1,703 results match your criteria Cystic Fibrosis Thoracic


Trial Refresh: A Case for an Adaptive Platform Trial for Pulmonary Exacerbations of Cystic Fibrosis.

Front Pharmacol 2019 28;10:301. Epub 2019 Mar 28.

Wesfarmers Centre of Vaccines & Infectious Diseases, Telethon Kids Institute, The University of Western Australia, Nedlands, WA, Australia.

Cystic fibrosis is a genetic disease typically characterized by progressive lung damage and premature mortality. Pulmonary exacerbations, or flare-ups of the lung disease, often require hospitalization for intensive treatment. Approximately 25% of patients with cystic fibrosis do not recover their baseline lung function after pulmonary exacerbations. Read More

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http://dx.doi.org/10.3389/fphar.2019.00301DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6447696PMC

Shadow cost of oral corticosteroids-related adverse events: A pharmacoeconomic evaluation applied to real-life data from the Severe Asthma Network in Italy (SANI) registry.

World Allergy Organ J 2019 26;12(1):100007. Epub 2019 Jan 26.

Personalized Medicine, Asthma and Allergy, Humanitas Research Hospital, Rozzano, MI, Italy.

Background: Asthma is one of the most common non-communicable respiratory diseases, affecting about 6% of the general population. Severe asthma, even if afflicts a minority of asthmatics, drives the majority of costs of the disease. The aim of this study is to create a pharmacoeconomic model to predict the costs of corticosteroid-related adverse events in severe asthmatics and applying it to the first published epidemiologic data from the Severe Asthma Network in Italy (SANI) registry. Read More

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http://dx.doi.org/10.1016/j.waojou.2018.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439414PMC
January 2019
2 Reads

Management and clinical outcomes after lung transplantation in patients with pre-transplant Mycobacterium abscessus infection: A single center experience.

Transpl Infect Dis 2019 Mar 29:e13084. Epub 2019 Mar 29.

Department of Medicine, University of California, San Francisco.

Background: Preoperative Mycobacterium abscessus infection is often considered a contraindication to lung transplantation because of its association with poor outcomes after transplant. Detailed strategies for bridging to transplant, post-operative management, and data regarding outcomes are lacking.

Methods: We reviewed outcomes in subjects with M abscessus infection who underwent lung transplantation between 2010 and 2018 at the University of California San Francisco. Read More

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http://dx.doi.org/10.1111/tid.13084DOI Listing
March 2019
1 Read

Congenital emphysematous lung disease associated with a novel Filamin A mutation. Case report and literature review.

BMC Pediatr 2019 Mar 29;19(1):86. Epub 2019 Mar 29.

Pediatrics and Adolescentology Unit, Department of Internal Medicine University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Background: Progressive lung involvement in Filamin A (FLNA)-related cerebral periventricular nodular heterotopia (PVNH) has been reported in a limited number of cases.

Case Presentation: We report a new pathogenic FLNA gene variant (c.7391_7403del; p. Read More

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https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887
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http://dx.doi.org/10.1186/s12887-019-1460-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440113PMC
March 2019
3 Reads

Transmission of bacteria in bronchiectasis and chronic obstructive pulmonary disease: Low burden of cough aerosols.

Respirology 2019 Mar 27. Epub 2019 Mar 27.

Lung Bacteria Group, QIMR Berghofer Medical Research Institute, Brisbane, QLD, Australia.

Background And Objective: Aerosol transmission of Pseudomonas aeruginosa has been suggested as a possible mode of respiratory infection spread in patients with cystic fibrosis (CF); however, whether this occurs in other suppurative lung diseases is unknown. Therefore, we aimed to determine if (i) patients with bronchiectasis (unrelated to CF) or chronic obstructive pulmonary disease (COPD) can aerosolize P. aeruginosa during coughing and (ii) if genetically indistinguishable (shared) P. Read More

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http://dx.doi.org/10.1111/resp.13544DOI Listing
March 2019
1 Read

Isolated pulmonary interstitial glycogenosis associated with alveolar growth abnormalities: A long-term follow-up study.

Pediatr Pulmonol 2019 Mar 25. Epub 2019 Mar 25.

Pediatric Allergy, Pulmonary and Cystic Fibrosis Section, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

Introduction: Pulmonary interstitial glycogenosis (PIG) is a rare infant interstitial lung disease characterized by an increase in the number of interstitial mesenchymal cells, presenting as enhanced cytoplasmic glycogen, and is considered to represent the expression of an underlying lung development disorder.

Methods: This study describes the clinical, radiological, and functional characteristics and long-term outcomes (median 12 years) of nine infants diagnosed with isolated PIG associated with alveolar simplification in the absence of other diseases.

Results: All patients presented with tachypnea. Read More

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http://dx.doi.org/10.1002/ppul.24324DOI Listing
March 2019
1 Read

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
1 Read

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
1 Read
1.671 Impact Factor

Cystic fibrosis-related diabetes before lung transplantation is associated with lower survival but does not affect long-term renal function.

Pediatr Pulmonol 2019 Mar 10. Epub 2019 Mar 10.

Department of Internal Medicine, Adult Cystic Fibrosis Care Center, Hospices Civils de Lyon, Lyon, France.

Objective: To describe the prevalence of cystic fibrosis-related diabetes (CFRD) before and after lung transplantation (LT); to analyse the survival and renal function after LT according to the CFRD status before LT.

Methods: Sixty cystic fibrosis (CF) patients transplanted at the Lyon University Hospital between 2004 and 2014 were included. Genotype, pancreatic status, age at LT, survival were recorded. Read More

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http://dx.doi.org/10.1002/ppul.24307DOI Listing
March 2019
2 Reads

Extracorporeal membrane oxygenation after lung transplantation: risk factors and outcomes analysis.

Ann Cardiothorac Surg 2019 Jan;8(1):54-61

Cardiac Surgery Division, Surgical Sciences Department, Città della Salute e della Scienza, University of Torino, Turin, Italy.

Background: Lung transplantation is the treatment of choice for end-stage pulmonary disease in selected patients. However, severe primary graft dysfunction is a significant complication of transplant and requires the implantation of an extracorporeal support. The aim of the study is to evaluate the impact of extracorporeal membrane oxygenation (ECMO) after transplant in our center. Read More

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http://dx.doi.org/10.21037/acs.2018.12.10DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6379200PMC
January 2019
1 Read

Improving Clinical Outcomes and Quality of Life with Massage Therapy in Youth and Young Adults with Cystic Fibrosis: a Pilot Study.

Int J Ther Massage Bodywork 2019 Mar 4;12(1):4-15. Epub 2019 Mar 4.

Patient Services, Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Background: Cystic Fibrosis (CF) is an autosomal recessive disorder of exocrine glands characterized by abnormal production of thick mucus, primarily in bronchi of the lungs. Individuals experience recurrent respiratory infections, increased work of breathing, cough and musculoskeletal changes with pain. Previous research found that massage therapy (MT) decreased pain, muscle tightness, and anxiety in individuals with CF, but did not use valid/reliable measurements of quality of life (QOL). Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6398987PMC
March 2019
1 Read

Origins of growth deficiencies in cystic fibrosis.

Thorax 2019 May 23;74(5):423-424. Epub 2019 Feb 23.

Pediatrics, Children's Hospital of Richmond at Virginia Commonwealth University, Richmond, Virginia, USA

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http://dx.doi.org/10.1136/thoraxjnl-2019-213076DOI Listing
May 2019
2 Reads

National Survey on the Management of Adult Bronchiectasis in Belgium.

COPD 2019 Feb 21:1-3. Epub 2019 Feb 21.

a Department of Respiratory Medicine , University Hospitals Leuven , Leuven , Belgium.

The increasing prevalence and incidence of bronchiectasis leads to a substantial health care burden. Quality standards for the management of bronchiectasis were formulated by the British Thoracic Society following publication of guidelines in 2010. They can be used as a benchmark for quality of care. Read More

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http://dx.doi.org/10.1080/15412555.2019.1566895DOI Listing
February 2019

Severe asthma and bronchiectasis.

J Asthma 2019 Feb 20:1-5. Epub 2019 Feb 20.

a Department of Pneumology , Hospital Universitario Central de Asturias , Oviedo , Spain.

Objective: The aim of our study was to determine the tomographic findings and prevalence of bronchiectasis in our population of patients with severe asthma, and to identify factors associated with the presence of bronchiectasis in these patients.

Materials And Methods: We retrospectively collected data from the medical histories of patients referred to the asthma unit of our hospital, with a diagnosis of severe asthma between 2015 and 2017. Patients with ABPA, cystic fibrosis, immunodeficiency or systemic disease were excluded. Read More

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http://dx.doi.org/10.1080/02770903.2019.1579832DOI Listing
February 2019
1 Read

Successful control of exacerbation of Allergic Bronchopulmonary Aspergillosis due to Aspergillus terreus in a cystic fibrosis patient with short-term adjunctive therapy with voriconazole: A case report.

J Mycol Med 2019 Feb 16. Epub 2019 Feb 16.

Invasive Fungi Research Center (IFRC)/ Department of Medical Mycology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran. Electronic address:

A 12-year-old boy with cystic fibrosis (CF) and a history of glucocorticoid-dependent allergic bronchopulmonary aspergillosis (ABPA) was referred to our hospital. The ABPA was diagnosed when he was 8 years old and he had been treated with several course of oral glucocorticoids for recurrent exacerbations. He was readmitted when aged 12 with a history of worsening shortness of breath and chest tightness. Read More

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http://dx.doi.org/10.1016/j.mycmed.2019.02.001DOI Listing
February 2019
1 Read

Help, hinder, hide and harm: what can we learn from the interactions between and during respiratory infections?

Thorax 2019 Feb 18. Epub 2019 Feb 18.

Departments of Pediatrics and Microbiology, University of Washington, Seattle, Washington, USA.

Recent studies of human respiratory secretions using culture-independent techniques have found a surprisingly diverse array of microbes. Interactions among these community members can profoundly impact microbial survival, persistence and antibiotic susceptibility and, consequently, disease progression. Studies of polymicrobial interactions in the human microbiota have shown that the taxonomic and structural compositions, and resulting behaviours, of microbial communities differ substantially from those of the individual constituent species and in ways of clinical importance. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2018-212616DOI Listing
February 2019
2 Reads

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

Magn Reson Med 2019 Jun 8;81(6):3808-3818. 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
June 2019
2 Reads

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

Thorax 2019 Apr 7;74(4):323-325. 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
April 2019
3 Reads

Outcomes in cystic fibrosis lung transplant recipients infected with organisms labeled as pan-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
5 Reads

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
3 Reads

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
14 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 May 28;276(5):1341-1347. 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
May 2019
8 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
18 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
8 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
12 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
17 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
14 Reads

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

Clin Transplant 2019 Mar 21;33(3):e13480. Epub 2019 Feb 21.

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 is 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
March 2019
9 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
13 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
March 2019
9 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
March 2019
15 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
March 2019
9 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
March 2019
7 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
13 Reads

A young woman with upper lobe predominant bronchiectasis.

BMJ 2019 Jan 10;364:k5244. Epub 2019 Jan 10.

Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Florida College of Medicine, Gainesville, FL, USA.

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http://dx.doi.org/10.1136/bmj.k5244DOI Listing
January 2019
3 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
5 Reads

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

Transpl Infect Dis 2019 Apr 13;21(2):e13046. Epub 2019 Jan 13.

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
April 2019
13 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 12 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
7 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
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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 2019 Apr 19;47(2):289-292. 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
April 2019
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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
2 Reads

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
2 Reads

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
16 Reads

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

JCI Insight 2018 11 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
14 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
5 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
19 Reads

Increased expression of ATP12A proton pump in cystic fibrosis airways.

JCI Insight 2018 10 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
10 Reads