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Molecular and Cellular Pediatrics

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https://www.readbyqxmd.com/read/28508352/pediatric-precursor-b-acute-lymphoblastic-leukemia-are-t-helper-cells-the-missing-link-in-the-infectious-etiology-theory
#1
REVIEW
Simone Bürgler, David Nadal
Precursor B acute lymphoblastic leukemia (BCP-ALL), the most common childhood malignancy, arises from an expansion of malignant B cell precursors in the bone marrow. Epidemiological studies suggest that infections or immune responses to infections may promote such an expansion and thus BCP-ALL development. Nevertheless, a specific pathogen responsible for this process has not been identified. BCP-ALL cells critically depend on interactions with the bone marrow microenvironment. The bone marrow is also home to memory T helper (Th) cells that have previously expanded during an immune response in the periphery...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28432664/human-perinatal-immunity-in-physiological-conditions-and-during-infection
#2
REVIEW
Gijs T J van Well, Leonie A Daalderop, Tim Wolfs, Boris W Kramer
The intrauterine environment was long considered sterile. However, several infectious threats are already present during fetal life. This review focuses on the postnatal immunological consequences of prenatal exposure to microorganisms and related inflammatory stimuli. Both the innate and adaptive immune systems of the fetus and neonate are immature, which makes them highly susceptible to infections. There is good evidence that prenatal infections are a primary cause of preterm births. Additionally, the association between antenatal inflammation and adverse neonatal outcomes has been well established...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28150141/role-of-second-hand-smoke-shs-induced-proteostasis-autophagy-impairment-in-pediatric-lung-diseases
#3
Neel Patel, Christopher D Trumph, Manish Bodas, Neeraj Vij
BACKGROUND: Exposure to second-hand tobacco smoke (SHS) is one of the prime risk factors for chronic lung disease development. Smoking during pregnancy may lead to birth defects in the newborn that include pulmonary dysfunction, increased susceptibility to opportunistic pathogens, or initiation of childhood respiratory manifestations such as bronchopulmonary dysplasia (BPD). Moreover, exposure to SHS in early childhood can have negative impact on lung health, although the exact mechanisms are unclear...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28101838/renal-consequences-of-preterm-birth
#4
REVIEW
Amelie Stritzke, Sumesh Thomas, Harish Amin, Christoph Fusch, Abhay Lodha
BACKGROUND: The developmental origin of health and disease concept identifies the brain, cardiovascular, liver, and kidney systems as targets of fetal adverse programming with adult consequences. As the limits of viability in premature infants have been pushed to lower gestational ages, the long-term impact of prematurity on kidneys still remains a significant burden during hospital stay and beyond. OBJECTIVES: The purpose of this study is to summarize available evidence, mechanisms, and short- and long-term renal consequences of prematurity and identify nephroprotective strategies and areas of uncertainty...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28097632/acquisition-and-adaptation-of-the-airway-microbiota-in-the-early-life-of-cystic-fibrosis-patients
#5
REVIEW
Sébastien Boutin, Alexander H Dalpke
Cystic fibrosis (CF) is a genetic disease in which bacterial infections of the airways play a major role in the long-term clinical outcome. In recent years, a number of next-generation sequencing (NGS)-based studies aimed at deciphering the structure and composition of the airways' microbiota. It was shown that the nasal cavity of CF patients displays dysbiosis early in life indicating a failure in the first establishment of a healthy microbiota. In contrast, within the conducting and lower airways, the establishment occurs normally first, but is sensitive to future dysbiosis including chronic infections with classical pathogens in later life...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28516419/abstracts-of-the-52nd-workshop-for-pediatric-research-frankfurt-germany-27-28-october-2016
#6
Rhea van den Bruck, Patrick P Weil, Thomas Ziegenhals, Philipp Schreiner, Stefan Juranek, Daniel Gödde, Silvia Vogel, Frauke Schuster, Valerie Orth, Johannes Dörner, Daniel Pembaur, Meike Röper, Stefan Störkel, Hubert Zirngibl, Stefan Wirth, Andreas C W Jenke, Jan Postberg, Nikolas Boy, Jana Heringer, Gisela Haege, Esther M Glahn, Georg F Hoffmann, Sven F Garbade, Peter Burgard, Stefan Kölker, Cho-Ming Chao, Faady Yahya, Alena Moiseenko, Amit Shrestha, Negah Ahmadvand, Jennifer Quantius, Jochen Wilhelm, Elie El-Agha, Klaus-Peter Zimmer, Saverio Bellusci, Christian Staufner, Stefan Kölker, Holger Prokisch, Georg F Hoffmann, Stephan Seeliger, Matthias Müller, Andreas Hippe, Henrik Steinkraus, Roland Wauer, Burkhard Lachmann, Sigrun R Hofmann, Christian M Hedrich, Jakob Zierk, Farhad Arzideh, Rainer Haeckel, Wolfgang Rascher, Manfred Rauh, Markus Metzler, Sebastian Thieme, Joanna Bandoła, Cornelia Richter, Martin Ryser, Arshad Jamal, Michelle P Ashton, Malte von Bonin, Matthias Kuhn, Christian M Hedrich, Ezio Bonifacio, Reinhard Berner, Sebastian Brenner, Johanna Hammersen, Cristina Has, Nora Naumann-Bartsch, Daniel Stachel, Dimitra Kiritsi, Stephan Söder, Mathilde Tardieu, Markus Metzler, Leena Bruckner-Tuderman, Holm Schneider, F Bohne, D Langer, R Cencic, T Eggermann, U Zechner, J Pelletier, F Zepp, T Enklaar, D Prawitt, Martin Pech, Markus Weckmann, Femke-Anouska Heinsen, Andre Franke, Christine Happle, Anna-Maria Dittrich, Gesine Hansen, Oliver Fuchs, Erika von Mutius, Brian G Oliver, Matthias V Kopp, Claudia Paret, Alexandra Russo, Johanna Theruvath, Bettina Keller, Khalifa El Malki, Nadine Lehmann, Arthur Wingerter, Marie A Neu, Gerhold-Ay Aslihan, Wolfgang Wagner, Clemens Sommer, Torsten Pietsch, Larissa Seidmann, Jörg Faber, Felix Schreiner, Merle Ackermann, Michael Michalik, Eva Rother, Andras Bilkei-Gorzo, Ildiko Racz, Laura Bindila, Beat Lutz, Jörg Dötsch, Andreas Zimmer, Joachim Woelfle, Hendrik S Fischer, Tim L Ullrich, Christoph Bührer, Christoph Czernik, Gerd Schmalisch, Robert Stein, Sigrun R Hofmann, Judith Hagenbuchner, Ursula Kiechl-Kohlendorfer, Petra Obexer, Michael J Ausserlechner, Niki T Loges, Adrien Tobias Frommer, Julia Wallmeier, Heymut Omran, Soner Öner-Sieben, Martina Gimpfl, Jan Rozman, Martin Irmler, Johannes Beckers, Martin Hrabe De Angelis, Adelbert Roscher, Eckhard Wolf, Regina Ensenauer, Karolina Nemes, Michael Frühwald, Martin Hasselblatt, Reiner Siebert, Uwe Kordes, Marcel Kool, Haicui Wang, Holly Hardy, Osama Refai, Katy E S Barwick, Holly H Zimmerman, Joachim Weis, Emma L Baple, Andrew H Crosby, Sebahattin Cirak, C Hellmuth, O Uhl, M Standl, J Heinrich, E Thiering, B Koletzko, Lena Blümel, Kornelius Kerl, Daniel Picard, Michael C Frühwald, Max C Liebau, Guido Reifenberger, Arndt Borkhardt, Martin Hasselblatt, Marc Remke, D Tews, M Wabitsch, P Fischer-Posovszky, Mike-Andrew Westhoff, Lisa Nonnenmacher, Julia Langhans, Lukas Schneele, Nancy Trenkler, Klaus-Michael Debatin
No abstract text is available yet for this article.
May 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27868161/neutrophil-plasticity-enables-the-development-of-pathological-microenvironments-implications-for-cystic-fibrosis-airway-disease
#7
REVIEW
Camilla Margaroli, Rabindra Tirouvanziam
INTRODUCTION: The pathological course of several chronic inflammatory diseases, including cystic fibrosis, chronic obstructive pulmonary disease, and rheumatoid arthritis, features an aberrant innate immune response dominated by neutrophils. In cystic fibrosis, neutrophil burden and activity of neutrophil elastase in the extracellular fluid have been identified as strong predictors of lung disease severity. REVIEW: Although neutrophils are generally considered to be rigid, pre-programmed effector leukocytes, recent studies suggest extensive plasticity in how neutrophil functions unfold upon recruitment to peripheral tissues, and how they choose their ultimate fate...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27868160/severe-changes-in-colon-epithelium-in-the-mecp2-null-mouse-model-of-rett-syndrome
#8
Pamela Millar-Büchner, Amber R Philp, Noemí Gutierrez, Sandra Villanueva, Bredford Kerr, Carlos A Flores
BACKGROUND: Rett syndrome is best known due to its severe and devastating symptoms in the central nervous system. It is produced by mutations affecting the Mecp2 gene that codes for a transcription factor. Nevertheless, evidence for MECP2 activity has been reported for tissues other than those of the central nervous system. Patients affected by Rett presented with intestinal affections whose origin is still not known. We have observed that the Mecp2-null mice presented with episodes of diarrhea, and decided to study the intestinal phenotype in these mice...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27830498/wilms-tumor-susceptibility-possible-involvement-of-foxp3-and-cxcl12-genes
#9
Patricia Midori Murobushi Ozawa, Carolina Batista Ariza, Roberta Losi-Guembarovski, Alda Losi Guembarovski, Carlos Eduardo Coral de Oliveira, Bruna Karina Banin-Hirata, Marina Okuyama Kishima, Diego Lima Petenuci, Maria Angelica Ehara Watanabe
BACKGROUND: Wilms' tumor is an embryonal neoplasm of the kidney that accounts for approximately 6 % of all childhood tumors. The chemokine CXCL12 (C-X-C chemokine ligand 12) and its ligand CXCR4 (C-X-C chemokine receptor type 4) are involved in the development of several organs, including the kidney, and are also associated with tumor growth and metastasis. FOXP3 (forkhead transcription factor 3) was initially described as a marker for regulatory T cells; however, its expression in several types of tumor cells has already been described and may have prognostic significance...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27778308/leukocyte-recruitment-in-preterm-and-term-infants
#10
Katinka Karenberg, Hannes Hudalla, David Frommhold
Impaired cellular innate immune defense accounts for susceptibility to sepsis and its high morbidity and mortality in preterm infants. Leukocyte recruitment is an integral part of the cellular immune response and follows a well-defined cascade of events from rolling of leukocytes along the endothelium to firm adhesion and finally transmigration which is concerted by a variety of adhesion molecules. Recent analytical advances such as fetal intravital microscopy have granted new insights into ontogenetic regulation and maturation of fetal immune cell recruitment...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27718180/linking-bronchopulmonary-dysplasia-to-adult-chronic-lung-diseases-role-of-wnt-signaling
#11
Chiharu Ota, Hoeke A Baarsma, Darcy E Wagner, Anne Hilgendorff, Melanie Königshoff
Bronchopulmonary dysplasia (BPD) is one of the most common chronic lung diseases in infants caused by pre- and/or postnatal lung injury. BPD is characterized by arrested alveolarization and vascularization due to extracellular matrix remodeling, inflammation, and impaired growth factor signaling. WNT signaling is a critical pathway for normal lung development, and its altered signaling has been shown to be involved in the onset and progression of incurable chronic lung diseases in adulthood, such as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF)...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27590627/the-experience-of-canakinumab-in-renal-amyloidosis-secondary-to-familial-mediterranean-fever
#12
Betul Sozeri, Nesrin Gulez, Malik Ergin, Erkin Serdaroglu
INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION: Herein, we reported our experience with clinical-laboratory features and treatment responses of a pediatric FMF patient with amyloidosis treated with canakinumab...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27518572/molecular-biology-of-the-gut
#13
EDITORIAL
Hassan Y Naim, Klaus-Peter Zimmer, Buford Nichols
No abstract text is available yet for this article.
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27510897/early-origins-of-asthma-and-allergy
#14
REVIEW
Michael Kabesch
Asthma is the most common chronic disease starting in childhood and persisting into adulthood in many cases. During childhood, different forms of asthma and wheezing disorders exist that can be discriminated by the mechanisms they are caused by. Specific genetic constellations and exposure against environmental factors during early childhood and in utero play a decisive role in the early development of the disease. Epigenetic mechanisms which are master regulators of gene transcription and thus govern the accessibility and use of genome information, have recently been identified as a "third power" determining many features in the early development of asthma and allergy...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27510896/transient-spontaneous-remission-in-congenital-mll-af10-rearranged-acute-myeloid-leukemia-presenting-with-cardiorespiratory-failure-and-meconium-ileus
#15
Tobias Gyárfás, Juergen Wintgens, Wolfgang Biskup, Ilske Oschlies, Wolfram Klapper, Reiner Siebert, Susanne Bens, Claudia Haferlach, Roland Meisel, Michaela Kuhlen, Arndt Borkhardt
BACKGROUND: Neonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of symptoms including hyperleucocytosis, cytopenia, hepatosplenomegaly, and skin infiltrates. Chromosomal rearrangements including mixed lineage leukemia (MLL) translocations are common in neonatal AML. CASE PRESENTATION: A female neonate born at 34 weeks gestation presented with cardiorespiratory failure, hepatosplenomegaly, pancytopenia, and coagulopathy...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27480877/lung-epithelial-gm-csf-improves-host-defense-function-and-epithelial-repair-in-influenza-virus-pneumonia-a-new-therapeutic-strategy
#16
REVIEW
Barbara Rösler, Susanne Herold
Influenza viruses (IVs) circulate seasonally and are a common cause of respiratory infections in pediatric and adult patients. Additionally, recurrent pandemics cause massive morbidity and mortality worldwide. Infection may result in rapid progressive viral pneumonia with fatal outcome. Since accurate treatment strategies are still missing, research refocuses attention to lung pathology and cellular crosstalk to develop new therapeutic options.Alveolar epithelial cells (AECs) play an important role in orchestrating the pulmonary antiviral host response...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27480876/vitamin-a-retinoid-signaling-in-pulmonary-development-and-disease
#17
REVIEW
Hector A Marquez, Wellington V Cardoso
Retinoic acid (RA), the active form of vitamin A, regulates key developmental processes in multiple organs. In the developing lung, RA is crucial for normal growth and differentiation of airways. Disruption in RA signaling or vitamin A deficiency (VAD) has been linked to aberrant development of the lung including alterations in the airway smooth muscle (SM) differentiation, development, and function. These alterations have been linked to disease states including asthma in both human and animal models.
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27468754/the-puzzle-of-immune-phenotypes-of-childhood-asthma
#18
REVIEW
Katja Landgraf-Rauf, Bettina Anselm, Bianca Schaub
Asthma represents the most common chronic childhood disease worldwide. Whereas preschool children present with wheezing triggered by different factors (multitrigger and viral wheeze), clinical asthma manifestation in school children has previously been classified as allergic and non-allergic asthma. For both, the underlying immunological mechanisms are not yet understood in depth in children. Treatment is still prescribed regardless of underlying mechanisms, and children are not always treated successfully...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27465412/the-need-for-coordination-of-research-activities-in-pediatric-lung-diseases
#19
EDITORIAL
Harald Ehrhardt, Klaus-Peter Zimmer
No abstract text is available yet for this article.
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27456476/neutrophil-elastase-and-matrix-metalloproteinase-12-in-cystic-fibrosis-lung-disease
#20
REVIEW
Claudius J Wagner, Carsten Schultz, Marcus A Mall
Chronic lung disease remains the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Recent studies in young children with CF diagnosed by newborn screening identified neutrophil elastase (NE), a major product released from neutrophils in inflamed airways, as a key risk factor for the onset and early progression of CF lung disease. However, the understanding of how NE and potentially other proteases contribute to the complex in vivo pathogenesis of CF lung disease remains limited. In this review, we summarize recent progress in this area based on studies in βENaC-overexpressing (βENaC-Tg) mice featuring CF-like lung disease and novel protease-specific Förster resonance energy transfer (FRET) sensors for localization and quantification of protease activity in the lung...
December 2016: Molecular and Cellular Pediatrics
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