Mesenchymal stromal cells in the development and therapy of bronchopulmonary dysplasia
Abstract
Bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, remains a major healthcare burden. Despite great progresses in perinatal medicine over the past decades, no cure for BPD has been found. The complex pathophysiology of the disease further hampers the development of effective treatment strategies, but recent insights into the biology of mesenchymal stem (MSCs) and progenitor cells in lung development and disease have ignited the hope of preventing or even treating BPD. The promising results of pre-clinical studies have lead to the first early phase clinical trials. Howeve...
Source: Molecular and Cellular Pediatrics - May 2, 2016 Category: Cytology Source Type: research
Alveologenesis: key cellular players and fibroblast growth factor 10 signaling
Conclusions
A better understanding of the cellular and molecular processes underlying the formation of the secondary septae is critical for the development of new therapies to protect or regenerate the alveoli. This review summarizes briefly the alveologenesis process in mouse and human. Further, it discusses the current knowledge on the epithelial and mesenchymal progenitor cells during early lung development giving rise to the key cellular players (e.g., alveolar epithelial cell type I, alveolar epithelial cell type II, alveolar myofibroblast, lipofibroblast) involved in alveologenesis. This review ...
Source: Molecular and Cellular Pediatrics - April 20, 2016 Category: Cytology Source Type: research
Inhaled protein/peptide-based therapies for respiratory disease
Abstract
Asthma, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (CF) are all chronic pulmonary diseases, albeit with different etiologies, that are characterized by airflow limitation, chronic inflammation, and abnormal mucus production/rheology. Small synthetic molecule-based therapies are commonly prescribed for all three diseases. However, there has been increased interest in “biologicals” to treat these diseases. Biologicals typically constitute protein- or peptide-based therapies and are often more potent than small molecule-based drugs. In this review, we shall describe the ...
Source: Molecular and Cellular Pediatrics - April 19, 2016 Category: Cytology Source Type: research
Can the preterm lung recover from perinatal stress?
Abstract
After birth, adequate lung function is necessary for the successful adaptation of a preterm baby. Both prenatal and postnatal insults and therapeutic interventions have an immediate effect on lung function and gas exchange but also interfere with fetal and neonatal lung development. Prenatal insults like chorioamnionitis and prenatal interventions like maternal glucocorticosteroids interact but might also determine the preterm baby’s lung response to postnatal interventions (“second hit”) like supplementation of oxygen and drug therapy. We review current experimental and clinical findings...
Source: Molecular and Cellular Pediatrics - April 12, 2016 Category: Cytology Source Type: research
The impact of hypoxia on intestinal epithelial cell functions: consequences for invasion by bacterial pathogens
Abstract
The maintenance of oxygen homeostasis in human tissues is mediated by several cellular adaptations in response to low-oxygen stress, called hypoxia. A decrease in tissue oxygen levels is initially counteracted by increasing local blood flow to overcome diminished oxygenation and avoid hypoxic stress. However, studies have shown that the physiological oxygen concentrations in several tissues are much lower than atmospheric (normoxic) conditions, and the oxygen supply is finely regulated in individual cell types. The gastrointestinal tract has been described to subsist in a state of physiological...
Source: Molecular and Cellular Pediatrics - March 21, 2016 Category: Cytology Source Type: research
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis
Abstract
Cystic fibrosis (CF) is a lethal monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that entails the (diagnostic) increase in sweat electrolyte concentrations, progressive lung disease with chronic inflammation and recurrent bacterial infections, pancreatic insufficiency, and male infertility. Therapies aimed at restoring the CFTR defect have emerged. Thus, a small molecule which facilitates chloride channel opening, the potentiator Ivacaftor, has been approved for the treatment of CF patients bearing a particular class of rare CFTR muta...
Source: Molecular and Cellular Pediatrics - March 14, 2016 Category: Cytology Source Type: research
Update on host-pathogen interactions in cystic fibrosis lung disease
Abstract
Bacterial and fungal infections are hallmarks of cystic fibrosis (CF) lung disease. In the era of long-term inhaled antibiotics and increasing CF patient survival, new “emerging” pathogens are detected in CF airways, yet their pathophysiological disease relevance remains largely controversial and incompletely defined. As a response to chronic microbial triggers, innate immune cells, particularly neutrophils, are continuously recruited into CF airways where they combat pathogens but also cause tissue injury through release of oxidants and proteases. The coordinated interplay between host imm...
Source: Molecular and Cellular Pediatrics - February 23, 2016 Category: Cytology Source Type: research
Underlying molecular and cellular mechanisms in childhood irritable bowel syndrome
Abstract
Irritable bowel syndrome (IBS) affects a large number of children throughout the world. The symptom expression of IBS is heterogeneous, and several factors which may be interrelated within the IBS biopsychosocial model play a role. These factors include visceral hyperalgesia, intestinal permeability, gut microbiota, psychosocial distress, gut inflammation, bile acids, food intolerance, colonic bacterial fermentation, and genetics. The molecular and cellular mechanisms of these factors are being actively investigated. In this mini-review, we present updates of these mechanisms and, where possibl...
Source: Molecular and Cellular Pediatrics - February 16, 2016 Category: Cytology Source Type: research
Fructose malabsorption
Abstract
Incomplete intestinal absorption of fructose might lead to abdominal complaints such as pain, flatulence and diarrhoea. Whether defect fructose transporters such as GLUT5 or GLUT2 are involved in the pathogenesis of fructose malabsorption is a matter of debate. The hydrogen production by colonic bacteria is used for diagnosis with the hydrogen breath test. However, the appropriate fructose test dose for correct diagnosis is unclear. Subjects with fructose malabsorption show increased breath hydrogen levels and abdominal symptoms after fructose administration but do not report any symptoms when ...
Source: Molecular and Cellular Pediatrics - February 16, 2016 Category: Cytology Source Type: research
Endocytosis and transcytosis of gliadin peptides
Conclusions
Gliadin is not completely digested by the intestinal proteases producing bioactive peptides that have different biological effects. These peptides are internalized in the cells by an active process of endocytosis and can traverse the intestinal mucosa with different kinetics and immunological effects. In vivo findings will also be discussed. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - February 16, 2016 Category: Cytology Source Type: research
Diverse roles of endoplasmic reticulum stress sensors in bacterial infection
Abstract
Bacterial infection often leads to cellular damage, primarily marked by loss of cellular integrity and cell death. However, in recent years, it is being increasingly recognized that, in individual cells, there are graded responses collectively termed cell-autonomous defense mechanisms that induce cellular processes designed to limit cell damage, enable repair, and eliminate bacteria. Many of these responses are triggered not by detection of a particular bacterial effector or ligand but rather by their effects on key cellular processes and changes in homeostasis induced by microbial effectors wh...
Source: Molecular and Cellular Pediatrics - February 16, 2016 Category: Cytology Source Type: research
Genetics of irritable bowel syndrome
Abstract
Irritable bowel syndrome (IBS) is a common condition with a complex and largely unknown etiology. There is no cure, and treatment options are mainly directed to the amelioration of symptoms. IBS causes reduced quality of life and poses considerable repercussions on health and socioeconomic systems. There is a heritable component in IBS, and genetic research is a valuable tool for the identification of causative pathways, which will provide important insight into the pathophysiology. However, although some gene-hunting efforts have been conducted and a few risk genes proposed, IBS genetic resear...
Source: Molecular and Cellular Pediatrics - February 12, 2016 Category: Cytology Source Type: research
Mesenchymal stem/stromal cells—a key mediator for regeneration after perinatal morbidity?
Abstract
Perinatal complications in both term- and preterm-born infants are a leading cause of neonatal morbidities and mortality. Infants face different challenges in the neonatal intensive care unit with long-term morbidities such as perinatal brain injury and bronchopulmonary dysplasia being particularly devastating. While advances in perinatal medicine have improved our understanding of the pathogenesis, effective therapies to prevent and/or reduce the severity of these disorders are still lacking. The potential of mesenchymal stem/stromal cell (MSC) therapy has emerged during the last two decades, ...
Source: Molecular and Cellular Pediatrics - February 11, 2016 Category: Cytology Source Type: research
Mesenchymal stem/stromal cells —a key mediator for regeneration after perinatal morbidity?
< h3 class= " a-plus-plus " > Abstract < /h3 > < p class= " a-plus-plus " > Perinatal complications in both term- and preterm-born infants are a leading cause of neonatal morbidities and mortality. Infants face different challenges in the neonatal intensive care unit with long-term morbidities such as perinatal brain injury and bronchopulmonary dysplasia being particularly devastating. While advances in perinatal medicine have improved our understanding of the pathogenesis, effective therapies to prevent and/or reduce the severity of these disorders are still lacking. The potential of mesenchymal stem/stromal cell (MSC) th...
Source: Molecular and Cellular Pediatrics - February 10, 2016 Category: Cytology Source Type: research
The clinical consequences of sucrase-isomaltase deficiency
Abstract
Primary sucrase-isomaltase deficiency, originally thought to be a homozygous recessive disorder, has been found to have numerous genetic variants that alone or in combination (compound heterozygosity) express varying degrees of clinical illness, most commonly causing chronic diarrhea, abdominal pain, and bloating. These symptoms are also present with secondary sucrase-isomaltase deficiency. Recent investigations are providing evidence that sucrase-isomaltase deficiency is more prevalent and of greater clinical significance than previously suspected. Further research is required to correlate the...
Source: Molecular and Cellular Pediatrics - February 8, 2016 Category: Cytology Source Type: research
