Articles

Post stroke hemi-dystonia in children: a neglected area of research

Childhood arterial ischemic stroke (CAIS) is increasingly recognized as an important cause of significant long-term morbidity in the pediatric population. Post stroke movement disorders, above all hemi-dystoni...

Authors: Daniel Tibussek, Ertan Mayatepek, Dirk Klee and Anne Koy

Airway reactivity and sphingolipids—implications for childhood asthma

Asthma is a clinically heterogeneous disorder, whose onset and progression results from a complex interplay between genetic susceptibility, allergens, and viral triggers. Sphingolipids and altered sphingolipid...

Authors: Jennie G. Ono, Tilla S. Worgall and Stefan Worgall

GM-CSF and the role of myeloid regulatory cells in the pathogenesis and treatment of Crohn’s disease

Intestinal monocytes/macrophages sustain the intestinal immune homeostasis and might be an attractive therapeutic target for the management of inflammatory bowel disease (IBD). Granulocyte macrophage colony-s...

Authors: Jan Däbritz

Modified mRNA as a new therapeutic option for pediatric respiratory diseases and hemoglobinopathies

The immunogenicity and limited stability of conventional messenger RNA (mRNA) has traditionally restricted its potential therapeutic use. In 1992, the first clinical application of mRNA was reported as a poten...

Authors: Justin S. Antony, Alexander Dewerth, Ashiqul Haque, Rupert Handgretinger and Michael S.D. Kormann

Stent implantation into the tracheo-bronchial system in rabbits: histopathologic sequelae in bare metal vs. drug-eluting stents

Stent implantation into the tracheo-bronchial system may be life-saving in selected pediatric patients with otherwise intractable stenosis of the upper airways. Following implantation, significant tissue proli...

Authors: Matthias Sigler, Julia Klötzer, Thomas Quentin, Thomas Paul and Oliver Möller

Lessons learned from metabolomics in cystic fibrosis

Cystic fibrosis is a mono-genetic multi-system disease; however, respiratory manifestations cause the main morbidity and mortality where chronic bacterial infections lead to bronchiectasis and ultimately respi...

Authors: Marianne S. Muhlebach and Wei Sha

Pursuing novel molecular mechanisms and treatment strategies to enhance lung growth in neonatal chronic lung disease (CLD)

Authors: Miguel A Alejandre Alcazar, Mark Kaschwich, Stefanie Preuss, Robert Ertsey, Sana Mujahid, Eva Rother, Katharina Dinger, Jörg Dötsch, Marlene Rabinovitch and Richard Bland

Cilia-associated cellular function of the AAA ATPases RUVBL1 and RUVBL2

Authors: Claudia Dafinger, Markus Rinschen, Carolin Ehrenberg, Mareike Franke, Heike Göbel, Jörg Dötsch, Thomas Benzing, Bernhard Schermer and Max Christoph Liebau

Simultaneously double infection of oncolytic viruses leads to addition of cell death induction in glioblastoma cell lines

Authors: Michael Ehrhardt, Julia Frank, Sigrun Smola and Norbert Graf

A translational approach to dystroglycanopathies: a frequent type of muscular dystrophy

Authors: Sebahattin Cirak, Haicui Wang, Raul Heredia, Francesco Muntoni, Yetrib Hathout, Peter Herkenrath, Jörg Dötsch and Peter Nürnberg

Fibrocystin in MDCK cells: impact on cell adhesion and epithelial morphogenesis

Authors: Birga Sötje, Dieter Haffner and Wolfgang H Ziegler

TMEM18 is a regulator of adipogenesis and involved in PPARG signalling in vivo

Authors: Kathrin Landgraf, Julian Schwartze, Denise Rockstroh, Wieland Kiess and Antje Körner

Identification of a putative new therapeutical target for Hodgkin's lymphoma

Authors: Stefanie Kewitz, Ines Volkmer and Martin S Staege

Disturbed B-lymphocytes selection in autoimmune lymphoproliferative syndrome

Authors: Aleš Janda, Klaus Schwarz, Mirjam van der Burg, Werner Vach, Hanna Ijspeert, Myriam Ricarda Lorenz, Magdeldin Elgizouli, Kathrin Pieper, Paul Fisch, Joachim Hagel, Raquel Lorenzetti, Maximilian Seidl, Joachim Roesler, Fabian Hauck, Elisabetta Traggiai, Carsten Speckmann…

What we learned from bench to bedside with the neuroblastoma targeting CD171-specific CAR

Authors: A Künkele, AJ Johnson, C Berger, L Finn, J Park and MC Jensen

Neutralization of interleukin-17 produced by gamma delta T cells constrains inflammation in experimental biliary atresia

Authors: Christian Klemann, Arne Schröder, Anika Dreier, Nora Moehn, Stephanie Dippel, Thomas Winterberg, Anne Wilde, Yi Yu, Faikah Gueler, Anne Joerns, Eva Tolosa, Johannes Leonhardt, Jan D Haas, Immo Prinz, Claus Petersen and Joachim F Kuebler

Hypoxia potentiates cytotoxicity of LPS-activated microglial BV2 cells in vitro by synergistic effects on cytokine and nitric oxide secretion

Authors: Susan Jung, Daniel Frey, Florian Brackmann, Mandy Richter-Kraus and Regina Trollmann

Regulation of PACAP receptors in respiratory distress syndrome (RDS) of preterms and newborns

Authors: MJ Müller, A Kislat, A Hippe, A Poppe, S Goldmann, T Paul and S Seeliger

A potential role of PI3K inhibition in radiotherapy of glioblastoma multiforme

Authors: Julia Langhans, Lukas Schneele, Klaus-Michael Debatin and Mike-Andrew Westhoff

Is the implementation of a microbiological surveillance screening beneficial in a neonatal intensive care unit?

Authors: I Schmeh, A Welk, T Schwanz, A Diefenbach, B Jansen and E Mildenberger

Modern chronic heart failure therapy in context of pulmonary banding to avoid heart transplantation

Authors: S Recla, B Steinbrenner, T Logeswaran, L Rüblinger, D Schmidt, J Bauer, C Apitz, J Thul and D Schranz

Mutations in CCNO and MCIDAS lead to a mucociliary clearance disorder due to reduced generation of multiple motile cilia

Authors: J Wallmeier, M Boon, D Al Mutairi, NT Loges, L Ma, C-T Chen, H Olbrich, P Pennekamp, T Menchen, G Dougherty, C Werner, M Jaspers, M Griese, E Horak, C Körner-Rettberg, S Schmitt-Grohé…

Impact of embryonic Fgf10 expression deficiency on embryonic mouse lung development and repair following hyperoxia injury

Authors: CM Chao, D Al Alam, S Schermuly, H Ehrhardt, KP Zimmer and S Bellusci

MYCN transcriptionally represses CD9 to trigger an invasion-metastasis cascade in neuroblastoma

Authors: Johannes Fabian, Desirée Opitz, Kristina Althoff, Marco Lodrini, Kathy Astrahantseff, Barbara Hero, Ruth Volland, Anneleen Beckers, Katleen de Preter, Nitin S Patil, Mohammed L Abba, Theresa M Thole, Jasmin Wünschel, Annette Künkele, Jamie Hu, Leonille Schweizer…

Singleton-Merten Syndrome: a rare autoimmune disorder caused by a specific IFIH1 mutation

Authors: I Buers, Y Nitschke, RC Hennekam, M MacDougall, C Lu, O Mamaeva, GI Rice, H Erlandsen, HG Kehl, H Thiele, P Nürnberg, W Höhne, YJ Crow, A Feigenbaum and F Rutsch

Neuroprotection after infection-sensitized neonatal hypoxic-ischemic brain injury

Authors: Hemmen Sabir, Damjan Osredkar, Elke Maes, Thomas Wood and Marianne Thoresen

Integrated genomics elucidates relative spatial homogeneity of embryonal brain tumors

Authors: Marc Remke, Florence Cavalli, A Sorana Morrissy, Vijay Ramaswamy, David Jones, Roger Packer, Eric Bouffet, Gary Bader, Arndt Borkhardt, Stefan Pfister, Nada Jabado, Marco Marra and Michael D Taylor

Mutations in the NKX2.1 and the PAX8 genes in a boy with thyroid dysgenesis, respiratory and neurological disorders

Authors: Pia Hermanns, Małgorzata Kumorowicz-Czoch and Joachim Pohlenz

Quantitative stress echocardiography in pediatric patients with type-1 diabetes mellitus

Authors: Kai O Hensel, Franziska Grimmer, Markus Roskopf, Andreas Heusch, Andreas Jenke and Stefan Wirth

Nexilin mutations are associated with left ventricular noncompaction cardiomyopathy

Authors: E Pardun, K Wenzel, H-H Kramer, F Berger, B Gerull and S Klaassen

Introduction of formula feeding induces subclinical inflammation and altered chromatin structure in the intestine of preterm pigs

Authors: Rhea Willems, Verena Rybicki, Pingping Jiang, Per Torp Sangild, René Liang Shen, Kai O Hensel, Stefan Wirth, Jan Postberg and Andreas C Jenke

Differential expression of mucosal trefoil factors and mucins in pediatric inflammatory bowel diseases

Authors: Andreas C Jenke, Veronika Boland, Jan Postberg, Silvia Vogel, Daniel Gödde, Matthias Zilbauer, Robert Heuschkel, Stefan Wirth and Kai O Hensel

Myeloid regulatory cells: cross talk of innate and adaptive immunity to maintain the intestinal immune homeostasis in inflammatory bowel disease

Authors: Jan Däbritz, Toni Weinhage, Georg Varga, Timo Wirth, Jan M Ehrchen, Louise M Judd, Trevelyan R Menheniott, Andrew S Giraud and Dirk Foell

Functional genomic screening reveals asparagine dependence as a metabolic vulnerability in sarcoma

Authors: Simone Hettmer, Anna C Schinzel, Daria Tchessalova, Nigel Richards, William C Hahn and Amy J Wagers

A regulatory SNP modifies cystic fibrosis by disrupting NFκB complex binding on FAS

Authors: Chidiebere U Awah, Silke Hedtfeld, Burkhard Tümmler and Frauke Stanke

Novel orphan medicines and abandoned pathways - the US Orphan Drug Act of 1983 and the impact on rare rheumatologic diseases and lysosomal storage disorders

Authors: Markus Ries, Thomas Lutz, Anette Lampert, William Mountford, Konstantin Mechler and Georg F Hoffmann

Ciliopathies - from rare inherited cystic kidney diseases to basic cellular function

Primary cilia are membrane-bound microtubule-based protuberances of the cell membrane projecting to the extracellular environment. While little attention was paid to this subcellular structure over a long time...

Authors: Sandra Habbig and Max Christoph Liebau

Submicroscopic copy-number variations associated with 46,XY disorders of sex development

Mutations in known causative genes and cytogenetically detectable chromosomal rearrangements account for a fraction of cases with 46,XY disorders of sex development (DSD). Recent advances in molecular cytogene...

Authors: Masafumi Kon and Maki Fukami

Metabolic origins of childhood asthma

Childhood obesity and incidence of asthma are increasing globally. The parallel increase of the two suggests that obesity and asthma may be related and that abnormalities in the lipid and/or glucose metabolism...

Authors: Hartmut Grasemann

Characterization of maturation of neuronal voltage-gated sodium channels SCN1A and SCN8A in rat myocardium

Sodium channels predominantly expressed in brain are expressed in myocardial tissue and play an important role in cardiac physiology. Alterations of sodium channels are known to result in neurological disease ...

Authors: Ulrich Krause, Christian Alflen, Michael Steinmetz, Matthias J Müller, Thomas Quentin and Thomas Paul

Propofol administration to the maternal-fetal unit improved fetal EEG and influenced cerebral apoptotic pathway in preterm lambs suffering from severe asphyxia

Term and near-term infants are at high risk of developing brain injury and life-long disability if they have suffered from severe perinatal asphyxia. We hypothesized that propofol administration to the materna...

Authors: Matthias Seehase, Ward Jennekens, Alex Zwanenburg, Peter Andriessen, Jennifer JP Collins, Elke Kuypers, Luc J Zimmermann, Johan SH Vles, Antonio WD Gavilanes and Boris W Kramer

The role of chitin, chitinases, and chitinase-like proteins in pediatric lung diseases

Chitin, after cellulose, the second most abundant biopolymer on earth, is a key component of insects, fungi, and house-dust mites. Lower life forms are endowed with chitinases to defend themselves against chit...

Authors: Ines Mack, Andreas Hector, Marlene Ballbach, Julius Kohlhäufl, Katharina J Fuchs, Alexander Weber, Marcus A Mall and Dominik Hartl

Bronchopulmonary dysplasia - an overview about pathophysiologic concepts

Neonatal chronic lung disease in the preterm infant, i.e. bronchopulmonary dysplasia (BPD) is characterized by impaired pulmonary development with its effects persisting into adulthood. Triggered in the immatu...

Authors: Sophie Niedermaier and Anne Hilgendorff

miRNA-221 is elevated in cystic fibrosis airway epithelial cells and regulates expression of ATF6

MicroRNA (miRNA) and messenger RNA (mRNA) expression differs in cystic fibrosis (CF) versus non-CF bronchial epithelium. Here, the role of miRNA in basal regulation of the transcription factor ATF6 was investi...

Authors: Irene K Oglesby, Raman Agrawal, Marcus A Mall, Noel G McElvaney and Catherine M Greene

Proteostasis in pediatric pulmonary pathology

Protein homeostasis describes the tight supervision of protein synthesis, correct protein maturation and folding, as well as the timely disposal of unwanted and damaged proteins by the ubiquitin-proteasome pat...

Authors: Silke Meiners and Korbinian Ballweg

Mechanisms of hypothermia-induced cell protection in the brain

Therapeutic hypothermia is an effective cytoprotectant and promising intervention shown to improve outcome in patients following cardiac arrest and neonatal hypoxia-ischemia. However, despite our clinical and ...

Authors: Katharina Rose Luise Schmitt, Giang Tong and Felix Berger

Matrix metalloproteinases and epileptogenesis

Matrix metalloproteinases are vital drivers of synaptic remodeling in health and disease. It is suggested that at early stages of epileptogenesis, inhibition of matrix metalloproteinases may help ameliorate ce...

Authors: Chrysanthy Ikonomidou

Fanconi anemia: young patients at high risk for squamous cell carcinoma

Fanconi anemia is one of the best studied inherited cancer-prone diseases. Greatly improved protocols for hematopoietic stem cell transplantation increasingly save the lives of these young patients. However, i...

Authors: Eunike Velleuer and Ralf Dietrich

Cancer therapy: know your enemy?

Most cancer therapies are devised for adult or even elder patients. However, when dealing with pediatric cancers, additional considerations are needed.

Authors: Mike-Andrew Westhoff, Oliver Brühl and Klaus-Michael Debatin

Are microRNAs suitable biomarkers of immunity to tuberculosis?

MicroRNAs (miRNAs) are crucial regulators of human immunity e.g. against Mycobacterium tuberculosis. Against the background of still alarming high mortality of tuberculosis effective biomarkers to improve diagnos...

Authors: Bianca Ueberberg, Malte Kohns, Ertan Mayatepek and Marc Jacobsen