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 clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Montgomery GS,Sagel SD,Taylor AL,Abman SHdoi
10.1002/ppul.20393subject
Has Abstractpub_date
2006-04-01 00:00:00pages
383-5issue
4eissn
8755-6863issn
1099-0496journal_volume
41pub_type
杂志文章abstract:OBJECTIVE:Among older children, sleep-disordered breathing (SDB) is associated with measurable neurocognitive consequences. However, diagnostic SDB thresholds are lacking for infants < 12 months. We sought to evaluate the relationship between SDB indices, gestational age (GA), and postmenstrual age (PMA) for infants wh...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24354
更新日期:2019-08-01 00:00:00
abstract:BACKGROUND:Patients with bronchopulmonary dysplasia (BPD) may require tracheostomy for long-term mechanical ventilation. Polysomnography (PSG) may predict successful decannulation in children, however it is unclear how this success compares with children without a PSG. To better evaluate this role, we compared decannul...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24474
更新日期:2019-11-01 00:00:00
abstract::Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple body organs. The lungs and pancreas are the most affected which results in progressive lung damage and pancreatic insufficiency. Due to the disease process, CF patients require significantly higher caloric intake than recommended for other ind...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20766
更新日期:2008-03-01 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::Phrenic nerve paralysis is a condition typically occurring after invasive procedures in the chest and neck. Here we describe a case of transient unilateral diaphragmatic paralysis in a child with status asthmaticus complicated by complete right lung atelectasis. Common causes of this disorder and possible implications...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20040
更新日期:2004-09-01 00:00:00
abstract::The efficacy and tolerability of high-dose salmeterol (100 mcg, BID) and albuterol (2.5 mg, BID) were compared with those of albuterol (2.5 mg, BID) in outpatients with cystic fibrosis in a randomized, double-blind, double-dummy, placebo-controlled, crossover study with both short- (4 weeks of each) and long-term (24 ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.10162
更新日期:2002-10-01 00:00:00
abstract::Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systemati...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23632
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND:Childhood wheeze is an important, well-known risk factor for asthma, yet little is known about the contribution of nocturnal dry cough. We investigated the association of nocturnal dry cough at ages 1-7 years with doctor-diagnosed asthma at 8 years of age, both in the presence and absence of wheeze. METHODS...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23092
更新日期:2015-09-01 00:00:00
abstract::Bronchial reactivity was assessed in 66 children with bronchial asthma (aged 8-15 years) by provocation with histamine-HCl during a symptom-free period. A significant bronchial reaction to histamine was defined as a 50% increase in the resistance of the respiratory system (Rrs) determined by the forced oscillation tec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950150308
更新日期:1993-03-01 00:00:00
abstract:INTRODUCTION:There are few published data on the efficacy of systemic corticosteroids in preterm infants with very severe forms of bronchopulmonary dysplasia (BPD), requiring respiratory support after 3 months of age. The aim of this study was to report the use of pulses of methylprednisolone in this population and its...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25109
更新日期:2021-01-01 00:00:00
abstract::Even though childhood asthma is assumed to comprise reversible airway obstruction, some children develop irreversible airway obstruction (not reversed by a bronchodilator or corticosteroids); this may be due to inflammation that has caused remodeling. Lately, it has been claimed that in the absence of treatment with i...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10090
更新日期:2002-04-01 00:00:00
abstract::Plasminogen deficiency is characterized by fibrin-rich pseudomembrane formation on all mucosal surfaces, particularly the conjunctiva. Respiratory system involvement is common; fibrin often obstructs the upper or lower respiratory tract, causing death. Although many treatments have been applied, no definitive treatmen...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24563
更新日期:2020-01-01 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:BACKGROUND:Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM. METHODS AND PATIENTS:Clinical manifestations, pulmonary ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.22742
更新日期:2013-10-01 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::In order to help specialists involved in CF care and clinical research to know the current best evidence about clinical effectiveness of interventions in CF, we designed and developed a web-based, free access tool called "CFDB"--Cystic Fibrosis DataBase (www.inetflow.it/CFDB). The database was built by searching in Me...
journal_title:Pediatric pulmonology
pub_type: 信件
doi:10.1002/ppul.22954
更新日期:2014-09-01 00:00:00
abstract::Exposure to chronic constant or intermittent hypoxia (CCH or CIH) may have different effects on growth and development in early life. In this work, we exposed postnatal day 2 (P2) CD1 mice to CCH or CIH (11% O2) for 4 weeks and examined the effect of hypoxia on body and organ growth until P30. Regression analysis show...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20729
更新日期:2008-01-01 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:SUMMARY:Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder caused by mutations in the ATM gene, resulting in faulty repair of breakages in double-stranded DNA. The clinical phenotype is complex and is characterized by neurologic abnormalities, immunodeficiencies, susceptibility to malignancies, recurren...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21277
更新日期:2010-09-01 00:00:00
abstract::Intrauterine inflammation generates inflammatory mediators that damage the developing bronchoalveolar epithelium, resulting in neonatal lung injury. Lung fluid transport disorders are the main reasons for the development of pulmonary edema, an important pathology of lung injury. Previous studies suggested that epithel...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24242
更新日期:2019-03-01 00:00:00
abstract:RATIONALE AND OBJECTIVES:Little is known about molecular changes in lungs of fetal rabbits with surgically induced diaphragmatic hernia (DH). Therefore, we examined in this model gene expressions of pivotal molecules for the developing lung. METHODS:At day 23 of gestation, DH was created in 12 fetuses from 4 does. Bot...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22512
更新日期:2012-11-01 00:00:00
abstract::To assess whether there is a maturation of the response to tube breathing during the first 10 days of life, we have measured the dynamic ventilatory responses of nine term infants (34 studies) to added tubes equivalent to two anatomical dead spaces. Such a method allows an assessment of the baby's ability to increase ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950090203
更新日期:1990-01-01 00:00:00
abstract:UNLABELLED:SUMMARY RATIONALE: Asthma therapy should be stepped up or stepped down in response to changes in asthma control. However, there is little evidence available on the optimal timing, sequence, and degree of medication reductions. In this study we analyzed clinically stable asthmatic children who underwent a med...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21278
更新日期:2010-09-01 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::Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive type of combined immunodeficiency with elevated IgE. In this report, we describe a Japanese girl of non-consanguineous family suffering from acute eosinophilic pneumonia (AEP) as a presenting feature of DOCK8 deficiency. Although AEP was self-limi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22814
更新日期:2014-03-01 00:00:00
abstract::A group of 153 children (51 with a history of bronchiolitis and 102 matched controls) were evaluated in a historical cohort study at a mean age of 8 years and again at 13 years to test the primary hypothesis that mild bronchiolitis, far more common than severe (hospitalized) bronchiolitis, predicts wheezing. A seconda...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060303
更新日期:1989-01-01 00:00:00
abstract::To determine the extent of pulmonary dysfunction following primary closure of an abdominal wall defect, we obtained pulmonary function tests (PFT) in 11 newborn infants with gastroschisis and 6 with large omphaloceles admitted to a newborn ICU in a children's hospital. Patients were 1 to 30 days of age at the time of ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120309
更新日期:1992-03-01 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
abstract::Our objective was to determine whether postnatal respiratory function, lung growth, and lung structure are affected by preterm birth which did not require neonatal respiratory support. Two groups of preterm (P) lambs were delivered 2 weeks before term, at 133 days of gestational age (GA). Tissue was collected at term ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20274
更新日期:2005-10-01 00:00:00
abstract::While the use of the raised volume rapid thoraco-abdominal compression (RVRTC) technique has been shown to provide new insights into airway and pulmonary pathophysiology in infants, and appears to resemble the spirometric techniques used in older subjects, there is as yet no consensus regarding measurement procedures,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10170
更新日期:2002-11-01 00:00:00