Abstract:
:One hundred fifty-four children with recurrent or chronic infections of the lower respiratory tract compatible with the diagnosis of primary ciliary dyskinesia (PCD) were evaluated for the presence of ultrastructural ciliary abnormalities. Studies were performed on multiple samples of respiratory mucosa obtained by nasal and bronchial brushing. Twenty-eight children showed ultrastructural ciliary defects compatible with the diagnosis of PCD: Twenty-four presented dynein arm deficiency (either as isolated defect or in association with microtubular abnormalities), two had ciliary aplasia, and two showed microtubular abnormalities. Eleven patients with PCD had situs viscerum inversus, bronchiectasis, and chronic sinusitis (Kartagener's syndrome); one child with Kartagener's syndrome had normal ciliary structure. The appearance of respiratory symptoms within the first month of life, the colonization by Haemophilus influenzae, and a history of recurrent rhinitis and otitis were characteristically present in children with PCD. The clinical status of those patients who reached adolescence was, in our experience, remarkably good. An early diagnosis with adequate prevention and therapy of respiratory infections may have an important role in minimizing irreversible lung damage.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Barlocco EG,Valletta EA,Canciani M,Lungarella G,Gardi C,De Santi MM,Mastella Gdoi
10.1002/ppul.1950100104subject
Has Abstractpub_date
1991-01-01 00:00:00pages
11-7issue
1eissn
8755-6863issn
1099-0496journal_volume
10pub_type
杂志文章abstract:BACKGROUND:Neuroendocrine cell hyperplasia of infancy (NEHI) is a recently described children's interstitial lung disease (chILD) disorder of unknown etiology. It manifests clinically with tachypnea, retractions, hypoxemia, and crackles. The characteristic radiographic appearance consists of pulmonary hyperexpansion an...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21219
更新日期:2010-08-01 00:00:00
abstract:INTRODUCTION:The prevalence of both childhood obesity and childhood asthma has increased dramatically over the past few decades. Little is known concerning the role of body composition and lifestyle influences on airway health in children. PURPOSE:To determine whether body composition, fruit and vegetable intake (FV) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21393
更新日期:2011-05-01 00:00:00
abstract::This study sought to determine if a clinical pathway developed and executed by specialists in pediatric asthma would reduce hospital costs and length of stay (LOS). The study design was a retrospective, nonrandomized, controlled trial. Subjects were children aged 2-18 years (N = 1,004) with a history of recurrent whee...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1110
更新日期:2001-09-01 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: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::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: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: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:OBJECTIVE:Alveolar concentration (C(A)NO) and bronchial flux (J(aw)NO) of nitric oxide (NO) characterize the contributions of peripheral and proximal airways to exhaled NO. Both parameters can be estimated using a two-compartment model if the fraction of NO in orally exhaled air (FE(NO)) is measured at multiple constan...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22550
更新日期:2012-11-01 00:00:00
abstract::Episodes of respiratory distress with chest retraction and wheezing, sometimes associated with facial edema, were noted after administering the proton pump inhibitors omeprazole and esomeprazole in an infant with gastroesophageal reflux. The disturbances relieved dramatically after withdrawing the proton pump inhibito...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20616
更新日期:2007-06-01 00:00:00
abstract::We report on a 7-month old infant with severe respiratory distress secondary to a paratracheal bronchogenic cyst. Respiratory relief was achieved by transtracheal puncture of the cyst. Surgical removal of the cyst was performed 1 week later because of radiological evidence of reaccumulation of fluid. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10408
更新日期:2004-12-01 00:00:00
abstract::With dual energy X-ray absorptiometry (DEXA), it is possible to quantitate important aspects of growth in children with cystic fibrosis (CF), supplementing the usual measures of height and weight. Of particular concern during growth is the accumulation of bone mineral, since osteoporosis and fractures are well-recogni...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199902)27:2<80::aid-ppul3>
更新日期:1999-02-01 00:00:00
abstract::Randomized trials of ribavirin therapy have used clinical scores to assess illness severity. Little information on agreement for these findings between observers has been published. We decided to determine interobserver agreement for (1) a history for apnea or respiratory failure; (2) assessment of cyanosis, respirato...
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(199607)22:1<23::AID-PPUL4>
更新日期:1996-07-01 00:00:00
abstract::In contrast to its effect on airway smooth muscle in the adult, in vitro studies have shown that caffeine significantly increases active tension in airway smooth muscle in the neonatal lamb. To determine if caffeine has a physiological effect on airway function during early development, we studied the effect of caffei...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120106
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:Matrix metalloproteinase-9 (MMP-9) plasma levels correlate with C-reactive protein (CRP) concentrations and they are both increased in adults with obstructive sleep apnea (OSA). No studies have evaluated MMP-9 levels in children with sleep apnea and CRP is not consistently elevated in pediatric OSA. The aim ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21251
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND:Socioeconomic status (SES) is a strong predictor of outcomes in cystic fibrosis (CF); however, there are no published studies evaluating this relationship in Canadians with CF. The objective of this study was to assess the effect of SES on annual hospitalization rates in a large cohort of pediatric and adult...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21368
更新日期:2011-04-01 00:00:00
abstract::"Pulmonary Interstitial Glycogenosis: Diagnostic Evaluation and Clinical Course," written by Liptzin et al is a timely and insightful phenotypic summary of a rare pediatric interstitial lung disease. Twenty-four infants with biopsy-proven pulmonary interstitial glycogenosis (PIG) were reviewed at their center. Genetic...
journal_title:Pediatric pulmonology
pub_type: 评论,杂志文章
doi:10.1002/ppul.24167
更新日期:2018-12-01 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:OBJECTIVES:To investigate the adherence and the self-reported barriers to general and respiratory exercises reported by individuals with cystic fibrosis (CF). STUDY DESIGN:An exploratory, experimental study. METHODS:Community-dwelling individuals aged 16 years and over, diagnosed with CF, who were accompanied in refe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24912
更新日期:2020-10-01 00:00:00
abstract::Fetuses of diabetic mothers who were exposed to excessive glucose show delayed maturation. Under these conditions, altered growth factor expression or signaling may have important regulatory influences. We examined the role of epidermal growth factor (EGF) in lung development and maternal diabetes in the rat. In order...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200002)29:2<103::aid-ppul4
更新日期:2000-02-01 00:00:00
abstract:BACKGROUND:Pressure rise time (PRT), also known as slope time to the peak inflating pressure can be set on some modern neonatal ventilators. On other ventilators, PRT is determined by the set circuit flow. Changing slope time can affect mean airway pressure (MAP), oxygenation, and carbon dioxide elimination. Our aim wa...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.24724
更新日期:2020-05-01 00:00:00
abstract:OBJECTIVES:To report the respiratory function of school-aged children with infantile Pompe disease (IPD) who started enzyme replacement therapy (ERT) in infancy and early childhood. STUDY DESIGN:This is a retrospective chart review of pulmonary function tests of: (a) patients with IPD 5 to 18 years of age, (b) who wer...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24621
更新日期:2020-03-01 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: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::Deficiency of mannose-binding lectin has been shown to be a risk factor for cystic fibrosis (CF) patients. We, therefore, decided to treat a patient with CF, mannose-binding lectin deficiency, severe bronchopulmonary Pseudomonas aeruginosa infection, and rapid deterioration of lung function with purified mannose-bindi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10064
更新日期:2002-03-01 00:00:00
abstract:OBJECTIVES:First, create a clinical severity score for patients with chronic lung disease of infancy (CLDi) following neonatal intensive care unit (NICU) stay. Second, using California wide population-based data, identify factors associated with clinical severity of CLDi at 4-9 months corrected gestational age (CGA). ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23148
更新日期:2015-09-01 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: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::We used an in vitro model system to examine the sites of deposition of aqueous therapeutic aerosols administered through 3-mm, 6-mm, and 9-mm endotracheal tubes (commonly used in infants, children, and adults) at clinically relevant inspiratory flow rates. Aerosol was delivered to the endotracheal tube via a "T" piece...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020108
更新日期:1986-01-01 00:00:00
abstract::The prevalence and morbidity of asthma are growing problems that appear to affect urban populations and particularly impoverished centers disproportionately. Poor children are more likely to be hospitalized for asthma than nonpoor children, and are more likely to experience clinical and social dysfunction due to asthm...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.10029
更新日期:2002-01-01 00:00:00