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"Cystic fibrosis"

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https://www.readbyqxmd.com/read/27922234/long-term-azithromycin-therapy-in-patients-with-cystic-fibrosis
#1
Nagehan Emiralioğlu, Zeynelabidin Öztürk, Ebru Yalçın, Deniz Doğru, Uğur Özçelik, Nural Kiper
Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27922233/diagnosis-of-cystic-fibrosis-with-chloride-meter-sherwood-m926s-chloride-analyzer%C3%A2-and-sweat-test-analysis-system-cf%C3%AE-collection-system%C3%A2-compared-to-the-gibson-cooke-method
#2
Nagehan Emiralioğlu, Uğur Özçelik, Ebru Yalçın, Deniz Doğru, Nural Kiper
Sweat test with Gibson Cooke (GC) method is the diagnostic gold standard for cystic fibrosis (CF). Recently, alternative methods have been introduced to simplify both the collection and analysis of sweat samples. Our aim was to compare sweat chloride values obtained by GC method with other sweat test methods in patients diagnosed with CF and whose CF diagnosis had been ruled out. We wanted to determine if the other sweat test methods could reliably identify patients with CF and differentiate them from healthy subjects...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27922232/changing-epidemiology-of-non-cystic-fibrosis-bronchiectasis
#3
Semiha Bahçeci, Sait Karaman, Hikmet Tekin Nacaroğlu, Selçuk Yazıcı, Saniye Girit, Şule Ünsal-Karkıner, Demet Can
Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27921442/nebulized-antibiotics-in-mechanically-ventilated-patients-roadmap-and-challenges
#4
G Poulakou, G Siakallis, S Tsiodras, A Arfaras Melainis, G Dimopoulos
Nebulized antibiotics use has become common practice in the therapeutics of pneumonia in cystic fibrosis patients. There is an increasing interest in their use for respiratory infections in mechanically ventilated (MV) patients in order to a) overcome pharmacokinetic issues in the lung compartment with traditional systemic antibiotic use and b) prevent the emergence of multi-drug-resistant (MDR) pathogens. Areas covered: The beneficial effects of antibiotic nebulization in MV patients e.g. increasing efficacy, reduced toxicity and prevention of resistance are described...
December 6, 2016: Expert Review of Anti-infective Therapy
https://www.readbyqxmd.com/read/27921040/new-insights-into-the-molecular-mechanisms-targeting-tubular-channels-transporters-in-pkd-development
#5
REVIEW
Ming Wu, Shengqiang Yu
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD. SUMMARY: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920537/factors-related-to-depression-and-anxiety-in-adults-with-bronchiectasis
#6
Elif Yelda Özgün Niksarlioglu, Gülcihan Özkan, Gülşah Günlüoğlu, Mehmet Atilla Uysal, Sule Gül, Lütfiye Kilic, Ayse Yeter, Güngör Çamsarı
INTRODUCTION AND BACKGROUND: Patients with chronic lung diseases frequently have depressive and anxiety symptoms, but there are very few studies looking at this in patients with bronchiectasis. AIM: This study aimed to investigate depression and anxiety and related factors among patients with non-cystic fibrosis bronchiectasis. PATIENTS AND METHODS: This was a prospective study of 133 patients with bronchiectasis. Patients with confirmed diagnosis of bronchiectasis with high-resolution computed tomography were enrolled in the study...
2016: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/27919886/cardiopulmonary-exercise-testing-in-children-with-cystic-fibrosis-one-centre-s-experience
#7
Elise Weir, Paul D Burns, Anne Devenny, David Young, James Y Paton
BACKGROUND: While exercise testing is increasingly used as a prognostic indicator in cystic fibrosis (CF), it is reported to be underused in UK CF centres, particularly in children. Here, we evaluated the cardiopulmonary exercise testing (CPET) results in children and young people with CF at CF annual review and its possible clinical value. METHOD: An observational study comparing CPET results using a cycle ergometer ramp test (peak oxygen uptake (Vo2peak)) and pulmonary function (forced expiratory volume in 1 s (FEV1)) was performed with body mass index (BMI) used as a disease severity marker...
December 5, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27919570/agreement-between-multiple-breath-nitrogen-washout-systems-in-children-and-adults
#8
William Poncin, Florian Singer, Anne-Sophie Aubriot, Patrick Lebecque
BACKGROUND: Comparability of multiple breath washout (MBW) systems has been little explored. We assessed agreement in lung clearance index (LCI) from two similar, commercial nitrogen MBW setups in patients with Cystic Fibrosis (CF) and controls. METHODS: The EasyOne Pro (NDD) and Exhalyzer D (EM) were randomly applied in 85 adults (34 with CF) and 97 children (47 with CF and normal forced expiratory volume in one second). We assessed differences between setups in LCI, lung volumes and breathing pattern and diagnostic performance for detecting abnormal lung function...
December 2, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27919253/cystic-fibrosis-lung-environment-and-pseudomonas-aeruginosa-infection
#9
Anjali Y Bhagirath, Yanqi Li, Deepti Somayajula, Maryam Dadashi, Sara Badr, Kangmin Duan
BACKGROUND: The airways of patients with cystic fibrosis (CF) are highly complex, subject to various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa is recognized as one of the most important pulmonary pathogens and the predominant cause of morbidity and mortality in CF. A multifarious interplay between the host, pathogens, microbiota, and the environment shapes the course of the disease. There have been several excellent reviews detailing CF pathology, Pseudomonas and the role of environment in CF but only a few reviews connect these entities with regards to influence on the overall course of the disease...
December 5, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27917292/deep-resequencing-of-cftr-in-762-f508del-homozygotes-reveals-clusters-of-non-coding-variants-associated-with-cystic-fibrosis-disease-traits
#10
Briana Vecchio-Pagán, Scott M Blackman, Melissa Lee, Melis Atalar, Matthew J Pellicore, Rhonda G Pace, Arianna L Franca, Karen S Raraigh, Neeraj Sharma, Michael R Knowles, Garry R Cutting
Extensive phenotypic variability is commonly observed in individuals with Mendelian disorders, even among those with identical genotypes in the disease-causing gene. To determine whether variants within and surrounding CFTR contribute to phenotypic variability in cystic fibrosis (CF), we performed deep sequencing of CFTR in 762 patients homozygous for the common CF-causing variant, F508del. In phase 1, ~200 kb encompassing CFTR and extending 10 kb 5' and 5 kb 3' of the gene was sequenced in 486 F508del homozygotes selected from the extremes of sweat chloride concentration...
2016: Human Genome Variation
https://www.readbyqxmd.com/read/27916878/burkholderia-contaminans-colonization-from-contaminated-liquid-docusate-colace-in-a-immunocompetent-adult-with-legionnaire-s-disease-infection-control-implications-and-the-potential-role-of-candida-pellucosa
#11
Burke A Cunha, John Gian, Bertamaria Dieguez, Elsa Santos-Cruz, Daniela Matassa, Steve Gerson, Pat Daniels, Carlos Rosales, Rodger P Silletti
Objective:B. contaminans was cultured from respiratory secretions and liquid docusate (Colace) in a Neurosurgical Intensive Care Unit (NICU) patient with community-acquired Legionnaire's disease but not from another bottle given to the patient. Unexpectedly, C. pelliculosa was cultured from two bottles, but not the B. contaminans bottle or respiratory secretions. Methods:B. cepacia, later identified as B. contaminans, was cultured from a bottle of liquid docusate (Colace) dispensed to a non-cystic fibrosis patient...
November 30, 2016: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/27916834/role-of-cytokine-induced-glycosylation-changes-in-regulating-cell-interactions-and-cell-signaling-in-inflammatory-diseases-and-cancer
#12
REVIEW
Justine H Dewald, Florent Colomb, Marie Bobowski-Gerard, Sophie Groux-Degroote, Philippe Delannoy
Glycosylation is one of the most important modifications of proteins and lipids, and cell surface glycoconjugates are thought to play important roles in a variety of biological functions including cell-cell and cell-substrate interactions, bacterial adhesion, cell immunogenicity and cell signaling. Alterations of glycosylation are observed in number of diseases such as cancer and chronic inflammation. In that context, pro-inflammatory cytokines have been shown to modulate cell surface glycosylation by regulating the expression of glycosyltransferases involved in the biosynthesis of carbohydrate chains...
November 29, 2016: Cells
https://www.readbyqxmd.com/read/27916649/new-horizons-for-cystic-fibrosis-treatment
#13
REVIEW
Isabelle Fajac, Kris De Boeck
Cystic fibrosis is an inherited multi-system disease associated with chronic lung infection, malabsorption, salt loss syndromes, male infertility and leading to numerous comorbidities. The landscape in cystic fibrosis care has changed markedly with currently more adult patients than children in many countries. Over 2000 different mutations in theCFTRgene have been reported and the majority are extremely rare. Understanding howCFTRmutations translate to disturbed synthesis or function of the CFTR protein has opened the way to 'personalized' treatments to correct the basic defect...
December 1, 2016: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/27916551/environmental-scan-of-cystic-fibrosis-research-worldwide
#14
Janice Kelly
BACKGROUND: Cystic fibrosis (CF) is a rare fatal genetic disease, affecting 70,000 to 100,000 people worldwide [1]. Numerous countries have specific charitable organizations dedicated to CF, with many funding research to find a cure or effective control for the disease. Cystic Fibrosis Canada, the largest charity in Canada dedicated to funding research and care in CF, conducted an environmental scan of these organizations to learn and better understand their research goals and funding process...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27916550/bmi-fails-to-identify-poor-nutritional-status-in-stunted-children-with-cf
#15
Michael W Konstan, David J Pasta, Jeffrey S Wagener, Donald R VanDevanter, Wayne J Morgan
BACKGROUND: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18years. RESULTS: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA <10th percentile and 26...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27914512/moderate-intake-of-docosahexaenoic-acid-raises-plasma-and-platelet-vitamin-e-levels-in-cystic-fibrosis-patients
#16
Evelyne Véricel, Stéphane Mazur, Romain Colas, Véronique Delaup, Catherine Calzada, Philippe Reix, Isabelle Durieu, Michel Lagarde, Gabriel Bellon
Patients with cystic fibrosis have increased oxidative stress and impaired antioxidant systems. Moderate intake of docosahexaenoic acid (DHA) may favor the lowering of oxidative stress. In this randomized, double-blind, cross-over study, DHA or placebo capsules, were given daily to 10 patients, 5mg/kg for 2 weeks then 10mg/kg DHA for the next 2 weeks (or placebo). After 9 weeks of wash-out, patients took placebo or DHA capsules. Biomarkers of lipid peroxidation and vitamin E were measured at baseline, and after 2 and 4 weeks of treatment in each phase...
December 2016: Prostaglandins, Leukotrienes, and Essential Fatty Acids
https://www.readbyqxmd.com/read/27913761/best-practices-in-the-treatment-of-early-cystic-fibrosis-lung-disease
#17
REVIEW
Marijke Proesmans
For many years, management of cystic fibrosis (CF) lung disease was focused on symptomatic treatment of chronic lung infection, which is characterized by cough and sputum production, leading to progressive lung damage. With increasing survival and better knowledge of the pathogenesis of CF lung disease, it has become clear that treatment has to start very early because lung damage occurs in young patients, often before obvious symptoms appear. The arrival of new cystic fibrosis transmembrane conductance-regulator (CFTR)-correcting therapies will bring more opportunities to prevent the disease, apart from only treating chronic lung infection...
December 2, 2016: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/27913277/specific-stabilization-of-cftr-by-phosphatidylserine
#18
Ellen Hildebrandt, Netaly Khazanov, John C Kappes, Qun Dai, Hanoch Senderowitz, Ina L Urbatsch
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR, ABCC7) is a plasma membrane chloride ion channel in the ABC transporter superfamily. CFTR is a key target for cystic fibrosis drug development, and its structural elucidation would advance those efforts. However, the limited in vivo and in vitro stability of the protein, particularly its nucleotide binding domains, has made structural studies challenging. Here we demonstrate that phosphatidylserine uniquely stimulates and thermally stabilizes the ATP hydrolysis function of purified human CFTR...
November 29, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27912062/atomic-structure-of-the-cystic-fibrosis-transmembrane-conductance-regulator
#19
Zhe Zhang, Jue Chen
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from the ATP-binding cassette (ABC) transporter family. In this study, we determined the structure of zebrafish CFTR in the absence of ATP by electron cryo-microscopy to 3.7 Å resolution. Human and zebrafish CFTR share 55% sequence identity, and 42 of the 46 cystic-fibrosis-causing missense mutational sites are identical. In CFTR, we observe a large anion conduction pathway lined by numerous positively charged residues...
December 1, 2016: Cell
https://www.readbyqxmd.com/read/27911585/early-lung-disease-in-infants-and-pre-school-children-with-cystic-fibrosis-what-have-we-learnt-and-what-should-we-do-about-it
#20
Sarath C Ranganathan, Graham L Hall, Peter D Sly, Stephen M Stick, Tonia A Douglas
The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis. Pulmonary inflammation, infection and structural lung damage manifest very early in life and is prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer term clinical trajectory for individuals with CF...
December 2, 2016: American Journal of Respiratory and Critical Care Medicine
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