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https://www.readbyqxmd.com/read/28222031/a-retrospective-analysis-of-longitudinal-changes-in-bone-mineral-content-in-cystic-fibrosis
#1
Adela Chirita-Emandi, Sheila Shepherd, Andreas Kyriakou, Jane D McNeilly, Carol Dryden, Donna Corrigan, Anne Devenny, Syed Faisal Ahmed
BACKGROUND: We aimed to describe the longitudinal changes in bone mineral content and influencing factors, in children with cystic fibrosis (CF). METHODS: One hundred children (50 females) had dual X-ray absorptiometry (DXA) performed. Of these, 48 and 24 children had two to three scans, respectively over 10 years of follow-up. DXA data were expressed as lumbar spine bone mineral content standard deviation score (LSBMCSDS) adjusted for age, gender, ethnicity and bone area...
February 21, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28220034/exhaled-and-nasal-nitric-oxide-in-relation-to-lung-function-blood-cell-counts-and-disease-characteristics-in-cystic-fibrosis
#2
Christina Krantz, Christer Janson, Annika Hollsing, Kjell Alving, Andrei Malinovschi
BACKGROUND: Patients with CF have similar or lower exhaled nitric oxide (FeNO) and lower nasal nitric oxide (nNO) levels than controls. There are divergent results on alveolar NO (CalvNO) concentrations in relation to CF. There are inconsistent results on correlation between different nitric oxide parameters and lung function and inflammation in CF. AIM: To compare FeNO, CalvNO and nNO levels between subjects with CF, asthma and healthy controls and to study whether these parameters are related to lung function, blood cell counts or clinical characteristics in CF patients...
February 21, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28219060/steady-state-therapy-with-azithromycin-or-low-dose-prednisolone-in-paediatric-cystic-fibrosis-patients-inflammatory-markers-and-disease-progression
#3
Galina Shmarina, Alexander Pukhalsky, Lucine Avakian, Sergey Semykin, Daria Pukhalskaya, Vladimir Alioshkin
BACKGROUND: Anti-inflammatory therapy is a logical approach to slowing the inevitable lung function deterioration in cystic fibrosis (CF) patients. This study's aim was to evaluate inflammatory markers and disease progression in paediatric CF patients chronically treated with azithromycin or low-dose prednisolone. METHODS: The study included 204 patients with CF and 100 healthy controls; 102 CF patients were treated with basic therapy only (without anti-inflammatory treatment; WAT), and 102 individuals received basic therapy along with azithromycin (n = 59) or low-dose prednisolone (n = 43)...
February 21, 2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28213960/association-of-rhinovirus-with-exacerbations-in-young-children-affected-by-cystic-fibrosis-preliminary-data
#4
Sacha Stelzer-Braid, Nancy Liu, Michael Doumit, Russell D'Cunha, Yvonne Belessis, Adam Jaffe, William D Rawlinson
Rhinovirus (RV) is a common respiratory viral infection linked to worsening of chronic respiratory diseases including cystic fibrosis (CF) and asthma. RV was tested by RT-PCR in samples (n = 465) collected from the upper (nasal swab, oropharyngeal suction and sputum) and lower (bronchoalveolar washings) respiratory tract of 110 children with CF. Air samples (n = 52) collected from the operating theatres and outpatient clinics were tested for RV. RV was found in 43% of children <5 years suffering an exacerbation, and 12% of older children (5-17 years)...
February 18, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28202251/high-incidence-of-non-tuberculous-mycobacteria-positive-cultures-among-adolescent-with-cystic-fibrosis
#5
Zoé Cavalli, Quitterie Reynaud, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
BACKGROUND: We evaluated the prevalence of non-tuberculous mycobacteria (NTM)-positive cultures among our cystic fibrosis (CF) center patients, reviewed risk factors for NTM positivity, and determined its impact on lung function evolution. METHODS: From 2009 to 2014, CF adults and children attending the CF center of Lyon (France) and having at least one positive NTM isolate were included. Each case was matched by age and gender with two CF patients with no NTM isolate (controls)...
February 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28188011/effect-of-posture-on-lung-ventilation-distribution-and-associations-with-structure-in-children-with-cystic-fibrosis
#6
Kathryn A Ramsey, Caroline McGirr, Stephen M Stick, Graham L Hall, Shannon J Simpson
BACKGROUND: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease. METHODS: Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease...
February 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28186714/lactic-acidosis-with-chloramphenicol-treatment-in-a-child-with-cystic-fibrosis
#7
Isabelle Goyer, Massimiliano Iseppon, Céline Thibault, Rachid Abaji, Maja Krajinovic, Julie Autmizguine
Children with cystic fibrosis are commonly colonized with multi-resistant bacteria. In such patients, infectious exacerbation may require salvage therapy with uncommonly used antimicrobials, including chloramphenicol. Chloramphenicol is rarely used nowadays because of the associated severe adverse events. We describe the case of a 15-year-old female with terminal cystic fibrosis who required intravenous (IV) chloramphenicol treatment for a Burkholderia cepacia (B. cepacia) exacerbation. The child subsequently developed lactic acidosis and secondary respiratory compensation adding to her baseline respiratory distress...
January 30, 2017: Journal of Population Therapeutics and Clinical Pharmacology
https://www.readbyqxmd.com/read/28186386/investigating-self-efficacy-disease-knowledge-and-adherence-to-treatment-in-adolescents-with-cystic-fibrosis
#8
Nicholas R Faint, Janelle M Staton, Stephen M Stick, Juliet M Foster, André Schultz
AIM: Patient adherence is integral to the effectiveness of prescribed treatment, and is associated with beneficial disease outcomes, yet in adolescents with cystic fibrosis, adherence is often sub-optimal. Multiple factors may contribute to treatment adherence, including disease knowledge and self-efficacy. In adolescents with cystic fibrosis: (i) to compare the disease knowledge of adolescents and their parents before transition to adult care; (ii) to determine the relationship between disease knowledge (adolescent, parent) and adherence; and (iii) to evaluate self-efficacy and its association with disease knowledge and adherence...
February 10, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28185886/macronutrient-intake-in-preschoolers-with-cystic-fibrosis-and-the-relationship-between-macronutrients-and-growth
#9
Stephanie S Filigno, Shannon M Robson, Rhonda D Szczesniak, Leigh A Chamberlin, Meredith A Baker, Stephanie M Sullivan, John Kroner, Scott W Powers
BACKGROUND: Adequate nutrition is essential for growth in children with cystic fibrosis (CF). The new CF Foundation Clinical Practice Guidelines bring attention to monitoring macronutrient intake as well as total energy. METHODS: Dietary intake of 75 preschool children with CF and pancreatic insufficiency was examined and compared to the Clinical Practice Guidelines. Regression analyses examined relationships between macronutrient intake and growth. RESULTS: Approximately 45% of children met the 110% minimum recommended dietary allowance (RDA) recommendation...
February 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28164596/genetic-expression-in-cystic-fibrosis-related-bone-disease-an-observational-transversal-cross-sectional-study
#10
Ioana M Ciuca, Liviu L Pop, Alexandru F Rogobete, Dan I Onet, Bogdan Guta-Almajan, Zoran Popa, Florin G Horhat
BACKGROUND: Cystic fibrosis (CF) is the most frequent monogenic genetic disease with autosomal recessive transmission and characterized by important clinical polymorphism and significant lethal prospective. CF related bone disease occurs frequently in adults with CF. Childhood is the period of bone formation, and therefore, children are more susceptible to low bone density. Several factors like pancreatic insufficiency, hormone imbalance, and physical inactivity contribute to CF bone disease development...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28161117/new-drugs-for-rare-diseases-in-children
#11
Klaus Rose
PURPOSE: United States (US) Pediatric Legislation (PL) was introduced in 1997 to improve children's health. The European Union PL (EUPL) has been in force since 2007. Both PLs facilitate additional pediatric research on primarily adult drugs. The EUPL declares that the forces of the market are not sufficient for children. Without a pediatric investigation plan, new drugs can no longer be registered with the European Union. New ways on how to facilitate drug development for rare pediatric diseases are being proposed...
February 1, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28155088/the-enigmatic-gut-in-cystic-fibrosis-linking-inflammation-dysbiosis-and-the-increased-risk-of-malignancy
#12
REVIEW
Millie Garg, Chee Y Ooi
PURPOSE OF REVIEW: Intestinal inflammation, dysbiosis, and increased gastrointestinal malignancy risks are well-described in patients with cystic fibrosis (CF). However, there is limited understanding of their pathophysiology. This review aims to discuss these issues and assess potential links between them. RECENT FINDINGS: Evidence of links between intestinal inflammation and dysbiosis (an imbalance in intestinal microbial populations) exist. Recent studies have demonstrated reduction in intestinal inflammation with probiotic administration...
February 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28143919/growth-in-prepubertal-children-with-cystic-fibrosis-treated-with-ivacaftor
#13
Michael S Stalvey, Jesse Pace, Minoo Niknian, Mark N Higgins, Valerie Tarn, Joy Davis, Sonya L Heltshe, Steven M Rowe
BACKGROUND AND OBJECTIVES: Cystic fibrosis (CF) is known for its impact on the lung and pancreas of individuals; however, impaired growth is also a common complication. We hypothesized that targeting the biological defect in the CF transmembrane conductance regulator (CFTR) protein may affect growth outcomes. METHODS: In this post hoc analysis, we assessed linear growth and weight in 83 children (aged 6-11 years) enrolled in 2 clinical trials, the longitudinal-observation GOAL study and the placebo-controlled ENVISION study, to evaluate the effects of ivacaftor, a CFTR potentiator...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28135043/household-proximity-to-water-and-nontuberculous-mycobacteria-in-children-with-cystic-fibrosis
#14
Jennifer M Bouso, James J Burns, Raid Amin, Floyd R Livingston, Okan Elidemir
BACKGROUND: Nontuberculous mycobacteria (NTM) have a particular affinity for patients with cystic fibrosis (CF). Recent studies suggest a possible relationship between acquiring NTM and the level of environmental water in a given area. We sought to determine if there is an association between household proximity to water and NTM in children with CF. MATERIALS AND METHODS: An IRB-approved retrospective chart review was completed on 150 children with CF in Florida...
January 30, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28132571/the-diagnosis-and-management-of-respiratory-viral-infections-in-cystic-fibrosis
#15
William Flight, Andrew Jones
Respiratory viruses, such as those that cause influenza and the common cold, are a regular feature of life for the entire human population. Among people with CF, these viruses are associated with prolonged respiratory illness and show a clear association with pulmonary exacerbations which in turn are associated with lung function decline and risk of death. Human rhinovirus is the most commonly encountered respiratory viral pathogen in CF although adenovirus, bocavirus, coronavirus, influenza, parainfluenza, metapneumovirus and respiratory syncytial virus are all also responsible for infections in this population...
February 3, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28131546/a-qualitative-analysis-of-the-information-needs-of-parents-of-children-with-cystic-fibrosis-prior-to-first-admission
#16
Vera Fixter, Catherine Butler, Jo Daniels, Samantha Phillips
PURPOSE: Hospitalization can be stressful for patients and their families. Pre-hospitalization information is crucial in establishing a good basis for patient satisfaction. In order to develop better preparatory material for parents, this qualitative study explored whether parents of children with cystic fibrosis, admitted to a UK Children's Hospital, felt adequately prepared for their child's admission. DESIGN AND METHODS: Data were collected from twelve parents whose children had been admitted within the last two years for routine intravenous antibiotics...
January 25, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28126911/characterization-of-inpatient-cystic-fibrosis-pulmonary-exacerbations
#17
Jonathan D Cogen, Assaf P Oron, Ronald L Gibson, Lucas R Hoffman, Matthew P Kronman, Thida Ong, Margaret Rosenfeld
BACKGROUND AND OBJECTIVES: Pulmonary exacerbations lead to significant morbidity and mortality in patients with cystic fibrosis (CF). National consensus guidelines exist, but few studies report current practice in the treatment and monitoring of pulmonary exacerbations. The goal of this study was to characterize consistency and variability in the inpatient management of CF-related pulmonary exacerbations. We focused on the use of guideline-recommended maintenance therapies, antibiotic selection and treatment regimens, use of systemic corticosteroids, and frequency of lung function testing...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#18
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/28114723/respiratory-muscle-training-improves-respiratory-muscle-endurance-but-not-exercise-tolerance-in-children-with-cystic-fibrosis
#19
Christian Bieli, Selina Summermatter, Urs Boutellier, Alexander Moeller
BACKGROUND: Respiratory muscle endurance (RME) training has been shown to increase exercise endurance and lung function in adults with cystic fibrosis (CF). We conducted an interventional study to investigate the effectiveness of RME training on CF-related health outcomes in children. METHODS: In a crossover trial, 22 children, aged 9-18 years, with CF performed 8 weeks of RME training and standard chest physiotherapy in a randomized sequence separated by a 1 week washout period...
January 23, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28103851/cystic-fibrosis-physicians-perspectives-on-the-timing-of-referral-for-lung-transplant-evaluation-a-survey-of-physicians-in-the-united-states
#20
Kathleen J Ramos, Ranjani Somayaji, Erika D Lease, Christopher H Goss, Moira L Aitken
BACKGROUND: Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge. METHODS: We developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015...
January 19, 2017: BMC Pulmonary Medicine
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