1,959 results match your criteria Cystic Fibrosis Thoracic

Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis.

J Cyst Fibros 2021 May 21;20(3):525-532. Epub 2020 Oct 21.

Department of Nutrition, Faculty of Medicine, Université de Montreal, 2405 Cote Sainte-Catherine Rd, Montreal, Quebec H3T 1A8, Canada; Research Centre, CHU Sainte-Justine, 3175 Cote Sainte-Catherine Rd, Montreal, Quebec H3T 1C5, Canada. Electronic address:

Background: Little is known about long-term bone mineral density (BMD) changes and fractures in lung transplant recipients with cystic fibrosis (CF). We examined femur and lumbar spine (LS) BMD changes in men and women with CF up to 10 years post-transplant and documented post-transplant fracture prevalence.

Methods: Retrospective study of individuals who had undergone a lung transplant (2000-2015) and had a pre-transplant and at least one BMD measurement after transplant. Read More

View Article and Full-Text PDF

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

SAGE Open Med Case Rep 2021 20;9:2050313X211016993. Epub 2021 May 20.

Department of General, Visceral, Thoracic and Vascular Surgery, Greifswald University Medical Centre, Greifswald, Germany.

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. Read More

View Article and Full-Text PDF

The Anti-Infectious Role of Sphingosine in Microbial Diseases.

Cells 2021 05 4;10(5). Epub 2021 May 4.

Department of Molecular Biology, University of Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany.

Sphingolipids are important structural membrane components and, together with cholesterol, are often organized in lipid rafts, where they act as signaling molecules in many cellular functions. They play crucial roles in regulating pathobiological processes, such as cancer, inflammation, and infectious diseases. The bioactive metabolites ceramide, sphingosine-1-phosphate, and sphingosine have been shown to be involved in the pathogenesis of several microbes. Read More

View Article and Full-Text PDF

Airway mucin MUC5AC and MUC5B concentrations and the initiation and progression of chronic obstructive pulmonary disease: an analysis of the SPIROMICS cohort.

Lancet Respir Med 2021 May 28. Epub 2021 May 28.

Marsico Lung Institute/Cystic Fibrosis and Pulmonary Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA. Electronic address:

Background: We previously described the contributions of increased total airway mucin concentrations to the pathogenesis and diagnosis of the chronic bronchitic component of chronic obstructive pulmonary disease (COPD). Here, we investigated the relative contribution of each of the major airway gel-forming mucins, MUC5AC and MUC5B, to the initiation, progression, and early diagnosis of airways disease in COPD.

Methods: SPIROMICS was a multicentre, observational study in patients aged 40-80 years recruited from six clinical sites and additional subsites in the USA. Read More

View Article and Full-Text PDF

Would it have happened without Christmas? Thoracic emphysema and allergic bronchopulmonary aspergillosis in a juvenile cystic fibrosis patient with the G551D mutation receiving ivacaftor.

ERJ Open Res 2021 Apr 24;7(2). Epub 2021 May 24.

Cystic Fibrosis Center, Brandenburg Medical School (MHB) University, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

https://bit.ly/2P96PTy. Read More

View Article and Full-Text PDF

Gastric cancer, inflammatory bowel disease and polyautoimmunity in a 17-year-old boy: CTLA-4 deficiency successfully treated with Abatacept.

Eur J Gastroenterol Hepatol 2021 May 21. Epub 2021 May 21.

Unit of Digestive Endoscopy and Surgery Unit of Immune and Infectious Diseases, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, Scientific Institute for Research and Healthcare Department of Systems Medicine, University of Rome Tor Vergata Department of Pathology and Molecular Histopathology Unit of General and Thoracic Surgery, Department of Pediatric Surgery Department of Pediatric Onco-Hematology, Cell and Gene Therapy, Bambino Gesù Children's Hospital - Scientific Institute for Research and Healthcare, Rome Unit of Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood 'G. Barresi,' University of Messina, Messina Unit of Pediatric Oncology and Hematology, Department of Pediatrics, Vittorio Emanuele University Hospital, University of Catania, Catania Translational Cytogenomics Research Unit, Laboratory of Medical Genetics, Bambino Gesù Children's Hospital, Scientific Institute for Research and Healthcare, Rome, Italy.

Gut involvement is frequent in immunologic disorders, especially with inflammatory manifestations but also with cancer. In the last years, advances in functional and genetic testing have improved the diagnostic and therapeutic approach to immune dysregulation syndromes. CTLA-4 deficiency is a rare disease with variable phenotype, ranging from absence of symptoms to severe multisystem manifestations and complications. Read More

View Article and Full-Text PDF

The Lung Allocation Score and Its Relevance.

Semin Respir Crit Care Med 2021 Jun 24;42(3):346-356. Epub 2021 May 24.

Division of Pulmonary & Critical Care Medicine, Department of Internal Medicine, Michigan Medicine/University of Michigan School of Medicine, Ann Arbor, Michigan.

Lung transplantation in the United States, under oversight by the Organ Procurement Transplantation Network (OPTN) in the 1990s, operated under a system of allocation based on location within geographic donor service areas, wait time of potential recipients, and ABO compatibility. On May 4, 2005, the lung allocation score (LAS) was implemented by the OPTN Thoracic Organ Transplantation Committee to prioritize patients on the wait list based on a balance of wait list mortality and posttransplant survival, thus eliminating time on the wait list as a factor of prioritization. Patients were categorized into four main disease categories labeled group A (obstructive lung disease), B (pulmonary hypertension), C (cystic fibrosis), and D (restrictive lung disease/interstitial lung disease) with variables within each group impacting the calculation of the LAS. Read More

View Article and Full-Text PDF

Effects of bilateral lung transplantation on cardiac autonomic modulation and cardiorespiratory coupling: a prospective study.

Respir Res 2021 May 21;22(1):156. Epub 2021 May 21.

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

Background: Although cardiac autonomic modulation has been studied in several respiratory diseases, the evidence is limited on lung transplantation, particularly on its acute and chronic effects. Thus, we aimed to evaluate cardiac autonomic modulation before and after bilateral lung transplantation (BLT) through a prospective study on patients enrolled while awaiting transplant.

Methods: Twenty-two patients on the waiting list for lung transplantation (11 women, age 33 [24-51] years) were enrolled in a prospective study at Ospedale Maggiore Policlinico Hospital in Milan, Italy. Read More

View Article and Full-Text PDF

Robotic thoracic surgery for inflammatory and infectious lung disease: initial experience in Brazil.

Rev Col Bras Cir 2021 17;48:e20202872. Epub 2021 May 17.

- Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, Departamento de Cirurgia Torácica - São Paulo - SP - Brasil.

Objective: in Latin America, especially Brazil, the use of a robotic platform for thoracic surgery is gradually increasing in recent years. However, despite tuberculosis and inflammatory pulmonary diseases are endemic in our country, there is a lack of studies describing the results of robotic surgical treatment of bronchiectasis. This study aims to evaluate the surgical outcomes of robotic surgery for inflammatory and infective diseases by determining the extent of resection, postoperative complications, operative time, and length of hospital stay. Read More

View Article and Full-Text PDF

Clinical Pharmacokinetic and Pharmacodynamic Considerations in the Drug Treatment of Non-Tuberculous Mycobacteria in Cystic Fibrosis.

Clin Pharmacokinet 2021 May 13. Epub 2021 May 13.

Faculty of Medicine, University of Queensland Centre for Clinical Research, The University of Queensland, Brisbane, QLD, Australia.

Non-tuberculous mycobacteria (NTM) are an emerging group of pulmonary infectious pathogens of increasing importance to the management of patients with cystic fibrosis (CF). NTM include slow-growing mycobacteria such as Mycobacterium avium complex (MAC) and rapidly growing mycobacteria such as Mycobacterium abscessus. The incidence of NTM in the CF population is increasing and infection contributes to significant morbidity to the patient and costs to the health system. Read More

View Article and Full-Text PDF

Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies.

Respirol Case Rep 2021 Jun 7;9(6):e00755. Epub 2021 May 7.

Department of Internal Medicine University of Texas Southwestern Medical Center Dallas TX USA.

Catamenial haemoptysis, the expectoration of blood during menses, has not been extensively reported in the cystic fibrosis (CF) literature. We describe four cases (age range: 25-34 years) of catamenial haemoptysis across four CF centres in the United States. These cases may represent thoracic endometriosis versus hormonal fluctuations in airway inflammation or infection resulting in bronchial artery bleeding. Read More

View Article and Full-Text PDF

Lung function in children with cystic fibrosis in the USA and UK: a comparative longitudinal analysis of national registry data.

Thorax 2021 May 11. Epub 2021 May 11.

Department of Public Health, Policy and Systems, University of Liverpool, Liverpool, UK.

Rationale: A previous analysis found significantly higher lung function in the US paediatric cystic fibrosis (CF) population compared with the UK with this difference apparently decreasing in adolescence and adulthood. However, the cross-sectional nature of the study makes it hard to interpret these results.

Objectives: To compare longitudinal trajectories of lung function in children with CF between the USA and UK and to explore reasons for any differences. Read More

View Article and Full-Text PDF

Combination CFTR modulator therapy in children and adults with cystic fibrosis.

Lancet Respir Med 2021 May 6. Epub 2021 May 6.

Division of Pulmonary, Critical Care, and Sleep Medicine, and Division of Pediatric Pulmonary Medicine, National Jewish Health, Denver, CO 80206, USA. Electronic address:

View Article and Full-Text PDF

The effect of diet in infancy on asthma in young adults: the Merthyr Allergy Prevention Study.

Thorax 2021 May 7. Epub 2021 May 7.

Respiratory/Cystic Fibrosis Unit, Children's Hospital for Wales, Cardiff, UK

Introduction: Early infant diet might influence the risk of subsequent allergic disease.

Methods: The Merthyr Allergy Prevention Study (MAPS) was a randomised controlled trial in infants at high risk of allergic disease. The trial determined whether a cow's milk exclusion diet for the first 4 months of life decreased the risk of allergic disease including asthma compared with a normal diet. Read More

View Article and Full-Text PDF

Physiotherapist involvement in the pandemic era: a Lombardy region survey.

Monaldi Arch Chest Dis 2021 May 4. Epub 2021 May 4.

Italian Association of Respiratory Physiotherapists (ARIR); Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan.

In February 2020 the first COVID-19 case was reported in Italy and afterwards the virus started spreading rapidly, increasing dramatically the number of infected subjects. To face the pandemic outbreak, hospitals converted wards to assure COVID-19 patients' care and adopted measures to reduce virus diffusion. The aim of this study was to determine how many physiotherapists, in the Lombardy region, worked during pandemic, whether they continued their usual practice or were employed in COVID-19 wards and in which tasks they were involved. Read More

View Article and Full-Text PDF

Patient personalized translational tools in Cystic fibrosis to transform data from bench to bed-side and back.

Am J Physiol Gastrointest Liver Physiol 2021 May 5. Epub 2021 May 5.

Division of Pulmonary Medicine, grid.239573.9Cincinnati Children's Hospital Medical Center, United States.

Cystic fibrosis is a deadly multi-organ disorder caused by loss of function mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator (CFTR) chloride/bicarbonate ion channel. More than 1700 CFTR genetic variants exist that can cause CF, and, majority of these are extremely rare. Due to genetic and environmental influences, CF patients exhibit large phenotypic variation. Read More

View Article and Full-Text PDF

Lung transplantation for non-cystic fibrosis bronchiectasis in Turkey: Initial institutional experience.

Asian J Surg 2021 Apr 28. Epub 2021 Apr 28.

Thoracic Surgery, Kartal Kosuyolu Training and Research Hospital, Istanbul, Turkey.

Background/objective: Lung transplantation is a well-established treatment in patients who have bronchiectasis with diffuse involvement, and with a progressive decline in respiratory function despite maximal medical therapy. We have aimed to present pre-transplantation factors and our results of lung transplantation for non-cystic fibrosis bronchiectasis.

Methods: Patients who underwent lung transplantation for non-cystic fibrosis bronchiectasis between the dates of December 2016 and July 2019 were included. Read More

View Article and Full-Text PDF

Huff and puff of exercise for airway clearance in cystic fibrosis: how clear is the evidence?

Tiffany Dwyer

Thorax 2021 Apr 29. Epub 2021 Apr 29.

Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia

View Article and Full-Text PDF

Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.

Thorax 2021 Apr 29. Epub 2021 Apr 29.

Department of Respiratory Medicine, Children's Hospital at Westmead, Westmead, New South Wales, Australia

Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV decline in adolescence and adulthood have not slowed. Read More

View Article and Full-Text PDF

Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.

Eur Respir J 2021 May 28. Epub 2021 May 28.

Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France

Introduction: A reduction in pulmonary artery (PA) relaxation is a key event in pulmonary arterial hypertension (PAH) pathogenesis. CFTR dysfunction in airway epithelial cells plays a central role in cystic fibrosis (CF); CFTR is also expressed in PAs and has been shown to control endothelium-independent relaxation.

Aim And Objectives: We aimed to delineate the role of CFTR in PAH pathogenesis through observational and interventional experiments in human tissues and animal models. Read More

View Article and Full-Text PDF

Role of Polymorphisms in the Susceptibility and Severity of Interstitial Lung Disease.

Biomedicines 2021 Apr 22;9(5). Epub 2021 Apr 22.

Genetic epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, 39011 Santander, Spain.

The search for biomarkers that can help to establish an early diagnosis and prognosis of interstitial lung disease (ILD) is of potential interest. polymorphisms have been implicated in the development of several lung disorders. Consequently, we assessed, for the first time, the role of polymorphisms in the susceptibility and severity of ILD. Read More

View Article and Full-Text PDF

Rapid macrolide and amikacin resistance testing for in people with cystic fibrosis.

J Med Microbiol 2021 Apr;70(4)

The University of Queensland Centre for Clinical Research, University of Queensland, Brisbane, Queensland, Australia.

complex (MABSC) is an environmental organism and opportunistic pathogen. MABSC pulmonary infections in people with cystic fibrosis are of growing clinical concern. Resistance data guide the use of macrolides and amikacin in MABSC pulmonary disease treatment. Read More

View Article and Full-Text PDF

Pulmonary volume-feedback and ventilatory pattern after bilateral lung transplantation using neurally adjusted ventilatory assist ventilation.

Br J Anaesth 2021 Apr 20. Epub 2021 Apr 20.

Department of Anesthesia, Critical Care and Emergency, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, University of Milan, Italy.

Background: Bilateral lung transplantation results in pulmonary vagal denervation, which potentially alters respiratory drive, volume-feedback, and ventilatory pattern. We hypothesised that Neurally Adjusted Ventilatory Assist (NAVA) ventilation, which is driven by diaphragm electrical activity (EAdi), would reveal whether vagally mediated pulmonary-volume feedback is preserved in the early phases after bilateral lung transplantation.

Methods: We prospectively studied bilateral lung transplant recipients within 48 h of surgery. Read More

View Article and Full-Text PDF

Lipopolysaccharides induce a RAGE-mediated sensitization of sensory neurons and fluid hypersecretion in the upper airways.

Sci Rep 2021 Apr 16;11(1):8336. Epub 2021 Apr 16.

Department of Anatomy, Physiology and Pharmacology (APP), College of Medicine, University of Saskatchewan, 107 Wiggins Road, Saskatoon, SK, S7N 5E5, Canada.

Thoracic dorsal root ganglia (tDRG) contribute to fluid secretion in the upper airways. Inflammation potentiates DRG responses, but the mechanisms remain under investigation. The receptor for advanced glycation end-products (RAGE) underlies potentiation of DRG responses in pain pathologies; however, its role in other sensory modalities is less understood. Read More

View Article and Full-Text PDF

Can clinical benefits of modulators effectively 'modulate' adherence in people with CF?

Thorax 2021 Apr 16. Epub 2021 Apr 16.

Division of Pulmonary and Sleep Medicine, Department of Pediatrics, University of Washington, Seattle, Washington, USA.

View Article and Full-Text PDF

Rectal organoid morphology analysis (ROMA) as a promising diagnostic tool in cystic fibrosis.

Thorax 2021 Apr 15. Epub 2021 Apr 15.

Department of Development and Regeneration, Woman and Child Unit, CF Research Lab, KU Leuven, Leuven, Flanders, Belgium

Diagnosing cystic fibrosis (CF) when sweat chloride is not in the CF range and less than 2 disease-causing mutations are found requires physiological CFTR assays, which are not always feasible or available. We developed a new physiological CFTR assay based on the morphological differences between rectal organoids from subjects with and without CF. In organoids from 167 subjects with and 22 without CF, two parameters derived from a semi-automated image analysis protocol (rectal organoid morphology analysis, ROMA) fully discriminated CF subjects with two disease-causing mutations from non-CF subjects (p<0. Read More

View Article and Full-Text PDF

Once daily tacrolimus conversion in lung transplantation: A prospective study on safety and medication adherence.

J Heart Lung Transplant 2021 Jun 27;40(6):467-477. Epub 2021 Feb 27.

Department of Respiratory Diseases, Lung Transplantation Group, UZ Leuven, Campus Gasthuisberg, Leuven, Belgium; Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department CHROMETA, KU Leuven, Leuven, Belgium.

Background: Lung transplantation (LTx) requires a calcineurin inhibitor-based immunosuppressive regimen. A once daily (QD) tacrolimus regimen was developed to increase medication adherence. However, data concerning its safety and efficacy in LTx are lacking. Read More

View Article and Full-Text PDF

Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis.

J Biol Chem 2021 Apr 8:100650. Epub 2021 Apr 8.

Department of Molecular Biology, University Clinic, University of Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany; Department of Thoracic and Cardiovascular Surgery, University Hospital Essen, University of Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany. Electronic address:

Most patients with cystic fibrosis (CF) suffer from acute and chronic pulmonary infections with bacterial pathogens, which often determine their life quality and expectancy. Previous studies have demonstrated a down-regulation of the acid ceramidase in CF epithelial cells resulting in an increase of ceramide and a decrease of sphingosine. Sphingosine kills many bacterial pathogens and the down-regulation of sphingosine seems to determine the infection susceptibility of cystic fibrosis mice and patients. Read More

View Article and Full-Text PDF

Elevated serum calprotectin (S100A8/A9) in patients with severe asthma.

J Asthma 2021 Apr 19:1-6. Epub 2021 Apr 19.

Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases, Metabolism and Ageing (CHROMETA), KU Leuven, Leuven, Belgium.

Objective: Asthma is a heterogeneous disease consisting of several inflammatory phenotypes of which neutrophilic asthma is associated with poorer responses to classic therapies, namely (inhaled) corticosteroids. The development of targeted therapies requires the identification of biomarkers to distinguish these phenotypes. Currently, we lack validated biomarkers for non-eosinophilic asthma. Read More

View Article and Full-Text PDF