7 results on '"C Smaczny"'
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2. Mukoviszidose (Zystische Fibrose): Ernährung und exokrine Pankreasinsuffizienz
- Author
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B. Palm, C. Smaczny, H. Ellemunter, M. Stern, and H.-G. Posselt
- Subjects
business.industry ,Medicine ,business - Published
- 2007
3. Autorenverzeichnis
- Author
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L. Graul-Neumann, D. Horn, C. Hübner, P. Huppke, R. König, F. Majewski, P. Meinecke, R. Pankau, T. Rosenbaum, D. Schnabel, M. Schuelke, J. Spranger, U. Theile, S. Tinschert, E. Wilichowski, H.A. Wollmann, M. Zenker, P. Bartmann, D. Bassler, C. Bührer, A.W. Flemmer, J. Forster, A. Franz, M. Gonser, L. Gortner, P. Groneck, R. Hentschel, E. Herting, U.B. Hoyme, H. Hummler, C. Jandeck, G. Jorch, R. Korinthenberg, J. Liese, R.F. Maier, J. Martius, A. Merkenschlager, C.F. Poets, F. Pohlandt, C. Roll, R. Roos, B. Roth, K.T.M. Schneider, Ch. Speer, H. Stopfkuchen, A. Teichmann, W. Thomas, K. Vetter, A. von der Wense, S. Zielen, B. Assmann, G.F. Hoffmann, S. Kölker, M. Lindner, E. Mönch, R. Santer, U. Spiekerkötter, J. Zschocke, K. Bauer, H.-J. Böhles, Jack Sinclair, K.W. Jauch, F. Jochum, Thomas Kauth, B. Koletzko, M. Krawinkel, K. Krohn, Walter Mihatsch, A. Moß, S. Mühlebach, S. Verwied-Jorky, M. Wabitsch, K.-P. Zimmer, N. Albers, D. L'Allemand, G. Binder, J.H. Brämswig, H.G. Dörr, A. Grüters-Kieslich, B.P. Hauffa, S. Heger, O. Hiort, R. Holl, P.M. Holterhus, B. Köhler, Eckhard Korsch, J. Kratzsch, H. Krude, K. Mohnike, A. Neu, R. Pfäffle, A. Richter-Unruh, F.G. Riepe, G. Simic-Schleicher, E. Schönau, G. Sinnecker, W. Sippell, H. Willgerodt, J. Wölfle, S.A. Wudy, E. Aygören-Pürsün, M. Bas, U. Baumann, T. Biedermann, J. Blume, B. Buchholz, G. Dückers, D. Dunsch, M. Edelhäuser, S. Ehl, C. Feiterna-Sperling, M. Funk, K. Hartmann, C. Königs, W. Kreuz, J. Krudewig, H.-J. Laws, R. Linde, I. Martinez-Saguer, M. Maurer, David Nadal, T. Niehues, G. Notheis, H. Ott, I. Schulze, B. Wedi, U. Wintergerst, G. Bürk, I. Foeldvari, M. Frosch, H. Girschick, K. Gerhold, N. Guellac, J.P. Haas, R. Häfner, W. Häuser, A. Heiligenhaus, T. Hospach, G. Horneff, H.-I. Huppertz, A. Illhardt, A.F. Jansson, T. Kallinich, H. Michels, K. Mönkemöller, U. Neudorf, M. Richter, E. Schnöbel-Müller, A. Thon, B. Zernikow, W. Behnisch, H. Cario, R. Dickerhoff, S. Eber, M. Führer, E. Kohne, A.E. Kulozik, J. Kunz, M. Muckenthaler, W. Eberl, G. Gaedicke, W. Muntean, W. Streif, J.D. Beck, F. Berthold, S. Bielack, G. Calaminus, A. Claviez, U. Creutzig, U. Dirksen, M. Dworzak, U. Göbel, N. Graf, B. Grießmeier, G. Henze, B. Hero, H. Jürgens, U. Kaiser, T. Klingebiel, E. Koscielniak, C. Kramm, T. Langer, B. Lawrenz, T. Lehrnbecher, U. Leiss, H.-J. Mentzel, M. Minkov, J. Peitz, R. Placzek, D. Reinhardt, A. Reiter, S. Rutkowski, P. Schmittenbecher, D.T. Schneider, B.M. Schreiber-Gollwitzer, M. Schrappe, H. Schroten, H.M. Schröder, V. Schuster, D. von Schweinitz, N. Sörensen, G. Tallen, B. Timmermann, M. Warmuth-Metz, M. Weckesser, L. Wessel, T. Wirth, J.E.A. Wolff, W. Wößmann, A. am Zehnhoff-Dinnesen, C. Apitz, R. Arnold, H. Baumgartner, G. Bennink, H. Bertram, M. Blankenburg, G. Bönner, J. von der Breek, J. Breuer, R. Buchhorn, J. Bürsch, R. Cesnjevar, I. Dähnert, I. Deisenhofer, G.-P. Diller, T. Doenst, K.-O. Dubowy, A. Eicken, P. Ewert, C. Fink, J. Franke, R. Gebauer, M. Gorenflo, null Grabitz, N.A. Haas, H.-J. Häusler, A. Hager, J. Hebebrand, W. Henschel, M. Hirt, M.M. Hoeper, J. Hörer, M. Hofbeck, A. Horke, V. Hraska, M. Hulpke-Wette, J. Janou šek, C. Jux, L. Kändler, R. Kandolf, R. Kaulitz, W. Kienast, S. Klaassen, W. Knirsch, H.H. Kramer, J.G. Kreuder, T. Kriebel, S. Läer, K.T. Laser, T.-P. Lê, M.A.G. Lewin, A. Lindinger, C.R. Mackenzie, S. Mebus, S.H. van der Mei, O. Miera, S. Ovroutski, T. Paul, J. Photiadis, R. Dalla Pozza, C. Rickers, W. Rosendahl, W. Ruschewski, J.S. Sachweh, H.-J. Schäfers, J. Scheewe, K.-R. Schirmer, C. Schlensak, M. Schlez, A.A. Schmaltz, K. Schmitt, H. Schneider, M.B. Schneider, D. Schranz, C. Schreiber, I. Schulze-Neick, L.F.J. Sieverding, H. Singer, J. Stieh, N. Sreeram, W.-R. Thies, J. Thul, R. Trauzeddel, C. Tschöpe, A. Uebing, H.E. Ulmer, M. Vogel, M. Vogt, J. Weil, A. Wessel, J.C. Will, E. Wühl, M. Ballmann, J. Barben, C.P. Bauer, J. Bend, D. Berdel, O. Blankenstein, W. Bremer, F. Brunsmann, T. Buchholz, A. Bufe, N. Derichs, E. Eber, F. Friedrichs, T. Frischer, U. Gembruch, U. Gieler, M. Götz, W.H. Haas, E. Hamelmann, J. Hammer, M. Hellermann, J. Jacobeit, A. Jung, V. Keim, R. Kitz, A. Kleinheinz, S. Koletzko, I. Kopp, M. Kopp, S. Lau, R. Lauener, null Loff, K. Magdorf, C. Muche-Borowski, F.-M. Müller, H. Müsken, L. Naehrlich, T. Nicolai, Th. Nüßlein, E. Paditz, Frau B. Palm, K. Paul, S. Pfeiffer-Auler, Frau D. Pfeiffer-Kascha, H.-G. Posselt, B. Przybilla, H.-C. Räwer, F. Ratjen, I. Reese, J. Riedler, E. Rietschel, M. Rose, R. Rossi, F. Ruëff, T. Schäfer, S. Schmidt, S. Schmitt-Grohé, J. Schulze, A. Schuster, J. Seidenberg, H. Sitter, C. Smaczny, T. Spindler, D. Staab, M. Stern, C.P. Strassburg, K. Strömer, M. Stuhrmann-Spangenberg, R. Szczepanski, A. Tacke, M. Tiedgen, M.S. Urschitz, J. Vagts, C. Vogelberg, U. Wahn, A. Walker, T. Werfel, J.H. Wildhaber, M. Zach, Th. Zimmermann, A. Ballauff, N. Bannert, I. Böhn, S. Buderus, P. Bufler, M. Burdelski, P. Gerner, K.-P. Grosse, J. Henker, P. Henneke, W. Huber, T. Lang, M.J. Lentze, M. Melter, T. Müller, E.-D. Pfister, B. Rodeck, A. Schmidt-Choudhury, H. Skopnik, S. Wirth, H. Witt, H. Bachmann, J. Dötsch, J.H. Ehrich, Arno Fuchshuber, B. Hoppe, P.F. Hoyer, M.J. Kemper, D. Michalk, D. Müller, D.E. Müller-Wiefel, M. Pohl, B. Tönshoff, K. Zerres, T. Bast, F.A.M. Baumeister, R. Berner, H. Bode, H.J. Christen, H. Collmann, F. Ebinger, H. Eiffert, S. Evers, R. Gold, S. Groß, F. Hanefeld, F. Heinen, H. Holthausen, A. Hübner, G. Jacobi, D. Karch, C. Kauschke, G. Kerkhoff, C. Kiese-Himmel, J. Klepper, A. Kohlschütter, E. Korn-Merker, I. Krägeloh-Mann, P. Kropp, G. Kurlemann, U. de Langen-Müller, H.G. Lenard, Th. Michael, A. von Moers, U. Felderhoff-Müser, R. Nau, B.A. Neubauer, G. Neuhäuser, K. Neumann, M. Noterdaeme, R. Pothmann, D. Rating, B. Reitter, E. Rickels, A.M. Ritz, H. Rosenkötter, B. Schmitt, U. Stephani, B. Stöver, D. Tibussek, R. Trollmann, G. Trommer, I. Tuxhorn, G. Wohlrab, K.P. Boergen, S. Brosch, W. Delb, R. Frank, B. Herrmann, N. von Hofacker, O. Kraus de Camargo, R.v. Kries, R. Michaelis, M. Papousek, H.G. Schlack, J. Schriever, K. Skrodzki, H.-M. Straßburg, U. Thyen, K. Becker, T. Fels, G. Fitze, S. Grasshoff-Derr, P. Göbel, P. Illing, J. Lieber, A. Schmidt, L.M. Wessel, L.D. Berthold, G. Hahn, W. Hirsch, J.D. Moritz, C. Schröder, R. Schumacher, J. Stegmann, M. Steinborn, R. Tietze, R. Wunsch, W. Deppe, T. Hermann, D. Kiosz, E. Leidig, H. Mayer, J. Oepen, R. Stachow, F. Ahrens, G. Frey, I. Huttegger, M.-L. Preil, P.P. Schmittenbecher, H. Traupe, O. Eberhardt, C. Hasler, R. Krauspe, N.M. Meenen, A. Meurer, R. Rödl, R. Stücker, and C. Zilkens
- Published
- 2007
4. Every CFTR variant counts - Target-capture based next-generation-sequencing for molecular diagnosis in the German CF Registry.
- Author
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Ahting S, Nährlich L, Held I, Henn C, Krill A, Landwehr K, Meister J, Nährig S, Nolde A, Remke K, Ruppel R, Sauer-Heilborn A, Schebek M, Schopper G, Schulte-Hubbert B, Schwarz C, Smaczny C, Wege S, and Hentschel J
- Subjects
- Humans, Germany, Genetic Testing methods, Female, Male, Genotype, Mutation, Cystic Fibrosis genetics, Cystic Fibrosis diagnosis, Cystic Fibrosis Transmembrane Conductance Regulator genetics, Registries, High-Throughput Nucleotide Sequencing methods
- Abstract
Background: In times of genotype guided therapy options, a total of 3.2 % of people with CF (pwCF) in the German CF Registry[1] only have one or no CFTR-variant detected after genetic analysis. Additionally, genetic data in the Registry can be documented as free text and can therefore be prone to error. In order to allow the greatest possible amount of pwCF access to modern therapies, we conducted a re-evaluation of free text entries and established a custom-whole-CFTR-locus NGS-approach for all pwCF who remained without genetic confirmation afterwards., Methods: To this end, we assembled 731 free text variants of 655 pwCF in the German CF Registry. All variants were evaluated using ClinVar, HGMD and CFTR1/2, corrected in the Registries' database and uploaded to ClinVar. PwCF whose diagnosis remained uncertain as well as additional pwCF or pwCFTR-RD that were assembled through a nationwide call for testing of unclear cases were offered genetic analysis. Samples were analysed using a target-capture based NGS-custom-design-panel covering the entire CFTR-locus., Results: Evaluation of free text variants led to the discovery of 43 variants not formerly reported in the context of CF. The Registries' dropdown list was extended by 497 variants and over 500 pwCF were provided with their most up-to-date genotype. Samples of 47 pwCF/pwCFTR-RD were sequenced via NGS with an overall success rate of 61.7 %, resulting in implementation of entire CFTR-genotyping into routine diagnostics., Conclusion: Entire CFTR-genotyping can greatly increase the genetic diagnostic rate of pwCF/pwCFTR-RD and should be considered after inconspicuous CFTR screening panels in CFTR-diagnostics., Competing Interests: Declaration of Competing Interest The following authors declare a conflict of interest in preparing this article: SN has received grants from the CTN-ECFS, DZL and a Speaker’s fee from Vertex pharmaceuticals. LN has received institutional fees from the German Center for Lung Research, Vertex Pharmaceuticals and the Mukoviszidose Institute. He participated in the Trial Steering Committee for CF STORM and is the Medical lead of the German CF-registry and the Pharmacovigilance study manager of the ECFSPR. CS has received a Speaker’s fee from Vertex, TFF Pharma, Chiesi and Viatris and is an ECFS Board Member.All other author’s declare no conflicts of interest., (Copyright © 2023 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)
- Published
- 2024
- Full Text
- View/download PDF
5. Airway inflammation in mild cystic fibrosis.
- Author
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Eckrich J, Zissler UM, Serve F, Leutz P, Smaczny C, Schmitt-Grohé S, Fussbroich D, Schubert R, Zielen S, and Eickmeier O
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- Adolescent, Biomarkers analysis, Cell Count methods, Child, Female, Humans, Male, Patient Acuity, Respiratory Function Tests methods, Sputum immunology, Statistics as Topic, Cystic Fibrosis complications, Cystic Fibrosis diagnosis, Inflammation diagnosis, Inflammation etiology, Inflammation immunology, Interleukins analysis, Respiratory System immunology, Respiratory System physiopathology, Respiratory Tract Infections diagnosis, Respiratory Tract Infections etiology, Respiratory Tract Infections immunology, Tumor Necrosis Factor-alpha analysis
- Abstract
Background: Airway infection and inflammation play major roles in the progression of cystic fibrosis (CF) lung disease. In patients with mild disease, airway inflammation is a clinically relevant and often underdiagnosed feature. Lung function, sputum cell counts, and cytokine profiles in CF with mild disease might be different in patients with and without involvement of small airway disease (SAD)., Methods: Patients with mild CF (n=32) and 22 healthy controls were enrolled in this study. Patients with CF were assigned to two groups: (1) patients without SAD (n=19, median age 12.3years, MEF
25 >50% predicted), and (2) patients with SAD (n=13 median age, 13.2years, MEF25 <50% predicted). Lung function parameters were measured, cells in induced sputum were counted, and cytokines/chemokines (IL-1β, IL-6, IL-8, TNF-α) were analyzed by real-time quantitative PCR (qRT-PCR) and cytometric bead array (CBA)., Results: Patients with CF had significant elevated levels of pro-inflammatory genes in qRT-PCR and secreted gene products in CBA compared to controls. Patients with CF and SAD had significantly increased trapped air (RV/TLC) and pronounced airway inflammation compared to controls as indicated by elevated levels of sputum biomarkers like total cells, neutrophils, and IL6., Conclusions: Our study demonstrated that patients with CF with mild disease defined by lung function might be further endotyped according to their involvement of SAD. In patients with CF and SAD, airway neutrophilic inflammation is more pronounced and is in part distinct from that seen in patients without SAD., (Copyright © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)- Published
- 2017
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6. BIIL 284 reduces neutrophil numbers but increases P. aeruginosa bacteremia and inflammation in mouse lungs.
- Author
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Döring G, Bragonzi A, Paroni M, Aktürk FF, Cigana C, Schmidt A, Gilpin D, Heyder S, Born T, Smaczny C, Kohlhäufl M, Wagner TO, Loebinger MR, Bilton D, Tunney MM, Elborn JS, Pier GB, Konstan MW, and Ulrich M
- Subjects
- Adult, Animals, Anti-Inflammatory Agents administration & dosage, Anti-Inflammatory Agents adverse effects, Disease Models, Animal, Female, Humans, Inflammation metabolism, Inflammation physiopathology, Leukocyte Count, Lung microbiology, Lung pathology, Male, Mice, Pseudomonas Infections blood, Pseudomonas Infections complications, Pseudomonas Infections physiopathology, Receptors, Leukotriene B4 antagonists & inhibitors, Treatment Outcome, Amidines administration & dosage, Amidines adverse effects, Bacteremia etiology, Carbamates administration & dosage, Carbamates adverse effects, Cystic Fibrosis blood, Cystic Fibrosis complications, Cystic Fibrosis physiopathology, Inflammation drug therapy, Neutrophils drug effects, Neutrophils metabolism, Pseudomonas aeruginosa drug effects, Pseudomonas aeruginosa isolation & purification
- Abstract
Background: A clinical study to investigate the leukotriene B(4) (LTB(4))-receptor antagonist BIIL 284 in cystic fibrosis (CF) patients was prematurely terminated due to a significantly increased risk of adverse pulmonary events. We aimed to establish the effect of BIIL284 in models of Pseudomonas aeruginosa lung infection, thereby contributing to a better understanding of what could have led to adverse pulmonary events in CF patients., Methods: P. aeruginosa DNA in the blood of CF patients during and after acute pulmonary exacerbations and in stable patients with non-CF bronchiectasis (NCFB) and healthy individuals was assessed by PCR. The effect of BIIL 284 treatment was tested in an agar bead murine model of P. aeruginosa lung infection. Bacterial count and inflammation were evaluated in lung and other organs., Results: Most CF patients (98%) and all patients with NCFB and healthy individuals had negative P. aeruginosa DNA in their blood. Similarly, the P. aeruginosa-infected mice showed bacterial counts in the lung but not in the blood or spleen. BIIL 284 treatment decreased pulmonary neutrophils and increased P. aeruginosa numbers in mouse lungs leading to significantly higher bacteremia rates and lung inflammation compared to placebo treated animals., Conclusions: Decreased airway neutrophils induced lung proliferation and severe bacteremia in a murine model of P. aeruginosa lung infection. These data suggest that caution should be taken when administering anti-inflammatory compounds to patients with bacterial infections., (© 2013.)
- Published
- 2014
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7. Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis.
- Author
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Friedrich-Rust M, Schlueter N, Smaczny C, Eickmeier O, Rosewich M, Feifel K, Herrmann E, Poynard T, Gleiber W, Lais C, Zielen S, Wagner TO, Zeuzem S, and Bojunga J
- Subjects
- Adolescent, Adult, Child, Female, Fibrosis diagnostic imaging, Fibrosis epidemiology, Fibrosis etiology, Humans, Liver Cirrhosis etiology, Male, Middle Aged, Prevalence, Prospective Studies, Young Adult, Cystic Fibrosis complications, Elasticity Imaging Techniques methods, Liver Cirrhosis diagnostic imaging, Liver Cirrhosis epidemiology, Pancreas diagnostic imaging, Pancreas pathology
- Abstract
Background: Patients with cystic fibrosis (CF) have a relevant morbidity and mortality caused by CF-related liver-disease. While transient elastography (TE) is an established elastography method in hepatology centers, Acoustic-Radiation-Force-Impulse (ARFI)-Imaging is a novel ultrasound-based elastography method which is integrated in a conventional ultrasound-system. The aim of the present study was to evaluate the prevalence of liver-fibrosis in patients with CF using TE, ARFI-imaging and fibrosis blood tests., Methods: 106 patients with CF were prospectively included in the present study and received ARFI-imaging of the left and right liver-lobe, ARFI of the pancreas TE of the liver and laboratory evaluation., Results: The prevalence of liver-fibrosis according to recently published best practice guidelines for CFLD was 22.6%. Prevalence of significant liver-fibrosis assessed by TE, ARFI-right-liver-lobe, ARFI-left-liver-lobe, Fibrotest, Fibrotest-corrected-by-haptoglobin was 17%, 24%, 40%, 7%, and 16%, respectively. The best agreement was found for TE, ARFI-right-liver-lobe and Fibrotest-corrected-by-haptoglobin. Patients with pancreatic-insufficiency had significantly lower pancreas-ARFI-values as compared to patients without., Conclusions: ARFI-imaging and TE seem to be promising non-invasive methods for detection of liver-fibrosis in patients with CF., (Copyright © 2013 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)
- Published
- 2013
- Full Text
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