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https://www.readbyqxmd.com/read/27911585/early-lung-disease-in-infants-and-pre-school-children-with-cystic-fibrosis-what-have-we-learnt-and-what-should-we-do-about-it
#1
Sarath C Ranganathan, Graham L Hall, Peter D Sly, Stephen M Stick, Tonia A Douglas
The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis. Pulmonary inflammation, infection and structural lung damage manifest very early in life and is prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer term clinical trajectory for individuals with CF...
December 2, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27905200/induced-sputum-to-detect-lung-pathogens-in-young-children-with-cystic-fibrosis
#2
Pamela D'Sylva, Daan Caudri, Nicole Shaw, Lidija Turkovic, Tonia Douglas, Jane Bew, Anthony D Keil, Stephen Stick, André Schultz
INTRODUCTION: Induced sputum sampling holds promise as a method for obtaining samples representative of the lower airways in young children. Collection of induced sputum samples in young children differs from older children and adults' as pharyngeal suctioning is often required. Our aim was to determine the sensitivity and specificity of induced sputum with and without airway clearance techniques to detect lower airway pathogens in children less than age 7 with cystic fibrosis. METHODS: Microbiological culture results were compared between 61 paired induced sputum and bronchoalveolar lavage fluid samples from young children with cystic fibrosis...
November 30, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27902490/detection-of-staphylococcus-aureus-in-cystic-fibrosis-patients-using-breath-voc-profiles
#3
A H Neerincx, B P Geurts, J van Loon, V Tiemes, J J Jansen, F J M Harren, L A J Kluijtmans, P J F M Merkus, S M Cristescu, L M C Buydens, R A Wevers
Staphylococcus aureus (S. aureus) is a common bacterium infecting children with cystic fibrosis (CF). Since current detection methods are difficult to perform in children, there is need for an alternative. This proof of concept study investigates whether breath profiles can discriminate between S. aureus infected and non-infected CF patients based on volatile organic compounds (VOCs). We collected exhaled breath of CF patients with and without S. aureus airways infections in which VOCs were identified using gas chromatography-mass spectrometry...
November 30, 2016: Journal of Breath Research
https://www.readbyqxmd.com/read/27897275/an-ex-vivo-model-contributing-to-the-diagnosis-and-evaluation-of-new-drugs-in-cystic-fibrosis
#4
A M Di Lullo, M Scorza, F Amato, M Comegna, V Raia, L Maiuri, G Ilardi, E Cantone, G Castaldo, M Iengo
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene. About 2000 mutations have been described so far. We setup an ex vivo model of human nasal epithelial cells (HNECs) to study CF patients testing the effect of novel mutations and molecular therapies. We performed the sampling (by brushing), followed by culture and analysis of HNECs using a series of molecular techniques. We performed 50 brushings from CF patients and controls...
November 29, 2016: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/27894912/hearing-thresholds-at-high-frequency-in-patients-with-cystic-fibrosis-a-systematic-review
#5
Debora T M Caumo, Lúcia B Geyer, Adriana R Teixeira, Sérgio S M Barreto
INTRODUCTION: High-frequency audiometry may contribute to the early detection of hearing loss caused by ototoxic medications. Many ototoxic drugs are widely used in the treatment of patients with cystic fibrosis. Early detection of hearing loss should allow known harmful drugs to be identified before the damage affects speech frequencies. The damage caused by ototoxicity is irreversible, resulting in important social and psychological consequences. In children, hearing loss, even when restricted to high frequencies, can affect the development of language...
November 9, 2016: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/27889352/oxidized-glutathione-and-uric-acid-as-biomarkers-of-early-cystic-fibrosis-lung-disease
#6
Nina Dickerhof, Rufus Turner, Irada Khalilova, Emmanuelle Fantino, Peter D Sly, Anthony J Kettle
BACKGROUND: In cystic fibrosis (CF) there is an urgent need for earlier diagnosis of pulmonary infections and inflammation using blood- and urine-based biomarkers. METHODS: Using mass spectrometry, oxidation products of glutathione and uric acid were measured in matched samples of bronchoalveolar lavage (BAL), serum and urine from 36 infants and children with CF, and related to markers of neutrophilic inflammation and infection in BAL. RESULTS: Oxidation products of glutathione (glutathione sulfonamide, GSA) and uric acid (allantoin), were elevated in BAL of children with pulmonary infections with Pseudomonas aeruginosa (PsA) compared to those without (p<0...
November 24, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27886635/multiplex-pcr-reveals-that-viruses-are-more-frequent-than-bacteria-in-children-with-cystic-fibrosis
#7
Sílvia Miró-Cañís, Sílvia Capilla-Rubio, Laura Marzo-Checa, Dionisia Fontanals-Aymerich, Isabel Sanfeliu-Sala, Mateu Espasa-Soley, Oscar Asensio-de-la-Cruz
BACKGROUND: Cystic fibrosis is a degenerative disease characterized by progressive epithelial secretory gland dysfunction associated with repeated respiratory infections. Bacterial infections are very frequent in children with cystic fibrosis, but because rapid METHODS: for screening for the wide variety of potentially involved viruses were unavailable until recently, the frequency of viral presence is unknown. Multiplex PCR enables screening for many viruses involved in respiratory infections...
November 13, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/27884144/assessment-of-serology-and-spirometry-and-the-combination-of-both-to-complement-microbiological-isolation-for-earlier-detection-of-pseudomonas-aeruginosa-infection-in-children-with-cystic-fibrosis
#8
Ana Kotnik Pirš, Uroš Krivec, Saša Simčič, Katja Seme
BACKGROUND: The aim of this study was to assess whether serology and spirometry and the combination of both can complement culture-based detection for earlier recognition of Pseudomonas aeruginosa infection in children with cystic fibrosis. METHODS: A 4 year longitudinal prospective study that included 67 Slovenian children with cystic fibrosis with a mean age of 10.5 years was conducted. Serology, spirometry and a scoring system combining serology and spirometry were assessed and compared...
November 25, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27878805/pancreatic-enzyme-replacement-therapy-for-people-with-cystic-fibrosis
#9
REVIEW
Usha Rani Somaraju, Arturo Solis-Moya
BACKGROUND: Most people with cystic fibrosis (80% to 90%) need pancreatic enzyme replacement therapy to prevent malnutrition. Enzyme preparations need to be taken whenever food is taken, and the dose needs to be adjusted according to the food consumed. A systematic review on the efficacy and safety of pancreatic enzyme replacement therapy is needed to guide clinical practice, as there is variability between centres with respect to assessment of pancreatic function, time of commencing treatment, dose and choice of supplements...
November 23, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#10
Belma Haliloglu, Yasemin Gokdemir, Zeynep Atay, Saygin Abali, Tulay Guran, Fazilet Karakoc, Refika Ersu, Bulent Karadag, Serap Turan, Abdullah Bereket
OBJECTIVE: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. RESEARCH DESIGN AND METHODS: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured...
November 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27869567/use-of-the-daily-phone-diary-to-study-religiosity-and-mood-convergent-validity
#11
Rhonda D Szczesniak, Yuanshu Zou, Sophia M Dimitriou, Alexandra L Quittner, Daniel H Grossoehme
Studies of religious/spiritual behavior frequently rely on self-reported questionnaire data, which is susceptible to bias. The Daily Phone Diary (DPD) was developed to minimize bias in reporting activities and behavior across a 24-hour period. A cross-sectional study of 126 parents of children with cystic fibrosis was used to establish the validity of the DPD to study religious/spiritual behaviors. Longitudinal models were used to determine the odds of improved mood during religious/spiritual activities. Convergent validity was found...
November 21, 2016: Journal of Health Care Chaplaincy
https://www.readbyqxmd.com/read/27863612/complementary-and-alternative-medicine-use-in-children-with-cystic-fibrosis
#12
Sandra Giangioppo, Odion Kalaci, Arun Radhakrishnan, Erin Fleischer, Jennifer Itterman, Brian Lyttle, April Price, Dhenuka Radhakrishnan
PURPOSE: To estimate the overall prevalence of complementary and alternative medicine use among children with cystic fibrosis, determine specific modalities used, predictors of use and subjective helpfulness or harm from individual modalities. RESULTS: Of 53 children attending the cystic fibrosis clinic in London, Ontario (100% recruitment), 79% had used complementary and alternative medicine. The most commonly used modalities were air purifiers, humidifiers, probiotics, and omega-3 fatty acids...
November 2016: Complementary Therapies in Clinical Practice
https://www.readbyqxmd.com/read/27862309/study-of-greek-children-and-youths-with-cystic-fibrosis-identifies-immunisation-gaps-and-delays
#13
Helena C Maltezou, Stavros Doudounakis, Maria Lekaditi, Kalliopi Tanou, Panos Katerelos, Maria Theodoridou
AIM: Data about immunisation rates in cystic fibrosis (CF) patients are scarce. We estimated the rates and timeliness of immunisations in CF patients aged 0.55-22 years. METHODS: We studied 122 subjects at a hospital in Greece in 2014. A standard questionnaire was used to collect data and parents' opinions about immunisations. RESULTS: The complete immunisation rates were 92.6% for diphtheria-tetanus-acellular pertussis-inactivated poliomyelitis-Haemophilus influenzae (DTaP-IPV-Hib), 96...
November 10, 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27859825/do-children-with-cystic-fibrosis-receiving-outreach-care-have-poorer-clinical-outcomes-than-those-treated-at-a-specialist-cystic-fibrosis-centre
#14
Heinrich C Weber, Philip F Robinson, Nicole Saxby, Sean A Beggs, Ingrid Els, Rodney I Ehrlich
INTRODUCTION: Although cystic fibrosis (CF) centre care is generally considered ideal, children living in regional Australia receive outreach care supported by the academic CF centres. METHODS: This is a retrospective database review of children with CF treated at the Royal Children's Hospital in Melbourne and its outreach clinics in Albury (Victoria), and Tasmania. The aim was to compare the outcomes of children with CF managed at an academic centre with that of outreach care, using lung function, nutritional status and Pseudomonas aeruginosa colonisation...
November 17, 2016: Australian Journal of Rural Health
https://www.readbyqxmd.com/read/27856730/multidrug-and-carbapenem-resistant-pseudomonas-aeruginosa-in-children-united-states-1999-2012
#15
Latania K Logan, Sumanth Gandra, Siddhartha Mandal, Eili Y Klein, Jordan Levinson, Robert A Weinstein, Ramanan Laxminarayan
BACKGROUND: Pseudomonas aeruginosa is a common cause of healthcare-associated infection. Multidrug-resistant (MDR) (>3 classes) and carbapenem-resistant (CR) P aeruginosa are significant threats globally. We used a large reference-laboratory database to study the epidemiology of P aeruginosa in children in the United States. METHODS: Antimicrobial susceptibility data from the Surveillance Network were used to phenotypically identify MDR and CR P aeruginosa isolates in children aged 1 to 17 years between January 1999 and July 2012...
November 16, 2016: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/27856213/ralstonia-species-do-these-bacteria-matter-in-cystic-fibrosis
#16
REVIEW
Anna-Rose Prior, Cedric Gunaratnam, Hilary Humphreys
Ralstonia species, often regarded as an environmental organism of low pathogenicity, can cause significant disease in certain at-risk patient groups, including those with cystic fibrosis. Difficulties with its identification in the clinical laboratory mean that it may be misidentified and therefore under recognised as a cause of disease. A number of outbreaks have been associated with the use of devices for inhaled respiratory therapy, putting those with chronic respiratory conditions at risk. Antimicrobial treatment of infection is challenging and limited due to frequent antimicrobial resistance...
October 21, 2016: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/27846621/unstimulated-saliva-related-caries-risk-factors-in-individuals-with-cystic-fibrosis-a-cross-sectional-analysis-of-unstimulated-salivary-flow-ph-and-buffering-capacity
#17
Alaa A Alkhateeb, Lloyd A Mancl, Richard B Presland, Marilynn L Rothen, Donald L Chi
Salivary flow rate, pH, and buffering capacity are associated with dental caries, but studies from the cystic fibrosis (CF) literature are inconclusive regarding these salivary factors and caries. The aim of this study was to evaluate these factors and their associations with dental caries in individuals with CF. Unstimulated whole saliva was collected from individuals aged 6-20 years at Seattle Children's Hospital CF Clinic, USA (n = 83). Salivary flow rate was measured in milliliters per minute. Salivary pH was assessed using a laboratory pH meter...
November 16, 2016: Caries Research
https://www.readbyqxmd.com/read/27836957/metabolomic-biomarkers-predictive-of-early-structural-lung-disease-in-cystic-fibrosis
#18
Charles R Esther, Lidija Turkovic, Tim Rosenow, Marianne S Muhlebach, Richard C Boucher, Sarath Ranganathan, Stephen M Stick
Neutrophilic airway inflammation plays a role in early structural lung disease in cystic fibrosis, but the mechanisms underlying this pathway are incompletely understood.Metabolites associated with neutrophilic inflammation were identified by discovery metabolomics on bronchoalveolar lavage fluid supernatant from 20 preschool children (2.9±1.3 years) with cystic fibrosis. Targeted mass-spectrometric detection of relevant metabolites was then applied to 34 children (3.5±1.5 years) enrolled in the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) who underwent chest computed tomography and bronchoalveolar lavage from two separate lobes during 42 visits...
December 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27805749/they-know-its-safe-they-know-what-to-expect-from-that-face-perceptions-toward-a-cognitive-behavioral-counseling-program-among-caregivers-of-children-with-cystic-fibrosis
#19
Fiona J Moola, Lauren A V Henry, Elizabeth Huynh, Jenna A Stacey, Guy E J Faulkner
BACKGROUND: Youth with cystic fibrosis (CF) experience significant behavioral and psychosocial challenges, such as depression, anxiety, and poor treatment adherence. Caregivers are critical to the provision of care and treatment to young people living with cystic fibrosis. Caregivers of youth with CF experience psychosocial morbidity. Thus, the development of counseling interventions are required to enhance psychosocial wellbeing among the caregivers of youth with cystic fibrosis. AIMS AND OBJECTIVES: In this qualitative study, we explored the experiences of eight caregivers who provide care to children with cystic fibrosis in an eight-week cognitive behavioral counseling program at a children's hospital in Winnipeg, Canada...
November 2, 2016: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/27803137/validity-of-the-stage-of-exercise-scale-in-children-with-rheumatologic-conditions
#20
Samantha L Stephens, Mark S Tremblay, Guy Faulkner, Joseph Beyene, Tri H Nguyen, Suneye Koohsari, Elizaveta Limenis, Brian M Feldman
OBJECTIVE: To determine the face, content, and construct validity of the Stages of Exercise Scale (SOES) in children with rheumatologic conditions [juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM)], and if the validity of the SOES differs by disease type by comparing it with a disease control with a chronic respiratory illness [cystic fibrosis (CF)]. METHODS: Sixty-seven children and adolescents (43 female) ages 11 to 18 years with a diagnosis of either JDM (n = 15), JIA (n = 39), or CF (n = 13) completed the SOES; scales of sensibility, process of change, decisional balance, and self-efficacy; the Child Health Assessment Questionnaire; and patient/physician ratings of disease severity...
December 2016: Journal of Rheumatology
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