Abstract:
OBJECTIVES:Childhood interstitial lung disease (chILD) is a group of rare chronic and complex disorders of variable pathology. There has been no systematic review of published chILD research. This study aimed to describe chILD classification systems, epidemiology, morbidity, treatments, outcomes, and the impact of chILD on families and the burden on health services. METHODS:A systematic literature search for original studies on chILD was undertaken in the major biomedical databases to the end of December 2013. Epidemiological studies, case series and studies describing classification systems were included. Single case studies were excluded. RESULTS:The search yielded 37 publications that met study criteria. Four different chILD classification systems have been proposed in the past decade. The incidence of chILD has been estimated at 0.13-16.2 cases/100,000 children/year. One to five new cases presented to individual hospitals each year. In developed countries, the median mortality was 13% (6-19%). Morbidity and outcomes were highly variable and not systematically reported. Corticosteroids and hydroxychloroquine were the most common treatments. The impact of chILD on families and the burden on health services has not been studied. CONCLUSIONS:The heterogeneity of the chILD group of disorders, different determinations of what constitutes a chILD disorder and, a paucity of large epidemiological studies precludes consolidation of results across studies. Consensus on chILD classification is needed to support diagnosis and allow direct comparisons of research evidence. Active disease surveillance and international patient registries are required to advance understanding and management of chILD.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Hime NJ,Zurynski Y,Fitzgerald D,Selvadurai H,Phu A,Deverell M,Elliott EJ,Jaffe Adoi
10.1002/ppul.23183subject
Has Abstractpub_date
2015-12-01 00:00:00pages
1383-92issue
12eissn
8755-6863issn
1099-0496journal_volume
50pub_type
杂志文章,评审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:OBJECTIVE:To define the benefits of a flexible bronchoscopy (FB) service in a Paediatric Intensive Care Unit (PICU). DESIGN:Review of the first 200 FBs undertaken in a large PICU. SETTING:Large cardiac and medical PICU in the United Kingdom, also providing extra-corporeal life support. PATIENTS:129 patients (78 male...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20910
更新日期:2008-12-01 00:00:00
abstract:INTRODUCTION:Acute bronchiolitis is a major cause of acute respiratory distress in infants. The soluble receptor for advanced glycation end-products (sRAGE) is a biomarker of pulmonary damage processes, with a diagnostic and a prognostic value in acute respiratory distress syndrome (ARDS). The RAGE pathway is also impl...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24141
更新日期:2018-10-01 00:00:00
abstract:OBJECTIVE:Medication adherence among adolescents with cystic fibrosis (CF) is often suboptimal and this has significant impact on their health and quality of life. The purpose of the study was to evaluate the impact of frequent home pulmonary function (PFT) monitoring on medication adherence among adolescents with CF. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23950
更新日期:2018-04-01 00:00:00
abstract::Cystic fibrosis (CF) is an illness associated with high healthcare utilization and healthcare costs, even when compared to other chronic illnesses. In a variety of medical populations, depression has been found to be associated with lower adherence and poorer medical outcomes. The current study is a retrospective char...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22990
更新日期:2014-12-01 00:00:00
abstract:BACKGROUND:Pulmonary function testing is commonly performed for diagnosis and clinical management of respiratory diseases. It is important to use appropriate reference equations from healthy subjects for interpretation of data from infants with lung disease. This study aimed to determine if published reference equation...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24063
更新日期:2018-07-01 00:00:00
abstract::Twenty patients with cystic fibrosis (CF) were trained to mobilize intrabronchial secretions by a new method of positive expiratory pressure mask (PEP mask) physiotherapy (PT). Patients repeatedly expired forcefully through the PEP mask; expiratory resistance was varied by eight different internal diameter resistors a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020608
更新日期:1986-11-01 00:00:00
abstract::Pulmonary function testing and monitoring plays an important role in the respiratory management of neonates. A noninvasive and complete bedside evaluation of the respiratory status is especially useful in critically ill neonates to assess disease severity and resolution and the response to pharmacological intervention...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23245
更新日期:2015-10-01 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:Cystic fibrosis (CF) genotyping has garnered increased attention since the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 led to the identification of over 1700 mutations on chromosome 7. Yet, little is known about the genetic profile of CF patients in Turkey. This s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24299
更新日期:2019-06-01 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 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 o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23247
更新日期:2015-10-01 00:00:00
abstract::We herein report a case of antiphospholipid syndrome (APS) primarily presenting with asthma-like symptoms that had been misdiagnosed as severe asthma. Patients presenting with severe asthma symptoms along with systemic thrombosis should be systematically evaluated for APS. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23079
更新日期:2015-02-01 00:00:00
abstract::Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown. In childhood, it is extremely rare, with only three reported cases so far. Here we report on an 8-year-old girl,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10242
更新日期:2003-05-01 00:00:00
abstract::Bronchoalveolar lavage (BAL) performed with a fiberoptic bronchoscope (FOB) is a useful method for sampling alveolar contents. Since the smallest FOB with a channel has a diameter of 3.6 mm, BAL is difficult to accomplish through artificial airways (AA) less than 5.0 mm I.D. We used a 4F balloon wedge pressure cathete...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950130110
更新日期:1992-05-01 00:00:00
abstract::The free running test is a useful method for evaluation of exercise-induced bronchospasm in children. In young children this test simulates real-life circumstances and can be done more easily than histamine or methacholine challenges. The interrupter technique is a noninvasive method for measuring airflow resistance d...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1099-0496(199903)27:3<203::aid-ppul9
更新日期:1999-03-01 00:00:00
abstract:OBJECTIVES:We sought to compare gas exchange, respiratory mechanics, and asynchronies during pressure support ventilation (PSV), sigh adjunct to PSV (PSV SIGH), and neurally adjusted ventilatory assist (NAVA) in hypoxemic infants after cardiac surgery. DESIGN:Prospective, single-center, crossover, randomized physiolog...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24335
更新日期:2019-07-01 00:00:00
abstract:OBJECTIVE:Since preventive therapies for bronchopulmonary dysplasia (BPD) are limited we treated preterm infants with azithromycin to decrease the incidence of BPD. METHODS:Infants less than 1,250 g birth weight were randomized to azithromycin or placebo within 12 hr of beginning mechanical ventilation and within 72 h...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21352
更新日期:2011-02-01 00:00:00
abstract::Aerosolized recombinant human DNase (dornase alfa) reduces mucus viscoelasticity in vitro and improves pulmonary function in patients with cystic fibrosis (CF). We postulated that if dornase alfa could be delivered more peripherally to small airways in the lung in the form of smaller aerosol droplets in patients with ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/(sici)1099-0496(199802)25:2<83::aid-ppul2>
更新日期:1998-02-01 00:00:00
abstract::Foreign-body aspiration (FBA) is a commonly encountered serious problem during childhood, accounting for 7% of lethal accidents in children aged 1-3 years. Bronchoscopy is indicated when a clear history of FBA is present, but the history may be unreliable. Clinical manifestations can be seen in various diseases, and w...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20128
更新日期:2005-02-01 00:00:00
abstract:OBJECTIVES:Respiratory syncytial virus (RSV) is a leading cause of lower respiratory tract infections worldwide, causing disproportionate morbidity and mortality in infants and children. Infants with stronger Th1 responses have less severe disease, yet little is known about the infant T-cell response within the air spa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23911
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVE:While the impact of fertility treatments on the perinatal outcome is well established, the long-term effects on offspring are yet to be determined. The current study aimed to investigate the risk of long-term obstructive sleep apnea (OSA) among children born following in vitro fertilization (IVF) and ovulatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24428
更新日期:2019-10-01 00:00:00
abstract::The quantity and composition of airway surface liquid (ASL) are essential to host defense. To date, attempts to harvest ASL and measure its composition have yielded conflicting results. We investigated the physical principles underlying two techniques that were proposed for harvesting ASL: filter paper pledgets and po...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10409
更新日期:2004-02-01 00:00:00
abstract::We conducted a survey to determine parent-reported asthma prevalence, morbidity, and healthcare utilization among 3527 children attending public schools (n = 2849) and private schools (n = 678) in San Juan, Puerto Rico. Schools were randomly selected from each of 12 geographic regions of the San Juan metropolitan area...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20022
更新日期:2004-05-01 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:INTRODUCTION:The efficacy of nebulized hypertonic saline (HS) therapy for shortening hospital length of stay (LOS) or improving bronchiolitic symptoms remains controversial. Most studies enrolled small numbers of subjects and did not consider the role of respiratory syncytial virus (RSV), the most common cause of acute...
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.23945
更新日期:2018-03-01 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::To determine the role of calmodulin in postnatal lung growth and development, 4-week-old rats were injected intraperitoneally on consecutive days with trifluoperazine (TFP), a potent and specific calmodulin antagonist, for a period fo 3 weeks and studied in comparison with normal controls and undernourished weight-mat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950100110
更新日期:1991-01-01 00:00:00
abstract::A 16-year-old female with cystic fibrosis (CF) presented with an acute respiratory exacerbation during which black flecks were observed in the spontaneously expectorated sputum. The production of this pigmented sputum was subsequently attributed to Exophiala dermatitidis hyphae. Treatment with antibiotics, corticoster...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21187
更新日期:2010-05-01 00:00:00
abstract::We present two infants with congenital tracheal stenosis with complete tracheal rings. Both had associated congenital anomalies. The first case showed cardiac malformations, and the second case had agenesis of the right lung. We review the literature, in particular with reference to tracheal stenosis and pulmonary age...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020414
更新日期:1986-07-01 00:00:00