1,682 results match your criteria Cystic Fibrosis Thoracic


A black-blood ultra-short echo time (UTE) sequence for 3D isotropic resolution imaging of the lungs.

Magn Reson Med 2019 Feb 8. Epub 2019 Feb 8.

Adult CF unit, Neuchatelois-Pourtales Hospital, Neuchatel, Switzerland.

Purpose: Ultra-short echo time MRI is a promising alternative to chest CT for cystic fibrosis patients. Black-blood imaging in particular could help discern small-sized anomalies, such as mucoid plugging, which may otherwise be confused with neighboring blood vessels, particularly when contrast agent is not used. We, therefore, implemented and tested an ultra-short echo time sequence with black-blood preparation. Read More

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http://dx.doi.org/10.1002/mrm.27679DOI Listing
February 2019
1 Read

Can't see the wood for the trees: confounders, colliders and causal inference - a statistician's approach.

Thorax 2019 Feb 7. Epub 2019 Feb 7.

Division of Biostatistics & Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

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http://dx.doi.org/10.1136/thoraxjnl-2018-212780DOI Listing
February 2019
1 Read

Outcomes in cystic fibrosis lung transplant recipients infected with organisms labeled aspan-resistant: An ISHLT Registry‒based analysis.

J Heart Lung Transplant 2019 Jan 25. Epub 2019 Jan 25.

Division of Infectious Diseases and Global Public Health, University of California San Diego, La Jolla, California, USA. Electronic address:

Background: The presence of pan-resistant organisms in patients with cystic fibrosis (CF) potentially impacts mortality after lung transplant (LT). In this study we aimed to study LT mortality in CF patients with and without pan-resistant infection.

Methods: The International Society for Heart and Lung Transplantation (ISHLT) Thoracic Transplant Registry was used to identify adults with CF, first-time, bilateral LT from 1991 to 2015. Read More

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http://dx.doi.org/10.1016/j.healun.2019.01.1306DOI Listing
January 2019
1 Read

stimulates nuclear sphingosine-1-phosphate generation and epigenetic regulation of lung inflammatory injury.

Thorax 2019 Feb 5. Epub 2019 Feb 5.

Department of Pharmacology, University of Illinois, Chicago, Illinois, USA.

Introduction: Dysregulated sphingolipid metabolism has been implicated in the pathogenesis of various pulmonary disorders. Nuclear sphingosine-1-phosphate (S1P) has been shown to regulate histone acetylation, and therefore could mediate pro-inflammatory genes expression.

Methods: Profile of sphingolipid species in bronchoalveolar lavage fluids and lung tissue of mice challenged with () was investigated. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2018-212378DOI Listing
February 2019
1 Read

Oxygen desaturation during the 6-min walk test as a risk for osteoporosis in non-cystic fibrosis bronchiectasis.

BMC Pulm Med 2019 Feb 4;19(1):28. Epub 2019 Feb 4.

Department of Thoracic Medicine, Chang Gung Memorial Hospital and College of Medicine, Chang Gung University, 199 Tun-Hwa North Road, Taipei, 105, Taiwan.

Background: Osteoporosis is a common comorbidity in non-cystic fibrosis (non-CF) bronchiectasis patients. We determined whether desaturation during 6-min walk test (6MWT) can be a predictor for osteoporosis risk.

Methods: This was a retrospective cross-sectional study. Read More

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https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890
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http://dx.doi.org/10.1186/s12890-019-0794-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360688PMC
February 2019
6 Reads

Cumulative radiation dose after lung transplantation in patients with cystic fibrosis.

Diagn Interv Imaging 2019 Jan 30. Epub 2019 Jan 30.

Radiology Department, Hôpital Européen Georges-Pompidou, Université Paris 5-Descartes, 20, rue Leblanc, 75015 Paris, France. Electronic address:

Purpose: The purpose of this study was first to evaluate the imaging-related cumulative post-transplantation radiation dose in cystic fibrosis (CF) lung transplantation (LT) recipients and second, to identify the occurrence and type of malignancies observed after LT.

Materials And Methods: A total of 52 patients with CF who underwent LT at our institution between January 2001 and December 2006 with at least 3 years of survival were retrospectively included. There were 27 men and 25 women with a mean age of 24. Read More

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http://dx.doi.org/10.1016/j.diii.2018.12.006DOI Listing
January 2019

Outcomes of endoscopic sinus surgery in adult lung transplant patients with cystic fibrosis.

Eur Arch Otorhinolaryngol 2019 Jan 28. Epub 2019 Jan 28.

Department of Surgery and Translational Medicine, Clinic of Otolaryngology, Head and Neck Surgery, Careggi University Hospital, University of Florence, Largo Brambilla, 3-50134, Florence, Italy.

Purpose: Cystic Fibrosis (CF) is the most common autosomal recessive disease in Caucasian population. Due to its pathological mechanism, chronic rhino sinusitis (CRS) associated or not with nasal polyposis usually occurs in adults and affects close to one-half of all CF patients. The goal of our work was to evaluate the impact of Endoscopic Sinus Surgery (ESS) in the quality of life (QoL) of the CF patients and demonstrate an improvement of the functional outcomes in the patients underwent the surgical procedure rather than in the not treated ones, particulary in lung transplant patients. Read More

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http://link.springer.com/10.1007/s00405-019-05308-9
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http://dx.doi.org/10.1007/s00405-019-05308-9DOI Listing
January 2019
2 Reads

Microbiome networks and change-point analysis reveal key community changes associated with cystic fibrosis pulmonary exacerbations.

NPJ Biofilms Microbiomes 2019 21;5. Epub 2019 Jan 21.

1Department of Cell and Systems Biology, University of Toronto, Toronto, ON Canada.

Over 90% of cystic fibrosis (CF) patients die due to chronic lung infections leading to respiratory failure. The decline in CF lung function is greatly accelerated by intermittent and progressively severe acute pulmonary exacerbations (PEs). Despite their clinical impact, surprisingly few microbiological signals associated with PEs have been identified. Read More

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http://dx.doi.org/10.1038/s41522-018-0077-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341074PMC
January 2019
5 Reads

JCF - progress in 2018.

J Cyst Fibros 2019 Jan;18(1):1-5

Department of Medicine and Pediatrics, Medical University of South Carolina, Charleston, SC, USA.

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http://dx.doi.org/10.1016/j.jcf.2018.12.008DOI Listing
January 2019
3 Reads

The Effect of Pulmonary Rehabilitation on the Physical Activity Level and General Clinical Status of Patients with Bronchiectasis.

Turk Thorac J 2019 Jan 1;20(1):30-35. Epub 2019 Jan 1.

Clinic of Pulmonary Rehabilitation, Health Sciences University, Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, İstanbul, Turkey.

Objectives: We aimed to investigate the effects of pulmonary rehabilitation (PR) on the level of physical activity (PA) and the general clinical status in adult patients with non-cystic fibrosis bronchiectasis.

Materials And Methods: The patients were included prospectively in the study and followed a home-based PR program for 2 months comprising breathing exercises, training in chest hygiene techniques, peripheral muscle strengthening training, and self-walking. The outcomes measurements were the following: 6-minute walking distance, pulmonary function test, peripheral and respiratory muscle strength measurements, International Physical Activity Questionnaire (IPAQ), Saint George Respiratory Questionnaire, and modified Medical Research Council dyspnea scores. Read More

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http://dx.doi.org/10.5152/TurkThoracJ.2018.18093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340688PMC
January 2019
4 Reads

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers.

Pediatr Pulmonol 2019 Jan 20. Epub 2019 Jan 20.

School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.

Introduction: Vitamin D acts on the immune system and lung response. Patients with cystic fibrosis (CF) may be deficient in this vitamin. The aims of the study were to evaluate vitamin D levels and severity of lung disease in infants and preschoolers diagnosed with CF, and to compare them to a group of children without pancreatic insufficiency (PI). Read More

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http://doi.wiley.com/10.1002/ppul.24260
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http://dx.doi.org/10.1002/ppul.24260DOI Listing
January 2019
7 Reads

Secondhand smoke alters arachidonic acid metabolism and inflammation in infants and children with cystic fibrosis.

Thorax 2019 Mar 19;74(3):237-246. Epub 2019 Jan 19.

Center for Vaccines and Immunity, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio, USA.

Background: Mechanisms that facilitate early infection and inflammation in cystic fibrosis (CF) are unclear. We previously demonstrated that children with CF and parental-reported secondhand smoke exposure (SHSe) have increased susceptibility to bacterial infections. SHSe hinders arachidonic acid (AA) metabolites that mediate immune function in patients without CF, and may influence CF immune dysfunction. Read More

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http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2018-2118
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http://dx.doi.org/10.1136/thoraxjnl-2018-211845DOI Listing
March 2019
8 Reads

Extracorporeal membrane oxygenation for grade 3 primary graft dysfunction after lung transplantation: long-term outcomes.

Clin Transplant 2019 Jan 18:e13480. Epub 2019 Jan 18.

Thoracic Surgery Department, Foch Hospital, Suresnes, France.

Introduction: Extracorporeal membrane oxygenation (ECMO) is an efficient and innovative therapeutic tool for primary graft dysfunction (PGD). However, its effect on survival and long-term lung function are not well-known. This study evaluated those parameters in patients with PGD requiring ECMO. Read More

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http://doi.wiley.com/10.1111/ctr.13480
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http://dx.doi.org/10.1111/ctr.13480DOI Listing
January 2019
4 Reads

CT-measured pulmonary artery diameter as an independent predictor of pulmonary hypertension in cystic fibrosis.

Pol J Radiol 2018 23;83:e401-e406. Epub 2018 Aug 23.

Mycobacteriology Research Center (MRC), National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Purpose: The role of computed tomography (CT) scan, as a promising prognostic imaging modality in cystic fibrosis (CF), has been widely investigated, focusing on parenchymal abnormalities. The aim of the present study was to evaluate the diagnostic performance of thoracic vascular parameters on CT to detect pulmonary hypertension (PH).

Material And Methods: CF patients who contemporaneously underwent CT and echocardiography were retrospectively enrolled. Read More

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https://www.termedia.pl/doi/10.5114/pjr.2018.79204
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http://dx.doi.org/10.5114/pjr.2018.79204DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334125PMC
August 2018
7 Reads

Epstein-Barr Virus-Related Post-Transplant Lymphoproliferative Disorders in Cystic Fibrosis Lung Transplant Recipients: A Case Series.

Transplant Proc 2019 Jan - Feb;51(1):194-197. Epub 2018 Jun 30.

Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Background: Solid organ transplantation is associated with a higher risk of Epstein-Barr virus (EBV)-related lymphoproliferative disease due to immunosuppressive regimen. Little evidence is currently available on post-transplant lymphoproliferative disorders (PTLDs) in the lung transplant (LuTx) setting, particularly in cystic fibrosis (CF) recipients.

Methods: We retrospectively analyzed all the cases of PTLDs that occurred in our LuTx center between January 2015 and December 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183087
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http://dx.doi.org/10.1016/j.transproceed.2018.05.032DOI Listing
June 2018
7 Reads

Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report.

Transplant Proc 2019 Jan - Feb;51(1):235-238. Epub 2018 Jun 28.

Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183084
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http://dx.doi.org/10.1016/j.transproceed.2018.04.071DOI Listing
June 2018
8 Reads

Surveillance Transbronchial Biopsy Program to Evaluate Acute Rejection After Lung Transplantation: A Single Institution Experience.

Transplant Proc 2019 Jan - Feb;51(1):198-201. Epub 2018 Jun 28.

Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Background: There is no unanimity in the literature regarding the value of transbronchial biopsies (TBBs) performed at a scheduled time after lung transplantation (surveillance TBBs [SBs]), compared to biopsies performed for suspected clinical acute rejection (clinically indicated TBBs [CIBs]). This study exposes an assessment of our experience over the last 4 years through a retrospective analysis of the data collected.

Methods: In our center, SBs are performed at 3, 6, and 12 months after a transplant. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183084
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http://dx.doi.org/10.1016/j.transproceed.2018.04.073DOI Listing
June 2018
6 Reads

Lung Transplant From Donor With Tracheal Bronchus: Case Report and Literature Review.

Transplant Proc 2019 Jan - Feb;51(1):239-241. Epub 2018 Jun 30.

Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

Donor lung abnormalities are quite rare; one of them is the presence of bronchial anomalies, whose incidence range is from 0.1% to 0.5%. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183086
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http://dx.doi.org/10.1016/j.transproceed.2018.04.082DOI Listing
June 2018
4 Reads

Current Status and Future Opportunities in Lung Precision Medicine Research with a Focus on Biomarkers. An American Thoracic Society/National Heart, Lung, and Blood Institute Research Statement.

Am J Respir Crit Care Med 2018 Dec;198(12):e116-e136

Background: Thousands of biomarker tests are either available or under development for lung diseases. In many cases, adoption of these tests into clinical practice is outpacing the generation and evaluation of sufficient data to determine clinical utility and ability to improve health outcomes. There is a need for a systematically organized report that provides guidance on how to understand and evaluate use of biomarker tests for lung diseases. Read More

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https://www.atsjournals.org/doi/10.1164/rccm.201810-1895ST
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http://dx.doi.org/10.1164/rccm.201810-1895STDOI Listing
December 2018
7 Reads

Improving the Affordability of Prescription Medications for People with Chronic Respiratory Disease. An Official American Thoracic Society Policy Statement.

Am J Respir Crit Care Med 2018 Dec;198(11):1367-1374

Background: Mounting evidence indicates that out-of-pocket costs for prescription medications, particularly among low- and middle-income patients with chronic diseases, are imposing financial burden, reducing medication adherence, and worsening health outcomes. This problem is exacerbated by a paucity of generic alternatives for prevalent lung diseases, such as asthma and chronic obstructive pulmonary disease, as well as high-cost medicines for rare diseases, such as cystic fibrosis. Affordability and access challenges are especially salient in the United States, as citizens of many other countries pay lower prices for and have greater access to prescription medications. Read More

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http://dx.doi.org/10.1164/rccm.201810-1865STDOI Listing
December 2018
3 Reads

Successful lung transplantation for chronic Mycobacterium abscessus infection in advanced cystic fibrosis, a case series.

Transpl Infect Dis 2018 Dec 30:e13046. Epub 2018 Dec 30.

Department of Pneumology, University Hospitals Leuven, Leuven, Belgium.

Pulmonary Mycobacterium abscessus infection in cystic fibrosis (CF) patients is difficult to treat and considered a contra-indication for lung transplantation in most centers. We present four CF patients with chronic pulmonary M abscessus infection, in whom lung transplantation was performed. Through intensive treatment before transplantation, we achieved control of the infection in all but one patient. Read More

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http://doi.wiley.com/10.1111/tid.13046
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http://dx.doi.org/10.1111/tid.13046DOI Listing
December 2018
4 Reads

A real life evaluation of non invasive ventilation in acute cardiogenic pulmonary edema: a multicenter, perspective, observational study for the ACPE SIMEU study group.

BMC Emerg Med 2018 Dec 29;18(1):61. Epub 2018 Dec 29.

Emergency Medicine Department, ASST Papa Giovanni XIII, Bergamo, Italy.

Background: During the past three decades conflicting evidences have been published on the use of non-invasive ventilation (NIV) in patients with acute cardiogenic pulmonary edema (ACPE). The aim of this study is to describe the management of acute respiratory failure (ARF) due to ACPE in twelve Italian emergency departments (EDs). We evaluated prevalence, characteristics and outcomes of ACPE patients treated with oxygen therapy, continuous positive airway pressure (CPAP) or Bi-level positive airway pressure (BiPAP) on admission to the EDs. Read More

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http://dx.doi.org/10.1186/s12873-018-0216-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310941PMC
December 2018
2 Reads

High-Throughput Sequencing in Respiratory, Critical Care, and Sleep Medicine Research. An Official American Thoracic Society Workshop Report.

Ann Am Thorac Soc 2019 Jan;16(1):1-16

High-throughput, "next-generation" sequencing methods are now being broadly applied across all fields of biomedical research, including respiratory disease, critical care, and sleep medicine. Although there are numerous review articles and best practice guidelines related to sequencing methods and data analysis, there are fewer resources summarizing issues related to study design and interpretation, especially as applied to common, complex, nonmalignant diseases. To address these gaps, a single-day workshop was held at the American Thoracic Society meeting in May 2017, led by the American Thoracic Society Section on Genetics and Genomics. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201810-716WSDOI Listing
January 2019
1 Read

Survival After Lung Transplant for Cystic Fibrosis in Italy: A Single Center Experience With 20 Years of Follow-up.

Transplant Proc 2018 Dec 7;50(10):3732-3738. Epub 2018 Sep 7.

Department of Pediatrics, Cystic Fibrosis Center, Sapienza University of Rome, Italy.

Objectives: Lung transplantation is currently the only treatment for end-stage respiratory failure in patients with cystic fibrosis (CF). In this study we retrospectively analyzed our experience since the start of the transplantation program in 1996 with focus on survival analysis.

Methods: All patients with CF who underwent lung transplant at our center were included (1996-2016). Read More

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http://dx.doi.org/10.1016/j.transproceed.2018.08.020DOI Listing
December 2018

Lung transplantation in two cystic fibrosis patients infected with previously pandrug-resistant Burkholderia cepacia complex treated with ceftazidime-avibactam.

Infection 2018 Dec 19. Epub 2018 Dec 19.

Infectious Diseases Department, Hospital Universitari Vall d'Hebron, P° Vall d'Hebron 119-129, 08035, Barcelona, Spain.

We describe two cystic fibrosis patients infected with pandrug-resistant Burkholderia cepacia complex, with the exception of ceftazidime-avibactam, who received prophylaxis with this antibiotic during lung transplantation. Although both patients had a post-operative relapse of respiratory infection, one with positive blood cultures, ceftazidime-avibactam treatment yielded a favourable outcome. 12 months after transplantation, one patient presented an excellent clinical outcome. Read More

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http://link.springer.com/10.1007/s15010-018-1261-y
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http://dx.doi.org/10.1007/s15010-018-1261-yDOI Listing
December 2018
3 Reads

A genome-wide association analysis reveals a potential role for recombination in the evolution of antimicrobial resistance in Burkholderia multivorans.

PLoS Pathog 2018 12 7;14(12):e1007453. Epub 2018 Dec 7.

Department of Cell and Systems Biology, University of Toronto, Toronto, Ontario, Canada.

Cystic fibrosis (CF) lung infections caused by members of the Burkholderia cepacia complex, such as Burkholderia multivorans, are associated with high rates of mortality and morbidity. We performed a population genomics study of 111 B. multivorans sputum isolates from one CF patient through three stages of infection including an early incident isolate, deep sampling of a one-year period of chronic infection occurring weeks before a lung transplant, and deep sampling of a post-transplant infection. Read More

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http://dx.doi.org/10.1371/journal.ppat.1007453DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300292PMC
December 2018

Microbiological testing of adults hospitalised with community-acquired pneumonia: an international study.

ERJ Open Res 2018 Oct 8;4(4). Epub 2018 Oct 8.

South Texas Veterans Health Care System and University of Texas Health San Antonio, San Antonio, TX, USA.

This study aimed to describe real-life microbiological testing of adults hospitalised with community-acquired pneumonia (CAP) and to assess concordance with the 2007 Infectious Diseases Society of America (IDSA)/American Thoracic Society (ATS) and 2011 European Respiratory Society (ERS) CAP guidelines. This was a cohort study based on the Global Initiative for Methicillin-resistant Pneumonia (GLIMP) database, which contains point-prevalence data on adults hospitalised with CAP across 54 countries during 2015. In total, 3702 patients were included. Read More

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http://dx.doi.org/10.1183/23120541.00096-2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174282PMC
October 2018
1 Read

Transplant options for end stage chronic obstructive pulmonary disease in the context of multidisciplinary treatments.

J Thorac Dis 2018 Oct;10(Suppl 27):S3356-S3365

Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Lung transplantation (LTx) in advanced stage chronic obstructive pulmonary disease (COPD) patients is associated with significant improvement in lung function and exercise capacity. However, demonstration that the procedure also provides a survival benefit has been more elusive compared to other respiratory conditions. Identification of patients with increased risk of mortality is crucial: a low forced expiratory volume in 1 second (FEV) is perhaps the most common reason for referral to a lung transplant center, but in itself is insufficient to identify which COPD patients will benefit from LTx. Read More

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http://jtd.amegroups.com/article/view/21080/18463
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http://dx.doi.org/10.21037/jtd.2018.04.166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204341PMC
October 2018
10 Reads

Elevated circulating MMP-9 is linked to increased COPD exacerbation risk in SPIROMICS and COPDGene.

JCI Insight 2018 Nov 15;3(22). Epub 2018 Nov 15.

The SPIROMICS and COPDGene groups are detailed in the Supplemental Acknowledgments.

Background: Matrix metalloprotease 9 (MMP-9) is associated with inflammation and lung remodeling in chronic obstructive pulmonary disease (COPD). We hypothesized that elevated circulating MMP-9 represents a potentially novel biomarker that identifies a subset of individuals with COPD with an inflammatory phenotype who are at increased risk for acute exacerbation (AECOPD).

Methods: We analyzed Subpopulations and Intermediate Outcome Measures in COPD Study (SPIROMICS) and Genetic Epidemiology of COPD (COPDGene) cohorts for which baseline and prospective data were available. Read More

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https://insight.jci.org/articles/view/123614
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http://dx.doi.org/10.1172/jci.insight.123614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302944PMC
November 2018
9 Reads

CFTR inhibits the invasion and growth of esophageal cancer cells by inhibiting the expression of NF-κB.

Cell Biol Int 2018 Dec;42(12):1680-1687

Department of Gastroenterology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, No. 20 Yuhuangding Dong Road, Yantai, 264000, Shandong, China.

Our study aimed to explore the function of cystic fibrosis transmembrane conductance regulator (CFTR) in esophageal cancer. Twenty patients with esophageal squamous cell carcinoma (ESCC) and 20 patients with esophageal adenocarcinoma (EA) were enrolled in this study. The levels of CFTR and NF-κB in tumor tissues and adjacent normal tissues were detected, respectively. Read More

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http://doi.wiley.com/10.1002/cbin.11069
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http://dx.doi.org/10.1002/cbin.11069DOI Listing
December 2018
4 Reads
1.933 Impact Factor

The influence of retransplantation on survival for pediatric lung transplant recipients.

J Thorac Cardiovasc Surg 2018 Nov 6;156(5):2025-2034.e2. Epub 2018 Jun 6.

Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria. Electronic address:

Objectives: We reviewed our 25-year experience in pediatric lung transplantation with the aim to identify trends and influencing factors over time.

Methods: We reviewed our prospectively maintained database and analyzed all patients younger than age 18 years who underwent primary lung transplantation at Medical University of Vienna between 1990 and 2015.

Results: Eighty-six consecutive patients were enrolled with a mean age of 12. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00225223183151
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http://dx.doi.org/10.1016/j.jtcvs.2018.05.080DOI Listing
November 2018
13 Reads

Increased expression of ATP12A proton pump in cystic fibrosis airways.

JCI Insight 2018 Oct 18;3(20). Epub 2018 Oct 18.

Telethon Institute of Genetics and Medicine (TIGEM), Pozzuoli NA, Italy.

Proton secretion mediated by ATP12A protein on the surface of the airway epithelium may contribute to cystic fibrosis (CF) lung disease by favoring bacterial infection and airway obstruction. We studied ATP12A in fresh bronchial samples and in cultured epithelial cells. In vivo, ATP12A expression was found almost exclusively at the apical side of nonciliated cells of airway epithelium and in submucosal glands, with much higher expression in CF samples. Read More

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http://dx.doi.org/10.1172/jci.insight.123616DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237449PMC
October 2018
9 Reads

Gaps in the evidence for treatment decisions in cystic fibrosis: a systematic review.

Thorax 2019 Mar 9;74(3):229-236. Epub 2018 Oct 9.

Evidence Based Child Health Group, Division of Child Health, Obstetrics and Gynaecology, University of Nottingham, Nottingham, UK.

Introduction: Cystic fibrosis (CF) is a multisystem disorder. Treatment is complex and evidence for treatment decisions may be absent. Characterising gaps in the research evidence will highlight treatment uncertainties and help prioritise research questions. Read More

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http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2017-2108
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http://dx.doi.org/10.1136/thoraxjnl-2017-210858DOI Listing
March 2019
5 Reads

In-vitro analysis of a novel 'add-on' silicone cuff to improve sealing properties of tracheal tubes.

Anaesthesia 2018 Nov;73(11):1372-1381

Department Hospital Clínic, Thorax Institute, Pneumology Department, and University of Barcelona, Barcelona, Spain.

Leakage of colonised oropharyngeal secretions across the tracheal tube cuff may cause iatrogenic pulmonary infection. We studied a novel 'add-on' cuff, which can be inserted over an existing tracheal tube and advanced into the subglottic region. The physical properties of the novel silicone cuff (BronchoGuard, Ciel Medical, USA) were evaluated in comparison with the Hi-Lo tracheal tube. Read More

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http://dx.doi.org/10.1111/anae.14413DOI Listing
November 2018
2 Reads

Deep learning in chest radiography: Detection of findings and presence of change.

PLoS One 2018 4;13(10):e0204155. Epub 2018 Oct 4.

Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts, United States of America.

Background: Deep learning (DL) based solutions have been proposed for interpretation of several imaging modalities including radiography, CT, and MR. For chest radiographs, DL algorithms have found success in the evaluation of abnormalities such as lung nodules, pulmonary tuberculosis, cystic fibrosis, pneumoconiosis, and location of peripherally inserted central catheters. Chest radiography represents the most commonly performed radiological test for a multitude of non-emergent and emergent clinical indications. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0204155PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171827PMC
October 2018
7 Reads

Patient outcomes from time of listing for lung transplantation in the UK: are there disease-specific differences?

Thorax 2019 Jan 3;74(1):60-68. Epub 2018 Oct 3.

Department of Cardiothoracic Transplantation, Royal Papworth NHS Foundation Trust, Cambridge, UK.

Background: The demand for lung transplantation vastly exceeds the availability of donor organs. This translates into long waiting times and high waiting list mortality. We set out to examine factors influencing patient outcomes from the time of listing for lung transplantation in the UK, examining for differences by patient characteristics, lung disease category and transplant centre. Read More

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http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2018-2117
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http://dx.doi.org/10.1136/thoraxjnl-2018-211731DOI Listing
January 2019
17 Reads

The contribution of respiratory microbiome analysis to a treatable traits model of care.

Respirology 2019 Jan 3;24(1):19-28. Epub 2018 Oct 3.

South Australian Health and Medical Research Institute, Adelaide, SA, Australia.

The composition of the airway microbiome in patients with chronic airway diseases, such as severe asthma, chronic obstructive pulmonary disease (COPD), bronchiectasis and cystic fibrosis (CF), has the potential to inform a precision model of clinical care. Patients with these conditions share overlapping disease characteristics, including airway inflammation and airflow limitation. The clinical management of chronic respiratory conditions is increasingly moving away from a one-size-fits-all model based on primary diagnosis, towards care targeting individual disease traits, and is particularly useful for subgroups of patients who respond poorly to conventional therapies. Read More

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http://dx.doi.org/10.1111/resp.13411DOI Listing
January 2019
3 Reads

and progression of lung disease in children with cystic fibrosis.

Thorax 2019 Feb 1;74(2):125-131. Epub 2018 Oct 1.

School of Pharmacy, University of Queensland, Brisbane, Queensland, Australia.

Background: The impact of on lung disease in young children with cystic fibrosis is uncertain.

Aims: To determine if positive respiratory cultures of species are associated with: (1) increased structural lung injury at age 5 years; (2) accelerated lung function decline between ages 5 years and 14 years and (3) to identify explanatory variables.

Methods: A cross-sectional analysis of association between positive bronchoalveolar lavage (BAL) cultures and chest high-resolution CT (HRCT) scan findings at age 5 years in subjects from the Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study was performed. Read More

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http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2018-2115
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http://dx.doi.org/10.1136/thoraxjnl-2018-211550DOI Listing
February 2019
17 Reads

Antimicrobial resistance in cystic fibrosis: Does it matter?

J Cyst Fibros 2018 Nov 27;17(6):687-689. Epub 2018 Sep 27.

Imperial College and Royal Brompton Hospital, London, Queen's University Belfast, Ireland. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183080
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http://dx.doi.org/10.1016/j.jcf.2018.08.015DOI Listing
November 2018
9 Reads

Defining antimicrobial resistance in cystic fibrosis.

J Cyst Fibros 2018 Nov 25;17(6):696-704. Epub 2018 Sep 25.

Division of Infectious Diseases, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, USA. Electronic address:

Antimicrobial resistance (AMR) can present significant challenges in the treatment of cystic fibrosis (CF) lung infections. In CF and other chronic diseases, AMR has a different profile and clinical consequences compared to acute infections and this requires different diagnostic and treatment approaches. This review defines AMR, explains how it occurs, describes the methods used to measure AMR as well as their limitations, and concludes with future directions for research and development in the area of AMR in CF. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183079
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http://dx.doi.org/10.1016/j.jcf.2018.08.014DOI Listing
November 2018
6 Reads

Exenatide corrects postprandial hyperglycaemia in young people with cystic fibrosis and impaired glucose tolerance: A randomized crossover trial.

Diabetes Obes Metab 2019 Mar 25;21(3):700-704. Epub 2018 Oct 25.

Department of Endocrinology and Diabetes, Women's and Children's Hospital, Adelaide, South Australia, Australia.

Impaired glucose tolerance (IGT) in cystic fibrosis (CF) manifests as postprandial hyperglycaemia. Pancreatic enzyme supplementation reduces the latter; restoring incretin secretion and slowing gastric emptying. We aimed to determine the acute effect of exenatide on postprandial glycaemia in young people with CF and IGT. Read More

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http://doi.wiley.com/10.1111/dom.13544
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http://dx.doi.org/10.1111/dom.13544DOI Listing
March 2019
3 Reads

Ultra-low-dose chest computed tomography without anesthesia in the assessment of pediatric pulmonary diseases.

J Pediatr (Rio J) 2018 Sep 17. Epub 2018 Sep 17.

Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil; Universidade Federal de Ciências da Saúde de Porto Alegre, Laboratório de Pesquisa em Imagens Médicas, Porto Alegre, RS, Brazil. Electronic address:

Objective: To evaluate the feasibility of using ultra-low-dose computed tomography of the chest with iterative reconstruction without anesthesia for assessment of pulmonary diseases in children.

Methods: This prospective study enrolled 86 consecutive pediatric patients (ranging from 1 month to 18 years) that underwent ultra-low-dose computed tomography due to suspicion of pulmonary diseases, without anesthesia and contrast. Parameters used were: 80kVp; 15-30mA; acquisition time, 0. Read More

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http://dx.doi.org/10.1016/j.jped.2018.07.010DOI Listing
September 2018
4 Reads
0.940 Impact Factor

Reply to: Use of Masks in Cystic Fibrosis Patients.

Am J Respir Crit Care Med 2018 Sep 20. Epub 2018 Sep 20.

The Prince Charles Hospital, Thoracic Medicine, Brisbane, Queensland, Australia.

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http://dx.doi.org/10.1164/rccm.201808-1476LEDOI Listing
September 2018
1 Read

Identification of genes and pathways in esophageal adenocarcinoma using bioinformatics analysis.

Biomed Rep 2018 Oct 25;9(4):305-312. Epub 2018 Jul 25.

Department of Clinical Laboratory, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, P.R. China.

Esophageal adenocarcinoma (EAC) is one of the most common subtypes of esophageal cancer, and is associated with a low 5-year survival rate. The present study aimed to identify key genes and pathways associated with EAC using bioinformatics analysis. The gene expression profiles of GSE92396, which includes 12 EAC samples and 9 normal esophageal samples, were downloaded from the Gene Expression Omnibus database. Read More

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http://dx.doi.org/10.3892/br.2018.1134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142036PMC
October 2018
3 Reads

Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis.

United European Gastroenterol J 2018 Aug 17;6(7):1049-1055. Epub 2018 May 17.

Department of Internal Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria.

Background: Nutritional status is an important prognostic factor in patients with cystic fibrosis (CF) prior to lung transplantation.

Objective: To investigate the impact of nutritional status on pulmonary function in CF transplant recipients.

Methods: Adult double lung transplanted CF patients were consecutively included. Read More

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http://dx.doi.org/10.1177/2050640618778381DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6137582PMC
August 2018
7 Reads

Successful Lung Re-transplant in a Patient with Cepacia Syndrome due to Burkholderia ambifaria.

J Cyst Fibros 2019 Jan 14;18(1):e1-e4. Epub 2018 Sep 14.

Division of Transplant Pulmonology, Norton Thoracic Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, United States. Electronic address:

Chronic airway inflammation and infection drive morbidity and mortality among patients with cystic fibrosis (CF). While Haemophilus influenzae and Staphylococcus aureus predominate in children, the prevalence of Pseudomonas aeruginosa increases as patients age. Other bacteria, including species within the Burkholderia cepacia complex (Bcc), are also more prevalent among adults with CF. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.08.011DOI Listing
January 2019
1 Read

Alignment of Inhaled Chronic Obstructive Pulmonary Disease Therapies with Published Strategies. Analysis of the Global Initiative for Chronic Obstructive Lung Disease Recommendations in SPIROMICS.

Ann Am Thorac Soc 2019 Feb;16(2):200-208

1 Division of Pulmonary Diseases and Critical Care Medicine, Department of Medicine, and.

Rationale: Despite awareness of chronic obstructive pulmonary disease (COPD) treatment recommendations, uptake is poor. The Subpopulations and Intermediate Outcome Measures in COPD Study (SPIROMICS) spans 2010-2016, providing an opportunity to assess integration of 2011 Global Initiative for Obstructive Lung Disease (GOLD) treatment strategies over time in a large observational cohort study.

Objectives: To evaluate how COPD treatment aligns with 2011 GOLD strategies and determine factors associated with failure to align with recommendations. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201804-283OCDOI Listing
February 2019
6 Reads

Work environment risks for health care workers with cystic fibrosis.

Respirology 2018 Dec 14;23(12):1190-1197. Epub 2018 Sep 14.

Department of Infectious Diseases, Monash Health, Melbourne, VIC, Australia.

In Australia and New Zealand, >50% of people with cystic fibrosis (CF) are adults and many of these people are pursuing vocational training and undertaking paid employment. More than 6% of adults with CF are working in health care. There is limited guidance in literature to support health care workers with CF (HCWcf) in training and in employment to support safe practice and to provide protection for themselves and their patients from the acquisition of health care associated infection. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/resp.13404
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http://dx.doi.org/10.1111/resp.13404DOI Listing
December 2018
12 Reads

Bronchiectasis: Treatment decisions for pulmonary exacerbations and their prevention.

Respirology 2018 Nov 11;23(11):1006-1022. Epub 2018 Sep 11.

Department of Paediatrics, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.

Interest in bronchiectasis has increased over the past two decades, as shown by the establishment of disease-specific registries in several countries, the publication of management guidelines and a growing number of clinical trials to address evidence gaps for treatment decisions. This review considers the evidence for defining and treating pulmonary exacerbations, the approaches for eradication of newly identified airway pathogens and the methods to prevent exacerbations through long-term treatments from a pragmatic practice-based perspective. Areas for future studies are also explored. Read More

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http://doi.wiley.com/10.1111/resp.13398
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http://dx.doi.org/10.1111/resp.13398DOI Listing
November 2018
4 Reads