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 clinical trials network, to be followed by all sites performing collaborative studies. Key variables in the measurement included type of voltmeter, exploring probe, reference electrodes, and solutions used to assess both sodium transport and chloride conductance. Eight sites submitted data on 3-8 normal and 4-5 CF subjects. Baseline voltage, an index of sodium transport, was -18.2 +/- 8.3 mV (mean +/- SD) for normals, and -45.3 +/- 11.4 mV for CF patients. There was no CFTR-mediated chloride secretion in CF subjects, as evidenced by the lack of response to perfusion with zero chloride + beta agonist solutions (+3.2 +/- 3.5 mV) vs. that in normals (-23.7 +/- 10.2 mV). The standardized nasal potential difference measurement minimizes variability between operators and study sites. Valid and consistent results can be attained with trained operators and attention to technical details. These data demonstrate the procedure to be sufficient for multicenter studies in the CF Foundation network.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Standaert TA,Boitano L,Emerson J,Milgram LJ,Konstan MW,Hunter J,Berclaz PY,Brass L,Zeitlin PL,Hammond K,Davies Z,Foy C,Noone PG,Knowles MRdoi
10.1002/ppul.10448subject
Has Abstractpub_date
2004-05-01 00:00:00pages
385-92issue
5eissn
8755-6863issn
1099-0496journal_volume
37pub_type
杂志文章,多中心研究abstract::Pulmonary hemorrhage (PH) is a serious complication causing acute respiratory distress in the premature infant, and it is associated with significant mortality and morbidity. The role of inflammatory mediators in this condition is largely undefined. Serial tracheal aspirates (TA) were obtained at intervals from 65 mec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10141
更新日期:2002-08-01 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::Respiratory symptoms, atopy, and bronchial reactivity were measured in primary school children living in Lake Munmorah (LM), a coastal town near two power stations, and in Nelson Bay (NB), a coastal town free from any possible major sources of outdoor air pollution. A prevalence survey and longitudinal follow-up study...
journal_title:Pediatric pulmonology
pub_type: 评论,杂志文章
doi:10.1002/ppul.1950110209
更新日期:1991-01-01 00:00:00
abstract::Pneumatoceles and bronchiectasis are secondary complications of respiratory infections in patients with hyper IgE syndrome (HIgES). We report on a patient with HIgES and recurrent pneumonias since 1 year of age, with progression to pneumatoceles and bronchiectasis and fatal outcome, with disseminated infection and sus...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22532
更新日期:2013-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20854
更新日期:2009-03-01 00:00:00
abstract::This review summarizes current knowledge about the role of nitric oxide (NO) in cystic fibrosis (CF) lung disease. NO is endogenously produced by a group of enzymes, the NO synthases (NOSs). There are three isoforms of NOS, each encoded by different genes: neuronal (nNOS), immune or inducible (iNOS), and endothelial (...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1099-0496(199912)28:6<442::aid-ppul1
更新日期:1999-12-01 00:00:00
abstract::The aim of our study was to determine the effects of pulmonary vascular engorgement on airways and pulmonary tissues in juvenile animals before and after methacholine (Mch)-induced changes in lung function. Five anesthetized, paralyzed, and thoracotomized piglets were studied before and during pulmonary vascular engor...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199801)25:1<45::aid-ppul5>
更新日期:1998-01-01 00:00:00
abstract:BACKGROUND/AIM:There has been recent interest in dry powder inhaled mannitol as a therapeutic agent in patients with cystic fibrosis (CF). It is has been shown to increase mucociliary clearance (MCC) by rehydrating the airway. To date there have been no studies exclusively in children with CF examining the effect of dr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20903
更新日期:2008-11-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10149
更新日期:2002-07-01 00:00:00
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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
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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: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::We assessed the ability of innovative lung function tests to detect bronchial obstruction induced by methacholine bronchial challenge. Fifty-five recurrently wheezy infants (mean age 16 +/- 5.2 months) free of respiratory symptoms underwent baseline lung function tests. Forty-two completed the methacholine challenge. ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950180508
更新日期:1994-11-01 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::Appropriate, regular exercise is of benefit to patients with cystic fibrosis (CF). As with other segments of the population, it has been difficult to devise exercise programs to which most patients will adhere for long periods of time. In healthy children, factors that are related to positive exercise compliance inclu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.10126
更新日期:2002-07-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24462
更新日期:2019-11-01 00:00:00
abstract::To determine the natural history of lung vascular remodeling and cardiac changes in the rat model of persistent pulmonary hypertension syndrome (PPHN) of the newborn, we studied fetal rats subjected to maternal indomethacin administration initiated on day 19 of gestation and continued for 2, 3, or 4 days. Animals rece...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1057
更新日期:2001-05-01 00:00:00
abstract::The assessment of apnea and asynchronous breathing requires the application of a facemask connected to a pneumotachograph and inductive transducer bands placed around the chest wall. These contact devices may alter the breathing pattern and are difficult to implement, especially in infants and children. This study val...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21570
更新日期:2012-05-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.20909
更新日期:2008-11-01 00:00:00
abstract::In a double-blind, randomized trial, we compared the effects of aerosolized metaproterenol to subcutaneous epinephrine in 35 episodes of acute asthma in children between 6 and 19 years of age. Patients were randomized to two parallel groups and then received both a placebo medication and an active medication to a maxi...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1986-07-01 00:00:00
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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
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23434
更新日期:2016-10-01 00:00:00
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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::To determine whether doses of ipratropium bromide (IB) greater than those usually administered by aerosol (75-250 micrograms) give a greater degree of protection from exercise-induced asthma (EIA) in children, 12 patients with chronic asthma, ages 7-13 yr, were challenged with methacholine and exercise after inhalatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060205
更新日期:1989-01-01 00:00:00
abstract:BACKGROUND:Quantifying the chest wall is useful in documenting thoracoabdominal synchrony during the neonatal period. Subjective measures are often used rather than gold-standard methods due to their practicality in clinical practice. The aim of the present study is to compare the reliability between a newly proposed m...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24709
更新日期:2020-05-01 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::The objective of this study was to determine the neurodevelopmental and temperamental outcome of infants who suffered an idiopathic apparent life-threatening event (IALTE) and their mothers' perceptions of such an event, and to compare such infants with a matched group of babies hospitalized for nonthreatening events....
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/(sici)1099-0496(199907)28:1<47::aid-ppul8>
更新日期:1999-07-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22558
更新日期:2012-10-01 00:00:00
abstract:RATIONALE:Asthma is one of the most common chronic disorders of childhood and is associated with significant healthcare utilization and costs. Comorbid psychiatric illnesses, specifically depression and anxiety, are more prevalent in patients with asthma and associated with worse asthma control, more emergency departme...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25061
更新日期:2020-11-01 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