Abstract:
BACKGROUND:Disease burden in cystic fibrosis (CF) impacts quality of life, distress, and treatment adherence. The promoting resilience in stress management (PRISM), is a brief patient-focused intervention to promote resilience in adolescents and young adults (AYAs), which may mitigate the negative outcomes, and is proven to be feasible and acceptable in other diseases. OBJECTIVE:Our aim was to test the feasibility and acceptability of PRISM among AYAs with CF in addition to collecting pilot data regarding patient-reported resilience, distress, and quality of life. METHODS:Eligible English speaking, 12 to 21 year patients admitted to the hospital were enrolled. We defined feasibility as 80% completion of all sessions. Acceptability was defined qualitatively based on feedback about timing, content and delivery of intervention. As an exploratory aim, questionnaires measuring resilience (Connor-Davidson resilience scale), distress (Kessler-6 scale), and disease-specific health-related quality of life (CF questionnaire-revised [CFQ-R]) were given at baseline and postintervention. RESULTS:10 out of 17 (59%) patients consented to participate. Eight were Caucasian, eight female with age range 13 to 20 years (median: 18). Nine completed all PRISM sessions with universally positive feedback. Health perception and respiratory domain scores of the CFQ-R improved (47.2-65.1; 95% confidence interval [CI], 2.6-35.6; 50.9-61.9; 95% CI, 1.7-19.9, respectively), however in the setting of inpatient exacerbation treatment it would be hard to attribute these changes to PRISM. CONCLUSION:PRISM was feasible and highly acceptable among AYAs with CF. Future research is needed to test the efficacy of PRISM among a larger group of patients with CF in a multicenter trial.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Toprak D,Nay L,McNamara S,Rosenberg AR,Rosenfeld M,Yi-Frazier JPdoi
10.1002/ppul.24574subject
Has Abstractpub_date
2020-03-01 00:00:00pages
638-645issue
3eissn
8755-6863issn
1099-0496journal_volume
55pub_type
临床试验,杂志文章abstract::The purpose of the study was to analyze the respiratory-dependent intraarterial blood pressure variations (pulsus paradoxus, PP) in intensive care unit patients with obstructive and restrictive pulmonary disease, and to evaluate the usefulness of PP for assessment of disease severity. One-minute paper recordings of ar...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200102)31:2<138::aid-ppul1022>3.
更新日期:2001-02-01 00:00:00
abstract::There is a need to judge general exercise tolerance in children with cystic fibrosis (CF) under normal daily activity conditions and -when more extensive testing is required-in an exercise laboratory in a specialized center. We investigated the reproducibility, validity, and criterion for a 6-minute walking test, whic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(SICI)1099-0496(199608)22:2<85::AID-PPUL1>
更新日期:1996-08-01 00:00:00
abstract:OBJECTIVES:We evaluated safety and efficacy of recombinant human growth hormone (rhGH) for improving growth, lean body mass (LBM), pulmonary function, and exercise tolerance in children with cystic fibrosis (CF) and growth restriction. STUDY DESIGN:Multicenter, open-label, controlled clinical trial comparing outcomes ...
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.21546
更新日期:2012-03-01 00:00:00
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 treatmen...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24563
更新日期:2020-01-01 00:00:00
abstract::Inhaled corticosteroids are commonly used in cystic fibrosis (CF), but there are few studies evaluating their safety in young children. We, therefore, prospectively administered beclomethasone diproprionate (BDP) to 12 clinically stable young children with CF to examine the safety of this therapy with respect to adren...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1122
更新日期:2001-10-01 00:00:00
abstract:OBJECTIVES:This study was designed to establish a porcine model of reversible obstruction of endobronchial foreign body (FB) and to assess the natural pulmonary reexpansion process using radiology and histopathology after removal of the bronchial FB. METHODS:A metal stent was placed in the right lower lobe bronchial l...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23947
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Community-acquired pneumonia (CAP) is a major cause of childhood morbidity and mortality worldwide. The angiotensin-converting enzyme (ACE) gene is a potential candidate gene for CAP risk. OBJECTIVES:In this study, we aimed to investigate whether the ACE insertion/deletion (I/D) polymorphism (rs4340) could ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23886
更新日期:2017-12-01 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:OBJECTIVE:Extremely low gestational age neonates (ELGANs) are at risk for pulmonary hypertension (PH). We hypothesized that PH, defined by echocardiogram at 36 weeks gestational age (GA), would associate with respiratory morbidity, increased oxidant stress, and reduced nitric oxide production. STUDY DESIGN:ELGANs in t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23913
更新日期:2018-03-01 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::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:SUMMARY:Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder caused by mutations in the ATM gene, resulting in faulty repair of breakages in double-stranded DNA. The clinical phenotype is complex and is characterized by neurologic abnormalities, immunodeficiencies, susceptibility to malignancies, recurren...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21277
更新日期:2010-09-01 00:00:00
abstract::Previous research has demonstrated a significant reciprocal relationship between psychosocial factors and asthma morbidity in children. The National Cooperative Inner-City Asthma Study investigated both asthma-specific and non-specific psychosocial variables, including asthma knowledge beliefs and management behavior,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/(sici)1099-0496(199710)24:4<263::aid-ppul5
更新日期:1997-10-01 00:00:00
abstract::This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absen...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10285
更新日期:2003-06-01 00:00:00
abstract::Patients with cystic fibrosis (CF) often experience acute pulmonary exacerbations (APE) and may be treated with a wide variety of intravenous antibiotics. The aim of this review is to provide an evidence-based summary of pharmacokinetic/pharmacodynamic (PK/PD), tolerability, and efficacy studies utilizing the intraven...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22664
更新日期:2013-01-01 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::The efficacy and tolerability of high-dose salmeterol (100 mcg, BID) and albuterol (2.5 mg, BID) were compared with those of albuterol (2.5 mg, BID) in outpatients with cystic fibrosis in a randomized, double-blind, double-dummy, placebo-controlled, crossover study with both short- (4 weeks of each) and long-term (24 ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.10162
更新日期:2002-10-01 00:00:00
abstract::The impulse oscillometry system (IOS) was introduced as a new technique to assess airflow obstruction in patients who are not able to perform forced breathing maneuvers, e.g., subjects with cerebral palsy or severe mental retardation, and young children. This study evaluates the sensitivity and specificity of IOS para...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10235
更新日期:2003-03-01 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:Lung involvement in children with Niemann-Pick disease has rarely been studied systematically. OBJECTIVE:To assess the involvement of the lung and the value of bronchoalveolar lavage in children with Niemann-Pick diseases. DESIGN:Retrospective analysis of patient records. PATIENTS:Thirteen patients, with ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20725
更新日期:2007-12-01 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:RATIONALE:Nitric oxide (NO) produced in the lung is an important mediator of normal lung development, vascular smooth muscle relaxation, and ventilation perfusion matching. NO is synthesized from arginine by the action of NO-synthase (NOS). Asymmetric dimethylarginine (ADMA), an endogenous derivate of arginine, inhibit...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20886
更新日期:2008-12-01 00:00:00
abstract:BACKGROUND:sTREM-1 (soluble triggering receptor expressed on myeloid cells-1) is a novel inflammatory marker that may be of clinical use in cystic fibrosis (CF). Dysregulation of the TREM pathway has been demonstrated in other inflammatory diseases and modulation in animal models has therapeutic benefit. We hypothesise...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23650
更新日期:2017-04-01 00:00:00
abstract:OBJECTIVE:Lung ultrasound (LUS) is an emerging tool that may be used in the diagnosis and follow-up of children with viral bronchiolitis. In this study, we describe LUS abnormalities in children receiving invasive mechanical ventilation (IMV) for severe bronchiolitis in the pediatric intensive care unit (PICU). Our aim...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24974
更新日期:2020-10-01 00:00:00
abstract::During the final prenatal period of fetal lung development in humans, important maturational processes occur, including the production of surfactant necessary to decrease surface tension at the air-liquid interface of the alveoli. During early gestation, the glucocorticoid receptor is expressed in the fetal lung, and ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.1092
更新日期:2001-07-01 00:00:00
abstract::Most pneumatoceles disappear spontaneously and do not cause severe symptoms. Treatment alternatives include various conventional or surgical methods. However, an enlarging, complicated pneumatocele with cardiorespiratory instability requires imaging-guided catheter drainage or surgery. Here, we report the case of a ne...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23311
更新日期:2016-02-01 00:00:00
abstract::Bronchiolitis in infancy is a risk factor for development of asthma in the first decades of life, although the majority may be asymptomatic at school age. Respiratory symptoms are common in early life, and prediction of later asthma may be challenging. We aimed to study if simple clinical variables assessed at 2 years...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22675
更新日期:2013-06-01 00:00:00
abstract::Specific airway resistance (SRaw) is conventionally determined by multiplying the plethysmographically measured values of airway resistance and functional residual capacity (FRC). An alternative single-step method, which avoids the need for airway occlusion during determination of FRC, has been described by Dab and Al...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060212
更新日期:1989-01-01 00:00:00
abstract::We report a case of fungal pleural effusion secondary to presumed valproate induced pancreatitis with pseudocyst and stricture formation. A child with dyskinetic cerebral palsy who had been on sodium valproate for several years was transferred for drainage of a left sided pleural effusion. Pleural fluid culture consis...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20982
更新日期:2009-06-01 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