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Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society

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https://www.readbyqxmd.com/read/29042177/antibiotic-exposure-and-interpersonal-variance-mask-the-effect-of-ivacaftor-on-respiratory-microbiota-composition
#1
Anton Y Peleg, Jocelyn Choo, Katherine Langan, Deirdre Edgeworth, Dominic Keating, John Wilson, Geraint B Rogers, Tom Kotsimbos
BACKGROUND: G551D is a class III mutation of the cystic fibrosis transmembrane regulator (CFTR) that results in impaired chloride channel function in cystic fibrosis (CF). Ivacaftor, a CFTR-potentiating agent improves sweat chloride, weight, lung function, and pulmonary exacerbation rate in CF patients with G551D mutations, but its effect on the airway microbiome remains poorly characterised. METHODS: Twenty CF patients with at least one G551D mutation from a single centre were recruited to a 4month double-blind, placebo-controlled, crossover study of ivacaftor with 28days of active treatment...
October 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29037538/glucose-trajectories-in-cystic-fibrosis-and-their-association-with-pulmonary-function
#2
Quitterie Reynaud, Muriel Rabilloud, Sylvain Roche, Stéphanie Poupon-Bourdy, Jean Iwaz, Raphaële Nove-Josserand, Emilie Blond, Martine Laville, Cathy Llerena, Sébastien Quetant, Philippe Reix, Sandrine Touzet, Isabelle Durieu
BACKGROUND: The prevalence of cystic fibrosis-related diabetes is increasing. This condition is potentially responsible for respiratory decline. METHODS: At inclusion, then yearly (over three years), 111 children and 117 adults with cystic fibrosis had oral glucose tolerance and insulin tests at one (G1) and 2h (G2). KmL analysis identified homogeneous G1 and G2 glucose trajectories. A linear mixed model quantified the relationships between trajectories and FEV1 changes...
October 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29033275/mechanisms-of-humoral-immune-response-against-pseudomonas-aeruginosa-biofilm-infection-in-cystic-fibrosis
#3
REVIEW
Renan Marrichi Mauch, Peter Østrup Jensen, Claus Moser, Carlos Emilio Levy, Niels Høiby
P. aeruginosa chronic lung infection is the major cause of morbidity and mortality in patients with cystic fibrosis (CF), and is characterized by a biofilm mode of growth, increased levels of specific IgG antibodies and immune complex formation. However, despite being designed to combat this infection, such elevated humoral response is not associated with clinical improvement, pointing to a lack of anti-pseudomonas effectiveness. The mode of action of specific antibodies, as well as their structural features, and even the background involving B-cell production, stimulation and differentiation into antibody-producing cells in the CF airways are poorly understood...
October 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29032179/corrigendum-to-does-current-reporting-of-lung-function-by-the-uk-cystic-fibrosis-registry-allow-a-fair-comparison-of-adult-centres-j-cyst-fibros-2017-585-591
#4
J A Nightingale, C Osmond
No abstract text is available yet for this article.
October 11, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29032178/igg-avidity-to-pseudomonas-aeruginosa-over-the-course-of-chronic-lung-biofilm-infection-in-cystic-fibrosis
#5
Renan Marrichi Mauch, Lena Lingren Nørregaard, Oana Ciofu, Carlos Emilio Levy, Niels Høiby
BACKGROUND AND OBJECTIVES: The mechanisms leading to low effectiveness of the humoral immune response against P. aeruginosa in cystic fibrosis (CF) are poorly understood. The aim of the present study was to assess the avidity maturation of specific antipseudomonal IgG before and during the development of chronic lung infection in a cohort of Danish CF patients. METHODS: Avidity maturation was assessed against a pooled P. aeruginosa antigen (St-Ag) and against P...
October 11, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29032177/exercise-assessment-and-training-in-cystic-fibrosis-can-less-achieve-more
#6
EDITORIAL
Narelle S Cox, Anne E Holland
No abstract text is available yet for this article.
October 11, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29029973/comparing-the-management-of-constipation-and-distal-intestinal-obstruction-syndrome-between-paediatricians-and-adult-physicians
#7
LETTER
J A Green, W D Carroll, F J Gilchrist
No abstract text is available yet for this article.
October 10, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986017/recent-progress-in-translational-cystic-fibrosis-research-using-precision-medicine-strategies
#8
Deborah M Cholon, Martina Gentzsch
Significant progress has been achieved in developing precision therapies for cystic fibrosis; however, highly effective treatments that target the ion channel, CFTR, are not yet available for many patients. As numerous CFTR therapeutics are currently in the clinical pipeline, reliable screening tools capable of predicting drug efficacy to support individualized treatment plans and translational research are essential. The utilization of bronchial, nasal, and rectal tissues from individual cystic fibrosis patients for drug testing using in vitro assays such as electrophysiological measurements of CFTR activity and evaluation of fluid movement in spheroid cultures, has advanced the prediction of patient-specific responses...
October 3, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28964647/real-life-practice-of-sweat-testing-in-europe
#9
N Cirilli, K W Southern, R Buzzetti, J Barben, L Nährlich, A Munck, M Wilschanski, K De Boeck, N Derichs
Evidence based guidelines exist for sweat testing, which remains a key component of a diagnosis of cystic fibrosis (CF), especially following newborn bloodspot screening (NBS). There are emerging challenges with respect to maintaining a valid sweat test service, notably a smaller number of sweat tests ordered in regions with established NBS programmes where Pediatricians refer less children for sweat testing, younger patients and equipment becoming obsolete. The ECFS Diagnostic Network Working Group has undertaken a comprehensive survey to better define sweat test practice across Europe...
September 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986024/cystic-fibrosis-and-gastroesophageal-reflux-disease
#10
Asim Maqbool, Ans Pauwels
Gastroesophageal reflux is common in children and adults with cystic fibrosis (CF). Pathological gastroesophageal reflux disease (GERD) is also frequent in patients of all ages with CF. This article reviews the pathophysiology, diagnostic work-up, management options, complications, and future directions in the evaluation and management of GERD - unique to and pertinent for - patients with CF in particular.
September 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986021/approach-to-chronic-abdominal-pain-in-cystic-fibrosis
#11
Sarah Shrager Lusman, Richard Grand
Abdominal pain in individuals with CF is challenging for the patient as well as the physician, as the differential diagnosis can be complex. Most gastrointestinal manifestations of CF present with regional abdominal pain. Pain localization, which requires knowledge of gut development and innervation, is crucial to understanding the pathophysiology of abdominal pain in CF. The location of the pain, together with the clinical presentation, shapes the differential diagnosis and thus guides the evaluation and management...
September 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28951068/progress-in-understanding-mucus-abnormalities-in-cystic-fibrosis-airways
#12
Jeffrey J Wine, Gunnar C Hansson, Peter König, Nam Soo Joo, Anna Ermund, Mario Pieper
Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), loss of CFTR function compromises these defences, and with present standard of care virtually all people with CF eventually develop mucus accumulation, plugging and chronic infections. This symposium focused on how mucus is affected by CFTR loss...
September 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986023/gallbladder-and-bile-duct-disease-in-cystic-fibrosis
#13
David N Assis, Dominique Debray
Cystic fibrosis (CF) is a multi-organ, clinically diverse disorder caused by mutations in the cystic fibrosis transmembrane conductance receptor (CFTR). Awareness of extra-pulmonary manifestations, including gastrointestinal and hepatobiliary disturbances, is an increasingly important part of providing high-quality care to patients with CF. Furthermore, biliary disorders, including gallbladder and bile duct disease, are common complications of CF. Therefore, a thorough understanding and efficient clinical evaluation of the gallbladder and biliary tree is an important aspect of integrated care for the patient with CF in order to prevent progression of undetected pathology...
September 20, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986019/pancreatic-insufficiency-in-cystic-fibrosis
#14
Vikesh K Singh, Sarah Jane Schwarzenberg
Pancreatic insufficiency (PI) affects about 85% of the cystic fibrosis population. Although most are PI soon after birth, some will have pancreatic sufficiency (PS) for some or all of their life. Understanding the clinical presentation, diagnosis, and management of PI is crucial to the care of people with cystic fibrosis.
September 20, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986028/pancreatitis-and-pancreatic-cystosis-in-cystic-fibrosis
#15
A Jay Freeman, Chee Y Ooi
The pathologic effects of an altered cystic fibrosis transmembrane receptor (CFTR) protein on the exocrine pancreas is ubiquitous and of varying severity. In this section, pancreatitis and pancreatic cystosis are covered.
September 19, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28939349/animal-and-model-systems-for-studying-cystic-fibrosis
#16
Bradley H Rosen, Marc Chanson, Lara R Gawenis, Jinghua Liu, Aderonke Sofoluwe, Alice Zoso, John F Engelhardt
The cystic fibrosis (CF) field is the beneficiary of five species of animal models that lack functional cystic fibrosis transmembrane conductance regulator (CFTR) channel. These models are rapidly informing mechanisms of disease pathogenesis and CFTR function regardless of how faithfully a given organ reproduces the human CF phenotype. New approaches of genetic engineering with RNA-guided nucleases are rapidly expanding both the potential types of models available and the approaches to correct the CFTR defect...
September 19, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986025/introduction
#17
EDITORIAL
Steven D Freedman, Michael Wilschanski
No abstract text is available yet for this article.
September 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986027/cystic-fibrosis-related-cirrhosis
#18
Daniel H Leung, Michael R Narkewicz
While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.
September 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986026/nutrition-prevention-and-management-of-nutritional-failure-in-cystic-fibrosis
#19
Jillian S Sullivan, Maria R Mascarenhas
Close monitoring of nutritional status is critical to the overall health of a patient with CF. As part of routine CF care, measurement of weight and height (and calculation of weight/length or BMI as appropriate) should be performed and analyzed at each visit. Early recognition of nutritional risk is imperative and evaluation with a multidisciplinary team should be performed to assess for caloric intake, caloric malabsorption, and other causes of poor weight gain and growth. Many tools are available to use for intervention, including oral supplementation, behavioral interventions, medications, nutritional therapies, and enteral tube feeding...
September 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986022/bacterial-overgrowth-dysbiosis-inflammation-and-dysmotility-in-the-cystic-fibrosis-intestine
#20
Jill Dorsey, Tanja Gonska
No abstract text is available yet for this article.
September 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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