Abstract:
:Diffuse panbronchiolitis is a progressive fatal respiratory disease of unknown cause seen predominantly in Southeast Asian adults. We report this condition in a 10-year-old child of Korean birth because of the uncommon presentation at this age and the favorable outcome associated with early diagnosis. Our objective was also to demonstrate the gradual but complete resolution of the disease and sustained remission from early institution of azithromycin.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Weinberger M,Fischer A,Kao Sdoi
10.1002/ppul.23176subject
Has Abstractpub_date
2015-09-01 00:00:00pages
E32-4issue
9eissn
8755-6863issn
1099-0496journal_volume
50pub_type
杂志文章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:BACKGROUND:The aim of this study was to investigate the association among a validated symptom-based questionnaire for asthma control in children (CAN), forced expiratory volume in 1 sec (FEV(1)), and fractional exhaled nitric oxide (FE(NO)). METHODS:Observational cross-sectional study was performed in a consecutive sa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21144
更新日期:2010-05-01 00:00:00
abstract:OBJECTIVE:To determine whether non-invasive positive pressure ventilation (NIPPV) delivered via nasal prongs can alleviate the need for tracheal intubation and invasive ventilation in infants admitted to the pediatric intensive care unit (PICU) with impending respiratory failure, and to find predictive factors for succ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22561
更新日期:2012-10-01 00:00:00
abstract::A potentially useful method to monitor respiratory mechanics in artificially ventilated patients consists of analyzing the relationship between tracheal pressure (P), lung volume (V), and gas flow (V) by multiple linear regression (MLR) using a suitable model. Contrary to other methods, it does not require any particu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950130307
更新日期:1992-07-01 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 aim of our study was to examine the effect of labor and birth canal compression on the rate of clearance of lung fluid. We recruited 10 babies born by vaginal delivery and 10 born by elective cesarean section (ECS). Thoracic gas volume (TGV) was measured using total body plethysmography, and functional residual ca...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199905)27:5<318::aid-ppul4
更新日期:1999-05-01 00:00:00
abstract:OBJECTIVE:To determine if fetal growth restriction (FGR) in the setting of sterile intra-uterine milieu would be associated with a decrease in respiratory distress syndrome (RDS) of preterm-neonates. METHODS:The relationship between FGR and neonatal RDS was examined in 92 singleton preterm-neonates (gestational age [G...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23369
更新日期:2016-08-01 00:00:00
abstract::Assessment of dynamic inspiratory function may provide valuable information about the degree and progression of pulmonary involvement in patients with Duchenne muscular dystrophy (DMD). The aims of this study were to characterize inspiratory function and to assess the efficacy of idebenone on this pulmonary function o...
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.23547
更新日期:2017-04-01 00:00:00
abstract::Eight patients with cystic fibrosis [CF] colonized with Pseudomonas aeruginosa (P. aeruginosa) had serial lung function, peripheral blood inflammatory markers, and serum IgG antibodies to Burkholderia cepacia (B. cepacia) lipopolysaccharide measured in the months preceding and following colonisation with B. cepacia. O...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200001)29:1<8::aid-ppul2>3
更新日期:2000-01-01 00:00:00
abstract:OBJECTIVES:We sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co-morbidities, high resolution computed tomography findings, and outcomes. METHODS:With institutional review board approval, we performed a retrospective review of patients with biopsy-proven P.I.G. Bio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24123
更新日期:2018-12-01 00:00:00
abstract::In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1099-0496(200005)29:5<382::aid-ppul7
更新日期:2000-05-01 00:00:00
abstract::Our aim was to determine whether antenatal corticosteroids improve perinatal adaptation of the pulmonary circulation in lambs with lung hypoplasia (LH). LH was induced in 12 ovine fetuses between 105 and 140 days gestation (term approximately 147 days); in 6 of these the ewe was given a single dose of betamethasone (1...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20453
更新日期:2006-09-01 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:OBJECTIVE:To validate the bronchiolitis score of Sant Joan de Déu (BROSJOD) and to examine the previously defined scoring cutoff. PATIENTS AND METHODS:Prospective, observational study. BROSJOD scoring was done by two independent physicians (at admission, 24 and 48 hr). Internal consistency of the score was assessed us...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23546
更新日期:2017-04-01 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:INTRODUCTION:Laryngomalacia is the most prevalent cause of congenital stridor. Flexible laryngobronchoscopy (FLB) is the gold standard for diagnosis. However, FLB requires venous access, deep sedation or general anesthesia, and is associated with patient's and parental discomfort and a considerable cost. Laryngeal ultr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23964
更新日期:2018-06-01 00:00:00
abstract::Our purpose was to identify and further characterize physiologic mechanisms relevant to autoresuscitation from hypoxic apnea in infants dying suddenly and unexpectedly. We studied cardiorespiratory recordings of 24 infants (age range, 0.8-21 months) who died suddenly while being monitored at home. These recordings wer...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10287
更新日期:2003-08-01 00:00:00
abstract::Tracheal mucosal damage has been reported in autopsy specimens of cases with proximal esophageal atresia and distal tracheoesophageal fistula (EA-TEF) (Gross classification type C). Such changes have not been reported for isolated EA (Gross classification type A). Our hypothesis is that passage of amniotic fluid (AF) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20490
更新日期:2006-11-01 00:00:00
abstract::Extralobar pulmonary sequestration is a congenital lung malformation characterized by a non-functional lung segment with systemic feeding vessel. Over 90% of sequestrations are found in the thorax with less than 10% located in the abdomen. We present an unusual case of intra abdominal pulmonary sequestration, located ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22891
更新日期:2014-05-01 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:Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM:The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHODS:Participants underwe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23477
更新日期:2017-01-01 00:00:00
abstract::Inadequate intake and suboptimal growth are common problems for patients with CF and a critical target for intervention. The purpose of this study was to compare the growth outcomes of children with CF who participated in a randomized clinical trial to improve energy intake and weight to children with CF receiving sta...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21322
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Ivacaftor is a significant innovation in the treatment of cystic fibrosis (CF) with gating mutations. A substantial percentage of patients with CF have severe lung involvement, but these patients are usually excluded from phase III clinical trials. Thus, the effectiveness of ivacaftor in this population has ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24424
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND:Chronic colonization with Pseudomonas (P.) aeruginosa worsens the prognosis of cystic fibrosis (CF) patients. This study aims to analyze the functional properties of neutrophils in CF patients with P. aeruginosa colonization. METHODS:Patients with CF (n = 16) were grouped by positivity of P. aeruginosa in s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25294
更新日期:2021-01-29 00:00:00
abstract::Atrial natriuretic peptide (ANP) has known natriuretic, diuretic, and vasodilatatory effects. It is synthesized and stored in the atrial cells. Stretching of the atrial muscle fibers during an increase in venous return sets a response of ANP release into the blood stream. High levels of ANP were measured in a number o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20233
更新日期:2005-10-01 00:00:00
abstract::This study evaluates different ventilator strategies during gas (GV) and partial liquid ventilation (PLV) in spontaneously breathing animals. We hypothesized that during PLV, spontaneously breathing animals would self-regulate respiratory parameters by increasing respiratory rate (RR) and minute ventilation (V'E) when...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199811)26:5<319::aid-ppul3
更新日期:1998-11-01 00:00:00
abstract::Angiogenesis is an important mechanism of airway remodeling in lung disease. We previously demonstrated that serum vascular endothelial growth factor (VEGF) is elevated in cystic fibrosis (CF) patients and declines with therapy for pulmonary exacerbation. We hypothesized that VEGF is elevated early in the course of CF...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21428
更新日期:2011-07-01 00:00:00
abstract::Changes in the relative contribution of ribcage and abdomen during augmented breaths were assessed in two groups of infants (less than 2 weeks and 4-12 weeks old). In both groups ribcage and abdominal movements increased during augmented breaths in quiet sleep. In the older infants the relative increase was greater fo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950040303
更新日期:1988-01-01 00:00:00
abstract::We have evaluated the role of eosinophil cationic protein (ECP) concentrations in serum in predicting wheezing after bronchiolitis, during infancy and early childhood. A prospective study at a university hospital serving all pediatric patients in a defined area was designed. Serum ECP concentrations were measured in 9...
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(199706)23:6<397::aid-ppul1
更新日期:1997-06-01 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