Abstract:
:The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment. We present three cases where the use of Mycophenolate in the context of autoimmune cytopenias proved beneficial also on the lung disease.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Bucciol G,Petrone A,Putti MCdoi
10.1002/ppul.23757subject
Has Abstractpub_date
2017-10-01 00:00:00pages
E73-E76issue
10eissn
8755-6863issn
1099-0496journal_volume
52pub_type
杂志文章abstract::The analysis of exhaled breath condensate (EBC) is a promising new method to measure airway inflammation. So far only limited data exist about methodological issues of EBC sampling in infants and young children. We evaluated 18 children with acute wheezy bronchitis (median age 24.3 months (min-max: 4-89.9)), 54 childr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20712
更新日期:2007-12-01 00:00:00
abstract::There is increasing interest in using the Multiple Breath Washout technique and the lung clearance index (LCI) for detecting early pulmonary changes, for example, in cystic fibrosis lung disease. However, there are still limited data regarding equipment specific reference ranges, repeatability and reproducibility. The...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21093
更新日期:2009-12-01 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:BACKGROUND:How pediatricians manage bronchiolitis and the derived total costs (direct and indirect) in the emergency department (ED) have not been fully characterized. The aim of the present study is to calculate those costs in a European country. METHODS:A prospective and observational study, including 10 EDs of tert...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.22906
更新日期:2014-10-01 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::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: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::Standard chemotherapy for tuberculosis (TB) in children uses hepatotoxic drugs. Published data and guidelines on monitoring of liver function during TB treatment are often contradictory and not directly relevant to the pediatric population. We carefully monitored 43 children (age 6.6 years, 0.7-15.1 [median, range]; 4...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1099-0496(199901)27:1<37::aid-ppul8>
更新日期:1999-01-01 00:00:00
abstract:BACKGROUND:Social and family factors may influence the probability of achieving asthma control in children. Parents' quality of life has been insufficiently explored as a predictive factor linked to the probability of achieving disease control in asthmatic children. OBJECTIVE:Determine whether the parents' quality of ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23336
更新日期:2016-07-01 00:00:00
abstract::Pseudomonas aeruginosa is often cultured from the airways of children with tracheostomies. P. aeruginosa produces exotoxin A (ETA) and type III cytotoxins. This study tested the hypothesis that children with tracheostomies are colonized by P. aeruginosa that express these virulence factors and will have antibodies dir...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20194
更新日期:2005-05-01 00:00:00
abstract:RATIONALE:Whether asthma constitutes a risk factor for coronavirus disease-2019 (COVID-19) is unclear. Here, we aimed to assess whether asthma, the most common chronic disease in children, is associated with higher COVID-19 risk or severity in pediatric populations. METHODS:We performed a systematic literature search ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,meta分析
doi:10.1002/ppul.24909
更新日期:2020-09-01 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::We determined the effects of an immediately antecedent viral lower respiratory tract infection (LRI) on the severity of clinical illness, changes in lung function and airway histamine responsiveness produced by a subsequent LRI in 9-12 week old beagle puppies inoculated with canine adenovirus 2, followed in 2 weeks by...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950100403
更新日期:1991-01-01 00:00:00
abstract:INTRODUCTION:Interpregnancy interval (IPI) is defined as the period between a live birth and the conception of a subsequent fetus. Both short (IPI < 6 months) and long IPI (IPI > 60 months) have been shown to increase the risk for adverse perinatal outcomes, some of which, are known risk factors for obstructive sleep a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25240
更新日期:2020-12-31 00:00:00
abstract:OBJECTIVE:To carry out a cost-effectiveness analysis of omitting chest radiography in the diagnosis of infant bronchiolitis. HYPOTHESIS:Omitting chest radiographs in the diagnosis of typical bronchiolitis was expected to reduce costs without adversely affecting the detection rate of alternate diseases. STUDY DESIGN:A...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20948
更新日期:2009-02-01 00:00:00
abstract::Follow-up studies have demonstrated that bronchiolitis caused by respiratory syncytial virus (RSV) is strongly associated with wheezing in the ensuing years. During the acute infection the immune response may induce long-lasting detrimental effects, thereby contributing to post-bronchiolitis wheezing (PBW). Therefore,...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/1099-0496(200008)30:2<92::aid-ppul3>3.0.co
更新日期:2000-08-01 00:00:00
abstract::We determined lung function at age 10 years in very low birthweight (VLBW, journal_title:Pediatric pulmonology pub_type: 杂志文章 doi:10.1002/ppul.20662 更新日期:2007-09-01 00:00:00
abstract::Although Mycobacterium fortuitum (MF) is a non-tuberculous mycobacterium that rarely causes disease, there are reported cases of pneumonia, lung abscess, and empyema in subjects with predisposing lung disease. We report a neonate, without predisposing disease or risk factors, who manifested pneumonia and lung abscess....
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22558
更新日期:2012-10-01 00:00:00
abstract::We present a child with interstitial lung disease (ILD) who was diagnosed with desquamative interstitial pneumonitis following CT thorax and open lung biopsy aged 2 years. Subsequently, surfactant protein gene analysis revealed mutations in the ABCA3 gene. This case of ABCA3 deficient ILD describes a stable clinical c...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22811
更新日期:2014-03-01 00:00:00
abstract::Measurements of thoracic gas volume (TGV), airway resistance (Raw), and airway conductance (Gaw) were calculated in a group of 42 normal infants using a whole-body plethysmograph. Maximum expiratory flow at functional residual capacity was measured in a separate group of 108 normal infants. Using data obtained from th...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950140303
更新日期:1992-11-01 00:00:00
abstract::Pneumomediastinum is uncommon in pediatric medical practice, outside the neonatal period. While asthma or respiratory infections are the most frequent underlying causes, it is important not to forget the possibility of foreign body aspiration, particularly after the clinical presentation. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10295
更新日期:2003-07-01 00:00:00
abstract:AIM:To analyze breathing pattern and mechanical ventilatory constraints during incremental exercise in healthy and cystic fibrosis (CF) children. METHODS:Thirteen healthy children and 6 children with cystic fibrosis volunteered to perform an incremental test on a treadmill. Exercise tidal flow/volume loops were plotte...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22804
更新日期:2014-03-01 00:00:00
abstract::A continuous subcutaneous infusion of terbutaline (CSIT) was used to treat 8 children with chronic severe asthma who continued to experience frequent symptoms, despite treatment with regular oral prednisolone. Five patients experienced a symptomatic improvement from CSIT, leading to a reduction in regular medication. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10081
更新日期:2002-05-01 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::Electronic compensation to overcome thermal artifacts during plethysmographic estimations of airway resistance is now used routinely in adults and school-age children, and was shown to be a valuable means of discriminating airway function between preschool children with and without lung disease. A similar system is no...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.20217
更新日期:2005-07-01 00:00:00
abstract:OBJECTIVES:To determine 1) what proportion of abstracts of randomized controlled trials (RCTs) presented at international conferences on cystic fibrosis (CF) are published as full reports, 2) time to publication, and 3) factors that might delay or prevent publication. METHODS:At the end of 1995, the Cochrane CF Group'...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199808)26:2<101::aid-ppul5
更新日期:1998-08-01 00:00:00
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::There is currently no validated scoring system for quantification of airway secretions in children. A user friendly, valid scoring system of airway secretions during flexible bronchoscopy (FB) would be useful for comparative purposes in clinical medicine and research. The objective of this study was to validate our br...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20478
更新日期:2006-09-01 00:00:00
abstract::Polyvinyl chloride (PVC) nasogastric feeding catheters are used clinically to measure intraesophageal pressure as an estimate of pleural pressure for calculating lung compliance in infants. The accuracy of pressure measurement of 4 French gauge (FG) catheter sizes and three brands of liquid-filled catheter manometer s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199705)23:5<362::aid-ppul8
更新日期:1997-05-01 00:00:00
abstract::Indoor aeroallergen exposures increased asthma symptoms in Caucasians, but their determinants and relationship to asthma and allergy in Asians are unclear. This study investigated exposures to cat, cockroach, and Blomia tropicalis allergens in 115 Hong Kong families with asthmatic children. Patients underwent exhaled ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21391
更新日期:2011-07-01 00:00:00