Ten-year follow up of hydroxychloroquine treatment for ABCA3 deficiency.

abstract：:Bronchopulmonary dysplasia (BPD) is a chronic obstructive pulmonary disease of prematurely born infants following prolonged mechanical ventilation and oxygen therapy. Developmental changes in pulmonary function of children with BPD during their early years have been difficult to study. We longitudinally studied maxima...

journal_title：Pediatric pulmonology

authors： Mallory GB Jr,Chaney H,Mutich RL,Motoyama EK

更新日期：1991-01-01 00:00:00

abstract：:We compared the performance of selected ultrasonic and jet nebulizers when aerosolizing several antibiotic formulations to determine optimum combinations for delivery of a respirable antibiotic aerosol. Three ultrasonic devices were tested: the UltraNeb 99/100, the UltraAIR and the Aerosonic. The reusable jet nebulize...

journal_title：Pediatric pulmonology

authors： Weber A,Morlin G,Cohen M,Williams-Warren J,Ramsey B,Smith A

更新日期：1997-04-01 00:00:00

abstract：:We used an in vitro model system to examine the sites of deposition of aqueous therapeutic aerosols administered through 3-mm, 6-mm, and 9-mm endotracheal tubes (commonly used in infants, children, and adults) at clinically relevant inspiratory flow rates. Aerosol was delivered to the endotracheal tube via a "T" piece...

journal_title：Pediatric pulmonology

authors： Ahrens RC,Ries RA,Popendorf W,Wiese JA

更新日期：1986-01-01 00:00:00

abstract：:Neutrophil elastase is present at high levels in airway fluid of patients with cystic fibrosis (CF), and is responsible for considerable inflammatory damage. Human monocyte/neutrophil elastase inhibitor (MNEI), a 42-kDa serpin protein, is an effective inhibitor of neutrophil elastase, cathepsin G, and proteinase-3, re...

journal_title：Pediatric pulmonology

authors： Woods DE,Cantin A,Cooley J,Kenney DM,Remold-O'Donnell E

更新日期：2005-02-01 00:00:00

abstract：:Lung transplantation in childhood is a highly specialized clinical practice confined to a few centers around the world. Organ availability remains an important limiting factor in extending the application of this procedure to more infants, children and adolescents. The lungs are the organ most vulnerable to injury, in...

journal_title：Pediatric pulmonology

authors： Mallory GB Jr,Schecter MG,Elidemir O

更新日期：2009-06-01 00:00:00

abstract：:The introduction of NBS in Ireland in July 2011, provided a unique opportunity to investigate clinical outcomes using a comparative historical cohort study. Clinical cohort: children clinically diagnosed with CF born 1 July 2008 to 30 June 2011, and NBS cohort: children diagnosed with CF through NBS born 1 July 2011 t...

journal_title：Pediatric pulmonology

authors： Fitzgerald C,Linnane B,George S,Ni Chroinin M,Mullane D,Herzig M,Greally P,Elnazir B,Healy F,Mc Nally P,Javadpour S,Cox D,Fitzpatrick P

更新日期：2020-09-01 00:00:00

abstract：:Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple body organs. The lungs and pancreas are the most affected which results in progressive lung damage and pancreatic insufficiency. Due to the disease process, CF patients require significantly higher caloric intake than recommended for other ind...

journal_title：Pediatric pulmonology

authors： Nasr SZ,Drury D

更新日期：2008-03-01 00:00:00

abstract：:Environmental tobacco smoke, also called passive smoking, was shown to have adverse effects on the health of children. Hydrogen peroxide (H2O2) is proposed as a sensitive marker of oxidative injury and inflammatory processes in the airways, being increased in adult active cigarette smokers. We tested whether passive s...

journal_title：Pediatric pulmonology

authors： Doniec Z,Nowak D,Tomalak W,Pisiewicz K,Kurzawa R

更新日期：2005-01-01 00:00:00

abstract：:Spirometry is used to monitor respiratory progress in children with Duchenne muscular dystrophy (DMD). Mucociliary clearance depends on cough strength, which can be measured by peak cough flow (PCF). It is not routinely measured in most centers. When the PCF falls below 270 l/min, mucociliary clearance is likely to be...

journal_title：Pediatric pulmonology

authors： Gauld LM,Boynton A

更新日期：2005-05-01 00:00:00

abstract：:We report on a 1-month-old boy who presented with a "horseshoe" lung, complete "O" rings of the trachea, and an unusual course of the left pulmonary artery mimicking a left pulmonary artery sling. Computed tomography, cardiac catheterization, and bronchoscopy were performed to confirm the rare coexistence of these ano...

journal_title：Pediatric pulmonology

authors： Hwang HK,Chen MR,Chen SJ,Kao HA,Lee KS,Shih SL

更新日期：2002-11-01 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：:Lung hypoplasia is the main cause of congenital diaphragmatic hernia (CDH)-associated death but pathogenesis remains unclear. MiR-455-5p is involved in lung hypoplasia. We hypothesized that nitrofen causes abnormal miR-455-5p expression during lung development and designed this study to determine the relationship betw...

journal_title：Pediatric pulmonology

authors： Zheng J,He Q,Tang H,Li J,Xu H,Mao X,Liu G

更新日期：2020-06-01 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

authors： van Woensel JB,Kimpen JL,Sprikkelman AB,Ouwehand A,van Aalderen WM

更新日期：2000-08-01 00:00:00

abstract：:We describe a method for measuring carbon monoxide diffusing capacity (DL(CO)) and alveolar volume (V(A)) in sleeping infants, using a single 4-sec breath-hold technique. The breath-hold maneuver is obtained by inducing a respiratory pause of the respiratory system. Several inflations of the respiratory system with ro...

journal_title：Pediatric pulmonology

authors： Castillo A,Llapur CJ,Martinez T,Kisling J,Williams-Nkomo T,Coates C,Tepper RS

更新日期：2006-06-01 00:00:00

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

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

更新日期：2018-11-01 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

authors： Forrester DL,Barr HL,Fogarty A,Knox A

更新日期：2017-04-01 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：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：INTRODUCTION:There are few published data on the efficacy of systemic corticosteroids in preterm infants with very severe forms of bronchopulmonary dysplasia (BPD), requiring respiratory support after 3 months of age. The aim of this study was to report the use of pulses of methylprednisolone in this population and its...

journal_title：Pediatric pulmonology

authors： Billion E,Hadchouel A,Garcelon N,Delacourt C,Drummond D

更新日期：2021-01-01 00:00:00

abstract：:The purpose of this study was to determine if the interrupter technique, a noninvasive method for measuring airflow resistance, could be used to assess airway obstruction in children. In 107 children (74 with asthma, 12 with cystic fibrosis, and 21 without lung disease) conductance (mostly of airways) measured with th...

journal_title：Pediatric pulmonology

authors： Carter ER,Stecenko AA,Pollock BH,Jaeger MJ

更新日期：1994-04-01 00:00:00

abstract：BACKGROUND:Studies on the association between asthma and anxiety in Asian children are limited. Therefore, we aimed to evaluate the association between asthma and anxiety among Japanese elementary school students. METHODS:A cross-sectional study of the association between asthma and anxiety in primary school children ...

journal_title：Pediatric pulmonology

authors： Kimura M,Ikeda A,Suzuki Y,Maruyama K,Wada H,Tanigawa T

更新日期：2020-10-01 00:00:00

abstract：:Wheezing is the most widely reported adventitious lung sound in the English language. It is recognized by health professionals as well as by lay people, although often with a different meaning. Wheezing is an indicator of airway obstruction and therefore of interest particularly for the assessment of young children an...

journal_title：Pediatric pulmonology

authors： Pasterkamp H

更新日期：2018-02-01 00:00:00

abstract：BACKGROUND:The diagnosis of community-acquired pneumonia (CAP) is based mainly on the patient's medical history and physical examination. However, in severe cases a further evaluation including chest X-ray (CXR) may be necessary. At present, lung ultrasound (LUS) is not included in the diagnostic work-up of pneumonia. ...

journal_title：Pediatric pulmonology

authors： Caiulo VA,Gargani L,Caiulo S,Fisicaro A,Moramarco F,Latini G,Picano E,Mele G

更新日期：2013-03-01 00:00:00

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

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

更新日期：1987-01-01 00:00:00

abstract：:"Pulmonary Interstitial Glycogenosis: Diagnostic Evaluation and Clinical Course," written by Liptzin et al is a timely and insightful phenotypic summary of a rare pediatric interstitial lung disease. Twenty-four infants with biopsy-proven pulmonary interstitial glycogenosis (PIG) were reviewed at their center. Genetic...

journal_title：Pediatric pulmonology

authors： Yonker LM,Kinane TB

更新日期：2018-12-01 00:00:00

abstract：:Neurally adjusted ventilatory assistance (NAVA) can overcome technical difficulties with synchronizing noninvasive ventilation breaths with the patient, a modality often used in very low birthweight infants (VLBW) with apnea of prematurity (AOP). This study is a retrospective single-center investigation into whether N...

journal_title：Pediatric pulmonology

authors： Tabacaru CR,Moores RR Jr,Khoury J,Rozycki HJ

更新日期：2019-11-01 00:00:00

abstract：INTRODUCTION:To date, no consensus has been reached on the optimal management of congenital lung abnormalities, and factors predicting postnatal outcome have not been identified. We developed an objective quantitative computed tomography (CT) scoring method, and assessed its value for clinical decision-making. METHODS...

journal_title：Pediatric pulmonology

authors： Hermelijn SM,Dragt OV,Bosch JJ,Hijkoop A,Riera L,Ciet P,Wijnen RMH,Schnater JM,Tiddens HAWM

更新日期：2020-11-01 00:00:00

abstract：BACKGROUND:Interferon-gamma (IFN-γ) and interferon-inducible protein of 10 kDa (IP-10) are potent inflammatory mediators and contribute to acute lung injury in adults. Recently, a potential role for IFN-γ and IP-10 in the pathogenesis of bronchopulmonary dysplasia (BPD) has been reported in animal models. OBJECTIVE:To...

journal_title：Pediatric pulmonology

authors： Aghai ZH,Saslow JG,Mody K,Eydelman R,Bhat V,Stahl G,Pyon K,Bhandari V

更新日期：2013-01-01 00:00:00

abstract：:Since persistent pulmonary hypertension of the newborn (PPHN) often occurs as a life-threatening illness, it would be advantageous to identify the highest-risk infants within the first 24 hours of life so that transfer to centers with extracorporeal membrane oxygenation (ECMO) or high-frequency ventilation can be faci...

journal_title：Pediatric pulmonology

authors： Davis JM,Spitzer AR,Cox C,Fox WW

更新日期：1988-01-01 00:00:00

abstract：:IL-10 is an anti-inflammatory cytokine that may have a protective role in acute lung injury. IL-10 expression is affected by a single-nucleotide polymorphism (SNP) located at position -1082 (G to A). The A allele is associated with lower IL-10 production. Low IL-10 production has been linked to the development of BPD....

journal_title：Pediatric pulmonology

authors： Yanamandra K,Boggs P,Loggins J,Baier RJ

更新日期：2005-05-01 00:00:00