Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations.

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

authors： Jochmann A,Artusio L,Robson K,Nagakumar P,Collins N,Fleming L,Bush A,Saglani S

更新日期：2016-08-01 00:00:00

abstract：INTRODUCTION:The 2017-2018 National Survey of Children's Health estimates that 30 million (42%) US children have experienced at least one adverse childhood experience (ACE), including abuse, neglect, and household dysfunction. ACEs negatively impact long-term health, and there has been no study of ACEs in cystic fibros...

journal_title：Pediatric pulmonology

authors： Williams WA 2nd,Jain M,Laguna TA,McColley SA

更新日期：2020-12-26 00:00:00

abstract：:Cystic fibrosis (CF) is one of the most common autosomal recessive and multisystemic diseases. CF affects many systems. One of these systems is the endocrine and exocrine functions of the pancreas, causing cystic fibrosis-related diabetes, which is extremely complex and has unique pathogenesis. Maturity-onset diabetes...

journal_title：Pediatric pulmonology

authors： Hangül M,Erdoğan M,Hatipoğlu N,Köse M

更新日期：2020-05-01 00:00:00

abstract：BACKGROUND:Child exposure to cigarette smoke is harmful. It should be reduced through parental smoking cessation interventions. The aim of our study was to determine the impact of simple advice provided by the pediatrician on the smoking habits of parents of children with cystic fibrosis (CF), diabetes mellitus (DM) an...

journal_title：Pediatric pulmonology

authors： Lafont M,Morin C,Arrouy A,Rabeau A,Labouret G,Roditis L,Michelet M,Mittaine M

更新日期：2021-01-22 00:00:00

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

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

更新日期：2016-08-01 00:00:00

abstract：BACKGROUND:In young children with cystic fibrosis (CF) the forced expiratory volume in 1 second (FEV1 ) is often normal and a more sensitive measure to detect early obstructive lung disease is needed. AIM:To evaluate the progression of selected spirometry parameters with age in a cohort of CF patients and healthy chil...

journal_title：Pediatric pulmonology

authors： Bakker EM,Borsboom GJ,van der Wiel-Kooij EC,Caudri D,Rosenfeld M,Tiddens HA

更新日期：2013-11-01 00:00:00

abstract：OBJECTIVES:The present pilot study was performed to evaluate the HPA axis and ANS activity by measuring salivary cortisol and α-amylase diurnal trajectory and production, respectively, in mild or moderate-to-severe (MS) OSA-affected, but otherwise healthy, children. Moreover, a correlative analysis was performed betwee...

journal_title：Pediatric pulmonology

authors： Patacchioli FR,Tabarrini A,Ghiciuc CM,Dima-Cozma LC,Prete A,Bianchini C,Nicoletti F,Gozal D,Villa MP

更新日期：2014-11-01 00:00:00

abstract：INTRODUCTION:Lung disease in cystic fibrosis (CF) begins early in life but the capabilities for detecting abnormalities of pulmonary dysfunction in children remain limited. OBJECTIVE:The study aimed to evaluate the early progression of lung function by the analysis of pulmonary hyperinflation, ventilation inhomogeneit...

journal_title：Pediatric pulmonology

authors： Walicka-Serzysko K,Postek M,Milczewska J,Sands D

更新日期：2020-11-01 00:00:00

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

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

更新日期：2015-02-01 00:00:00

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

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

更新日期：2008-08-01 00:00:00

abstract：:During the past five decades there has been increasing interest in the potential anti-inflammatory effects of macrolide antibiotics. Low-dose macrolide therapy has dramatically increased survival in patients with diffuse panbronchiolitis, a disease with many similarities to cystic fibrosis (CF). This has led to furthe...

journal_title：Pediatric pulmonology

authors： Jaffé A,Bush A

更新日期：2001-06-01 00:00:00

abstract：:Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive type of combined immunodeficiency with elevated IgE. In this report, we describe a Japanese girl of non-consanguineous family suffering from acute eosinophilic pneumonia (AEP) as a presenting feature of DOCK8 deficiency. Although AEP was self-limi...

journal_title：Pediatric pulmonology

authors： Tsuge I,Ito K,Ohye T,Kando N,Kondo Y,Nakajima Y,Inuo C,Kurahashi H,Urisu A

更新日期：2014-03-01 00:00:00

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 Me...

journal_title：Pediatric pulmonology

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

更新日期：2014-09-01 00:00:00

abstract：:In order to better understand the regulation of lung maturation by glucocorticoid-glucocorticoid receptor signaling, we studied glucocorticoid receptor (GR) hypomorphic mice with a mixed C57Bl6/129 sv background, in which disruption of exon 2 of the GR gene produces an N-terminal truncated GR protein. Four groups of m...

journal_title：Pediatric pulmonology

authors： Nemati B,Atmodjo W,Gagnon S,Humes D,McKerlie C,Kaplan F,Sweezey NB

更新日期：2008-02-01 00:00:00

abstract：:Pneumatoceles and bronchiectasis are secondary complications of respiratory infections in patients with hyper IgE syndrome (HIgES). We report on a patient with HIgES and recurrent pneumonias since 1 year of age, with progression to pneumatoceles and bronchiectasis and fatal outcome, with disseminated infection and sus...

journal_title：Pediatric pulmonology

authors： Roxo P Jr,Torres LA,Menezes UP,Melo JM

更新日期：2013-01-01 00:00:00

abstract：:Multiresistance in Stenotrophomonas maltophilia limits the effectiveness of antimicrobial therapy for infections due to this organism. It can be of special concern in cystic fibrosis (CF) patients due to frequent antimicrobial administration. The in vitro activity of 41 antimicrobial agents against 76 epidemiologicall...

journal_title：Pediatric pulmonology

authors： Cantón R,Valdezate S,Vindel A,Sánchez Del Saz B,Maíz L,Baquero F

更新日期：2003-02-01 00:00:00

abstract：:Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly pa...

journal_title：Pediatric pulmonology

authors： Shah AR,Dinwiddie R,Woolf D,Ramani R,Higgins JN,Matthew DJ

更新日期：1992-10-01 00:00:00

abstract：:Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systemati...

journal_title：Pediatric pulmonology

authors： Bromley S,Vizcaya D

更新日期：2017-05-01 00:00:00

abstract：BACKGROUND:Several studies have shown that exposure to air pollutants affects lung growth and development and can result in poor respiratory health in early life. METHODS:We included a subsample of 772 Mexican preschoolers whose mothers participated in a Prenatal Omega-3 fatty acid Supplements, GRowth, And Development...

journal_title：Pediatric pulmonology

authors： Gutiérrez-Delgado RI,Barraza-Villarreal A,Escamilla-Núñez MC,Hernández-Cadena L,Cortez-Lugo M,Sly P,Romieu I

更新日期：2020-08-01 00:00:00

abstract：BACKGROUND:Children's perception of their symptoms has proved reliable and relevant to disease management and should be considered when assessing their asthma control. The aim of the study is to validate the Brazilian Portuguese version of the Childhood Asthma Control Test (c-ACT) in children aged 4-11 years. METHODS:...

journal_title：Pediatric pulmonology

authors： Oliveira SG,Sarria EE,Roncada C,Stein RT,Pitrez PM,Mattiello R

更新日期：2016-04-01 00:00:00

abstract：AIM:Our aim was to evaluate the correlation of Adenosine monophosphate challenge test (AMP-PCW) results with the patients' subsequent clinical course. METHODS:We performed a 6-year retrospective cohort study of young children with suspected asthma who underwent AMP-PCW test. RESULTS:Fifty four children were included ...

journal_title：Pediatric pulmonology

authors： Levin G,Rottensctreich A,Picard E,Avital A,Springer C,Cohen S

更新日期：2019-07-01 00:00:00

abstract：OBJECTIVE:There is currently no evidence-based method for defining competency in pediatric flexible bronchoscopy (FB). Based on expert opinion, guidelines using numbers of procedures have been published in defining competency for pediatric FB. The purpose of this study was to formally survey the opinion of USA pediatri...

journal_title：Pediatric pulmonology

authors： Leong AB,Green CG,Kurland G,Wood RE

更新日期：2014-06-01 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

authors： Snell C,Fernandes S,Bujoreanu IS,Garcia G

更新日期：2014-12-01 00:00:00

abstract：:With better understanding of the role of type 2 inflammation in allergic asthma, there has been progress made in the development of new biologic therapies targeting these specific pathways. This review will consider diagnostic criteria for using biologic therapies for pediatric asthma with special emphasis on populati...

journal_title：Pediatric pulmonology

authors： Lovinsky-Desir S

更新日期：2020-03-01 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

authors： Leung TF,Wong YS,Chan IH,Yung E,Sy HY,Lam CW,Wong GW

更新日期：2011-07-01 00:00:00

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

authors： Smyth A

更新日期：2005-12-01 00:00:00

abstract：:The occurrence of a tracheoesophageal fistula (TEF) in the setting of lymphoma has only rarely been reported in the world literature. Most cases reported were associated with radiation therapy vs. chemotherapy alone. This report presents one case illustrating the difficulty encountered managing a TEF that developed wh...

journal_title：Pediatric pulmonology

authors： Moree JS,Bhakta MG,Ledbetter J

更新日期：2006-07-01 00:00:00

abstract：OBJECTIVES:To identify the spirometric equations that are most appropriate for use in children and adolescents living in Bogota, Colombia after evaluating a set of relevant previously-developed equations, including the Global Lung Function Initiative (GLI) 2012 spirometry reference equations. METHODS:Healthy children ...

journal_title：Pediatric pulmonology

authors： Aristizabal-Duque R,Castiblanco E,Rodriguez I,Sossa-Briceño MP,Rodriguez-Martinez CE

更新日期：2019-06-01 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

authors： O'Connor MG,Suthar D,Vera K,Slaughter JC,Maitre NL,Steele S,Beller A,Fike CD,Aschner JL,Moore PE,Austin ED

更新日期：2018-03-01 00:00:00

abstract：OBJECTIVE:Medication adherence among adolescents with cystic fibrosis (CF) is often suboptimal and this has significant impact on their health and quality of life. The purpose of the study was to evaluate the impact of frequent home pulmonary function (PFT) monitoring on medication adherence among adolescents with CF. ...

journal_title：Pediatric pulmonology

authors： Shakkottai A,Kaciroti N,Kasmikha L,Nasr SZ

更新日期：2018-04-01 00:00:00