1,841 results match your criteria Cystic Fibrosis Thoracic


The Use of Amikacin Liposome Inhalation Suspension (Arikayce) in the Treatment of Refractory Nontuberculous Mycobacterial Lung Disease in Adults.

Drug Des Devel Ther 2020 10;14:2287-2294. Epub 2020 Jun 10.

Division of Pulmonary Disease and Critical Care Medicine, Department of Medicine, Virginia Commonwealth University, Richmond, VA, USA.

Nontuberculous mycobacteria (NTM) can cause and perpetuate chronic inflammation and lung infection. Despite having the diagnostic criteria, as defined by the American Thoracic Society (ATS) and Infectious Diseases Society of America (IDSA), clinicians find it challenging to diagnose and treat NTM-induced lung disease. Inhaled antibiotics are suitable for patients with lung infection caused by and other organisms, but until recently, their utility in NTM-induced infection was not established. Read More

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http://dx.doi.org/10.2147/DDDT.S146111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7293904PMC

Evidence of Small Airways Disease and the Immediate Effects of Lumacaftor/Ivacaftor in Children with Cystic Fibrosis.

Ir Med J 2020 May 7;113(5):70. Epub 2020 May 7.

Department of Respiratory, Children's University Hospital, Temple Street, Dublin 1.

Aim The aim of this study was to explore risk factors for acute changes in lung function following initiation of lumacaftor/ivacaftor (LUM/IVA) in children with cystic fibrosis. Methods Retrospective review of all children commenced on LUM/IVA treatment over a one-year period. CT Thorax images were reviewed for evidence of air trapping using the Brody score. Read More

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Antimicrobial resistance: Concerns of healthcare providers and people with CF.

J Cyst Fibros 2020 Jun 17. Epub 2020 Jun 17.

Department of Pediatrics, University of North Carolina, Chapel Hill, NC, USA. Electronic address:

Background: Chronic lung infections and their treatment pose risks for the development of antimicrobial resistance (AMR) in people with cystic fibrosis (PWCF). In this study, we evaluated the attitudes of healthcare providers' (HCP) and PWCF or their parents' toward AMR within the international CF community.

Methods: HCP and PWCF identified through listservs and CF-related organizations were asked to complete an AMR centered survey, with additional questions on antimicrobial stewardship (AMS) for HCP. Read More

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http://dx.doi.org/10.1016/j.jcf.2020.05.009DOI Listing

"Management of Community-Acquired Pneumonia in Immunocompromised Adults: A Consensus Statement Regarding Initial Strategies".

Chest 2020 Jun 16. Epub 2020 Jun 16.

Pulmonary and Critical Care, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Background: Community-acquired pneumonia (CAP) guidelines have improved the management and outcomes of patients with CAP, primarily by standardization of initial empiric therapy. But current society-published guidelines exclude immunocompromised patients.

Research Question: There is no concensus regarding the initial management of immunocompromised patients with suspected CAP. Read More

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http://dx.doi.org/10.1016/j.chest.2020.05.598DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297164PMC
June 2020
7.483 Impact Factor

Bronchoalveolar Lavage-microRNAs Are Potential Novel Biomarkers of Outcome After Lung Transplantation.

Transplant Direct 2020 May 9;6(5):e547. Epub 2020 Apr 9.

Division of Pathology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Primary graft dysfunction, infections, and acute rejection (AR) worsen lung transplantation (LTx) outcome and patient survival. Despite significant efforts, reliable biomarkers of acute lung allograft dysfunction are lacking. To address this issue, we profiled the bronchoalveolar lavage (BAL) miRNome in LTx patients. Read More

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http://dx.doi.org/10.1097/TXD.0000000000000994DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213607PMC

Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations.

J Cyst Fibros 2020 Jun 13. Epub 2020 Jun 13.

Faculty of Medicine, University of Queensland, South Brisbane, Queensland, Australia. Electronic address:

Background: Tezacaftor/ivacaftor is a CFTR modulator approved to treat people with cystic fibrosis (pwCF) who are homozygous (F/F) or heterozygous for the F508del-CFTR mutation and a residual function mutation (F/RF). This randomized, double-blind, placebo-controlled Phase 3 study evaluated the efficacy, safety, tolerability, and pharmacokinetics (PK) of tezacaftor/ivacaftor in participants ≥12 years of age heterozygous for the F508del-CFTR mutation and a minimal function mutation (F/MF), which produces no CFTR protein or a protein unresponsive to tezacaftor/ivacaftor in vitro.

Methods: Participants were randomized 1:1 to receive tezacaftor/ivacaftor or placebo for 12 weeks. Read More

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http://dx.doi.org/10.1016/j.jcf.2020.04.015DOI Listing

COVID-19 in lung transplant recipients: A case series from Milan, Italy.

Transpl Infect Dis 2020 Jun 8:e13356. Epub 2020 Jun 8.

Respiratory Unit and Adult Cystic, Fibrosis Centre, Internal Medicine Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy.

Limited data are currently available regarding the course of COVID-19 in lung and solid organ transplant recipients. We hereby present four cases of SARS-CoV-2 pneumonia in lung transplant recipients from our center, set in Milan, Italy. We reduced immunosuppressive regimen in all these patients, typically holding the antiproliferative agent and augmenting steroids; everybody received hydroxychloroquine, initial empiric antibiotic treatment with piperacillin/tazobactam, and high-dose low molecular weight heparin. Read More

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http://dx.doi.org/10.1111/tid.13356DOI Listing

Outcomes of pulmonary infection.

Int J Mycobacteriol 2020 Jan-Mar;9(1):48-52

Oxford Adult Cystic Fibrosis Centre, Oxford University Hospitals NHS Foundation Trust; University of Oxford, Oxford, UK.

Background: Treatment of Mycobacterium abscessus pulmonary disease (PD) is challenging with frequent side effects and uncertain rates of success.

Methods: We performed a retrospective review of all patients at our center with at least one respiratory sample positive for M. abscessus between 2014 and 2019. Read More

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http://dx.doi.org/10.4103/ijmy.ijmy_3_20DOI Listing

Pulmonary fibrosis secondary to COVID-19: a call to arms?

Lancet Respir Med 2020 May 15. Epub 2020 May 15.

National Institute for Health Research, Respiratory Clinical Research Facility, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College, London, UK.

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http://dx.doi.org/10.1016/S2213-2600(20)30222-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7228737PMC

Tablet and web-based audiometry to screen for hearing loss in adults with cystic fibrosis.

Thorax 2020 May 14. Epub 2020 May 14.

Adult Cystic Fibrosis Centre, Royal Brompton and Harefield NHS Foundation Trust, London, UK

Introduction: Individuals with chronic lung disease (eg, cystic fibrosis (CF)) often receive antimicrobial therapy including aminoglycosides resulting in ototoxicity. Extended high-frequency audiometry has increased sensitivity for ototoxicity detection, but diagnostic audiometry in a sound-booth is costly, time-consuming and requires a trained audiologist. This cross-sectional study analysed tablet-based audiometry (Shoebox MD) performed by non-audiologists in an outpatient setting, alongside home web-based audiometry (3D Tune-In) to screen for hearing loss in adults with CF. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2019-214177DOI Listing

Probiotics may be considered for children and adults with cystic fibrosis.

Evid Based Nurs 2020 May 13. Epub 2020 May 13.

Evidence Synthesis, University of Central Lancashire, Preston, Lancashire, UK.

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http://dx.doi.org/10.1136/ebnurs-2020-103272DOI Listing

The Detection of Bile Acids in the Lungs of Paediatric Cystic Fibrosis Patients Is Associated with Altered Inflammatory Patterns.

Diagnostics (Basel) 2020 May 6;10(5). Epub 2020 May 6.

Human Microbiome Programme, School of Pharmacy and Biomedical Sciences, Curtin University, Perth 6102, Australia.

: Cystic fibrosis (CF) is a hereditary disorder in which persistent unresolved inflammation and recurrent airway infections play major roles in the initiation and progression of the disease. Little is known about triggering factors modulating the transition to chronic microbial infection and inflammation particularly in young children. Cystic fibrosis respiratory disease starts early in life, with the detection of inflammatory markers and infection evident even before respiratory symptoms arise. Read More

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http://dx.doi.org/10.3390/diagnostics10050282DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7277992PMC

ACR Appropriateness Criteria® Hemoptysis.

J Am Coll Radiol 2020 May;17(5S):S148-S159

Specialty Chair, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

Hemoptysis, the expectoration of blood, ranges in severity from nonmassive to massive. This publication reviews the literature on the imaging and treatment of hemoptysis. Based on the literature, the imaging recommendations for massive hemoptysis are both a chest radiograph and CT with contrast or CTA with contrast. Read More

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http://dx.doi.org/10.1016/j.jacr.2020.01.043DOI Listing

The role of innate immunity in the long-term outcome of lung transplantation.

Ann Transl Med 2020 Mar;8(6):412

Latner Thoracic Research Laboratories, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Long-term survival after lung transplantation remains suboptimal due to chronic lung allograft dysfunction (CLAD), a progressive scarring process affecting the graft. Although anti-donor alloimmunity is central to the pathogenesis of CLAD, its underlying mechanisms are not fully elucidated and it is neither preventable nor treatable using currently available immunosuppression. Recent evidence has shown that innate immune stimuli are fundamental to the development of CLAD. Read More

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http://dx.doi.org/10.21037/atm.2020.03.20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186608PMC

Esophageal Motility Disorders Associated With Death or Allograft Dysfunction After Lung Transplantation? Results of a Retrospective Monocentric Study.

Clin Transl Gastroenterol 2020 Mar;11(3):e00137

Hospices Civils de Lyon, Digestive Physiology, Hopital E Herriot, Lyon, France.

Objectives: Pathological gastroesophageal reflux (GER) is a known risk factor for bronchiolitis obliterans syndrome (BOS) after lung transplantation. This study aimed at determining whether functional esophageal evaluation might predict BOS occurrence and survival in this setting.

Methods: Ninety-three patients who underwent esophageal high-resolution manometry and 24-hour pH-impedance monitoring within the first year after lung transplantation were retrospectively included. Read More

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http://dx.doi.org/10.14309/ctg.0000000000000137DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7145052PMC

Oscillating devices for airway clearance in people with cystic fibrosis.

Cochrane Database Syst Rev 2020 04 30;4:CD006842. Epub 2020 Apr 30.

Queen Elizabeth University Hospital, Glasgow, UK.

Background: Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis. Oscillating devices generate intra- or extra-thoracic oscillations orally or external to the chest wall. Internally they create variable resistances within the airways, generating controlled oscillating positive pressure which mobilises mucus. Read More

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http://dx.doi.org/10.1002/14651858.CD006842.pub5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7197699PMC

Are We Missing the Opportunity to Measure Muscle Mass on Computed Tomography Thorax?

J Thorac Imaging 2020 Apr 28. Epub 2020 Apr 28.

All Wales Adult Cystic Fibrosis Centre, University Hospital of Llandough.

Hand grip strength (HGS) and fat free mass index (FFMI) are important indicators of skeletal muscle mass and correlate with prognosis in patients with respiratory diseases. It is also possible to estimate muscle mass by measuring muscle density and volume on cross sectional imaging. We reviewed all patients of the All Wales Cystic Fibrosis Centre who had a computed tomography thorax as part of routine clinical care between 2013 and 2017. Read More

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http://dx.doi.org/10.1097/RTI.0000000000000517DOI Listing

Exercise and Cystic Fibrosis.

Adv Exp Med Biol 2020 ;1228:381-391

Department of Thoracic and Cardiovascular Surgery, The Second Affiliated Hospital of Nantong University, Nantong, China.

Cystic fibrosis (CF) is an autosomal recessive, inherited congenital disease caused by the mutation of the family autosomal CF gene, with cumulative exocrine secretion characterized by inflammation, tracheal remodeling, and mucus accumulation. With the development of modern medical technology, CF patients are living longer lives and receiving more and more treatments, including traditional drugs, physical therapy, and gene therapy. Exercise is widely used to prevent and treat metabolic diseases such as cardiovascular diseases, obesity, diabetes, and metabolic syndrome. Read More

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http://dx.doi.org/10.1007/978-981-15-1792-1_26DOI Listing
January 2020

Enhanced inflammasome activation and reduced sphingosine-1 phosphate S1P signalling in a respiratory mucoobstructive disease model.

J Inflamm (Lond) 2020 21;17:16. Epub 2020 Apr 21.

1Department of Thoracic Medicine, Royal Adelaide Hospital, Adelaide, Australia.

Background: Inflammasomes and sphingosine-1-phosphate (S1P) signalling are increasingly subject to intensive research in human diseases. We hypothesize that in respiratory muco-obstructive diseases, mucus obstruction enhances NLRP3 inflammasome activation and dysregulated S1P signalling.

Methods: Lung tissues from mice overexpressing the beta-unit of the epithelial sodium channel (βENaC) and their littermate controls were examined by histology, immunofluorescence and confocal microscopy, followed by ImageJ quantitative analysis. Read More

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http://dx.doi.org/10.1186/s12950-020-00248-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7175514PMC

Sympatho-Vagal Dysfunction in Patients with End-Stage Lung Disease Awaiting Lung Transplantation.

J Clin Med 2020 Apr 17;9(4). Epub 2020 Apr 17.

Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Although the literature demonstrates that cardiac autonomic control (CAC) might be impaired in patients with chronic pulmonary diseases, the interplay between CAC and disease severity in end-stage lung disease has not been studied yet. We investigated the effects of end-stage lung disease on CAC through the analysis of heart rate variability (HRV) among patients awaiting lung transplantation. Forty-nine patients on the waiting list for lung transplantation (LTx; 19 men, age 38 ± 15 years) and 49 healthy non-smoking controls (HC; 22 men, age 40 ± 16 years) were enrolled in a case-control study at Policlinico Hospital in Milan, Italy. Read More

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http://dx.doi.org/10.3390/jcm9041146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230240PMC

Evaluating the Alimentary and Respiratory Tracts in Health and disease (EARTH) research programme: a protocol for prospective, longitudinal, controlled, observational studies in children with chronic disease at an Australian tertiary paediatric hospital.

BMJ Open 2020 Apr 14;10(4):e033916. Epub 2020 Apr 14.

Discipline of Paediatrics, School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia

Introduction: Chronic gastrointestinal and respiratory conditions of childhood can have long-lasting physical, psychosocial and economic effects on children and their families. Alterations in diet and intestinal and respiratory microbiomes may have important implications for physical and psychosocial health. Diet influences the intestinal microbiome and should be considered when exploring disease-specific alterations. Read More

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http://dx.doi.org/10.1136/bmjopen-2019-033916DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200033PMC

The history of respiratory disease management.

Authors:
Duncan Geddes

Medicine (Abingdon) 2020 Apr 4;48(4):239-243. Epub 2020 Mar 4.

is an Honorary Consultant at the Royal Brompton Hospital, London and Professor of Respiratory Medicine at Imperial College London, UK. His main clinical and research interests are cystic fibrosis, lung cancer and emphysema. He was President of the British Thoracic Society and Chairman of Asthma UK. Competing interests: none declared.

Over the past 200 years lung diseases have shifted from infections - tuberculosis, pneumonia - to diseases of dirty air - chronic obstructive pulmonary disease, asthma and lung cancer. New diseases have emerged from industrial pollution and HIV infection, while better imaging has revealed others previously unrecognized. Scientific advances in microbiology, imaging and clinical measurement have improved diagnosis and allowed better targeted treatment. Read More

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http://dx.doi.org/10.1016/j.mpmed.2020.01.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102619PMC

Cystic Fibrosis from Childhood to Adulthood: What Is New in Imaging Assessment?

Radiol Clin North Am 2020 May 26;58(3):475-486. Epub 2020 Feb 26.

Division of Thoracic Imaging, Department of Radiology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA. Electronic address:

Advanced pulmonary disease continues to remain the leading cause of morbidity and mortality in patients with cystic fibrosis (CF), with pulmonary imaging playing a crucial role in early detection, longitudinal monitoring, as well as prelung and postlung transplant evaluation. This article reviews the specific imaging features of CF using conventional imaging modalities (chest radiographs and high-resolution computed tomography [HRCT]) as well as emerging imaging technologies (digital chest tomosynthesis and MR imaging). In addition, the authors review the CF-specific HRCT imaging findings that are essential in the evaluation of these patients in the pre-lung transplant and post-lung transplant settings. Read More

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http://dx.doi.org/10.1016/j.rcl.2019.12.003DOI Listing

Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis.

Thorax 2020 Jun 7;75(6):449-458. Epub 2020 Apr 7.

School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin 2, Ireland

Rationale: Mutations in the cystic fibrosis transmembrane regulator () gene form the basis of cystic fibrosis (CF). There remains an important knowledge gap in CF as to how diminished CFTR activity leads to the dominant inflammatory response within CF airways.

Objectives: To investigate if extracellular vesicles (EVs) contribute to inflammatory signalling in CF. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2019-214027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279202PMC

Seven P's of publication practices.

J Cyst Fibros 2020 May 31;19(3):333-335. Epub 2020 Mar 31.

Cystic Fibrosis Centre, IRCCS Giannina, Gaslini Institute, Genoa, Italy.

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http://dx.doi.org/10.1016/j.jcf.2020.02.007DOI Listing

Bacterial etiology of community-acquired pneumonia in immunocompetent hospitalized patients and appropriateness of empirical treatment recommendations: an international point-prevalence study.

Eur J Clin Microbiol Infect Dis 2020 Apr 3. Epub 2020 Apr 3.

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

An accurate knowledge of the epidemiology of community-acquired pneumonia (CAP) is key for selecting appropriate antimicrobial treatments. Very few etiological studies assessed the appropriateness of empiric guideline recommendations at a multinational level. This study aims at the following: (i) describing the bacterial etiologic distribution of CAP and (ii) assessing the appropriateness of the empirical treatment recommendations by clinical practice guidelines (CPGs) for CAP in light of the bacterial pathogens diagnosed as causative agents of CAP. Read More

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http://dx.doi.org/10.1007/s10096-020-03870-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7222990PMC

Physical therapies for postural abnormalities in people with cystic fibrosis.

Cochrane Database Syst Rev 2020 03 30;3:CD013018. Epub 2020 Mar 30.

Federal University of Rio Grande do Norte, Department of Physical Therapy, Avenida Senador Salgado Filho 3000, Lagoa Nova, Natal, Rio Grande do Norte, Brazil, 59078-970.

Background: Cystic fibrosis (CF) is the most common life-threatening, inherited disease in white populations which causes several dysfunctions, including postural abnormalities. Physical therapy may help in some consequences of these postural abnormalities, such as pain, trunk deformity and quality of life.

Objectives: To determine the effects of a range of physical therapies for managing postural abnormalities in people with cystic fibrosis, specifically on quality of life, pain and trunk deformity. Read More

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http://dx.doi.org/10.1002/14651858.CD013018.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7104790PMC

Early pulmonary function and mid-term outcome in lung transplantation after ex-vivo lung perfusion - a single-center, retrospective, observational, cohort study.

Transpl Int 2020 Jul 26;33(7):773-785. Epub 2020 Apr 26.

Department of Anesthesia, Critical Care and Emergency, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.

Outcomes after transplantation of lungs (LuTX) treated with ex-vivo lung perfusion (EVLP) are debated. In a single-center 8 years of retrospective analysis, we compared: donors' and recipients' characteristics, gas exchange and lung mechanics at ICU admission, 3, 6, and 12 months, and patients' survival of LuTX from standard donors compared with EVLP-treated grafts. A total of 193 LuTX were performed. Read More

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http://dx.doi.org/10.1111/tri.13606DOI Listing

Utility and validity of dynamic chest radiography in cystic fibrosis (dynamic CF): an observational, non-controlled, non-randomised, single-centre, prospective study.

BMJ Open Respir Res 2020 Mar;7(1)

Adult CF Unit, Liverpool Heart and Chest Hospital NHS Trust, Liverpool, UK.

Introduction: Dynamic chest radiography (DCR) uses novel, low-dose radiographic technology to capture images of the thoracic cavity while in motion. Pulmonary function testing is important in cystic fibrosis (CF). The tolerability, rapid acquisition and lower radiation and cost compared with CT imaging may make DCR a useful adjunct to current standards of care. Read More

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http://dx.doi.org/10.1136/bmjresp-2020-000569DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7206905PMC

Single Cell Flow Cytometry Profiling of Bronchoalveolar Lavage in Children.

Am J Respir Cell Mol Biol 2020 Mar 24. Epub 2020 Mar 24.

Murdoch Children's Research Institute, Parkville, Victoria, Australia.

Childhood pulmonary diseases not only cause childhood morbidity and mortality, but can also cause long term pulmonary impairment. The clinical management of many childhood pulmonary diseases is hampered by a limited understanding of the underlying pathophysiological mechanisms. Flow cytometry, which can be used to phenotype individual cell populations or isolate cells for downstream analysis, represents a crucial technology which can help to elucidate the pathophysiology of these conditions. Read More

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http://dx.doi.org/10.1165/rcmb.2019-0453MADOI Listing
March 2020
3.985 Impact Factor

Comparison of a handheld turbine spirometer to conventional spirometry in children with cystic fibrosis.

Pediatr Pulmonol 2020 Jun 23;55(6):1394-1399. Epub 2020 Mar 23.

Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.

Background: In pediatric cystic fibrosis (CF) ambulatory care, handheld spirometry in individual clinic rooms would improve patient flow and potentially reduce patient-to-patient contact. A validation study was conducted to examine the accuracy of an entirely handheld turbine spirometer vs a standard laboratory device in pediatric CF patients.

Methods: Spirometric data were obtained from 76 CF patients aged less than 18 years in the ambulatory setting using the Micro Loop Spirometer (CareFusion) and compared to same-day data from conventional laboratory spirometry. Read More

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http://dx.doi.org/10.1002/ppul.24743DOI Listing

Vitamin D Deficiency and Radiological Findings in Adult Non-Cystic Fibrosis Bronchiectasis.

Turk Thorac J 2020 03 1;21(2):87-92. Epub 2020 Mar 1.

Department of Chest Diseases, Health Science University, Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, İstanbul, Turkey.

Objectives: Vitamin D may play an important role in immunity and its deficiency has been related to increased respiratory infections. The aim of this study was to detect the prevalence of vitamin D deficiency and to investigate the relationship between radiological and clinical effects on adult bronchiectasis (BR) patients.

Materials And Methods: A total of 130 patients with BR and 73 healthy individuals (control group) were enrolled in this study. Read More

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http://dx.doi.org/10.5152/TurkThoracJ.2019.18139DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7089704PMC

Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?

Nutrients 2020 Mar 14;12(3). Epub 2020 Mar 14.

Institute of Pathophysiology and Allergy Research, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, 1090 Vienna, Austria.

Recent research has linked sphingolipid (SL) metabolism with cystic fibrosis transmembrane conductance regulator (CFTR) activity, affecting bioactive lipid mediator sphingosine-1-phosphate (S1P). We hypothesize that loss of CFTR function in cystic fibrosis (CF) patients influenced plasma S1P levels. Total and unbound plasma S1P levels were measured in 20 lung-transplanted adult CF patients and 20 healthy controls by mass spectrometry and enzyme-linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3390/nu12030765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7146441PMC

Infection prevention and chronic disease management in cystic fibrosis and noncystic fibrosis bronchiectasis.

Ther Adv Respir Dis 2020 Jan-Dec;14:1753466620905272

National Jewish Health, 1400 Jackson Street, Denver, CO 80206-2762, USA.

Bronchiectasis is a chronic lung disease (CLD) characterized by irreversible bronchial dilatation noted on computed tomography associated with chronic cough, ongoing viscid sputum production, and recurrent pulmonary infections. Patients with bronchiectasis can be classified into two groups: those with cystic fibrosis and those without cystic fibrosis. Individuals with either cystic fibrosis related bronchiectasis (CFRB) or noncystic fibrosis related bronchiectasis (NCFRB) experience continuous airway inflammation and suffer airway architectural changes that foster the acquisition of a unique polymicrobial community. Read More

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http://dx.doi.org/10.1177/1753466620905272DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7068740PMC

Lung transplantation for cystic fibrosis.

J Heart Lung Transplant 2020 Jun 20;39(6):553-560. Epub 2020 Feb 20.

Toronto Lung Transplant Program, Toronto General Hospital, Toronto, Ontario, Canada; Division of Thoracic Surgery, Toronto General Hospital, Toronto, Ontario, Canada. Electronic address:

Background: The contribution of lung transplantation to the treatment of patients with end-stage cystic fibrosis (CF) has been debated. We aimed to describe achievable outcomes from high-volume CF and lung transplant programs. This study reports on the largest single-center experience of lung transplantation for adult and pediatric patients with CF. Read More

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http://dx.doi.org/10.1016/j.healun.2020.02.010DOI Listing
June 2020
6.650 Impact Factor

Finding the relevance of antimicrobial stewardship for cystic fibrosis.

J Cyst Fibros 2020 Jul 29;19(4):511-520. Epub 2020 Feb 29.

Department of Thoracic Medicine, The Prince Charles Hospital, and QIMR Berghofer Medical Research Institute, Brisbane, QLD, Australia.

Antimicrobials have undoubtedly improved the lives of people with CF, but important antimicrobial-related toxicities and the emergence of antimicrobial-resistant bacteria associated with their use must be considered. Antimicrobial stewardship (AMS) is advocated across the spectrum of healthcare to promote the appropriate use of antimicrobials to preserve their current effectiveness and to optimise treatment, and it is clear that AMS strategies are applicable to and can benefit both non-CF and CF populations. This perspective explores the definition and components of an AMS program, the current evidence for AMS, and the reasons why AMS is a challenging concept in the provision of CF care. Read More

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http://dx.doi.org/10.1016/j.jcf.2020.02.012DOI Listing

Effect of Sex Differences on Computed Tomography Findings in Adults With Cystic Fibrosis: A Multicenter Study.

Arch Bronconeumol 2020 Feb 24. Epub 2020 Feb 24.

Pulmonology Service, Hospital Universitario La Princesa, Madrid, Spain.

Background: The survival of women with cystic fibrosis (CF) is lower than that of men by approximately 5 years. While various factors have been put forward to account for this discrepancy, no specific reasons have been established. Our hypothesis was that anatomical-structural involvement is more pronounced in women with CF than in men and that this is reflected in thoracic HRCT findings. Read More

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http://dx.doi.org/10.1016/j.arbres.2019.12.028DOI Listing
February 2020

Barriers and facilitators to physical activity among children, adolescents, and young adults with cystic fibrosis: a systematic review and thematic synthesis of qualitative research.

BMJ Open 2020 02 20;10(2):e035261. Epub 2020 Feb 20.

Children's Health & Exercise Research Centre, Sport and Health Sciences, University of Exeter, Exeter, UK.

Objectives: Physical activity is widely recommended in the treatment and management of cystic fibrosis (CF). Despite the numerous physical and psychological benefits, many young people with CF are not achieving the recommended levels of physical activity. The aim of this systematic review was to identify and synthesise available qualitative investigations exploring the motives for, barriers to and facilitators of physical activity among young people with CF. Read More

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http://dx.doi.org/10.1136/bmjopen-2019-035261DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045130PMC
February 2020

Etiology and characteristics of patients with bronchiectasis in Taiwan: a cohort study from 2002 to 2016.

BMC Pulm Med 2020 Feb 18;20(1):45. Epub 2020 Feb 18.

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

Background: Bronchiectasis is a chronic infectious respiratory disease with diverse causes and ethnic or geographic differences. However, few large-scale studies of its etiology have been conducted in Asia. This study aimed to determine the etiology and clinical features of bronchiectasis in Taiwan. Read More

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http://dx.doi.org/10.1186/s12890-020-1080-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7029505PMC
February 2020
2.489 Impact Factor

Unilateral temporary diaphragmatic paralysis secondary to bronchial artery embolization in a girl with cystic fibrosis and massive hemoptysis: a case report.

BMC Pulm Med 2020 Feb 11;20(1):38. Epub 2020 Feb 11.

Cystic Fibrosis Centre, Department of Pediatric Medicine, Anna Meyer Children's University Hospital, Viale Gaetano Pieraccini 24, 50139, Florence, Italy.

Background: Massive hemoptysis is a serious complication in Cystic Fibrosis (CF), occurring commonly in older patients. Bronchial artery embolization (BAE) can be performed to stop the bleeding. BAE is generally safe and effective, but can sometimes lead to serious complications. Read More

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http://dx.doi.org/10.1186/s12890-020-1076-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7014762PMC
February 2020

A Prospective Study of Hematologic Complications and Long-Term Survival of Italian Patients Affected by Shwachman-Diamond Syndrome.

J Pediatr 2020 Apr 6;219:196-201.e1. Epub 2020 Feb 6.

Cystic Fibrosis Center, Department of Cardiovascular and Thoracic Surgery, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.

Objective: To describe the hematologic outcome and long-term survival of patients enrolled in the Shwachman-Diamond syndrome Italian Registry.

Study Design: A retrospective and prospective study of patients recorded in the Shwachman-Diamond syndrome Italian Registry.

Results: The study population included 121 patients, 69 males and 52 females, diagnosed between 1999 and 2018. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.12.041DOI Listing

Exendin-4 restores airway mucus homeostasis through the GLP1R-PKA-PPARγ-FOXA2-phosphatase signaling.

Mucosal Immunol 2020 Jul 7;13(4):637-651. Epub 2020 Feb 7.

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

Goblet cell hyperplasia and metaplasia and excessive mucus are prominent pathologies of chronic airway diseases such as chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), and chronic bronchitis. Chronic infection by respiratory pathogens, including Pseudomonas aeruginosa, exacerbates cyclical proinflammatory responses and mucus hypersecretion. P. Read More

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http://dx.doi.org/10.1038/s41385-020-0262-1DOI Listing

Bronchiectasis in rheumatoid arthritis. A clinical appraisial.

Joint Bone Spine 2020 Jan 30. Epub 2020 Jan 30.

Center for Rheumatology, University College London Hospitals NHS Foundation Trust, 235, Euston Road, Bloomsbury, NW1 2BU London, United Kingdom.

Bronchiectasis is defined as irreversibly damaged and dilated bronchi and is one of the most common pulmonary manifestations in patients with rheumatoid arthritis (RA). The model of RA-associated autoimmunity induced in some individuals by chronic bacterial infection in bronchiectasis is becoming increasingly acceptable, although a genetic predisposition to RA-associated bronchiectasis has also been demonstrated. Bronchiectasis should be suspected in RA patients with chronic cough and sputum production or frequent respiratory infections and the diagnosis must be confirmed by thoracic high-resolution computed tomography. Read More

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http://dx.doi.org/10.1016/j.jbspin.2019.12.006DOI Listing
January 2020

Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.

J Cyst Fibros 2020 Jul 24;19(4):e25-e27. Epub 2020 Jan 24.

Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 E. Monument St 5th Floor, Baltimore, MD 21205, United States.

Background: Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy.

Case Summary: We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF). Read More

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http://dx.doi.org/10.1016/j.jcf.2020.01.005DOI Listing
July 2020
3.475 Impact Factor

An atypical acute exacerbation of COPD due to Aspergillus fumigatus.

Monaldi Arch Chest Dis 2020 Jan 27;90(1). Epub 2020 Jan 27.

Department of Pulmonary Medicine, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University.

A 64-year-old male with a history of stabile chronic obstructive pulmonary disease (COPD) presented with increasing dyspnea and sputum for the last two months. Complete blood count showed WBC 14x103/ml, Hgb: 14.2 g/dL and eosinophilia. Read More

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http://dx.doi.org/10.4081/monaldi.2020.1155DOI Listing
January 2020

Early markers of cystic fibrosis structural lung disease: follow-up of the ACFBAL cohort.

Eur Respir J 2020 Apr 3;55(4). Epub 2020 Apr 3.

Dept of Respiratory and Sleep Medicine, Queensland Children's Hospital, Brisbane, Australia.

Little is known about early predictors of later cystic fibrosis (CF) structural lung disease. This study examined early predictors of progressive structural lung abnormalities in children who completed the Australasian CF Bronchoalveolar Lavage (ACFBAL) clinical trial at age 5-years and participated in an observational follow-up study (CF-FAB).Eight Australian and New Zealand CF centres participated in CF-FAB and provided follow-up chest computed-tomography (CT) scans for children who had completed the ACFBAL study with baseline scans at age 5-years. Read More

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http://dx.doi.org/10.1183/13993003.01694-2019DOI Listing

Clinical Use and Barriers of Thoracic Ultrasound: A Survey of Italian Pulmonologists.

Respiration 2020;99(2):171-176. Epub 2020 Jan 10.

Casa Cura Policlinico, Department of Neurorehabilitation Sciences, Milan, Italy.

Introduction: Thoracic ultrasound is accurate in the diagnosis of a wide range of respiratory diseases. Yet the extent of its use is unknown. Through a national survey, we aimed to explore the clinical use of thoracic ultrasound and the barriers to the diffusion of the technique in Italy. Read More

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http://dx.doi.org/10.1159/000504632DOI Listing
January 2020

Outcomes and risk factors identification in urgent lung transplantation: a multicentric study.

J Thorac Dis 2019 Nov;11(11):4746-4754

Thoracic Surgery and Lung Transplantation Unit, University-Hospital of Padova, Padova, Italy.

Background: In rapidly lung deteriorating patients, urgent lung transplantation (ULT) seems the only definitive therapy. Few publications on this topic report conflicting results, putting a word of caution about ULT programs.

Methods: A national ULT program was introduced in 2010: patients on mechanical support may be transplanted with the first available graft. Read More

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http://dx.doi.org/10.21037/jtd.2019.10.55DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6940257PMC
November 2019