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https://www.readbyqxmd.com/read/28526275/increasing-total-serum-ige-allergic-bronchopulmonary-aspergillosis-and-lung-function-in-cystic-fibrosis
#1
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
https://www.readbyqxmd.com/read/28523349/diagnosis-of-bronchiectasis-and-airway-wall-thickening-in-children-with-cystic-fibrosis-objective-airway-artery-quantification
#2
Wieying Kuo, Marleen de Bruijne, Jens Petersen, Kazem Nasserinejad, Hadiye Ozturk, Yong Chen, Adria Perez-Rovira, Harm A W M Tiddens
OBJECTIVES: To quantify airway and artery (AA)-dimensions in cystic fibrosis (CF) and control patients for objective CT diagnosis of bronchiectasis and airway wall thickness (AWT). METHODS: Spirometer-guided inspiratory and expiratory CTs of 11 CF and 12 control patients were collected retrospectively. Airway pathways were annotated semi-automatically to reconstruct three-dimensional bronchial trees. All visible AA-pairs were measured perpendicular to the airway axis...
May 18, 2017: European Radiology
https://www.readbyqxmd.com/read/28508992/palivizumab-prophylaxis-in-infants-with-cystic-fibrosis-does-not-delay-first-isolation-of-pseudomonas-aeruginosa-or-staphylococcus-aureus
#3
Clélia Buchs, Marie-Laure Dalphin, Stéphane Sanchez, Marie Perceval, Laurianne Coutier, Catherine Mainguy, Behrouz Kassaï-Koupaï, Philippe Reix
Respiratory syncytial virus (RSV) infections may worsen cystic fibrosis (CF) lung disease and favor Pseudomonas aeruginosa (Pa) or Staphylococcus aureus (Sa) acquisition, which is of particular importance in the youngest patients. We aimed to determine the effectiveness of PVZ on microbiological outcomes in young children with CF. We conducted a retrospective case-control study to compare these outcomes in children who systematically received PVZ (PVZ+; n = 40) or not (PVZ-; n = 140). One case was matched with at least three same-gender controls born the same year and month...
May 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28508087/the-vitamin-d-for-enhancing-the-immune-system-in-cystic-fibrosis-disc-trial-rationale-and-design-of-a-multi-center-double-blind-placebo-controlled-trial-of-high-dose-bolus-administration-of-vitamin-d3-during-acute-pulmonary-exacerbation-of-cystic-fibrosis
#4
Vin Tangpricha, Ellen M Smith, Jose Binongo, Suzanne E Judd, Thomas R Ziegler, Seth Walker, Rabindra Tirouvanziam, Susu M Zughaier, Moon Jeong Lee, Supavit Chesdachai, Wendy A Hermes, James F Chmiel, Amit Gaggar, Ruth E Grossmann, Patricia M Joseph, Jessica A Alvarez
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized, placebo-controlled trial that will test the hypothesis of whether high dose vitamin D given as a single oral bolus of 250,000 IU to adults with CF during a pulmonary exacerbation followed by a maintenance dose of vitamin D will improve time to next pulmonary exacerbation and re-hospitalization, improve survival and lung function compared to placebo and reduce the rates of pulmonary exacerbation,...
June 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/28507430/investigating-the-feasibility-of-text-message-reminders-to-improve-adherence-to-nebulized-medication-in-children-and-adolescents-with-cystic-fibrosis
#5
Robert W Morton, Heather E Elphick, Elaine Edwards, William J Daw, Noreen S West
BACKGROUND: Children with cystic fibrosis (CF) often have suboptimal adherence rates to nebulized medication. Adherence barriers cited include forgetting to take the nebulizers, due to busy home and social lives. Text message reminders have been shown to be effective at improving adherence rates in other chronic diseases such as asthma and diabetes. OBJECTIVE: The objective of this study was to assess the feasibility and efficacy of sending text reminders for a prolonged period of time to children with CF...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28505188/early-childhood-lung-function-is-a-stronger-predictor-of-adolescent-lung-function-in-cystic-fibrosis-than-early-pseudomonas-aeruginosa-infection
#6
Jessica E Pittman, Hannah Noah, Hollin E Calloway, Stephanie D Davis, Margaret W Leigh, Mitchell Drumm, Scott D Sagel, Frank J Accurso, Michael R Knowles, Marci K Sontag
OBJECTIVE: Pseudomonas aeruginosa has been suggested as a major determinant of poor pulmonary outcomes in cystic fibrosis (CF), although other factors play a role. Our objective was to investigate the association of early childhood Pseudomonas infection on differences in lung function in adolescence with CF. METHODS: Two populations of subjects with CF were studied: from the Gene Modifier Study (GMS), 346 F508del homozygotes with severe vs. mild adolescent lung disease, and from the Colorado Newborn Screen Study (NBS) 172 subjects diagnosed with CF by newborn screening...
2017: PloS One
https://www.readbyqxmd.com/read/28502446/pancreatic-disorders
#7
REVIEW
Aliye Uc, Douglas S Fishman
Once considered uncommon, pancreatic diseases are increasingly recognized in the pediatric age group. Acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis occur in children with an incidence approaching that of adults. Risk factors are broad, prompting the need for a completely different diagnostic and therapeutic approach in children. Although cystic fibrosis remains the most common cause of exocrine pancreatic insufficiency, other causes such as chronic pancreatitis may be as common as Shwachman Diamond syndrome...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28500860/interventions-for-preventing-silent-cerebral-infarcts-in-people-with-sickle-cell-disease
#8
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Carolyn Doree, Miguel R Abboud
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Silent cerebral infarcts are the commonest neurological complication in children and probably adults with SCD. Silent cerebral infarcts also affect academic performance, increase cognitive deficits and may lower intelligence quotient...
May 13, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28497760/the-intestinal-microbiome-and-paediatric-liver-disease
#9
REVIEW
Daniel H Leung, Dean Yimlamai
The intestinal microbiome has been the intense focus of recent study, but how the microbiota affects connected organs, such as the liver, has not been fully elucidated. The microbiome regulates intestinal permeability and helps to metabolise the human diet into small molecules, thus directly affecting liver health. Several studies have linked intestinal dysbiosis to the severity and progression of liver diseases, such as non-alcoholic fatty liver disease, non-alcoholic steatohepatitis, primary sclerosing cholangitis, total parenteral nutrition-associated liver disease, and cystic fibrosis-associated liver disease...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28492126/bacteria-isolated-from-the-airways-of-paediatric-patients-with-bronchiectasis-according-to-hiv-status
#10
Charl Verwey, Sithembiso Velaphi, Riaz Khan
BACKGROUND: Knowledge of which bacteria are found in the airways of paediatric patients with bronchiectasis unrelated to cystic fibrosis (CF) is important in defining empirical antibiotic guidelines for the treatment of acute infective exacerbations. OBJECTIVE: To describe the bacteria isolated from the airways of children with non-CF bronchiectasis according to their HIV status. METHODS: Records of children with non-CF bronchiectasis who attended the paediatric pulmonology clinic at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa, from April 2011 to March 2013, or were admitted to the hospital during that period, were reviewed...
April 25, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28489686/scaling-the-oxygen-uptake-efficiency-slope-for-body-size-in-cystic-fibrosis
#11
Owen William Tomlinson, Alan Robert Barker, Patrick John Oades, Craig Anthony Williams
PURPOSE: The aim of this study was to describe the relationship between body size and the oxygen uptake efficiency slope (OUES) in paediatric patients with cystic fibrosis (CF) and healthy controls (CON), in order to identify appropriate scaling procedures to adjust the influence of body size upon OUES. METHODS: The OUES was derived using maximal and submaximal points from cardiopulmonary exercise testing in 72 children (36 CF and 36 CON). OUES was subsequently scaled for stature, body mass (BM) and body surface area (BSA) using ratio-standard (Y/X) and allometric (Y/X) methods...
May 9, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28482390/-clinical-analysis-of-11-children-with-pancreatic-cystic-fibrosis
#12
G L Wang, C N Zhao, J Zhou, F H Yu, H Q Shen, J Zhang, S Y Zhao, X W Xu
Objective: To increase the recognition of pancreatic cystic fibrosis (PCF) in children and facilitate diagnosing and treatment of this rare entity. Method: This is a retrospective analysis of children who presented to Beijing Children's Hospital affiliated to Capital Medical University from January 2010 to December 2015. We describe their clinical features, laboratory testing and management. Result: Eleven children were diagnosed with PCF by genetic testing or sweat chloride test during these 5 years, including 4 boys and 7 girls...
May 4, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28481640/multiple-breath-washout-outcomes-are-sensitive-to-inflammation-and-infection-in-children-with-cystic-fibrosis
#13
Kathryn A Ramsey, Rachel E Foong, Jasmine Grdosic, Alana Harper, Billy Skoric, Charles Clem, Miriam Davis, Lidija Turkovic, Stephen M Stick, Stephanie D Davis, Sarath C Ranganathan, Graham L Hall
RATIONALE: The lung clearance index is a measure of ventilation distribution from the multiple breath washout technique. The lung clearance index is increased in the presence of lower respiratory tract inflammation and infection in infants with cystic fibrosis, however the associations during the preschool years are unknown. OBJECTIVES: We assessed the ability of the lung clearance index to detect the presence and extent of lower respiratory tract inflammation and infection in preschool children with cystic fibrosis...
May 8, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28479021/what-keeps-children-with-cystic-fibrosis-awake-at-night
#14
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
BACKGROUND: Sleep disturbance is common in children with cystic fibrosis (CF) however there are limited studies investigating the causes for poor sleep quality. In a cross sectional observational study we aimed to evaluate the clinical correlates of sleep disturbance in this population. METHODS: Children with CF (7-18years) free from pulmonary exacerbation completed medical review, overnight oximetry, the OSA-18 and 14days of actigraphy recordings with a sleep diary...
May 4, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28473256/malnutrition-among-hospitalized-children-in-the-united-states-changing-prevalence-clinical-correlates-and-practice-patterns-between-2002-and-2011
#15
Jennifer Carvalho-Salemi, Jason L Salemi, Molly R Wong-Vega, Kiara K Spooner, Marisa D Juarez, Stacey S Beer, Nicki L Canada
BACKGROUND: Pediatric malnutrition has been associated with adverse clinical outcomes, longer lengths of stay, and higher health care costs. OBJECTIVE: To characterize prevalence, temporal trends, and short-term clinical outcomes of coded diagnoses of pediatric malnutrition (CDM) across sociodemographic, clinical, and hospital characteristics from 2002 to 2011. DESIGN: This study is a retrospective cross-sectional analysis of nationally representative data from the Nationwide Inpatient Sample and the Kids' Inpatient Database...
April 28, 2017: Journal of the Academy of Nutrition and Dietetics
https://www.readbyqxmd.com/read/28470972/oral-calorie-supplements-for-cystic-fibrosis
#16
REVIEW
Rosalind L Smyth, Oli Rayner
BACKGROUND: Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. OBJECTIVES: To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life...
May 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28470018/use-of-carbapenems-polymyxins-and-tigecycline-in-united-states-children-s-hospitals-2010-2014
#17
Kathleen Chiotos, Rachael K Ross, Jennifer H Han, Matthew Miller, Jeffrey S Gerber
We characterized use of the carbapenems, polymyxins, and tigecycline in United States children's hospitals between 2010 and 2014. We found substantial variability in use across hospitals and overall decreased use over time. Most polymyxin and tigecycline use occurred in cystic fibrosis patients, and appendectomy was a common indication for carbapenem therapy.
2017: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/28465863/low-beta-adrenergic-sweat-responses-in-cystic-fibrosis-and-cystic-fibrosis-transmembrane-conductance-regulator-related-metabolic-syndrome-children
#18
Danieli Barino Salinas, Lucia Kang, Colleen Azen, Paul Quinton
β-adrenergically stimulated sweat secretion depends on the function of the cystic fibrosis transmembrane conductance regulator (CFTR) and discriminates between cystic fibrosis (CF) patients and healthy controls. Therefore, we sought to determine the feasibility, safety, and efficacy of assaying β-adrenergic sweating in children identified by CF newborn screening to help determine prognoses for individuals with CFTR-related metabolic syndrome (CRMS). Preschool age children with a positive newborn screening test for CF participated in this cross-sectional study...
March 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/28455785/hypertonic-saline-as-a-useful-tool-for-sputum-induction-and-pathogen-detection-in-cystic-fibrosis
#19
Adriana Carolina Marques Ferreira, Fernando Augusto Lima Marson, Milena Antonelli Cohen, Carmen Silvia Bertuzzo, Carlos Emilio Levy, Antonio Fernando Ribeiro, Jose Dirceu Ribeiro
PURPOSE: The aim of this study was to compare the qualitative and semi-quantitative detection of pathogens in the airway secretions of patients with cystic fibrosis (CF) and the sputum induction capacity before and after inhalation of 7% hypertonic saline solution (HSS). METHODS: The study enrolled 64 patients with CF. Airway secretions were collected from all enrolled patients with CF before and after inhalation of 7% HSS, and the samples were screened for pathogens...
April 28, 2017: Lung
https://www.readbyqxmd.com/read/28455274/protocol-of-a-pilot-study-of-technology-enabled-coproduction-in-pediatric-chronic-illness-care
#20
Heather C Kaplan, Sunny Narendra Thakkar, Lisa Burns, Barbara Chini, Dana Mh Dykes, Gary L McPhail, Erin Moore, Shehzad Ahmed Saeed, Ian Eslick, Peter A Margolis, Lisa Opipari-Arrigan
BACKGROUND: Pediatric chronic illness care models are traditionally organized around acute episodes of care and may not meet the needs of patients and their families. Interventions that extend the patient-clinician interaction beyond the health care visit, allow for asynchronous and bidirectional feedback loops that span visits and daily life, and facilitate seamless sharing of information are needed to support a care delivery system that is more collaborative, continuous, and data-driven...
April 28, 2017: JMIR Research Protocols
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