2014 year in review: Cystic fibrosis.

Abstract:

:In this article, we highlight cystic fibrosis (CF) research published in Pediatric Pulmonology during 2014, as well as related articles published in other journals.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Savant AP,McColley SA

doi

10.1002/ppul.23309

subject

Has Abstract

pub_date

2015-11-01 00:00:00

pages

1147-56

issue

11

eissn

8755-6863

issn

1099-0496

journal_volume

50

pub_type

杂志文章,评审
  • Severe systemic adverse reaction to proton pump inhibitors in an infant.

    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

    authors: Baldassarre E,Sagaon MM,Ferrarini A,Bianchetti MG

    更新日期:2007-06-01 00:00:00

  • Comparison of different dosing strategies of intratracheally instilled budesonide on meconium injured piglet lungs.

    abstract:BACKGROUND:Severe inflammation plays a vital role in the pathogenesis of meconium aspiration syndrome (MAS). Intratracheal (IT) instillation of corticosteroids may be beneficial for MAS in optimizing local effect and reducing systemic adverse effects, but the optimum dosing course remains open to question. METHODS:Thi...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.23697

    authors: Lin CH,Jeng MJ,Yang YC,Hsiao YH,Kou YR

    更新日期:2017-07-01 00:00:00

  • Terbutaline alleviates the lung injury in the neonatal rats exposed to endotoxin: Potential roles of epithelial sodium channels.

    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

    authors: Lu K,Chen X,Zhu W,Mao X,Yang Y,Qiu J,Zhang M,Cheng R

    更新日期:2019-03-01 00:00:00

  • Magnetic resonance imaging of the larynx in the pediatric population: A systematic review.

    abstract:BACKGROUND:Magnetic Resonance Imaging (MRI) techniques to image the larynx have evolved rapidly into a promising and safe imaging modality, without need for sedation or ionizing radiation. MRI is therefore of great interest to image pediatric laryngeal diseases. Our aim was to review MRI developments relevant for the p...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24250

    authors: Elders BBLJ,Hermelijn SM,Tiddens HAWM,Pullens B,Wielopolski PA,Ciet P

    更新日期:2019-04-01 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

  • AMPK and FoxO1 regulate catalase expression in hypoxic pulmonary arterial smooth muscle.

    abstract:BACKGROUND:Hypoxia and reactive oxygen species (ROS) including H(2)O(2) play major roles in triggering and progression of pulmonary vascular remodeling in persistent pulmonary hypertension. Catalase (CAT), the major endogenous enzyme scavenging H(2)O(2), is regulated in a tissue- and context-specific manner. OBJECTIVE...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.22919

    authors: Awad H,Nolette N,Hinton M,Dakshinamurti S

    更新日期:2014-09-01 00:00:00

  • Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: a 10-year retrospective study in a French region (Brittany).

    abstract::Until the year 2000, systematic cystic fibrosis (CF) neonatal screening was only performed in a few regions of France. The Brittany region began in 1989, but not the neighboring region of Loire-Atlantique. The present study compares the clinical evolution of both affected populations 10 years after screening was start...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.10259

    authors: Siret D,Bretaudeau G,Branger B,Dabadie A,Dagorne M,David V,de Braekeleer M,Moisan-Petit V,Picherot G,Rault G,Storni V,Roussey M

    更新日期:2003-05-01 00:00:00

  • US pediatric pulmonology workforce.

    abstract:AIM:Children with respiratory conditions benefit from care provided by pediatric pulmonologists. As these physicians are a small portion of the overall pediatric workforce, it is necessary to understand the practices and career plans of these specialists. METHODS:An internet survey was developed by the American Academ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24253

    authors: Harris C,Katkin J,Cataletto M,Dorkin H,Laskosz L,Ruch-Ross H

    更新日期:2019-04-01 00:00:00

  • Successful treatment of pulmonary hemangioma with propranolol.

    abstract::Pulmonary hemangioma is a rare benign tumor of the lungs. Airway hemangiomas in particular may lead to feeding difficulties, barking cough, stridor, respiratory distress, and even acute airway obstruction. Hence, such hemangiomas usually require early and aggressive treatment; however, the treatment modalities employe...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.23008

    authors: Emiralioğlu N,Oğuz B,Akyüz C,Yalçın B,Kiper N,Ersöz DD,Yalçın E,Özçelik U

    更新日期:2014-08-01 00:00:00

  • Human amniotic fluid stem cells protect rat lungs exposed to moderate hyperoxia.

    abstract:BACKGROUND:Treatment of bronchopulmonary dysplasia (BPD) remains as yet an unmet clinical need and recently stem cells have been proposed as a therapeutic tool in animal models. We investigated the role of amniotic fluid stem cells (AFS) in an adult rat model of hyperoxia lung injury. METHODS:Fifty Sprague-Dawley rats...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.22791

    authors: Grisafi D,Pozzobon M,Dedja A,Vanzo V,Tomanin R,Porzionato A,Macchi V,Salmaso R,Scarpa M,Cozzi E,Fassina A,Navaglia F,Maran C,Onisto M,Caenazzo L,De Coppi P,De Caro R,Chiandetti L,Zaramella P

    更新日期:2013-11-01 00:00:00

  • Early oxygen uptake recovery following exercise testing in children with chronic chest diseases.

    abstract::The value of exercise testing as an objective measure of disease severity in patients with chronic chest diseases (CCD) is becoming increasingly recognized. The aim of this study was to investigate changes in oxygen uptake (VO2) during early recovery following maximal cardiopulmonary exercise testing (CPXT) in relatio...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21024

    authors: Stevens D,Oades PJ,Armstrong N,Williams CA

    更新日期:2009-05-01 00:00:00

  • Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in association with celiac disease.

    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

    authors: Sethi GR,Singhal KK,Puri AS,Mantan M

    更新日期:2011-03-01 00:00:00

  • Acquired somatic mutations in the microsatellite DNA, in children with bronchial asthma.

    abstract:OBJECTIVES:High incidence of genetic alterations at the microsatellite (MS) DNA level has been reported in asthmatic adults. WORKING HYPOTHESIS:The aim of this study was to investigate whether microsatellite instability (MSI) and loss of heterozygosity (LOH) were detectable phenomena in children with asthma. METHODOL...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21097

    authors: Thomou C,Paraskakis E,Neofytou E,Kalmanti M,Siafakas NM,Tzortzaki EG

    更新日期:2009-10-01 00:00:00

  • Treatment of anorexia and weight loss with megestrol acetate in patients with cystic fibrosis.

    abstract::Four patients with severe cystic fibrosis lung disease, anorexia and weight loss, received Megestrol Acetate (MA), as an appetite stimulant. The initial dose was 400-800 mg daily and was continued for 6-15 months. Appetite was improved, with significant weight gain in all patients and an increase in their weight for a...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/(sici)1099-0496(199911)28:5<380::aid-ppul1

    authors: Nasr SZ,Hurwitz ME,Brown RW,Elghoroury M,Rosen D

    更新日期:1999-11-01 00:00:00

  • Spanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children.

    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

    authors: Moreno M,Castillo-Corullón S,Pérez-Ruiz E,Luna MC,Antón-Pacheco JL,Mondejar-Lopez P,De-la-Serna O,Villa JR,Osona B,Torres-Borrego J,Santiago-Burruchaga M,Asensio O,Andres-Martin A,Delgado-Pecellin I,González Y,Palmero A,E

    更新日期:2019-10-01 00:00:00

  • Respiratory pattern at hospital discharge in asymptomatic preterm infants.

    abstract::Pneumogram (PG) recordings were performed in 87 very low birthweight (VLBW) asymptomatic infants just prior to hospital discharge to determine the relationships between: 1) a prior history of apnea of prematurity (AOP) and cardiorespiratory pattern; and 2) cardiorespiratory pattern at hospital discharge and postconcep...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950040204

    authors: Hageman JR,Holmes D,Suchy S,Hunt CE

    更新日期:1988-01-01 00:00:00

  • DENND1B gene variants associate with elevated exhaled nitric oxide in healthy high-risk neonates.

    abstract:RATIONALE OF THE STUDY:Increased neonatal fraction of exhaled nitric oxide (FeNO) is associated with lung symptoms early in life, while predictors of neonatal FeNO levels are unknown. The objective of this study was to investigate perinatal and genetic predictors of FeNO in healthy at-risk neonates. METHODS:FeNO was m...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.22958

    authors: Chawes BL,Bischoff AL,Kreiner-Møller E,Buchvald F,Hakonarson H,Bisgaard H

    更新日期:2015-02-01 00:00:00

  • Effect of night symptoms and disease severity on subjective sleep quality in children with non-cystic-fibrosis bronchiectasis.

    abstract:BACKGROUND:Night-time symptoms and hypoxemia during sleep may affect sleep quality in children with chronic lung disease such as bronchiectasis. Poor sleep quality may impair growth, learning, and emotional development of children. Our aim was to assess the sleep quality and associated factors in children with non-cyst...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21454

    authors: Erdem E,Ersu R,Karadag B,Karakoc F,Gokdemir Y,Ay P,Akpinar IN,Dagli E

    更新日期:2011-09-01 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

  • Polysomnography Provides Useful Clinical Information in the Liberation from Respiratory Technology: A Retrospective Review.

    abstract::Background The prevalence of respiratory-technology dependent children is increasing although for most children the goal is liberation from technology. Liberation from home mechanical ventilation (HMV) and decannulation strategies vary due to the lack of clinical practice standards. The primary objective of this study...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.24164

    authors: Bashir A,Henningfeld JK,Thompson NE,D'Andrea LA

    更新日期:2018-11-01 00:00:00

  • Surfactant administration via a thin endotracheal catheter during spontaneous breathing in preterm infants.

    abstract::To systematically review the clinical outcomes of surfactant administration via a thin endotracheal catheter during spontaneous breathing compared with conventional administration involving tracheal intubation, mechanical ventilation (MV), and tracheal extubation, in preterm infants. PubMed, EMBASE, and the Cochrane L...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章,评审

    doi:10.1002/ppul.23651

    authors: Wu W,Shi Y,Li F,Wen Z,Liu H

    更新日期:2017-06-01 00:00:00

  • Acute pulmonary toxicity following intralesional administration of bleomycin for a lymphovenous malformation.

    abstract:OBJECTIVE:To describe the clinical course and treatment of an infant with acute pulmonary toxicity following intralesional administration of bleomycin for a lymphovenous malformation. DESIGN:Case report. SETTING:A tertiary care University-affiliated hospital. PATIENT, INTERVENTION, AND RESULTS:An 8-month-old girl de...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21139

    authors: Atwa K,Abuhasna S,Shihab Z,Hashaykeh N,Hasan R

    更新日期:2010-02-01 00:00:00

  • Acid steatocrit determination is not helpful in cystic fibrosis patients without or with mild steatorrhea.

    abstract:BACKGROUND:Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed to asse...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.21149

    authors: Walkowiak J,Lisowska A,Blask-Osipa A,Drzymała-Czyz S,Sobkowiak P,Cichy W,Breborowicz A,Herzig KH,Radzikowski A

    更新日期:2010-03-01 00:00:00

  • Five years of experience with biochemical cystic fibrosis newborn screening based on IRT/PAP in Germany.

    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

    authors: Sommerburg O,Hammermann J,Lindner M,Stahl M,Muckenthaler M,Kohlmueller D,Happich M,Kulozik AE,Stopsack M,Gahr M,Hoffmann GF,Mall MA

    更新日期:2015-07-01 00:00:00

  • Comparison of normal infants and infants with cystic fibrosis using forced expiratory flows breathing air and heliox.

    abstract::SUMMARY. The detection of early airway disease in infants with cystic fibrosis (CF) may lead to earlier intervention and an improved prognosis. We hypothesized that the ratio of maximal expiratory flows while breathing a mixture of helium and oxygen (heliox) and air, referred to as density dependence (DD), would ident...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/1099-0496(200101)31:1<17::aid-ppul1002>3.0

    authors: Davis S,Jones M,Kisling J,Howard J,Tepper RS

    更新日期:2001-01-01 00:00:00

  • A comprehensive approach to the prevention of bronchopulmonary dysplasia.

    abstract::The current bronchopulmonary dysplasia (BPD) is seen in infants born extremely premature, with less severe respiratory distress syndrome (RDS) and who received prenatal steroids-"new BPD". The pathophysiology of BPD is based on an impairment of lung maturation with prenatal and postnatal multi-hit insults and genetic ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章,评审

    doi:10.1002/ppul.21508

    authors: Kugelman A,Durand M

    更新日期:2011-12-01 00:00:00

  • Correlation between lung scintigraphy and long-term outcome in survivors of congenital diaphragmatic hernia.

    abstract::Lung scintigraphy has been used to evaluate the degree of pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH). However, the relationship between lung scintigraphy and long-term outcome of CDH remains unclear. The aim of this study is to determine whether lung scintigraphy correlates with long-te...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.20466

    authors: Okuyama H,Kubota A,Kawahara H,Oue T,Kitayama Y,Yagi M

    更新日期:2006-09-01 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

  • FGR in the setting of preterm sterile intra-uterine milieu is associated with a decrease in RDS.

    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

    authors: Park CW,Park JS,Jun JK,Yoon BH

    更新日期:2016-08-01 00:00:00

  • The bronchodilator effect of a fixed-combination metered aerosol (fenoterol and ipratropium bromide).

    abstract::The fixed-combination metered aerosol lK6 (fenoterol 0.05 mg/puff, ipratropium bromide 0.02 mg/puff--Berodual, Boehringer-Ingelheim Ltd., Ridgefield, Conn.) was administered to 12 children (8 boys, 4 girls) aged 3 1/2 to 6 2/12 years who had extrinsic bronchial asthma. Three forms of administration, each with a differ...

    journal_title:Pediatric pulmonology

    pub_type: 杂志文章

    doi:10.1002/ppul.1950010605

    authors: Berdel D,Kellersmann U

    更新日期:1985-11-01 00:00:00