TY  - JOUR
A1  - Tony, Hans-Peter
A1  - Burmester, Gerd
A1  - Schulze-Koops, Hendrik
A1  - Grunke, Mathias
A1  - Henes, Joerg
A1  - Kötter, Ina
A1  - Haas, Judith
A1  - Unger, Leonore
A1  - Lovric, Svjetlana
A1  - Haubitz, Marion
A1  - Fischer-Betz, Rebecca
A1  - Chehab, Gamal
A1  - Rubbert-Roth, Andrea
A1  - Specker, Christof
A1  - Weinerth, Jutta
A1  - Holle, Julia
A1  - Müller-Ladner, Ulf
A1  - König, Ramona
A1  - Fiehn, Christoph
A1  - Burgwinkel, Philip
A1  - Budde, Klemens
A1  - Sörensen, Helmut
A1  - Meurer, Michael
A1  - Aringer, Martin
A1  - Kieseier, Bernd
A1  - Erfurt-Berge, Cornelia
A1  - Sticherling, Michael
A1  - Veelken, Roland
A1  - Ziemann, Ulf
A1  - Strutz, Frank
A1  - von Wussow, Praxis
A1  - Meier, Florian MP
A1  - Hunzelmann, Nico
A1  - Schmidt, Enno
A1  - Bergner, Raoul
A1  - Schwarting, Andreas
A1  - Eming, Rüdiger
A1  - Schwarz-Eywill, Michael
A1  - Wassenberg, Siegfried
A1  - Fleck, Martin
A1  - Metzler, Claudia
A1  - Zettl, Uwe
A1  - Westphal, Jens
A1  - Heitmann, Stefan
A1  - Herzog, Anna L.
A1  - Wiendl, Heinz
A1  - Jakob, Waltraud
A1  - Schmidt, Elvira
A1  - Freivogel, Klaus
A1  - Dörner, Thomas
A1  - Hertl, Michael
A1  - Stadler, Rudolf
T1  - Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID)
JF  - Arthritis Research & Therapy
N2  - Introduction: 

Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkin’s lymphoma in a real-life clinical setting. 

Methods: 

Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases. The main outcome measures were safety and clinical response, as judged at the discretion of the investigators. 

Results: 

A total of 370 patients (299 patient-years) with various autoimmune diseases (23.0% with systemic lupus erythematosus, 15.7% antineutrophil cytoplasmic antibody-associated granulomatous vasculitides, 15.1% multiple sclerosis and 10.0% pemphigus) from 42 centres received a mean dose of 2,440 mg of rituximab over a median (range) of 194 (180 to 1,407) days. The overall rate of serious infections was 5.3 per 100 patient-years during rituximab therapy. Opportunistic infections were infrequent across the whole study population, and mostly occurred in patients with systemic lupus erythematosus. There were 11 deaths (3.0% of patients) after rituximab treatment (mean 11.6 months after first infusion, range 0.8 to 31.3 months), with most of the deaths caused by infections. Overall (n = 293), 13.3% of patients showed no response, 45.1% showed a partial response and 41.6% showed a complete response. Responses were also reflected by reduced use of glucocorticoids and various immunosuppressives during rituximab therapy and follow-up compared with before rituximab. Rituximab generally had a positive effect on patient well-being (physician’s visual analogue scale; mean improvement from baseline of 12.1 mm)
KW  - GRAID
Y1  - 2011
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-142856
VL  - 13
IS  - R75
ER  - 
TY  - JOUR
A1  - El-Helou, Sabine M.
A1  - Biegner, Anika-Kerstin
A1  - Bode, Sebastian
A1  - Ehl, Stephan R.
A1  - Heeg, Maximilian
A1  - Maccari, Maria E.
A1  - Ritterbusch, Henrike
A1  - Speckmann, Carsten
A1  - Rusch, Stephan
A1  - Scheible, Raphael
A1  - Warnatz, Klaus
A1  - Atschekzei, Faranaz
A1  - Beider, Renata
A1  - Ernst, Diana
A1  - Gerschmann, Stev
A1  - Jablonka, Alexandra
A1  - Mielke, Gudrun
A1  - Schmidt, Reinhold E.
A1  - Schürmann, Gesine
A1  - Sogkas, Georgios
A1  - Baumann, Ulrich H.
A1  - Klemann, Christian
A1  - Viemann, Dorothee
A1  - Bernuth, Horst von
A1  - Krüger, Renate
A1  - Hanitsch, Leif G.
A1  - Scheibenbogen, Carmen M.
A1  - Wittke, Kirsten
A1  - Albert, Michael H.
A1  - Eichinger, Anna
A1  - Hauck, Fabian
A1  - Klein, Christoph
A1  - Rack-Hoch, Anita
A1  - Sollinger, Franz M.
A1  - Avila, Anne
A1  - Borte, Michael
A1  - Borte, Stephan
A1  - Fasshauer, Maria
A1  - Hauenherm, Anja
A1  - Kellner, Nils
A1  - Müller, Anna H.
A1  - Ülzen, Anett
A1  - Bader, Peter
A1  - Bakhtiar, Shahrzad
A1  - Lee, Jae-Yun
A1  - Heß, Ursula
A1  - Schubert, Ralf
A1  - Wölke, Sandra
A1  - Zielen, Stefan
A1  - Ghosh, Sujal
A1  - Laws, Hans-Juergen
A1  - Neubert, Jennifer
A1  - Oommen, Prasad T.
A1  - Hönig, Manfred
A1  - Schulz, Ansgar
A1  - Steinmann, Sandra
A1  - Klaus, Schwarz
A1  - Dückers, Gregor
A1  - Lamers, Beate
A1  - Langemeyer, Vanessa
A1  - Niehues, Tim
A1  - Shai, Sonu
A1  - Graf, Dagmar
A1  - Müglich, Carmen
A1  - Schmalzing, Marc T.
A1  - Schwaneck, Eva C.
A1  - Tony, Hans-Peter
A1  - Dirks, Johannes
A1  - Haase, Gabriele
A1  - Liese, Johannes G.
A1  - Morbach, Henner
A1  - Foell, Dirk
A1  - Hellige, Antje
A1  - Wittkowski, Helmut
A1  - Masjosthusmann, Katja
A1  - Mohr, Michael
A1  - Geberzahn, Linda
A1  - Hedrich, Christian M.
A1  - Müller, Christiane
A1  - Rösen-Wolff, Angela
A1  - Roesler, Joachim
A1  - Zimmermann, Antje
A1  - Behrends, Uta
A1  - Rieber, Nikolaus
A1  - Schauer, Uwe
A1  - Handgretinger, Rupert
A1  - Holzer, Ursula
A1  - Henes, Jörg
A1  - Kanz, Lothar
A1  - Boesecke, Christoph
A1  - Rockstroh, Jürgen K.
A1  - Schwarze-Zander, Carolynne
A1  - Wasmuth, Jan-Christian
A1  - Dilloo, Dagmar
A1  - Hülsmann, Brigitte
A1  - Schönberger, Stefan
A1  - Schreiber, Stefan
A1  - Zeuner, Rainald
A1  - Ankermann, Tobias
A1  - Bismarck, Philipp von
A1  - Huppertz, Hans-Iko
A1  - Kaiser-Labusch, Petra
A1  - Greil, Johann
A1  - Jakoby, Donate
A1  - Kulozik, Andreas E.
A1  - Metzler, Markus
A1  - Naumann-Bartsch, Nora
A1  - Sobik, Bettina
A1  - Graf, Norbert
A1  - Heine, Sabine
A1  - Kobbe, Robin
A1  - Lehmberg, Kai
A1  - Müller, Ingo
A1  - Herrmann, Friedrich
A1  - Horneff, Gerd
A1  - Klein, Ariane
A1  - Peitz, Joachim
A1  - Schmidt, Nadine
A1  - Bielack, Stefan
A1  - Groß-Wieltsch, Ute
A1  - Classen, Carl F.
A1  - Klasen, Jessica
A1  - Deutz, Peter
A1  - Kamitz, Dirk
A1  - Lassy, Lisa
A1  - Tenbrock, Klaus
A1  - Wagner, Norbert
A1  - Bernbeck, Benedikt
A1  - Brummel, Bastian
A1  - Lara-Villacanas, Eusebia
A1  - Münstermann, Esther
A1  - Schneider, Dominik T.
A1  - Tietsch, Nadine
A1  - Westkemper, Marco
A1  - Weiß, Michael
A1  - Kramm, Christof
A1  - Kühnle, Ingrid
A1  - Kullmann, Silke
A1  - Girschick, Hermann
A1  - Specker, Christof
A1  - Vinnemeier-Laubenthal, Elisabeth
A1  - Haenicke, Henriette
A1  - Schulz, Claudia
A1  - Schweigerer, Lothar
A1  - Müller, Thomas G.
A1  - Stiefel, Martina
A1  - Belohradsky, Bernd H.
A1  - Soetedjo, Veronika
A1  - Kindle, Gerhard
A1  - Grimbacher, Bodo
T1  - The German national registry of primary immunodeficiencies (2012-2017)
JF  - Frontiers in Immunology
N2  - 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.
KW  - registry for primary immunodeficiency
KW  - primary immunodeficiency (PID)
KW  - German PID-NET registry
KW  - PID prevalence
KW  - European Society for Immunodeficiencies (ESID)
KW  - IgG substitution therapy
KW  - CVID
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-226629
VL  - 10
ER  - 
TY  - JOUR
A1  - Recke, Andreas
A1  - Konitzer, Sarah
A1  - Lemcke, Susanne
A1  - Freitag, Miriam
A1  - Sommer, Nele Maxi
A1  - Abdelhady, Mohammad
A1  - Amoli, Mahsa M.
A1  - Benoit, Sandrine
A1  - El-Chennawy, Farha
A1  - Eldarouti, Mohammad
A1  - Eming, Rüdiger
A1  - Gläser, Regine
A1  - Günther, Claudia
A1  - Hadaschik, Eva
A1  - Homey, Bernhard
A1  - Lieb, Wolfgang
A1  - Peitsch, Wiebke K.
A1  - Pföhler, Claudia
A1  - Robati, Reza M.
A1  - Saeedi, Marjan
A1  - Sárdy, Miklós
A1  - Sticherling, Michael
A1  - Uzun, Soner
A1  - Worm, Margitta
A1  - Zillikens, Detlef
A1  - Ibrahim, Saleh
A1  - Vidarsson, Gestur
A1  - Schmidt, Enno
T1  - The p.Arg435His Variation of IgG3 With High Affinity to FcRn Is Associated With Susceptibility for Pemphigus Vulgaris-Analysis of Four Different Ethnic Cohorts
JF  - frontiers in Immunology
N2  - IgG3 is the IgG subclass with the strongest effector functions among all four IgG subclasses and the highest degree of allelic variability among all constant immunoglobulin genes. Due to its genetic position, IgG3 is often the first isotype an antibody switches to before IgG1 or IgG4. Compared with the other IgG subclasses, it has a reduced half-life which is probably connected to a decreased affinity to the neonatal Fc receptor (FcRn). However, a few allelic variants harbor an amino acid replacement of His435 to Arg that reverts the half-life of the resulting IgG3 to the same level as the other IgG subclasses. Because of its functional impact, we hypothesized that the p.Arg435His variation could be associated with susceptibility to autoantibody-mediated diseases like pemphigus vulgaris (PV) and bullous pemphigoid (BP). Using a set of samples from German, Turkish, Egyptian, and Iranian patients and controls, we were able to demonstrate a genetic association of the p.Arg435His variation with PV risk, but not with BP risk. Our results suggest a hitherto unknown role for the function of IgG3 in the pathogenesis of PV.
KW  - immunology
KW  - dermatology
KW  - autoantibodies
KW  - allotype
KW  - pemphigus
KW  - Diagnose
KW  - pemphigoid
KW  - half-life
KW  - functional genetics
Y1  - 2018
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-225073
VL  - 9
ER  - 
TY  - THES
A1  - Schmidt, Claudia
T1  - Ausbildung von oraler Motor Fluency unter Manipulation der Zunge und der sensiblen Nervenbahn mittels Elektrostimulation und Leitungsanästhesie
T1  - Formation of oral motor fluency during manipulation of the tongue muscles and the sensory nerve tract by means of electrostimulation and anesthesia
N2  - Diese Dissertation untersucht die Ausbildung motorischer Verarbeitungsflüssigkeit bei verbalen Stimuli, welche als Ursache für die Entstehung des Mere-Exposure Effekts angesehen wird. Ziel war es herauszufinden, ob eine Bewegung der Zunge, die external mittels Elektrostimulation verursacht wurde, Einfluss auf den Mere-Exposure Effekt ausübt. Des Weiteren wurde untersucht, ob der Effekt unter Elektrostimulation und gleichzeitiger Störung der Afferenz mittels Lokalanästhesie auftritt. Ergebnis dieser Studie war, dass der Mere-Exposure Effekt in der Gesamtstichprobe mit N = 68 Probanden nachzuweisen war. Eine bloße Motorinterferenz der Zunge ist demnach nicht der entscheidende Faktor für die Ausbildung von Fluency. Auch eine Ausschaltung der Afferenz mittels Anästhesie bei N = 37 Probanden konnte den Effekt nicht eliminieren und scheint somit keinen maßgeblichen Einfluss auf Fluency für verbale Stimuli zu haben.
N2  - This thesis investigates the occurrence of motor processing fluency in verbal stimuli, which is considered to be responsible for the development of the mere exposure effect. The target was to find out, if a movement of the tongue muscles, caused externally by electrostimulation, has an impact on the mere exposure effect. Furthermore, it was investigated whether the effect occurs during electrostimulation and concomitant interference of the afference by means of local anesthesia. The result of this study was that the mere exposure effect was to be detected in the total sample with N = 68 subjects. Thus, mere movement of the tongue muscles is not the deciding factor in the formation of fluency. Moreover, it was shown that the elimination of the afference by anesthesia in N = 37 subjects could not obliterate the effect and therefore does not seem to have a significant influence on fluency.
KW  - Mere-Exposure-Effekt
KW  - Wortflüssigkeit
KW  - Implizites Gedächtnis
KW  - Mere-Exposure-Effekt
KW  - mere exposure effect
KW  - Verarbeitungsflüssigkeit
KW  - fluency
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-179146
ER  - 
TY  - JOUR
A1  - Sonnenschein-van der Voort, Agnes M. M.
A1  - Arends, Lidia R.
A1  - de Jongste, Johan C.
A1  - Annesi-Maesano, Isabella
A1  - Arshad, S. Hasan
A1  - Barros, Henrique
A1  - Basterrechea, Mikel
A1  - Bisgaard, Hans
A1  - Chatzi, Leda
A1  - Corpeleijn, Eva
A1  - Correia, Sofia
A1  - Craig, Leone C.
A1  - Devereux, Graham
A1  - Dogaru, Cristian
A1  - Dostal, Miroslav
A1  - Duchen, Karel
A1  - Eggesbø, Merete
A1  - van der Ent, C. Kors
A1  - Fantini, Maria P.
A1  - Forastiere, Francesco
A1  - Frey, Urs
A1  - Gehring, Ulrike
A1  - Gori, Davide
A1  - van der Gugten, Anne C.
A1  - Hanke, Wojciech
A1  - Henderson, A. John
A1  - Heude, Barbara
A1  - Iñiguez, Carmen
A1  - Inskip, Hazel M.
A1  - Keil, Thomas
A1  - Kelleher, Cecily C.
A1  - Kogevinas, Manolis
A1  - Kreiner-Møller, Eskil
A1  - Kuehni, Claudia E.
A1  - Küpers, Leanne K.
A1  - Lancz, Kinga
A1  - Larsen, Pernille S.
A1  - Lau, Susanne
A1  - Ludvigsson, Johnny
A1  - Mommers, Monique
A1  - Andersen, Anne-Marie Nybo
A1  - Palkovicova, Lubica
A1  - Pike, Katherine C.
A1  - Pizzi, Constanza
A1  - Polanska, Kinga
A1  - Porta, Daniela
A1  - Richiardi, Lorenzo
A1  - Roberts, Graham
A1  - Schmidt, Anne
A1  - Sram, Radim J.
A1  - Sunyer, Jordi
A1  - Thijs, Carel
A1  - Torrent, Maties
A1  - Viljoen, Karien
A1  - Wijga, Alet H.
A1  - Vrijheid, Martine
A1  - Jaddoe, Vincent W. V.
A1  - Duijts, Liesbeth
T1  - Preterm birth, infant weight gain, and childhood asthma risk: A meta-analysis of 147,000 European children
JF  - The Journal of Allergy and Clinical Immunology
N2  - Background
Preterm birth, low birth weight, and infant catch-up growth seem associated with an increased risk of respiratory diseases in later life, but individual studies showed conflicting results.

Objectives
We performed an individual participant data meta-analysis for 147,252 children of 31 birth cohort studies to determine the associations of birth and infant growth characteristics with the risks of preschool wheezing (1-4 years) and school-age asthma (5-10 years).

Methods
First, we performed an adjusted 1-stage random-effect meta-analysis to assess the combined associations of gestational age, birth weight, and infant weight gain with childhood asthma. Second, we performed an adjusted 2-stage random-effect meta-analysis to assess the associations of preterm birth (gestational age <37 weeks) and low birth weight (<2500 g) with childhood asthma outcomes.

Results
Younger gestational age at birth and higher infant weight gain were independently associated with higher risks of preschool wheezing and school-age asthma (P < .05). The inverse associations of birth weight with childhood asthma were explained by gestational age at birth. Compared with term-born children with normal infant weight gain, we observed the highest risks of school-age asthma in children born preterm with high infant weight gain (odds ratio [OR], 4.47; 95% CI, 2.58-7.76). Preterm birth was positively associated with an increased risk of preschool wheezing (pooled odds ratio [pOR], 1.34; 95% CI, 1.25-1.43) and school-age asthma (pOR, 1.40; 95% CI, 1.18-1.67) independent of birth weight. Weaker effect estimates were observed for the associations of low birth weight adjusted for gestational age at birth with preschool wheezing (pOR, 1.10; 95% CI, 1.00-1.21) and school-age asthma (pOR, 1.13; 95% CI, 1.01-1.27).

Conclusion
Younger gestational age at birth and higher infant weight gain were associated with childhood asthma outcomes. The associations of lower birth weight with childhood asthma were largely explained by gestational age at birth."
KW  - gestational age
KW  - low birth weight
KW  - infant growth
KW  - wheezing
KW  - asthma
KW  - epidemiology
KW  - cohort studies
KW  - children
Y1  - 2014
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-120714
VL  - 133
IS  - 5
ER  - 
TY  - JOUR
A1  - Hohenauer, Tobias
A1  - Berking, Carola
A1  - Schmidt, Andreas
A1  - Haferkamp, Sebastian
A1  - Senft, Daniela
A1  - Kammerbauer, Claudia
A1  - Fraschka, Sabine
A1  - Graf, Saskia Anna
A1  - Irmler, Martin
A1  - Beckers, Johannes
A1  - Flaig, Michael
A1  - Aigner, Achim
A1  - Höbel, Sabrina
A1  - Hoffmann, Franziska
A1  - Hermeking, Heiko
A1  - Rothenfusser, Simon
A1  - Endres, Stefan
A1  - Ruzicka, Thomas
A1  - Besch, Robert
T1  - The neural crest transcription factor Brn3a is expressed in melanoma and required for cell cycle progression and survival
JF  - EMBO Molecular Medicine
N2  - Pigment cells and neuronal cells both are derived from the neural crest. Here, we describe the Pit-Oct-Unc (POU) domain transcription factor Brn3a, normally involved in neuronal development, to be frequently expressed in melanoma, but not in melanocytes and nevi. RNAi-mediated silencing of Brn3a strongly reduced the viability of melanoma cell lines and decreased tumour growth in vivo. In melanoma cell lines, inhibition of Brn3a caused DNA double-strand breaks as evidenced by Mre11/Rad50-containing nuclear foci. Activated DNA damage signalling caused stabilization of the tumour suppressor p53, which resulted in cell cycle arrest and apoptosis. When Brn3a was ectopically expressed in primary melanocytes and fibroblasts, anchorage-independent growth was increased. In tumourigenic melanocytes and fibroblasts, Brn3a accelerated tumour growth in vivo. Furthermore, Brn3a cooperated with proliferation pathways such as oncogenic BRAF, by reducing oncogene-induced senescence in non-malignant melanocytes. Together, these results identify Brn3a as a new factor in melanoma that is essential for melanoma cell survival and that promotes melanocytic transformation and tumourigenesis.
KW  - oncogene-induced senescence
KW  - BRN-3A
KW  - DNA
KW  - DNA damage
KW  - tumourigenesis
KW  - P53
KW  - in-vitro
KW  - neural crest factors
KW  - family
KW  - apoptosis
KW  - melanoma
KW  - BRAF mutations
KW  - domain
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-122193
SN  - 1757-4676
VL  - 5
ER  - 
TY  - JOUR
A1  - Schoffer, Olaf
A1  - Schülein, Stefanie
A1  - Arand, Gerlinde
A1  - Arnholdt, Hans
A1  - Baaske, Dieter
A1  - Bargou, Ralf C.
A1  - Becker, Nikolaus
A1  - Beckmann, Matthias W.
A1  - Bodack, Yves
A1  - Böhme, Beatrix
A1  - Bozkurt, Tayfun
A1  - Breitsprecher, Regine
A1  - Buchali, Andre
A1  - Burger, Elke
A1  - Burger, Ulrike
A1  - Dommisch, Klaus
A1  - Elsner, Gudrun
A1  - Fernschild, Karin
A1  - Flintzer, Ulrike
A1  - Funke, Uwe
A1  - Gerken, Michael
A1  - Göbel, Hubert
A1  - Grobe, Norbert
A1  - Gumpp, Vera
A1  - Heinzerling, Lucie
A1  - Kempfer, Lana Raffaela
A1  - Kiani, Alexander
A1  - Klinkhammer-Schalke, Monika
A1  - Klöcking, Sabine
A1  - Kreibich, Ute
A1  - Knabner, Katrin
A1  - Kuhn, Peter
A1  - Lutze, Stine
A1  - Mäder, Uwe
A1  - Maisel, Tanja
A1  - Maschke, Jan
A1  - Middeke, Martin
A1  - Neubauer, Andreas
A1  - Niedostatek, Antje
A1  - Opazo-Saez, Anabelle
A1  - Peters, Christoph
A1  - Schell, Beatrice
A1  - Schenkirsch, Gerhard
A1  - Schmalenberg, Harald
A1  - Schmidt, Peter
A1  - Schneider, Constanze
A1  - Schubotz, Birgit
A1  - Seide, Anika
A1  - Strecker, Paul
A1  - Taubenheim, Sabine
A1  - Wackes, Matthias
A1  - Weiß, Steffen
A1  - Welke, Claudia
A1  - Werner, Carmen
A1  - Wittekind, Christian
A1  - Wulff, Jörg
A1  - Zettl, Heike
A1  - Klug, Stefanie J.
T1  - Tumour stage distribution and survival of malignant melanoma in Germany 2002-2011
JF  - BMC Cancer
N2  - Background

Over the past two decades, there has been a rising trend in malignant melanoma incidence worldwide. In 2008, Germany introduced a nationwide skin cancer screening program starting at age 35. The aims of this study were to analyse the distribution of malignant melanoma tumour stages over time, as well as demographic and regional differences in stage distribution and survival of melanoma patients.

Methods

Pooled data from 61 895 malignant melanoma patients diagnosed between 2002 and 2011 and documented in 28 German population-based and hospital-based clinical cancer registries were analysed using descriptive methods, joinpoint regression, logistic regression and relative survival.

Results

The number of annually documented cases increased by 53.2% between 2002 (N = 4 779) and 2011 (N = 7 320). There was a statistically significant continuous positive trend in the proportion of stage UICC I cases diagnosed between 2002 and 2011, compared to a negative trend for stage UICC II. No trends were found for stages UICC III and IV respectively. Age (OR 0.97, 95% CI 0.97–0.97), sex (OR 1.18, 95% CI 1.11–1.25), date of diagnosis (OR 1.05, 95% CI 1.04–1.06), ‘diagnosis during screening’ (OR 3.24, 95% CI 2.50–4.19) and place of residence (OR 1.23, 95% CI 1.16–1.30) had a statistically significant influence on the tumour stage at diagnosis. The overall 5-year relative survival for invasive cases was 83.4% (95% CI 82.8–83.9%).

Conclusions

No distinct changes in the distribution of malignant melanoma tumour stages among those aged 35 and older were seen that could be directly attributed to the introduction of skin cancer screening in 2008.
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KW  - Malignant melanoma
KW  - TNM staging
KW  - Survival analysis
KW  - Skin cancer screening
KW  - Stage distribution
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-164544
VL  - 16
IS  - 936
ER  - 
TY  - JOUR
A1  - Stengel, Helena
A1  - Vural, Atay
A1  - Brunder, Anna-Michelle
A1  - Heinius, Annika
A1  - Appeltshauser, Luise
A1  - Fiebig, Bianca
A1  - Giese, Florian
A1  - Dresel, Christian
A1  - Papagianni, Aikaterini
A1  - Birklein, Frank
A1  - Weis, Joachim
A1  - Huchtemann, Tessa
A1  - Schmidt, Christian
A1  - Körtvelyessy, Peter
A1  - Villmann, Carmen
A1  - Meinl, Edgar
A1  - Sommer, Claudia
A1  - Leypoldt, Frank
A1  - Doppler, Kathrin
T1  - Anti–pan-neurofascin IgG3 as a marker of fulminant autoimmune neuropathy
JF  - Neurology: Neuroimmunology & Neuroinflammation
N2  - Objective 
To identify and characterize patients with autoantibodies against different neurofascin (NF) isoforms.

Methods 
Screening of a large cohort of patient sera for anti-NF autoantibodies by ELISA and further characterization by cell-based assays, epitope mapping, and complement binding assays.

Results 
Two different clinical phenotypes became apparent in this study: The well-known clinical picture of subacute-onset severe sensorimotor neuropathy with tremor that is known to be associated with IgG4 autoantibodies against the paranodal isoform NF-155 was found in 2 patients. The second phenotype with a dramatic course of disease with tetraplegia and almost locked-in syndrome was associated with IgG3 autoantibodies against nodal and paranodal isoforms of NF in 3 patients. The epitope against which these autoantibodies were directed in this second phenotype was the common Ig domain found in all 3 NF isoforms. In contrast, anti–NF-155 IgG4 were directed against the NF-155–specific Fn3Fn4 domain. The description of a second phenotype of anti–NF-associated neuropathy is in line with some case reports of similar patients that were published in the last year.

Conclusions 
Our results indicate that anti–pan-NF-associated neuropathy differs from anti–NF-155-associated neuropathy, and epitope and subclass play a major role in the pathogenesis and severity of anti–NF-associated neuropathy and should be determined to correctly classify patients, also in respect to possible differences in therapeutic response.
KW  - neurology
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-202462
VL  - 6
IS  - 5
ER  - 
TY  - JOUR
A1  - Bumiller-Bini Hoch, Valéria
A1  - Kohler, Ana Flávia
A1  - Augusto, Danillo G.
A1  - Lobo-Alves, Sara Cristina
A1  - Malheiros, Danielle
A1  - Cipolla, Gabriel Adelman
A1  - Winter Boldt, Angelica Beate
A1  - Braun-Prado, Karin
A1  - Wittig, Michael
A1  - Franke, Andre
A1  - Pföhler, Claudia
A1  - Worm, Margitta
A1  - van Beek, Nina
A1  - Goebeler, Matthias
A1  - Sárdy, Miklós
A1  - Ibrahim, Saleh
A1  - Busch, Hauke
A1  - Schmidt, Enno
A1  - Hundt, Jennifer Elisabeth
A1  - Araujo-Souza, Patrícia Savio de
A1  - Petzl-Erler, Maria Luiza
T1  - Genetic associations and differential mRNA expression levels of host genes suggest a viral trigger for endemic pemphigus foliaceus
JF  - Viruses
N2  - The long search for the environmental trigger of the endemic pemphigus foliaceus (EPF, fogo selvagem) has not yet resulted in any tangible findings. Here, we searched for genetic associations and the differential expression of host genes involved in early viral infections and innate antiviral defense. Genetic variants could alter the structure, expression sites, or levels of the gene products, impacting their functions. By analyzing 3063 variants of 166 candidate genes in 227 EPF patients and 194 controls, we found 12 variants within 11 genes associated with differential susceptibility (p < 0.005) to EPF. The products of genes TRIM5, TPCN2, EIF4E, EIF4E3, NUP37, NUP50, NUP88, TPR, USP15, IRF8, and JAK1 are involved in different mechanisms of viral control, for example, the regulation of viral entry into the host cell or recognition of viral nucleic acids and proteins. Only two of nine variants were also associated in an independent German cohort of sporadic PF (75 patients, 150 controls), aligning with our hypothesis that antiviral host genes play a major role in EPF due to a specific virus–human interaction in the endemic region. Moreover, CCL5, P4HB, and APOBEC3G mRNA levels were increased (p < 0.001) in CD4+ T lymphocytes of EPF patients. Because there is limited or no evidence that these genes are involved in autoimmunity, their crucial role in antiviral responses and the associations that we observed support the hypothesis of a viral trigger for EPF, presumably a still unnoticed flavivirus. This work opens new frontiers in searching for the trigger of EPF, with the potential to advance translational research that aims for disease prevention and treatment.
KW  - endemic pemphigus foliaceus
KW  - virus
KW  - genetic association
KW  - differential gene expression
KW  - autoimmune disease
KW  - environmental factors
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-270572
SN  - 1999-4915
VL  - 14
IS  - 5
ER  - 
TY  - JOUR
A1  - Buhl, Timo
A1  - Beissert, Stefan
A1  - Gaffal, Evelyn
A1  - Goebeler, Matthias
A1  - Hertl, Michael
A1  - Mauch, Cornelia
A1  - Reich, Kristian
A1  - Schmidt, Enno
A1  - Schön, Michael P.
A1  - Sticherling, Michael
A1  - Sunderkötter, Cord
A1  - Traidl‐Hoffmann, Claudia
A1  - Werfel, Thomas
A1  - Wilsman‐Theis, Dagmar
A1  - Worm, Margitta
T1  - COVID‐19 and implications for dermatological and allergological diseases
JF  - JDDG: Journal der Deutschen Dermatologischen Gesellschaft
N2  - COVID‐19, caused by the coronavirus SARS‐CoV‐2, has become pandemic. A further level of complexity opens up as soon as we look at diseases whose pathogenesis and therapy involve different immunological signaling pathways, which are potentially affected by COVID‐19. Medical treatments must often be reassessed and questioned in connection with this infection.
This article summarizes the current knowledge of COVID‐19 in the light of major dermatological and allergological diseases. It identifies medical areas lacking sufficient data and draws conclusions for the management of our patients during the pandemic. We focus on common chronic inflammatory skin diseases with complex immunological pathogenesis: psoriasis, eczema including atopic dermatitis, type I allergies, autoimmune blistering and inflammatory connective tissue diseases, vasculitis, and skin cancers. Since several other inflammatory skin diseases display related or comparable immunological reactions, clustering of the various inflammatory dermatoses into different disease patterns may help with therapeutic decisions. Thus, following these patterns of skin inflammation, our review may supply treatment recommendations and thoughtful considerations for disease management even beyond the most frequent diseases discussed here.
KW  - COVID-19
KW  - dermatology
KW  - allergies
Y1  - 2020
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-217860
VL  - 18
IS  - 8
SP  - 815
EP  - 824
ER  -