Abstract:
:We compared proliferation (growth) and differentiation (development) related proteins in normal and hypoplastic fetal murine lungs. The hypoplastic lungs were created in CD-1 fetal mice by nitrofen exposure (25 mg per pregnant mouse given intragastrically on gestational day 8 [Gd8]), as published earlier. The lungs were harvested at Gd14, 16, 19 and from neonates. Immunoblot analyses were carried out for transcription factors (oncogenic proteins, nuclear receptor, and transmembrane receptor proteins) in severely hypoplastic murine fetal lungs with coexistent diaphragmatic hernia, and results were compared with those derived from normal lungs of equivalent age. These proteins have proposed roles in the regulation of proliferation and differentiation processes of fetal lungs. We have shown that the product of the oncogene c-myc was reduced in hypoplastic lungs at all stages of gestation, whereas c-Fos protein levels were variable. These proteins are known to regulate transcription of various developmental proteins, such as those responsible for proliferation and differentiation. Further, the nuclear transcription factors thyroid transcription factor-1 (TITF-1) and glucocorticoid receptor (GR) were reduced, and thyroid hormone receptor (TR) and retinoic acid receptors (RARs) were inhibited in severely hypoplastic lungs compared to normal lungs of equivalent gestational stage, except in neonatal lungs, where signals for RARs were seen. TITF-1 is known to localize in bronchial epithelial cells in developing lungs. It is restricted to type II pneumocytes with gestational development in the normal lungs and regulates surfactant proteins. Earlier, we have reported that surfactant proteins are reduced in hypoplastic lungs. In the current study, reduced GR and TITF-1 proteins may play a role in reducing surfactant proteins in the hypoplastic lungs. The significant inhibition in TR and RARalpha in the severely hypoplastic lungs reflects on affected epithelial cell maturation and alveolar formation, respectively. Altered RARbeta levels correlate with affected lung growth and branching morphogenesis of nitrofen-exposed lungs. A transmembrane receptor protein EGFR was reduced in hypoplastic lungs, suggesting the involvement of altered mesenchymal-epithelial signal transduction pathways. We conclude (1) Our data suggest altered levels of various nuclear transcription factors in the murine fetal hypoplastic lungs; (2) Reduced levels TITF-1 protein in hypoplastic lungs may have caused the functional immaturity of distal lung, immature airways and thus may affect overall differentiation of lungs. These results correlated with low levels of surfactant proteins in these lungs; (3) TR and RAR inhibition indicate their roles through reduced or retarded proliferation and differentiation processes in the severely hypoplastic lungs; (4) GR down-regulation in developing fetal murine hypoplastic lungs indicate delayed development, and GR up-regulation in affected neonates may be induced by stress/stretch caused at birth due to air-breathing; (5) Down- regulation of EGFR indicate altered mesenchymal-epithelial interactions and possible influence on lung proliferation and differentiation.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Chinoy MR,Chi X,Cilley REdoi
10.1002/ppul.1099subject
Has Abstractpub_date
2001-08-01 00:00:00pages
129-41issue
2eissn
8755-6863issn
1099-0496pii
10.1002/ppul.1099journal_volume
32pub_type
杂志文章abstract::Dysfunctional swallowing is an uncommon, but important cause of bronchiectasis. We describe a child with a brainstem tumor, who developed bronchiectasis caused by chronic aspiration secondary to a dysfunctional swallow. The case highlights the importance of thorough and repeated evaluation before a diagnosis of idiopa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21152
更新日期:2010-02-01 00:00:00
abstract::Pneumatoceles and bronchiectasis are secondary complications of respiratory infections in patients with hyper IgE syndrome (HIgES). We report on a patient with HIgES and recurrent pneumonias since 1 year of age, with progression to pneumatoceles and bronchiectasis and fatal outcome, with disseminated infection and sus...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22532
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Although the benefits of recombinant human deoxyribonuclease (dornase alfa) in patients with cystic fibrosis (CF) are established, its optimal timing in relation to physiotherapy is unknown. As its enzymatic effect lasts for 6-11 hr, dornase alfa may be more efficacious if the time interval between inhalatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ppul.20704
更新日期:2007-12-01 00:00:00
abstract::Episodes of respiratory distress with chest retraction and wheezing, sometimes associated with facial edema, were noted after administering the proton pump inhibitors omeprazole and esomeprazole in an infant with gastroesophageal reflux. The disturbances relieved dramatically after withdrawing the proton pump inhibito...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20616
更新日期:2007-06-01 00:00:00
abstract:OBJECTIVES:Sildenafil, tezosentan, and prostacyclin reduce pulmonary vascular pressures in pulmonary hypertension, but have potential to vasodilate the systemic circulation. Nebulized vasodilators allow targeted drug delivery, high local drug concentrations, less systemic hypotension, and better matching of the lung's ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20431
更新日期:2006-07-01 00:00:00
abstract::It has been suggested that cystic fibrosis (CF) patients harboring multiresistant (MR) Pseudomonas aeruginosa (PA) should be seen in separate clinics. The aim of this study was to test the feasibility of this by longitudinally studying the consistency of isolates of MRPA in individuals. We analyzed all respiratory tra...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10262
更新日期:2003-04-01 00:00:00
abstract::Pneumomediastinum is uncommon in pediatric medical practice, outside the neonatal period. While asthma or respiratory infections are the most frequent underlying causes, it is important not to forget the possibility of foreign body aspiration, particularly after the clinical presentation. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10295
更新日期:2003-07-01 00:00:00
abstract:BACKGROUND:Acquiring sputum cultures from infants is considered challenging. We describe their yield in infants with cystic fibrosis (CF) and other chronic suppurative lung diseases (CSLDs). METHODS:Retrospective medical record review over a 4-year period, for infants aged 0-2 years with ≥2 airway bacterial cultures a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25103
更新日期:2020-12-01 00:00:00
abstract:BACKGROUND:The measurement of specific airway resistance during tidal breathing (sRaw(tb)) has gained popularity in children, but methodological concerns have been raised regarding the electronic compensation for the thermal artifact. The panting method (sRaw(p)) is efficient in minimizing the latter, but may be associ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22829
更新日期:2014-03-01 00:00:00
abstract:BACKGROUND:In young children with cystic fibrosis (CF) the forced expiratory volume in 1 second (FEV1 ) is often normal and a more sensitive measure to detect early obstructive lung disease is needed. AIM:To evaluate the progression of selected spirometry parameters with age in a cohort of CF patients and healthy chil...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22777
更新日期:2013-11-01 00:00:00
abstract::End-tidal PCO2 (PETCO2) measurements from two commercially available neonatal infrared capnometers with different sampling systems and a mass spectrometer were compared with arterial PCO2 (PaCO2) to determine whether the former could predict the latter in mechanically ventilated rabbits with and without lung injury. T...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950170309
更新日期:1994-03-01 00:00:00
abstract::Pulmonary hemorrhage (PH) is a serious complication causing acute respiratory distress in the premature infant, and it is associated with significant mortality and morbidity. The role of inflammatory mediators in this condition is largely undefined. Serial tracheal aspirates (TA) were obtained at intervals from 65 mec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10141
更新日期:2002-08-01 00:00:00
abstract:OBJECTIVE:Both healthy preterm infants and those with bronchopulmonary dysplasia (BPD) have poor lung function during childhood and adolescence, although there is no evidence whether prematurity alone explains the reduction in lung function found in BPD infants. Our study seeks to know if lung function, measured in inf...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21609
更新日期:2012-07-01 00:00:00
abstract:OBJECTIVE:To examine cystic fibrosis (CF) physician adherence to the 2007 CF Foundation (CFF) Pulmonary Guidelines for Chronic Medications. Specifically adherence and barriers to prescribing level A medication recommendations (i.e., inhaled tobramycin and dornase alfa) and level B medication recommendations (i.e., macr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21573
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND:Prone sleeping position, use of soft mattresses and head covering by bedclothes are known risk factors for sudden infant death syndrome (SIDS). Rebreathing carbon dioxide (CO(2) ) may be a possible mechanism or a confounding factor of SIDS. OBJECTIVE:To compare the aeration properties of a new concept of in...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21351
更新日期:2011-02-01 00:00:00
abstract::Our objective was to determine whether postnatal respiratory function, lung growth, and lung structure are affected by preterm birth which did not require neonatal respiratory support. Two groups of preterm (P) lambs were delivered 2 weeks before term, at 133 days of gestational age (GA). Tissue was collected at term ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20274
更新日期:2005-10-01 00:00:00
abstract:BACKGROUND:Respiratory viral and atypical bacterial infections are associated with pulmonary exacerbations and hospitalisations in cystic fibrosis patients. We wanted to study the impact of such infections on children attending the outpatient clinic. METHODS:Seventy-five children were followed for 12 months at regular...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20517
更新日期:2006-12-01 00:00:00
abstract::Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systemati...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23632
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND:Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed to asse...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21149
更新日期:2010-03-01 00:00:00
abstract:BACKGROUND:Pneumonia is a leading killer of children under-5 years, with a high burden in Nigeria. We aimed to quantify the regional burden and risks of pediatric pneumonia in Nigeria, and specifically the states of Lagos and Jigawa. METHODS:We conducted a scoping literature search for studies of pneumonia morbidity a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24626
更新日期:2020-06-01 00:00:00
abstract::Randomized trials of ribavirin therapy have used clinical scores to assess illness severity. Little information on agreement for these findings between observers has been published. We decided to determine interobserver agreement for (1) a history for apnea or respiratory failure; (2) assessment of cyanosis, respirato...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/(SICI)1099-0496(199607)22:1<23::AID-PPUL4>
更新日期:1996-07-01 00:00:00
abstract::Bronchial reactivity was assessed in 66 children with bronchial asthma (aged 8-15 years) by provocation with histamine-HCl during a symptom-free period. A significant bronchial reaction to histamine was defined as a 50% increase in the resistance of the respiratory system (Rrs) determined by the forced oscillation tec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950150308
更新日期:1993-03-01 00:00:00
abstract:AIMS:To explore whether gender differences in the Scandinavian Cystic Fibrosis (CF) patients exist in the areas of key clinical parameters, complications, and medication. METHODS:Cross-sectional data on 890 (416 female) pancreatic insufficient CF patients were evaluated regarding chronic infection, body mass index, lu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21265
更新日期:2010-10-01 00:00:00
abstract::In order to better understand the regulation of lung maturation by glucocorticoid-glucocorticoid receptor signaling, we studied glucocorticoid receptor (GR) hypomorphic mice with a mixed C57Bl6/129 sv background, in which disruption of exon 2 of the GR gene produces an N-terminal truncated GR protein. Four groups of m...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20746
更新日期:2008-02-01 00:00:00
abstract::It has been previously shown that prophylactic, intravenous dexamethasone (DEX) and intratracheal recombinant human Cu/Zn superoxide dismutase (SOD) ameliorate lung injury in newborn piglets treated with 48 hr of hyperoxia and mechanical ventilation. DEX has many pharmacologic effects, including the possible induction...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950200210
更新日期:1995-08-01 00:00:00
abstract:BACKGROUND:The diagnosis of community-acquired pneumonia (CAP) is based mainly on the patient's medical history and physical examination. However, in severe cases a further evaluation including chest X-ray (CXR) may be necessary. At present, lung ultrasound (LUS) is not included in the diagnostic work-up of pneumonia. ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.22585
更新日期:2013-03-01 00:00:00
abstract:BACKGROUND:Plastic bronchitis (PB) is a rare, variable, and potentially fatal disease. This study aimed to assess the efficacy of fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL) in treating children with PB. METHODS:In total, 15 children with PB, between 2012 and 2020, were enrolled in our study. Within...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25016
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Most international asthma guidelines recommend that children ≤5 years with asthma or recurrent wheezing be treated with daily low- moderate dose inhaled corticosteroids (ICS) as the preferred controller and leukotriene receptor antagonists (LTRA) as alternative therapy. There is no systematic review comparin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24176
更新日期:2018-12-01 00:00:00
abstract:OBJECTIVE:Recent advances in medicine have allowed children with chronic life-threatening disorders to survive longer than ever before with the use of complex medical device technology (e.g., mechanical ventilation, dialysis, etc.). The care of children with chronic pulmonary disorders and respiratory-technology depend...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23155
更新日期:2015-12-01 00:00:00
abstract::Our objective was to examine the characteristics of preterm and full-term infants < or = 6 weeks old that influence the success of obtaining sufficient sweat for diagnosis of CF, and corresponding sweat chloride concentrations. A retrospective chart review of 119 sweat tests was performed on 103 preterm and full-term ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20235
更新日期:2005-07-01 00:00:00