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Cystic Fibrosis

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https://www.readbyqxmd.com/read/28822728/variability-of-lung-clearance-index-in-clinically-stable-cystic-fibrosis-lung-disease-in-school-age-children
#1
Marcus Svedberg, Per M Gustafsson, Paul D Robinson, Monica Rosberg, Anders Lindblad
BACKGROUND: Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis (CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI variability in clinically stable CF lung disease in school age children. METHODS: Paediatric patients, aged 6 to 17years, attending the outpatient CF clinic performed Multiple Breath Nitrogen Washout (Exhalyzer® D) and spirometry every third month over a period of one year. Clinical stability was assessed by the Cystic Fibrosis Clinical Score (CFCS) at each visit...
August 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28822537/impaired-renal-function-should-not-be-a-barrier-to-transplantation-in-patients-with-cystic-fibrosis
#2
Todd C Crawford, J Trent Magruder, Joshua C Grimm, Alejandro Suarez-Pierre, Xun Zhou, Jinny S Ha, Robert S Higgins, Stephen R Broderick, Jonathan B Orens, Pali Shah, Christian A Merlo, Bo S Kim, Errol L Bush
BACKGROUND: Previous studies have demonstrated an association between pretransplantation renal dysfunction (PRD) and increased mortality after lung transplantation (LT). The purpose of this study was to determine whether PRD impacts survival after LT in patients with cystic fibrosis (CF). METHODS: We queried the United Network for Organ Sharing (UNOS) database to identify all adult (≥18 years) recipients with CF who underwent isolated LT from May 4, 2005 to December 31, 2014...
August 16, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28819878/aspergillus-bronchitis-in-patients-with-cystic-fibrosis
#3
Claudia Brandt, Jobst Roehmel, Volker Rickerts, Volker Melichar, Nadja Niemann, Carsten Schwarz
Aspergillus fumigatus frequently colonizes the airways of patients with cystic fibrosis (CF) and may cause various severe infections, such as bronchitis. Serological data, sputum dependent markers and longitudinal data of treated cases of Aspergillus bronchitis were evaluated for further description of this infection. This study, which comprises three substudies, aimed to analyze epidemiological data of Aspergillus in CF and the entity of Aspergillus bronchitis. In a first step, data of the German Cystic Fibrosis Registry were used to evaluate the frequency of Aspergillus colonization in patients with CF (n = 2599)...
August 17, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28819175/in-vivo-therapeutic-efficacy-of-frog-skin-derived-peptides-against-pseudomonas-aeruginosa-induced-pulmonary-infection
#4
Chen Chen, Maria Luisa Mangoni, Y Peter Di
Pseudomonas aeruginosa is an opportunistic and frequently drug-resistant pulmonary pathogen especially in cystic fibrosis sufferers. Recently, the frog skin-derived antimicrobial peptide (AMP) Esc(1-21) and its diastereomer Esc(1-21)-1c were found to possess potent in vitro antipseudomonal activity. Here, they were first shown to preserve the barrier integrity of airway epithelial cells better than the human AMP LL-37. Furthermore, Esc(1-21)-1c was more efficacious than Esc(1-21) and LL-37 in protecting host from pulmonary bacterial infection after a single intra-tracheal instillation at a very low dosage of 0...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28819167/artificial-dnaj-protein-for-protein-production-and-conformational-diseases
#5
Akinori Hishiya, Keizo Koya
For secreted proteins, proper protein folding is essential not only for biological function but also for secretion itself. Proteins with folding problems are trapped in the endoplasmic reticulum (ER) and are eventually degraded in the cytoplasm. In this study, we exploited co-expression of an artificial fusion protein, based on the sequence of a DnaJ protein, which could interact as co-chaperones in the Hsp70-based protein-folding system, with target recombinant secreted proteins to enhance their production and secretion...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28816423/infants-with-cystic-fibrosis-still-lag-on-some-growth-measures-babies-weight-on-par-with-healthy-peers-but-growth-is-still-stunted
#6
(no author information available yet)
No abstract text is available yet for this article.
September 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28816015/liver-transplantation-in-adult-cystic-fibrosis-clinical-imaging-and-pathological-evidence-of-obliterative-portal-venopathy
#7
Sophie Hillaire, Dominique Cazals-Hatem, Onorina Bruno, Sandra De Miranda, Dominique Grenet, Nicolas Poté, Olivier Soubrane, Serge Erlinger, Florence Lacaille, Francois Mellot, Valerie Vilgrain, Valerie Paradis
No abstract text is available yet for this article.
August 16, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28815999/incident-stenotrophomonas-maltophilia-infection-and-lung-function-decline-in-cystic-fibrosis
#8
Emily E Barsky, Kathryn A Williams, Gregory P Priebe, Gregory S Sawicki
OBJECTIVES: To determine whether incident detection of Stenotrophomonas maltophilia (SM) in patients with cystic fibrosis (CF) is associated with accelerated lung function decline and increased hospitalizations and to determine whether this effect is more pronounced in individuals with subsequent chronic infection. METHODS: We performed a longitudinal, retrospective single-center, pre-post study of 88 patients with CF, ages 6-51 years, with first positive respiratory culture for SM between 2008 and 2014...
August 16, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28815937/the-airway-microbiota-in-early-cystic-fibrosis-lung-disease
#9
Katherine B Frayman, David S Armstrong, Keith Grimwood, Sarath C Ranganathan
Infection plays a critical role in the pathogenesis of cystic fibrosis (CF) lung disease. Over the past two decades, the application of molecular and extended culture-based techniques to microbial analysis has changed our understanding of the lungs in both health and disease. CF lung disease is a polymicrobial disorder, with obligate and facultative anaerobes recovered alongside traditional pathogens in varying proportions, with some differences observed to correlate with disease stage. While healthy lungs are not sterile, differences between the lower airway microbiota of individuals with CF and disease-controls are already apparent in childhood...
August 16, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28815197/autosomal-recessive-inheritance-cystic-fibrosis
#10
D Yitzchak Goldstein, Michael Prystowsky
The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.
January 2017: Acad Pathol
https://www.readbyqxmd.com/read/28815024/stem-cell-derived-organoids-to-model-gastrointestinal-facets-of-cystic-fibrosis
#11
REVIEW
Meike Hohwieler, Lukas Perkhofer, Stefan Liebau, Thomas Seufferlein, Martin Müller, Anett Illing, Alexander Kleger
Cystic fibrosis (CF) is one of the most frequently occurring inherited human diseases caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) which lead to ample defects in anion transport and epithelial fluid secretion. Existing models lack both access to early stages of CF development and a coeval focus on the gastrointestinal CF phenotypes, which become increasingly important due increased life span of the affected individuals. Here, we provide a comprehensive overview of gastrointestinal facets of CF and the opportunity to model these in various systems in an attempt to understand and treat CF...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28814498/both-ways-at-once-keeping-small-airways-clean
#12
REVIEW
Paul M Quinton
The small airways of the lungs are under constant assault from the pathogens and debris in the air that they must conduct to alveoli. Although hygiene is of paramount importance for respiratory health, the underlying principles of airway clearance have not been well integrated or established. Newly emerging concepts of simultaneous absorption and secretion of airway surface liquid (ASL) and the role of [Formula: see text] in the maturation of mucins have advanced from experimental evidence as well as observations from the congenital disease cystic fibrosis (CF) to present a novel model that integrates microanatomy with organ physiology to meet the constant challenge of cleaning small airways...
September 2017: Physiology
https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#13
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28814218/diagnosis-and-management-of-pancreatic-exocrine-insufficiency
#14
Mehrdad Nikfarjam, Jeremy S Wilson, Ross C Smith
In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic stimulation; synthesis, release or transportation of pancreatic enzymes; or synchronisation of secretions to mix with ingested food...
August 21, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28812224/risk-factors-for-totally-implantable-venous-access-device-associated-complications-in-cystic-fibrosis
#15
C McCarthy, O O'Carroll, M E O'Brien, T McEnery, A Franciosi, C Gunaratnam, N G McElvaney
BACKGROUND: Candidaemia is an important nosocomial infection, seen frequently in immunocompromised and critically ill patients and increasingly recognised in cystic fibrosis (CF) patients with totally implantable venous access devices (TIVADs). This study aims to investigate the incidence and risk factors for the development of TIVAD-associated candidaemia and to assess the rate of TIVAD-related complications in CF patients. METHODS: A 10-year retrospective study was carried out on adult CF patients attending a single centre...
August 15, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28811770/clinical-and-laboratory-profile-of-children-with-cystic-fibrosis-experience-of-a-tertiary-care-center-in-pakistan
#16
Danish Abdul Aziz, Abdul Gaffar Billoo, Ahad Qureshi, Misha Khalid, Salman Kirmani
OBJECTIVE: To determine the clinical presentation, diagnostic investigations and laboratory workup done in admitted children with cystic fibrosis at Aga Khan University Hospital Karachi, Pakistan. METHODS: This is athree years retrospective study from January 2013 to December 2015 conducted at The Aga Khan University Hospital Karachi Pakistan, enrolling admitted patient from birth to 15 years of either gender, diagnosed with CF on the basis of clinical features and positive sweat chloride test...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28811674/regenerative-medicine-and-cell-based-approaches-to-restore-pancreatic-function
#17
REVIEW
Cara Ellis, Adam Ramzy, Timothy J Kieffer
The pancreas is a complex organ with exocrine and endocrine components. Many pathologies impair exocrine function, including chronic pancreatitis, cystic fibrosis and pancreatic ductal adenocarcinoma. Conversely, when the endocrine pancreas fails to secrete sufficient insulin, patients develop diabetes mellitus. Pathology in either the endocrine or exocrine pancreas results in devastating economic and personal consequences. The current standard therapy for treating patients with type 1 diabetes mellitus is daily exogenous insulin injections, but cell sources of insulin provide superior glycaemic regulation and research is now focused on the goal of regenerating or replacing β cells...
August 16, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28811149/three-center-feasibility-of-lung-clearance-index-in-infants-and-preschool-children-with-cystic-fibrosis-and-other-lung-diseases
#18
Mirjam Stahl, Simon Y Graeber, Cornelia Joachim, Sandra Barth, Isabell Ricklefs, Gesa Diekmann, Matthias V Kopp, Lutz Naehrlich, Marcus A Mall
BACKGROUND: Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting. METHODS: Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2...
August 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28809446/deferasirox-for-managing-iron-overload-in-people-with-thalassaemia
#19
REVIEW
Claudia Bollig, Lisa K Schell, Gerta Rücker, Roman Allert, Edith Motschall, Charlotte M Niemeyer, Dirk Bassler, Joerg J Meerpohl
BACKGROUND: Thalassaemia is a hereditary anaemia due to ineffective erythropoiesis. In particular, people with thalassaemia major develop secondary iron overload resulting from regular red blood cell transfusions. Iron chelation therapy is needed to prevent long-term complications.Both deferoxamine and deferiprone are effective; however, a review of the effectiveness and safety of the newer oral chelator deferasirox in people with thalassaemia is needed. OBJECTIVES: To assess the effectiveness and safety of oral deferasirox in people with thalassaemia and iron overload...
August 15, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28808008/mathematical-model-reveals-role-of-nucleotide-signaling-in-airway-surface-liquid-homeostasis-and-its-dysregulation-in-cystic-fibrosis
#20
Conner I Sandefur, Richard C Boucher, Timothy C Elston
Mucociliary clearance is composed of three components (i.e., mucin secretion, airway surface hydration, and ciliary-activity) which function coordinately to clear inhaled microbes and other foreign particles from airway surfaces. Airway surface hydration is maintained by water fluxes driven predominantly by active chloride and sodium ion transport. The ion channels that mediate electrogenic ion transport are regulated by extracellular purinergic signals that signal through G protein-coupled receptors. These purinoreceptors and the signaling pathways they activate have been identified as possible therapeutic targets for treating lung disease...
August 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
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