Abstract:
INTRODUCTION:Exercise ventilation efficiency index in cardiopulmonary exercise testing (CPET) is elevated in patients with heart failure providing useful information on disease progression and prognosis. Few data, however, exist for ventilation efficiency index among cystic fibrosis (CF) patients. AIMS:To assess ventilation efficiency index (ΔVE/ΔVCO2 or V'E/V'CO2 slope) and intercept of ventilation (VE-intercept) in CF patients with mild, moderate, and severe cystic fibrosis (CF) lung disease. To assess possible correlations with ventilation inhomogeneity and structural damages as seen on high resolution computed tomography (HRCT). METHODS:CF patients with mild (FEV1 > 80%, n = 47), moderate (60% < FEV1 < 80%, n = 21), and severe (FEV1 < 60%, n = 9) lung disease, mean age 14.9 years participated. Peak oxygen uptake (VO2 peak), pulmonary ventilation at peak exercise (VE), respiratory equivalent ratios for oxygen and carbon dioxide at peak exercise (VE/VO2 , VE/VCO2 ), end-tidal CO2 (PetCO2 ), and ΔVE/ΔVCO2 , ΔVE/ΔVO2 in a maximal CPET along with spirometry and multiple breath washout indices were examined. HRCT scans were performed and scored using Bhalla score. RESULTS:Mean ΔVE/ΔVCO2 showed no significant differences among the three groups (P = .503). Mean VEint discriminated significantly among the different groups (p 2 < 0.001). Ventilation efficiency index did not correlate either with LCI or Bhalla score. However, VE together with ΔVE/ΔVCO2 slope could predict Bhalla score (r 2 = 0.869, P = .006). CONCLUSION:No significant differences were found regarding ΔVE/ΔVCO2 slope levels between the three groups. Ventilation intercept (VEint ) was elevated significantly as disease progresses reflecting increased dead space ventilation. CF patients retain their ventilation efficiency to exercise even as lung function deteriorates by adopting a higher respiratory rate along with increased dead space ventilation.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Kampouras A,Hatziagorou E,Avramidou V,Georgopoulou V,Kirvassilis F,Hebestreit H,Tsanakas Jdoi
10.1002/ppul.24438subject
Has Abstractpub_date
2019-10-01 00:00:00pages
1584-1590issue
10eissn
8755-6863issn
1099-0496journal_volume
54pub_type
杂志文章abstract:INTRODUCTION:In moderate-severe asthma exacerbation, salbutamol by inhaler (MDI) is superior to salbutamol delivered by nebulizer (NEB); however, to our knowledge, no studies in children with exclusively severe exacerbations were performed. OBJECTIVE:To compare the efficacy of salbutamol and ipratropium bromide by MDI...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24244
更新日期:2019-04-01 00:00:00
abstract:BACKGROUND:Hypoxia and reactive oxygen species (ROS) including H(2)O(2) play major roles in triggering and progression of pulmonary vascular remodeling in persistent pulmonary hypertension. Catalase (CAT), the major endogenous enzyme scavenging H(2)O(2), is regulated in a tissue- and context-specific manner. OBJECTIVE...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22919
更新日期:2014-09-01 00:00:00
abstract::Home mattresses of 24 asthmatic children with house dust mite allergy were sprayed with either benzyl-benzoate foam or placebo in a double blind fashion, 10 days before the children left the residential house for asthmatic children Istituto Pio XII (located in the Italian Alps in an environment free of mites) and went...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1950180405
更新日期:1994-10-01 00:00:00
abstract::In this article, we describe existing CF registries with a focus on US registry data collected through the CF Foundation Patient Registry (CFFPR) and the Epidemiologic Study of CF (ESCF); highlight what registries have taught us regarding epidemiology of CF; showcase the impact of registries on research and clinical c...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23790
更新日期:2017-11-01 00:00:00
abstract::Drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a rare and severe side-effect, mainly described after intake of anticonvulsants, allopurinol, or antibiotics. It usually begins within 2 months after drug introduction. Symptoms include cutaneous rash, hematologic abnormalities, and internal orga...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23567
更新日期:2017-04-01 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::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::Respiration rate is an important indicator of a person's health, and thus it is monitored when performing clinical evaluations. There are different approaches for respiration monitoring, but generally they can be classed as contact or noncontact. For contact methods, the sensing device (or part of the instrument conta...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.21416
更新日期:2011-06-01 00:00:00
abstract::Tracheobronchomegaly (Mounier-Kuhn syndrome) is characterized by dilatation of the central airways, tracheobronchial outpouchings, and chronic tracheobronchitis. Most cases are diagnosed in adulthood. We report the clinical, radiographic, and bronchoscopic findings in a 14-year-old boy with tracheobronchomegaly, ptosi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200006)29:6<476::aid-ppul1
更新日期:2000-06-01 00:00:00
abstract::A 14-year-old Korean boy was admitted with cough, hemoptysis, and fever. A chest X-ray showed a solitary pulmonary mass and pneumonitis. Bronchial biopsy by fiberoptic bronchoscopy revealed a poorly differentiated small cell carcinoma. All of the staging information indicated that the patient had limited disease. Duri...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200004)29:4<317::aid-ppul1
更新日期:2000-04-01 00:00:00
abstract::Atrial natriuretic peptide (ANP) has known natriuretic, diuretic, and vasodilatatory effects. It is synthesized and stored in the atrial cells. Stretching of the atrial muscle fibers during an increase in venous return sets a response of ANP release into the blood stream. High levels of ANP were measured in a number o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20233
更新日期:2005-10-01 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::To determine the extent of pulmonary dysfunction following primary closure of an abdominal wall defect, we obtained pulmonary function tests (PFT) in 11 newborn infants with gastroschisis and 6 with large omphaloceles admitted to a newborn ICU in a children's hospital. Patients were 1 to 30 days of age at the time of ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120309
更新日期:1992-03-01 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:INTRODUCTION:The efficacy of nebulized hypertonic saline (HS) therapy for shortening hospital length of stay (LOS) or improving bronchiolitic symptoms remains controversial. Most studies enrolled small numbers of subjects and did not consider the role of respiratory syncytial virus (RSV), the most common cause of acute...
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.23945
更新日期:2018-03-01 00:00:00
abstract::Patients with cystic fibrosis (CF) can be discriminated from healthy subjects by measurement of the nasal potential difference, which has become a useful outcome measure for therapies directed toward correcting defective electrolyte transport in CF. A standard operating procedure was developed by a CF Foundation clini...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.10448
更新日期:2004-05-01 00:00:00
abstract::The clinical course of sarcoidosis in children has not been well defined. Eight children with symptomatic sarcoidosis included in this study underwent pulmonary function tests and bronchoscopy with bronchoalveolar lavage (BAL) before treatment and during steroid therapy. At the start of therapy, functional parameters,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950160109
更新日期:1993-07-01 00:00:00
abstract::While the use of the raised volume rapid thoraco-abdominal compression (RVRTC) technique has been shown to provide new insights into airway and pulmonary pathophysiology in infants, and appears to resemble the spirometric techniques used in older subjects, there is as yet no consensus regarding measurement procedures,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10170
更新日期:2002-11-01 00:00:00
abstract:OBJECTIVE:To determine the interpretative consequences of adopting the Global Lungs 2012 (GLI-2012) spirometric prediction equations in a pediatric hospital population. MATERIAL:Spirometric records from 2,192 white boys and 1,842 white girls, and 412 and 334 African-American boys and girls, respectively, aged 6.0-18.0...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22876
更新日期:2014-02-01 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::Some studies have suggested that lung clearance index (LCI) is age-independent among healthy subjects early in life, which implies that ventilation distribution does not vary with growth. However, other studies of older children and adolescents suggest that ventilation becomes more homogenous with somatic growth. We d...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21553
更新日期:2012-04-01 00:00:00
abstract:BACKGROUND:'Profound intellectual and multiple disability' (PIMD) is defined as a profound cognitive disability with severe sensory and motor impairments. The aim of this study was to evaluate the respiratory morbidity in children with PIMD and investigate possible risk factors. METHODS:In 10 specialized facilities fo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23114
更新日期:2015-10-01 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::The current bronchopulmonary dysplasia (BPD) is seen in infants born extremely premature, with less severe respiratory distress syndrome (RDS) and who received prenatal steroids-"new BPD". The pathophysiology of BPD is based on an impairment of lung maturation with prenatal and postnatal multi-hit insults and genetic ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.21508
更新日期:2011-12-01 00:00:00
abstract::Pertussis carries a high risk of mortality in very young infants. The mechanism of refractory cardio-respiratory failure is complex and not clearly delineated. We aimed to examine the clinico-pathological features and suggest how they may be related to outcome, by multi-center review of clinical records and post-morte...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.21082
更新日期:2009-10-01 00:00:00
abstract:OBJECTIVES:We evaluated safety and efficacy of recombinant human growth hormone (rhGH) for improving growth, lean body mass (LBM), pulmonary function, and exercise tolerance in children with cystic fibrosis (CF) and growth restriction. STUDY DESIGN:Multicenter, open-label, controlled clinical trial comparing outcomes ...
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.21546
更新日期:2012-03-01 00:00:00
abstract::Ten children with mild bronchial asthma underwent bronchial challenge with carbachol on three consecutive days; using a double-blind technique, they were given inhaled saline placebo on the first day and placebo or sodium cromoglycate (SCG) on the second and third days 15 min prior to the bronchial provocation test wi...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950030309
更新日期:1987-05-01 00:00:00
abstract::In children, pulmonary sequelae contribute to early and late morbidity after bone marrow transplantation (BMT). Between 1975-1999, we performed 152 BMTs in 138 pediatric patients with malignant and nonmalignant diseases. Allogenic bone marrow was used from 99 HLA identical siblings and from 23 other related or unrelat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200011)30:5<393::aid-ppul5>3.0.c
更新日期:2000-11-01 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:OBJECTIVES:The present pilot study was performed to evaluate the HPA axis and ANS activity by measuring salivary cortisol and α-amylase diurnal trajectory and production, respectively, in mild or moderate-to-severe (MS) OSA-affected, but otherwise healthy, children. Moreover, a correlative analysis was performed betwee...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22972
更新日期:2014-11-01 00:00:00