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https://www.readbyqxmd.com/read/28822728/variability-of-lung-clearance-index-in-clinically-stable-cystic-fibrosis-lung-disease-in-school-age-children
#1
Marcus Svedberg, Per M Gustafsson, Paul D Robinson, Monica Rosberg, Anders Lindblad
BACKGROUND: Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis (CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI variability in clinically stable CF lung disease in school age children. METHODS: Paediatric patients, aged 6 to 17years, attending the outpatient CF clinic performed Multiple Breath Nitrogen Washout (Exhalyzer® D) and spirometry every third month over a period of one year. Clinical stability was assessed by the Cystic Fibrosis Clinical Score (CFCS) at each visit...
August 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28815937/the-airway-microbiota-in-early-cystic-fibrosis-lung-disease
#2
Katherine B Frayman, David S Armstrong, Keith Grimwood, Sarath C Ranganathan
Infection plays a critical role in the pathogenesis of cystic fibrosis (CF) lung disease. Over the past two decades, the application of molecular and extended culture-based techniques to microbial analysis has changed our understanding of the lungs in both health and disease. CF lung disease is a polymicrobial disorder, with obligate and facultative anaerobes recovered alongside traditional pathogens in varying proportions, with some differences observed to correlate with disease stage. While healthy lungs are not sterile, differences between the lower airway microbiota of individuals with CF and disease-controls are already apparent in childhood...
August 16, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28811770/clinical-and-laboratory-profile-of-children-with-cystic-fibrosis-experience-of-a-tertiary-care-center-in-pakistan
#3
Danish Abdul Aziz, Abdul Gaffar Billoo, Ahad Qureshi, Misha Khalid, Salman Kirmani
OBJECTIVE: To determine the clinical presentation, diagnostic investigations and laboratory workup done in admitted children with cystic fibrosis at Aga Khan University Hospital Karachi, Pakistan. METHODS: This is athree years retrospective study from January 2013 to December 2015 conducted at The Aga Khan University Hospital Karachi Pakistan, enrolling admitted patient from birth to 15 years of either gender, diagnosed with CF on the basis of clinical features and positive sweat chloride test...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28811149/three-center-feasibility-of-lung-clearance-index-in-infants-and-preschool-children-with-cystic-fibrosis-and-other-lung-diseases
#4
Mirjam Stahl, Simon Y Graeber, Cornelia Joachim, Sandra Barth, Isabell Ricklefs, Gesa Diekmann, Matthias V Kopp, Lutz Naehrlich, Marcus A Mall
BACKGROUND: Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting. METHODS: Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2...
August 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28804700/abridged-version-of-the-awmf-guideline-for-the-medical-clinical-diagnostics-of-indoor-mould-exposure-s2k-guideline-of-the-german-society-of-hygiene-environmental-medicine-and-preventive-medicine-ghup-in-collaboration-with-the-german-association-of-allergists
#5
Gerhard A Wiesmüller, Birger Heinzow, Ute Aurbach, Karl-Christian Bergmann, Albrecht Bufe, Walter Buzina, Oliver A Cornely, Steffen Engelhart, Guido Fischer, Thomas Gabrio, Werner Heinz, Caroline E W Herr, Jörg Kleine-Tebbe, Ludger Klimek, Martin Köberle, Herbert Lichtnecker, Thomas Lob-Corzilius, Rolf Merget, Norbert Mülleneisen, Dennis Nowak, Uta Rabe, Monika Raulf, Hans Peter Seidl, Jens-Oliver Steiß, Regine Szewszyk, Peter Thomas, Kerttu Valtanen, Julia Hurraß
This article is an abridged version of the AWMF mould guideline "Medical clinical diagnostics of indoor mould exposure" presented in April 2016 by the German Society of Hygiene, Environmental Medicine and Preventive Medicine (Gesellschaft für Hygiene, Umweltmedizin und Präventivmedizin, GHUP), in collaboration with the above-mentioned scientific medical societies, German and Austrian societies, medical associations and experts. Indoor mould growth is a potential health risk, even if a quantitative and/or causal relationship between the occurrence of individual mould species and health problems has yet to be established...
2017: Allergo Journal International
https://www.readbyqxmd.com/read/28802361/functional-endoscopic-sinus-surgery-improves-the-quality-of-life-in-children-suffering-from-chronic-rhinosinusitis-with-nasal-polyps
#6
Melina Fetta, Nikolaos S Tsilis, John V Segas, Thomas P Nikolopoulos, Petros V Vlastarakos
OBJECTIVE: To evaluate the impact of FESS in children with chronic rhinosinusitis with nasal polyps, regarding their overall postoperative quality-of-life (QoL) and constituent QoL domains. Potential differences between cystic fibrosis (CF) sufferers and non-sufferers, or cases with recurrent sinonasal polyposis versus single-operations were also explored. METHODS: 39 children were studied. The mean patient age was 10.9 years; four children suffered from cystic fibrosis...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28800360/prerequisites-for-a-dry-powder-inhaler-for-children-with-cystic-fibrosis
#7
Anne J Lexmond, Paul Hagedoorn, Henderik W Frijlink, Bart L Rottier, Anne H de Boer
Correct inhalation technique is essential for effective use of dry powder inhalers (DPIs), as their effectiveness largely depends on the patient's inhalation manoeuvre. Children are an especially challenging target population for DPI development due to the large variability in understanding and inspiratory capacities. We previously performed a study in which we determined the prerequisites for a paediatric DPI in a mostly healthy paediatric population, for which we used an empty test inhaler with variable internal airflow resistance and mouthpiece...
2017: PloS One
https://www.readbyqxmd.com/read/28800247/reply-from-the-authors-of-comparison-of-lung-clearance-index-and-magnetic-resonance-imaging-for-assessment-of-lung-disease-in-children-with-cystic-fibrosis
#8
Mirjam Stahl, Mark O Wielpütz, Hans-Ulrich Kauczor, Marcus A Mall
No abstract text is available yet for this article.
August 11, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28795206/children-and-adolescents-with-cystic-fibrosis-display-moderate-bone-microarchitecture-abnormalities-data-from-high-resolution-peripheral-quantitative-computed-tomography
#9
C Braun, J Bacchetta, P Braillon, R Chapurlat, J Drai, P Reix
We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only. INTRODUCTION: Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs)...
August 9, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28774563/adverse-events-following-live-attenuated-intranasal-influenza-vaccination-of-children-with-cystic-fibrosis-results-from-two-influenza-seasons
#10
Constantina Boikos, Lawrence Joseph, David Scheifele, Larry C Lands, Gaston De Serres, Jesse Papenburg, Nicholas Winters, Mark Chilvers, Caroline Quach
BACKGROUND: Despite the approved use of live-attenuated intranasal influenza vaccine (LAIV) for seasonal immunization of patients with cystic fibrosis (CF), many questions remain unanswered regarding the timing, duration, and types of adverse events that occur following administration of this vaccine. METHODS: In 2012 and 2013, 264 LAIV doses were administered to 198 patients aged 2-19 with CF. Vaccinees were followed prospectively for 55 days after vaccination (day 0) and information on adverse events was collected...
July 31, 2017: Vaccine
https://www.readbyqxmd.com/read/28766082/quality-of-life-among-german-parents-of-children-with-cystic-fibrosis-the-effects-of-being-a-single-caregiver
#11
Astrid Wallenwein, Mona Schwarz, Lutz Goldbeck
PURPOSE: Quality of life (QoL) has not been studied up to now in single parents of children with cystic fibrosis (CF). We hypothesized lower QoL compared to parents living together with a partner. We explored whether single parents benefit in a comparable manner from a family-oriented inpatient rehabilitation (FOR) program provided in Germany. METHODS: 260 parents of a child with CF (0-17 years), 40 of them single parents, were included. Their QoL was compared to partnered parents using Student's t test for independent samples...
August 1, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28765819/a-review-of-42-asthmatic-children-with-allergic-bronchopulmonary-aspergillosis
#12
Ashok Shah, Shekhar Kunal
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) in children with asthma, not associated with cystic fibrosis, is yet to receive the recognition it deserves. OBJECTIVE: To highlight the presentation of ABPA in children with asthma. METHODS: This retrospective review documents the occurrence of pediatric ABPA over a period of 31 years in one unit. Children with asthma, eosinophilia and infiltrates on chest radiograph were screened for ABPA...
July 2017: Asia Pacific Allergy
https://www.readbyqxmd.com/read/28754328/age-related-levels-of-fecal-m2-pyruvate-kinase-in-children-with-cystic-fibrosis-and-healthy-children-0-to-10years-old
#13
Millie Garg, Steven T Leach, Tamara Pang, Bronwen Needham, Michael J Coffey, Tamarah Katz, Roxanne Strachan, John Widger, Penelope Field, Yvonne Belessis, Sandra Chuang, Andrew S Day, Adam Jaffe, Chee Y Ooi
BACKGROUND: The pathogenesis of gut inflammation, bacterial dysbiosis and increased rates of malignancy in CF is unclear. Fecal M2-pyruvate kinase (M2-PK) is a biomarker indicative of cellular proliferation that may be raised in intestinal malignancy and inflammation. Biomarkers, including M2-PK, may be useful in assessing effects of novel therapies on the gastrointestinal tract. METHODS: M2-PK was measured in stools collected from patients with CF and HC (0-10years)...
July 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28751295/pseudo-bartter-syndrome-as-the-sole-manifestation-of-cystic-fibrosis-in-a-child-with-711-g-t-ivs8-5t-mutation-a-new-face-of-an-old-disease
#14
Faten Tinsa, Sondes Hadj Fredj, Imen Bel Hadj, Fatma Khalsi, Sonia Abdelhak, Khadija Boussetta, Taieb Messaoud
Pseudo-Bartter syndrome (PBS) describes an uncommon complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. PBS as the sole manifestation of cystic fibrosis in children is extremely rare and has never been described in patients carrying 5T variant. We report a clinical, biochemical and genetic study of a four year-old boy presenting a pseudo-Bartter syndrome as the sole manifestation of cystic fibrosis. All 27 exons and the flanking intron regions of the CFTR gene were analysed by PCR and direct sequencing...
August 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/28750627/air-pollution-exposure-is-associated-with-mrsa-acquisition-in-young-u-s-children-with-cystic-fibrosis
#15
Kevin J Psoter, Anneclaire J De Roos, Jon Wakefield, Jonathan D Mayer, Margaret Rosenfeld
BACKGROUND: The role of air pollution in increasing susceptibility to respiratory tract infections in the cystic fibrosis (CF) population has not been well described. We recently demonstrated that chronic PM2.5 exposure is associated with an increased risk of initial Pseudomonas aeruginosa acquisition in young children with CF. The purpose of this study was to determine whether PM2.5 exposure is a risk factor for acquisition of other respiratory pathogens in young children with CF. METHODS: We conducted a retrospective study of initial acquisition of methicillin susceptible and methicillin resistant Staphylococcus aureus (MSSA and MRSA), Stenotrophomonas maltophilia and Achromobacter xylosoxidans in U...
July 27, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28745925/clinical-care-of-children-with-primary-ciliary-dyskinesia
#16
Jane S Lucas, Mikkel Christian Alanin, Samuel Collins, Amanda Harris, Helle Krogh Johansen, Kim G Nielsen, Jean Francois Papon, Phil Robinson, Woolf T Walker
Primary ciliary dyskinesia (PCD) is a rare heterogeneous disorder, usually inherited as an autosomal recessive condition but X-linked inheritance is also described. Abnormal ciliary function in childhood leads to neonatal respiratory distress in term infants, persistent wet cough, bronchiectasis, chronic rhinosinusitis, and hearing impairment; approximately 50% of patients have situs inversus. There is a paucity of evidence for treating PCD, hence consensus guidelines are predominantly influenced by knowledge from cystic fibrosis (CF)...
July 26, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28737140/bone-mineral-density-of-indian-children-and-adolescents-with-cystic-fibrosis
#17
Sumita Gupta, Aparna Mukherjee, Rajesh Khadgawat, Madhulika Kabra, Rakesh Lodha, Sushil K Kabra
OBJECTIVE: To document bone mineral density of children and adolescents with cystic fibrosis. DESIGN: Cross-sectional study. SETTING: Tertiary-care center of Northern India, July 2012 to August 2015. PARTICIPANTS: 52 children aged 6-18 years with cystic fibrosis and 62 healthy controls of similar age and sex. METHODS: Both patients and controls were stratified into two groups, as pre-pubertal and peri-/post-pubertal, and compared for whole body bone mineral density, measured using dual energy X-ray absorptiometry...
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28728770/don-t-push-your-luck-educational-family-board-not-bored-game-for-school-age-children-living-with-chronic-conditions
#18
Andrea Kennedy, Lisa Semple, Kerri Alderson, Vanessa Bouskill, Janice Karasevich, Brenda Riske, Sheri van Gunst
PURPOSE: Children who are living with chronic conditions may be supported in self-care through enjoyable active learning and family social processes. This research focused on development and evaluation of "Don't Push Your Luck!", an educational board game designed to inspire family discussion about chronic conditions, and help affected children learn about self-care choices and consequences. DESIGN AND METHODS: Mixed-method research was conducted with families from one outpatient Cystic Fibrosis Clinic and four Hemophilia Treatment Centres in Canada and United States (N=72)...
July 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28727186/telephone-monitoring-and-home-visits-significantly-improved-the-quality-of-life-treatment-adherence-and-lung-function-in-children-with-cystic-fibrosis
#19
Elisavet-Anna Chrysochoou, Elpis Hatziagorou, Fotis Kirvassilis, John Tsanakas
Cystic Fibrosis (CF) is a chronic and systemic disease with a progressive course. As survival rates continue to improve, there is a growing demand for new therapeutic options that improve treatment adherence, disease management and quality of life (QoL) (1). The aim of this study was to evaluate the safety and effectiveness of a home care programme for children with CF and to assess the value of regular telephone contact with the CF team based at Hippokration Hospital, Thessaloniki, Greece. This article is protected by copyright...
July 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28726737/respiratory-tract-infections-and-the-role-of-biologically-active-polysaccharides-in-their-management-and-prevention
#20
REVIEW
Milos Jesenak, Ingrid Urbancikova, Peter Banovcin
Respiratory tract infections (RTIs) are the most common form of infections in every age category. Recurrent respiratory tract infections (RRTIs), a specific form of RTIs, represent a typical and common problem associated with early childhood, causing high indirect and direct costs on the healthcare system. They are usually the consequence of immature immunity in children and high exposure to various respiratory pathogens. Their rational management should aim at excluding other severe chronic diseases associated with increased morbidity (e...
July 20, 2017: Nutrients
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