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Pediatric Pulmonology

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https://www.readbyqxmd.com/read/28085238/comparison-of-sitting-and-supine-forced-vital-capacity-in-collagen-vi-related-dystrophy-and-laminin-%C3%AE-2-related-dystrophy
#1
Katherine G Meilleur, Melody M Linton, Joseph Fontana, Anne Rutkowski, Jeffrey Elliott, Mark Barton, Peter McGraw, Angela Kokkinis, Sandra Donkervoort, Meganne Leach, Minal Jain, Jahannaz Dastgir, James Collins, Rhonda Szczesniak, Kelly Yang, Hemant Sawnani, Carsten G Bönnemann
BACKGROUND: Progressive, restrictive, respiratory insufficiency is the major cause of morbidity and mortality in Congenital Muscular Dystrophy (CMD). Nocturnal hypoventilation precedes daytime alveolar hypoventilation, and if untreated, may lead to respiratory failure and cor pulmonale. CMD consensus care guidelines recommend screening for respiratory insufficiency by conventional and dynamic (sitting to supine) pulmonary function testing (PFT) and evaluating for sleep disordered breathing if there is more than 20% relative reduction from sitting to supine FVC(L) (ΔFVC)...
January 13, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28085236/budesonide-reduces-hospital-admission-rates-in-preschool-children-with-acute-wheezing
#2
Cem Hasan Razi, Nazlı Cörüt, Nesibe Andıran
The object of this study was to determine whether high doses of inhaled budesonide provide additional benefits to a standardized treatment regimen that includes systemic steroids and salbutamol in preschool patients presented to the emergency department (ED) with acute wheezing attacks. Methods This randomized, double-blind, placebo-controlled, parallel group trial was conducted in children, 6 months-6 years with moderate or severe acute wheezing epizode, as determined based on a pulmonary index score (PIS) of 7-13 points...
January 13, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28076664/sensitization-predicts-asthma-development-among-wheezing-toddlers-in-secondary-healthcare
#3
Nienke A Boersma, Ruud W H Meijneke, Johannes C Kelder, Cornelis K van der Ent, Walter A F Balemans
INTRODUCTION: Some wheezing toddlers develop asthma later in childhood. Sensitization is known to predict asthma in birth cohorts. However, its predictive value in secondary healthcare is uncertain. AIM: This study examines the predictive value of sensitization to inhalant allergens among wheezing toddlers in secondary healthcare for the development of asthma at school age (≥6 years). METHODS: Preschool children (1-3 years) who presented with wheezing in secondary healthcare were screened on asthma at school age with the International Study of Asthma and Allergies in Childhood questionnaire...
January 11, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28068001/in-vivo-and-in-vitro-ivacaftor-response-in-cystic-fibrosis-patients-with-residual-cftr-function-n-of-1-studies
#4
Meghan E McGarry, Beate Illek, Ngoc P Ly, Lorna Zlock, Sabrina Olshansky, Courtney Moreno, Walter E Finkbeiner, Dennis W Nielson
RATIONALE: Ivacaftor, a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, decreases sweat chloride concentration, and improves pulmonary function in 6% of cystic fibrosis (CF) patients with specific CFTR mutations. Ivacaftor increases chloride transport in many other CFTR mutations in non-human cells, if CFTR is in the epithelium. Some CF patients have CFTR in the epithelium with residual CFTR function. The effect of ivacaftor in these patients is unknown. METHODS: This was a series of randomized, crossover N-of-1 trials of ivacaftor and placebo in CF patients ≥8 years old with potential residual CFTR function (intermediate sweat chloride concentration, pancreatic sufficient, or mild bronchiectasis on chest CT)...
January 9, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28068000/strem-1-is-elevated-in-cystic-fibrosis-and-correlates-with-proteases
#5
D L Forrester, H L Barr, A Fogarty, A Knox
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 hypothesised that sTREM-1 could act as a biomarker of disease in cystic fibrosis. METHODS: Plasma from 17 patients with CF (stable and pre and post pulmonary exacerbation) and eight healthy volunteers was analyzed for sTREM-1 and proteases (matrix metalloproteinase-8 (MMP-8), MMP-9, and human neutrophil elastase HNE)...
January 9, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28052594/network-meta-analysis-of-probiotics-to-prevent-respiratory-infections-in-children-and-adolescents
#6
REVIEW
Marina Azambuja Amaral, Gabriela Helena Barbosa Ferreira Guedes, Matias Epifanio, Mario Bernardes Wagner, Marcus Herbert Jones, Rita Mattiello
Probiotics have emerged as a promising intervention for the prevention of respiratory tract infections (RTIs) in children. Assess the effect of probiotics on prevention of RTIs in children and adolescents. MEDLINE, EMBASE, LILACS, SCIELO, CINAHL, SCOPUS, and Web of Science. Key words: "respiratory tract infections" AND probiotics. Randomized controlled trials RCT assessing the effect of probiotics on RTIs in children and adolescents were included. Two reviewers, working independently, to identify studies that met the eligibility criteria...
January 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28052587/pilot-study-of-dornase-alfa-pulmozyme-therapy-for-acquired-ventilator-associated-infection-in-preterm-infants
#7
Melissa Scala, Deborah Hoy, Maria Bautista, Judith Jones Palafoutas, Kabir Abubakar
OBJECTIVE: Evaluate the feasibility, safety, and efficacy of adjunctive treatment with dornase alfa in preterm patients with ventilator-associated pulmonary infection (VAPI) compared to standard care. WORKING HYPOTHESIS: We hypothesize that therapy with dornase alfa will be safe and well tolerated in the preterm population with no worsening of symptoms, oxygen requirement, or need for respiratory support. STUDY DESIGN: Prospective, randomized, blinded, pilot study comparing adjunctive treatment with dornase alfa to sham therapy...
January 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28052585/diagnostic-yield-of-bronchoalveolar-lavage-in-immunocompromised-children-with-malignant-and-non-malignant-disorders
#8
Sruti Nadimpalli, Marc Foca, Prakash Satwani, Maria Luisa Sulis, Andrei Constantinescu, Lisa Saiman
BACKGROUND: The diagnostic yield of bronchoalveolar lavage (BAL) in the Immunocompromised pediatric population has ranged from 28% to 68%. We hypothesized that the diagnostic yield of BALs would be higher in more recent years due to new diagnostic assays. METHODS: A retrospective case series was performed among immunocompromised children ≤18 years old who underwent BALs from 2001 to 2012, to assess the yield of microbiologic diagnostic studies and to determine the impact of BAL findings on antimicrobial management...
January 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28052557/adenotonsillectomy-to-treat-obstructive-sleep-apnea-is-it-enough
#9
REVIEW
A Boudewyns, F Abel, E Alexopoulos, M Evangelisti, A Kaditis, S Miano, M P Villa, S L Verhulst
Although adenotonsillectomy is the first line treatment for children with obstructive sleep apnea syndrome (0SAS),(1) improvement in objectively documented outcomes is often inadequate and a substantial number of children have residual disease. Early recognition and treatment of children with persistent OSAS is required to prevent long-term morbidity. The management of these children is frequently complex and a multidisciplinary approach is required as most of them have additional risk factors for OSAS and comorbidities...
January 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28029756/when-and-why-to-treat-the-child-who-snores
#10
Hui-Leng Tan, Maria Luz Alonso Alvarez, Marina Tsaoussoglou, Silke Weber, Athanasios G Kaditis
Obstructive sleep-disordered breathing (SDB) can result in cardiovascular and neurocognitive morbidity as well as adversely affect behavior, growth, quality of life, and nocturnal continence. This article summarizes the latest evidence regarding the morbidity related to obstructive SDB, commenting on the impact of severity of obstruction, that is, the difference in effects seen of moderate to severe obstructive sleep apnea syndrome (OSAS) compared to those of mild OSAS or primary snoring. The impact of therapy is discussed, focusing on which children are likely to benefit from treatment interventions; namely those with moderate or severe OSAS irrespective of the presence of morbidity, children with mild OSAS with associated morbidity or predictors of SDB persistence such as obesity, and children with complex conditions accompanied by upper airway obstruction like craniosynostosis and Prader-Willi syndrome...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28029754/pulmonary-hemorrhage-as-a-complication-of-respiratory-syncyntial-virus-rsv-bronchiolitis
#11
Audrey K S Soo, David P Inwald
Respiratory Syncytial Virus (RSV) is a common cause of bronchiolitis. Although there are a number of recognized complications, pulmonary hemorrhage has not been reported previously. A retrospective case notes review was performed through an electronic search of a Pediatric Intensive Care Unit's medical records. Seven patients with RSV infection and pulmonary hemorrhage were identified and included in this case series. Six of the seven patients were born prematurely (30-36 weeks gestation). All patients required blood transfusion...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#12
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28029743/statins-ameliorate-pulmonary-hypertension-secondary-to-left-ventricular-dysfunction-through-the-rho-kinase-pathway-and-nadph-oxidase
#13
I-Chen Chen, Mian-Shin Tan, Bin-Nan Wu, Chee-Yin Chai, Jwu-Lai Yeh, Shah-Hwa Chou, Ing-Jun Chen, Zen-Kong Dai
BACKGROUND: Pulmonary hypertension (PH) is a devastating disorder, for which no therapy is curative. It has been reported that pulmonary vascular remodeling, associated with increasing mean pulmonary arterial pressure and upregulated expression of endothelial nitric oxide synthase (eNOS), endothelin-1 (ET-1), RhoA/RhoH-kinase results in the development of PH. Oxidative stress and the RhoA/Rho-kinase pathway are also thought to be involved in the pathophysiology of PH. Statins are 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (HMG-CoA reductase inhibitors) with pleiotropic effects and are potential agents for the treatment of PH...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27905200/induced-sputum-to-detect-lung-pathogens-in-young-children-with-cystic-fibrosis
#14
Pamela D'Sylva, Daan Caudri, Nicole Shaw, Lidija Turkovic, Tonia Douglas, Jane Bew, Anthony D Keil, Stephen Stick, André Schultz
INTRODUCTION: Induced sputum sampling holds promise as a method for obtaining samples representative of the lower airways in young children. Collection of induced sputum samples in young children differs from older children and adults' as pharyngeal suctioning is often required. Our aim was to determine the sensitivity and specificity of induced sputum with and without airway clearance techniques to detect lower airway pathogens in children less than age 7 with cystic fibrosis. METHODS: Microbiological culture results were compared between 61 paired induced sputum and bronchoalveolar lavage fluid samples from young children with cystic fibrosis...
November 30, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27893197/importance-to-question-sinonasal-symptoms-and-to-perform-rhinoscopy-and-rhinomanometry-in-cystic-fibrosis-patients
#15
J M Bock, M Schien, C Fischer, L Naehrlich, M Kaeding, O Guntinas-Lichius, A Gerber, C Arnold, J G Mainz
OBJECTIVES: Cystic fibrosis (CF) patients almost regularly reveal sinonasal pathology. The purpose of this study was to assess association between objective and subjective measurements of sinonasal involvement comparing nasal airflow obtained by active anterior rhinomanometry (AAR), nasal endoscopic findings, and symptoms assessed with the Sino-Nasal Outcome Test-20 (SNOT-20). METHODS: Nasal cavities were explored by anterior rigid rhinoscopy and findings were compared to inspiratory nasal airflow measured by AAR to quantify nasal patency and subjective health-related quality of life in sinonasal disease obtained with the SNOT-20 questionnaire...
November 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27880037/suppressed-erythropoietin-expression-in-a-nitrofen-induced-congenital-diaphragmatic-hernia
#16
Hajime Takayasu, Koki Hagiwara, Kouji Masumoto
BACKGROUND: Erythropoietin (EPO), an essential stimulator of erythropoiesis produced by the fetal liver, is important both in vascular remodeling and modulation of the endothelial response in the pulmonary vasculature. In addition, EPO guides alveolar development, along with retinoic acid (RA). EPO is a direct target of RA, and the retinoid pathway is altered in the nitrofen-induced congenital diaphragmatic hernia (CDH) model. In the present study, we tested the hypothesis that the synthesis of EPO is suppressed in a rat model of CDH...
November 23, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27875026/disseminated-bcg-pneumonitis-revealing-severe-combined-immunodeficiencyxs-in-charge-syndrome
#17
Hyung Young Kim, Yoo-Mi Kim, Hee Ju Park
CHARGE (coloboma, heart defect, atresia choanae, retarded growth and development, genital hypoplasia, and ear anomalies/deafness) syndrome is a rare genetic disorder caused by CHD7 mutation and is related to immunodeficiency. A 6-month-old girl with right lung agenesis, congenital heart defects, and ear anomalies developed repeated and serious respiratory infection for a short period. She was clinically diagnosed with typical CHARGE syndrome with severe combined immunodeficiency (T-, B+, NK-); however, CHD7 mutation was not detected...
November 22, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27875025/adherence-and-barriers-to-hyperinsufflation-in-children-with-congenital-muscular-dystrophy
#18
John E Pascoe, Hemant Sawnani, Oscar H Mayer, Keith McConnell, Joseph M McDonough, Cynthia White, Anne M Rutkowski, Raouf S Amin, Avani C Modi
BACKGROUND: Congenital muscular dystrophy (CMD) is a rare, inherited neuromuscular disease characterized by progressive muscle weakness, thoracic insufficiency, and ultimately respiratory failure. Adherence to respiratory therapies in children with neuromuscular disorders is unknown. This study examined the multimodal assessment of adherence and barriers to 15 min, twice daily hyperinsufflation in children with CMD. Adherence was hypothesized to be greater than 50% and discomfort, embarrassment, and difficulty finding time were hypothesized to be barriers...
November 22, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27870384/high-flow-nasal-cannula-use-outside-of-the-icu-factors-associated-with-failure
#19
Kristina A Betters, Scott E Gillespie, Judson Miller, David Kotzbauer, Kiran B Hebbar
OBJECTIVES: High flow nasal cannula (HFNC) use is increasing in pediatrics. Few studies exist examining the safety of HFNC use outside the intensive care unit (ICU). This study aimed to characterize patient qualities associated with failure of HFNC use outside the ICU. STUDY DESIGN: A retrospective chart review of patients placed on HFNC outside the ICU from September 2011 to July 2013 was completed. Failure was defined as intubation or cardiopulmonary arrest. Two-sample tests and binary logistic regression determined significant factors associated with failed HFNC administration, both unadjusted and adjusted for hospital...
November 21, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27870227/aspergillosis-and-the-role-of-mucins-in-cystic-fibrosis
#20
REVIEW
Abigail C Cowley, David J Thornton, David W Denning, Alexander Horsley
The prevalence of aspergillosis in CF patients has until recently been underestimated, but increasing evidence suggests that it may play an important role in the progression of CF lung disease. In healthy airways, Aspergillus fumigatus can be efficiently removed from the lung by mechanisms such as mucociliary clearance and cough. However, these mechanisms are defective in CF, allowing pathogens such as A. fumigatus to germinate and establish chronic infections within the airways. The precise means by which A...
November 21, 2016: Pediatric Pulmonology
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