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https://www.readbyqxmd.com/read/29226186/prevalence-of-pelvic-incontinence-in-patients-with-cystic-fibrosis
#1
Fatima Neemuchwala, Fahmida Ahmed, Samya Z Nasr
Cystic fibrosis (CF) patients are at risk for developing pelvic (urinary and/or fecal) incontinence due to progressive weakness of pelvic floor muscles secondary to recurrent episodes of coughing and respiratory infections. Many patients do not bring these symptoms to the attention of their health care providers because of social embarrassment and lack of knowledge of available effective treatment. Several studies have identified the prevalence of incontinence in CF adults and adolescents. However, few studies identified the problem in children with CF...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/29225146/microbial-diversity-within-the-airway-microbiome-in-chronic-pediatric-lung-diseases
#2
Andrea Hahn, Stephanie Warnken, Marcos Pérez-Losada, Robert J Freishtat, Keith A Crandall
The study of the airway microbiome in children is an area of emerging research, especially in relation to the role microbial diversity may play in acute and chronic inflammation. Three such pediatric airway diseases include cystic fibrosis, asthma, and chronic lung disease of prematurity. In cystic fibrosis, the presence of Pseudomonas spp. is associated with decreased microbial diversity. Decreasing microbial diversity is also associated with poor lung function. In asthma, early viral infections appear to drive changes in bacterial diversity which may be associated with asthma risk...
December 7, 2017: Infection, Genetics and Evolution
https://www.readbyqxmd.com/read/29224788/long-term-intestinal-obstruction-sequelae-and-growth-in-children-with-cystic-fibrosis-operated-for-meconium-ileus-expectancies-and-surprises
#3
Anastasia Mentessidou, Ioanna Loukou, Georgios Kampouroglou, Anastasia Livani, Ioannis Georgopoulos, Petros Mirilas
BACKGROUND/PURPOSE: In the few studies on intestinal complications and growth of cystic fibrosis (CF) patients with a history of meconium ileus (MI), operated MI has not been investigated separately. We aimed to investigate the incidence of long-term intestinal obstruction sequelae [constipation, distal intestinal obstruction syndrome (DIOS)] and growth in CF patients operated for MI. METHODS: Retrospective study (1989-2016) including operative diagnoses and procedures, constipation and DIOS events, yearly Body Mass Index (BMI) measurements...
November 15, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29224659/bronchial-artery-embolization-for-the-treatment-of-acute-hemoptysis
#4
M Cody O'Dell, Anne E Gill, C Matthew Hawkins
Massive hemoptysis is a life-threatening condition often defined as coughing up 300-600mL of blood in 24 hours in an adult, or >8mL/kg in 24 hours in a child. Although the definition is controversial, one should view massive hemoptysis as any volume of expectorated blood that can cause respiratory failure. This is because mortality in the setting of hemoptysis is usually associated with asphyxiation, rather than exsanguination. Massive hemoptysis accounts for only about 5% of cases of hemoptysis, but when treated conservatively, has a reported mortality rate between 50% and 85%...
December 2017: Techniques in Vascular and Interventional Radiology
https://www.readbyqxmd.com/read/29222447/structural-abnormalities-in-islets-from-very-young-children-with-cystic-fibrosis-may-contribute-to-cystic-fibrosis-related-diabetes
#5
Marika Bogdani, Scott M Blackman, Cecilia Ridaura, Jean-Pierre Bellocq, Alvin C Powers, Lydia Aguilar-Bryan
Cystic fibrosis (CF)-related diabetes (CFRD) is thought to result from beta-cell injury due in part to pancreas exocrine damage and lipofibrosis. CFRD pancreata exhibit reduced islet density and altered cellular composition. To investigate a possible etiology, we tested the hypothesis that such changes are present in CF pancreata before the development of lipofibrosis. We evaluated pancreas and islet morphology in tissues from very young CF children (<4 years of age), and adult patients with CF and CFRD...
December 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29220099/supportive-palliative-care-should-be-integrated-into-routine-care-for-paediatric-patients-with-life-limiting-kidney-disease
#6
REVIEW
Julia Thumfart, Tobias Reindl, Cornelia Rheinlaender, Dominik Müller
AIM: Paediatric palliative care is no longer restricted to patients with cancer and has been extended to patients with other chronic conditions, such as cystic fibrosis or neuromuscular disorders. This review focused on the current state of palliative care for children and adolescents with chronic kidney disease (CKD). METHOD: We assessed the literature on CKD published up to August 2017. All the papers, except one from 1996, were published this century. This review discusses the role that palliative care plays in the process of decision-making and explores the possibilities of implementing palliative care into the routine therapy of affected patients and providing support for their families...
December 8, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29218501/evaluating-safety-reporting-in-paediatric-antibiotic-trials-2000-2016-a-systematic-review-and-meta-analysis
#7
REVIEW
Paola Pansa, Yingfen Hsia, Julia Bielicki, Irja Lutsar, A Sarah Walker, Mike Sharland, Laura Folgori
BACKGROUND: There are very few options to treat multidrug-resistant bacterial infections in children. A major barrier is the duration and complexity of regulatory trials of new antibiotics. Extrapolation of safety data from adult trials could facilitate drug development for children. OBJECTIVE: We performed a systematic review on the safety of antibiotic clinical trials (CTs) in children (0-18 years) to evaluate the overall quality of safety trials conducted in children and to determine if age-specific adverse events (AEs) could be identified for specific antibiotic classes...
December 7, 2017: Drugs
https://www.readbyqxmd.com/read/29217601/novel-magnetic-resonance-technique-for-functional-imaging-of-cystic-fibrosis-lung-disease
#8
Sylvia Nyilas, Grzegorz Bauman, Gregor Sommer, Enno Stranzinger, Orso Pusterla, Urs Frey, Insa Korten, Florian Singer, Carmen Casaulta, Oliver Bieri, Philipp Latzin
Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2-MBW)...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29216871/mental-health-literacy-among-pediatric-hospital-staff-in-the-united-arab-emirates
#9
Nabeel Al-Yateem, Rachel Rossiter, Walter Robb, Alaa Ahmad, Mahmoud Saleh Elhalik, Sumaya Albloshi, Shameran Slewa-Younan
BACKGROUND: In the United Arab Emirates (UAE) 35% of the population are aged 0-24 years. A significant proportion of these young people are living with chronic conditions (e.g., asthma, type 1 diabetes, cardiac conditions, and genetically-transmitted conditions such as thalassemia and cystic fibrosis). This group has increased vulnerability to developmental delays and mental health problems, and is increasingly coming to the attention of service providers in mainstream schools, primary healthcare centers, and pediatric hospitals...
December 8, 2017: BMC Psychiatry
https://www.readbyqxmd.com/read/29206744/detectable-concentrations-of-inhaled-tobramycin-in-critically-ill-children-without-cystic-fibrosis-should-routine-monitoring-be-recommended
#10
Jamie L Miller, Trisha M Lepa, Courtney Ranallo, Hala Chaaban, Grant H Skrepnek, Peter N Johnson
OBJECTIVES: To determine the percentage of detectable tobramycin troughs and acute kidney injury in critically ill children without cystic fibrosis on inhaled therapy. DESIGN: Historic cohort. SETTING: Academic hospital. PATIENTS: Forty children less than 18 years receiving inhaled tobramycin across 6.5 years. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary objective was to determine the percentage of detectable tobramycin troughs greater than or equal to 0...
December 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29176899/supine-posture-changes-lung-volumes-and-increases-ventilation-heterogeneity-in-cystic-fibrosis
#11
Laurie J Smith, Kenneth A Macleod, Guilhem J Collier, Felix C Horn, Helen Sheridan, Ina Aldag, Chris J Taylor, Steve Cunningham, Jim M Wild, Alex Horsley
INTRODUCTION: Lung Clearance Index (LCI) is recognised as an early marker of cystic fibrosis (CF) lung disease. The effect of posture on LCI however is important when considering longitudinal measurements from infancy and when comparing LCI to imaging studies. METHODS: 35 children with CF and 28 healthy controls (HC) were assessed. Multiple breath washout (MBW) was performed both sitting and supine in triplicate and analysed for LCI, Scond, Sacin, and lung volumes...
2017: PloS One
https://www.readbyqxmd.com/read/29173301/targeted-gene-next-generation-sequencing-in-chinese-children-with-chronic-pancreatitis-and-acute-recurrent-pancreatitis
#12
Yuan Xiao, Wentao Yuan, Bo Yu, Yan Guo, Xu Xu, Xinqiong Wang, Yi Yu, Yi Yu, Biao Gong, Chundi Xu
OBJECTIVE: To identify causal mutations in certain genes in children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP). STUDY DESIGN: After patients were enrolled (CP, 55; ARP, 14) and their clinical characteristics were investigated, we performed next-generation sequencing to detect nucleotide variations among the following 10 genes: cationic trypsinogen protease serine 1 (PRSS1), serine protease inhibitor, Kazal type 1 (SPINK1), cystic fibrosis transmembrane conductance regulator gene (CFTR), chymotrypsin C (CTRC), calcium-sensing receptor (CASR), cathepsin B (CTSB), keratin 8 (KRT8), CLAUDIN 2 (CLDN2), carboxypeptidase A1 (CPA1), and ATPase type 8B member 1 (ATP8B1)...
December 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29164174/early-life-pseudomonas-aeruginosa-infection-in-cystic-fibrosis-and-lung-disease-progression
#13
Argyri Petrocheilou, Maria Papagrigoriou-Theodoridou, Athanasios Michos, Stavros-Eleftherios Doudounakis, Ioanna Loukou, Athanasios Kaditis
Lung disease in cystic fibrosis (CF) starts early, with studies identifying abnormalities on chest computed tomography (CT) scan even in infancy. In this retrospective study, abnormal chest CT was the main outcome; body mass index (BMI) z score and forced expiratory volume percent predicted (FEV1%) predicted at age 6 to 7 years were secondary outcomes. Pseudomonas aeruginosa infection prior to 12 months of age was the main explanatory variable. There was no association between early P aeruginosa infection and abnormal CT after adjustment for CFTR (cystic fibrosis transmembrane conductance regulator) functional mutation class, gender, and other pathogens (odds ratio = 0...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/29162121/the-involvement-of-musculoskeletal-system-and-its-influence-on-postural-stability-in-children-and-young-adults-with-cystic-fibrosis
#14
Ozge Kenis-Coskun, Evrim Karadag-Saygi, Yeliz Bahar-Ozdemir, Yasemin Gokdemir, Bulent Karadag, Onder Kayhan
BACKGROUND: Cystic fibrosis (CF) affects the musculoskeletal system via a multifactorial pathway that includes vitamin D deficiency and involvement of respiratory muscles such as intercostals due to recurrent upper and lower respiratory tract infections. Eventual result is the deterioration of musculoskeletal health and posture in CF patients. Postural stability is directly affected by posture and can be compromised in every musculoskeletal problem. The aim of this study is to evaluate musculoskeletal system and postural stability in patients with CF...
November 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29157921/pooling-of-bronchoalveolar-lavage-in-children-with-cystic-fibrosis-does-not-adversely-affect-the-microbiological-yield-or-sensitivity-in-detecting-pulmonary-inflammation
#15
Paul McNally, Jennifer O'Rourke, Emmanuelle Fantino, Archana Chacko, Rishi Pabary, Andrew Turnbull, Tim Grant, Niamh O'Sullivan, Claire Wainwright, Barry Linnane, Jane C Davies, Peter D Sly
BACKGROUND: Bronchoalveolar lavage (BAL) is a potentially useful outcome measure for clinical trials in children with CF but its use is limited by variations in approach internationally. We sought to determine if pooling adversely affected the diagnostic properties of BAL. METHODS: Children undergoing bronchoscopy for clinical reasons were included. A multi-step study protocol ensured BAL was collected and analysed both separately and as a pooled fluid. RESULTS: Eighty-five children (53 CF, 32 control) were recruited...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29155436/enzyme-replacement-therapy-for-infantile-onset-pompe-disease
#16
REVIEW
Min Chen, Lingli Zhang, Shuyan Quan
BACKGROUND: Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency...
November 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29154891/acute-pain-perception-during-different-sampling-methods-for-respiratory-culture-in-cystic-fibrosis-patients
#17
Hanneke Eyns, Elke De Wachter, Anne Malfroot, Peter Vaes
BACKGROUND: Reliable identification of lower respiratory tract pathogens is crucial in the management of cystic fibrosis (CF). As part of another study (NCT02363764) investigating the bacterial yield of three sampling methods: nasal swabs (NS), cough swabs (CS) and (induced) sputum samples ((I)SS) in both expectorating (EPs) and non-expectorating (NEPs) patients with CF, the present study aimed to explore the prevalence of respiratory culture sampling-related pain as assessed by self-report within a cohort of children and adults...
November 14, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29151314/effect-of-inhaled-steroids-on-clinical-and-inflammatoryparameters-in-children-with-cystic-fibrosis
#18
Zeynep Seda Uyan, Göksenin Ünlügüzel Üstün, Goncagül Haklar, Erkan Çakır, Sedat Oktem, Refika Ersu, Bülent Taner Karadağ, Fazilet Karakoç, Elif Dağlı
Background/aim: The effectiveness of inhaled corticosteroids (ICSs) in cystic fibrosis (CF) is controversial. The aim of this study was to investigate the effect of an ICS on bronchial hyperreactivity (BHR), oxidative status, and clinical and inflammatory parameters in CF patients. Materials and methods: CF patients were randomized to receive either 2 mg/day nebulized budesonide or 0.9% normal saline as placebo for 8 weeks. Results: Twenty-nine CF patients (mean age: 10.5 ± 2.9 years) were enrolled in the study...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29150357/caregiver-burden-of-parents-of-young-children-with-cystic-fibrosis
#19
C Fitzgerald, S George, R Somerville, B Linnane, P Fitzpatrick
BACKGROUND: There is a paucity of research examining the impact of informal caregiving on parents of young children with cystic fibrosis (CF). The aim of this study was to examine caregiver burden and identify risk factors associated with high caregiver burden in mothers and fathers of young children with CF. METHODS: This was a cross-sectional study of parents of young children with CF. A total of 213 families were invited to complete the CarerQoL questionnaire, a validated tool composed of two parts: (i) the CarerQol-7D which describes the care situation in terms of the negative and positive effects of caregiving and (ii) the visual analogue scale (VAS) which measures happiness on a scale from 0 to 10 (0=completely unhappy and 10=completely happy)...
November 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29146601/airway-microbiota-across-age-and-disease-spectrum-in-cystic-fibrosis
#20
Edith T Zemanick, Brandie D Wagner, Charles E Robertson, Richard C Ahrens, James F Chmiel, John P Clancy, Ronald L Gibson, William T Harris, Geoffrey Kurland, Theresa A Laguna, Susanna A McColley, Karen McCoy, George Retsch-Bogart, Kurtis T Sobush, Pamela L Zeitlin, Mark J Stevens, Frank J Accurso, Scott D Sagel, J Kirk Harris
Our objectives were to characterise the microbiota in cystic fibrosis (CF) bronchoalveolar lavage fluid (BALF), and determine its relationship to inflammation and disease status.BALF from paediatric and adult CF patients and paediatric disease controls undergoing clinically indicated bronchoscopy was analysed for total bacterial load and for microbiota by 16S rDNA sequencing.We examined 191 BALF samples (146 CF and 45 disease controls) from 13 CF centres. In CF patients aged <2 years, nontraditional taxa (e...
November 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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