Abstract:
OBJECTIVES:Forced expiratory volume in 1 second (FEV1) is an important predictor of outcome in cystic fibrosis (CF). This study aimed to describe the change in lung function, nutritional status, and mortality of children with CF at a single center in Cape Town, South Africa, and identify factors associated with poor lung function and mortality. METHODS:A retrospective study was conducted of children aged between 5 and 18 years between January 2007 and December 2016. At least two separate best annual FEV1 measurements were required for inclusion in the study. RESULTS:A total of 143 children were followed up from which 107 study participants (median diagnosis age 5.5 months) were included. There was no statistically significant improvement from 2007 to 2016 in population mean FEV1 (2.5 ± 1.70 to -1.9 ± 1.70 [P = .1]) and body mass index (-0.7 ± 1.2 to -0.4 ± 1.2 [P = .3]) Z scores. FEV1 Z score declined by 0.17 per year. No significant correlation between FEV1 and age of diagnosis, sex, ethnicity, genotype, geographical location, pancreatic status, or infections was identified. On multiple stepwise regression analysis, FEV1 at age 6 was found to be the only independent predictor of mortality (adjusted odds ratio [95% CI] 0.5 [0.3-0.8]; P = .005). CONCLUSION:FEV1 at age 6 was an independent predictor for CF-related mortality. Measurement of lung function in preschool children in SA with CF using more sensitive methods than spirometry is important to identify children at risk of poor outcomes.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Vandenbroucke NJ,Zampoli M,Morrow Bdoi
10.1002/ppul.24726subject
Has Abstractpub_date
2020-06-01 00:00:00pages
1381-1387issue
6eissn
8755-6863issn
1099-0496journal_volume
55pub_type
杂志文章abstract:BACKGROUND:Patient-reported outcomes (PROs) are increasingly used to evaluate the efficacy of new treatments and the progression of chronic diseases. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) is a disease-specific, PRO measure of health-related quality of life (HRQOL). We evaluated associations between changes ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21325
更新日期:2011-01-01 00:00:00
abstract:OBJECTIVE:Since preventive therapies for bronchopulmonary dysplasia (BPD) are limited we treated preterm infants with azithromycin to decrease the incidence of BPD. METHODS:Infants less than 1,250 g birth weight were randomized to azithromycin or placebo within 12 hr of beginning mechanical ventilation and within 72 h...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21352
更新日期:2011-02-01 00:00:00
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::Viral colds are an important cause of respiratory symptoms in normal children. Studies in adults suggest that inflammation in the lower respiratory tract is associated with viral colds, but there are no data regarding inflammation and viral infection in the lower airway of normal children with colds. We, therefore, st...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199908)28:2<109::aid-ppul6
更新日期:1999-08-01 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:RATIONALE:Nitric oxide (NO) produced in the lung is an important mediator of normal lung development, vascular smooth muscle relaxation, and ventilation perfusion matching. NO is synthesized from arginine by the action of NO-synthase (NOS). Asymmetric dimethylarginine (ADMA), an endogenous derivate of arginine, inhibit...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20886
更新日期:2008-12-01 00:00:00
abstract::The incidence of necrotizing pneumonia and empyema complicated by bronchopleural fistula is rising. We describe the case of a 2-year-old boy who presented with empyema thoracis and necrotizing pneumonia who developed a bronchopleural fistula. At initial thoracotomy for decortication, necrotic lung was found and resect...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22649
更新日期:2013-06-01 00:00:00
abstract::Inadequate intake and suboptimal growth are common problems for patients with CF and a critical target for intervention. The purpose of this study was to compare the growth outcomes of children with CF who participated in a randomized clinical trial to improve energy intake and weight to children with CF receiving sta...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21322
更新日期:2011-01-01 00:00:00
abstract::We used an in vitro model system to examine the sites of deposition of aqueous therapeutic aerosols administered through 3-mm, 6-mm, and 9-mm endotracheal tubes (commonly used in infants, children, and adults) at clinically relevant inspiratory flow rates. Aerosol was delivered to the endotracheal tube via a "T" piece...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020108
更新日期:1986-01-01 00:00:00
abstract::Aspiration is common in the intubated human neonate. Thus, the ventilatory and blood gas responses to citric acid and saline instillation into different airway sites were studied in ten awake, unanesthetised lambs, breathing spontaneously via a tracheostomy tube. With a system of balloons, 1 ml of saline or citric aci...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030112
更新日期:1987-01-01 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::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: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::Polyvinyl chloride (PVC) nasogastric feeding catheters are used clinically to measure intraesophageal pressure as an estimate of pleural pressure for calculating lung compliance in infants. The accuracy of pressure measurement of 4 French gauge (FG) catheter sizes and three brands of liquid-filled catheter manometer s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199705)23:5<362::aid-ppul8
更新日期:1997-05-01 00:00:00
abstract:BACKGROUND:Matrix metalloproteinase-9 (MMP-9) plasma levels correlate with C-reactive protein (CRP) concentrations and they are both increased in adults with obstructive sleep apnea (OSA). No studies have evaluated MMP-9 levels in children with sleep apnea and CRP is not consistently elevated in pediatric OSA. The aim ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21251
更新日期:2010-07-01 00:00:00
abstract::There is a need to judge general exercise tolerance in children with cystic fibrosis (CF) under normal daily activity conditions and -when more extensive testing is required-in an exercise laboratory in a specialized center. We investigated the reproducibility, validity, and criterion for a 6-minute walking test, whic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199608)22:2<85::AID-PPUL1>
更新日期:1996-08-01 00:00:00
abstract:BACKGROUND:There is minimal literature available on the long-term outcome of pediatric non-cystic fibrosis (CF) bronchiectasis. AIM:To document 5-year outcomes of children with chest computerized tomography (CT) scan diagnosed bronchiectasis from a tertiary New Zealand (NZ) respiratory clinic. METHODS:Review of a cli...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21331
更新日期:2011-02-01 00:00:00
abstract::Respiratory syncytial virus (RSV) infection can be severe in pediatric patients. Risk factors for severe disease include age less than 6 months, prematurity, preexisting heart or lung disease or malformations, gastroesophageal reflux, and immunodeficiency. The aim of the present study was to investigate the influence ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200010)30:4<302::aid-ppul5>3.0.c
更新日期:2000-10-01 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::Previous research has demonstrated a significant reciprocal relationship between psychosocial factors and asthma morbidity in children. The National Cooperative Inner-City Asthma Study investigated both asthma-specific and non-specific psychosocial variables, including asthma knowledge beliefs and management behavior,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/(sici)1099-0496(199710)24:4<263::aid-ppul5
更新日期:1997-10-01 00:00:00
abstract::The introduction of NBS in Ireland in July 2011, provided a unique opportunity to investigate clinical outcomes using a comparative historical cohort study. Clinical cohort: children clinically diagnosed with CF born 1 July 2008 to 30 June 2011, and NBS cohort: children diagnosed with CF through NBS born 1 July 2011 t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24876
更新日期:2020-09-01 00:00:00
abstract::We report on a 1-month-old boy who presented with a "horseshoe" lung, complete "O" rings of the trachea, and an unusual course of the left pulmonary artery mimicking a left pulmonary artery sling. Computed tomography, cardiac catheterization, and bronchoscopy were performed to confirm the rare coexistence of these ano...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10172
更新日期:2002-11-01 00:00:00
abstract::Pneumocyte apoptosis is implicated in the pathophysiology of acute inflammatory lung injuries in newborns and adults. Pulmonary angiotensin (ANG) II contributes to lung epithelial apoptosis in vitro, but its role in acute lung injury in vivo is unclear. We therefore studied the effects of ANG II receptor action on the...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20187
更新日期:2005-04-01 00:00:00
abstract::Previously, there was insufficient evidence to confirm that pneumonia in infants and children might lead to the development of pulmonary hypertension. Recently, it has been shown that acceleration time corrected for heart rate (ATc) and the ratios of right ventricular preejection period to right ventricular ejection t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950100413
更新日期:1991-01-01 00:00:00
abstract::We studied the left lung using multi-focus microphotography in 378 rat fetuses, assessing airway branching from day 13 to day 19 of gestation, and lung growth variables from day 13 to day 21. Longitudinal growth, and monopodial and dichotomous branching brought about a consistent airway pattern with variations within ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199604)21:4<219::AID-PPUL3
更新日期:1996-04-01 00:00:00
abstract:BACKGROUND:Mesenchymal stromal cell (MSC)-mediated therapeutic effects have been observed in the treatment of lung diseases. For the first time, this treatment was used as rescue therapy in a pediatric patient with a life-threatening respiratory syndrome associated with the filamin A (FLNA) gene mutation. METHODS:A ch...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24497
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM:The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHODS:Participants underwe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23477
更新日期:2017-01-01 00:00:00
abstract::Aerosolized recombinant human DNase (dornase alfa) reduces mucus viscoelasticity in vitro and improves pulmonary function in patients with cystic fibrosis (CF). We postulated that if dornase alfa could be delivered more peripherally to small airways in the lung in the form of smaller aerosol droplets in patients with ...
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(199802)25:2<83::aid-ppul2>
更新日期:1998-02-01 00:00:00
abstract::We studied the effect of low-protein diet (8% casein) on lung growth in rats from 3 to 7 weeks of age. Their diet was isocaloric with that of control animals fed a diet of 20% casein. The calorie intake of experimental animals was increased during the first 3 weeks of the experiment, but they increased less (about 10%...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950070308
更新日期:1989-01-01 00:00:00
abstract:BACKGROUND:Social and family factors may influence the probability of achieving asthma control in children. Parents' quality of life has been insufficiently explored as a predictive factor linked to the probability of achieving disease control in asthmatic children. OBJECTIVE:Determine whether the parents' quality of ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23336
更新日期:2016-07-01 00:00:00