Abstract:
:Plasminogen deficiency is characterized by fibrin-rich pseudomembrane formation on all mucosal surfaces, particularly the conjunctiva. Respiratory system involvement is common; fibrin often obstructs the upper or lower respiratory tract, causing death. Although many treatments have been applied, no definitive treatment (especially of the respiratory involvement) yet exists. Although excision of tracheobronchial tree membranes affords temporary improvement, this should be performed only for patients in severe respiratory distress; the procedure triggers fibrin redeposition. Here, we share our experience with an 8-year-old plasminogen-deficient female with severe lung involvement; we successfully delivered local tissue plasminogen activator followed by nebulized fresh frozen plasma.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Kilinc AA,Tarcin G,Kurugoglu S,Schuster V,Cokugras H,Celkan Tdoi
10.1002/ppul.24563subject
Has Abstractpub_date
2020-01-01 00:00:00pages
E1-E3issue
1eissn
8755-6863issn
1099-0496journal_volume
55pub_type
杂志文章abstract:RATIONALE:Asthma is one of the most common chronic disorders of childhood and is associated with significant healthcare utilization and costs. Comorbid psychiatric illnesses, specifically depression and anxiety, are more prevalent in patients with asthma and associated with worse asthma control, more emergency departme...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25061
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:There are limited data on the effect of bronchopulmonary dysplasia (BPD) on sleep disordered breathing (SDB). We hypothesized that both the severity of prematurity and BPD would increase the likelihood of SDB in early childhood. Our secondary aim was to evaluate the association of demographic factors on the ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23769
更新日期:2017-12-01 00:00:00
abstract::Short-acting β2-adrenergic receptor agonists are commonly used bronchodilators for symptom relief in asthmatics. Recent evidence demonstrated that prolonged exposure of cultured airway smooth muscle cells to β2 agonists directly augments procontractile signaling pathways with the change in expression of regulator of G...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22723
更新日期:2013-10-01 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::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:OBJECTIVES:Forced expiratory volume in 1 second (FEV1) is an important predictor of outcome in cystic fibrosis (CF). This study aimed to describe the change in lung function, nutritional status, and mortality of children with CF at a single center in Cape Town, South Africa, and identify factors associated with poor lu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24726
更新日期:2020-06-01 00:00:00
abstract::To promote early screening of patients with suspected Primary Ciliary Dyskinesia (PCD), nasal nitric oxide (nNO) measurements during tidal breathing (TB) have been developed for children unable to ensure velum closure (VC) during breath hold or expiration against resistance. To investigate technical and practical issu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.23167
更新日期:2015-12-01 00:00:00
abstract:BACKGROUND:We hypothesized airway inflammation can be detected non-invasively by induced sputum (IS) or peripheral blood eosinophilia, and IS can detect bacterial and viral infection in preschool children with airway disease, with results comparable to broncho-alveolar lavage (BAL). METHODS:Preschool children with cys...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23366
更新日期:2016-08-01 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: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::Lane-Hamilton syndrome refers to the uncommon co-occurrence of idiopathic pulmonary hemosiderosis and celiac disease (CD). Three children aged between 7 and 14 years with IPH were detected to have co-existing non-diarrheal CD. Institution of gluten-free diet in each of the three children resulted in amelioration of th...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21357
更新日期:2011-03-01 00:00:00
abstract::Tracheal bronchus (TRB) has been generally considered an anatomical variant of the tracheobronchial tree without a precise pathological effect. Its prevalence is estimated to be between 0.2% to 3% of all children undergoing bronchoscopy and scientific information has been limited to case reports or small case series. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.24435
更新日期:2019-10-01 00:00:00
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::In past decades, several chest radiograph scoring systems for cystic fibrosis were developed. This study was performed to compare interobserver variability of six different radiograph scores and to correlate them with clinical parameters. Thirty chest radiographs of 30 patients with cystic fibrosis were scored accordi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10280
更新日期:2003-06-01 00:00:00
abstract::Intrauterine inflammation generates inflammatory mediators that damage the developing bronchoalveolar epithelium, resulting in neonatal lung injury. Lung fluid transport disorders are the main reasons for the development of pulmonary edema, an important pathology of lung injury. Previous studies suggested that epithel...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24242
更新日期:2019-03-01 00:00:00
abstract::Our objective was to determine the repeatability of bronchial responsiveness to mannitol dry powder (MDP) as an objective marker of asthma in children. MDP challenge was performed in children with stable asthma at the same time of the day on two separate occasions within a week. The test was terminated after a 15% fal...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.10378
更新日期:2003-12-01 00:00:00
abstract::Respiratory system compliance (Crs) can be used to assess lung stiffness in sick, intubated infants, avoiding the use of an esophageal balloon. Crs was assessed in a group of 15 sick, intubated infants using the occlusion and inflation techniques. The occlusion technique gave satisfactory results in 13 infants. Apneic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030207
更新日期:1987-03-01 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::A new method that permits the measurement of adult-type maximal expiratory flow-volume curves and fractional lung volumes in sedated infants was recently described. The purpose of this study was to define the normal range for these new measures of pulmonary function in infants and young children. Measurements of force...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/1099-0496(200009)30:3<215::aid-ppul6>3.0.c
更新日期:2000-09-01 00:00:00
abstract::Pulmonary surfactant, a unique developmentally regulated, phospholipid-rich lipoprotein, is synthesized by the type II epithelial cells (AECII) of the pulmonary alveolus, where it is stored in organelles termed lamellar bodies. The synthesis of pulmonary surfactant is under multifactorial control and is regulated by a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22975
更新日期:2014-09-01 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::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:BACKGROUND:Plastic bronchitis (PB) is a rare, variable, and potentially fatal disease. This study aimed to assess the efficacy of fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL) in treating children with PB. METHODS:In total, 15 children with PB, between 2012 and 2020, were enrolled in our study. Within...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25016
更新日期:2020-11-01 00:00:00
abstract::It has been suggested that cystic fibrosis (CF) patients harboring multiresistant (MR) Pseudomonas aeruginosa (PA) should be seen in separate clinics. The aim of this study was to test the feasibility of this by longitudinally studying the consistency of isolates of MRPA in individuals. We analyzed all respiratory tra...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10262
更新日期:2003-04-01 00:00:00
abstract::It has been suggested that children with asthma recover more quickly from exercise-induced bronchoconstriction than adults. On the basis of clinical observation we hypothesized that recovery rate from exercise-induced asthma (EIA) in childhood also decreases with age. In 14 children (aged 7-12 years) with a history of...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1950200309
更新日期:1995-09-01 00:00:00
abstract::We examined the effects of within-breath changes in compliance (C) upon the accuracy of measurements of compliance and resistance (R) by linear regression analysis and by Mead and Wittenberger's method. These effects were illustrated by a computer model and by lung models with linear and nonlinear pressure/volume rela...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950120304
更新日期:1992-03-01 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::We tested a health education intervention program to reduce passive smoking in infancy. The aim was to develop an instrument for study of tobacco smoke exposure and childhood respiratory illness. One hundred and eighty-four women who had smoked during pregnancy were allocated by month of delivery to an intervention gr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030311
更新日期:1987-05-01 00:00:00
abstract::Lung transplantation in childhood is a highly specialized clinical practice confined to a few centers around the world. Organ availability remains an important limiting factor in extending the application of this procedure to more infants, children and adolescents. The lungs are the organ most vulnerable to injury, in...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20997
更新日期:2009-06-01 00:00:00
abstract:PURPOSE:To investigate the common indications for polysomnogram (PSG) associated co-morbid conditions, evaluation strategies, treatment options, and outcomes in a series of infants diagnosed with obstructive sleep apnea (OSA) by a PSG. METHODS:Retrospective chart review of infants who underwent PSG over a 7-year perio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22867
更新日期:2014-06-01 00:00:00