Search Our Scientific Publications & Authors

Mycopathologia 2021 May 15. Epub 2021 May 15.

Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK.

Introduction: Fungal infection and sensitization are common in chronic respiratory patient populations such as bronchiectasis, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (CF) and are often associated with prolonged antifungal therapy (Hohmann et al. in Clin Infect Dis 15:939-940, 2010; Vissichelli et al. in Infect Prev Pract 1:100029, 2019), morbidity, and mortality. Read More

Pediatr Pulmonol 2021 May 15. Epub 2021 May 15.

Pediatric Respiratory Medicine, Inselspital, University Children's Hospital of Bern, University of Bern, Switzerland.

Background: Multiple-breath inert gas washout (MBW) is a sensitive technique to assess lung volumes and ventilation inhomogeneity in infancy. Poor agreement amongst commercially available setups and a lack of transparency in the underlying algorithms for the computation of infant MBW outcomes currently limit the widespread application of MBW as a surveillance tool in early lung disease.

Methods: We determined all computational steps in signal processing and the calculation of MBW outcomes in the current infant WBreath/Exhalyzer D setup (Exhalyzer D device, Eco Medics AG; WBreath software version 3. Read More

Ital J Pediatr 2021 May 14;47(1):112. Epub 2021 May 14.

Department of Translational Medical Sciences, Cystic Fibrosis Center, University Federico II, Naples, Italy.

Background: The sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapies. However, false positives have been reported in patients with different diseases. We describe and discuss 4 cases due to different clinical conditions in which we recorded false positive ST, and the test remained altered for a period of varying length. Read More

Sleep Med 2021 Apr 22;83:21-25. Epub 2021 Apr 22.

Department of Pneumology, University Medicine Essen - Ruhrlandklinik, University Duisburg-Essen, Tüschener Weg 40, 45239, Essen, Germany. Electronic address:

Purpose: In our study we aimed to analyze sleep variability and activity in patients with cystic fibrosis (CF) during their hospital stay.

Methods: Forty-three CF patients were recruited and have been divided into two subgroups: exacerbated (n = 18) and non-exacerbated (n = 25). During the course of their hospital stay we used VitaLog, a minimal-impact biomotion device, in order to determine total sleep time (TST), time in bed (TIB), sleep efficiency (SE) and intra patient standard deviation (IPSD) of TST. Read More

Biosens Bioelectron 2021 May 9;187:113313. Epub 2021 May 9.

School of Pharmaceutical Sciences, Qilu University of Technology (Shandong Academy of Sciences), Jinan, 250014, China. Electronic address:

The abnormal levels of trypsin in biological fluids can cause some acute illnesses, such as acute pancreatitis, cystic fibrosis and malnutrition. In this paper, we report the development of an integrated liquid crystal (LC) sensing device for simple, rapid and sensitive detection of trypsin assisted by the surfactant-embedded smart hydrogel. The gelatin hydrogel mixed with CTAB is added into the side channel of the LC sensing device. Read More

Biotechnol Appl Biochem 2021 May 14. Epub 2021 May 14.

Institute of Nano Electronic Engineering.

Prostate cancer is one of the predominant cancers affecting men and has been widely reported. In the past, various therapies and drugs have been proposed to treat prostate cancer. Among these treatments, gene therapy has been considered to be an optimal and widely applicable treatment. Read More

Acta Biomed 2021 May 12;92(2):e2021164. Epub 2021 May 12.

Pediatric Respiratory Unit, San Marco Hospital, Department of Clinical and Experimental Medicine, University of Catania.

Background And Aim: In patients with cystic fibrosis (CF) non-invasive ventilation (NIV) improves lung mechanics and gas exchange, and decreases the work of breathing. Domiciliary NIV is mainly used in hypercapnic patients with severe disease, because it counteracts the progression of lung functional impairment and it is often used as a useful "bridge" to lung transplantation. However, to date, there are no standardized criteria to indicate the effect of a precocious starting of NIV in patients with functional ventilation inhomogeneity without hypercapnia. Read More

Netw Syst Med 2020 Dec 31;3(1):159-181. Epub 2020 Dec 31.

National Jewish Health, Denver, Colorado, USA.

Small studies have recently suggested that there are specific plasma metabolic signatures in chronic obstructive pulmonary disease (COPD), but there have been no large comprehensive study of metabolomic signatures in COPD that also integrate genetic variants. Fresh frozen plasma from 957 non-Hispanic white subjects in COPDGene was used to quantify 995 metabolites with Metabolon's global metabolomics platform. Metabolite associations with five COPD phenotypes (chronic bronchitis, exacerbation frequency, percent emphysema, post-bronchodilator forced expiratory volume at one second [FEV]/forced vital capacity [FVC], and FEV percent predicted) were assessed. Read More

Ann Transl Med 2021 Apr;9(8):738

Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry, RWTH University Hospital Aachen, Aachen, Germany.

Primary sclerosing cholangitis (PSC) is a rare progressive cholangitis resulting in cirrhosis and cholangiocellular carcinoma. The pathogenesis is unclear and an effective medical therapy is not available. It is highly associated to ulcerative colitis for which recently a disturbance of the tight junction (TJ) barrier has been claimed as etiologic feature. Read More

Front Med (Lausanne) 2021 27;8:616200. Epub 2021 Apr 27.

Department of Hepatology, The First Hospital of Jilin University, Jilin University, Changchun, China.

Chronic inflammatory pulmonary diseases are characterized by recurrent and persistent inflammation of the airways, commonly associated with poor clinical outcomes. Although their etiologies vary tremendously, airway neutrophilia is a common feature of these diseases. Neutrophils, as vital regulators linking innate and adaptive immune systems, are a double-edged sword in the immune response of the lung involving mechanisms such as phagocytosis, degranulation, neutrophil extracellular trap formation, exosome secretion, release of cytokines and chemokines, and autophagy. Read More

Front Pharmacol 2021 27;12:662110. Epub 2021 Apr 27.

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

Cystic fibrosis (CF) is a severe genetic disease for which curative treatment is still lacking. Next generation biotechnologies and more efficient cell-based and disease models are accelerating the development of novel therapies for CF. Gene editing tools, like CRISPR-based systems, can be used to make targeted modifications in the genome, allowing to correct mutations directly in the Cystic Fibrosis Transmembrane conductance Regulator () gene. Read More

J Pediatr Nurs 2021 May 10. Epub 2021 May 10.

Emeritus Professor, Department of Physiological Nursing, School of Nursing; and Department of Epidemiology & Biostatistics, School of Medicine, University of California San Francisco, CA 94143-0610, USA. Electronic address:

Background: Cystic fibrosis (CF) is a common autosomal recessive disease with an incidence of 1:2560 live births in Jordan. It affects the quality of life for both the child and the parents. In Jordan knowledge about parents' experience with children diagnosed with CF is limited. Read More

PLoS One 2021 13;16(5):e0251527. Epub 2021 May 13.

Azienda Ospedaliera-Universitaria Integrata di Verona, Verona, Italy.

Objective: To describe the symptoms and clinical course of SARS-CoV-2 infection in patients with cystic fibrosis (CF).

Methods: We carried out a prospective multicentre cohort study based on 32 CF centres and 6597 patients. Centres were contacted to collect baseline and follow-up data of patients who reported symptoms suggestive of COVID-19 or who had contact with a positive/suspected case between the end of February and July 2020. Read More

Pediatr Pulmonol 2021 May 13. Epub 2021 May 13.

Department of Pharmacy, Cancer and Hematology Centers of West Michigan, Grand Rapids, Michigan, USA.

Aminoglycosides are commonly used for the treatment of Pseudomonas aeruginosa (PsA) in the setting of acute pulmonary exacerbations (PEx) in pediatric patients with cystic fibrosis (CF). There are controversies and practice differences between institutions related to aminoglycoside dosing and monitoring strategies. The purpose of this review article is to summarize the currently available literature and identify gaps in the literature related to pharmacokinetic parameter goals, aminoglycoside dosing strategies, and methods for monitoring serum aminoglycoside concentrations for treatment of PsA in CF PEx, and throughout will discuss anticipated changes with the increasing availability of highly effective CF transmembrane conductance regulator modulators. Read More

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

Front Immunol 2021 26;12:659523. Epub 2021 Apr 26.

Department of Microbiology and Immunology, Geisel School of Medicine at Dartmouth, Lebanon, NH, United States.

Chronic infection mysteriously occurs in the airways of patients with cystic fibrosis (CF), bronchiectasis (BE), and chronic obstructive pulmonary disease (COPD) in the absence of neutrophil dysfunction or neutropenia and is strongly associated with autoimmunity to bactericidal permeability-increasing protein (BPI). Here, we define a critical role for BPI in immunity against Wild type and BPI-deficient ( mice were infected with , and bacterial clearance, cell infiltrates, cytokine production, and phagocytosis were quantified. mice exhibited a decreased ability to clear in concert with increased neutrophil counts and cytokine release. Read More

Int J Integr Care 2021 Apr 30;21(2):15. Epub 2021 Apr 30.

Department of Economics, Management and Quantitative Methods (DEMM), Università degli Studi di Milano, via Conservatorio, 7, 20122, Milan, Italy.

Introduction: Co-production is more and more considered as a promising tool for dealing with the main challenges in the health sector (e.g., growing rates of chronic diseases, budget constraints, higher patients' expectations of the quality and the value of services, equity to access of care, etc. Read More

Eur Respir Rev 2021 Jun 11;30(160). Epub 2021 May 11.

Sydney School of Public Health, The University of Sydney, Sydney, Australia.

There is no consensus on how best to measure responses to interventions among children and adults with cystic fibrosis (CF). We have systematically reviewed and summarised the characteristics and measurement properties of tests and tools that have been used to capture outcomes in studies among people with CF, including their reliability, validity and responsiveness. This review is intended to guide researchers when selecting tests or tools for measuring treatment effects in CF trials. Read More

BMC Pulm Med 2021 May 12;21(1):158. Epub 2021 May 12.

School of Medicine, University of Limerick, Limerick, Ireland.

Background: Oscillating Positive Expiratory Pressure (OPEP) devices are important adjuncts to airway clearance therapy in patients with cystic fibrosis (CF). Current devices are typically reusable and require daily, or often more frequent, cleaning to prevent risk of infection by acting as reservoirs of potentially pathogenic organisms. In response, a daily disposable OPEP device, the UL-OPEP, was developed to mitigate the risk of contamination and eliminate the burdensome need for cleaning devices. Read More

Small 2021 May 12:e2100531. Epub 2021 May 12.

Clinical and Health Science, University of South Australia, North Tce, Adelaide, SA, 5000, Australia.

Pseudomonas aeruginosa biofilms cause persistent and chronic infections, most known clinically in cystic fibrosis (CF). Tobramycin (TOB) is a standard anti-pseudomonal antibiotic; however, in biofilm infections, its efficacy severely decreases due to limited permeability across the biofilm matrix. Herewith, a biomimetic, nanostructured, lipid liquid crystal nanoparticle-(LCNP)-formulation is discovered to significantly enhance the efficacy of TOB and eradicate P. Read More

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

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

Eur J Pharmacol 2021 May 8:174123. Epub 2021 May 8.

Department of Medical Biochemistry and Cell Biology, University of Gothenburg, Sweden. Electronic address:

Cystic fibrosis (CF) is a recessive inherited disease caused by mutations affecting anion transport by the epithelial ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The disease is characterized by mucus accumulation in the airways and intestine, but the major cause of mortality in CF is airway mucus accumulation, leading to bacterial colonization, inflammation and respiratory failure. Several drug targets are under evaluation to alleviate airway mucus obstruction in CF and one of these targets is the epithelial sodium channel ENaC. Read More

Pediatr Pulmonol 2021 May 11. Epub 2021 May 11.

Pediatric Pulmonology and Sleep Unit, Department of Pediatrics.

Cystic fibrosis (CF) is a chronic multisystem disease with manifestations from birth. It involves the entire respiratory system, with increased cough, and recurrent pulmonary infections, and it also leads to intestinal malabsorption, all of which can have an impact on sleep. In this review, we summarize the available literature on the various sleep disturbances in children with CF. Read More

Adv Exp Med Biol 2021 ;1318:109-118

Department of Internal Medicine, Ghent University, Ghent, Belgium.

The outbreak of the COVID-19 pandemic shows a marked geographical variation in its prevalence and mortality. The question arises if the host genetic variation may (partly) affect the prevalence and mortality of COVID-19. We postulated that the geographical variation of human polymorphisms might partly explain the variable prevalence of the infection. Read More

Thorax 2021 May 10. Epub 2021 May 10.

Pediatrics, University of Arizona, Tucson, Arizona, USA

Redox Biol 2021 Apr 30;43:101998. Epub 2021 Apr 30.

Department of Otolaryngology Head & Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USA; Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA. Electronic address:

Lipopolysaccharide (LPS) serves as the interface between gram-negative bacteria (GNB) and the innate immune response in respiratory epithelial cells (REC). Herein, we describe a novel biological role of LPS that permits GNB to persist in the respiratory tract through inducing CFTR and mucociliary dysfunction. LPS reduced cystic fibrosis transmembrane conductance regulater (CFTR)-mediated short-circuit current in mammalian REC in Ussing chambers and nearly abrogated CFTR single channel activity (defined as forskolin-activated Cl currents) in patch clamp studies, effects of which were blocked with toll-like receptor (TLR)-4 inhibitor. Read More

Purinergic Signal 2021 May 10. Epub 2021 May 10.

Bayer GU Oncology, St. Louis, MO, USA.

Systemic pools of ATP are elevated in individuals homozygous for cystic fibrosis (CF) as evidenced by elevated blood and plasma ATP levels. This elevated ATP level seems to provide benefit in the presence of advanced solid tumors (Abraham et al., Nature Medicine 2(5):593-596, 1996). Read More

FEMS Microbiol Rev 2021 May 10. Epub 2021 May 10.

bioMérieux, Open Innovation and Partnerships, 3 Route du Port Michaud, 38390 La Balme Les Grottes, France.

Antimicrobial resistance (AMR) has become a global medical priority that needs urgent resolution. Pseudomonas aeruginosa is a versatile, adaptable bacterial species with widespread environmental occurrence, strong medical relevance, a diverse set of virulence genes and a multitude of intrinsic and possibly acquired antibiotic resistance traits. P. Read More

J Drug Assess 2021 Apr 5;10(1):62-67. Epub 2021 Apr 5.

Graduate School of Pharmaceutical Sciences, Duquesne University, Pittsburgh, PA, USA.

Background: There have been significant advances in Cystic Fibrosis (CF) treatment, with the introduction of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators. Adherence is an important goal for CF management, as nonadherence is linked to poor health outcomes.

Objective: To calculate the medication adherence in patients taking CFTR modulators using a national specialty pharmacy database. Read More