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https://www.readbyqxmd.com/read/28627192/-excess-weight-in-patients-with-cystic-fibrosis-is-it-always-beneficial
#1
David González Jiménez, Rosana Muñoz-Codoceo, María Garriga-García, Manuel Molina-Arias, Marina Álvarez-Beltrán, Ruth García-Romero, Cecilia Martínez-Costa, Silvia María Meavilla-Olivas, Luis Rodrigo Peña-Quintana, Silvia Gallego Gutiérrez, José Manuel Marugán de Miguelsanz, Lucrecia Suárez Cortina, Esperanza Natividad Castejón Ponce, Rosaura Leis Trabazo, Fayna Martín Cruz, Juan José Díaz Martín, Carlos Bousoño García
INTRODUCTION: The primary objective of this study was to find out the prevalence of overweight and obese status, as well as their association to pulmonary function, total cholesterol and vitamin D in patients with cystic fibrosis (CF). MATERIALS AND METHODS: This is a multicenter descriptive and cross-sectional study. Twelve Spanish hospitals participated. 451 patients with CF were included. Adults were classified according to body mass index (BMI) and children were classified according to BMI percentiles (WHO tables)...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28616421/cystic-fibrosis-prevalence-among-a-group-of-high-risk-children-in-the-main-referral-children-hospital-in-iran
#2
Mohammad Reza Modaresi, Jamal Faghinia, Mohsen Reisi, Majid Keivanfar, Shiva Navaie, Javad Seyyedi, Faride Baharzade
BACKGROUND: Knowledge about cystic fibrosis (CF) in Iran is very limited. The objective of this study was to determine the prevalence of CF among a group of high-risk children with suggestive clinical features in the main referral hospital in Iran. MATERIALS AND METHODS: This study children consisted of 505 patients who had presented with one or more of the following symptoms: chronic or recurrent respiratory symptoms, gastrointestinal symptoms as rectal prolapse, steatorrhea, hepatobiliary disease as prolonged jaundice, failure to thrive, hyperglycemia and glycosuria, hypochloremic metabolic alkalosis, hypoprothrombinemia, anemia or edema, and positive family history of CF...
2017: Journal of Education and Health Promotion
https://www.readbyqxmd.com/read/28611970/bronchiectasis-in-children-current-concepts-in-immunology-and-microbiology
#3
REVIEW
Susan J Pizzutto, Kim M Hare, John W Upham
Bronchiectasis is a complex chronic respiratory condition traditionally characterized by chronic infection, airway inflammation, and progressive decline in lung function. Early diagnosis and intensive treatment protocols can stabilize or even improve the clinical prognosis of children with bronchiectasis. However, understanding the host immunologic mechanisms that contribute to recurrent infection and prolonged inflammation has been identified as an important area of research that would contribute substantially to effective prevention strategies for children at risk of bronchiectasis...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28608624/four-case-reports-of-chinese-cystic-fibrosis-patients-and-literature-review
#4
Juan Xu, Yong Yin, Lei Zhang, Jing Zhang, Shuhua Yuan, Hao Zhang
AIM: Cystic fibrosis (CF) is an extremely rare disease in Asians. Here, we report four Chinese children with CF and review the literature about Chinese CF patients. METHODS: The cystic fibrosis transmembrane conductance regulator (CFTR) gene testing was performed on four suspected patients for CF screening. We also reviewed the literature about Chinese CF patients from 1970s. The clinical data of all these CF patients were summarized. RESULTS: We diagnosed four CF patients who had mutations in the CFTR gene...
June 13, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28606619/cystic-fibrosis-transmembrane-conductance-regulator-modulators-for-children
#5
Carla Colombo
No abstract text is available yet for this article.
June 8, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28599957/pulmonary-surfactant-dysfunction-in-pediatric-cystic-fibrosis-mechanisms-and-reversal-with-a-lipid-sequestering-drug
#6
Lasantha Gunasekara, Mustafa Al-Saiedy, Francis Green, Ryan Pratt, Candice Bjornson, Ailian Yang, W Michael Schoel, Ian Mitchell, Mary Brindle, Mark Montgomery, Elizabeth Keys, John Dennis, Grishma Shrestha, Matthias Amrein
BACKGROUND: Airway surfactant is impaired in cystic fibrosis (CF) and associated with declines in pulmonary function. We hypothesized that surfactant dysfunction in CF is due to an excess of cholesterol with an interaction with oxidation. METHODS: Surfactant was extracted from bronchial lavage fluid from children with CF and surface tension, and lipid content, inflammatory cells and microbial flora were determined. Dysfunctional surfactant samples were re-tested with a lipid-sequestering agent, methyl-β-cyclodextrin (MβCD)...
June 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28599639/seasonality-of-acquisition-of-respiratory-bacterial-pathogens-in-young-children-with-cystic-fibrosis
#7
Kevin J Psoter, Anneclaire J De Roos, Jon Wakefield, Jonathan D Mayer, Margaret Rosenfeld
BACKGROUND: Seasonal variations are often observed for respiratory tract infections; however, limited information is available regarding seasonal patterns of acquisition of common cystic fibrosis (CF)-related respiratory pathogens. We previously reported differential seasonal acquisition of Pseudomonas aeruginosa in young children with CF and no such variation for methicillin-susceptible Staphylococcus aureus acquisition. The purpose of this study was to describe and compare the seasonal incidence of acquisition of other respiratory bacterial pathogens in young children with CF...
June 9, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28597092/factors-associated-with-changes-in-health-related-quality-of-life-in-children-with-cystic-fibrosis-during-1-year-follow-up
#8
Marieke van Horck, Bjorn Winkens, Geertjan Wesseling, Karin de Winter-de Groot, Ilja de Vreede, Quirijn Jöbsis, Edward Dompeling
There are limited data on health-related quality of life (HRQoL) changes over time in children with cystic fibrosis (CF). We investigated associations between clinical and treatment variables with changes in HRQoL during 1 year. Forty-nine children with CF aged 6-18 years were followed in this multicentre, observational cohort study during 1 year. HRQoL was measured by the validated disease specific cystic fibrosis questionnaire-revised (CFQ-R). The CFQ-R total score as well as most domain scores improved significantly (8...
June 9, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28596950/review-quality-of-life-in-children-with-non-cystic-fibrosis-bronchiectasis
#9
REVIEW
Anna Marie Nathan, Jessie Anne de Bruyne, Kah Peng Eg, Surendran Thavagnanam
Non-cystic fibrosis bronchiectasis (NCFB) has gained renewed interest, due to its increasing health-care burden. Annual mortality statistics in England and Wales showed that under 1,000 people die from bronchiectasis each year, and this number is increasing by 3% yearly. Unfortunately, there is a severe lack of well-powered, randomized controlled trials to guide clinicians how to manage NCFB effectively. Quality-of-life (QOL) measures in NCFB are an important aspect of clinical care that has not been studied well...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28586522/is-sweat-chloride-predictive-of-severity-of-cystic-fibrosis-lung-disease-assessed-by-chest-computed-tomography
#10
Daan Caudri, David Zitter, Inez Bronsveld, Harm Tiddens
BACKGROUND: Cystic Fibrosis (CF) lung disease is characterized by a marked heterogeneity. Sweat chloride-level is a functional marker of the CF Transmembrane Regulator (CFTR) protein and could be an important predictor of later disease severity. METHODS: In this retrospective analysis children from the Rotterdam CF clinic with available sweat chloride level at diagnosis and at least one routine spirometry-controlled volumetric chest CT scan in follow-up were included...
June 6, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28583497/safety-of-endoscopic-sinus-surgery-in-children-with-cystic-fibrosis
#11
Dmitry Tumin, Don Hayes, Stephen E Kirkby, Joseph D Tobias, Christopher McKee
INTRODUCTION: Data on the safety of endoscopic sinus surgery (ESS) are limited in children with cystic fibrosis (CF). We used a multi-institutional surgical registry to examine ESS outcomes in children with CF. METHODS: The 2014-2015 American College of Surgeons' National Surgical Quality Improvement Program-Pediatric database was queried for patients age <18 years undergoing elective ESS. Prolonged hospital stay (>1 day), 30-day readmission, and 30-day unplanned reoperation were compared according to presence of CF diagnosis...
July 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28579360/complications-of-long-and-intermediate-term-venous-catheters-in-cystic-fibrosis-patients-a-multicenter-study
#12
Teresa L May, Alex H Gifford, Thomas Lahiri, Adam Black, Janet Trang, Alexandra G Cornell, Karyll Gonzalez, Scott Morin, Mark Napier, Christine W Duarte, Jonathan B Zuckerman
BACKGROUND: Totally implantable venous access devices (TIVADs) or peripherally inserted central venous catheters (PICCs) are commonly used in the care of patients with cystic fibrosis (CF), but they are associated with various complications, including thrombosis, infection, and insertion site symptoms. METHODS: We conducted a retrospective review of PICC and TIVAD use in adults and children with CF over an 8-year period at 3 accredited care centers. Patient attributes included CFTR genotype, comorbidities, lung function, body mass index, use of anticoagulation, and respiratory tract microbiology...
June 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28575039/assessment-of-pathologic-increase-in-liver-stiffness-enables-earlier-diagnosis-of-cfld-results-from-a-prospective-longitudinal-cohort-study
#13
Victoria Klotter, Caroline Gunchick, Enno Siemers, Timo Rath, Helge Hudel, Lutz Naehrlich, Martin Roderfeld, Elke Roeb
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6...
2017: PloS One
https://www.readbyqxmd.com/read/28559466/inhaled-drug-therapy-2016-the-year-in-review
#14
Rajiv Dhand
Some recent salient publications related to inhaled drug therapy are discussed. Unexpectedly, a 2.5-μg once-daily dose of tiotropium (Respimat) had greater efficacy than the 5.0-μg daily dose. Occurrence of a reverse dose response serves to caution us that administering more drug is not always beneficial. Small-airway inflammation contributes to pathogenesis of asthma, especially severe asthma. However, there is no conclusive evidence that the use of small-particle aerosols to target small airways improves clinical outcomes in controlled clinical trials...
May 30, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28554723/new-treatments-targeting-the-basic-defects-in-cystic-fibrosis
#15
Isabelle Fajac, Claire E Wainwright
Cystic fibrosis (CF) is a monogenic autosomal recessive disorder affecting around 75,000 individuals worldwide. It is a multi-system disease but the main morbidity and mortality is caused by chronic lung disease. Due to newborn screening, a multidisciplinary approach to care and intensive symptomatic treatment, the prognosis has dramatically improved over the last decades and there are currently more adults than children in many countries. However, CF is still a very severe disease with a current median age of life expectancy in the fourth decade of life...
May 26, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28552547/spirometry-assisted-high-resolution-chest-computed-tomography-in-children-is-it-worth-the-effort
#16
Jeffrey Parke Otjen, Jonathan Ogden Swanson, Assaf Oron, Robert M DiBlasi, Tim Swortzel, Jade Adriana Marie van Well, Eva Anna Elisabeth Gommers, Margaret Rosenfeld
BACKGROUND: Image quality of high resolution chest computed tomographies (HRCTs) depends on adequate breath holds at end inspiration and end expiration. We hypothesized that implementation of spirometry-assisted breath holds in children undergoing HRCTs would improve image quality over that obtained with voluntary breath holds by decreasing motion artifact and atelectasis. METHODS: This is a retrospective case-control study of HRCTs obtained at a tertiary care children's hospital before and after implementation of a spirometry-assisted CT protocol, in which children ≥8 years of age are first trained in supine slow vital capacity maneuvers and then repeat the maneuvers in the CT scanner, coached by a respiratory therapist...
April 7, 2017: Current Problems in Diagnostic Radiology
https://www.readbyqxmd.com/read/28547678/immunosuppression-drug-therapy-in-lung-transplantation-for-cystic-fibrosis
#17
REVIEW
Pamela Burcham, Lisa Sarzynski, Sabrina Khalfoun, Kimberly J Novak, Julie C Miller, Dmitry Tumin, Don Hayes
Cystic fibrosis (CF) is a common indication for lung transplantation (LTx) in children and adults with severe and irreversible lung disease. In the setting of LTx in the CF population, immunosuppressive medications are used to prevent allograft rejection despite the majority of these patients being chronically infected with numerous, and often antibiotic-resistant, pathogens. There is limited evidence for the optimal post-LTx immunosuppression regimen in patients with CF, particularly in children. This article provides a review of immunosuppression regimens in the pediatric and adult CF post-LTx population, investigating drug dosing and monitoring, and medication combinations...
May 25, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28543609/understanding-the-school-experiences-of-children-and-adolescents-with-serious-chronic-illness-a-systematic-meta-review
#18
REVIEW
A Lum, C E Wakefield, B Donnan, M A Burns, J E Fardell, G M Marshall
BACKGROUND: Serious chronic illness can have a detrimental effect on school attendance, participation and engagement, leaving affected students at risk of failing to meet their developmental potential. An improved understanding of factors that help to explain or mitigate this risk can help educators and health professionals deliver the most effective support. This meta-review critiqued the available evidence examining the link between six chronic illnesses (asthma, cancer, chronic kidney diseases, heart diseases, cystic fibrosis and gastrointestinal diseases) and children's and adolescents' school experiences and outcomes, as well as investigating the medical, school, psychosocial and sociodemographic factors that are linked to poorer or better school outcomes...
May 23, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28540765/an-update-on-pediatric-bronchiectasis
#19
Danielle F Wurzel, Anne B Chang
The prevalence and awareness of bronchiectasis not related to cystic fibrosis (CF) is increasing and it is now recognized as a major cause of respiratory morbidity, mortality and healthcare utilization worldwide. The need to elucidate the early origins of bronchiectasis is increasingly appreciated and has been identified as an important research priority. Current treatments for pediatric bronchiectasis are limited to antimicrobials, airway clearance techniques and vaccination. Several new drugs targeting airway inflammation are currently in development...
June 5, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28526275/increasing-total-serum-ige-allergic-bronchopulmonary-aspergillosis-and-lung-function-in-cystic-fibrosis
#20
Florian Gothe, Matthias Kappler, Matthias Griese
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disorder contributing to lung disease in cystic fibrosis (CF) and challenging to diagnose. OBJECTIVE: This study analyzed the predictive value of increasing total IgE (t-IgE) levels in a CF cohort alongside with clinical and serologic data. METHODS: A total of 387 children and young adults were followed from 2000 to 2006 and retrospectively classified into 6 groups...
May 16, 2017: Journal of Allergy and Clinical Immunology in Practice
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