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 to 30 June 2016. Clinical data were collected from the CF Registry of Ireland, medical charts, and data on weight/height before diagnosis from public health nurses and family doctors. SPSS was used for analysis. A total of 232 patients were recruited (response 93%) (93 clinically diagnosed, 139 NBS-detected). Following exclusions of meconium ileus (MI) (40), diagnosis outside Ireland (4), and being designated as CFSPID (2), a total of 77 clinically diagnosed patients and 109 NBS detected children were included in analysis. Over half were homozygous for F508del mutation. Being clinically diagnosed was independently associated with hospitalization for infective exacerbation of CF < 36 months (OR, 2.80; 95%CI 1.24-6.29). Diagnosis to first acquisition of Pseudomonas aeruginosa was significantly longer in NBS than clinically detected; from birth there was no significant difference. Weight and length/height were significantly greater in NBS cohort at 6 and 12 months. We provide evidence of improved growth, reduced hospitalization for acute exacerbations, and delayed P. aeruginosa acquisition (from diagnosis) to age 3 for the NBS cohort. Screening practices likely account for the non-significant difference in P. aeruginosa acquisition from birth.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Fitzgerald C,Linnane B,George S,Ni Chroinin M,Mullane D,Herzig M,Greally P,Elnazir B,Healy F,Mc Nally P,Javadpour S,Cox D,Fitzpatrick Pdoi
10.1002/ppul.24876subject
Has Abstractpub_date
2020-09-01 00:00:00pages
2323-2329issue
9eissn
8755-6863issn
1099-0496journal_volume
55pub_type
杂志文章abstract::Ten infants with evidence of impending respiratory failure from severe bronchiolitis were successfully treated with continuous positive airway pressure (NCPAP) with double nasal prongs. Their mean (SD) age was 6.7 (3.8) months and mean (SD) body weight was 7.1 (2.1) kg. Respiratory assessments were made immediately be...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950160305
更新日期:1993-09-01 00:00:00
abstract:BACKGROUND:Asthma is associated with poorer outcomes in sickle cell disease (SCD). Whether AHR can exist in SCD as a distinct entity, separate and independent of asthma, is unknown. AIMS:Our goal was to elucidate the prevalence of AHR, as measured by a methacholine challenge test (MCT), in children with SCD who did no...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23374
更新日期:2016-09-01 00:00:00
abstract:OBJECTIVE:Lung ultrasound (LUS) is an emerging tool that may be used in the diagnosis and follow-up of children with viral bronchiolitis. In this study, we describe LUS abnormalities in children receiving invasive mechanical ventilation (IMV) for severe bronchiolitis in the pediatric intensive care unit (PICU). Our aim...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24974
更新日期:2020-10-01 00:00:00
abstract::Measurements of arterial oxygen tension (PaO2) while breathing room air, and maximum expiratory flow volume curves were performed in 34 patients with cystic fibrosis (age range 7-27 years, 24 males and 10 females). Logistic regression was performed using forced vital capacity (FVC), forced expiratory volume in 1 secon...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060108
更新日期:1989-01-01 00:00:00
abstract:BACKGROUND:Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed to asse...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21149
更新日期:2010-03-01 00:00:00
abstract:OBJECTIVES:Swallowing disorders which lead to aspiration are common in premature infants with a postmenstrual age (PMA) of >36 weeks. Aspiration is often silent and the unique symptom is desaturation during feeding. The aim of this study was 1) to determine the number of prematures with desaturations during feeding due...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23946
更新日期:2018-04-01 00:00:00
abstract:BACKGROUND:The measurement of specific airway resistance during tidal breathing (sRaw(tb)) has gained popularity in children, but methodological concerns have been raised regarding the electronic compensation for the thermal artifact. The panting method (sRaw(p)) is efficient in minimizing the latter, but may be associ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22829
更新日期:2014-03-01 00:00:00
abstract::To evaluate the long-term effect of prematurity and/or hyaline membrane disease (HMD) on pulmonary function and airway reactivity, we studied 49 prematurely born children aged 10 to 13 years. They were divided into three groups according to birth weight and HMD status: Groups I and II comprised the children weighing l...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950070412
更新日期:1989-01-01 00:00:00
abstract::Campomelic dysplasia is a generalized disorder of cartilaginous growth and development, leading to early death from pulmonary insufficiency. We describe the airway dynamics as observed bronchoscopically in two affected infants. Both infants demonstrated anatomic compromise of the upper airway and diffuse laryngotrache...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030514
更新日期:1987-09-01 00:00:00
abstract:OBJECTIVES:To prospectively assess longitudinal lung function in children with sickle cell disease (SCD). WORKING HYPOTHESIS:Lung function in SCD children deteriorates with increasing age and the decline is more marked in younger children who have recently suffered ACS episodes. STUDY DESIGN:Two prospective longitudi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23367
更新日期:2016-07-01 00:00:00
abstract:AIM:The aim of this study was to investigate the status and effects of vitamin D supplement as adjuvant therapy in the management of respiratory distress syndrome (RDS) in preterm infants. PATIENT AND METHODS:Vitamin D was prospectively studied in 196 preterm neonates, 96 of them developed RDS, which further subdivide...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24552
更新日期:2020-01-01 00:00:00
abstract::We reviewed the literature on the effects of high flow nasal cannula (HFNC) and heated, humidified, high-flow, nasal cannula (HHHFNC) treatment in preterm infants. We found nine studies, but only two were randomized controlled trials. These studies show that: HFNC application is associated to the delivery of continuou...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.21051
更新日期:2009-07-01 00:00:00
abstract::Electronic compensation to overcome thermal artifacts during plethysmographic estimations of airway resistance is now used routinely in adults and school-age children, and was shown to be a valuable means of discriminating airway function between preschool children with and without lung disease. A similar system is no...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.20217
更新日期:2005-07-01 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::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::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::While many factors that contribute to the occurrence of lower respiratory tract infections (LRTI) in Canadian indigenous children have been described, the role of aspiration during swallowing has not been explored in these children. Because of an increase in referrals of indigenous children from our catchment area (Ma...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21489
更新日期:2011-12-01 00:00:00
abstract:INTRODUCTION:Exercise ventilation efficiency index in cardiopulmonary exercise testing (CPET) is elevated in patients with heart failure providing useful information on disease progression and prognosis. Few data, however, exist for ventilation efficiency index among cystic fibrosis (CF) patients. AIMS:To assess venti...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24438
更新日期:2019-10-01 00:00:00
abstract::Lung hypoplasia is the main cause of congenital diaphragmatic hernia (CDH)-associated death but pathogenesis remains unclear. MiR-455-5p is involved in lung hypoplasia. We hypothesized that nitrofen causes abnormal miR-455-5p expression during lung development and designed this study to determine the relationship betw...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24739
更新日期:2020-06-01 00:00:00
abstract::Better growth and nutritional status is strongly associated with better pulmonary function and survival in children with CF. Behavioral intervention is an efficacious treatment approach for improving calorie intake and weight gain in children with CF; and recently has been shown to facilitate maintenance of daily ener...
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.21147
更新日期:2010-01-01 00:00:00
abstract:BACKGROUND:Treatment of bronchopulmonary dysplasia (BPD) remains as yet an unmet clinical need and recently stem cells have been proposed as a therapeutic tool in animal models. We investigated the role of amniotic fluid stem cells (AFS) in an adult rat model of hyperoxia lung injury. METHODS:Fifty Sprague-Dawley rats...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22791
更新日期:2013-11-01 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::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::The cotton rat was evaluated as a model for anti-inflammatory and antiviral influenza therapy. Beginning 3 days after intranasal infection with 10(7) tissue culture infectious doses-50% (TCID)(50) of an H3N2 human influenza, animals were treated topically via intranasal lavage with a range of doses of triamcinolone ac...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10320
更新日期:2003-10-01 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:BACKGROUND:Although the benefits of recombinant human deoxyribonuclease (dornase alfa) in patients with cystic fibrosis (CF) are established, its optimal timing in relation to physiotherapy is unknown. As its enzymatic effect lasts for 6-11 hr, dornase alfa may be more efficacious if the time interval between inhalatio...
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.20704
更新日期:2007-12-01 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:OBJECTIVE:The prevalence of obstructive sleep apnea (OSA) is higher in children with poorly controlled asthma. We aimed to determine the validity of the Pediatric Sleep Questionnaire (PSQ) to screen for OSA in children with asthma. METHODS:This retrospective review encompassed sleep studies and medical records of asth...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23568
更新日期:2017-03-01 00:00:00
abstract::The purpose of this report is to describe the College of American Pathologists sweat testing (SW) proficiency testing program for cystic fibrosis, to evaluate its impact on test performance, and to describe the current practice of sweat testing in North America. The study analyzed participant summary reports of the SW...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200012)30:6<476::aid-ppul7>3.0.c
更新日期:2000-12-01 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