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https://www.readbyqxmd.com/read/29026731/not-all-children-with-cystic-fibrosis-have-abnormal-esophageal-neutralization-during-chemical-clearance-of-acid-reflux
#1
Frederick W Woodley, Melissa Moore-Clingenpeel, Rodrigo Strehl Machado, Christopher J Nemastil, Sudarshan R Jadcherla, Don Hayes, Benjamin T Kopp, Ajay Kaul, Carlo Di Lorenzo, Hayat Mousa
PURPOSE: Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range...
September 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29025281/understanding-treatment-adherence-with-the-health-belief-model-in-children-with-cystic-fibrosis
#2
Nicole R Dempster, Beth G Wildman, Tracy L Masterson, Gregory J Omlor
OBJECTIVE: Children's health beliefs are significantly related to their adherence; however, pediatric literature has rarely tested health-related theories as a whole. The goal of the present study was to evaluate the use of the health belief model (HBM) in understanding children's adherence, both globally and to individual treatment components. METHOD: Thirty-three patient-parent dyads completed questionnaires regarding health beliefs and adherence to medical regimens...
October 1, 2017: Health Education & Behavior: the Official Publication of the Society for Public Health Education
https://www.readbyqxmd.com/read/29018439/neutrophils-from-patients-with-primary-ciliary-dyskinesia-display-reduced-chemotaxis-to-cxcr2-ligands
#3
Maaike Cockx, Mieke Gouwy, Véronique Godding, Kris De Boeck, Jo Van Damme, Mieke Boon, Sofie Struyf
Primary ciliary dyskinesia (PCD), cystic fibrosis (CF), and chronic obstructive airway disease are characterized by neutrophilic inflammation in the lungs. In CF and chronic obstructive airway disease, improper functioning of neutrophils has been demonstrated. We hypothesized that the pulmonary damage in PCD might be aggravated by abnormal functioning neutrophils either as a primary consequence of the PCD mutation or secondary to chronic inflammation. We analyzed chemotactic responses and chemoattractant receptor expression profiles of peripheral blood neutrophils from 36 patients with PCD, 21 healthy children and 19 healthy adults...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28994899/prophylactic-antibiotics-for-preventing-pneumococcal-infection-in-children-with-sickle-cell-disease
#4
REVIEW
Angela E Rankine-Mullings, Shirley Owusu-Ofori
BACKGROUND: Persons with sickle cell disease (SCD) are particularly susceptible to infection. Infants and very young children are especially vulnerable. The 'Co-operative Study of Sickle Cell Disease' observed an incidence rate for pneumococcal septicaemia of 10 per 100 person years in children under the age of three years. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimens may be advisable for this population...
October 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28991007/the-optimal-approach-to-nutrition-and-cystic-fibrosis-latest-evidence-and-recommendations
#5
Francis M Hollander, Nicole M de Roos, Harry G M Heijerman
PURPOSE OF REVIEW: Cystic fibrosis (CF) is a progressive genetic disease that affects multiple organ systems. Therapy is directed to maintain and optimize nutritional status and pulmonary function, as these are key factors in survival. In this review, the most recent findings regarding nutritional management associated with pulmonary function and outcome will be explored. RECENT FINDINGS: Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status...
November 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28986027/cystic-fibrosis-related-cirrhosis
#6
Daniel H Leung, Michael R Narkewicz
While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.
September 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986024/cystic-fibrosis-and-gastroesophageal-reflux-disease
#7
Asim Maqbool, Ans Pauwels
Gastroesophageal reflux is common in children and adults with cystic fibrosis (CF). Pathological gastroesophageal reflux disease (GERD) is also frequent in patients of all ages with CF. This article reviews the pathophysiology, diagnostic work-up, management options, complications, and future directions in the evaluation and management of GERD - unique to and pertinent for - patients with CF in particular.
September 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28982773/inter-test-reproducibility-of-the-lung-clearance-index-measured-by-multiple-breath-washout
#8
Esther Oude Engberink, Felix Ratjen, Stephanie D Davis, George Retsch-Bogart, Reshma Amin, Sanja Stanojevic
The lung clearance index (LCI) has strong intra-test repeatability; however, the inter-test reproducibility of the LCI is poorly defined.The aim of the present study was to define a physiologically meaningful change in LCI in preschool children, which discriminates changes associated with disease progression from biological variability.Repeated LCI measurements from a longitudinal cohort study of children with cystic fibrosis and age-matched controls were collected to define the inter-visit reproducibility of the LCI...
October 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28982600/oxidative-stress-in-early-cystic-fibrosis-lung-disease-is-exacerbated-by-airway-glutathione-deficiency
#9
Nina Dickerhof, John F Pearson, Teagan S Hoskin, Luke J Berry, Rufus Turner, Peter D Sly, Anthony J Kettle
Neutrophil-derived myeloperoxidase (MPO) is recognized as a major source of oxidative stress at the airway surface of a cystic fibrosis (CF) lung where, despite limited evidence, the antioxidant glutathione is widely considered to be low. The aims of this study were to establish whether oxidative stress or glutathione status are associated with bronchiectasis and whether glutathione deficiency is inherently linked to CF or a consequence of oxidative stress. MPO was measured by ELISA in 577 bronchoalveolar lavage samples from 205 clinically-phenotyped infants and children with CF and 58 children without CF (ages 0...
October 2, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28981972/standard-versus-biofilm-antimicrobial-susceptibility-testing-to-guide-antibiotic-therapy-in-cystic-fibrosis
#10
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: The antibiotics used to treat pulmonary infections in people with cystic fibrosis are typically chosen based on the results of antimicrobial susceptibility testing performed on bacteria traditionally grown in a planktonic mode (grown in a liquid). However, there is considerable evidence to suggest that Pseudomonas aeruginosa actually grows in a biofilm (or slime layer) in the airways of people with cystic fibrosis with chronic pulmonary infections. Therefore, choosing antibiotics based on biofilm rather than conventional antimicrobial susceptibility testing could potentially improve response to treatment of Pseudomonas aeruginosa in people with cystic fibrosis...
October 5, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28978522/cigarette-smoke-activates-cftr-through-ros-stimulated-camp-signaling-in-human-bronchial-epithelial-cells
#11
Francis H Wong, Asmahan AbuArish, Elizabeth Matthes, Mark J Turner, Lana E Greene, Alexandre Cloutier, Renaud Robert, David Y Thomas, Gonzalo Cosa, Andre M Cantin, John W Hanrahan
Air pollution stimulates airway epithelial secretion through a cholinergic reflex that is unaffected in cystic fibrosis (CF), yet a strong correlation is observed between passive smoke exposure in the home and impaired lung function in CF children. Our aim was to study the effects of low smoke concentrations on CFTR function in vitro. Cigarette smoke extract stimulated robust anion secretion that was transient, mediated by cystic fibrosis transmembrane conductance regulator (CFTR), and dependent on cAMP-dependent protein kinase activation...
October 4, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28965267/exercise-performance-and-quality-of-life-in-children-with-cystic-fibrosis-and-mildly-impaired-lung-function-relation-with-antibiotic-treatments-and-hospitalization
#12
Kristof Vandekerckhove, Michiel Keyzer, Jasper Cornette, Ilse Coomans, Filip Pyl, Frans De Baets, Petra Schelstraete, Filomeen Haerynck, Daniel De Wolf, Sabine Van Daele, Jan Boone
This study evaluates the impact of antibiotic treatments and hospitalization on exercise performance and health-related quality of life (QOL) in children with mild cystic fibrosis (CF) lung disease. Forty-seven children between 7 and 17 years with mild CF underwent a maximal exercise test including spiro-ergometry and filled out a QOL-questionnaire (PedsQL™). Amount of antibiotic treatments (AB) and hospitalization days in the last 3 years were reviewed. FEV1% was mildly decreased (91.7 ± 17.9 L/min, p = 0...
September 30, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28964647/real-life-practice-of-sweat-testing-in-europe
#13
N Cirilli, K W Southern, R Buzzetti, J Barben, L Nährlich, A Munck, M Wilschanski, K De Boeck, N Derichs
Evidence based guidelines exist for sweat testing, which remains a key component of a diagnosis of cystic fibrosis (CF), especially following newborn bloodspot screening (NBS). There are emerging challenges with respect to maintaining a valid sweat test service, notably a smaller number of sweat tests ordered in regions with established NBS programmes where Pediatricians refer less children for sweat testing, younger patients and equipment becoming obsolete. The ECFS Diagnostic Network Working Group has undertaken a comprehensive survey to better define sweat test practice across Europe...
September 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28960390/prenatal-bowel-findings-in-male-siblings-with-a-confirmed-foxp3-mutation
#14
Catherine Griswold, Allison R Durica, Larry G Dennis, Ann F Jewell
There are multiple etiologies for fetal dilated bowel loops on ultrasonography (US), and we present a unique case of male siblings with a forkhead box P3 (FOXP3) mutation. Both children presented with fetal bowel anomalies on prenatal US. Family histories of cystic fibrosis and immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome were reported. Amniocentesis in both pregnancies identified a normal male karyotype and the familial mutation associated with IPEX syndrome. IPEX syndrome is one of a group of conditions known as congenital diarrhea disorders...
September 29, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28955051/early-detection-of-pulmonary-exacerbations-in-children-with-cystic-fibrosis-by-electronic-home-monitoring-of-symptoms-and-lung-function
#15
Marieke van Horck, Bjorn Winkens, Geertjan Wesseling, Dillys van Vliet, Kim van de Kant, Sanne Vaassen, Karin de Winter-de Groot, Ilja de Vreede, Quirijn Jöbsis, Edward Dompeling
Pulmonary exacerbations (PEx) in Cystic Fibrosis (CF) are associated with an increased morbidity and even mortality. We investigated whether early detection of PEx in children with CF is possible by electronic home monitoring of symptoms and lung function. During this one-year prospective multi-centre study, 49 children with CF were asked to use a home monitor three times a week. Measurements consisted of a respiratory symptom questionnaire and assessment of Forced Expiratory Volume in one second (FEV1). Linear mixed-effects and multiple logistic regression analyses were used...
September 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28952009/psychosocial-distress-and-knowledge-deficiencies-in-parents-of-children-in-ireland-who-carry-an-altered-cystic-fibrosis-gene
#16
S J Quigley, B Linnane, S Connellan, A Ward, P Ryan
Significant gaps have been identified in parental understanding of CF newborn screening and the consequences of carrying an altered CF gene. Seven potential causes of psychosocial adversity arising from false positive newborn screening for CF have been identified. The current study aimed to increase parents understanding of CF, reduce their levels of stress, and investigate psychosocial adversity arising from false-positive screening. This national study was run over one year in the Republic of Ireland. Parents were recruited for the study following a diagnostic sweat test confirming their child carried a single altered CF gene...
September 26, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28950435/lung-clearance-index-lci-as-a-predictor-of-exercise-limitation-among-cf-patients
#17
Vasiliki Avramidou, Elpis Hatziagorou, Asterios Kampouras, Helge Hebestreit, Eleana Kourouki, Fotis Kirvassilis, John Tsanakas
INTRODUCTION: FEV1 is often considered the gold standard to monitor lung disease in cystic fibrosis (CF). Recently, there has been increasing interest in multiple breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative or even more sensitive techniques. However, limited data exist on associations among the above methods. AIM: To evaluate the correlations between outcome measures of MBW and CPET and to examine if ventilation inhomogeneity can predict exercise intolerance...
September 26, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28950031/efficacy-and-effectiveness-of-rsv-immunoprophylaxis-in-children-with-cystic-fibrosis-an-unsolved-question-with-more-to-be-asked
#18
Rees L Lee, Rebekah F Brown, Tebeb Gebretsadik, Tina V Hartert, William D Dupont, Pingsheng Wu
The systematic review of the safety and efficacy of palivizumab in children with cystic fibrosis (CF) by Kua and Lee(1) demonstrates the woeful lack of definitive data in this area and correctly points out the critical need for additional well-designed studies. Their review examined 10 scientific reports and concluded that respiratory syncytial virus (RSV) immunoprophylaxis "may have a potential role in reducing RSV hospitalizations in children aged less than 2 years with CF".(1) While we fully agree with the authors' non-committal conclusions, it is important to highlight the extreme caution that must be exercised in interpreting this literature...
September 26, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28945313/severe-infantile-isolated-exocrine-pancreatic-insufficiency-caused-by-the-complete-functional-loss-of-the-spink1-gene
#19
Théa Venet, Emmanuelle Masson, Cécile Talbotec, Kareen Billiemaz, Renaud Touraine, Claire Gay, Sylvie Destombe, David N Cooper, Hugues Patural, Jian-Min Chen, Claude Férec
Exocrine pancreatic insufficiency (EPI) is rare in children, with most if not all cases occurring as part of syndromic conditions such as cystic fibrosis and Shwachman-Diamond syndrome. Here we report two cases, both presenting with severe EPI around 5 months of age. Characterized by diffuse pancreatic lipomatosis, they otherwise exhibited no remarkable deficiencies in other organs. Novel non-identical homozygous variants (a deletion removing the entire SPINK1 gene and an insertion of a full-length inverted Alu element into the 3'-untranslated region of the SPINK1 gene) resulting in the complete functional loss of the SPINK1 gene (encoding pancreatic secretory trypsin inhibitor) were identified in each patient...
September 25, 2017: Human Mutation
https://www.readbyqxmd.com/read/28941954/a-parental-report-of-youth-transition-readiness-the-parent-starx-questionnaire-starx-p-and-re-evaluation-of-the-starx-child-report
#20
Meaghan Nazareth, Laura Hart, Maria Ferris, Eniko Rak, Stephen Hooper, Miranda A L van Tilburg
PURPOSE: The STARx Questionnaire is a self-report measure of health care transition (HCT) readiness in youth with chronic diseases. We aimed to improve reliability and generalizability of the STARx and report initial reliability data on the STARx-P Questionnaire, a self-report measure of parent perspective on their child's HCT readiness. METHODS: Participants were recruited in several clinics from a large academic hospital in the southeastern USA and via the therapeutic summer camp for children with chronic disease...
September 20, 2017: Journal of Pediatric Nursing
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