Abstract:
:The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was identified in 1989, yet major pathophysiologic questions remain unanswered. There is emerging evidence that CFTR is a bicarbonate channel, a driver of chloride-bicarbonate exchange and through its action on local pH, a regulator of other ion channels and of proteins that function optimally in a neutral environment. In both the respiratory and gastrointestinal (GI) tracts, bicarbonate drives ionic content and fluid on epithelial surfaces, allows mucins to unfold and become slippery, and contributes to innate immunity. In the GI tract bicarbonate neutralizes gastric acid to support digestion and absorption. When CFTR is dysfunctional, lack of bicarbonate secretion disrupts these normal processes and thus leads directly to the clinical symptoms and signs of CF. This article synthesizes evidence from cell, animal, and human investigations that support these concepts. Bicarbonate secretion does not seem to be the same in all tissues and varies with physiologic demand. Thus, tissue type and whether conditions are baseline or stimulated needs to be taken into account when evaluating the evidence concerning the role of bicarbonate in the pathophysiology of CF as a regulator of local pH. Basic and applied research that focuses on the role of CFTR-mediated bicarbonate secretion helps explain many of the diverse clinical manifestations that are CF.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Borowitz Ddoi
10.1002/ppul.23247subject
Has Abstractpub_date
2015-10-01 00:00:00pages
S24-S30eissn
8755-6863issn
1099-0496journal_volume
50 Suppl 40pub_type
杂志文章,评审abstract:OBJECTIVE:The aim of this study was to assess pulmonary function and its predictors in very low birth weight (birth weight ≤1,500 g) children (VLBWc) with or without bronchopulmonary dysplasia (BPD), born at gestational age ≤32 weeks at a single tertiary center during 1996-1999, after the introduction of surfactant the...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22676
更新日期:2013-08-01 00:00:00
abstract::Changes in the relative contribution of ribcage and abdomen during augmented breaths were assessed in two groups of infants (less than 2 weeks and 4-12 weeks old). In both groups ribcage and abdominal movements increased during augmented breaths in quiet sleep. In the older infants the relative increase was greater fo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950040303
更新日期:1988-01-01 00:00:00
abstract:BACKGROUND:Multiple breath washout (MBW) in infants presents a number of technical challenges. Conventional MBW is based on simultaneous measurement of flow and gas concentrations. These two signals are aligned and combined to derive expired gas volumes from which lung volumes and measures of ventilation inhomogeneity ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23348
更新日期:2016-05-01 00:00:00
abstract::Few data are known about the effects of sleep-related breathing disorders (SRBD) on the cardiovascular system in adolescents. Forty healthy adolescents (mean age, 13.7 +/- 1.6 years) answered a questionnaire regarding symptoms of sleep-related breathing disorders (SRBD) and underwent a cardiorespiratory polygraphy and...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10303
更新日期:2003-07-01 00:00:00
abstract::This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absen...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10285
更新日期:2003-06-01 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: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::In past decades, several chest radiograph scoring systems for cystic fibrosis were developed. This study was performed to compare interobserver variability of six different radiograph scores and to correlate them with clinical parameters. Thirty chest radiographs of 30 patients with cystic fibrosis were scored accordi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10280
更新日期:2003-06-01 00:00:00
abstract::Since persistent pulmonary hypertension of the newborn (PPHN) often occurs as a life-threatening illness, it would be advantageous to identify the highest-risk infants within the first 24 hours of life so that transfer to centers with extracorporeal membrane oxygenation (ECMO) or high-frequency ventilation can be faci...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950050103
更新日期:1988-01-01 00:00:00
abstract::Short-acting β2-adrenergic receptor agonists are commonly used bronchodilators for symptom relief in asthmatics. Recent evidence demonstrated that prolonged exposure of cultured airway smooth muscle cells to β2 agonists directly augments procontractile signaling pathways with the change in expression of regulator of G...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22723
更新日期:2013-10-01 00:00:00
abstract::We have studied the effect of triamcinolone acetonide (TAC) on airway morphogenesis of the Sprague-Dawley fetal rat in whole organ lung cultures from day 15 to day 21 of equivalent gestational age (6 days in culture). TAC produced an increased number of peripheral buds from day 18 onward and the airway and airspaces h...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950140107
更新日期:1992-09-01 00:00:00
abstract:UNLABELLED:Vascular rings (VR) may produce extrinsic compression of trachea and/or esophagus. Diagnosis relies on nonspecific clinical data from resulting compression and image studies, mainly angiography, computed tomography, and cardiovascular MRI. Because of their low incidence, diagnosis is often missed. The role o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21216
更新日期:2010-06-01 00:00:00
abstract:BACKGROUND:Asthma is the most common chronic inflammatory disease in childhood and some reports have demonstrated systemic inflammation. The relevance of high-sensitivity assays for C-reactive protein (hs-CRP), which are known to be a sensitive marker of low-grade systemic inflammation, has not been fully studied in ch...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21539
更新日期:2012-03-01 00:00:00
abstract::Early detection of progressive lung disease in cystic fibrosis (CF) may lead to better treatment and prognosis. Routine lung function indices may be relatively insensitive markers of peripheral airway obstruction and alveolar collapse. We hypothesized that the single-breath diffusion capacity of the lung for carbon mo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10357
更新日期:2004-01-01 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 raised lung volume technique is increasingly used to measure forced expiratory maneuvers in infants. However, there is no consensus regarding the optimal airway inflation pressure (P(inf)) required for such maneuvers, or the influence of small changes in P(inf) within and between infants. The aim of this study was...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.10060
更新日期:2002-02-01 00:00:00
abstract::Indoor aeroallergen exposures increased asthma symptoms in Caucasians, but their determinants and relationship to asthma and allergy in Asians are unclear. This study investigated exposures to cat, cockroach, and Blomia tropicalis allergens in 115 Hong Kong families with asthmatic children. Patients underwent exhaled ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21391
更新日期:2011-07-01 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:BACKGROUND:Decreased lung function is common in preterm-born survivors. Increased fractional exhaled nitric oxide (FeNO) appears to be a reliable test for eosinophillic airway inflammation especially in asthma. We, systematically, reviewed the literature to compare FeNO levels in preterm-born children and adults who di...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,meta分析
doi:10.1002/ppul.24270
更新日期:2019-05-01 00:00:00
abstract:OBJECTIVE:Cystic fibrosis (CF) patients may develop hypoxemia during sleep. Limited information is available on nocturnal oxygen saturation in CF children with less severe lung disease. The aim of this study was to investigate the degree of nocturnal oxygen desaturation and factors that correlate with nocturnal oxygena...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20643
更新日期:2007-08-01 00:00:00
abstract:OBJECTIVES:To investigate the effect of position on the strength of the Hering-Breuer reflex in prematurely born infants and determine whether any differences seen were related to differences in lung or tidal volume between positions. WORKING HYPOTHESIS:Position related differences in the strength of the Hering-Breuer...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20855
更新日期:2008-08-01 00:00:00
abstract::Two male patients with cystic fibrosis (CF), both 18 years old, developed frontal lobe brain abscesses. Both patients presented with histories of intermittent headache over several days and occasional vomiting. Headache was not more evident in the mornings and not associated with visual disturbance in either patient. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950170609
更新日期:1994-06-01 00:00:00
abstract:BACKGROUND:Our objective was to determine those characteristics associated with reversibility of airflow obstruction and response to maximal bronchodilation in children with severe asthma through the Severe Asthma Research Program (SARP). METHODS:We performed a cross-sectional analysis evaluating children ages 6 to 17...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24473
更新日期:2019-11-01 00:00:00
abstract::Our objective was to compare the effectiveness of maximum forced expiratory flow measured at functional residual capacity (V'maxFRC) and the ratio of flow at 75% of the forced expiratory volume to peak forced expiratory flow (FEF(75)/FEF(peak)) for detecting bronchodilator-related changes in wheezy infants. In 55 infa...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.10325
更新日期:2003-09-01 00:00:00
abstract::The goal of the present investigation was to describe the prevalence of and clinical factors associated with sleep-disordered breathing in children and adolescents. Children and adolescents (3,680 in all, 1-18 years old) attending schools in central Greece were surveyed by questionnaires distributed to parents. We fou...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.20002
更新日期:2004-06-01 00:00:00
abstract::Sudden unexplained deaths have been reported in 13% [corrected] of Familial Dysautonomia (FD) subjects. To characterize cardiorespiratory dysregulation in children with FD that might contribute to potential sudden death, respiratory inductance plethysmography (chest/abdomen), ECG, hemoglobin saturation, and pulse wave...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20764
更新日期:2008-03-01 00:00:00
abstract:INTRODUCTION:The prognosis of postinfectious bronchiolitis obliterans (PIBO) has many implications, ranging between reduced quality of life and life-threatening complications. We evaluated the prognostic factors for PIBO using the baseline clinical characteristics of patients and built a prediction model for determinin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25220
更新日期:2020-12-11 00:00:00
abstract:BACKGROUND:Acquiring sputum cultures from infants is considered challenging. We describe their yield in infants with cystic fibrosis (CF) and other chronic suppurative lung diseases (CSLDs). METHODS:Retrospective medical record review over a 4-year period, for infants aged 0-2 years with ≥2 airway bacterial cultures a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25103
更新日期:2020-12-01 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
abstract::There is increasing interest in using the Multiple Breath Washout technique and the lung clearance index (LCI) for detecting early pulmonary changes, for example, in cystic fibrosis lung disease. However, there are still limited data regarding equipment specific reference ranges, repeatability and reproducibility. The...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21093
更新日期:2009-12-01 00:00:00