Abstract:
:Early detection of progressive lung disease in cystic fibrosis (CF) may lead to better treatment and prognosis. Routine lung function indices may be relatively insensitive markers of peripheral airway obstruction and alveolar collapse. We hypothesized that the single-breath diffusion capacity of the lung for carbon monoxide (DLCO) would change before tests of airway function in patients with CF. We assessed lung function longitudinally in 53 children with CF during a mean period of 3.8 years to determine whether the diffusion capacity of the lung becomes abnormal before more conventional indices of lung function do. Within patients, DLCO was slightly elevated and remained stable, while forced expired volume in 1 sec (FEV1) and forced vital capacity (FVC) declined progressively (mean individual decline, -1.8% and -0.8% of predicted). Cross-sectionally, this decline was faster (mean group decline -3.8% and -2.8% of predicted), indicating an additional cohort effect. Normalized diffusion capacity at an early stage of CF is slightly elevated and is preserved in spite of progressive obstructive lung disease. This can be attributed to alterations in pulmonary and bronchial circulation due to loss of function and/or number of alveolar units. Diffusion capacity at rest does not appear to be a suitable early marker of progressive deterioration of CF lung disease.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Merkus PJ,Govaere ES,Hop WH,Stam H,Tiddens HA,de Jongste JCdoi
10.1002/ppul.10357subject
Has Abstractpub_date
2004-01-01 00:00:00pages
56-60issue
1eissn
8755-6863issn
1099-0496journal_volume
37pub_type
杂志文章abstract::One hundred fifty-four children with recurrent or chronic infections of the lower respiratory tract compatible with the diagnosis of primary ciliary dyskinesia (PCD) were evaluated for the presence of ultrastructural ciliary abnormalities. Studies were performed on multiple samples of respiratory mucosa obtained by na...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950100104
更新日期:1991-01-01 00:00:00
abstract:BACKGROUND:Asthma is the most common chronic inflammatory disease in childhood and some reports have demonstrated systemic inflammation. The relevance of high-sensitivity assays for C-reactive protein (hs-CRP), which are known to be a sensitive marker of low-grade systemic inflammation, has not been fully studied in ch...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21539
更新日期:2012-03-01 00:00:00
abstract::Foreign-body aspiration (FBA) is a commonly encountered serious problem during childhood, accounting for 7% of lethal accidents in children aged 1-3 years. Bronchoscopy is indicated when a clear history of FBA is present, but the history may be unreliable. Clinical manifestations can be seen in various diseases, and w...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20128
更新日期:2005-02-01 00:00:00
abstract:BACKGROUND:In preschool children, measurement of airway resistance using interrupter technique (Rint) is feasible to assess the degree of bronchial obstruction. Although some studies measured Rint in infancy, values of Rint and its variability in preterm infants are unknown. In this study, Rint and its variability was ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23771
更新日期:2017-10-01 00:00:00
abstract::Assessment of dynamic inspiratory function may provide valuable information about the degree and progression of pulmonary involvement in patients with Duchenne muscular dystrophy (DMD). The aims of this study were to characterize inspiratory function and to assess the efficacy of idebenone on this pulmonary function o...
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.23547
更新日期:2017-04-01 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::New Zealand children's morbidity from respiratory disease is high. This study examines whether subclinical ciliary abnormalities underlie the increased prevalence of respiratory disease in indigenous New Zealand children. A prospective study enrolled a group of healthy children who were screened for respiratory diseas...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20155
更新日期:2005-03-01 00:00:00
abstract::Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20393
更新日期:2006-04-01 00:00:00
abstract::Lower respiratory tract infections (LRTI) are common in the first year of life and are mostly caused by viruses. Severity of LRTI in infants is associated with early-life environmental factors. Genetic association studies also suggest a role of heredity in susceptibility to acute bronchiolitis. We designed a case cont...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20069
更新日期:2004-10-01 00:00:00
abstract:INTRODUCTION:Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders characterized by tissue deposition of glycosaminoglycans (GAG). Their musculoskeletal abnormalities and the GAG storage in the airway result in increased risk for patients undergoing anesthesia. This study evaluates a multi-disciplinary...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22629
更新日期:2013-06-01 00:00:00
abstract:AIM:The aim of this study was to investigate the status and effects of vitamin D supplement as adjuvant therapy in the management of respiratory distress syndrome (RDS) in preterm infants. PATIENT AND METHODS:Vitamin D was prospectively studied in 196 preterm neonates, 96 of them developed RDS, which further subdivide...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24552
更新日期:2020-01-01 00:00:00
abstract::Two hundred twenty-six healthy school children, with a mean age of 8.8 years; 62 girls mean age 8.8, 48 boys mean age 12.6 and 51 girls mean age 12.6 years at the start, were enrolled in a longitudinal study of lung function and tested annually for 5 years. All were free of respiratory symptoms, and none smoked more t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950080308
更新日期:1990-01-01 00:00:00
abstract:OBJECTIVE:Respiratory illnesses compose the most common diagnoses of patients admitted to pediatric intensive care units. In pediatrics, high-flow nasal cannula (HFNC) therapy is an intermediate level of respiratory support with variability in practice. We conducted a pre-post intervention study of patients placed on H...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25214
更新日期:2020-12-09 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::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::The aim of this study was to compare safety and efficacy of bambuterol hydrochloride (10 mg) oral solution administered once daily in the evening with terbutaline sulphate (0.075 mg/kg body weight) oral solution administered three times daily in 2-5-year-old children with asthma. There were two treatment groups: (2/3)...
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(200003)29:3<194::aid-ppul7
更新日期:2000-03-01 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::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::Eight patients with cystic fibrosis [CF] colonized with Pseudomonas aeruginosa (P. aeruginosa) had serial lung function, peripheral blood inflammatory markers, and serum IgG antibodies to Burkholderia cepacia (B. cepacia) lipopolysaccharide measured in the months preceding and following colonisation with B. cepacia. O...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200001)29:1<8::aid-ppul2>3
更新日期:2000-01-01 00:00:00
abstract:BACKGROUND:Multiple breath washout (MBW) in infants presents a number of technical challenges. Conventional MBW is based on simultaneous measurement of flow and gas concentrations. These two signals are aligned and combined to derive expired gas volumes from which lung volumes and measures of ventilation inhomogeneity ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23348
更新日期:2016-05-01 00:00:00
abstract::The aim of our study was to examine the effect of labor and birth canal compression on the rate of clearance of lung fluid. We recruited 10 babies born by vaginal delivery and 10 born by elective cesarean section (ECS). Thoracic gas volume (TGV) was measured using total body plethysmography, and functional residual ca...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199905)27:5<318::aid-ppul4
更新日期:1999-05-01 00:00:00
abstract::Malacoplakia is an unusual inflammatory condition with distinctive histologic features. Involvement of the lung is quite uncommon and is rarely described in paediatrics. We report on a case of pulmonary malacoplakia in a teenage girl. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10386
更新日期:2003-11-01 00:00:00
abstract::Severe bronchopulmonary dysplasia (BPD) is frequently associated with asthma. The combination is often severe enough to necessitate corticosteroid therapy. There are no commercially available nebulizer solutions of corticosteroids for use in infants and young children. Seven infants and small children with very severe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950130407
更新日期:1992-08-01 00:00:00
abstract:OBJECTIVES:To determine 1) what proportion of abstracts of randomized controlled trials (RCTs) presented at international conferences on cystic fibrosis (CF) are published as full reports, 2) time to publication, and 3) factors that might delay or prevent publication. METHODS:At the end of 1995, the Cochrane CF Group'...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199808)26:2<101::aid-ppul5
更新日期:1998-08-01 00:00:00
abstract::The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was identified in 1989, yet major pathophysiologic questions remain unanswered. There is emerging evidence that CFTR is a bicarbonate channel, a driver of chloride-bicarbonate exchange and through its action on local pH, a regulator o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23247
更新日期:2015-10-01 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:INTRODUCTION:The 2017-2018 National Survey of Children's Health estimates that 30 million (42%) US children have experienced at least one adverse childhood experience (ACE), including abuse, neglect, and household dysfunction. ACEs negatively impact long-term health, and there has been no study of ACEs in cystic fibros...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25243
更新日期:2020-12-26 00:00:00
abstract::Little is known about the respiratory behavior of the intercostal muscles within a neonatal and developmental context. We, therefore, examined intercostal muscle electromyographic (EMG) activity in kittens (1 month old, n = 8; 2 months old, n = 7) during eupnea and heightened respiratory drive induced by hypercapnia. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950050407
更新日期:1988-01-01 00:00:00
abstract::Our aim was to determine whether antenatal corticosteroids improve perinatal adaptation of the pulmonary circulation in lambs with lung hypoplasia (LH). LH was induced in 12 ovine fetuses between 105 and 140 days gestation (term approximately 147 days); in 6 of these the ewe was given a single dose of betamethasone (1...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20453
更新日期:2006-09-01 00:00:00
abstract:OBJECTIVES:We assessed the performance of a tracheostomy decannulation protocol privileging safety over quickness, in pediatric patients undergoing rehabilitation from severe acquired brain injury. We analyzed factors associated with decannulation timing and possibility and examined cases of failure. HYPOTHESIS:A safe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23832
更新日期:2017-11-01 00:00:00