Pulmonary outcome measures in long-term survivors of infantile Pompe disease on enzyme replacement therapy: A case series.

abstract：:In this article, we describe existing CF registries with a focus on US registry data collected through the CF Foundation Patient Registry (CFFPR) and the Epidemiologic Study of CF (ESCF); highlight what registries have taught us regarding epidemiology of CF; showcase the impact of registries on research and clinical c...

journal_title：Pediatric pulmonology

authors： Fink AK,Loeffler DR,Marshall BC,Goss CH,Morgan WJ

更新日期：2017-11-01 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

authors： Kilinc AA,Tarcin G,Kurugoglu S,Schuster V,Cokugras H,Celkan T

更新日期：2020-01-01 00:00:00

abstract：:To assess whether there is a maturation of the response to tube breathing during the first 10 days of life, we have measured the dynamic ventilatory responses of nine term infants (34 studies) to added tubes equivalent to two anatomical dead spaces. Such a method allows an assessment of the baby's ability to increase ...

journal_title：Pediatric pulmonology

authors： Upton CJ,Milner AD,Stokes GM,Wilson AJ

更新日期：1990-01-01 00:00:00

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

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

abstract：:Respiratory system compliance (Crs) can be used to assess lung stiffness in sick, intubated infants, avoiding the use of an esophageal balloon. Crs was assessed in a group of 15 sick, intubated infants using the occlusion and inflation techniques. The occlusion technique gave satisfactory results in 13 infants. Apneic...

journal_title：Pediatric pulmonology

authors： Heaf DP,Turner H,Stocks J,Helms P

更新日期：1987-03-01 00:00:00

abstract：:Changes in the relative contribution of ribcage and abdomen during augmented breaths were assessed in two groups of infants (less than 2 weeks and 4-12 weeks old). In both groups ribcage and abdominal movements increased during augmented breaths in quiet sleep. In the older infants the relative increase was greater fo...

journal_title：Pediatric pulmonology

authors： Mathew OP,Pronske ML,Clark ML

更新日期：1988-01-01 00:00:00

abstract：BACKGROUND:Consolidation on chest radiography is widely used as the reference standard for defining pneumonia and variability in interpretation is well known but not well explored or explained. METHODS:Three pediatric sub-specialists (infectious diseases, radiology and respiratory medicine) viewed 3,033 chest radiogra...

journal_title：Pediatric pulmonology

authors： Williams GJ,Macaskill P,Kerr M,Fitzgerald DA,Isaacs D,Codarini M,McCaskill M,Prelog K,Craig JC

更新日期：2013-12-01 00:00:00

abstract：:We tested a health education intervention program to reduce passive smoking in infancy. The aim was to develop an instrument for study of tobacco smoke exposure and childhood respiratory illness. One hundred and eighty-four women who had smoked during pregnancy were allocated by month of delivery to an intervention gr...

journal_title：Pediatric pulmonology

authors： Woodward A,Owen N,Grgurinovich N,Griffith F,Linke H

更新日期：1987-05-01 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：:L-arginine is the precursor of nitric oxide which plays an important role on pulmonary circulation and pulmonary vascular tone. Our aim was to compare the levels of L-arginine between infants with respiratory distress syndrome and infants without respiratory distress syndrome and to determine the relationship between ...

journal_title：Pediatric pulmonology

authors： Canpolat FE,Yurdakök M,Yiğit S,Korkmaz A,Tekinalp G

更新日期：2005-12-01 00:00:00

abstract：BACKGROUND:Asthma and obstructive sleep apnea (OSA) in children share multiple epidemiological risk factors and the prevalence of snoring is higher in asthmatic children, suggesting that the latter may be at increased risk for OSA. Since both asthma and OSA are inflammatory disorders, we hypothesized that polysomnograp...

journal_title：Pediatric pulmonology

authors： Kheirandish-Gozal L,Dayyat EA,Eid NS,Morton RL,Gozal D

更新日期：2011-09-01 00:00:00

abstract：RATIONAL:This study aims at describing the use of bilevel positive airway pressure (BiPAP) in infants with severe bronchiolitis. WORKING HYPOTHESIS:The use of BiPAP in infants with bronchiolitis may be associated with a worst outcome. STUDY DESIGN:A single-center retrospective study performed from October 2013 to Apr...

journal_title：Pediatric pulmonology

authors： Delacroix E,Millet A,Pin I,Mortamet G

更新日期：2020-11-01 00:00:00

abstract：RATIONALE AND OBJECTIVES:Little is known about molecular changes in lungs of fetal rabbits with surgically induced diaphragmatic hernia (DH). Therefore, we examined in this model gene expressions of pivotal molecules for the developing lung. METHODS:At day 23 of gestation, DH was created in 12 fetuses from 4 does. Bot...

journal_title：Pediatric pulmonology

authors： Vuckovic A,Roubliova XI,Votino C,Naeije R,Jani JC

更新日期：2012-11-01 00:00:00

abstract：OBJECTIVE:To determine the interpretative consequences of adopting the Global Lungs 2012 (GLI-2012) spirometric prediction equations in a pediatric hospital population. MATERIAL:Spirometric records from 2,192 white boys and 1,842 white girls, and 412 and 334 African-American boys and girls, respectively, aged 6.0-18.0...

journal_title：Pediatric pulmonology

authors： Quanjer PH,Weiner DJ

更新日期：2014-02-01 00:00:00

abstract：:Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown. In childhood, it is extremely rare, with only three reported cases so far. Here we report on an 8-year-old girl,...

journal_title：Pediatric pulmonology

authors： Heinrich D,Gordjani N,Trusen A,Marx A,Hebestreit H

更新日期：2003-05-01 00:00:00

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

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

更新日期：2006-09-01 00:00:00

abstract：OBJECTIVE:To describe and analyze the characteristics and the early risk factors for mortality of noninvasive ventilation (NIV) in critically ill children. STUDY DESIGN:A multicenter, prospective, observational 2-year study carried out with critically ill patients (1 month - 18 years of age) who needed NIV. Clinical d...

journal_title：Pediatric pulmonology

authors： López J,Pons-Òdena M,Medina A,Molinos-Norniella C,Palanca-Arias D,Demirkol D,León-González JS,López-Fernández YM,Perez-Baena L,López-Herce J,NIV Research Group.

更新日期：2020-12-31 00:00:00

abstract：UNLABELLED:Vascular rings (VR) may produce extrinsic compression of trachea and/or esophagus. Diagnosis relies on nonspecific clinical data from resulting compression and image studies, mainly angiography, computed tomography, and cardiovascular MRI. Because of their low incidence, diagnosis is often missed. The role o...

journal_title：Pediatric pulmonology

authors： Furuya ME,Vargas MH,Ramírez-Figueroa JL,Félix-Heredia JL,González-Ortíz B,Rodríguez-Hernández L,Vera-Canelo JM

更新日期：2010-06-01 00:00:00

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

authors： Grigg J,Riedler J,Robertson CF

更新日期：1999-08-01 00:00:00

abstract：:The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was identified in 1989, yet major pathophysiologic questions remain unanswered. There is emerging evidence that CFTR is a bicarbonate channel, a driver of chloride-bicarbonate exchange and through its action on local pH, a regulator o...

journal_title：Pediatric pulmonology

authors： Borowitz D

更新日期：2015-10-01 00:00:00

abstract：BACKGROUND:Mesenchymal stromal cell (MSC)-mediated therapeutic effects have been observed in the treatment of lung diseases. For the first time, this treatment was used as rescue therapy in a pediatric patient with a life-threatening respiratory syndrome associated with the filamin A (FLNA) gene mutation. METHODS:A ch...

journal_title：Pediatric pulmonology

authors： Pelizzo G,Avanzini MA,Lenta E,Mantelli M,Croce S,Catenacci L,Acquafredda G,Ferraro AL,Giambanco C,D'Amelio L,Giordano S,Re G,Zennaro F,Calcaterra V

更新日期：2020-01-01 00:00:00

abstract：:The airway epithelium represents a primary site for the introduction and deposition of potentially pathogenic microorganisms into the body, through inspired air. The epithelial mucosa is an important component of the innate immune system that recognizes conserved structures in microorganisms and initiates appropriate ...

journal_title：Pediatric pulmonology

authors： Gómez MI,Prince A

更新日期：2008-01-01 00:00:00

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

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

abstract：:Fine needle aspiration biopsy cytology performed in three children with sarcoidosis expedited clinical investigation and diagnosis of their disease. Each patient had a different clinical presentation; in two of them lymphoma was part of the initial differential diagnosis. Aspiration cytology in all cases revealed coll...

journal_title：Pediatric pulmonology

authors： Wakely PE Jr,Silverman JF,Holbrook CT,Fairman RP,Daeschner CW 3rd,Joshi VV

更新日期：1992-06-01 00:00:00

abstract：OBJECTIVE:Among older children, sleep-disordered breathing (SDB) is associated with measurable neurocognitive consequences. However, diagnostic SDB thresholds are lacking for infants < 12 months. We sought to evaluate the relationship between SDB indices, gestational age (GA), and postmenstrual age (PMA) for infants wh...

journal_title：Pediatric pulmonology

authors： Matlen LB,Hassan F,Shellhaas RA

更新日期：2019-08-01 00:00:00

abstract：:Pseudomonas aeruginosa is often cultured from the airways of children with tracheostomies. P. aeruginosa produces exotoxin A (ETA) and type III cytotoxins. This study tested the hypothesis that children with tracheostomies are colonized by P. aeruginosa that express these virulence factors and will have antibodies dir...

journal_title：Pediatric pulmonology

authors： Rao AR,Splaingard MS,Gershan WM,Havens PL,Thill A,Barbieri JT

更新日期：2005-05-01 00:00:00

abstract：:Bronchial reactivity was assessed in 66 children with bronchial asthma (aged 8-15 years) by provocation with histamine-HCl during a symptom-free period. A significant bronchial reaction to histamine was defined as a 50% increase in the resistance of the respiratory system (Rrs) determined by the forced oscillation tec...

journal_title：Pediatric pulmonology

authors： Holmgren D,Engström I,Bjure J,Sixt R,Aberg N

更新日期：1993-03-01 00:00:00

abstract：INTRODUCTION:Chest impedance (CI) is the current standard for cardio-respiratory monitoring in preterm infants but fails to provide direct and quantitative information on diaphragmatic activity. Transcutaneous electromyography (dEMG) is able to measure diaphragmatic activity, but its feasibility and repeatability to mo...

journal_title：Pediatric pulmonology

authors： Kraaijenga JV,Hutten GJ,de Jongh FH,van Kaam AH

更新日期：2015-09-01 00:00:00

abstract：BACKGROUND:Discussions on the diagnostic and management of acquired pediatric lung pathology are usually published by large tertiary children's hospitals. It is likely that much of this pathology is actually seen and managed in nonacademic practices. METHODS:A 10-year retrospective review of patients under 18-years of...

journal_title：Pediatric pulmonology

authors： Kanitra JJ,Thampy CA,Cullen ML

更新日期：2021-01-02 00:00:00

abstract：:Pulmonary function testing and monitoring plays an important role in the respiratory management of neonates. A noninvasive and complete bedside evaluation of the respiratory status is especially useful in critically ill neonates to assess disease severity and resolution and the response to pharmacological intervention...

journal_title：Pediatric pulmonology

authors： Reiterer F,Sivieri E,Abbasi S

更新日期：2015-10-01 00:00:00