Abstract:
:A thorough understanding of the early natural history of cystic fibrosis (CF) lung disease is critical for the development of effective interventions in the youngest patients. We assessed the evolution of pulmonary infection, inflammation, and clinical course among 40 infants over a 2-year period through annual bronchoalveolar lavage (BAL) for culture and measurements of pro- and anti-inflammatory cytokines, semiannual infant pulmonary function testing, and quarterly clinical evaluations. Both the prevalence of CF pathogens and their density in BAL fluid increased with age. Infants had neutrophilic lower airway inflammation and elevated IL-8 concentrations independent of whether CF pathogens were recovered. Total leukocyte and neutrophil densities and IL-8 concentrations increased with density of CF pathogens in BAL fluid, whether the isolated organism was P. aeruginosa or another pathogen. IL-10 concentrations were similar in CF subjects and non-CF historical controls. Infants generally had suboptimal growth (low weight and height percentiles) and obstructive lung disease (decreased expiratory flows and air trapping). Subjects from whom CF pathogens were isolated at > 10(5) cfu/mL had the worst air trapping and lowest Brasfield chest X-ray scores. Our findings provide a foundation for future studies of early intervention in CF lung disease, including antimicrobial and anti-inflammatory therapy.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Rosenfeld M,Gibson RL,McNamara S,Emerson J,Burns JL,Castile R,Hiatt P,McCoy K,Wilson CB,Inglis A,Smith A,Martin TR,Ramsey BWdoi
10.1002/ppul.1144subject
Has Abstractpub_date
2001-11-01 00:00:00pages
356-66issue
5eissn
8755-6863issn
1099-0496pii
10.1002/ppul.1144journal_volume
32pub_type
杂志文章abstract:OBJECTIVES:To develop reference equations of maximal voluntary ventilation (MVV) in children and adolescents, and to test the validity and reproducibility of MVV. STUDY DESIGN:Cross-sectional study. PATIENT-SUBJECT SELECTION:A total of 348 healthy volunteers (6-17 years)-248 for the development of reference equations...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24576
更新日期:2020-02-01 00:00:00
abstract::This paper assesses the effectiveness of aerosolized tobramycin (TOBI) on cystic fibrosis (CF) lung disease, using a radiologic tool. The published tool, the age-based severity curve (ABS), is derived from Brasfield scoring of chest X-rays (CXR). This study evaluates both the usefulness of the ABS as an assessment too...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20032
更新日期:2004-07-01 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::The pediatric pneumogram is a frequently used tool in the diagnosis and management of apnea during infancy. We analyzed 287 pneumographic recordings from 123 full-term infants (63 males) obtained during the first 12 months of life to establish normative values for apnea, periodic breathing, and bradycardia. The result...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950010409
更新日期:1985-07-01 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:BACKGROUND:Community-acquired pneumonia (CAP) is a leading cause of childhood death. There are few published reports of radiographic findings among children with severe CAP. OBJECTIVE:To describe chest X-ray (CXR) findings and assess association between these radiographic findings and pneumococcal isolation in childre...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ppul.21287
更新日期:2010-10-01 00:00:00
abstract::Bronchiolitis in infancy is a risk factor for development of asthma in the first decades of life, although the majority may be asymptomatic at school age. Respiratory symptoms are common in early life, and prediction of later asthma may be challenging. We aimed to study if simple clinical variables assessed at 2 years...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22675
更新日期:2013-06-01 00:00:00
abstract::The optimal duration of therapy for acute exacerbations of cystic fibrosis (CF) has not been defined, and the utility of serial pulmonary function testing in predicting the duration of therapy has yet to be established. In a review of 90 pulmonary exacerbations of 39 patients with CF requiring hospitalization, we foun...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950160404
更新日期:1993-10-01 00:00:00
abstract:BACKGROUND:Gastro-esophageal reflux (GOR) may contribute to lung disease in children with cystic fibrosis (CF). There is conflicting evidence regarding the effect of chest physiotherapy (CPT) in the head-down position on GOR. Furthermore, there is currently no evidence on the impact of physiotherapy on GOR as assessed ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21524
更新日期:2012-02-01 00:00:00
abstract:OBJECTIVE:While the impact of fertility treatments on the perinatal outcome is well established, the long-term effects on offspring are yet to be determined. The current study aimed to investigate the risk of long-term obstructive sleep apnea (OSA) among children born following in vitro fertilization (IVF) and ovulatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24428
更新日期:2019-10-01 00:00:00
abstract:OBJECTIVES:To investigate the adherence and the self-reported barriers to general and respiratory exercises reported by individuals with cystic fibrosis (CF). STUDY DESIGN:An exploratory, experimental study. METHODS:Community-dwelling individuals aged 16 years and over, diagnosed with CF, who were accompanied in refe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24912
更新日期:2020-10-01 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: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: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::Multiple breath inert gas washout (MBW) is gaining popularity for measurements of resting lung volume and ventilation inhomogeneity. Test reproducibility is an important determinant of the clinical applicability of diagnostic tests. The between-test reproducibility of variables derived from MBW tests in newborn infant...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21113
更新日期:2010-01-01 00:00:00
abstract::Sudden unexplained deaths have been reported in 13% [corrected] of Familial Dysautonomia (FD) subjects. To characterize cardiorespiratory dysregulation in children with FD that might contribute to potential sudden death, respiratory inductance plethysmography (chest/abdomen), ECG, hemoglobin saturation, and pulse wave...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20764
更新日期:2008-03-01 00:00:00
abstract:OBJECTIVE:While pulmonary arterial hypertension (PAH) is rare in infants and children, it results in substantial morbidity and mortality. In recent years, prognosis has improved, coinciding with the introduction of new PAH-targeted therapies, although much of their use in children is off-label. Evidence to guide the tr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24442
更新日期:2019-10-01 00:00:00
abstract::We developed a simple method to identify neonates at high risk of bronchopulmonary dysplasia (BPD) and determined whether early (8 hours) and late (14 days) risk assessment is equally useful. A retrospective cohort design was utilized of subjects enrolled in multi-dose surfactant trials to develop each risk identifica...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199606)21:6<345::AID-PPUL1
更新日期:1996-06-01 00:00:00
abstract:BACKGROUND:We hypothesized airway inflammation can be detected non-invasively by induced sputum (IS) or peripheral blood eosinophilia, and IS can detect bacterial and viral infection in preschool children with airway disease, with results comparable to broncho-alveolar lavage (BAL). METHODS:Preschool children with cys...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23366
更新日期:2016-08-01 00:00:00
abstract::We evaluated 4 patients who developed severe, symptomatic stridor during maximal cardiopulmonary exercise testing, all referred due to exercise-related dyspnea. All underwent resting, unsedated transnasal fiberoptic laryngoscopy and had normal findings. Four patients performed repeat maximal exercise testing with fibe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20076
更新日期:2005-01-01 00:00:00
abstract::Although airway obstruction and chronic endobronchial infection have long been recognized as major factors in the pathogenesis of lung disease in cystic fibrosis (CF), only recently has it been recognized that the inflammatory process itself may be responsible in a major way for destroying the lungs. The most characte...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199708)24:2<137::aid-ppul1
更新日期:1997-08-01 00:00:00
abstract:BACKGROUND:Pentraxin 3 (PTX-3) is an acute-phase protein that increases in the plasma during inflammation. OBJECTIVE:We aimed to evaluate the usefulness of PTX-3 as a clinical marker in children with lower respiratory tract infection (LRTI) and examine the correlation of PTX-3 with other biomarkers such as C-reactive ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23199
更新日期:2016-01-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to assess pulmonary function and its predictors in very low birth weight (birth weight ≤1,500 g) children (VLBWc) with or without bronchopulmonary dysplasia (BPD), born at gestational age ≤32 weeks at a single tertiary center during 1996-1999, after the introduction of surfactant the...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22676
更新日期:2013-08-01 00:00:00
abstract::In cystic fibrosis the bronchiectatic conducting airways have large numbers of neutrophils in their walls and in their luminal contents. The neutrophil's primary granule enzyme activities of elastase and peroxidase are increased in the sputum of these patients. It has been postulated that these enzymes--together or in...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199707)24:1<29::aid-ppul5>
更新日期:1997-07-01 00:00:00
abstract::In children unable to perform reliable spirometry, the interrupter resistance (R(int) ) technique for assessing respiratory resistance is easy to perform. However, few data are available on the possibility to use R(int) as a surrogate for spirometry. We aimed at comparing R(int) and spirometry at baseline and after br...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22526
更新日期:2012-10-01 00:00:00
abstract::Our aim was to evaluate long-term effects of exogenous surfactant therapy on pulmonary functional outcome in children born very preterm. We examined 40 children aged 7-12 years who were born before 30 weeks of gestation with an immature surfactant system, and were randomized to one of three treatment groups: human sur...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/(sici)1099-0496(199803)25:3<182::aid-ppul8
更新日期:1998-03-01 00:00:00
abstract::Our objective was to review the clinical features and radiographic manifestations of chest wall lesions in a pediatric chest unit. Twenty-five patients (11 males and 14 females, aged 14 months to 15 years) were suspected of having a chest wall lesion on the basis of clinical examination and chest radiograph. A retrosp...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10440
更新日期:2004-03-01 00:00:00
abstract::Nine young asthmatic children aged 2-5 years underwent methacholine challenge after placebo or albuterol administered by metered dose inhaler through a spacer device (Babyhaler) with a face mask in a double-blind, cross-over, randomized study. The methacholine challenge was performed using chest auscultation to define...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1950170503
更新日期:1994-05-01 00:00:00
abstract:BACKGROUND:Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM. METHODS AND PATIENTS:Clinical manifestations, pulmonary ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.22742
更新日期:2013-10-01 00:00:00
abstract::Four patients with severe cystic fibrosis lung disease, anorexia and weight loss, received Megestrol Acetate (MA), as an appetite stimulant. The initial dose was 400-800 mg daily and was continued for 6-15 months. Appetite was improved, with significant weight gain in all patients and an increase in their weight for a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199911)28:5<380::aid-ppul1
更新日期:1999-11-01 00:00:00