Lung disease in Niemann-Pick disease.

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：:To develop and validate a questionnaire scale that can be used as a screening tool to investigate for the presence of childhood obstructive sleep apnea syndrome (OSAS) in Hong Kong Chinese children. Subjects suspected to have OSAS and controls were recruited. Parents completed a Hong Kong children sleep questionnaire ...

journal_title：Pediatric pulmonology

authors： Li AM,Cheung A,Chan D,Wong E,Ho C,Lau J,Wing YK

更新日期：2006-12-01 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：: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_title：Pediatric pulmonology

authors： Savant AP,McColley SA

更新日期：2015-11-01 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：: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

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

abstract：:Respiratory syncytial virus (RSV) lower respiratory tract infection (LRTI) during early childhood may be associated with subsequent pulmonary sequelae, including recurrent wheezing and asthma. We undertook a systematic review to investigate the pulmonary function sequelae following RSV LRTI in the first 3 years of lif...

journal_title：Pediatric pulmonology

authors： Verwey C,Nunes MC,Dangor Z,Madhi SA

更新日期：2020-07-01 00:00:00

abstract：:We developed a simple method to identify neonates at high risk of bronchopulmonary dysplasia (BPD) and determined whether early (8 hours) and late (14 days) risk assessment is equally useful. A retrospective cohort design was utilized of subjects enrolled in multi-dose surfactant trials to develop each risk identifica...

journal_title：Pediatric pulmonology

authors： Rozycki HJ,Narla L

更新日期：1996-06-01 00:00:00

abstract：:The goal of the present investigation was to describe the prevalence of and clinical factors associated with sleep-disordered breathing in children and adolescents. Children and adolescents (3,680 in all, 1-18 years old) attending schools in central Greece were surveyed by questionnaires distributed to parents. We fou...

journal_title：Pediatric pulmonology

authors： Kaditis AG,Finder J,Alexopoulos EI,Starantzis K,Tanou K,Gampeta S,Agorogiannis E,Christodoulou S,Pantazidou A,Gourgoulianis K,Molyvdas PA

更新日期：2004-06-01 00:00:00

abstract：:Pneumocyte apoptosis is implicated in the pathophysiology of acute inflammatory lung injuries in newborns and adults. Pulmonary angiotensin (ANG) II contributes to lung epithelial apoptosis in vitro, but its role in acute lung injury in vivo is unclear. We therefore studied the effects of ANG II receptor action on the...

journal_title：Pediatric pulmonology

authors： Lukkarinen HP,Laine J,Aho H,Zagariya A,Vidyasagar D,Kääpä PO

更新日期：2005-04-01 00:00:00

abstract：BACKGROUND:Although the benefits of recombinant human deoxyribonuclease (dornase alfa) in patients with cystic fibrosis (CF) are established, its optimal timing in relation to physiotherapy is unknown. As its enzymatic effect lasts for 6-11 hr, dornase alfa may be more efficacious if the time interval between inhalatio...

journal_title：Pediatric pulmonology

authors： Wilson CJ,Robbins LJ,Murphy JM,Chang AB

更新日期：2007-12-01 00:00:00

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

authors： Julius SM,Davenport KL,Davenport PW

更新日期：2002-12-01 00:00:00

abstract：:We determined the effects of an immediately antecedent viral lower respiratory tract infection (LRI) on the severity of clinical illness, changes in lung function and airway histamine responsiveness produced by a subsequent LRI in 9-12 week old beagle puppies inoculated with canine adenovirus 2, followed in 2 weeks by...

journal_title：Pediatric pulmonology

authors： Quan SF,Witten ML,Grad R,Ray CG,Lemen RJ

更新日期：1991-01-01 00:00:00

abstract：:Ten infants with evidence of impending respiratory failure from severe bronchiolitis were successfully treated with continuous positive airway pressure (NCPAP) with double nasal prongs. Their mean (SD) age was 6.7 (3.8) months and mean (SD) body weight was 7.1 (2.1) kg. Respiratory assessments were made immediately be...

journal_title：Pediatric pulmonology

authors： Soong WJ,Hwang B,Tang RB

更新日期：1993-09-01 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：:A computerized method for the measurement of alveolar ventilation (VA) and the mean alveolar partial pressures of CO2 and O2 is described and tested in healthy, awake fullterm (FT) newborns and preterm (PT) infants (postnatal age 40 days). This study emphasizes the technical pitfalls generally encountered when dealing...

journal_title：Pediatric pulmonology

authors： Lagneaux D,Mossay C,Geubelle F,Christiaens G

更新日期：1988-01-01 00:00:00

abstract：:In 1994-1995 a cross-sectional epidemiological study investigating the respiratory health of school children in two Taiwan areas was conducted; one area was located in a region with petrochemical manufacturing complexes (Linyuan), and the other was situated in a reference area with no local industrial emissions (Taihs...

journal_title：Pediatric pulmonology

authors： Yang CY,Wang JD,Chan CC,Hwang JS,Chen PC

更新日期：1998-05-01 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：INTRODUCTION:Acute bronchiolitis is a major cause of acute respiratory distress in infants. The soluble receptor for advanced glycation end-products (sRAGE) is a biomarker of pulmonary damage processes, with a diagnostic and a prognostic value in acute respiratory distress syndrome (ARDS). The RAGE pathway is also impl...

journal_title：Pediatric pulmonology

authors： Egron C,Roszyk L,Rochette E,Jabaudon M,Sapin V,Mulliez A,Labbé A,Coste K

更新日期：2018-10-01 00:00:00

abstract：:Maternal retinoid administration has beneficial effects on lung development in the nitrofen rodent toxic model of congenital diaphragmatic hernia (DH). We wanted to investigate the effects in a surgical model, where the retinoid signaling pathway is not primarily disrupted by the toxic agent. We created DH in fetal ra...

journal_title：Pediatric pulmonology

authors： Gallot D,Coste K,Jani J,Roubliova X,Marceau G,Velemir L,Verheyen A,Lemery D,Sapin V,Deprest J

更新日期：2008-06-01 00:00:00

abstract：:We compared proliferation (growth) and differentiation (development) related proteins in normal and hypoplastic fetal murine lungs. The hypoplastic lungs were created in CD-1 fetal mice by nitrofen exposure (25 mg per pregnant mouse given intragastrically on gestational day 8 [Gd8]), as published earlier. The lungs we...

journal_title：Pediatric pulmonology

authors： Chinoy MR,Chi X,Cilley RE

更新日期：2001-08-01 00:00:00

abstract：:Angiogenesis is an important mechanism of airway remodeling in lung disease. We previously demonstrated that serum vascular endothelial growth factor (VEGF) is elevated in cystic fibrosis (CF) patients and declines with therapy for pulmonary exacerbation. We hypothesized that VEGF is elevated early in the course of CF...

journal_title：Pediatric pulmonology

authors： Watts KD,McColley SA

更新日期：2011-07-01 00:00:00

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

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

abstract：BACKGROUND:Plastic bronchitis (PB) is a rare, variable, and potentially fatal disease. This study aimed to assess the efficacy of fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL) in treating children with PB. METHODS:In total, 15 children with PB, between 2012 and 2020, were enrolled in our study. Within...

journal_title：Pediatric pulmonology

authors： Wang L,Wang W,Sun JM,Ni SW,Ding JL,Zhu YL,Ding SG

更新日期：2020-11-01 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：:Little attention has been focused on the progressive pulmonary deterioration which occurs in mechanically ventilated infants with normal or mildly abnormal lungs. We hypothesized that lung function would deteriorate over a 24-hr period in anesthetized neonatal piglets with normal lungs mechanically ventilated at 2 cm ...

journal_title：Pediatric pulmonology

authors： Easa D,Finn KC,Balaraman V,Sood S,Wilkerson S,Takenaka W,Mundie TG

更新日期：1995-03-01 00:00:00

abstract：:Sleep disordered breathing (SDB) represents a spectrum of breathing disorders, ranging from snoring to obstructive sleep apnea syndrome (OSAS), that disrupt nocturnal respiration and sleep architecture. OSAS is a common disorder in children, with a prevalence of 2-3%. It is associated with neurobehavioral, cognitive, ...

journal_title：Pediatric pulmonology

authors： Goldbart AD,Tal A

更新日期：2008-12-01 00:00:00

abstract：:To evaluate the long-term effect of prematurity and/or hyaline membrane disease (HMD) on pulmonary function and airway reactivity, we studied 49 prematurely born children aged 10 to 13 years. They were divided into three groups according to birth weight and HMD status: Groups I and II comprised the children weighing l...

journal_title：Pediatric pulmonology

authors： Galdès-Sebaldt M,Sheller JR,Grogaard J,Stahlman M

更新日期：1989-01-01 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：:Lung diseases caused by surfactant protein C (SFTPC) mutations are inherited as autosomal traits with variable penetrance and severity or as sporadic disease caused by a de novo mutation on one allele. Here, we report the case of a child surviving with a homozygous surfactant protein C mutation after aggressive clinic...

journal_title：Pediatric pulmonology

authors： Arıkan-Ayyıldız Z,Caglayan-Sozmen S,Isık S,Deterding R,Dishop MK,Couderc R,Epaud R,Louha M,Uzuner N

更新日期：2014-03-01 00:00:00