Cystic fibrosis database (CFDB): a new web-based tool for cystic fibrosis specialists.

Abstract:

:In order to help specialists involved in CF care and clinical research to know the current best evidence about clinical effectiveness of interventions in CF, we designed and developed a web-based, free access tool called "CFDB"--Cystic Fibrosis DataBase (www.inetflow.it/CFDB). The database was built by searching in Medline, Embase, Cochrane Library and worldwide trials registries all studies involving clinical interventions in CF. The tool lets the user define queries starting from one or more types of pathological conditions and one or more interventions. The output of the queries is structured in three levels: (1) how many and which studies deal with the conditions formulated in the query; (2) which are the main results of these studies; (3) a critical summary of the literature related to the query. This tool, providing a quick overview of the available evidence in clinical research in CF, may help clinical decision making, designing of new trials and building guidelines.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Buzzetti R,Cirilli N,Minicucci L,Raia V,Salvatore D,Maffeis P

doi

10.1002/ppul.22954

subject

Has Abstract

pub_date

2014-09-01 00:00:00

pages

938-40

issue

9

eissn

8755-6863

issn

1099-0496

journal_volume

49

pub_type

信件
  • Relationship of symptoms to lung function abnormalities in preterm infants at follow-up.

    abstract::Recurrent respiratory symptoms are common in preterm infants in the first 2 years of life. The aim of this study was to determine the lung function abnormalities associated with such symptoms. Forty preterm infants, with a median gestational age of 29 weeks were studied at a median postnatal age of 12 months. Twenty-t...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950110304

    authors: Yuksel B,Greenough A

    更新日期:1991-01-01 00:00:00

  • Hering-Breuer reflex, lung volume and position in prematurely born infants.

    abstract:OBJECTIVES:To investigate the effect of position on the strength of the Hering-Breuer reflex in prematurely born infants and determine whether any differences seen were related to differences in lung or tidal volume between positions. WORKING HYPOTHESIS:Position related differences in the strength of the Hering-Breuer...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.20855

    authors: Landolfo F,Saiki T,Peacock J,Hannam S,Rafferty GF,Greenough A

    更新日期:2008-08-01 00:00:00

  • Socioeconomic status and risk of hospitalization among individuals with cystic fibrosis in Ontario, Canada.

    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

    authors: Stephenson A,Hux J,Tullis E,Austin PC,Corey M,Ray J

    更新日期:2011-04-01 00:00:00

  • Differential effects of chronic intermittent and chronic constant hypoxia on postnatal growth and development.

    abstract::Exposure to chronic constant or intermittent hypoxia (CCH or CIH) may have different effects on growth and development in early life. In this work, we exposed postnatal day 2 (P2) CD1 mice to CCH or CIH (11% O2) for 4 weeks and examined the effect of hypoxia on body and organ growth until P30. Regression analysis show...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.20729

    authors: Farahani R,Kanaan A,Gavrialov O,Brunnert S,Douglas RM,Morcillo P,Haddad GG

    更新日期:2008-01-01 00:00:00

  • Exhaled breath condensate pH in infants and children with acute and recurrent wheezy bronchitis.

    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

    authors: Vogelberg C,Würfel C,Knoetzsch A,Kahlert A,Range U,Leupold W

    更新日期:2007-12-01 00:00:00

  • Prevalence of respiratory syncytial virus infection in Italian infants hospitalized for acute lower respiratory tract infections, and association between respiratory syncytial virus infection risk factors and disease severity.

    abstract::This study was designed to collect data on the prevalence of respiratory syncytial virus (RSV) infection in Italy in infants hospitalized for lower respiratory tract infections, and to evaluate which of the recognized risk factors might be associated with disease severity. Thirty-two centers throughout Italy participa...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章,多中心研究

    doi:10.1002/ppul.10047

    authors: Lanari M,Giovannini M,Giuffré L,Marini A,Rondini G,Rossi GA,Merolla R,Zuccotti GV,Salvioli GP,Investigators R.A.DA.R. Study Group.

    更新日期:2002-06-01 00:00:00

  • Glucocorticoids and lung development in the fetus and preterm infant.

    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

    authors: Bolt RJ,van Weissenbruch MM,Lafeber HN,Delemarre-van de Waal HA

    更新日期:2001-07-01 00:00:00

  • Humoral immune response against 38- and 16-kDa mycobacterial antigens in childhood tuberculosis.

    abstract::Several enzyme-linked immunosorbent assays (ELISAs) based on mycobacterial antigens have been tried for the rapid diagnosis of tuberculosis (TB). In this study, the value of the 16 and 38-kDa mycobacterial antigens in the diagnosis of TB was investigated in pediatric patients in Izmir, Turkey in whom they were found u...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.20901

    authors: Senol G,Ecevit C,Oztürk A

    更新日期:2009-09-01 00:00:00

  • Diaphragmatic electromyography in preterm infants: The influence of electrode positioning.

    abstract:OBJECTIVE:To determine the effect of changing electrode positions on vital signs and respiratory effort parameters measured with transcutaneous electromyography of the diaphragm (dEMG) in preterm infants. METHODS:In this observational study, simultaneous dEMG measurements were performed at the standard position and at...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24585

    authors: van Leuteren RW,Bekhuis RE,de Waal CG,de Jongh FH,van Kaam AH,Hutten GJ

    更新日期:2020-02-01 00:00:00

  • Pilot study for the development of a monitoring device for ventilated children.

    abstract::Airway pressure and air flow were measured at the endotracheal tube in 13 children on a variety of ventilators. These signals were stored for analysis on a computer. Further data sets were obtained after 24 hours or following major interventions. Air flow rate was integrated to give volume. Pulmonary resistance and el...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950110315

    authors: Wensley DF,Noonan P,Seear MD,Werner H,Pirie GE

    更新日期:1991-01-01 00:00:00

  • Dissociation of symptom scores and bronchial hyperreactivity: study in asthmatic children on long-term treatment with inhaled beclomethasone dipropionate.

    abstract::The aim of the study was to evaluate the effects of inhaled steroids (IS) on the improvement of clinical asthma symptoms and on the decrease in bronchial hyperreactivity (BHR). Twenty-four children with severe asthma were given 1,000 micrograms beclomethasone dipropionate (BDP) daily and compared with ten asthmatic co...

    journal_title:Pediatric pulmonology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1002/ppul.1950130203

    authors: Benoist MR,Brouard JJ,Rufin P,Waernessyckle S,de Blic J,Paupe J,Scheinmann P

    更新日期:1992-06-01 00:00:00

  • Comparison of airway reactivity induced by cold air and metacholine challenges in asthmatic children.

    abstract::Bronchial responsiveness to isocapnic hyperventilation with cold air (CAH) and to inhaled methacholine (MCH) was compared in 17 children with bronchial asthma. The response to cold air was expressed as the percent drop in FEV1 from baseline at 4 min. after the challenge (delta % FEV1 CAH), and the response to methacho...

    journal_title:Pediatric pulmonology

    pub_type: 临床试验,杂志文章

    doi:10.1002/ppul.1950190603

    authors: de Benedictis FM,Canny GJ,MacLusky IB,Levison H

    更新日期:1995-06-01 00:00:00

  • Telomere length and lung function in a population-based cohort of children and mid-life adults.

    abstract:OBJECTIVE:Telomere length is associated with poorer lung health in older adults, possibly from cumulative risk factor exposure, but data are lacking in pediatric and population-based cohorts. We examined associations of telomere length with lung function in children and mid-life adults. METHODS:Data were drawn from a ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24489

    authors: Nguyen MT,Saffery R,Burgner D,Lycett K,Vryer R,Grobler A,Dwyer T,Ranganathan S,Wake M

    更新日期:2019-12-01 00:00:00

  • Sleep and breathing in Prader-Willi syndrome.

    abstract::Prader-Willi syndrome (PWS) is a genetic disorder, with hypotonia being the predominant feature in infancy, and developmental delay, obesity, and behavioral problems becoming more prominent in childhood and adolescence. Children with this disorder frequently suffer from excessive daytime sleepiness and have a primary ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章,评审

    doi:10.1002/ppul.10152

    authors: Nixon GM,Brouillette RT

    更新日期:2002-09-01 00:00:00

  • Tracking of lung function in healthy children and adolescents.

    abstract::Two hundred twenty-six healthy school children, with a mean age of 8.8 years; 62 girls mean age 8.8, 48 boys mean age 12.6 and 51 girls mean age 12.6 years at the start, were enrolled in a longitudinal study of lung function and tested annually for 5 years. All were free of respiratory symptoms, and none smoked more t...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950080308

    authors: Hibbert ME,Hudson IL,Lanigan A,Landau LI,Phelan PD

    更新日期:1990-01-01 00:00:00

  • Lung abscess due to non-tuberculous, non-Mycobacterium fortuitum in a neonate.

    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

    authors: Glatstein M,Scolnik D,Bensira L,Domany KA,Shah M,Vala S

    更新日期:2012-10-01 00:00:00

  • Hering-Breuer reflex in young asthmatic children.

    abstract::The aim of this study was to assess if the Hering-Breuer reflex could be provoked in young children. Thirty asthmatic children with a mean age of 5.3 years and 18 healthy children with a mean age of 7.0 years were studied. The presence of the reflex was indicated by prolongation of the spontaneous expiratory time foll...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950110412

    authors: Greenough A,Pool J

    更新日期:1991-01-01 00:00:00

  • Partitioning the respiratory effects of airway citric acid and normal saline in lambs.

    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

    authors: Hutchison AA,Caton D,Thomas RG,Bucciarelli RL

    更新日期:1987-01-01 00:00:00

  • Transition of respiratory technology dependent patients from pediatric to adult pulmonology care.

    abstract:OBJECTIVE:Recent advances in medicine have allowed children with chronic life-threatening disorders to survive longer than ever before with the use of complex medical device technology (e.g., mechanical ventilation, dialysis, etc.). The care of children with chronic pulmonary disorders and respiratory-technology depend...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.23155

    authors: Agarwal A,Willis D,Tang X,Bauer M,Berlinski A,Com G,Ward WL,Carroll JL

    更新日期:2015-12-01 00:00:00

  • Non-invasive assessment of exercise performance in children with cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis: is there a CF specific muscle defect?

    abstract:INTRODUCTION:Peripheral muscle dysfunction is increasingly recognized as complicating respiratory disease, but this is difficult to measure non-invasively. RESEARCH QUESTION:Can skeletal muscle function and efficiency be measured during exercise non-invasively using respiratory mass spectrometry (RMS); and is the know...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.20899

    authors: Rosenthal M,Narang I,Edwards L,Bush A

    更新日期:2009-03-01 00:00:00

  • Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis.

    abstract:BACKGROUND:Patient-reported outcomes (PROs) are increasingly used to evaluate the efficacy of new treatments and the progression of chronic diseases. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) is a disease-specific, PRO measure of health-related quality of life (HRQOL). We evaluated associations between changes ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21325

    authors: Sawicki GS,Rasouliyan L,McMullen AH,Wagener JS,McColley SA,Pasta DJ,Quittner AL

    更新日期:2011-01-01 00:00:00

  • Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis.

    abstract::Progressive lung disease in patients with cystic fibrosis (CF) is caused by thick secretions, which cause airway obstruction and subsequent colonization and infection by inhaled pathogenic microorganisms. Recently, recombinant human DNase has been shown to reduce the viscoelasticity of sputum in patients with cystic f...

    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(199602)21:2<77::AID-PPUL3>

    authors: Eng PA,Morton J,Douglass JA,Riedler J,Wilson J,Robertson CF

    更新日期:1996-02-01 00:00:00

  • 15-Year trends in respiratory care of extremely preterm infants: Contributing factors and consequences on health and growth during hospitalization.

    abstract:OBJECTIVES:To review 15-year trends in respiratory care of extremely preterm infants managed in a tertiary perinatal center; to identify the factors contributing to their evolution; and to determine whether these changes had an impact on infant mortality, severe morbidity, and growth. METHODS:Retrospective cohort stud...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24774

    authors: Habas F,Durand S,Milési C,Mesnage R,Combes C,Gavotto A,Picaud JC,Cambonie G

    更新日期:2020-08-01 00:00:00

  • The etiology of tracheal mucosal injury in proximal esophageal atresia plus distal tracheoesophageal fistula: an animal study.

    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

    authors: Ateş O,Hakgüder G,Olguner M,Ozer E,Akgür FM

    更新日期:2006-11-01 00:00:00

  • Prophylactic antibiotics in cystic fibrosis: a conviction without evidence?

    abstract::Pulmonary infection with Staphylococcus aureus occurs in young children with cystic fibrosis, and may contribute to the cycle of infection, inflammation, and destruction of lung tissue which leads to bronchiectasis. Practice guidelines in North America and the UK differ greatly with regard to the advice given on presc...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章,评审

    doi:10.1002/ppul.20305

    authors: Smyth A

    更新日期:2005-12-01 00:00:00

  • A decade of pediatric tracheostomies: Indications, outcomes, and long-term prognosis.

    abstract:OBJECTIVE:To define the mortality and long-term outcomes of children undergoing tracheostomy. DESIGN:Retrospective chart and Texas Department of Health Bureau of Vital Statistics review of patients admitted to a Pediatric Intensive Care Unit who underwent a tracheostomy between 2001 and 2011. Mortality and decannulati...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.23657

    authors: McPherson ML,Shekerdemian L,Goldsworthy M,Minard CG,Nelson CS,Stein F,Graf JM

    更新日期:2017-07-01 00:00:00

  • Perception of intrinsic and extrinsic respiratory loads in children with life-threatening asthma.

    abstract::There is a subpopulation of asthmatic patients with a history of life-threatening asthma (LTA) who have a reduced perception of respiratory loads. The aim of this study was to determine if these patients have a reduced perception of both intrinsic and extrinsic loads. Children with asthma were classified into life-thr...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.10199

    authors: Julius SM,Davenport KL,Davenport PW

    更新日期:2002-12-01 00:00:00

  • Bronchoalveolar lavage fluid cellularity and soluble intercellular adhesion molecule-1 in children with colds.

    abstract::Viral colds are an important cause of respiratory symptoms in normal children. Studies in adults suggest that inflammation in the lower respiratory tract is associated with viral colds, but there are no data regarding inflammation and viral infection in the lower airway of normal children with colds. We, therefore, st...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/(sici)1099-0496(199908)28:2<109::aid-ppul6

    authors: Grigg J,Riedler J,Robertson CF

    更新日期:1999-08-01 00:00:00

  • Predictors of proper inhaler technique and asthma control in pediatric patients with asthma.

    abstract:BACKGROUND:Improper use of asthma inhalers is one of the potential factors of poor asthma control among children. This study aimed to assess the proper handling of asthma inhalers and asthma control in addition to factors influencing them among pediatric patients who self-administer their inhalers. METHODS:A cross-sec...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.25263

    authors: Almomani BA,Al-Qawasmeh BS,Al-Shatnawi SF,Awad S,Alzoubi SA

    更新日期:2021-01-26 00:00:00

  • Corticosteroid treatment for pulmonary lymphoid hyperplasia in children with the acquired immune deficiency syndrome.

    abstract::Five children with positive serology for human immunodeficiency virus (HIV) infection by enzyme-linked immunosorbent assay and Western blot were followed for chronic pulmonary disease. Lung biopsies were performed in all patients, and confirmed the diagnosis of pulmonary lymphoid hyperplasia. All children demonstrated...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950040105

    authors: Rubinstein A,Bernstein LJ,Charytan M,Krieger BZ,Ziprkowski M

    更新日期:1988-01-01 00:00:00