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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
#1
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
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#2
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28093464/socioeconomic-status-smoke-exposure-and-health-outcomes-in-young-children-with-cystic-fibrosis
#3
Thida Ong, Michael Schechter, Jing Yang, Limin Peng, Julia Emerson, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld
BACKGROUND: Lower socioeconomic status (SES) and environmental tobacco smoke (ETS) exposure are both associated with poorer disease outcomes in cystic fibrosis (CF), and children with low SES are disproportionately exposed to ETS. We analyzed a large cohort of young children with CF to distinguish the impact of SES and ETS on clinical outcomes. METHODS: The Early Pseudomonas Infection Control Observational study enrolled Pseudomonas-negative young children with CF <13 years of age...
January 16, 2017: Pediatrics
https://www.readbyqxmd.com/read/28088130/vaccine-candidates-against-nontypeable-haemophilus-influenzae-a-review
#4
REVIEW
Ava Behrouzi, Farzam Vaziri, Fatemeh Rahimi-Jamnani, Parviz Afrough, Mohammad Rahbar, Fereshteh Satarian, Seyed Davar Siadat
Nonencapsulated, nontypeable Hemophilus influenzae (NTHi) remains an important cause of acute otitis and respiratory diseases in children and adults. NTHi bacteria are one of the major causes of respiratory tract infections, including acute otitis media, cystic fibrosis, and community-acquired pneumonia among children, especially in developing countries. The bacteria can also cause chronic diseases such as chronic bronchitis and chronic obstructive pulmonary disease in the lower respiratory tract of adults...
2017: Iranian Biomedical Journal
https://www.readbyqxmd.com/read/28087053/six-minute-walk-test-results-predict-risk-of-hospitalization-for-youths-with-cystic-fibrosis-a-5-year-follow-up-study
#5
Márcio V F Donadio, João P Heinzmann-Filho, Fernanda M Vendrusculo, Patrícia X H Frasson, Paulo J C Marostica
OBJECTIVE: To evaluate the association of 6-minute walk test (6MWT) and other variables (anthropometry, chronic Pseudomonas aeroginosa colonization, pulmonary function, and respiratory muscle strength) with the risk of hospitalization for pulmonary exacerbation in children and adolescents with cystic fibrosis (CF). STUDY DESIGN: Cohort study that included patients with CF aged 6-18 years. All participants underwent spirometry, manovacuometry, and 6MWT during the 5-year follow-up...
January 10, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28075671/hospitalization-rates-for-intussusception-in-children-aged-0-59%C3%A2-months-from-2009-to-2014-in-italy
#6
Vincenzo Restivo, Claudio Costantino, Fabio Tramuto, Francesco Vitale
The real cause of intussusception is not fully understood and a variety of conditions have been associated with it (Meckel diverticulum, polyps, duplication cysts, parasites, Henoch-Schönlein purpura, cystic fibrosis, hemolytic-uremic syndrome and infectious gastroenteritis). Furthermore few European countries, following WHO recommendation to monitor baseline incidence of intussusception before implementation of immunization program for rotavirus, used intussusception rate as a baseline value to compare the same figures in the period before and after introduction of vaccination...
January 11, 2017: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/28062483/the-sialic-acid-to-urea-ratio-as-a-measure-of-airway-surface-hydration
#7
Charles R Esther, David B Hill, Brian Button, Shuai Shi, Corey M Jania, Elizabeth A Duncan, Claire M Doerschuk, Gang Chen, Sarath Ranganathan, Stephen Michael Stick, Richard C Boucher
INTRODUCTION: Although airway mucus dehydration is key to pathophysiology of cystic fibrosis (CF) and other airways diseases, measuring mucus hydration is challenging. We explored a robust method to estimate mucus hydration using sialic acid as a marker for mucin content. METHODS: Terminal sialic acid residues from mucins were cleaved by acid hydrolysis from airway samples, and concentrations of sialic acid, urea, and other biomarkers analyzed using mass spectrometry...
January 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28053855/two-cases-of-non-cystic-fibrosis-cf-bronchiectasis-with-allergic-bronchopulmonary-aspergillosis
#8
Hriday De, Syed Md Azad, Prabhas P Giri, Priyankar Pal, Apurba Ghosh, Anirban Maitra
Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung function in children with cystic fibrosis. We present two cases of non CF bronchiectasis associated with ABPA who presented to our hospital with recurrent hospitalisations of undiagnosed aetiology...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28049743/alternative-indexes-to-estimate-the-functional-capacity-from-the-6-minute-walk-test-in-children-and-adolescents-with-cystic-fibrosis
#9
Renata Tiemi Okuro, Maria Angela Gonçalves de Oliveira Ribeiro, José Dirceu Ribeiro, Rafaela Coelho Minsky, Camila Isabel Santos Schivinski
BACKGROUND: Cystic fibrosis is a multi-systemic disease related to reduced functional capacity. The distance covered in the 6-min walk test (6MWT) has been known to assess functional capacity, but little is known about other indexes that can be derived. We sought to compare the performance during the 6MWT and the estimated indexes of functional capacity from the 6MWT between subjects with cystic fibrosis (CF) and healthy individuals as well as to assess the relationship among these indexes and disease severity, pulmonary function, and nutritional status in CF...
January 3, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28042521/cftr-modulator-therapies-in-pediatric-cystic-fibrosis-focus-on-ivacaftor
#10
Elizabeth L Kramer, John P Clancy
INTRODUCTION: Mutations in the cystic fibrosis transmembrane conductance regulator protein (CFTR) cause cystic fibrosis (CF), a disease with life threatening pulmonary and gastrointestinal manifestations. Recent breakthrough therapies restore function to select disease-causing CFTR mutations. Ivacaftor is a small molecule that increases the open channel probability of certain CFTR mutations, producing clear evidence of bioactivity and efficacy in pediatric CF patients. CFTR modulators represent a significant advancement in CF treatment...
October 2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/28040232/how-well-do-children-with-cystic-fibrosis-sleep-an-actigraphic-and-questionnaire-based-study
#11
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
OBJECTIVE: To measure sleep patterns and quality, objectively and subjectively, in clinically stable children with cystic fibrosis (CF) and healthy control children, and to examine the relationship between sleep quality and disease severity. STUDY DESIGN: Clinically stable children with CF and healthy control children (7-18 years of age) were recruited. Sleep patterns and quality were measured at home with actigraphy (14 days). Overnight peripheral capillary oxygen saturation was measured via the use of pulse oximetry...
December 28, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/28005688/respiratory-syncytial-virus-infection-associated-hospitalization-rates-in-infants-and-children-with-cystic-fibrosis
#12
Jakob Metz, Ernst Eber, Bernhard Resch
BACKGROUND: Infections with respiratory syncytial virus (RSV) are the leading cause for hospital admissions in infants and young children. The incidence of RSV-related hospitalizations in patients with cystic fibrosis (CF) is unclear. To date no effective treatment for RSV infections is available. Thus, prophylaxis with the monoclonal antibody palivizumab is an important option. METHODS: In a retrospective, single-center study at the Department of Paediatrics and Adolescent Medicine of the Medical University Graz, Austria, we analyzed all CF patients born between 1995 and 2012, who were admitted for respiratory problems between 1995 and 2014...
December 21, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27992070/exercise-for-haemophilia
#13
REVIEW
Karen Strike, Kathy Mulder, Rojer Michael
BACKGROUND: Haemophilia is a bleeding disorder associated with haemorrhaging into joints and muscles. Exercise is often used to aid recovery after bleeds, and to improve joint function in the presence of arthropathy. OBJECTIVES: Our objective was to systematically review the available evidence on the safety and effectiveness of exercise for people with haemophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register and electronic databases PubMed, OVID-Embase, and CINAHL...
19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27989470/differences-between-who-and-cdc-early-growth-measurements-in-the-assessment-of-cystic-fibrosis-clinical-outcomes
#14
Danielle Usatin, Elizabeth H Yen, Catherine McDonald, Fadi Asfour, John Pohl, Jacob Robson
BACKGROUND: Early childhood growth status has been used to predict long-term clinical outcomes in Cystic Fibrosis (CF) patients. Adulthood CF outcomes based on early weight-for-length (WFL) measurements, using either World Health Organization (WHO) or Centers for Disease Control (CDC) scales, have not been compared. METHODS: Cystic Fibrosis Foundation registry patients were studied (n=3014). Participants were categorized at age two years as WFL <50th percentile on both WHO and CDC scales, ≥50th percentile on WHO but not CDC, or ≥50th percentile on both...
December 15, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27986496/medical-diagnostics-for-indoor-mold-exposure
#15
REVIEW
Julia Hurraß, 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, Gerhard A Wiesmüller
In April 2016, the German Society of Hygiene, Environmental Medicine and Preventative Medicine (Gesellschaft für Hygiene, Umweltmedizin und Präventivmedizin (GHUP)) together with other scientific medical societies, German and Austrian medical societies, physician unions and experts has provided an AWMF (Association of the Scientific Medical Societies) guideline 'Medical diagnostics for indoor mold exposure'. This guideline shall help physicians to advise and treat patients exposed indoors to mold. Indoor mold growth is a potential health risk, even without a quantitative and/or causal association between the occurrence of individual mold species and health effects...
December 5, 2016: International Journal of Hygiene and Environmental Health
https://www.readbyqxmd.com/read/27977404/sensitivity-and-specificity-of-different-methods-for-cystic-fibrosis-related-diabetes-screening-is-the-oral-glucose-tolerance-test-still-the-standard
#16
Catherine Mainguy, Gabriel Bellon, Véronique Delaup, Tiphanie Ginoux, Behrouz Kassai-Koupai, Stéphane Mazur, Muriel Rabilloud, Laurent Remontet, Philippe Reix
BACKGROUND: Cystic fibrosis-related diabetes (CFRD) is a late cystic fibrosis (CF)-associated comorbidity whose prevalence is increasing sharply lifelong. Guidelines for glucose metabolism (GM) monitoring rely on the oral glucose tolerance test (OGTT). However, this test is neither sensitive nor specific. The aim of this study was to compare sensitivity and specificity of different methods for GM monitoring in children and adolescents with CF. METHODS: Continuous glucose monitoring system (CGMS), used as the reference method, was compared with the OGTT, intravenous glucose tolerance test (IGTT), homeostasis model assessment index of insulin resistance (HOMA-IR), homeostasis model assessment index of β-cell function (HOMA-%B) and glycated haemoglobin A1C...
January 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27964949/vitamin-d-and-the-lung
#17
REVIEW
Sofia Konstantinopoulou, Ignacio E Tapia
Vitamin D metabolism is complex and its deficiency has proven to be deleterious in bone health, and to have significant effects on the immune and cardiovascular systems. Vitamin D has also been associated with pulmonary diseases outcomes. This review will focus on vitamin D metabolism, and studies performed mostly in children with asthma or Cystic Fibrosis.
October 31, 2016: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/27957481/respiratory-infection-rates-differ-between-geographically-distant-paediatric-cystic-fibrosis-cohorts
#18
Kathryn A Ramsey, Emily Hart, Lidija Turkovic, Marc Padros-Goossens, Stephen M Stick, Sarath C Ranganathan
Respiratory infections are a major cause of pulmonary decline in children with cystic fibrosis (CF). We compared the prevalence of infection in early life at geographically distant CF treatment centres participating in the same surveillance programme in Australia. Lower airway microbiology, inflammation and structural lung disease at annual review were evaluated for 260 children 0-8 years old with CF at 1032 visits to CF treatment centres in Melbourne or Perth. Melbourne patients were more likely to be culture-positive for common respiratory pathogens at all age groups (odds ratio (OR) 1...
July 2016: ERJ Open Research
https://www.readbyqxmd.com/read/27943680/progression-of-lung-disease-in-preschool-patients-with-cystic-fibrosis
#19
Sanja Stanojevic, Stephanie D Davis, George Retsch-Bogart, Hailey Webster, Miriam Davis, Robin C Johnson, Renee Jensen, Maria Ester Pizarro, Mica Kane, Charles C Clem, Leah Schornick, Padmaja Subbarao, Felix A Ratjen
RATIONALE: Implementation of intervention strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures that capture and track lung disease. OBJECTIVE: To define the utility of the Lung Clearance Index (LCI), measured by Multiple Breath Washout (MBW), as a means to track disease progression in preschool children with CF. METHODS: CF children between the ages of 2.5 and 6 years with a confirmed diagnosis of CF and age-matched healthy controls were enrolled at three North American CF centers...
December 12, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27941194/-genetic-mutations-as-a-cause-of-acute-recurrent-pancreatitis-in-children-case-report-and-literature-review
#20
Tatiana Jamer, Barbara Iwańczak
Acute recurrent pancreatitis is not common in children. The epidemiology, etiology and clinical presentation of pediatric acute recurrent pancreatitis are not well understood. The etiology is diverse and multifactorial, with many cases being idiopathic. The most common etiology of acute recurrent pancreatitis in children are genetic factors, biliary duct disorders, anatomic anomalies of the pancreatobiliary system and metabolic diseases. Mutations are most commonly found in the cationic trypsynogen gene (PRSS1), the pancreatic secretory trypsin inhibitor gene (SPINK1) and the cystic fibrosis transmembrane conductance regulator gene (CFTR)...
2016: Developmental Period Medicine
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