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https://www.readbyqxmd.com/read/28334845/infantile-tremor-syndrome-and-subdural-hemorrhage-in-an-infant-with-cystic-fibrosis
#1
Anirban Mandal, Mayank Priyadarshi, Kanaram Jat, Sushil Kumar Kabra
Cystic fibrosis (CF), an autosomal recessive disease with multi-system involvement, may present with bleeding in infancy owing to vitamin K malabsorption. Infantile tremor syndrome (ITS) is an obscure condition associated with vitamin B12 and other micronutrient deficiencies, described predominantly in Indian subcontinent. We describe an infant presenting with ITS and chronic subdural hemorrhage. He was subsequently diagnosed to have CF. The ITS and subdural hemorrhage is rarely reported in children with CF...
February 23, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28333826/complex-relation-between-diet-and-phospholipid-fatty-acids-in-children-with-cystic-fibrosis
#2
Sara Moukarzel, Roger A Dyer, Sheila M Innis
OBJECTIVES: Altered total plasma n-6 and n-3 fatty acids are common in cystic fibrosis (CF). Whether alterations extend to plasma phosphatidylcholine (PC) and phosphatidylethanolamine (PE) and are explained by diet is unclear. The present study was to describe the dietary intake of a large group of children with CF and to determine whether dietary fat composition explains differences in plasma PC and PE fatty acids between children with and without CF. METHODS: Dietary intake was assessed using a food frequency questionnaire...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28332032/qualitative-assessment-of-the-symptoms-and-impact-of-pancreatic-exocrine-insufficiency-pei-to-inform-the-development-of-a-patient-reported-outcome-pro-instrument
#3
Colin D Johnson, Rob Arbuckle, Nicola Bonner, Gary Connett, Enrique Dominguez-Munoz, Philippe Levy, Doris Staab, Nicola Williamson, Markus M Lerch
BACKGROUND: Pancreatic exocrine insufficiency (PEI) affects patients with chronic pancreatitis (CP) and cystic fibrosis (CF) who produce insufficient digestive pancreatic enzymes. Common symptoms include steatorrhoea, diarrhea, and abdominal pain. OBJECTIVE: The objective of the study was to develop and test the content validity of a patient-reported outcome (PRO) instrument assessing PEI symptoms and their impact on health-related quality of life. METHODS: Instrument development was supported by a literature review, expert physician interviews (n = 10: Germany 4, UK 3, France 3), and exploratory, qualitative, concept-elicitation interviews with patients with CF and CP with PEI (n = 61: UK 29, Germany 18, France 14) and expert physicians (n = 10)...
March 22, 2017: Patient
https://www.readbyqxmd.com/read/28330773/a-3-year-prognostic-score-for-adults-with-cystic-fibrosis
#4
L Nkam, J Lambert, A Latouche, G Bellis, P R Burgel, M N Hocine
BACKGROUND: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients. METHODS: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic...
March 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28329612/aquagenic-wrinkling-of-the-palms-a-case-report-and-literature-review
#5
Matteo Megna, Mariateresa Cantelli, Donata Martellotta, Gabriella Calabrò, Anna Balato, Fabio Ayala
Aquagenic wrinkling of the palms (AWP) is an unusual and rare dermatological condition characterized by excessive palmar wrinkling, occurring within a few minutes of water exposure. Cystic fibrosis (CF) or CF carrier state associated forms, drug induced cases, and idiopathic forms have been described. We report the case of a 27-year-old woman with a 7-year history of transient excessive wrinkling of her palms after brief exposure to water. We present also a comprehensive review of the literature. We believe that AWP has been underdiagnosed thus far and we would like to encourage investigations such as sweat chloride test or genetic studies in these patients because of the association with CF or CF carrier state, particularly when AWP appears in younger ages...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28329534/acquired-aquagenic-papulotranslucent-acrokeratoderma
#6
Sarah E Yagerman, Marie Lager, Nicholas A Soter
Aquagenic papulotranslucent acrokeratoderma isa rare condition with the development of white-totransluscentpapules and plaques after exposureto water. While the first report was described asan autosomal dominant hereditary condition,there have since been acquired cases reported inassociation with cystic fibrosis, with prior exposureto a drug, or as an idiopathic condition. We presenta 24-year-old man with acquired aquagenicpapulotranslucent acrokeratoderma that has beenpresent since infancy, without a family history,without prior drug exposure, and without anypersonal or family history of cystic fibrosis...
December 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28329304/cystic-fibrosis-pulmonary-exacerbations-attributable-to-respiratory-syncytial-virus-and-influenza-a-population-based-study
#7
Ranjani Somayaji, Christopher H Goss, Umer Khan, Moni Neradilek, Kathleen M Neuzil, Justin R Ortiz
Background: Characterization of the role of respiratory viral pathogens on cystic fibrosis (CF) pulmonary disease is needed. We aimed to determine the association of influenza and respiratory syncytial virus (RSV) activity with risk of pulmonary exacerbation (PEx) in persons with CF in the United States. Methods: We conducted a cohort study from January 2003 - March 2009 using the CF Foundation Patient Registry merged with CDC respiratory virus surveillance data...
March 9, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28328157/lack-of-small-colony-variants-of-staphylococcus-aureus-from-lower-respiratory-tract-specimens
#8
Rosemary Carzino, Emily Hart, Philip Sutton, Louise King, Sarath Ranganathan
BACKGROUND: Small-colony variants (SCVs) of Staphylococcus aureus are associated with worse lung disease in children with Cystic Fibrosis (CF), exhibit a higher resistance to antibiotics and co-colonize more commonly with Pseudomonas aeruginosa compared to the normal phenotype. The prevalence of SCVs in lower airway specimens from children with CF is largely unknown. METHODS: Each visible morphotype of S. aureus was subcultured onto horse blood agar (HBA) to enable identification of SCVs...
March 22, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28325850/phenotypic-diversity-and-genotypic-flexibility-of-burkholderia-cenocepacia-during-long-term-chronic-infection-of-cystic-fibrosis-lungs
#9
Amy Huei-Yi Lee, Stephane Flibotte, Sunita Sinha, Adrianna Paiero, Rachel L Ehrlich, Sergey Balashov, Garth D Ehrlich, James E A Zlosnik, Joshua Chang Mell, Corey Nislow
Chronic bacterial infections of the lung are the leading cause of morbidity and mortality in cystic fibrosis patients. Tracking bacterial evolution during chronic infections can provide insights into how host selection pressures-including immune responses and therapeutic interventions-shape bacterial genomes. We carried out genomic and phenotypic analyses of 215 serially collected Burkholderia cenocepacia isolates from 16 cystic fibrosis patients, spanning a period of 2-20 yr and a broad range of epidemic lineages...
March 21, 2017: Genome Research
https://www.readbyqxmd.com/read/28325787/impaired-defenses-of-neonatal-mouse-alveolar-macrophage-with-cftr-deletion-are-modulated-by-glutathione-and-tgf%C3%AE-1
#10
Theresa W Gauthier, Jocelyn R Grunwell, Xiao-Du Ping, Frank L Harris, Lou Ann S Brown
Our understanding of the intrinsic effects of cystic fibrosis (CF) transmembrane conductance regulator (cftr) deletion on resident neonatal alveolar macrophage (AM) remains limited. We previously demonstrated that diminished glutathione (GSH) or excessive AM transforming growth factor beta one (TGFβ1) contributes to AM dysfunction in a variety of disease states. In this study, using a gut-corrected cftr neonatal knockout (KO) mouse model and a siRNA-manipulated macrophage-like cell line (THP-1 cell), we hypothesized (1) that cftr mutation alone increases neonatal AM oxidant stress and cellular TGFβ1 signaling via altered GSH, thereby impairing cellular function, and (2) that exogenous GSH attenuates AM alterations and dysfunction in the KO AM In neonatal KO mice, the baseline bronchoalveolar lavage fluid demonstrated a near doubling in mixed disulfides (P ≤ 0...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28325763/pseudomonas-aeruginosa-alginate-overproduction-promotes-coexistence-with-staphylococcus-aureus-in-a-model-of-cystic-fibrosis-respiratory-infection
#11
Dominique H Limoli, Gregory B Whitfield, Tomoe Kitao, Melissa L Ivey, Michael R Davis, Nora Grahl, Deborah A Hogan, Laurence G Rahme, P Lynne Howell, George A O'Toole, Joanna B Goldberg
While complex intra- and interspecies microbial community dynamics are apparent during chronic infections and likely alter patient health outcomes, our understanding of these interactions is currently limited. For example, Pseudomonas aeruginosa and Staphylococcus aureus are often found to coinfect the lungs of patients with cystic fibrosis (CF), yet these organisms compete under laboratory conditions. Recent observations that coinfection correlates with decreased health outcomes necessitate we develop a greater understanding of these interbacterial interactions...
March 21, 2017: MBio
https://www.readbyqxmd.com/read/28325727/effect-of-allergic-bronchopulmonary-aspergillosis-on-fev1-in-children-and-adolescents-with-cystic-fibrosis-a-european-cystic-fibrosis-society-patient-registry-analysis
#12
Athanasios G Kaditis, Michael Miligkos, Anna Bossi, Carla Colombo, Elpis Hatziagorou, Nataliya Kashirskaya, Isabelle de Monestrol, Muriel Thomas, Meir Mei-Zahav, George Chrousos, Anna Zolin
OBJECTIVE: To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1 percent predicted in children and adolescents with cystic fibrosis. DESIGN: Longitudinal data analysis (2008-2010). SETTING: Patients participating in the European Cystic Fibrosis Society Patient Registry. PARTICIPANTS: 3350 patients aged 6-17 years. MAIN OUTCOME MEASURE: FEV1percent predicted was the main outcome measure (one measurement per year per child)...
March 21, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28325531/real-life-initiation-of-lumacaftor-ivacaftor-combination-in-adults-with-cystic-fibrosis-homozygous-for-the-phe508del-cftr-mutation-and-severe-lung-disease
#13
Dominique Hubert, Raphaël Chiron, Boubou Camara, Dominique Grenet, Anne Prévotat, Laurence Bassinet, Stéphane Dominique, Gilles Rault, Julie Macey, Isabelle Honoré, Reem Kanaan, Sylvie Leroy, Nadine Desmazes Dufeu, Pierre-Régis Burgel
OBJECTIVE: To investigate the short-term adverse events and effectiveness of lumacaftor/ivacaftor combination treatment in adults with cystic fibrosis (CF) and severe lung disease in a real life setting. METHODS: A multicentre observational study investigated adverse events, treatment discontinuation, FEV1 and body mass index (BMI) one month and three months after lumacaftor/ivacaftor initiation in adults with CF and FEV1 below 40% predicted. RESULTS: Respiratory adverse events (AEs) were reported by 27 of 53 subjects (51%) and 16 (30%) discontinued treatment...
March 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28324670/relationship-of-antibiotic-treatment-to-recovery-after-acute-fev1-decline-in-children-with-cystic-fibrosis
#14
Wayne J Morgan, Jeffrey S Wagener, David J Pasta, Stefanie J Millar, Donald R VanDevanter, Michael W Konstan
RATIONALE: Children with cystic fibrosis often suffer acute declines in lung function. We previously showed that such declines are not always treated with antibiotics, but did not assess whether treatment improves the likelihood of recovery. OBJECTIVES: To determine whether new antibiotic treatment was associated with recovery from acute FEV1 decline. METHODS: We studied episodes of FEV1 decline (≥10% from baseline) from the Epidemiologic Study of Cystic Fibrosis...
March 21, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28323913/accrual-of-bone-mass-in-children-and-adolescents-with-cystic-fibrosis
#15
Sonakshi Sharma, Mirjana Jaksic, Sheryl Fenwick, Catherine Byrnes, Tim Cundy
Context: Low bone density is a recognised complication of cystic fibrosis (CF). Hypothesis: Accrual of bone mass is most impaired in the sickest children, as judged by nutritional status and pulmonary function. Design: Retrospective analysis of correlation between lumbar spine BMD, BMI and FEV1 z-score in children and adolescents with CF. Setting: Specialist CF service at a pediatric hospital. Patients: 60 participants aged 5...
February 22, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321912/boosting-joint-models-for-longitudinal-and-time-to-event-data
#16
Elisabeth Waldmann, David Taylor-Robinson, Nadja Klein, Thomas Kneib, Tania Pressler, Matthias Schmid, Andreas Mayr
Joint models for longitudinal and time-to-event data have gained a lot of attention in the last few years as they are a helpful technique clinical studies where longitudinal outcomes are recorded alongside event times. Those two processes are often linked and the two outcomes should thus be modeled jointly in order to prevent the potential bias introduced by independent modeling. Commonly, joint models are estimated in likelihood-based expectation maximization or Bayesian approaches using frameworks where variable selection is problematic and that do not immediately work for high-dimensional data...
March 21, 2017: Biometrical Journal. Biometrische Zeitschrift
https://www.readbyqxmd.com/read/28320883/the-pseudomonas-aeruginosa-two-component-regulator-algr-directly-activates-rsma-expression-in-a-phosphorylation-independent-manner
#17
Sean D Stacey, Danielle A Williams, Christopher L Pritchett
Pseudomonas aeruginosa is an important pathogen of the immunocompromised, causing both acute and chronic infections. In cystic fibrosis (CF) patients, P. aeruginosa causes chronic disease. The impressive sensory network of P. aeruginosa allows the bacteria to sense and respond to a variety of stimuli found in diverse environments. Transcriptional regulators, including alternative sigma factors and response regulators, integrate signals changing gene expression allowing P. aeruginosa to cause infection. The two-component transcriptional regulator AlgR is important in P...
March 20, 2017: Journal of Bacteriology
https://www.readbyqxmd.com/read/28320633/nutritional-status-nutrient-intake-and-use-of-enzyme-supplements-in-paediatric-patients-with-cystic-fibrosis-a-european-multicentre-study-with-reference-to-current-guidelines
#18
Joaquim Calvo-Lerma, Jessie M Hulst, Inês Asseiceira, Ine Claes, Maria Garriga, Carla Colombo, Victoria Fornés, Sandra Woodcock, Tiago Martins, Mieke Boon, Mar Ruperto, Sylvia Walet, Chiara Speziali, Peter Witters, Etna Masip, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
BACKGROUND: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines. METHODS: Cross sectional study in paediatric patients with CF from 6 European centres...
March 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28320629/is-acute-recurrent-pancreatitis-in-children-a-precursor-of-chronic-pancreatitis-a-long-term-follow-up-study-of-93-cases
#19
Ujjal Poddar, Surender K Yachha, Vibhor Borkar, Anshu Srivastava
BACKGROUND/AIMS: In view of paucity of literature we analyzed our experience of acute recurrent pancreatitis (ARP) to study clinical profile and long-term outcome. METHODS: Over 13 years, 93 consecutive children (≤18 years) diagnosed to have ARP were included in this study. Magnetic resonance cholangiopancreatography was done at baseline and on follow-up. Common mutations for serine-protease-inhibitor (SPINK1 N34S), protease inhibitor (PRSS1 R122S) and cystic fibrosis transmembrane conductance regulator (CFTR deltaF508, 5T) were studied in 22 idiopathic cases...
March 6, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28319489/autogenic-drainage-in-children-with-cystic-fibrosis
#20
Lieselotte Corten, Brenda M Morrow
PURPOSE: Airway clearance is an essential part of the management of cystic fibrosis (CF) as it facilitates clearance of viscous pulmonary secretions. This review aimed to determine the effect of autogenic drainage (AD) and assisted autogenic drainage (AAD) compared with no, sham, or other types of airway clearance in children with CF. SUMMARY OF KEY POINTS: Two pediatric randomized cross-over trials were identified on the use of AD in children with CF; no studies were available on the use of AAD...
March 17, 2017: Pediatric Physical Therapy
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