50,247 results match your criteria Cystic Fibrosis


Successful ceftazidime-avibactam treatment of post-surgery Burkholderia multivorans genomovar II bacteremia and brain abscesses in a young lung transplanted woman with cystic fibrosis.

Transpl Infect Dis 2019 Mar 20:e13082. Epub 2019 Mar 20.

Cystic Fibrosis Center, IRCCS Ca' Granda Foundation Milan, Italy.

Burkholderia cepacia complex (Bcc) includes several phenotypically similar but genotypically distinct gram-negative bacteria (GNB) that can colonize the respiratory tract of Cystic Fibrosis (CF) patients. Pathogens are difficult to treat due to intrinsic resistance to multiple antibiotics and are associated to a more rapid decline in lung function and to increased mortality, particularly after lung transplantation. For all these reasons chronic infection by Burkholderia (B) cenocepacia is presently considered a relative or absolute contraindication in almost all lung transplant centres. Read More

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http://dx.doi.org/10.1111/tid.13082DOI Listing

Infection prevention and control in cystic fibrosis: a systematic review of interventions.

Expert Rev Respir Med 2019 Mar 20. Epub 2019 Mar 20.

a Evidence Based Child Health Group, Division of Child Health , Obstetrics & Gynaecology, Queens Medical Centre , E Floor East Block, Nottingham NG7 2UH , UK.

Introduction: Cystic fibrosis is a life-limiting genetic condition characterized by recurrent pulmonary infection. Acquisition of infection can occur from environmental reservoirs, person-to-person transmission and from the healthcare environment. Primary prevention of infections through infection prevention and control measures is an important strategy in cystic fibrosis care. Read More

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http://dx.doi.org/10.1080/17476348.2019.1595594DOI Listing

Acetyl-CoA carboxylase inhibition regulates microtubule dynamics and intracellular transport in cystic fibrosis epithelial cells.

Am J Physiol Lung Cell Mol Physiol 2019 Mar 20. Epub 2019 Mar 20.

Pediatrics, Case Western Reserve University, United States.

The use of high-dose ibuprofen as an anti-inflammatory therapy in cystic fibrosis has been shown to be an effective intervention though use is limited due to potential adverse events. Identifying the mechanism of ibuprofen efficacy would aid in the development of new therapies that avoid these adverse events. Previous findings demonstrated that ibuprofen treatment restores the regulation of microtubule dynamics in cystic fibrosis (CF) epithelial cells through a 5' adenosine monophosphate-activated protein kinase (AMPK)-dependent mechanism. Read More

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http://dx.doi.org/10.1152/ajplung.00369.2018DOI Listing

in cystic fibrosis: A chronic cheater.

Proc Natl Acad Sci U S A 2019 Mar 19. Epub 2019 Mar 19.

Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322

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http://dx.doi.org/10.1073/pnas.1902734116DOI Listing

Persistent Adherence to Airway Clearance Therapy in Adults With Cystic Fibrosis.

Respir Care 2019 Mar 19. Epub 2019 Mar 19.

Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

Background: Airway clearance therapy (ACT) is a core component of daily treatment for cystic fibrosis (CF). However, surprisingly little is known about sustained or persistent use of ACT over time among adults with CF. This longitudinal study examined persistent adherence to ACT over 12 months and its modifiable predictors, drawing on aspects of Social Cognitive Theory and the Theory of Planned Behavior. Read More

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http://dx.doi.org/10.4187/respcare.06500DOI Listing

Higher Levels of Education Are Associated With Full-Time Work in Adults With Cystic Fibrosis.

Respir Care 2019 Mar 19. Epub 2019 Mar 19.

School of Physiotherapy and Exercise Science, Faculty of Health Sciences, Curtin University, Perth, Western Australia.

Background: This study sought to explore factors in adults with cystic fibrosis (CF) that predicted whether (i) someone was engaged in full-time paid work, and (ii) those engaged in paid work reported problems with absenteeism and/or presenteeism.

Methods: Adults with cystic fibrosis who live in Western Australia completed absenteeism and presenteeism questions from the World Health Organization's Health Performance Questionnaire. The participants were grouped by work status (full time vs part time or unemployed) and by self-reported absenteeism and presenteeism (evidence of vs no evidence of). Read More

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http://dx.doi.org/10.4187/respcare.06607DOI Listing

Choline Supplementation in Cystic Fibrosis-The Metabolic and Clinical Impact.

Nutrients 2019 Mar 18;11(3). Epub 2019 Mar 18.

Department of General Pediatrics, Hematology and Oncology, Children's Hospital, Eberhard-Karls-University, 72076 Tübingen, Germany.

Background: Choline is essential for the synthesis of liver phosphatidylcholine (PC), parenchymal maintenance, bile formation, and lipoprotein assembly to secrete triglycerides. In choline deficiency, the liver accretes choline/PC at the expense of lung tissue, thereby impairing pulmonary PC homoeostasis. In cystic fibrosis (CF), exocrine pancreas insufficiency results in impaired cleavage of bile PC and subsequent fecal choline loss. Read More

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https://www.mdpi.com/2072-6643/11/3/656
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http://dx.doi.org/10.3390/nu11030656DOI Listing
March 2019
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Higher Plasma Endothelial Markers in Adults with Cystic Fibrosis Compared to Healthy Age Matched Controls.

Ann Am Thorac Soc 2019 Mar 19. Epub 2019 Mar 19.

University of Washington, Medicine / Pulmonary and Critical Care, Seattle, Washington, United States.

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http://dx.doi.org/10.1513/AnnalsATS.201812-844RLDOI Listing

Correlating CFTR Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis.

Am J Respir Crit Care Med 2019 Mar 19. Epub 2019 Mar 19.

Johns Hopkins School of Medicine, 1500, McKusick-Nathans Institute of Genetic Medicine, Baltimore, Maryland, United States ;

Rationale: The advent of precision treatment for cystic fibrosis (CF) using small-molecule therapeutics has created a need to estimate potential clinical improvements attributable to increases in cystic fibrosis transmembrane conductance regulator (CFTR) function.

Objective: Derive CFTR function of a variety of CFTR genotypes and correlate with key clinical features (sweat chloride concentration, pancreatic exocrine status, and lung function) to develop benchmarks for assessing response to CFTR modulators.

Methods: CFTR function assigned to 226 unique CFTR genotypes was correlated with the clinical data of 54,671 individuals enrolled in the Clinical and Functional TRanslation of CFTR (CFTR2) project. Read More

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http://dx.doi.org/10.1164/rccm.201901-0145OCDOI Listing

Exophiala dermatitidis: key issues of an opportunistic fungal pathogen.

Virulence 2019 Mar 19. Epub 2019 Mar 19.

a Institute of Medical Microbiology, Center of Excellence in Clinical and Laboratory Mycology and Clinical Studies , University Hospital Essen, University of Duisburg-Essen , Essen , Germany.

The black yeast Exophiala dermatitidis is an opportunistic pathogen, causing phaeohyphomycosis in immunosuppressed patients, chromoblastomycosis and fatal infections of the central nervous system in otherwise healthy Asian patients. In addition, it is also regularly isolated from respiratory samples from cystic fibrosis patients, with rates varying between 1 and 19 %. Melanin, as part of the cell wall of black yeasts, is one major factor known contributing to pathogenicity of E. Read More

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http://dx.doi.org/10.1080/21505594.2019.1596504DOI Listing

Linkage of the CF foundation patient registry with the pediatric health information system database.

Pediatr Pulmonol 2019 Mar 18. Epub 2019 Mar 18.

Cystic Fibrosis Foundation, Bethesda, Maryland.

Introduction: The Cystic Fibrosis Foundation Patient Registry (CFFPR) contains clinical and demographic data from ∼85% of US cystic fibrosis (CF) patients across 120 care centers, but lacks robust inpatient hospitalization data. In contrast, the Pediatric Health Information System (PHIS) database includes inpatient clinical and resource utilization data from 49 US children's hospitals. The creation of a linked CFFPR-PHIS dataset can uniquely address questions related to in-hospital pediatric CF treatment and management. Read More

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http://dx.doi.org/10.1002/ppul.24272DOI Listing

Identifying pediatric lung disease: A comparison of forced oscillation technique outcomes.

Pediatr Pulmonol 2019 Mar 18. Epub 2019 Mar 18.

Telethon Kids Institute, Nedlands, Western Australia, Australia.

Rationale: Increasing evidence suggests the forced oscillation technique (FOT) has the capacity to provide non-invasive monitoring and diagnosis of respiratory disease in young children. However, which FOT outcomes provide the most pertinent clinical information is currently unknown. The aim of this study was to determine which FOT outcomes were most sensitive for differentiating between health and specific childhood respiratory disease. Read More

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http://dx.doi.org/10.1002/ppul.24286DOI Listing
March 2019
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Validity and Reliability of a Novel Multimodal Questionnaire for the Assessment of Abdominal Symptoms in People with Cystic Fibrosis (CFAbd-Score).

Patient 2019 Mar 18. Epub 2019 Mar 18.

Cystic Fibrosis Center for Children and Adults, Jena University Hospital, Am Klinikum 1, 07747, Jena, Germany.

Background And Objective: For people with cystic fibrosis, validated patient-reported outcome measures for the assessment of the complex abdominal involvement are lacking. The objective of this study was to examine whether the CFAbd-Score, a novel questionnaire consisting of 28 items, meets the essential requirements (validity and reliability) for a patient-reported outcome measure according to US Food and Drug Administration recommendations.

Methods: Content validity was assessed by recording the frequencies and severity of symptoms that occurred during the prior 2 weeks in patients with cystic fibrosis (n = 116; aged ≥ 6 years). Read More

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http://dx.doi.org/10.1007/s40271-019-00361-2DOI Listing

Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis.

Lung 2019 Mar 18. Epub 2019 Mar 18.

Pediatric Pulmonology and National CF Center, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.

Introduction: There is a strong association between cystic fibrosis and malnutrition, mainly because of the higher energy needs combined with lower intake. There is also a well-established correlation between good nutritional status and better lung function. To date, however, there are no studies examining nutritional status in childhood and adult lung function. Read More

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http://dx.doi.org/10.1007/s00408-019-00218-3DOI Listing

The Yin and Yang of Lung Infections in Cystic Fibrosis: A Model for Studying Polymicrobial Interactions.

J Bacteriol 2019 Mar 18. Epub 2019 Mar 18.

Department of Microbiology and Immunology, Geisel School of Medicine at Dartmouth, Hanover, NH 03733

The streptococci are increasingly recognized as a core component of the cystic fibrosis (CF) lung microbiome, yet the role that they play in CF lung disease is unclear. The presence of group (SMG, also known as the group streptococci, AGS) correlates with exacerbation when these microbes are the predominant species in the lung. In contrast, microbiome studies have indicated that increased relative abundance of streptococci in the lung, including members of the oral microflora, correlates with less severe impacts on lung disease compared to other CF-associated microflora, indicating a complex role for this genus in the context of CF. Read More

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http://dx.doi.org/10.1128/JB.00115-19DOI Listing

Diagnostic accuracy of MRI with MRCP and B-Mode-sonography with elastography of the pancreas in patients with cystic fibrosis: a point-to-point comparison.

BMC Res Notes 2019 Mar 18;12(1):150. Epub 2019 Mar 18.

Department of Internal Medicine I, University Hospital Ulm, Albert-Einstein-Allee 23, 89081, Ulm, Germany.

Objective: For patients with cystic fibrosis, the imaging of the pancreas is of crucial importance for the early detection of pancreatic carcinoma. Comparative studies between Magnetic Resonance Imaging (MRI) and sonographic pancreas sonography are not yet available. The aim of the study was to compare MRI, sonography and point-shearwave elastography (pSWE). Read More

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http://dx.doi.org/10.1186/s13104-019-4193-4DOI Listing

Computed Tomography Description of the Uncinate Process Angulation in Patients With Cystic Fibrosis and Comparison With Primary Ciliary Dyskinesia, Nasal Polyposis, and Controls.

Ear Nose Throat J 2019 Feb 7;98(2):89-93. Epub 2019 Feb 7.

1 Ear Nose and Throat Department and Cystic Fibrosis Centre, Intercommunal Hospital of Créteil, Paris, France.

Background:: There is a medial bulging of the lateral nasal wall in patients with cystic fibrosis (CF).

Aims:: Uncinate process (UP) angulation measurements in patients and controls to objectify this bulging.

Materials And Methods:: Thirty CF, 17 primary ciliary dyskinesia (PCD), 13 chronic rhinosinusitis with polyps (CRSwp), and 30 controls were included. Read More

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http://dx.doi.org/10.1177/0145561319828645DOI Listing
February 2019

Aerosolized agents for airway clearance in cystic fibrosis.

Pediatr Pulmonol 2019 Mar 18. Epub 2019 Mar 18.

Department of Respiratory and Sleep Medicine, Royal Children's Hospital, Melbourne, Victoria, Australia.

The outlook for people with cystic fibrosis (CF) has improved considerably as a result of conventional therapies including aerosolized agents for airway clearance. These will continue to play a significant role in maintaining well-being and improving survival, even as newer agents emerge that correct the underlying CF defect. In this review, we explore the evidence supporting the use of dornase alfa, hypertonic saline, and mannitol in improving mucus clearance in patients with CF from different age groups with differing disease severity. Read More

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http://dx.doi.org/10.1002/ppul.24306DOI Listing

Pulmonary Mycobacterium abscessus complex in children with cystic fibrosis: A practical management guideline.

J Paediatr Child Health 2019 Mar 18. Epub 2019 Mar 18.

Infectious Diseases Unit, Department of General Medicine, Royal Children's Hospital Melbourne, Melbourne, Victoria, Australia.

The treatment of Mycobacterium abscessus complex (MABSC) pulmonary infections is an emerging challenge in patients with cystic fibrosis (CF). Multidrug therapy for prolonged durations is required and carries the significant burden of drug-related toxicity, cost and selective pressure for multiresistant bacteria. International guidelines acknowledge that clinical and in vitro data to support treatment regimens are limited, particularly in children. Read More

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http://dx.doi.org/10.1111/jpc.14427DOI Listing

Hydroxyurea (hydroxycarbamide) for transfusion-dependent β-thalassaemia.

Cochrane Database Syst Rev 2019 Mar 16;3:CD012064. Epub 2019 Mar 16.

Department of Pediatric Hematology and Oncology, National Institute of Blood Disease & Bone Marrow Transplant, ST 2/A Block 17 Gulshan-e-Iqbal, KDA Scheme 24, Karachi, Sindh, Pakistan, 75300.

Background: Hydroxyurea (hydroxycarbamide) promotes the production of foetal haemoglobin (HbF) by reactivating gamma-genes. Evidence has shown clinical benefits of hydroxyurea in people with sickle cell anemia; however, only a few studies have assessed this treatment in people with beta (β)-thalassaemia.

Objectives: The primary objective is to review the efficacy of hydroxyurea in reducing or ameliorating the requirement of blood transfusions in people with transfusion-dependent β-thalassaemia. Read More

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http://dx.doi.org/10.1002/14651858.CD012064.pub2DOI Listing
March 2019
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Nebulized Amikacin and Fosfomycin for Severe Pseudomonas aeruginosa Pneumonia: An Experimental Study.

Crit Care Med 2019 Mar 15. Epub 2019 Mar 15.

Department of Pulmonary and Critical Care Medicine, Hospital Clinic, Barcelona, Spain.

Objectives: Latest trials failed to confirm merits of nebulized amikacin for critically ill patients with nosocomial pneumonia. We studied various nebulized and IV antibiotic regimens in a porcine model of severe Pseudomonas aeruginosa pneumonia, resistant to amikacin, fosfomycin, and susceptible to meropenem.

Design: Prospective randomized animal study. Read More

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http://dx.doi.org/10.1097/CCM.0000000000003724DOI Listing
March 2019
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Aerosol Transmission of Aspergillus fumigatus in Cystic Fibrosis Patients in the Netherlands.

Emerg Infect Dis 2019 Apr;25(4):797-799

We collected sputum samples and cough plates from 15 cystic fibrosis patients in the Netherlands who were colonized with Aspergillus fumigatus; we recovered A. fumigatus of the same genotype in cough aerosols and sputum samples from 2 patients. The belief that transmission of A. Read More

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http://dx.doi.org/10.3201/eid2504.181110DOI Listing

Accuracy of Transient Elastography Data Combined With APRI in Detection and Staging of Liver Disease in Pediatric Patients With Cystic Fibrosis.

Clin Gastroenterol Hepatol 2019 Mar 14. Epub 2019 Mar 14.

Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, Brisbane, Australia; Faculty of Medicine, The University of Queensland, Brisbane, Australia. Electronic address:

Background & Aims: Liver disease develops in 15%-72% of patients with cystic fibrosis, and 5%-10% develop cirrhosis or portal hypertension, usually during childhood. Transient elastography (TE) is a noninvasive method to measure liver stiffness. We aimed to validate its accuracy in detection of liver disease and assessment of fibrosis in children with cystic fibrosis. Read More

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http://dx.doi.org/10.1016/j.cgh.2019.03.015DOI Listing

Effects of Lumacaftor/Ivacaftor on physical activity and exercise tolerance in three adults with cystic fibrosis.

J Cyst Fibros 2019 Mar 14. Epub 2019 Mar 14.

Department of Public Health and Infectious Diseases, Adult Cystic Fibrosis Center, Sapienza University of Rome, 00185 Rome, Italy; Eleonora Lorrillard-Spencer Cenci Foundation, 00185 Rome, Italy. Electronic address:

The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.001DOI Listing

Caroli Disease Revisited: A Case of a Kidney Transplant Patient With Autosomal Polycystic Kidney Disease and Recurrent Episodes of Cholangitis.

Transplant Proc 2019 Mar 3;51(2):541-544. Epub 2019 Jan 3.

Division of Nephrology and Transplantation, Department of Medicine, University of Vermont Medical Center and Larner College of Medicine, Burlington, Vermont, USA. Electronic address:

Polycystic kidney disease (PKD) is a genetic disorder leading to end-stage renal disease more commonly in the fourth to sixth decades of life. Cyst formation in the kidneys and other organs such as the liver and pancreas is the main characteristic of this disease. A significant number of patients with PKD undergo kidney transplantation and receive significant immunosuppression, predisposing them to comorbidities such as infections and malignancies. Read More

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http://dx.doi.org/10.1016/j.transproceed.2018.12.025DOI Listing

Challenges and safety of beta lactam desensitization during extracorporeal membrane oxygenation.

Ann Allergy Asthma Immunol 2019 Mar 13. Epub 2019 Mar 13.

Brigham and Women's Hospital, Boston, Massachusetts; Drug Hypersensitivity and Desensitization Center at Brigham and Women's Hospital.

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http://dx.doi.org/10.1016/j.anai.2019.03.005DOI Listing

Posaconazole therapy in children with cystic fibrosis and Aspergillus-related lung disease.

Med Mycol 2019 Mar 16. Epub 2019 Mar 16.

Department of Respiratory Sciences, NIHR Biomedical Research Centre (Respiratory theme) and Institute for Lung Health, University of Leicester, Leicester, United Kingdom.

There is emerging evidence for the role of posaconazole in the management of Aspergillus-related cystic fibrosis (CF) lung disease. The tolerability and efficacy of posaconazole in paediatric CF is not well established. We report a prospective study over a fifty-three month period evaluating the safety, tolerability, and efficacy of posaconazole in pediatric CF. Read More

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http://dx.doi.org/10.1093/mmy/myz015DOI Listing
March 2019
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Biochemistry of very-long-chain and long-chain ceramides in cystic fibrosis and other diseases: The importance of side chain.

Prog Lipid Res 2019 Mar 12. Epub 2019 Mar 12.

Department of Human Genetics, McGill University, Montreal, QC, Canada; Institute of Molecular and Translational Medicine, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic; Department of Medicine, Division of Experimental Medicine, McGill University, Montreal, QC, Canada. Electronic address:

Ceramides, the principal building blocks of all sphingolipids, have attracted the attention of many scientists around the world interested in developing treatments for cystic fibrosis, the most common genetic disease of Caucasians. Many years of fruitful research in this field have produced some fundamentally important, yet controversial results. Here, we aimed to summarize the current knowledge on the role of long- and very-long- chain ceramides, the most abundant species of ceramides in animal cells, in cystic fibrosis and other diseases. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01637827193001
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http://dx.doi.org/10.1016/j.plipres.2019.03.001DOI Listing
March 2019
2 Reads

Lentiviral Vectors for the Treatment and Prevention of Cystic Fibrosis Lung Disease.

Genes (Basel) 2019 Mar 14;10(3). Epub 2019 Mar 14.

Stead Family Department of Pediatrics, The University of Iowa, Iowa City, IA 52242, USA.

Despite the continued development of cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs for the treatment of cystic fibrosis (CF), the need for mutation agnostic treatments remains. In a sub-group of CF individuals with mutations that may not respond to modulators, such as those with nonsense mutations, CFTR gene transfer to airway epithelia offers the potential for an effective treatment. Lentiviral vectors are well-suited for this purpose because they transduce nondividing cells, and provide long-term transgene expression. Read More

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http://dx.doi.org/10.3390/genes10030218DOI Listing
March 2019
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Mid-Term Reproducibility of Chest MRI in Adults with Clinically Stable Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

Am J Respir Crit Care Med 2019 Mar 15. Epub 2019 Mar 15.

Thoraxklinik at University Hospital Heidelberg, Diagnostic and Interventional Radiology with Nuclear Medicine, Heidelberg, Germany.

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https://www.atsjournals.org/doi/10.1164/rccm.201812-2356LE
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http://dx.doi.org/10.1164/rccm.201812-2356LEDOI Listing
March 2019
3 Reads

Shifting Landscape of Airway Infection in Early Cystic Fibrosis.

Am J Respir Crit Care Med 2019 Mar 15. Epub 2019 Mar 15.

University of Colorado School of Medicine, Pediatrics, Aurora, Colorado, United States.

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http://dx.doi.org/10.1164/rccm.201903-0529EDDOI Listing

Autophagy suppresses the pathogenic immune response to dietary antigens in cystic fibrosis.

Cell Death Dis 2019 Mar 15;10(4):258. Epub 2019 Mar 15.

European Institute for Research in Cystic Fibrosis, San Raffaele Scientific Institute, Milan, Italy.

Under physiological conditions, a finely tuned system of cellular adaptation allows the intestinal mucosa to maintain the gut barrier function while avoiding excessive immune responses to non-self-antigens from dietary origin or from commensal microbes. This homeostatic function is compromised in cystic fibrosis (CF) due to loss-of-function mutations in the CF transmembrane conductance regulator (CFTR). Recently, we reported that mice bearing defective CFTR are abnormally susceptible to a celiac disease-like enteropathy, in thus far that oral challenge with the gluten derivative gliadin elicits an inflammatory response. Read More

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http://dx.doi.org/10.1038/s41419-019-1500-xDOI Listing
March 2019
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The Effect of Oral Azithromycin on the Response to Pulmonary Exacerbations Treated with Intravenous Tobramycin in Children with Cystic Fibrosis.

Ann Am Thorac Soc 2019 Mar 15. Epub 2019 Mar 15.

University of Toronto/ Hospital for Sick Children, Infectious Disease, Toronto, Ontario, Canada ;

Rationale: Intravenous (IV) tobramycin is frequently used to treat pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF), but there is concern that azithromycin may interact with tobramycin making it less effective against Pseudomonas aeruginosa.

Objective: The objective of this study was to determine whether oral azithromycin use was associated with worse lung function response to intravenous tobramycin treatment for PExs in a cohort of pediatric CF patients with chronic P. aeruginosa infection. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201811-774OCDOI Listing
March 2019
1 Read

Slc26 Family of Anion Transporters in the Gastrointestinal Tract: Expression, Function, Regulation, and Role in Disease.

Compr Physiol 2019 Mar 15;9(2):839-872. Epub 2019 Mar 15.

Hannover Medical School, Department of Gastroenterology, Hannover, Germany.

SLC26 family members are multifunctional transporters of small anions, including Cl , HCO , sulfate, oxalate, and formate. Most SLC26 isoforms act as secondary (coupled) anion transporters, while others mediate uncoupled electrogenic transport resembling Cl channels. Of the 11 described SLC26 isoforms, the SLC26A1,2,3,6,7,9,11 are expressed in the gastrointestinal tract, where they participate in salt and water transport, surface pH-microclimate regulation, affect the microbiome composition, the absorption, and secretion of oxalate and sulfate, and other functions that require further study. Read More

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http://dx.doi.org/10.1002/cphy.c180027DOI Listing
March 2019
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Emerging Therapeutic Approaches for Cystic Fibrosis. From Gene Editing to Personalized Medicine.

Front Pharmacol 2019 27;10:121. Epub 2019 Feb 27.

INSERM U 1151, Institut Necker Enfants Malades, Université Paris Descartes, Paris, France.

An improved understanding of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein structure and the consequences of gene mutations have allowed the development of novel therapies targeting specific defects underlying CF. Some strategies are mutation specific and have already reached clinical development; some strategies include a read-through of the specific premature termination codons (read-through therapies, nonsense mediated decay pathway inhibitors for Class I mutations); correction of CFTR folding and trafficking to the apical plasma membrane (correctors for Class II mutations); and an increase in the function of CFTR channel (potentiators therapy for Class III mutations and any mutant with a residual function located at the membrane). Other therapies that are in preclinical development are not mutation specific and include gene therapy to edit the genome and stem cell therapy to repair the airway tissue. Read More

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http://dx.doi.org/10.3389/fphar.2019.00121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6400831PMC
February 2019
1 Read

Increased salt intake does not worsen the progression of renal cystic disease in high water-loaded PCK rats.

PLoS One 2019 14;14(3):e0207461. Epub 2019 Mar 14.

Department of Clinical Nutrition, Faculty of Health Science, Suzuka University of Medical Science, Suzuka, Mie, Japan.

The anti-diuretic hormone arginine vasopressin is thought to be a detrimental factor in polycystic kidney disease (PKD). We previously reported that high water intake (HWI) reduced urine osmolality and urinary arginine vasopressin, improved renal function, and reduced the kidney/body weight ratio in PCK rats, an orthologous model of human PKD. In PKD patients, however, it is reported that HWI increases total kidney volume, urine volume, and urine sodium excretion, which could be a consequence of high salt intake. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0207461PLOS
March 2019
2 Reads

Cystic fibrosis transmembrane conductance regulator modulators: Present and future in cystic fibrosis treatment. A review.

Arch Argent Pediatr 2019 Apr;117(2):e131-e136

Universidad Nacional de Colombia, Departamento de Pediatría, Bogotá DC, Colombia.

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are the present and future of drug management for patients with cystic fibrosis. The objective of this article is to review this therapeutic option. Scientific articles were reviewed by searching the MedLine database, which is available through the Cystic Fibrosis Foundation's official website, from 2009 to 2018, in English. Read More

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http://dx.doi.org/10.5546/aap.2019.eng.e131DOI Listing

Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia.

Nature 2019 Mar 13;567(7748):405-408. Epub 2019 Mar 13.

Department of Biochemistry, University of Illinois at Urbana-Champaign, Urbana, IL, USA.

Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) compromise epithelial HCO and Cl secretion, reduce airway surface liquid pH, and impair respiratory host defences in people with cystic fibrosis. Here we report that apical addition of amphotericin B, a small molecule that forms unselective ion channels, restored HCO secretion and increased airway surface liquid pH in cultured airway epithelia from people with cystic fibrosis. These effects required the basolateral Na, K-ATPase, indicating that apical amphotericin B channels functionally interfaced with this driver of anion secretion. Read More

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http://www.nature.com/articles/s41586-019-1018-5
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http://dx.doi.org/10.1038/s41586-019-1018-5DOI Listing
March 2019
2 Reads

A Practical Review of Proteasome Pharmacology.

Pharmacol Rev 2019 Apr;71(2):170-197

Department of Biochemistry, West Virginia University School of Medicine, Morgantown, West Virginia

The ubiquitin proteasome system (UPS) degrades individual proteins in a highly regulated fashion and is responsible for the degradation of misfolded, damaged, or unneeded cellular proteins. During the past 20 years, investigators have established a critical role for the UPS in essentially every cellular process, including cell cycle progression, transcriptional regulation, genome integrity, apoptosis, immune responses, and neuronal plasticity. At the center of the UPS is the proteasome, a large and complex molecular machine containing a multicatalytic protease complex. Read More

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http://dx.doi.org/10.1124/pr.117.015370DOI Listing
April 2019
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Dismantling the bacterial virulence program.

Microb Biotechnol 2019 Mar 12. Epub 2019 Mar 12.

Centre for Microbial Diseases and Immunity Research, Department of Microbiology and Immunology, University of British Columbia, Vancouver, BC, Canada.

In the face of rising antimicrobial resistance, there is an urgent need for the development of efficient and effective anti-infective compounds. Adaptive resistance, a reversible bacterial phenotype characterized by the ability to surmount antibiotic challenge without mutation, is triggered to cope in situ with several stressors and is very common clinically. Thus, it is important to target stress-response effectors that contribute to in vivo adaptations and associated lifestyles such as biofilm formation. Read More

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http://dx.doi.org/10.1111/1751-7915.13388DOI Listing

Nasal Resistome Development in Infants With Cystic Fibrosis in the First Year of Life.

Front Microbiol 2019 26;10:212. Epub 2019 Feb 26.

Institute for Infectious Diseases, University of Bern, Bern, Switzerland.

Polymicrobial infections of the respiratory tract due to antibiotic resistant bacteria are a great concern in patients with cystic fibrosis (CF). We therefore aimed at establishing a functional metagenomic method to analyze the nasal resistome in infants with CF within the first year of life. We included samples from patients before antibiotic treatment, which allowed obtaining information regarding natural status of the resistome. Read More

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http://dx.doi.org/10.3389/fmicb.2019.00212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399209PMC
February 2019
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A pragmatic behavior-based habit index for adherence to nebulized treatments among adults with cystic fibrosis.

Patient Prefer Adherence 2019 13;13:283-294. Epub 2019 Feb 13.

Department of Psychology, Institute of Psychiatry, Psychology and Neuroscience (IoPPN) King's College London, London, UK.

Background: Habit, a psychological process that automatically generates urges to perform a behavior in associated settings, is potentially an important determinant of medication adherence. Habit is challenging to measure because, as a psychological construct, it cannot be directly observed. We describe a method of using routinely available objective adherence data from electronic data capture (EDC) to generate a behavior-based index of adherence habit and demonstrate how this index can be applied. Read More

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http://dx.doi.org/10.2147/PPA.S186417DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6388736PMC
February 2019
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Characterisation and anti-biofilm activity of glycerol monolaurate nanocapsules against Pseudomonas aeruginosa.

Microb Pathog 2019 Mar 9. Epub 2019 Mar 9.

Post Graduate Program in Nanosciences, Universidade Franciscana, Santa Maria, Brazil; Microbiology and Parasitology Department, Health Sciences Center, Universidade Federal de Santa Maria, Santa Maria, Brazil.

Pseudomonas aeruginosa is a ubiquitous microorganism that commonly causes hospital-acquired infections, including pneumonia, bloodstream and urinary tract infections and it is well known for chronically colonising the respiratory tract of patients with cystic fibrosis, causing severe intermittent exacerbation of the condition. P. aeruginosa may appear in the free form cell but also grows in biofilm communities adhered to a surface. Read More

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http://dx.doi.org/10.1016/j.micpath.2019.03.007DOI Listing

The Relative Contribution of Food Groups to Macronutrient Intake in Children with Cystic Fibrosis: A European Multicenter Assessment.

J Acad Nutr Diet 2019 Mar 9. Epub 2019 Mar 9.

Background: Optimal nutrition for children with cystic fibrosis (CF) improves prognosis and survival, but an increased caloric intake recommendation for this population raises concerns about the nutrient profile of their diets.

Objective: Our aim was to assess the relative contribution of food groups to the total macronutrient intake of European pediatric patients with CF.

Design: We conducted a cross-sectional study in which the participants recorded dietary intake from 2016 to 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22122672183026
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http://dx.doi.org/10.1016/j.jand.2019.01.003DOI Listing
March 2019
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Factors associated with opioid use after endoscopic sinus surgery.

Laryngoscope 2019 Mar 12. Epub 2019 Mar 12.

Division of Head and Neck Surgery and Communication Sciences, Department of Surgery, Duke University, Durham, North Carolina.

Objectives/hypothesis: Responsible prescribing of postoperative pain medications is necessary in combatting the current opioid epidemic in the United States. The goal of this study was to determine which clinical factors affect opioid usage following functional endoscopic sinus surgery (FESS).

Study Design: Retrospective medical records study. Read More

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http://dx.doi.org/10.1002/lary.27921DOI Listing
March 2019
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Severe ileocecal inflammatory syndrome in adult patients with cystic fibrosis.

Z Gastroenterol 2019 Mar 12;57(3):312-316. Epub 2019 Mar 12.

University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany.

The relevance of gastrointestinal manifestations of cystic fibrosis (CF) is increasing due to an improved life expectancy. We report on 2 adult patients with prior lung transplantation who presented with a severe inflammatory disorder of the ileocecal region. One patient underwent ileocecal resection; the second patient died after emergency surgery for intestinal perforation. Read More

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http://dx.doi.org/10.1055/a-0829-7050DOI Listing
March 2019
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Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis.

PLoS One 2019 12;14(3):e0213216. Epub 2019 Mar 12.

Instituto de Investigación Sanitaria La Fe de Valencia, Valencia, Spain.

Background: A method to adjust Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis is not currently available.

Objectives: To assess the in vivo efficacy of a method to adjust the dose of enzymatic supplement in CF extrapolated from previous in vitro digestion studies (theoretical optimal dose, TOD). Secondly, to assess how individual patient characteristics influence the expected coefficient of fat absorption (CFA) and thus to identify an individual correction factor to improve TOD. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213216PLOS
March 2019
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Antimicrobial properties of basidiomycota macrofungi to isolated from patients with cystic fibrosis.

Int J Mycobacteriol 2019 Jan-Mar;8(1):93-97

Northern Ireland Public Health Laboratory, Belfast City Hospital, Lisburn Road, Belfast, Northern Ireland, BT9 7AD; School of Medicine, Dentistry and Biomedical Science, Centre for Experimental Medicine, Queen's University, 97 Lisburn Road, Belfast; School of Biomedical Sciences, Ulster University, Cromore Road, Coleraine, Co. Londonderry, Northern Ireland, BT52 1SA, UK.

Background: Antimicrobial resistance (AMR) has now emerged as a global public health crisis. Of particular concern is AMR associated with the genus Mycobacterium, including Mycobacterium tuberculosis and the nontuberculous mycobacteria (NTM). Emergence of the NTM, in particular Mycobacterium abscessus, in patients with cystic fibrosis (CF) represents both a diagnostic and a treatment dilemma. Read More

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http://dx.doi.org/10.4103/ijmy.ijmy_167_18DOI Listing
March 2019
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