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Molecular and Cellular Pediatrics

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https://www.readbyqxmd.com/read/27868161/neutrophil-plasticity-enables-the-development-of-pathological-microenvironments-implications-for-cystic-fibrosis-airway-disease
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/27406259/early-injury-of-the-neonatal-lung-contributes-to-premature-lung-aging-a-hypothesis
#15
REVIEW
Silke Meiners, Anne Hilgendorff
Chronic lung disease of the newborn, also known as bronchopulmonary dysplasia (BPD), is the most common chronic lung disease in early infancy and results in an increased risk for long-lasting pulmonary impairment in the adult. BPD develops upon injury of the immature lung by oxygen toxicity, mechanical ventilation, and infections which trigger sustained inflammatory immune responses and extensive remodeling of the extracellular matrix together with dysregulated growth factor signaling. Histopathologically, BPD is characterized by impaired alveolarization, disrupted vascular development, and saccular wall fibrosis...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27357257/pathogenesis-of-bronchopulmonary-dysplasia-when-inflammation-meets-organ-development
#16
REVIEW
Tayyab Shahzad, Sarah Radajewski, Cho-Ming Chao, Saverio Bellusci, Harald Ehrhardt
Bronchopulmonary dysplasia is a chronic lung disease of preterm infants. It is caused by the disturbance of physiologic lung development mainly in the saccular stage with lifelong restrictions of pulmonary function and an increased risk of abnormal somatic and psychomotor development. The contributors to this disease's entity are multifactorial with pre- and postnatal origin. Central to the pathogenesis of bronchopulmonary is the induction of a massive pulmonary inflammatory response due to mechanical ventilation and oxygen toxicity...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27317552/mendeliome-sequencing-enables-differential-diagnosis-and-treatment-of-neonatal-lactic-acidosis
#17
Walid Fazeli, Mert Karakaya, Peter Herkenrath, Anne Vierzig, Jörg Dötsch, Jürgen-Christoph von Kleist-Retzow, Sebahattin Cirak
BACKGROUND: Neonatal lactic acidosis can be associated to severe inborn errors of metabolism. Rapid identification of the underlying disorder may improve the clinical management through reliable counseling of the parents and adaptation of the treatment. METHODS: We present the case of a term newborn with persistent hypoglycemia on postnatal day 1, who developed severe lactic acidosis, aggravating under intravenous glucose administration. Routine metabolic investigations revealed elevated pyruvate and lactate levels in urine, and magnetic resonance spectroscopy showed a lactic acid peak and decreased N-acetylaspartate levels...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27251607/mechanism-of-human-rhinovirus-infections
#18
REVIEW
Dieter Blaas, Renate Fuchs
About 150 human rhinovirus serotypes are responsible for more than 50 % of recurrent upper respiratory infections. Despite having similar 3D structures, some bind members of the low-density lipoprotein receptor family, some ICAM-1, and some use CDHR3 for host cell infection. This is also reflected in the pathways exploited for cellular entry. We found that even rhinovirus serotypes binding the same receptor can travel along different endocytic pathways and release their RNA genome into the cytosol at different locations...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27251606/the-cross-talk-between-enterocytes-and-intraepithelial-lymphocytes
#19
REVIEW
Serena Vitale, Stefania Picascia, Carmen Gianfrani
The gut mucosa is continuously exposed to food and microbial antigens. Both enterocytes and intraepithelial lymphocytes have a pivotal role in maintaining the integrity of intestinal mucosa, as these cells guarantee a first line of defense against pathogens and toxic molecules. Enterocytes maintain a physical barrier against microbes and directly contribute to the gut homeostasis by sampling the luminal agents through several pattern recognition receptors or presenting antigen to mucosa T cells. Similarly, due to a close physical contact with the intestinal epithelial cells, the intraepithelial lymphocytes represent an important part of the gut lymphoid tissue, contrasting the entry and spread of pathogens...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27216745/microrna-in-late-lung-development-and-bronchopulmonary-dysplasia-the-need-to-demonstrate-causality
#20
REVIEW
Claudio Nardiello, Rory E Morty
MicroRNA are emerging as powerful regulators of cell differentiation and tissue and organ development. Several microRNA have been described to play a role in branching morphogenesis, a key step in early lung development. However, considerably less attention has been paid to microRNA as regulators of the process of secondary septation, which drives lung alveolarization during late lung development. Secondary septation is severely perturbed in bronchopulmonary dysplasia (BPD), a common complication of preterm birth characterized by blunted alveolarization...
December 2016: Molecular and Cellular Pediatrics
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