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Weber christian syndrome

Konstantinos Raymondos, Tamme Dirks, Michael Quintel, Ulrich Molitoris, Jörg Ahrens, Thorben Dieck, Kai Johanning, Dietrich Henzler, Rolf Rossaint, Christian Putensen, Hermann Wrigge, Ralph Wittich, Maximilian Ragaller, Thomas Bein, Martin Beiderlinden, Maxi Sanmann, Christian Rabe, Jörn Schlechtweg, Monika Holler, Fernando Frutos-Vivar, Andres Esteban, Hartmut Hecker, Simone Rosseau, Vera von Dossow, Claudia Spies, Tobias Welte, Siegfried Piepenbrock, Steffen Weber-Carstens
BACKGROUND: This study investigates differences in treatment and outcome of ventilated patients with acute respiratory distress syndrome (ARDS) between university and non-university hospitals in Germany. METHODS: This subanalysis of a prospective, observational cohort study was performed to identify independent risk factors for mortality by examining: baseline factors, ventilator settings (e.g., driving pressure), complications, and care settings-for example, case volume of ventilated patients, size/type of intensive care unit (ICU), and type of hospital (university/non-university hospital)...
May 30, 2017: Critical Care: the Official Journal of the Critical Care Forum
Frank Dodoo-Schittko, Susanne Brandstetter, Magdalena Brandl, Sebastian Blecha, Michael Quintel, Steffen Weber-Carstens, Stefan Kluge, Patrick Meybohm, Caroline Rolfes, Björn Ellger, Friedhelm Bach, Tobias Welte, Thomas Muders, Kathrin Thomann-Hackner, Thomas Bein, Christian Apfelbacher
BACKGROUND: Little is known about the characteristics and real world life circumstances of ARDS (acute respiratory distress syndrome) patient populations. This knowledge is essential for transferring evidence-based therapy into routine healthcare. The aim of this study was to report socio-demographic and clinical characteristics in an unselected population of ARDS patients and to compare these results to findings from other large ARDS cohorts. METHODS: A German based cross-sectional observational study was carried out...
March 2017: Journal of Thoracic Disease
Xiao Liu, Tica Pichulik, Olaf-Oliver Wolz, Truong-Minh Dang, Andrea Stutz, Carly Dillen, Magno Delmiro Garcia, Helene Kraus, Sabine Dickhöfer, Ellen Daiber, Lisa Münzenmayer, Silke Wahl, Nikolaus Rieber, Jasmin Kümmerle-Deschner, Amir Yazdi, Mirita Franz-Wachtel, Boris Macek, Markus Radsak, Sebastian Vogel, Berit Schulte, Juliane Sarah Walz, Dominik Hartl, Eicke Latz, Stephan Stilgenbauer, Bodo Grimbacher, Lloyd Miller, Cornelia Brunner, Christiane Wolz, Alexander N R Weber
BACKGROUND: The Nod-like receptor NACHT, LRR, and PYD domain-containing protein 3 (NLRP3) and Bruton tyrosine kinase (BTK) are protagonists in innate and adaptive immunity, respectively. NLRP3 senses exogenous and endogenous insults, leading to inflammasome activation, which occurs spontaneously in patients with Muckle-Wells syndrome; BTK mutations cause the genetic immunodeficiency X-linked agammaglobulinemia (XLA). However, to date, few proteins that regulate NLRP3 inflammasome activity in human primary immune cells have been identified, and clinically promising pharmacologic targeting strategies remain elusive...
February 16, 2017: Journal of Allergy and Clinical Immunology
Bettina Hartmann, Christian Fottner, Karin Herrmann, Tobias Limbourg, Matthias M Weber, Karlheinz Beckh
AIMS: A major proportion of patients with diabetic foot syndrome are older than 65 years. Little is known about outcomes of these elderly patients. METHODS: We analysed 245 treatment cases in an observational single-centre study for comorbidities and outcomes over a 6-month period. RESULTS: In all, 122 patients had peripheral arterial disease which was significantly increasing with age (n = 245, df = 1, χ(2) = 23.06, p ⩽ 0...
January 2017: Diabetes & Vascular Disease Research
H Christian Weber
PURPOSE OF REVIEW: Irritable bowel syndrome (IBS) is a highly prevalent gastrointestinal disorder with negative impact on quality of life and it represents a substantial economic burden on healthcare cost. The medical management of IBS remains largely symptomatic. This review provides an update related to the most recently published diagnostic Rome IV criteria for IBS and clinical trial data for novel treatment modalities in IBS targeting the peripheral opioid receptors of the enteric nervous system and the gut microbiota...
February 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
Felix Balzer, Mario Menk, Jannis Ziegler, Christian Pille, Klaus-Dieter Wernecke, Claudia Spies, Maren Schmidt, Steffen Weber-Carstens, Maria Deja
BACKGROUND: Currently there is no ARDS definition or classification system that allows optimal prediction of mortality in ARDS patients. This study aimed to examine the predictive values of the AECC and Berlin definitions, as well as clinical and respiratory parameters obtained at onset of ARDS and in the course of the first seven consecutive days. METHODS: The observational study was conducted at a 14-bed intensive care unit specialized on treatment of ARDS. Predictive validity of the AECC and Berlin definitions as well as PaO2/FiO2 and FiO2/PaO2*Pmean (oxygenation index) on mortality of ARDS patients was assessed and statistically compared...
November 8, 2016: BMC Anesthesiology
Ary Serpa Neto, Matthieu Schmidt, Luciano C P Azevedo, Thomas Bein, Laurent Brochard, Gernot Beutel, Alain Combes, Eduardo L V Costa, Carol Hodgson, Christian Lindskov, Matthias Lubnow, Catherina Lueck, Andrew J Michaels, Jose-Artur Paiva, Marcelo Park, Antonio Pesenti, Tài Pham, Michael Quintel, V Marco Ranieri, Michael Ried, Roberto Roncon-Albuquerque, Arthur S Slutsky, Shinhiro Takeda, Pier Paolo Terragni, Marie Vejen, Steffen Weber-Carstens, Tobias Welte, Marcelo Gama de Abreu, Paolo Pelosi, Marcus J Schultz
PURPOSE: Extracorporeal membrane oxygenation (ECMO) is a rescue therapy for patients with acute respiratory distress syndrome (ARDS). The aim of this study was to evaluate associations between ventilatory settings during ECMO for refractory hypoxemia and outcome in ARDS patients. METHODS: In this individual patient data meta-analysis of observational studies in adult ARDS patients receiving ECMO for refractory hypoxemia, a time-dependent frailty model was used to determine which ventilator settings in the first 3 days of ECMO had an independent association with in-hospital mortality...
November 2016: Intensive Care Medicine
Oliver Ristow, Christian Freudlsperger, Moritz Berger, Heidi Bächli, Jürgen Hoffmann, Michael Engel
Regarded singly, both Sturge-Weber syndrome and trigonocephaly are rare congenital disorders. The cardinal features of Sturge-Weber syndrome are facial cutaneous capillary malformation (port-wine stain), leptomeningeal angiomatosis, and glaucoma. Premature closure of the metopic suture results in trigonocephaly. However, to the best of our knowledge, the diagnosis of a combination of both Sturge-Weber syndrome and trigonocephaly has not as yet been reported. This brief clinical study thus presents a patient with the unusual findings of a Sturge-Weber syndrome and simultaneous trigonocephaly induced by premature metopic synostosis...
October 2016: Journal of Craniofacial Surgery
Johanna Stock, Johannes Kuenanz, Niklas Glonke, Joseph Sonntag, Jenny Frese, Burkhard Tönshoff, Britta Höcker, Bernd Hoppe, Markus Feldkötter, Lars Pape, Christian Lerch, Simone Wygoda, Manfred Weber, Gerhard-Anton Müller, Oliver Gross
BACKGROUND: Patients with autosomal or X-linked Alport syndrome (AS) with heterozygous mutations in type IV collagen genes have a 1-20 % risk of progressing to end-stage renal disease during their lifetime. We evaluated the long-term renal outcome of patients at risk of progressive disease (chronic kidney disease stages 1-4) with/without nephroprotective therapy. METHODS: This was a prospective, non-interventional, observational study which included data from a 4-year follow-up of AS patients with heterozygous mutations whose datasets had been included in an analysis of the 2010 database of the European Alport Registry...
July 11, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
A Kuehn, F Codreanu-Morel, C Lehners-Weber, V Doyen, S-A Gomez-André, F Bienvenu, J Fischer, N Ballardini, M van Hage, J-M Perotin, S Silcret-Grieu, H Chabane, F Hentges, M Ollert, C Hilger, M Morisset
BACKGROUND: Fish is one of the most allergenic foods. While clinical cross-reactivity among different fishes is a widely accepted feature of fish allergy, associations with other food allergies are not well understood. This study aims at analyzing the relevance of clinical cross-reactivity between fish and chicken meat in patients with allergy to chicken meat without sensitization to hen's eggs. METHODS: Patients with food allergy to fish and chicken meat (n = 29) or chicken meat only (n = 7) were recruited...
December 2016: Allergy
Philipp Christian Seppelt, Simon Schwill, Alexander Weymann, Rawa Arif, Antje Weber, Marcin Zaradzki, Karsten Richter, Stephan Ensminger, Peter Nicholas Robinson, Andreas H Wagner, Matthias Karck, Klaus Kallenbach
OBJECTIVES: Marfan syndrome is an autosomal dominant inherited disorder of connective tissue. The vascular complications of Marfan syndrome have the biggest impact on life expectancy. The aorta of Marfan patients reveals degradation of elastin layers caused by increased proteolytic activity of matrix metalloproteinases (MMPs). In this study we performed adenoviral gene transfer of human tissue inhibitor of matrix metalloproteinases-1 (hTIMP-1) in aortic grafts of fibrillin-1 deficient Marfan mice (mgR/mgR) in order to reduce elastolysis...
2016: PloS One
Walter Knirsch, Dominik C Benz, Patrick Bühr, Daniel Quandt, Roland Weber, Christian Kellenberger, Christian P Braegger, Oliver Kretschmar
OBJECTIVES: The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. BACKGROUND: CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management...
June 2016: Catheterization and Cardiovascular Interventions
Susanne Brandstetter, Frank Dodoo-Schittko, Sebastian Blecha, Philipp Sebök, Kathrin Thomann-Hackner, Michael Quintel, Steffen Weber-Carstens, Thomas Bein, Christian Apfelbacher
BACKGROUND: Health-related quality of life (HRQoL) and return to work are important outcomes in critical care medicine, reaching beyond mortality. Little is known on factors predictive of HRQoL and return to work in critical illness, including the acute respiratory distress syndrome (ARDS), and no evidence exists on the role of quality of care (QoC) for outcomes in survivors of ARDS. It is the aim of the DACAPO study ("Surviving ARDS: the influence of QoC and individual patient characteristics on quality of life") to investigate the role of QoC and individual patient characteristics on quality of life and return to work...
2015: BMC Health Services Research
Marie L Weber, Dominik T Schneider, Sonja Offenmüller, Peter Kaatsch, Hagen Graf Einsiedel, Martin Benesch, Alexander Claviez, Martin Ebinger, Christof Kramm, Christian Kratz, Jennifer Lawlor, Ivo Leuschner, Susanne Merkel, Markus Metzler, Rainer Nustede, Sabine Petsch, Karl-Heinz Seeger, Paul-Gerhardt Schlegel, Meinolf Suttorp, Oliver Zolk, Ines B Brecht
INTRODUCTION: Colorectal carcinoma (CRC) is the second most common adult cancer in Germany, however, it is extremely rare in children and adolescents. In these patients, previous literature describes aggressive behavior and diagnosis at advanced stage. METHOD: Thirty-one patients with CRC age ≤ 18 years and treated between 1990 and 2012 have been identified through the structures and registries of the German Society for Pediatric Oncology and Hematology. RESULTS: The age range was 9-18 years (median 13...
April 2016: Pediatric Blood & Cancer
Stefanie Weber, Anja K Büscher, Henning Hagmann, Max C Liebau, Christian Heberle, Michael Ludwig, Sabine Rath, Martin Alberer, Antje Beissert, Martin Zenker, Peter F Hoyer, Martin Konrad, Hanns-Georg Klein, Julia Hoefele
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a severe cause of progressive renal disease. Genetic forms of SRNS can present with autosomal recessive or autosomal dominant inheritance. Recent studies have identified mutations in multiple podocyte genes responsible for SRNS. Improved sequencing methods (next-generation sequencing, NGS) now promise rapid mutational testing of SRNS genes. METHODS: In the present study, a simultaneous screening of ten SRNS genes in 37 SRNS patients was performed by NGS...
January 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Christoph Liebetrau, Michael Weber, Stergios Tzikas, Lars Palapies, Helge Möllmann, Gerhard Pioro, Tanja Zeller, Andres Beiras-Fernandez, Christoph Bickel, Andreas M Zeiher, Karl J Lackner, Stephan Baldus, Holger M Nef, Stefan Blankenberg, Christian W Hamm, Thomas Münzel, Till Keller
BACKGROUND: The introduction of modern troponin assays has facilitated diagnosis of acute myocardial infarction due to improved sensitivity with corresponding loss of specificity. Atrial fibrillation (AF) is associated with elevated levels of troponin. The aim of the present study was to evaluate the diagnostic performance of troponin I in patients with suspected acute coronary syndrome and chronic AF. METHODS: Contemporary sensitive troponin I was assayed in a derivation cohort of 90 patients with suspected acute coronary syndrome and chronic AF to establish diagnostic cut-offs...
2015: BMC Medicine
Felicitas Becker, Julian Schubert, Sarah Weckhuysen, Arvid Suls, Steffen Grüninger, Elisabeth Korn-Merker, Anne Hofmann-Peters, Jürgen Sperner, Helen Cross, Kerstin Hallmann, Christian E Elger, Wolfram S Kunz, René Madeleyen, Holger Lerche, Yvonne G Weber
In the recent years, several neurological syndromes related to defects of the glucose transporter type 1 (Glut1) have been descried. They include the glucose transporter deficiency syndrome (Glut1-DS) as the most severe form, the paroxysmal exertion-induced dyskinesia (PED), a form of spastic paraparesis (CSE) as well as the childhood (CAE) and the early-onset absence epilepsy (EOAE). Glut1, encoded by the gene SLC2A1, is the most relevant glucose transporter in the brain. All Glut1 syndromes respond well to a ketogenic diet (KD) and most of the patients show a rapid seizure control...
August 2015: Epilepsy Research
Dennis Lal, Ann-Kathrin Ruppert, Holger Trucks, Herbert Schulz, Carolien G de Kovel, Dorothée Kasteleijn-Nolst Trenité, Anja C M Sonsma, Bobby P Koeleman, Dick Lindhout, Yvonne G Weber, Holger Lerche, Claudia Kapser, Christoph J Schankin, Wolfram S Kunz, Rainer Surges, Christian E Elger, Verena Gaus, Bettina Schmitz, Ingo Helbig, Hiltrud Muhle, Ulrich Stephani, Karl M Klein, Felix Rosenow, Bernd A Neubauer, Eva M Reinthaler, Fritz Zimprich, Martha Feucht, Rikke S Møller, Helle Hjalgrim, Peter De Jonghe, Arvid Suls, Wolfgang Lieb, Andre Franke, Konstantin Strauch, Christian Gieger, Claudia Schurmann, Ulf Schminke, Peter Nürnberg, Thomas Sander
Genetic generalised epilepsy (GGE) is the most common form of genetic epilepsy, accounting for 20% of all epilepsies. Genomic copy number variations (CNVs) constitute important genetic risk factors of common GGE syndromes. In our present genome-wide burden analysis, large (≥ 400 kb) and rare (< 1%) autosomal microdeletions with high calling confidence (≥ 200 markers) were assessed by the Affymetrix SNP 6.0 array in European case-control cohorts of 1,366 GGE patients and 5,234 ancestry-matched controls...
May 2015: PLoS Genetics
Richard Martin Sellei, Simon Johannes Hingmann, Philipp Kobbe, Christian Weber, John Edward Grice, Frauke Zimmerman, Sabine Jeromin, Frank Hildebrand, Hans-Christoph Pape
BACKGROUND: Decision-making in treatment of an acute compartment syndrome is based on clinical assessment, supported by invasive monitoring. Thus, evolving compartment syndrome may require repeated pressure measurements. In suspected cases of potential compartment syndromes clinical assessment alone seems to be unreliable. The objective of this study was to investigate the feasibility of a non-invasive application estimating whole compartmental elasticity by ultrasound, which may improve accuracy of diagnostics...
2015: Patient Safety in Surgery
Johannes Dorst, Luc Dupuis, Susanne Petri, Katja Kollewe, Susanne Abdulla, Joachim Wolf, Markus Weber, David Czell, Christian Burkhardt, Frank Hanisch, Stefan Vielhaber, Thomas Meyer, Gabriele Frisch, Dagmar Kettemann, Torsten Grehl, Bertold Schrank, Albert C Ludolph
Weight loss is increasingly considered as a negative prognostic marker in amyotrophic lateral sclerosis (ALS). Despite the critical importance of nutritional issues in ALS, and the common use of percutaneous endoscopic gastrostomy (PEG), there is a general lack of knowledge on peri-interventional treatment, optimal parameters of enteral nutrition, its timing during disease progression and its potential disease-modifying effects in ALS patients. Here we report the results of a multi-center prospective study of percutaneous endoscopic gastrostomy (PEG) in ALS...
2015: Journal of Neurology
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