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In: Europäische Sicherheit & Technik: ES & T ; europäische Sicherheit, Strategie & Technik, Band 62, Heft 9, S. 76-80
ISSN: 2193-746X
Bis vor wenigen Jahren waren Entwicklung, Beschaffung und Einsatz von IT-Systemen überwiegend teilstreitkraftspezifisch ausgerichtet. Abgestimmte Konzepte, Strukturen und Vorgehensweisen für streitkräfte- oder bundeswehrgemeinsame Lösungsansätze waren nicht erforderlich und daher kaum ausgeprägt. (Europäische Sicherheit & Technik / SWP)
World Affairs Online
In: Europäische Sicherheit: Politik, Streitkräfte, Wirtschaft, Technik, Band 60, Heft 3, S. 62-67
ISSN: 0940-4171
World Affairs Online
This introductory text is specifically targeted at ecologists and resource scientists, illustrating how sampling theory can be applied in a wide variety of resource contexts. The emphasis throughout is on design-based sampling from finite populations but attention is also given to model-based prediction and sampling from infinite populations.
World Affairs Online
Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1-25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0-88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%-subcutaneous; 29%-intravenous; 1%-unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment. ; peerReviewed
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Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment.
BASE
In: El-Helou, Sabine M., Biegner, Anika-Kerstin, Bode, Sebastian, Ehl, Stephan R., Heeg, Maximilian, Maccari, Maria E., Ritterbusch, Henrike, Speckmann, Carsten, Rusch, Stephan, Scheible, Raphael orcid:0000-0003-2067-5569 , Warnatz, Klaus, Atschekzei, Faranaz, Beider, Renata, Ernst, Diana, Gerschmann, Stev, Jablonka, Alexandra, Mielke, Gudrun, Schmidt, Reinhold E., Schuermann, Gesine, Sogkas, Georgios, Baumann, Ulrich H., Klemann, Christian, Viemann, Dorothee, von Bernuth, Horst, Krueger, Renate, Hanitsch, Leif G., Scheibenbogen, Carmen M., Wittke, Kirsten, Albert, Michael H., Eichinger, Anna, Hauck, Fabian, Klein, Christoph, Rack-Hoch, Anita, Sollinger, Franz M., Avila, Anne, Borte, Michael, Borte, Stephan, Fasshauer, Maria, Hauenherm, Anja, Kellner, Nils, Mueller, Anna H., Uelzen, Anett, Bader, Peter, Bakhtiar, Shahrzad, Lee, Jae-Yun, Hess, Ursula, Schubert, Ralf, Woelke, Sandra, Zielen, Stefan, Ghosh, Sujal, Laws, Hans-Juergen, Neubert, Jennifer, Oommen, Prasad T., Hoenig, Manfred, Schulz, Ansgar, Steinmann, Sandra, Schwarz, Klaus, Dueckers, Gregor, Lamers, Beate, Langemeyer, Vanessa, Niehues, Tim, Shai, Sonu, Graf, Dagmar, Mueglich, Carmen, Schmalzing, Marc T., Schwaneck, Eva C., Tony, Hans-Peter, Dirks, Johannes, Haase, Gabriele, Liese, Johannes G., Morbach, Henner, Foell, Dirk, Hellige, Antje, Wittkowski, Helmut, Masjosthusmann, Katja, Mohr, Michael, Geberzahn, Linda, Hedrich, Christian M., Mueller, Christiane, Roesen-Wolff, Angela, Roesler, Joachim, Zimmermann, Antje, Behrends, Uta, Rieber, Nikolaus, Schauer, Uwe, Handgretinger, Rupert, Holzer, Ursula, Henes, Joerg, Kanz, Lothar, Boesecke, Christoph, Rockstroh, Juergen K., Schwarze-Zander, Carolynne, Wasmuth, Jan-Christian, Dilloo, Dagmar, Huelsmann, Brigitte, Schoenberger, Stefan, Schreiber, Stefan, Zeuner, Rainald, Ankermann, Tobias, von Bismarck, Philipp, Huppertz, Hans-Iko, Kaiser-Labusch, Petra, Greil, Johann, Jakoby, Donate, Kulozik, Andreas E., Metzler, Markus, Naumann-Bartsch, Nora, Sobik, Bettina, Graf, Norbert orcid:0000-0002-2248-323X , Heine, Sabine, Kobbe, Robin, Lehmberg, Kai, Mueller, Ingo, Herrmann, Friedrich, Horneff, Gerd, Klein, Ariane, Peitz, Joachim, Schmidt, Nadine, Bielack, Stefan, Gross-Wieltsch, Ute, Classen, Carl F., Klasen, Jessica, Deutz, Peter, Kamitz, Dirk, Lassay, Lisa, Tenbrock, Klaus, Wagner, Norbert, Bernbeck, Benedikt, Brummel, Bastian, Lara-Villacanas, Eusebia, Muenstermann, Esther, Schneider, Dominik T., Tietsch, Nadine, Westkemper, Marco, Weiss, Michael, Kramm, Christof, Kuehnle, Ingrid, Kullmann, Silke, Girschick, Hermann, Specker, Christof orcid:0000-0003-2504-3229 , Vinnemeier-Laubenthal, Elisabeth, Haenicke, Henriette, Schulz, Claudia, Schweigerer, Lothar, Mueller, Thomas G., Stiefel, Martina, Belohradsky, Bernd H., Soetedjo, Veronika, Kindle, Gerhard and Grimbacher, Bodo (2019). The German National Registry of Primary Immunodeficiencies (2012-2017). Front. Immunol., 10. LAUSANNE: FRONTIERS MEDIA SA. ISSN 1664-3224
Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata((R)) and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1-25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0-88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE-syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%-subcutaneous; 29%-intravenous; 1%-unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment.
BASE