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cystic fibrosis children

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https://www.readbyqxmd.com/read/28324670/relationship-of-antibiotic-treatment-to-recovery-after-acute-fev1-decline-in-children-with-cystic-fibrosis
#1
Wayne J Morgan, Jeffrey S Wagener, David J Pasta, Stefanie J Millar, Donald R VanDevanter, Michael W Konstan
RATIONALE: Children with cystic fibrosis often suffer acute declines in lung function. We previously showed that such declines are not always treated with antibiotics, but did not assess whether treatment improves the likelihood of recovery. OBJECTIVES: To determine whether new antibiotic treatment was associated with recovery from acute FEV1 decline. METHODS: We studied episodes of FEV1 decline (≥10% from baseline) from the Epidemiologic Study of Cystic Fibrosis...
March 21, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28323913/accrual-of-bone-mass-in-children-and-adolescents-with-cystic-fibrosis
#2
Sonakshi Sharma, Mirjana Jaksic, Sheryl Fenwick, Catherine Byrnes, Tim Cundy
Context: Low bone density is a recognised complication of cystic fibrosis (CF). Hypothesis: Accrual of bone mass is most impaired in the sickest children, as judged by nutritional status and pulmonary function. Design: Retrospective analysis of correlation between lumbar spine BMD, BMI and FEV1 z-score in children and adolescents with CF. Setting: Specialist CF service at a pediatric hospital. Patients: 60 participants aged 5...
February 22, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28319489/autogenic-drainage-in-children-with-cystic-fibrosis
#3
Lieselotte Corten, Brenda M Morrow
PURPOSE: Airway clearance is an essential part of the management of cystic fibrosis (CF) as it facilitates clearance of viscous pulmonary secretions. This review aimed to determine the effect of autogenic drainage (AD) and assisted autogenic drainage (AAD) compared with no, sham, or other types of airway clearance in children with CF. SUMMARY OF KEY POINTS: Two pediatric randomized cross-over trials were identified on the use of AD in children with CF; no studies were available on the use of AAD...
March 17, 2017: Pediatric Physical Therapy
https://www.readbyqxmd.com/read/28315491/vibrating-mesh-nebulizer-maintenance-by-cf-patients-results-from-a-french-survey
#4
Melisande Baravalle-Einaudi, Nadine Dufeu, Christian Dupont, Laurent Vecellio, Bertrand Delaisi, Ania Carsin, Jean-Christophe Dubus
PURPOSE OF THE STUDY: Vibrating-mesh nebulizers are widely used at home for cystic fibrosis (CF) treatment, with a therapeutic efficiency closely linked to the mesh performance. This national study looks at the maintenance at home by CF patients and their families of the mesh of the eFlow(®)rapid nebulizer. Ninety-two patients from 34 French CF centers, treated at home with inhaled drugs delivered with a vibrating-mesh nebulizer, answered to a phone standardized questionnaire specifying the different techniques of maintenance of the nebulizer...
March 14, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28302638/innovative-approach-for-self-management-and-social-welfare-of-children-with-cystic-fibrosis-in-europe-development-validation-and-implementation-of-an-mhealth-tool-mycyfapp
#5
Joaquim Calvo-Lerma, Celia P Martinez-Jimenez, Juan-Pablo Lázaro-Ramos, Ana Andrés, Paula Crespo-Escobar, Erlend Stav, Cornelia Schauber, Lucia Pannese, Jessie M Hulst, Lucrecia Suárez, Carla Colombo, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
INTRODUCTION: For the optimal management of children with cystic fibrosis, there are currently no efficient tools for the precise adjustment of pancreatic enzyme replacement therapy, either for advice on appropriate dietary intake or for achieving an optimal nutrition status. Therefore, we aim to develop a mobile application that ensures a successful nutritional therapy in children with cystic fibrosis. METHODS AND ANALYSIS: A multidisciplinary team of 12 partners coordinate their efforts in 9 work packages that cover the entire so-called 'from laboratory to market' approach by means of an original and innovative co-design process...
March 16, 2017: BMJ Open
https://www.readbyqxmd.com/read/28283399/dietary-intake-and-lipid-profile-in-children-and-adolescents-with-cystic-fibrosis
#6
Janna W Woestenenk, Dorothea A Schulkes, Henk S Schipper, Cornelis K van der Ent, Roderick H J Houwen
BACKGROUND: Cystic fibrosis (CF) patients are advised to derive 35% of their daily energy intake from dietary fat. Whether this high fat intake is associated with dyslipidaemia is unknown. We described the lipid profile and dietary intake in paediatric patients with CF. METHODS: 110 fasting lipid concentrations of 110 Dutch patients with CF were studied, along with 86 measurements of dietary intake. For the total group and for boys and girls separately, the lipid profile and the dietary intake were investigated...
March 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28281010/early-detection-of-lung-function-decrements-in-children-and-adolescents-with-cystic-fibrosis-using-new-reference-values
#7
Angela Zacharasiewicz, Sabine Renner, Flora Haderer, Michael Weber, Eleonore Dehlink, Zsolt Szepfalusi, Thomas Frischer
Interpretation of lung function values in children with cystic fibrosis (CF) depends on the applied reference values. We hypothesize that differences between the new global lung function initiative (GLI) values and the formerly used Zapletal et al. values produce significantly different clinical results. We analyzed 3719 lung function measurements of 108 children and adolescents (n = 54 male; aged 6-18 years) with CF treated between September 1991 and July 2009. Data were analyzed in milliliters (ml) and % predicted (pred...
March 9, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28279431/psychosocial-response-to-uncertain-newborn-screening-results-for-cystic-fibrosis
#8
Robin Z Hayeems, Fiona A Miller, Carolyn J Barg, Yvonne Bombard, June C Carroll, Karen Tam, Elizabeth Kerr, Pranesh Chakraborty, Beth K Potter, Sarah Patton, Jessica P Bytautas, Louise Taylor, Christine Davies, Jennifer Milburn, April Price, Tanja Gonska, Katherine Keenan, Felix Ratjen, Astrid Guttmann
OBJECTIVE: To explore the psychosocial implications of diagnostic uncertainty that result from inconclusive results generated by newborn bloodspot screening (NBS) for cystic fibrosis (CF). STUDY DESIGN: Using a mixed methods prospective cohort study of children who received NBS for CF, we compared psychosocial outcomes of parents whose children who received persistently inconclusive results with those whose children received true positive or screen-negative results...
March 6, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28277226/nasal-polyposis-in-cystic-fibrosis-follow-up-of-children-and-adolescents-for-a-3-year-period
#9
Silke Anna Theresa Weber, Renata Mizusaki Iyomasa, Camila de Castro Corrêa, Wellington Novais Mafra Florentino, Giesela Fleischer Ferrari
INTRODUCTION: Nasal polyposis is often found in patients with cystic fibrosis. OBJECTIVE: To assess the incidence of nasal polyposis, the response to medical treatment, recurrence and the need for surgical intervention in children and adolescents with cystic fibrosis during a three-year follow-up. METHODS: Clinical symptoms (pulmonary, pancreatic insufficiency, malnutrition, nasal obstruction), two positive sweat chloride tests, and genotype findings in 23 patients with cystic fibrosis were analyzed...
October 17, 2016: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/28276750/red-blood-cell-distribution-width-is-not-a-reliable-biomarker-for-low-iron-stores-in-children-with-cystic-fibrosis
#10
M D Akkermans, L Uijterschout, M Nuijsink, D M Hendriks, J B van Goudoever, F Brus
Low iron stores in children, absolute iron deficiency (AID), can lead to impaired neurodevelopment and requires iron therapy. In the presence of infection/inflammation, like in cystic fibrosis (CF), serum ferritin (SF) is not a reliable biomarker for AID. Red blood cell distribution width (RDW) is a promising alternative reported not to be influenced by infection in healthy children. Currently, there are no data on the diagnostic capacity of RDW to detect AID in pediatric CF patients. This was a prospective observational study that investigated iron status biomarkers in 53 Dutch pediatric CF patients...
February 10, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28267483/when-is-too-little-care-too-much-harm-in-cystic-fibrosis-psychological-and-ethical-approaches-to-the-problem
#11
John Massie, Alice Morgan, Lynn Gillam
Some parents of children with cystic fibrosis (CF) do not adhere to treatments recommended by the CF team. This can be a challenging issue for CF clinicians and can create conflict between the parents and treating team. Both parents and treating team believe they are acting in the best interests of the child, but do not share a common opinion as to what that entails. In this paper we present an understanding of the psychological framework of parents' illness representation that may foster a better understanding by CF clinicians of how to approach parents who hold a conflicting opinion regarding optimal care...
March 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28267276/hyponatremia-in-children-with-acute-respiratory-infections-a-reappraisal
#12
REVIEW
Camilla Lavagno, Gregorio P Milani, Peter Uestuener, Giacomo D Simonetti, Carmen Casaulta, Mario G Bianchetti, Pietro B Fare, Sebastiano A G Lava
Hyponatremia (<135 mmol/L), typically associated with an elevated anti-diuretic hormone level, is common among children admitted with bronchiolitis, pneumonia, or pulmonary exacerbation of cystic fibrosis. The main consequences of acute hyponatremia include cerebral edema and Ayus-Arieff pulmonary edema. A widespread belief is that, in children with pneumonia or bronchiolitis, hyponatremia results from inappropriate anti-diuresis. By contrast, the pathogenic role of extracellular fluid volume depletion or decreased effective circulating blood volume is underscored...
March 7, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28262916/vitamin-e-supplementation-in-people-with-cystic-fibrosis
#13
REVIEW
Peter O Okebukola, Sonal Kansra, Joanne Barrett
BACKGROUND: People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency including vitamin E. Vitamin E deficiency can cause a host of conditions such as haemolytic anaemia, cerebellar ataxia and cognitive difficulties. Vitamin E supplementation is widely recommended in cystic fibrosis and aims to ameliorate this deficiency. This is an updated version of the review. OBJECTIVES: To determine the effects of any level of vitamin E supplementation on the frequency of vitamin E deficiency disorders in people with cystic fibrosis...
March 6, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28262569/pilot-trial-of-tobramycin-inhalation-powder-in-cystic-fibrosis-patients-with-chronic-burkholderia-cepacia-complex-infection
#14
Valerie Waters, Yvonne Yau, Trevor Beaudoin, Jillian Wettlaufer, Sean Kevin Tom, Nancy McDonald, Leena Rizvi, Michelle Klingel, Felix Ratjen, Elizabeth Tullis
There is no effective chronic suppressive therapy Burkholderia cepacia complex infection in cystic fibrosis (CF) patients. This was a pilot, open-label clinical trial of tobramycin inhalation powder (TIP) delivered via Podhaler twice daily for 28days in adults and children with CF and chronic B. cepacia complex infection in Toronto, Canada. A total of 10 subjects (4 pediatric, 6 adult patients) were treated. There was a mean drop of 1.4 log (CFU/ml) in sputum bacterial density (p=0.01) and sputum IL-8 levels decreased significantly after 28days of TIP (p=0...
March 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28258928/inhaled-dry-powder-mannitol-in-children-with-cystic-fibrosis-a-randomised-efficacy-and-safety-trial
#15
K De Boeck, E Haarman, J Hull, L C Lands, A Moeller, A Munck, J Riethmüller, H Tiddens, S Volpi, J Leadbetter, B Charlton, A Malfroot
INTRODUCTION: Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF). METHODS: The efficacy of inhaled mannitol in children with CF aged 6-17years was assessed in a phase 2, randomised, placebo-controlled crossover study. Subjects were randomly assigned to mannitol 400mg every 12h or matching placebo for 8weeks, followed by an 8week washout and an 8week period with the alternate treatment...
February 28, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28258742/current-and-future-approaches-to-large-airways-imaging-in-adults-and-children
#16
REVIEW
T Semple, A Calder, C M Owens, S Padley
"Large airways disease" is a catch-all phrase encompassing a wide variety of pathology affecting the trachea, main, lobar, segmental, and proximal sub-segmental bronchi. Relevant pathologies can be divided into focal or diffuse processes and many conditions have classic appearances on computed tomography (CT). We provide a review of the imaging specifics of a wide range of large airway pathologies in adult, childhood, and fetal life with examples of common and rare pathologies ranging from well-known entities such as cystic fibrosis and allergic bronchopulmonary aspergillosis to rarities such as Williams-Campbell, primary ciliary dyskinesia, and congenital high-airway obstruction syndrome (CHAOS)...
March 1, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28248714/nebulization-of-antiinfective-agents-in-invasively-mechanically-ventilated-adults-a-systematic-review-and-meta-analysis
#17
Candela Solé-Lleonart, Jean-Jacques Rouby, Stijn Blot, Garyfallia Poulakou, Jean Chastre, Lucy B Palmer, Matteo Bassetti, Charles-Edouard Luyt, Jose M Pereira, Jordi Riera, Tim Felton, Jayesh Dhanani, Tobias Welte, Jose M Garcia-Alamino, Jason A Roberts, Jordi Rello
BACKGROUND: Nebulization of antiinfective agents is a common but unstandardized practice in critically ill patients. METHODS: A systematic review of 1,435 studies was performed in adults receiving invasive mechanical ventilation. Two different administration strategies (adjunctive and substitute) were considered clinically relevant. Inclusion was restricted to studies using jet, ultrasonic, and vibrating-mesh nebulizers. Studies involving children, colonized-but-not-infected adults, and cystic fibrosis patients were excluded...
March 1, 2017: Anesthesiology
https://www.readbyqxmd.com/read/28245190/cystic-fibrosis-related-diabetes-cfrd-is-preceded-by-and-associated-with-growth-failure-and-deteriorating-lung-function
#18
Nicolas Terliesner, Mandy Vogel, Anna Steighardt, Ruth Gausche, Constance Henn, Julia Hentschel, Thomas Kapellen, Sabine Klamt, Julia Gebhardt, Wieland Kiess, Freerk Prenzel
BACKGROUND: Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents. METHODS: A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed...
February 28, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28236043/cystic-fibrosis-in-austria
#19
Thomas Frischer, Ernst Eber, Helmut Ellemunter, Angela Zacharasiewicz, Ingrid Kaluza, Josef Riedler, Sabine Renner
Registry data for patients with cystic fibrosis (CF) are increasingly used to evaluate the natural history, for benchmarking of therapy and in order to identify eligible patients for clinical studies. So far, no data on frequency and clinical status of CF patients have been available for Austria on a national level. We collected data of CF patients treated 2014 in Austrian CF outpatient clinics by means of a European CF registry and on an individual search basis. A total of 773 CF patients with a median age of 18...
February 24, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28230293/influence-of-complex-childhood-diseases-on-variation-in-growth-and-skeletal-development
#20
REVIEW
Babette S Zemel
The study of human growth and skeletal development by human biologists is framed by the larger theoretical concerns regarding the underpinnings of population variation and human evolution. This unique perspective is directly relevant to the assessment of child health and well-being at the individual and group level, as well as the construction of growth charts. Environmental, behavioral (nutrition and physical activity), and disease-related factors can prevent attainment of full genetic potential for growth...
February 23, 2017: American Journal of Human Biology: the Official Journal of the Human Biology Council
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