, Holger, Makay, Ozer, Patocs, Attila, Walz, Martin K., Huber, Tobias B., Seufert, Jochen, Hellman, Per, Ekaterina, Raymond H., Kuchinskaya, Ekaterina, Schiavi, Francesca, Malinoc, Angelica, Reisch, Nicole, Jarzab, Barbara, Barontini, Marta, Januszewicz, Andrzej, Shah, Nalini, Young, William F., Jr., Opocher, Giuseppe, Eng, Charis, Neumann, Hartmut P. H., and Bausch, Birke

Abstract

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.

Published
2018
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50. Minimally Invasive Surgery of Retroperitoneal Paragangliomas - Single-Center Experiences of 91 Tumors in 66 Patients

Author

Karakas, Elias, Alesina, Pier Francesco, Seeliger, Barbara, Neumann, Hartmut P. H., Zschiedrich, Stefan, and Walz, Martin K.

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Background: Surgery is the primary treatment of choice in retroperitoneal paragangliomas (rPG) as these tumors are potentially lethal by catecholamine excess. Up to now only very few and limited studies have demonstrated the option of minimally invasive surgery (MIS) in these patients. In the following[for full text, please go to the a.m. URL], 133. Kongress der Deutschen Gesellschaft für Chirurgie

Published
2016

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