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cystic fibrosis children

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https://www.readbyqxmd.com/read/29469935/are-children-with-chronic-illnesses-requiring-dietary-therapy-at-risk-for-disordered-eating-or-eating-disorders-a-systematic-review
#1
REVIEW
Jenny H Conviser, Sheehan D Fisher, Susanna A McColley
OBJECTIVE: Pediatric chronic illnesses (CI) can affect a child's mental health. Chronic illnesses with treatment regimens that specify a therapeutic diet may place the child at increased risk for disordered eating and specific eating disorders (ED). The aim of this review is to examine the relation between diet-treated CI and disordered eating and to determine the order of onset to infer directionality. Diet-treated CI is hypothesized to precede and to be associated with disordered eating...
February 22, 2018: International Journal of Eating Disorders
https://www.readbyqxmd.com/read/29469024/association-between-body-composition-and-pulmonary-function-in-children-and-young-people-with-cystic-fibrosis
#2
Patrizia Calella, Giuliana Valerio, Matt Thomas, Helen McCabe, Jake Taylor, Malcolm Brodlie, Mario Siervo
OBJECTIVES: Body mass index (BMI) has significant limitations when assessing nutritional status in pediatric patients with cystic fibrosis (CF). We evaluated whether measurements of lean body mass (LBM) and fat mass (FM) are more sensitive nutritional parameters by testing their association with pulmonary function in adolescent patients with CF. METHODS: Sixty-nine male and female adolescents with CF were studied (age: 14.5 ± 2.3, BMI: 19.5 ± 2.3 kg/m2 )...
December 5, 2017: Nutrition
https://www.readbyqxmd.com/read/29465158/evaluation-of-adipokines-in-children-with-cystic-fibrosis
#3
Edyta Machura, Maria Szczepańska, Elżbieta Świętochowska, Franciszek Halkiewicz, Małgorzata Barć-Czarnecka, Katarzyna Ziora, Dariusz Ziora
INTRODUCTION: Patients with CF present numerous pathological conditions such as malnutrition, depletion of fat-free mass, metabolic disturbances (abnormal glucose metabolism, increased insulin resistance, chronic energy deficit, local and chronic inflammation), which could affect or be associated with altered adipokines concentrationMaterial and Methods: We aimed in this study to investigate the levels of selected adipokines such as resistin, apelin, adiponectin to demonstrate their application as possible markers of inflammation...
February 21, 2018: Endokrynologia Polska
https://www.readbyqxmd.com/read/29459095/the-pharmacist-s-role-in-supporting-people-living-with-cystic-fibrosis
#4
Olufunmilola Abraham, Jenny S Li, Kathy E Monangai, Alison M Feathers, Daniel Weiner
OBJECTIVES: To describe the critical need for pharmacists' involvement in outpatient care for people living with cystic fibrosis (CF). DATA SOURCES: Not applicable. SUMMARY: CF is a pulmonary condition that affects more than 30,000 children and adults in the United States and 70,000 people worldwide. Various complex medication regimens are given to patients with CF, some depending on the type of mutation they have in their CF transmembrane conductance regulator protein...
February 16, 2018: Journal of the American Pharmacists Association: JAPhA
https://www.readbyqxmd.com/read/29457316/assessment-of-pulmonary-structure-function-relationships-in-young-children-and-adolescents-with-cystic-fibrosis-by-multivolume-proton-mri-and-ct
#5
Francesca Pennati, David J Roach, John P Clancy, Alan S Brody, Robert J Fleck, Andrea Aliverti, Jason C Woods
BACKGROUND: Lung disease is the most frequent cause of morbidity and mortality in patients with cystic fibrosis (CF), and there is a shortage of sensitive biomarkers able to regionally monitor disease progression and to assess early responses to therapy. PURPOSE: To determine the feasibility of noncontrast-enhanced multivolume MRI, which assesses intensity changes between expiratory and inspiratory breath-hold images, to detect and quantify regional ventilation abnormalities in CF lung disease, with a focus on the structure-function relationship...
February 19, 2018: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29449440/lung-clearance-index-to-monitor-treatment-response-in-pulmonary-exacerbations-in-preschool-children-with-cystic-fibrosis
#6
Jonathan H Rayment, Sanja Stanojevic, Stephanie D Davis, George Retsch-Bogart, Felix Ratjen
BACKGROUND: Antibiotic treatment for pulmonary symptoms in preschool children with cystic fibrosis (CF) varies among clinicians. The lung clearance index (LCI) is sensitive to early CF lung disease, but its utility to monitor pulmonary exacerbations in young children has not been assessed. OBJECTIVE: We aim to (1) understand how LCI changes during lower respiratory tract symptoms relative to a recent clinically stable measurement, (2) determine whether LCI can identify antibiotic treatment response and (3) compare LCI changes to changes in spirometric indices...
February 15, 2018: Thorax
https://www.readbyqxmd.com/read/29434062/glucose-tolerance-insulin-secretion-sensitivity-characteristics-in-indian-children-with-cystic-fibrosis-a-pilot-study
#7
Vandana Jain, Santosh Kumar, Naval K Vikram, Mani Kalaivani, Surya Prakash Bhatt, Rajni Sharma, Kumar Kabra Sushil
Background & objectives: Cystic fibrosis (CF) is a life-limiting genetic condition resulting in chronic respiratory infections, pancreatic enzyme insufficiency and associated complications. This pilot study was undertaken to assess the glucose tolerance and insulin secretion and sensitivity among Indian children with CF. Methods: Children with CF under regular follow up at the Paediatric Pulmonology Clinic of a tertiary care hospital in New Delhi, India, were enrolled...
October 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/29417077/ciprofloxacin-use-in-hospitalized-children-approved-or-off-label
#8
Toktam Faghihi, Leila Yavari Tekmehdash, Mania Radfar, Kheirollah Gholami
Objective: Fluoroquinolones are not routinely used as the first-line antimicrobial therapy in pediatrics. The American Academy of Pediatrics (AAP) and the United States Food and Drug Administration (FDA) approved fluoroquinolones on certain indications in children. The aim of this study was to evaluate to what extent and how ciprofloxacin is used on approved indication or as off-label. Besides, dose adequacy and treatment duration were assessed. Methods: In a 10-month observational study, all children receiving systemic ciprofloxacin were assessed...
October 2017: Journal of Research in Pharmacy Practice
https://www.readbyqxmd.com/read/29412427/association-of-nutritional-status-with-lung-function-and-morbidity-in-children-and-adolescents-with-cystic-fibrosis-a-36-month-cohort-study
#9
Daniela Barbieri Hauschild, Anauã Franco Rosa, Julia Carvalho Ventura, Eliana Barbosa, Emília Addison Machado Moreira, Norberto Ludwig Neto, Yara Maria Franco Moreno
OBJECTIVE: To evaluate the association between nutritional status, lung function and morbidity in a 36-month cohort in children and adolescents with cystic fibrosis. METHODS: Prospective cohort of children and adolescents with cystic fibrosis aged 1-15 years. At the baseline, the nutritional status was determined by weight-for-height and body mass index-for-age for children <2 years and ≥2 years, respectively, and classified as: nutritional failure, nutritional risk and acceptable; and by the 50th percentile, according to the World Health Organization (WHO) growth charts...
January 15, 2018: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/29412394/biographical-ruptures-and-flows-in-the-family-experience-and-trajectory-of-children-with-cystic-fibrosis
#10
Marcelo Eduardo Pfeiffer Castellanos, Nelson Filice de Barros, Sandra Straccialano Coelho
Biographical disruption (BD) became a core concept of sociological studies on the chronic illness experience by showing how this event can be strongly affected by ruptures in the ways of living and organizing the biographical trajectory through narratives. Critical reviews have pointed out that the widespread use of this concept was not sufficiently attentive to its analytical limits, e.g. addressing experiences of children with genetic diseases, when biographic flows (BF) rather than BD would be probably found...
February 2018: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/29411860/self-management-for-bronchiectasis
#11
REVIEW
Carol Kelly, Seamus Grundy, Dave Lynes, David Jw Evans, Sharada Gudur, Stephen J Milan, Sally Spencer
BACKGROUND: Bronchiectasis is a long term respiratory condition with an increasing rate of diagnosis. It is associated with persistent symptoms, repeated infective exacerbations, and reduced quality of life, imposing a burden on individuals and healthcare systems. The main aims of therapeutic management are to reduce exacerbations and improve quality of life. Self-management interventions are potentially important for empowering people with bronchiectasis to manage their condition more effectively and to seek care in a timely manner...
February 7, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29405664/comparison-of-bronchoscopy-and-bronchoalveolar-lavage-findings-in-three-types-of-suppurative-lung-disease
#12
Jorrit J V de Vries, Anne B Chang, Julie M Marchant
BACKGROUND: Endobronchial suppuration is present in children with protracted bacterial bronchitis (PBB), bronchiectasis, and cystic fibrosis (CF). However, no studies have directly compared bronchoscopy and bronchoalveolar lavage (BAL) findings across these conditions within a single center using the same techniques and with shared community pathogens. AIM: To determine; (i) the bronchoscopic findings and BAL microbiology and cellularity among children with these conditions and; (ii) the relationship between bacterial pathogens, airway cellularity and aberrant macroscopic bronchoscopic findings...
February 6, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29398489/young-patients-with-cystic-fibrosis-demonstrate-subtle-alterations-of-the-cardiovascular-system
#13
Jacobien B Eising, Cornelis K van der Ent, Arco J Teske, Maaike M Vanderschuren, Cuno S P M Uiterwaal, Folkert J Meijboom
BACKGROUND: As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children. METHODS: 33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study...
February 2, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29398400/micrornas-link-chronic-inflammation-in-childhood-to-growth-impairment-and-insulin-resistance
#14
REVIEW
Francesca Cirillo, Pietro Lazzeroni, Cecilia Catellani, Chiara Sartori, Sergio Amarri, Maria Elisabeth Street
MicroRNAs are involved in multiple pathophysiological networks and in the pathogenesis of a broad spectrum of human disorders, including cancer and inflammatory diseases. Impaired linear growth is encountered in children with chronic inflammatory conditions such as cystic fibrosis, inflammatory bowel diseases, juvenile idiopathic arthritis, celiac disease and in subjects born intrauterine growth restricted/small for gestational age. Children with inflammatory conditions may also be at risk of developing insulin resistance as a result of the inflammatory process and concurrent therapy...
February 2, 2018: Cytokine & Growth Factor Reviews
https://www.readbyqxmd.com/read/29396026/the-effect-of-enteral-tube-feeding-in-cystic-fibrosis-a-registry-based-study
#15
Denis Libeert, Dimitri Declercq, Simeon Wanyama, Muriel Thomas, Sabine Van Daele, Frans De Baets, Stephanie Van Biervliet
BACKGROUND: Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal. METHODS: A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls. RESULTS: At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls...
January 21, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29396025/early-life-growth-patterns-persist-for-12%C3%A2-years-and-impact-pulmonary-outcomes-in-cystic-fibrosis
#16
Don B Sanders, Zhumin Zhang, Philip M Farrell, HuiChuan J Lai
BACKGROUND: In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years. This study examined whether these benefits are sustained through 12 years of age. METHODS: Longitudinal growth from 76 children with CF enrolled in the Wisconsin CF Neonatal Screening Project was examined and categorized into 5 groups: R12, R6, and R2, representing Responders who maintained growth improvement to age 12, 6, and 2 years, respectively, and I6 and N6, representing Non-responders whose growth did and did not improve during ages 2-6 years, respectively...
January 30, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29385901/opat-proof-of-concept-in-a-peripheral-belgian-hospital-after-review-of-the-literature
#17
Annick Smismans, Astrid Vantrappen, Freija Verbiest, Christophe Indevuyst, Bea Van den Poel, Sandrina von Winckelmann, Annelore Peeters, Sara Ombelet, Peter Lybeert, Andre Heremans, Eric Frans, Erwin Ho, Johan Frans
Since its introduction in the 1970s in the United States, outpatient parenteral antibiotic/antimicrobial therapy (OPAT) has been adopted internationally for long-term intravenous (IV) treatment of stable infectious diseases. The aim is to provide a safe and successful completion of IV antimicrobial treatment at the ambulatory care center or at home without complications and costs associated with hospitalization. OPAT implementation has been accelerated by progress in vascular access devices, newly available antibiotics, the emphasis on cost-savings, as well as an improved patient comfort and a reduced incidence of health care associated infections with a similar outcome...
January 31, 2018: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29378945/adaptation-of-commensal-proliferating-escherichia-coli-to-the-intestinal-tract-of-young-children-with-cystic-fibrosis
#18
Susana Matamouros, Hillary S Hayden, Kyle R Hager, Mitchell J Brittnacher, Kristina Lachance, Eli J Weiss, Christopher E Pope, Anne-Flore Imhaus, Colin P McNally, Elhanan Borenstein, Lucas R Hoffman, Samuel I Miller
The mature human gut microbiota is established during the first years of life, and altered intestinal microbiomes have been associated with several human health disorders. Escherichia coli usually represents less than 1% of the human intestinal microbiome, whereas in cystic fibrosis (CF), greater than 50% relative abundance is common and correlates with intestinal inflammation and fecal fat malabsorption. Despite the proliferation of E. coli and other Proteobacteria in conditions involving chronic gastrointestinal tract inflammation, little is known about adaptation of specific characteristics associated with microbiota clonal expansion...
January 29, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29372944/determination-of-the-lung-clearance-index-lci-in-a-paediatric-cystic-fibrosis-cohort
#19
M Mulligan, L Collins, C P Dunne, L Keane, B Linnane
The pathogenesis of CF lung disease may start in infancy. Therefore, it is important to monitor the early stages of its progress. The Exhalyzer D is the first commercially available device designed to measure lung ventilation inhomogeneity at any age. This study was conducted to assess the performance and feasibility of using the Exhalyzer D in a paediatric CF clinic. A total of 91 subjects were recruited (23 controls, and 68 patients with CF). The majority of CF patients (79%) and controls (78%) completed at least two successful washouts...
September 18, 2017: Irish Medical Journal
https://www.readbyqxmd.com/read/29371133/hypertonic-saline-has-a-prolonged-effect-on-mucociliary-clearance-in-adults-with-cystic-fibrosis
#20
Aaron T Trimble, A Whitney Brown, Beth L Laube, Noah Lechtzin, Kirby L Zeman, Jihong Wu, Agathe Ceppe, David Waltz, William D Bennett, Scott H Donaldson
BACKGROUND: Inhaled hypertonic saline (HS) has been shown to increase mucociliary clearance (MCC) and improve clinical outcomes in adults and adolescents with cystic fibrosis (CF). However, in younger children with CF, a large study failed to demonstrate clinical benefits. This discrepancy could reflect pharmacodynamic differences in the MCC response to HS in different populations. We previously demonstrated the absence of a sustained effect of HS on MCC in healthy adults and in this study sought to characterize the durability of the MCC response to HS in adults with CF...
January 19, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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