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Pediatric Pulmonology

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https://www.readbyqxmd.com/read/28786560/pediatric-lung-transplantation-and-end-of-life-care-in-cystic-fibrosis-barriers-and-successful-strategies
#1
Elisabeth Dellon, Samuel B Goldfarb, Don Hayes, Gregory S Sawicki, Joanne Wolfe, Debra Boyer
Pediatric lung transplantation has advanced over the years, providing a potential life-prolonging therapy to patients with cystic fibrosis. Despite this, many challenges in lung transplantation remain and result in worse outcomes than other solid organ transplants. As CF lung disease progresses, children and their caregivers are often simultaneously preparing for lung transplantation and end of life. In this article, we will discuss the current barriers to success in pediatric CF lung transplantation as well as approaches to end of life care in this population...
August 8, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28771980/interrupter-technique-in-infancy-higher-airway-resistance-and-lower-short-term-variability-in-preterm-versus-term-infants
#2
Jakob Usemann, Désirée Demann, Pinelopi Anagnostopoulou, Insa Korten, Olga Gorlanova, Sven Schulzke, Urs Frey, Philipp Latzin
BACKGROUND: In preschool children, measurement of airway resistance using interrupter technique (Rint) is feasible to assess the degree of bronchial obstruction. Although some studies measured Rint in infancy, values of Rint and its variability in preterm infants are unknown. In this study, Rint and its variability was measured at infancy and compared between healthy term and preterm infants. METHODS: High quality Rint measurements in term (n = 50) and preterm (n = 48) infants were obtained at postmenstrual age of 42-50 weeks in two study centers in Switzerland...
August 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28771972/a-new-insight-into-cftr-allele-frequency-in-brazil-through-next-generation-sequencing
#3
Luisa M Nunes, Roberto Ribeiro, Vivian D T Niewiadonski, Ester Sabino, Guilherme L Yamamoto, Débora R Bertola, Nelson Gaburo, Luiz Vicente R F da Silva Filho
BACKGROUND: As of 2013, fewer than 20% of patients in the Brazilian CF Registry had two CFTR mutations identified. The aim of this study was to sequence the coding region of the CFTR in Brazilian CF patients and determine the frequency of mutations in this cohort. METHODS: Patients with CF and those with suspected atypical CF or CFTR-related disorders were invited to enroll. Total DNA was extracted from blood samples, quantified, and purified. Library preparation was performed using Ion Xpress™ Plus gDNA and Amplicon Library preparation kits (Life Technologies), as well as sequencing using the Ion Torrent platform (Life Technologies)...
August 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28759157/mtor-notch3-signaling-mediates-pulmonary-hypertension-in-hypoxia-exposed-neonatal-rats-independent-of-changes-in-autophagy
#4
Julijana Ivanovska, Sparsh Shah, Mathew J Wong, Crystal Kantores, Amish Jain, Martin Post, Behzad Yeganeh, Robert P Jankov
BACKGROUND/AIM: Mammalian target of rapamycin (mTOR) is a pivotal regulator of cell proliferation, survival, and autophagy. Autophagy is increased in adult experimental chronic pulmonary hypertension (PHT), but its contributory role to pulmonary vascular disease remains uncertain and has yet to be explored in the neonatal animal. Notch is a major pro-proliferative pathway activated by mTOR. A direct relationship between autophagy and Notch signaling has not been previously explored. Our aim was to examine changes in mTOR-, Notch-, and autophagy-related pathways and the therapeutic effects of autophagy modulators in experimental chronic neonatal PHT secondary to chronic hypoxia...
July 31, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28752681/spontaneous-pneumomediastinum-emphysema-and-pulmonary-bullae-associated-with-refractory-mycoplasma-pneumoniae-pneumonia-in-a-child
#5
Lilin Huang, Huajia Chen, Shumei Peng
We report a 21-month-old child with spontaneous pneumomediastinum (PM), emphysema, and pulmonary bullae caused by Mycoplasma pneumonia (M. pneumoniae) pneumonia. The patient presented with stubborn dyspnea and wheezing although received integrated treatment, including anti-infection, anti-inflammation, and intravenous immunoglobulin. This case was unique in that pulmonary bullae kept increasing. PM and emphysema were refractory in our patient, which have been reported as benign and limited. Surgical intervention was implemented to ultimately cure the case...
July 28, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28749088/anxiety-in-youth-with-asthma-a-meta-analysis
#6
Joanne Dudeney, Louise Sharpe, Adam Jaffe, Emma B Jones, Caroline Hunt
OBJECTIVES: Anxiety often presents comorbidly with asthma in youth under 18; however, prevalence rates are unclear. The aim of this review was to provide an up-to-date analysis of the literature investigating the prevalence of anxiety disorders, and comparisons of anxiety disorders and symptomatology in youth with asthma, compared to those without. METHODS: A systematic search was conducted using the databases PsycINFO, MEDLINE, EMBASE, and CINAHL. RESULTS: The search process produced 15 studies (n = 7443) reporting data on youth with asthma and anxiety disorders, 11 studies (n = 10 332) reporting data on youth with and without asthma and anxiety disorders, and 28 studies (n = 5848) reporting data on youth with and without asthma and anxiety symptomatology...
July 27, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28741891/children-with-refractory-ards
#7
LETTER
Don Hayes, Dmitry Tumin, Warren Joshua Frazier
No abstract text is available yet for this article.
July 25, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28737283/prevalence-of-hearing-and-vestibular-loss-in-cystic-fibrosis-patients-exposed-to-aminoglycosides
#8
Jaynee A Handelsman, Samya Z Nasr, Crystal Pitts, William M King
AIM: Cystic Fibrosis (CF) patients frequently use aminoglycosides (AGS) to treat CF exacerbation due to colonization with Pseudomonas aeruginosa. Although AGS can cause vestibular and auditory sensory losses that can negatively impact quality of life, little is known about the prevalence of vestibular loss in this population. The aim of this study was to determine the prevalence of hearing loss and/or vestibular dysfunction in CF patients treated with AGS. METHODS: The relationship between hearing status and vestibular status was also investigated...
July 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28737279/exercise-capacity-is-not-decreased-in-children-who-have-undergone-lung-resection-early-in-life-for-congenital-thoracic-malformations-compared-to-healthy-age-matched-children
#9
Ashlee Dunn, Kasey Pearce, Robin Callister, Adam Collison, Matthew Morten, Payal Mandaliya, Lauren Platt, Ben Dascombe, Rajendra Kumar, Hiran Selvadurai, Paul D Robinson, Joerg Mattes
PURPOSE: The purpose of this study was to compare (i) the exercise capacity and (ii) lung function prior to and immediately post cardiopulmonary exercise tests (CPET) of children who underwent early life lung resection for Congenital Pulmonary Airway Malformations (CPAM) to healthy control children. METHOD: Eight children with CPAM (four males, age 9.6 ± 1.8 years) and eight control children without respiratory disease (three males, age 9.4 ± 1.4 years) performed a CPET on a cycle ergometer, during which maximal oxygen consumption (V̇O2max ) and heart rate were measured...
July 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28737262/overnight-delivery-of-hypertonic-saline-by-nasal-cannula-aerosol-for-cystic-fibrosis
#10
Timothy E Corcoran, Joseph E Godovchik, Karl H Donn, David R Busick, Jennifer Goralski, Landon W Locke, Matthew R Markovetz, Michael M Myerburg, Ashok Muthukrishnan, Lawrence Weber, Ryan T Lacy, Joseph M Pilewski
AIM: Inhaled hypertonic saline increases mucociliary clearance, improves pulmonary function, and decreases exacerbations in cystic fibrosis (CF) but contributes to the already significant treatment burden of CF. Overnight delivery of inhaled medications via a specially designed nasal cannula-aerosol device (Trans-nasal Pulmonary Aerosol Delivery [tPAD]) is an alternative approach. Here, we test whether overnight inhalation of hypertonic saline via tPAD improves mucociliary clearance and assess the tolerability of the device...
July 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28714612/weaning-from-long-term-continuous-positive-airway-pressure-or-noninvasive-ventilation-in-children
#11
Meriem Mastouri, Alessandro Amaddeo, Lucie Griffon, Annick Frapin, Samira Touil, Adriana Ramirez, Sonia Khirani, Brigitte Fauroux
OBJECTIVES: A significant number of children are able to discontinue long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) but the underlying disorders, weaning criteria, and outcome of these children have not been studied. STUDY DESIGN: Retrospective cohort follow up. SUBJECT SELECTION: Consecutive children who were weaned from long term CPAP/NIV between October 2013 and January 2016. METHODOLOGY: Underlying disorders, weaning criteria, and clinical outcome were analyzed...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28714611/mapping-targetable-inflammation-and-outcomes-with-cystic-fibrosis-biomarkers
#12
Olivia Giddings, Charles R Esther
Cystic fibrosis is characterized by an overly exuberant neutrophilic inflammatory response to pathogens and other stimuli that starts very early in disease. The overwhelming nature of this response is a primary cause of remodeling and destruction of the airways, suggesting that anti-inflammatory therapies could be beneficial in CF. However, finding therapies that can effectively reduce the inflammatory response without compromising host defenses remains elusive. New approaches towards mapping inflammatory targets promise to aid in developing novel therapeutic strategies and improve outcomes in individuals with CF...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28714601/cf-related-diabetes-containing-the-metabolic-miscreant-of-cystic-fibrosis
#13
Amir Moheet, Antoinette Moran
Cystic fibrosis-related diabetes (CFRD) is associated with both an increase in morbidity and mortality in people with cystic fibrosis (CF). With increased screening and improved life expectancy of people with CF, the prevalence of CFRD is expected to rise further. The underlying pathophysiological mechanisms causing glucose intolerance and diabetes in patients with CF are not well understood but both functional and structural abnormalities in islet cells are likely to have key roles. Insulin therapy improves health outcomes in patients with CF...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28714267/pulmonary-hypoplasia-resulting-from-prolonged-rupture-of-membranes-a-distinct-clinical-entity-with-instructive-experimental-models
#14
EDITORIAL
Lewis P Rubin
No abstract text is available yet for this article.
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28696535/bmi-predicts-exercise-induced-bronchoconstriction-in-asthmatic-boys
#15
Wilma J van Veen, Jean M M Driessen, Elin T G Kersten, Janneke C van Leeuwen, Marjolein G J Brusse-Keizer, Wim M C van Aalderen, Bernard J Thio
BACKGROUND: Exercise induced bronchoconstriction (EIB) is a frustrating morbidity of asthma in children. Obesity has been associated with asthma and with more severe EIB in asthmatic children. OBJECTIVES: To quantify the effect of BMI on the risk of the occurrence of EIB in children with asthma. METHODS: Data were collected from six studies in which exercise challenge tests were performed according to international guidelines. We included 212 Children aged 7-18 years, with a pediatrician-diagnosed mild-to-moderate asthma...
July 11, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28682006/air-trapping-in-early-cystic-fibrosis-lung-disease-does-ct-tell-the-full-story
#16
Tim Rosenow, Kathryn Ramsey, Lidija Turkovic, Conor P Murray, L Clara Mok, Graham L Hall, Stephen M Stick
INTRODUCTION: Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping". It is presumed to be localized hyperinflation due to small airway obstruction. In order to test this assumption, we compared air trapping extent to lung volumes measured on CT in young children with CF. MATERIALS AND METHODS: Children aged below 7 years undergoing inspiratory/expiratory CT were recruited from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis cohort...
July 6, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28682001/weight-correction-of-carbon-dioxide-diffusion-coefficient-dco2-reduces-its-inter-individual-variability-and-improves-its-correlation-with-blood-carbon-dioxide-levels-in-neonates-receiving-high-frequency-oscillatory-ventilation
#17
Gusztav Belteki, Benjamin Lin, Colin J Morley
BACKGROUND: Carbon-dioxide elimination during high-frequency oscillatory ventilation (HFOV) is thought to be proportional to the carbon dioxide diffusion coefficient (DCO2 ) which is calculated as frequency x (tidal volume)(2) . DCO2 can be used to as an indicator of CO2 elimination but values obtained in different patients cannot be directly compared. OBJECTIVES: To analyze the relationship between DCO2 , the weight-corrected DCO2 (DCO2 corr) and blood gas PCO2 values obtained from infants receiving HFOV...
July 6, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#18
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
July 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28672069/the-heterogeneity-of-viral-bronchiolitis-a-lack-of-universal-consensus-definitions
#19
REVIEW
David G Hancock, Billie Charles-Britton, Dani-Louise Dixon, Kevin D Forsyth
Viral bronchiolitis is one of the most common hospital presentations in infancy and as such represents a major healthcare burden worldwide. However despite this, there are currently no effective targeted therapies nor can those infants at highest risk for developing severe disease or subsequent respiratory morbidity be predicted on initial hospital presentation. Current definitions of bronchiolitis in the published literature vary significantly in terms of the age range at presentation, specific clinical symptoms, causative virus, and the inclusion or exclusion of infants with previous presentations and/or various comorbidities...
July 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28672068/effect-of-extending-the-time-after-bronchodilator-administration-on-identifying-bronchodilator-responsiveness-in-a-pediatric-pulmonary-clinic
#20
Jonathan D Cogen, Robert M DiBlasi, Ronald L Gibson, Jason S Debley
OBJECTIVES: American Thoracic Society/European Respiratory Society (ATS/ERS) spirometry interpretation guidelines recommend ≥15 min between pre- and post-bronchodilator testing to evaluate for a bronchodilator response. We aimed to lengthen the time between albuterol administration and post-bronchodilator testing to adhere to ATS/ERS guidelines and evaluated if lengthening this wait time would increase the percentage of patients classified as bronchodilator responsive. METHODS: We compared the proportion of patients with a positive bronchodilator response between two groups of children with asthma, one group in which post-bronchodilator administration wait times were not standardized (pre-intervention) to another in which the wait time was extended to 15 min to adhere to ATS/ERS standards (post-intervention)...
July 3, 2017: Pediatric Pulmonology
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