Abstract:
:Progressive lung disease in patients with cystic fibrosis (CF) is caused by thick secretions, which cause airway obstruction and subsequent colonization and infection by inhaled pathogenic microorganisms. Recently, recombinant human DNase has been shown to reduce the viscoelasticity of sputum in patients with cystic fibrosis and to improve lung function. Ultrasonically nebulized hypertonic saline (HS) has been demonstrated to enhance mucociliary clearance and sputum expectoration by rehydrating airway secretions, and may therefore provide a low cost alternative. We studied the changes in pulmonary function and symptoms in a group of patients with CF who have moderate to severe lung disease. The patients were evaluated following 2 weeks of treatment with HS in an open-label study. Subjects were randomly allocated to receive 10 ml of either 0.9% NaCl (IS) or 6% NaCl (HS). Twice daily, prior to physiotherapy, treatments were delivered by a portable ultrasonic nebulizer. To prevent bronchoconstriction, 600 mg of salbutamol was administered prior to the nebulized solutions. A symptom score was recorded and spirometry was performed on day 0 before therapy was started, on day 14 (the last day of therapy), and on day 28 (14 days after the last treatment with either IS or HS). Fifty-two patients (32 males), with a mean age of 16.2 (range 7-36) years completed the study. There was no difference in baseline characteristics between the two groups. Following 2 weeks of treatment, there was a significant improvement from baseline in FEV1 of 15.0 +/- 16.0% (mean +/- SD) in patients treated with HS, compared with a change of 2.8 +/- 13% in those on IS therapy (P = 0.004). Furthermore, there was a subjective improvement in the effectiveness of chest physiotherapy as reported by those using HS (P = 0.02). The treatment was well tolerated. We conclude that in patients with CF, ultrasonically nebulized hypertonic saline improves lung function in a way similar to that reported for human recombinant DNase when inhaled over a 2 week period. Nebulized saline also enhances the perception of effectiveness of chest physiotherapy.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Eng PA,Morton J,Douglass JA,Riedler J,Wilson J,Robertson CFdoi
10.1002/(SICI)1099-0496(199602)21:2<77::AID-PPUL3>subject
Has Abstractpub_date
1996-02-01 00:00:00pages
77-83issue
2eissn
8755-6863issn
1099-0496pii
10.1002/(SICI)1099-0496(199602)21:2<77::AID-PPUL3>journal_volume
21pub_type
临床试验,杂志文章,多中心研究,随机对照试验abstract::Respiratory input impedance (Zin) is a potentially informative test of pulmonary function in infants who are unable to perform standard tests commonly performed in children and adults Analysis of Zin in dogs using the six-element model of DuBois et al. (J Appl Physiol 8:587, 1956) provides estimates of airways resista...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199612)22:6<364::AID-PPUL5
更新日期:1996-12-01 00:00:00
abstract::To promote early screening of patients with suspected Primary Ciliary Dyskinesia (PCD), nasal nitric oxide (nNO) measurements during tidal breathing (TB) have been developed for children unable to ensure velum closure (VC) during breath hold or expiration against resistance. To investigate technical and practical issu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.23167
更新日期:2015-12-01 00:00:00
abstract::During the final prenatal period of fetal lung development in humans, important maturational processes occur, including the production of surfactant necessary to decrease surface tension at the air-liquid interface of the alveoli. During early gestation, the glucocorticoid receptor is expressed in the fetal lung, and ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.1092
更新日期:2001-07-01 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: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::Sleep disordered breathing (SDB) represents a spectrum of breathing disorders, ranging from snoring to obstructive sleep apnea syndrome (OSAS), that disrupt nocturnal respiration and sleep architecture. OSAS is a common disorder in children, with a prevalence of 2-3%. It is associated with neurobehavioral, cognitive, ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20943
更新日期:2008-12-01 00:00:00
abstract:INTRODUCTION:The prevalence of both childhood obesity and childhood asthma has increased dramatically over the past few decades. Little is known concerning the role of body composition and lifestyle influences on airway health in children. PURPOSE:To determine whether body composition, fruit and vegetable intake (FV) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21393
更新日期:2011-05-01 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::Diffuse panbronchiolitis is a progressive fatal respiratory disease of unknown cause seen predominantly in Southeast Asian adults. We report this condition in a 10-year-old child of Korean birth because of the uncommon presentation at this age and the favorable outcome associated with early diagnosis. Our objective wa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23176
更新日期:2015-09-01 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::We recently reported that prenatal glucocorticoid therapy is less effective at promoting an improvement in lung function in male than in female sheep. This observation, and the higher incidence of respiratory distress syndrome in human males, suggests that the male fetal lung may be less responsive to glucocorticoids ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1082
更新日期:2001-07-01 00:00:00
abstract:BACKGROUND:Chest computerized tomography (CT) scores are associated with the frequency of future pulmonary exacerbations in people with cystic fibrosis (CF). However, cut-off values to identify children with mild lung disease with different risks for frequent future pulmonary exacerbations have not been identified. ME...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24144
更新日期:2018-10-01 00:00:00
abstract:OBJECTIVE:To define the mortality and long-term outcomes of children undergoing tracheostomy. DESIGN:Retrospective chart and Texas Department of Health Bureau of Vital Statistics review of patients admitted to a Pediatric Intensive Care Unit who underwent a tracheostomy between 2001 and 2011. Mortality and decannulati...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23657
更新日期:2017-07-01 00:00:00
abstract::The impulse oscillometry system (IOS) was introduced as a new technique to assess airflow obstruction in patients who are not able to perform forced breathing maneuvers, e.g., subjects with cerebral palsy or severe mental retardation, and young children. This study evaluates the sensitivity and specificity of IOS para...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10235
更新日期:2003-03-01 00:00:00
abstract::Ten infants with evidence of impending respiratory failure from severe bronchiolitis were successfully treated with continuous positive airway pressure (NCPAP) with double nasal prongs. Their mean (SD) age was 6.7 (3.8) months and mean (SD) body weight was 7.1 (2.1) kg. Respiratory assessments were made immediately be...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950160305
更新日期:1993-09-01 00:00:00
abstract:UNLABELLED:The main goal of the study was to assess possible association between transplacental exposure to genotoxic PAH compounds assessed by the cord blood PAH-DNA adducts and fractional exhaled nitric oxide (FeNO) measured in healthy non-asthmatic children at the age of 7 years. The subjects included the subsample ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22570
更新日期:2012-11-01 00:00:00
abstract::Bronchoalveolar lavage (BAL) performed with a fiberoptic bronchoscope (FOB) is a useful method for sampling alveolar contents. Since the smallest FOB with a channel has a diameter of 3.6 mm, BAL is difficult to accomplish through artificial airways (AA) less than 5.0 mm I.D. We used a 4F balloon wedge pressure cathete...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950130110
更新日期:1992-05-01 00:00:00
abstract:BACKGROUND:sTREM-1 (soluble triggering receptor expressed on myeloid cells-1) is a novel inflammatory marker that may be of clinical use in cystic fibrosis (CF). Dysregulation of the TREM pathway has been demonstrated in other inflammatory diseases and modulation in animal models has therapeutic benefit. We hypothesise...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23650
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:Child exposure to cigarette smoke is harmful. It should be reduced through parental smoking cessation interventions. The aim of our study was to determine the impact of simple advice provided by the pediatrician on the smoking habits of parents of children with cystic fibrosis (CF), diabetes mellitus (DM) an...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25277
更新日期:2021-01-22 00:00:00
abstract::Prader-Willi syndrome (PWS) is a genetic disorder, with hypotonia being the predominant feature in infancy, and developmental delay, obesity, and behavioral problems becoming more prominent in childhood and adolescence. Children with this disorder frequently suffer from excessive daytime sleepiness and have a primary ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.10152
更新日期:2002-09-01 00:00:00
abstract::The gasdermin A (GSDMA) and gasdermin B (GSDMB) genes are located at 17q21.2. The GSDM family genes have been studied in the gastrointestinal tract but recent reports suggest that GSDMB is associated with childhood asthma in several populations. We investigated the association of the GSDMA and GSDMB variants with asth...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21424
更新日期:2011-07-01 00:00:00
abstract::In contrast to its effect on airway smooth muscle in the adult, in vitro studies have shown that caffeine significantly increases active tension in airway smooth muscle in the neonatal lamb. To determine if caffeine has a physiological effect on airway function during early development, we studied the effect of caffei...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120106
更新日期:1992-01-01 00:00:00
abstract::Respiratory system compliance (Crs) can be used to assess lung stiffness in sick, intubated infants, avoiding the use of an esophageal balloon. Crs was assessed in a group of 15 sick, intubated infants using the occlusion and inflation techniques. The occlusion technique gave satisfactory results in 13 infants. Apneic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030207
更新日期:1987-03-01 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::Maternal retinoid administration has beneficial effects on lung development in the nitrofen rodent toxic model of congenital diaphragmatic hernia (DH). We wanted to investigate the effects in a surgical model, where the retinoid signaling pathway is not primarily disrupted by the toxic agent. We created DH in fetal ra...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20829
更新日期:2008-06-01 00:00:00
abstract::Our objective was to determine the repeatability of bronchial responsiveness to mannitol dry powder (MDP) as an objective marker of asthma in children. MDP challenge was performed in children with stable asthma at the same time of the day on two separate occasions within a week. The test was terminated after a 15% fal...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.10378
更新日期:2003-12-01 00:00:00
abstract:BACKGROUND:How pediatricians manage bronchiolitis and the derived total costs (direct and indirect) in the emergency department (ED) have not been fully characterized. The aim of the present study is to calculate those costs in a European country. METHODS:A prospective and observational study, including 10 EDs of tert...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.22906
更新日期:2014-10-01 00:00:00
abstract::To develop and validate a questionnaire scale that can be used as a screening tool to investigate for the presence of childhood obstructive sleep apnea syndrome (OSAS) in Hong Kong Chinese children. Subjects suspected to have OSAS and controls were recruited. Parents completed a Hong Kong children sleep questionnaire ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20505
更新日期:2006-12-01 00:00:00
abstract:BACKGROUND:Chronic colonization with Pseudomonas (P.) aeruginosa worsens the prognosis of cystic fibrosis (CF) patients. This study aims to analyze the functional properties of neutrophils in CF patients with P. aeruginosa colonization. METHODS:Patients with CF (n = 16) were grouped by positivity of P. aeruginosa in s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25294
更新日期:2021-01-29 00:00:00
abstract::Wheezing is the most widely reported adventitious lung sound in the English language. It is recognized by health professionals as well as by lay people, although often with a different meaning. Wheezing is an indicator of airway obstruction and therefore of interest particularly for the assessment of young children an...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23930
更新日期:2018-02-01 00:00:00