Abstract:
INTRODUCTION:To date, no consensus has been reached on the optimal management of congenital lung abnormalities, and factors predicting postnatal outcome have not been identified. We developed an objective quantitative computed tomography (CT) scoring method, and assessed its value for clinical decision-making. METHODS:Volumetric CT-scans of all patients born with a congenital lung abnormality between January 1999 and 2018 were assessed. Lung disease was quantified using the newly-developed congenital lung abnormality quantification (CLAQ) scoring method. In 20 equidistant axial slices, cells of a square grid were scored according to the abnormality within. The scored CT parameters were used to predict development of symptoms, and SD scores for spirometry and exercise tolerance (Bruce treadmill test) at 8 years of age. RESULTS:CT-scans of 124 patients with a median age of 5 months were scored. Clinical diagnoses included congenital pulmonary airway malformation (49%), bronchopulmonary sequestration (27%), congenital lobar overinflation (22%), and bronchogenic cyst (1%). Forty-four patients (35%) developed symptoms requiring surgery of whom 28 (22%) patients became symptomatic before a CT-scan was scheduled. Lesional hyperdensity was found as an important predictor of symptom development and decreased exercise tolerance. Using receiver operating characteristic analysis, an optimal cut-off value for developing symptoms was found at 18% total disease. CONCLUSION:CT-quantification of congenital lung abnormalities using the CLAQ method is an objective and reproducible system to describe congenital lung abnormalities on chest CT. The risk for developing symptoms may increase when more than a single lung lobe is affected.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Hermelijn SM,Dragt OV,Bosch JJ,Hijkoop A,Riera L,Ciet P,Wijnen RMH,Schnater JM,Tiddens HAWMdoi
10.1002/ppul.25032subject
Has Abstractpub_date
2020-11-01 00:00:00pages
3152-3161issue
11eissn
8755-6863issn
1099-0496journal_volume
55pub_type
杂志文章abstract:BACKGROUND:Evidence from recent studies suggests that IRT/PAP protocols may be successfully used as a purely biochemical newborn screening (NBS) for cystic fibrosis (CF) that does not require genetic screening. However, the experience with the performance of different IRT/PAP protocols remains limited. In this study, w...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23190
更新日期:2015-07-01 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::The main purpose of this study was to evaluate four different forms of treatment in young infants admitted for acute wheezing (AW). Seventy-nine infants less than one year of age were randomly assigned to one of five groups. Group 1 received nebulized fenoterol plus ipratropium bromide, group 2 fenoterol, group 3 feno...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1950030504
更新日期:1987-09-01 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::Most pneumatoceles disappear spontaneously and do not cause severe symptoms. Treatment alternatives include various conventional or surgical methods. However, an enlarging, complicated pneumatocele with cardiorespiratory instability requires imaging-guided catheter drainage or surgery. Here, we report the case of a ne...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23311
更新日期:2016-02-01 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:OBJECTIVE:Recent advances in medicine have allowed children with chronic life-threatening disorders to survive longer than ever before with the use of complex medical device technology (e.g., mechanical ventilation, dialysis, etc.). The care of children with chronic pulmonary disorders and respiratory-technology depend...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23155
更新日期:2015-12-01 00:00:00
abstract::Respiratory syncytial virus (RSV) lower respiratory tract infection (LRTI) during early childhood may be associated with subsequent pulmonary sequelae, including recurrent wheezing and asthma. We undertook a systematic review to investigate the pulmonary function sequelae following RSV LRTI in the first 3 years of lif...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24804
更新日期:2020-07-01 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::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::Aspiration is common in the intubated human neonate. Thus, the ventilatory and blood gas responses to citric acid and saline instillation into different airway sites were studied in ten awake, unanesthetised lambs, breathing spontaneously via a tracheostomy tube. With a system of balloons, 1 ml of saline or citric aci...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030112
更新日期:1987-01-01 00:00:00
abstract:BACKGROUND:In young children with cystic fibrosis (CF) the forced expiratory volume in 1 second (FEV1 ) is often normal and a more sensitive measure to detect early obstructive lung disease is needed. AIM:To evaluate the progression of selected spirometry parameters with age in a cohort of CF patients and healthy chil...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22777
更新日期:2013-11-01 00:00:00
abstract::This study was designed to collect data on the prevalence of respiratory syncytial virus (RSV) infection in Italy in infants hospitalized for lower respiratory tract infections, and to evaluate which of the recognized risk factors might be associated with disease severity. Thirty-two centers throughout Italy participa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.10047
更新日期:2002-06-01 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
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::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::Inhaled corticosteroids are commonly used in cystic fibrosis (CF), but there are few studies evaluating their safety in young children. We, therefore, prospectively administered beclomethasone diproprionate (BDP) to 12 clinically stable young children with CF to examine the safety of this therapy with respect to adren...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1122
更新日期:2001-10-01 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:INTRODUCTION:In this cohort study spanning an 18-year period, we evaluated the prevalence and associated mortality rate of epidemic strains of pseudomonas aeruginosa (PsA), especially Australian Epidemic Strain Type 1 (AES1), in a pediatric cystic fibrosis center practicing cohort segregation and early PsA eradication....
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24173
更新日期:2018-11-01 00:00:00
abstract::The reliability and predictive value of salivary theophylline levels was investigated in 58 children who were treated with oral sustained-release theophylline preparations for bronchial asthma. Serum and saliva levels were assessed by fluorescence polarization immunoassay (FPIA), and Seralyzer (reagent strip reflectan...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060407
更新日期:1989-01-01 00:00:00
abstract::Prenatal cigarette smoke (CS) exposure, in combination with hypoxia and/or hyperthermia can lead to gasping and attenuated recovery from hypoxia in 7 days old rat pups. We studied 95 unanesthetized spontaneously breathing 14 days old rat pups to investigate if the destabilizing effects of increased ambient temperature...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21578
更新日期:2012-05-01 00:00:00
abstract::Physicians and physiotherapists who care for CF patients have recommended the use of trampolines as a physiotherapeutic tool for enhancing cardiopulmonary performance, encouraging sputum production, and improving general well-being. Despite some therapeutic and recreational benefits associated with trampoline use, pap...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20133
更新日期:2005-01-01 00:00:00
abstract::Lung diseases caused by surfactant protein C (SFTPC) mutations are inherited as autosomal traits with variable penetrance and severity or as sporadic disease caused by a de novo mutation on one allele. Here, we report the case of a child surviving with a homozygous surfactant protein C mutation after aggressive clinic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22976
更新日期:2014-03-01 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:BACKGROUND:Magnetic Resonance Imaging (MRI) techniques to image the larynx have evolved rapidly into a promising and safe imaging modality, without need for sedation or ionizing radiation. MRI is therefore of great interest to image pediatric laryngeal diseases. Our aim was to review MRI developments relevant for the p...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24250
更新日期:2019-04-01 00:00:00
abstract:BACKGROUND:Different modalities of noninvasive respiratory support have been recommended for the management of acute bronchiolitis in the pediatric intensive care unit (PICU). High-flow nasal cannula (HFNC) is among the new modalities that have been widely used in the last decade. METHODS:This is a retrospective study...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24553
更新日期:2020-02-01 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:BACKGROUND:Patient-reported outcomes (PROs) are increasingly used to evaluate the efficacy of new treatments and the progression of chronic diseases. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) is a disease-specific, PRO measure of health-related quality of life (HRQOL). We evaluated associations between changes ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21325
更新日期:2011-01-01 00:00:00
abstract:OBJECTIVE:Newborns exhibit the lowest immediate respiratory morbidity rates when born following 39 completed weeks of gestation. We sought to determine whether early-term delivery (37-38 + 6 weeks' gestation) impacts on long-term pediatric respiratory morbidity. STUDY DESIGN:In this population-based prospective cohort...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23529
更新日期:2017-02-01 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