2,093 results match your criteria Cystic Fibrosis Thoracic


One Size Does Not Fit All: The Past, Present and Future of Cystic Fibrosis Causal Therapies.

Cells 2022 Jun 8;11(12). Epub 2022 Jun 8.

Molecular Virology and Gene Therapy, Department of Pharmaceutical and Pharmacological Sciences, KU Leuven, 3000 Leuven, Flanders, Belgium.

Cystic fibrosis (CF) is the most common monogenic disorder, caused by mutations in the CF transmembrane conductance regulator () gene. Over the last 30 years, tremendous progress has been made in understanding the molecular basis of CF and the development of treatments that target the underlying defects in CF. Currently, a highly effective CFTR modulator treatment (Kalydeco™/Trikafta™) is available for 90% of people with CF. Read More

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Bronchiectasis: Retrospective Analysis of Clinical and Pathological Findings in a Tertiary-Care Hospital.

Int J Clin Pract 2022 31;2022:8773204. Epub 2022 Jan 31.

Department of Thoracic Surgery, Inonu University School of Medicine, Malatya, Turkey.

Background: Bronchiectasis is still a challenging chronic lung disease in developing countries. Patients with bronchiectasis can also have pulmonary hypertension. There are sparse data on the prevalence of pulmonary hypertension in patients with bronchiectasis. Read More

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Lung Biomolecular Profile and Function of Grafts from Donors after Cardiocirculatory Death with Prolonged Donor Warm Ischemia Time.

J Clin Med 2022 May 29;11(11). Epub 2022 May 29.

Department of Anesthesia, Critical Care and Emergency, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, 20122 Milan, Italy.

The acceptable duration of donor warm ischemia time (DWIT) after cardiocirculatory death (DCD) is still debated. We analyzed the biomolecular profile and function during ex vivo lung perfusion (EVLP) of DCD lungs and their correlation with lung transplantation (LuTx) outcomes. Donor data, procurement times, recipient outcomes, and graft function up to 1 year after LuTx were collected. Read More

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No patient left behind! Therapeutic options for cystic fibrosis patients living with lung transplantation.

J Cyst Fibros 2022 Jun 3. Epub 2022 Jun 3.

Université Paris Cité, Inserm U1016, Institut Cochin, Paris, France; Pulmonary Department and National Cystic Fibrosis Reference Centre, Cochin Hospital; Assistance Publique Hôpitaux de Paris, Paris, France. Electronic address:

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The MUC5B Promoter Polymorphism is Not Associated With Non-ILD Chronic Respiratory Diseases or Post-transplant Outcome.

Transpl Int 2022 16;35:10159. Epub 2022 May 16.

BREATHE, Department of Chronic Diseases and Metabolism, KU Leuven, Leuven, Belgium.

The MUC5B promoter polymorphism (rs35705950) has been associated with interstitial lung disease (ILD) and with prolonged pre-transplant survival in idiopathic pulmonary fibrosis (IPF), but no information is available regarding its prevalence in other respiratory diseases and its influence on post-transplant outcome. We included the Leuven lung transplantation cohort between 1991 and 2015 ( = 801). We assessed the minor allele frequency (MAF) of the MUC5B variant in the entire study cohort and investigated the influence of recipient MUC5B promoter polymorphism on post-transplant outcome in patients who were transplanted after 2004. Read More

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The Role of Zinc in the Pathogenesis of Lung Disease.

Nutrients 2022 May 19;14(10). Epub 2022 May 19.

Department of Thoracic Surgery, Shengjing Hospital, China Medical University, Shenyang 110022, China.

Lung diseases, such as asthma, chronic obstructive pulmonary diseases (COPD), and cystic fibrosis (CF), are among the leading causes of mortality and morbidity globally. They contribute to substantial economic burdens on society and individuals. Currently, only a few treatments are available to slow the development and progression of these diseases. Read More

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ERS International Congress 2021: highlights from the Respiratory Infections Assembly.

ERJ Open Res 2022 Apr 23;8(2). Epub 2022 May 23.

Division of Molecular and Clinical Medicine, Medical School, University of Dundee, Dundee, UK.

The European Respiratory Society International Congress 2021 took place virtually for the second year running due to the coronavirus pandemic. The Congress programme featured more than 400 sessions and 3000 abstract presentations, covering the entire field of respiratory science and medicine. In this article, early career members of the Respiratory Infections Assembly summarise a selection of sessions across a broad range of topics, including presentations on bronchiectasis, non-tuberculosis mycobacteria, tuberculosis, cystic fibrosis and COVID-19. Read More

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British Thoracic Society survey of the career intentions of respiratory medicine specialty trainees in the UK.

BMJ Open Respir Res 2022 04;9(1)

All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.

There were respiratory consultant post vacancies in 82% of surveyed UK hospitals in 2021. Understanding respiratory trainees' career intentions is vital to plan and train a future respiratory workforce. In 2020, the British Thoracic Society surveyed trainee members (n=144) to assess career plans and perceived barriers and facilitators when applying for consultant posts. Read More

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Lung transplantation in the COVID-19 Era: A multi-faceted challenge.

Respir Med Res 2022 05 17;81:100866. Epub 2021 Dec 17.

Paris-Saclay University, School of Medicine, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies Unit, Marie Lannelongue Hospital, Paris Saint-Joseph Hospital Group, Le Plessis-Robinson, France; Marie Lannelongue Hospital, Paris Saint-Joseph Hospital Group, Department of transplantation and thoracic and vascular surgery, Pulmonary Hypertension National Referral Centre, Le Plessis Robinson, France.

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Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients.

J Cyst Fibros 2022 May 4. Epub 2022 May 4.

Department of Biomedical Engineering, Columbia University, New York, NY, USA; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA; College of Dental Medicine, Columbia University Irving Medical Center, New York, NY, USA. Electronic address:

Background: Manifestations of cystic fibrosis, although well-characterized in the proximal airways, are understudied in the distal lung. Characterization of the cystic fibrosis lung 'matrisome' (matrix proteome) has not been previously described, and could help identify biomarkers and inform therapeutic strategies.

Methods: We performed liquid chromatography-mass spectrometry, gene ontology analysis, and multi-modal imaging, including histology, immunofluorescence, and electron microscopy for a comprehensive evaluation of distal human lung extracellular matrix (matrix) structure and composition in end-stage cystic fibrosis. Read More

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Centralized Organ Recovery and Reconditioning Centers.

Thorac Surg Clin 2022 May;32(2):167-174

Department of Surgery, Washington University School of Medicine, Campus Box 8234, 660 South Euclid Avenue, St Louis, MO 63110, USA; Department of Pathology & Immunology, Washington University School of Medicine, Campus Box 8234, 660 South Euclid Avenue, St Louis, MO 63110, USA.

An increased focus on improving efficiency and decreasing costs has resulted in alternative models of donor management and organ recovery. The specialized donor care facility model provides highly efficient and cost-effective donor care at a free-standing facility, resulting in improved organ yield, shorter ischemic times, decreased travel, and fewer nighttime operations. Ex vivo lung perfusion (EVLP) improves utilization of extended criteria donor lungs, and centralized EVLP facilities have the potential to increase transplant volumes for smaller transplant programs in specified geographic regions. Read More

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Impacts of Nontuberculous Mycobacteria Isolates in Non-cystic Fibrosis Bronchiectasis: A 16-Year Cohort Study in Taiwan.

Front Microbiol 2022 18;13:868435. Epub 2022 Apr 18.

Department of Thoracic Medicine, Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Background: The prevalence of nontuberculous mycobacteria (NTM) in patients with chronic respiratory disease has increased. The implication of NTM in non- bronchiectasis remained controversial. This study investigated the impact of NTM in non- bronchiectasis in Taiwan. Read More

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Invited editorial: Q and A on hereditary lung cancer.

Respir Med Res 2022 May 30;81:100881. Epub 2022 Apr 30.

Department of Pneumology and Thoracic Oncology, Tenon Hospital, Assistance Publique Hôpitaux de Paris and GRC 4, Theranoscan, Sorbonne Université, Paris, France. Electronic address:

Advances in the field of genetic susceptibility to respiratory diseases (e.g. pulmonary fibrosis, emphysema, cystic fibrosis, pulmonary hypertension) have led pneumologists to integrate the familial risk dimension and work with genetics clinical teams and laboratories. Read More

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Cumulative Incidence and Risk Factors for Severe COVID-19 in French People with Cystic Fibrosis.

Clin Infect Dis 2022 Apr 27. Epub 2022 Apr 27.

Respiratory Medicine and National Reference CF Center, AP-HP, Hôpital Cochin, Paris, France.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections are closely monitored in people with cystic fibrosis (pwCF), with a special emphasis on severe cases. Previous studies used hospitalization rates as proxy for severity.

Methods: We evaluated data from coronavirus disease 2019 (COVID-19) cases diagnosed in French pwCF followed in one of the 47 French CF center over the first year of the pandemic. Read More

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Evaluation of clinically relevant changes in the lung clearance index in children with cystic fibrosis and healthy controls.

Thorax 2022 Apr 15. Epub 2022 Apr 15.

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

Background: The limits of reproducibility of the lung clearance index (LCI) are higher in children with cystic fibrosis (CF) compared with healthy children, and it is currently unclear what defines a clinically meaningful change.

Methods: In a prospective multisite observational study of children with CF and healthy controls (HCs), we measured LCI, FEV% predicted and symptom scores at quarterly visits over 2 years. Two reviewers performed a detailed review of visits to evaluate the frequency that between visit LCI changes outside ±10%, ±15%, ±20% represented a clinically relevant signal. Read More

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Feasibility and efficacy of combined pancreatic islet-lung transplantation in cystic fibrosis-related diabetes-PIM study: A multicenter phase 1-2 trial.

Am J Transplant 2022 Apr 11. Epub 2022 Apr 11.

Department of Endocrinology, Diabetes and Nutrition, Strasbourg University Hospital, France.

Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis (CF), and restoring metabolic control in these patients may improve their management after lung transplantation. In this multicenter, prospective, phase 1-2 trial, we evaluate the feasibility and metabolic efficacy of combined pancreatic islet-lung transplantation from a single donor in patients with CFRD, terminal respiratory failure, and poorly controlled diabetes. Islets were infused via the portal vein under local anesthesia, 1 week after lung transplantation. Read More

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Surprise Foreign Body Aspiration with Pistachios in a Patient with Cystic Fibrosis with Persistent Atelectasis on Chest Radiography.

Turk Arch Pediatr 2022 Mar;57(2):247-248

Department of Pediatric Allergy and Immunology, Gaziantep University, Faculty of Medicine, Gaziantep, Turkey.

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Microbial Community Composition in Explanted Cystic Fibrosis and Control Donor Lungs.

Front Cell Infect Microbiol 2021 16;11:764585. Epub 2022 Mar 16.

Halo Research Group, School of Pharmacy, Queen's University Belfast, Belfast, United Kingdom.

To date, investigations of the microbiota in the lungs of people with Cystic Fibrosis (PWCF) have primarily focused on microbial community composition in luminal mucus, with fewer studies observing the microbiota in tissue samples from explanted lung tissue. Here, we analysed both tissue and airway luminal mucus samples extracted from whole explanted lungs of PWCF and unused donor lungs. We determined if the lung microbiota in end-stage CF varied within and between patients, was spatially heterogeneous and related to localized structural damage. Read More

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The 49th parallel: Does geographic position affect longevity of patients with cystic fibrosis?

J Thorac Cardiovasc Surg 2022 Mar 1. Epub 2022 Mar 1.

Division of Thoracic Surgery, School of Medicine, University of Pennsylvania, Philadelphia, Pa. Electronic address:

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Efficacy of -acetylcysteine on idiopathic or postinfective non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis protocol.

BMJ Open 2022 03 31;12(3):e053625. Epub 2022 Mar 31.

Division of Respiratory Medicine, Department of Integrated Traditional and Western Medicine, Sichuan University of West China Hospital, Chengdu, Sichuan, China

Introduction: Non-cystic fibrosis (non-CF) bronchiectasis is a chronic pulmonary disorder that causes destruction and permanent dilatation of the airways, resulting in excessive sputum production, repeated infection and inflammation. A need for high-quality and specialised care has been highlighted in recent years. -acetylcysteine (NAC) is a widely used mucolytic agent in respiratory diseases that not only possesses a property to enhance secretion clearance, but also exhibits antioxidant and anti-inflammatory effects. Read More

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Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials.

Front Pharmacol 2022 14;13:863280. Epub 2022 Mar 14.

Department of Thoracic Surgery, The First Hospital of China Medical University, Shenyang, China.

Cystic fibrosis is a rare, recessive, progressive genetic disease caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Small molecules have recently been developed to treat the molecular consequences of CFTR mutations and restore CFTR protein function. However, the data on triple combination therapy (mainly from Vertex Pharmaceuticals, which is most tested in clinical trials) are limited. Read More

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HPV-associated complications post lung transplantation: why prevention is better than 'no' cure.

Thorax 2022 Jun 30;77(6):539. Epub 2022 Mar 30.

Cardiothoracic Transplant Unit, Manchester University NHS Foundation Trust, Manchester, UK.

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Joint patient and clinician priority setting to identify 10 key research questions regarding the long-term sequelae of COVID-19.

Thorax 2022 Jul 30;77(7):717-720. Epub 2022 Mar 30.

Respiratory Epidemiology, Occupational Medicine and Public Health, Imperial College London, London, UK.

Given the large numbers of people infected and high rates of ongoing morbidity, research is clearly required to address the needs of adult survivors of COVID-19 living with ongoing symptoms (long COVID). To help direct resource and research efforts, we completed a research prioritisation process incorporating views from adults with ongoing symptoms of COVID-19, carers, clinicians and clinical researchers. The final top 10 research questions were agreed at an independently mediated workshop and included: identifying underlying mechanisms of long COVID, establishing diagnostic tools, understanding trajectory of recovery and evaluating the role of interventions both during the acute and persistent phases of the illness. Read More

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Azole-Induced Myositis after Combined Lung-Liver Transplantation.

Case Rep Transplant 2022 25;2022:7323755. Epub 2022 Mar 25.

Department of Respiratory Diseases and Lung Transplantation, University Hospital Gasthuisberg, Leuven, Belgium.

Lung transplant recipients experience a high rate of invasive pulmonary aspergillosis infections, for which voriconazole is the treatment of choice. We report a patient who developed voriconazole-induced myositis that relapsed after one dose of isavuconazole. Our patient was a 38-year-old man who received a single sequential lung transplantation and liver transplantation because of end-stage cystic fibrosis. Read More

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Profile of Clinical and Analytical Parameters in Bronchiectasis Patients during the COVID-19 Pandemic: A One-Year Follow-Up Pilot Study.

J Clin Med 2022 Mar 21;11(6). Epub 2022 Mar 21.

Muscle Wasting and Cachexia in Chronic Respiratory Diseases and Lung Cancer Research Group, Pulmonology Department, IMIM-Hospital del Mar, Parc de Salut Mar, Parc de Recerca Biomèdica de Barcelona (PRBB), 08003 Barcelona, Spain.

Whether the COVID-19 pandemic may have modified the clinical planning and course in bronchiectasis patients remains to be fully elucidated. We hypothesized that the COVID-19 pandemic may have influenced the management and clinical outcomes of bronchiectasis patients who were followed up for 12 months. In bronchiectasis patients (n = 30, 23 females, 66 years), lung function testing, disease severity [FEV, age, colonization, radiological extension, dyspnea (FACED), exacerbation (EFACED)] and dyspnea scores, exacerbation numbers and hospitalizations, body composition, sputum microbiology, and blood analytical biomarkers were determined at baseline and after a one-year follow-up. Read More

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Implementation of prognostic machine learning algorithms in paediatric chronic respiratory conditions: a scoping review.

BMJ Open Respir Res 2022 03;9(1)

Community Health and Epidemiology, Dalhousie University, Halifax, Nova Scotia, Canada.

Machine learning (ML) holds great potential for predicting clinical outcomes in heterogeneous chronic respiratory diseases (CRD) affecting children, where timely individualised treatments offer opportunities for health optimisation. This paper identifies rate-limiting steps in ML prediction model development that impair clinical translation and discusses regulatory, clinical and ethical considerations for ML implementation. A scoping review of ML prediction models in paediatric CRDs was undertaken using the PRISMA extension scoping review guidelines. Read More

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Use of Dynamic Chest Radiography to Assess Treatment of Pulmonary Exacerbations in Cystic Fibrosis.

Radiology 2022 06 15;303(3):675-681. Epub 2022 Mar 15.

From the Adult CF Unit (T.S.F., D.S.N., M.J.W.) and Department of Radiology (C.M.), Liverpool Heart and Chest Hospital, Thomas Drive, Liverpool L14 3PE, UK; Institute of Life Course and Medical Sciences (T.S.F., P.S.M.) and Institute of Infection and Global Health (D.S.N., M.J.W.), University of Liverpool, Liverpool, UK; and Institute in the Park (University of Liverpool), Alder Hey Children's Hospital, Liverpool, UK (P.S.M.).

Background Although spirometry is an important marker in the management of pulmonary exacerbations in cystic fibrosis (CF), it is a forced maneuver and can generate aerosol. Therefore, it may be difficult to perform in some individuals. Dynamic chest radiography (DCR) provides real-time information regarding pulmonary dynamics alongside fluoroscopic-style thoracic imaging. Read More

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Three-hole oesophagectomy following bilateral lung transplant for cystic fibrosis.

BMJ Case Rep 2022 Mar 14;15(3). Epub 2022 Mar 14.

Thoracic Surgery, Vancouver Coastal Health Authority, Vancouver, British Columbia, Canada.

Cystic fibrosis (CF) is associated with increased rates of malignancy, particularly in lung transplant recipients requiring long-term immunosuppression. We present a unique case of post-bilateral lung transplant (LTx) three-hole oesophagectomy for de-novo oesophageal adenocarcinoma. Preoperative planning and careful fluid management allowed for a successful treatment course. Read More

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