Transient spontaneous remission in congenital MLL-AF10 rearranged acute myeloid leukemia presenting with cardiorespiratory failure and meconium ileus
ConclusionsOur patient exhibited a unique manifestation of neonatal MLL-AF10 rearranged AML with cardiorespiratory failure and intestinal infiltration. It highlights the importance of leukemia in the differential diagnosis of neonatal distress, congenital hematological abnormalities, and skin lesions. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - August 28, 2016 Category: Cytology Source Type: research
The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
ConclusionsOur data, though limited to only one patient, emphasize that therapeutic intervention with canakinumab seems to be improve kidney function in colchicine-resistant FMF with renal amyloidosis. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - August 14, 2016 Category: Cytology Source Type: research
Molecular biology of the gut
(Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - August 11, 2016 Category: Cytology Source Type: research
Early origins of asthma (and allergy)
SummaryAsthma 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 ma...
Source: Molecular and Cellular Pediatrics - August 9, 2016 Category: Cytology Source Type: research
Vitamin A-retinoid signaling in pulmonary development and disease
AbstractRetinoic 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. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - July 31, 2016 Category: Cytology Source Type: research
Lung epithelial GM-CSF improves host defense function and epithelial repair in influenza virus pneumonia —a new therapeutic strategy?
AbstractInfluenza 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. After IV infection they release a cascade of immune mediators,...
Source: Molecular and Cellular Pediatrics - July 31, 2016 Category: Cytology Source Type: research
The puzzle of immune phenotypes of childhood asthma
< h3 class= " a-plus-plus " > Abstract < /h3 > < p class= " a-plus-plus " > 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. This review summarizes recent key finding...
Source: Molecular and Cellular Pediatrics - July 27, 2016 Category: Cytology Source Type: research
The need for coordination of research activities in pediatric lung diseases
(Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - July 26, 2016 Category: Cytology Source Type: research
Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease
< h3 class= " a-plus-plus " > Abstract < /h3 > < p class= " a-plus-plus " > 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 progre...
Source: Molecular and Cellular Pediatrics - July 24, 2016 Category: Cytology Source Type: research
Early injury of the neonatal lung contributes to premature lung aging: a hypothesis
Abstract
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 sac...
Source: Molecular and Cellular Pediatrics - July 11, 2016 Category: Cytology Source Type: research
Pathogenesis of bronchopulmonary dysplasia: when inflammation meets organ development
Abstract
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. The extent of the pro-inflammatory reaction and the disturb...
Source: Molecular and Cellular Pediatrics - June 28, 2016 Category: Cytology Source Type: research
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis
Conclusions
Mendeliome sequencing was successfully used to disentangle the underlying cause of severe neonatal lactic acidosis. Indeed, it is one of several targeted sequencing approaches that allow rapid and reliable counseling of the parents, adaptation of the clinical management, and renunciation of unnecessary, potentially invasive and often costly diagnostic measures. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - June 16, 2016 Category: Cytology Source Type: research
Mechanism of human rhinovirus infections
Abstract
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. How this may account for distinct immune responses elicited by various rh...
Source: Molecular and Cellular Pediatrics - May 31, 2016 Category: Cytology Source Type: research
The cross-talk between enterocytes and intraepithelial lymphocytes
Abstract
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...
Source: Molecular and Cellular Pediatrics - May 31, 2016 Category: Cytology Source Type: research
MicroRNA in late lung development and bronchopulmonary dysplasia: the need to demonstrate causality
Abstract
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. A number of studies to date have reporte...
Source: Molecular and Cellular Pediatrics - May 22, 2016 Category: Cytology Source Type: research
