Abstract:
:Mucus plugging constitutes a nutrient-rich nidus for a bacterial infection that has long been recognized as a potent stimulus for neutrophilic airway inflammation driving progressive lung damage in people with cystic fibrosis (CF). However, mucus plugging and neutrophilic inflammation are already present in many infants and young children with CF even in the absence of detectable bacterial infection. A series of observational studies in young children with CF, as well as investigations in animal models with CF-like lung disease support the concept that mucus plugging per se can trigger inflammation before the onset of airways infection. Here we review emerging evidence suggesting that activation of the interleukin-1 (IL-1) signaling pathway by hypoxic epithelial cell necrosis, leading to the release of IL-1α in mucus-obstructed airways, may be an important mechanistic link between mucus plugging and sterile airway inflammation in early CF lung disease. Furthermore, we discuss recent data from preclinical studies demonstrating that treatment with the IL-1 receptor (IL-1R) antagonist anakinra has anti-inflammatory as well as mucus modulating effects in mice with CF-like lung disease and primary cultures of human CF airway epithelia. Collectively, these studies support an important role of the IL-1 signaling pathway in sterile neutrophilic inflammation and mucus hypersecretion and suggest inhibition of this pathway as a promising anti-inflammatory strategy in patients with CF and potentially other muco-obstructive lung diseases.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Balázs A,Mall MAdoi
10.1002/ppul.24462subject
Has Abstractpub_date
2019-11-01 00:00:00pages
S5-S12eissn
8755-6863issn
1099-0496journal_volume
54 Suppl 3pub_type
杂志文章,评审abstract::"Pulmonary Interstitial Glycogenosis: Diagnostic Evaluation and Clinical Course," written by Liptzin et al is a timely and insightful phenotypic summary of a rare pediatric interstitial lung disease. Twenty-four infants with biopsy-proven pulmonary interstitial glycogenosis (PIG) were reviewed at their center. Genetic...
journal_title:Pediatric pulmonology
pub_type: 评论,杂志文章
doi:10.1002/ppul.24167
更新日期:2018-12-01 00:00:00
abstract:INTRODUCTION:Foreign body (FB) aspiration is a frequent and preventable source of morbidity and mortality, especially in children under 4 years of age. Few comprehensive studies exist on presentation and outcome of apple aspirations in children. METHODS:In a retrospective analysis of bronchoscopy records of a tertiary...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24768
更新日期:2020-07-01 00:00:00
abstract::Nicotine, 1 mg/kg body weight/day, was injected subcutaneously in 3 female rabbits during gestational and lactating periods, and the lungs of the offspring were studied by scanning and transmission electron microscopy (SEM and TEM) on days 5, 10, and 25 postpartum. Three other female rabbits served as controls. The si...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950010606
更新日期:1985-11-01 00:00:00
abstract::We examined the effects of within-breath changes in compliance (C) upon the accuracy of measurements of compliance and resistance (R) by linear regression analysis and by Mead and Wittenberger's method. These effects were illustrated by a computer model and by lung models with linear and nonlinear pressure/volume rela...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120304
更新日期:1992-03-01 00:00:00
abstract:BACKGROUND:Treatment regimens for patients with cystic fibrosis (CF) are time-consuming and complex, resulting in consistently low adherence rates. To date, few studies have evaluated innovative technologies to improve adherence in this population. Current infection control guidelines for patients with CF seek to minim...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21164
更新日期:2010-02-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a well-known complication of cystic fibrosis (CF), with an estimated incidence of up to 11%. In patients with CF, the diagnosis of ABPA must be based on significant elevation of Aspergillus fumigatus (Af) antibody and total serum IgE, since it is common to already have...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950170210
更新日期:1994-02-01 00:00:00
abstract::Fetuses of diabetic mothers who were exposed to excessive glucose show delayed maturation. Under these conditions, altered growth factor expression or signaling may have important regulatory influences. We examined the role of epidermal growth factor (EGF) in lung development and maternal diabetes in the rat. In order...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200002)29:2<103::aid-ppul4
更新日期:2000-02-01 00:00:00
abstract::We conducted a survey to determine parent-reported asthma prevalence, morbidity, and healthcare utilization among 3527 children attending public schools (n = 2849) and private schools (n = 678) in San Juan, Puerto Rico. Schools were randomly selected from each of 12 geographic regions of the San Juan metropolitan area...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20022
更新日期:2004-05-01 00:00:00
abstract:OBJECTIVE:To determine the effect of changing electrode positions on vital signs and respiratory effort parameters measured with transcutaneous electromyography of the diaphragm (dEMG) in preterm infants. METHODS:In this observational study, simultaneous dEMG measurements were performed at the standard position and at...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24585
更新日期:2020-02-01 00:00:00
abstract::The airway occlusion techniques for assessing passive respiratory mechanics have become well established methods in fullterm neonates and older infants. The single breath technique (SBT) is frequently used for assessing lung function in intubated infants on neonatal intensive care units. However, less is known about t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950150508
更新日期:1993-05-01 00:00:00
abstract::The long-term efficacy and safety of aminoglycoside aerosol therapy for Pseudomonas aeruginosa colonization/infection in cystic fibrosis has not been fully investigated. In the present study, 14 patients with cystic fibrosis, ages 8-19 years (mean: 13.3 years), received tobramycin aerosol therapy for a mean duration o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060207
更新日期:1989-01-01 00:00:00
abstract:OBJECTIVE:To validate the bronchiolitis score of Sant Joan de Déu (BROSJOD) and to examine the previously defined scoring cutoff. PATIENTS AND METHODS:Prospective, observational study. BROSJOD scoring was done by two independent physicians (at admission, 24 and 48 hr). Internal consistency of the score was assessed us...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23546
更新日期:2017-04-01 00:00:00
abstract::Pseudomonas aeruginosa is often cultured from the airways of children with tracheostomies. P. aeruginosa produces exotoxin A (ETA) and type III cytotoxins. This study tested the hypothesis that children with tracheostomies are colonized by P. aeruginosa that express these virulence factors and will have antibodies dir...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20194
更新日期:2005-05-01 00:00:00
abstract:BACKGROUND:Disease burden in cystic fibrosis (CF) impacts quality of life, distress, and treatment adherence. The promoting resilience in stress management (PRISM), is a brief patient-focused intervention to promote resilience in adolescents and young adults (AYAs), which may mitigate the negative outcomes, and is prov...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.24574
更新日期:2020-03-01 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::Our study was aimed at elucidating the effect of strenuous maternal exercise (running at a speed of 20 m/min) on fetal somatic growth and lung development. Dams were separated into three groups: (1) exercising during the entire course of pregnancy; (2) exercising from the 16th to the 20th gestational day; and (3) cont...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950150604
更新日期:1993-06-01 00:00:00
abstract::The reliability and predictive value of salivary theophylline levels was investigated in 58 children who were treated with oral sustained-release theophylline preparations for bronchial asthma. Serum and saliva levels were assessed by fluorescence polarization immunoassay (FPIA), and Seralyzer (reagent strip reflectan...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060407
更新日期:1989-01-01 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:RATIONALE AND OBJECTIVES:Little is known about molecular changes in lungs of fetal rabbits with surgically induced diaphragmatic hernia (DH). Therefore, we examined in this model gene expressions of pivotal molecules for the developing lung. METHODS:At day 23 of gestation, DH was created in 12 fetuses from 4 does. Bot...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22512
更新日期:2012-11-01 00:00:00
abstract::Lung hypoplasia is the main cause of congenital diaphragmatic hernia (CDH)-associated death but pathogenesis remains unclear. MiR-455-5p is involved in lung hypoplasia. We hypothesized that nitrofen causes abnormal miR-455-5p expression during lung development and designed this study to determine the relationship betw...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24739
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:Severe inflammation plays a vital role in the pathogenesis of meconium aspiration syndrome (MAS). Intratracheal (IT) instillation of corticosteroids may be beneficial for MAS in optimizing local effect and reducing systemic adverse effects, but the optimum dosing course remains open to question. METHODS:Thi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23697
更新日期:2017-07-01 00:00:00
abstract::With better understanding of the role of type 2 inflammation in allergic asthma, there has been progress made in the development of new biologic therapies targeting these specific pathways. This review will consider diagnostic criteria for using biologic therapies for pediatric asthma with special emphasis on populati...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24613
更新日期:2020-03-01 00:00:00
abstract:OBJECTIVE:To evaluate safety and efficacy of oral posaconazole and terbinafine for Lomentospora prolificans and Scedosporium apiospermum in children with cystic fibrosis. METHODS:Retrospective case review. RESULTS:There were five children (four girls), median age 15.0 years; three had S. apiospermum and two had L. pr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24789
更新日期:2020-08-01 00:00:00
abstract:OBJECTIVE:To carry out a cost-effectiveness analysis of omitting chest radiography in the diagnosis of infant bronchiolitis. HYPOTHESIS:Omitting chest radiographs in the diagnosis of typical bronchiolitis was expected to reduce costs without adversely affecting the detection rate of alternate diseases. STUDY DESIGN:A...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20948
更新日期:2009-02-01 00:00:00
abstract::Neonatal exposure to intermittent hypoxia results in altered ventilatory response to subsequent hypoxia in animal models. The effect of similar exposure in human infants is unknown. Our objective was to determine the impact of sleep disordered breathing (SDB) in early infancy on ventilatory response in infants. We rec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22568
更新日期:2013-03-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20520
更新日期:2007-01-01 00:00:00
abstract::Pediatric Pulmonology continues to publish research and clinical topics related to the entire range of children's respiratory disorders. As we have done annually in recent years, we here summarize some of the past year's publications in our major topic areas, as well as selected literature in these areas from other co...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23719
更新日期:2017-09-01 00:00:00
abstract::The cytokines tumor necrosis factor-alpha (TNF-alpha), interleukin-8 (IL-8), and intercellular adhesion molecule-1 (ICAM-1) have important roles in regulating neutrophil migration and the inflammatory response. To determine whether the concentration of these cytokines and soluble ICAM-1 (sICAM-1) in sputum was increas...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199601)21:1<11::AID-PPUL2>
更新日期:1996-01-01 00:00:00
abstract:INTRODUCTION:Chest impedance (CI) is the current standard for cardio-respiratory monitoring in preterm infants but fails to provide direct and quantitative information on diaphragmatic activity. Transcutaneous electromyography (dEMG) is able to measure diaphragmatic activity, but its feasibility and repeatability to mo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23116
更新日期:2015-09-01 00:00:00
abstract::With dual energy X-ray absorptiometry (DEXA), it is possible to quantitate important aspects of growth in children with cystic fibrosis (CF), supplementing the usual measures of height and weight. Of particular concern during growth is the accumulation of bone mineral, since osteoporosis and fractures are well-recogni...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199902)27:2<80::aid-ppul3>
更新日期:1999-02-01 00:00:00