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 care; and discuss future directions. This manuscript complements the plenary address given by Dr Wayne Morgan at the 2016 North American CF Conference by summarizing the key points from the presentation and providing additional detail and information.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Fink AK,Loeffler DR,Marshall BC,Goss CH,Morgan WJdoi
10.1002/ppul.23790subject
Has Abstractpub_date
2017-11-01 00:00:00pages
S44-S51issue
S48eissn
8755-6863issn
1099-0496journal_volume
52pub_type
杂志文章,评审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::The structure, distribution, and frequency of neuroendocrine (NE) cells in human fetal lung from early stages of development to term are described. Neuroendocrine cells were studied by electron microscopy and immunostaining for serotonin and bombesin, recently identified markers of these cells in human lung. The diffe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:
更新日期:1985-05-01 00:00:00
abstract::The quantity and composition of airway surface liquid (ASL) are essential to host defense. To date, attempts to harvest ASL and measure its composition have yielded conflicting results. We investigated the physical principles underlying two techniques that were proposed for harvesting ASL: filter paper pledgets and po...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10409
更新日期:2004-02-01 00:00:00
abstract:INTRODUCTION:Lung disease in cystic fibrosis (CF) begins early in life but the capabilities for detecting abnormalities of pulmonary dysfunction in children remain limited. OBJECTIVE:The study aimed to evaluate the early progression of lung function by the analysis of pulmonary hyperinflation, ventilation inhomogeneit...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25013
更新日期:2020-11-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a complication commonly encountered in patients with CF that produces significant respiratory morbidity. Chronic airway colonization with Aspergillus induces strong inflammatory responses with high IgE levels. Current guidelines for therapy include prolonged courses of...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20804
更新日期:2008-06-01 00:00:00
abstract:INTRODUCTION:Advance care planning (ACP), though recommended, has not been studied in adolescents with cystic fibrosis (CF). This quality improvement project engaged adolescents with advanced CF disease in ACP and assessed patient and CF provider attitudes and preferences regarding ACP discussions and tools. MATERIALS...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23559
更新日期:2016-12-01 00:00:00
abstract:OBJECTIVE:Obstructive sleep apnea/hypopnea syndrome (OSAHS) is a common sleep problem. The aim of this study is to investigate the association between OSAHS and blood pressure (BP) in snoring children. METHODS:Snoring children were recruited from January 2009 to December 2010. Clinical history was taken accompanied by...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22595
更新日期:2013-03-01 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: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::The jet nebulizer is a common device used for administering aerosol medication in young children. However, compared to a metered dose inhaler-spacer (MDI-spacer), it takes more time and personnel. This study aimed to compare the efficacy of salbutamol aerosol therapy given via these two devices in young wheezing child...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.20204
更新日期:2005-05-01 00:00:00
abstract:RATIONALE OF THE STUDY:Increased neonatal fraction of exhaled nitric oxide (FeNO) is associated with lung symptoms early in life, while predictors of neonatal FeNO levels are unknown. The objective of this study was to investigate perinatal and genetic predictors of FeNO in healthy at-risk neonates. METHODS:FeNO was m...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22958
更新日期:2015-02-01 00:00:00
abstract::The occurrence of a tracheoesophageal fistula (TEF) in the setting of lymphoma has only rarely been reported in the world literature. Most cases reported were associated with radiation therapy vs. chemotherapy alone. This report presents one case illustrating the difficulty encountered managing a TEF that developed wh...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20405
更新日期:2006-07-01 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::Until the year 2000, systematic cystic fibrosis (CF) neonatal screening was only performed in a few regions of France. The Brittany region began in 1989, but not the neighboring region of Loire-Atlantique. The present study compares the clinical evolution of both affected populations 10 years after screening was start...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10259
更新日期:2003-05-01 00:00:00
abstract::For the last thirty years, oral chloral hydrate has been used for sedation of infants for lung function testing. Recently, however, availability of chloral hydrate became severely limited in the United States after two manufacturers discontinued manufacturing in 2012. Due to these limitations and the recent and ongoin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23100
更新日期:2015-02-01 00:00:00
abstract:OBJECTIVE:To develop and validate a questionnaire to assess asthma control in children (CAN). DESIGN:Two versions of the CAN (for carers and children) were developed. Both versions were validated in an observational, prospective, multicenter study performed in 38 hospital outpatient clinics throughout Spain. Four hund...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20929
更新日期:2009-01-01 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::Several enzyme-linked immunosorbent assays (ELISAs) based on mycobacterial antigens have been tried for the rapid diagnosis of tuberculosis (TB). In this study, the value of the 16 and 38-kDa mycobacterial antigens in the diagnosis of TB was investigated in pediatric patients in Izmir, Turkey in whom they were found u...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20901
更新日期:2009-09-01 00:00:00
abstract::SUMMARY. Patients with idiopathic scoliosis are reported to have a restrictive pattern on pulmonary function tests. A case is presented of a teenage girl with juvenile idiopathic scoliosis who had evidence of airways obstruction in addition to restriction on pulmonary function tests (PFT). Examination of flow-volume l...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200101)31:1<86::aid-ppul1012>3.0
更新日期:2001-01-01 00:00:00
abstract::Respiratory inductive plethysmography (RIP) is a simple technique for an objective, noninvasive assessment of thoracoabdominal asynchrony, which in turn is an indirect measure of airway obstruction. We evaluated different indices of asynchrony obtained by RIP before and after methacholine-induced airway obstruction. B...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950200610
更新日期:1995-12-01 00:00:00
abstract::Measurements of thoracic gas volume (TGV), airway resistance (Raw), and airway conductance (Gaw) were calculated in a group of 42 normal infants using a whole-body plethysmograph. Maximum expiratory flow at functional residual capacity was measured in a separate group of 108 normal infants. Using data obtained from th...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950140303
更新日期:1992-11-01 00:00:00
abstract::The purpose of the study was to analyze the respiratory-dependent intraarterial blood pressure variations (pulsus paradoxus, PP) in intensive care unit patients with obstructive and restrictive pulmonary disease, and to evaluate the usefulness of PP for assessment of disease severity. One-minute paper recordings of ar...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200102)31:2<138::aid-ppul1022>3.
更新日期:2001-02-01 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 effect of viral infection on nasopharyngeal carriage of Streptococcus pneumoniae during childhood is not well known. We studied dynamics of pneumococcal colonization by quantitative PCR during the natural course of viral bronchiolitis. At time of admission, 47 (47%) of 100 patients with bronchiolitis carried pneum...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23390
更新日期:2016-08-01 00:00:00
abstract::The prognosis for patients with acute respiratory distress syndrome (ARDS) in adults and children has improved since its formal acceptance as a clinical entity in 1967. Because acute hypoxemic respiratory failure is the hallmark of acute lung injury and ARDS, the management of oxygenation is crucial. Physicians managi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.1159
更新日期:2001-12-01 00:00:00
abstract:OBJECTIVE:To determine whether non-invasive positive pressure ventilation (NIPPV) delivered via nasal prongs can alleviate the need for tracheal intubation and invasive ventilation in infants admitted to the pediatric intensive care unit (PICU) with impending respiratory failure, and to find predictive factors for succ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22561
更新日期:2012-10-01 00:00:00
abstract::SUMMARY. The detection of early airway disease in infants with cystic fibrosis (CF) may lead to earlier intervention and an improved prognosis. We hypothesized that the ratio of maximal expiratory flows while breathing a mixture of helium and oxygen (heliox) and air, referred to as density dependence (DD), would ident...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200101)31:1<17::aid-ppul1002>3.0
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:Consolidation on chest radiography is widely used as the reference standard for defining pneumonia and variability in interpretation is well known but not well explored or explained. METHODS:Three pediatric sub-specialists (infectious diseases, radiology and respiratory medicine) viewed 3,033 chest radiogra...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22806
更新日期:2013-12-01 00:00:00
abstract::The airway epithelium represents a primary site for the introduction and deposition of potentially pathogenic microorganisms into the body, through inspired air. The epithelial mucosa is an important component of the innate immune system that recognizes conserved structures in microorganisms and initiates appropriate ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20735
更新日期:2008-01-01 00:00:00
abstract::Pseudomonas aeruginosa is often cultured from the airways of children with tracheostomies. P. aeruginosa produces exotoxin A (ETA) and type III cytotoxins. This study tested the hypothesis that children with tracheostomies are colonized by P. aeruginosa that express these virulence factors and will have antibodies dir...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20194
更新日期:2005-05-01 00:00:00