Impact of a Gap Junction Protein Alpha 4 Variant on Clinical Disease Phenotype in F508del Homozygous Patients With Cystic Fibrosis
BackgroundLung disease phenotype varies widely even in the F508del (homozygous) genotype. Leukocyte-driven inflammation is important for pulmonary disease pathogenesis in cystic fibrosis (CF). Blood cytokines correlate negatively with pulmonary function in F508del homozygous patients, and gap junction proteins (GJA) might be related to the influx of blood cells into the lung and influence disease course. We aimed to assess the relationship between GJA1/GJA4 genotypes and the clinical disease phenotype.MethodsOne-hundred-and-sixteen homozygous F508del patients (mean age 27 years, m/f 66/50) were recruited from the CF center...
Source: Frontiers in Genetics - October 28, 2020 Category: Genetics & Stem Cells Source Type: research

Peptidorhamnomannans From Scedosporium and Lomentospora Species Display Microbicidal Activity Against Bacteria Commonly Present in Cystic Fibrosis Patients
Scedosporium and Lomentospora species are filamentous fungi that cause a wide range of infections in humans. They are usually found in the lungs of cystic fibrosis (CF) patients and are the second most frequent fungal genus after Aspergillus species. Several studies have been recently performed in order to understand how fungi and bacteria interact in CF lungs, since both can be isolated simultaneously from patients. In this context, many bacterial molecules were shown to inhibit fungal growth, but little is known about how fungi could interfere in bacterial development in CF lungs. Scedosporium and Lomentospora species pr...
Source: Frontiers in cellular and infection microbiology - October 28, 2020 Category: Microbiology Source Type: research

Synthesis of the Hydroxamate Siderophore N α-Methylcoprogen B in Scedosporium apiospermum Is Mediated by sidD Ortholog and Is Required for Virulence
Scedosporium species rank second among the filamentous fungi capable to colonize chronically the respiratory tract of patients with cystic fibrosis (CF). Nevertheless, there is little information on the mechanisms underpinning their virulence. Iron acquisition is critical for the growth and pathogenesis of many bacterial and fungal genera that chronically inhabit the CF lungs. In a previous study, we showed the presence in the genome of Scedosporium apiospermum of several genes relevant for iron uptake, notably SAPIO_CDS2806, an ortholog of sidD, which drives the synthesis of the extracellular hydroxamate-type siderophore ...
Source: Frontiers in cellular and infection microbiology - October 28, 2020 Category: Microbiology Source Type: research

Cystic fibrosis: physiopathology and the latest pharmacological treatments
Publication date: Available online 27 October 2020Source: Pharmacological ResearchAuthor(s): Carla Fonseca, Joana Bicker, Gilberto Alves, Amílcar Falcão, Ana Fortuna (Source: Pharmacological Research)
Source: Pharmacological Research - October 27, 2020 Category: Drugs & Pharmacology Source Type: research

Use of medical marijuana in cystic fibrosis patients
The usage and attitudes towards medical marijuana in Cystic Fibrosis (CF) patients is unknown. Through the use of a survey we aim to clarify rates and reasons for use. (Source: BMC Complementary and Alternative Medicine)
Source: BMC Complementary and Alternative Medicine - October 27, 2020 Category: Complementary Medicine Authors: Michael J. Stephen, Jared Chowdhury, Luis Arzeno Tejada, Robert Zanni and Denis Hadjiliadis Tags: Research article Source Type: research

Sickle cell trait newborn screen results: disclosure and management
This study aims to evaluate the notification process of sickle cell trait (SCT) identified by newborn screening in Alberta. On April 1, 2019, Alberta began newborn screening for sickle cell disease (SCD) and elected to report sickle cell trait (SCT). For 1  year, healthcare providers (HCPs) were sent a questionnaire which addressed the perceived importance of disclosing the SCT results, whether HCPs felt competent in disclosing the result, knowledge of available resources, and comfort with coordinating and interpreting testing for the newborn’s par ents. As a control, we collected data from HCPs receiving positi...
Source: Journal of Community Genetics - October 26, 2020 Category: Genetics & Stem Cells Source Type: research

Which score is better in association with exercise capacity and health status in non-cystic fibrosis bronchiectasis patients?
CONCLUSION: Although both FACED and BSI scores were negatively correlated with walking distance and health status in patients with NCFB, BSI was more associated. PMID: 33081435 [PubMed - as supplied by publisher] (Source: Turkish Journal of Medical Sciences)
Source: Turkish Journal of Medical Sciences - October 24, 2020 Category: General Medicine Tags: Turk J Med Sci Source Type: research

Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents
Conclusions: In total, 8 patients (9.5%) in this pediatric CF population were categorized as having CFLD according to both biochemical and sonographic tests. Consistency was found among the results of FibroScan and SWE. We suggest WUSS and either FibroScan or SWE, combined with GGT as diagnostic markers for CFLD. (Source: Journal of Pediatric Gastroenterology and Nutrition)
Source: Journal of Pediatric Gastroenterology and Nutrition - October 24, 2020 Category: Gastroenterology Tags: Original Article: Pancreatology Source Type: research

Learning to breathe with Tai Chi online - qualitative data from a randomized controlled feasibility study of patients with cystic fibrosis
Publication date: Available online 22 October 2020Source: European Journal of Integrative MedicineAuthor(s): Patricia Ronan, Awais Mian, Siobhán B. Carr, Susan L. Madge, Ava Lorenc, Professor Nicola Robinson (Source: European Journal of Integrative Medicine)
Source: European Journal of Integrative Medicine - October 23, 2020 Category: Complementary Medicine Source Type: research

High-level antibiotic tolerance of a clinically-isolated Enterococcus faecalis strain.
Abstract Bacteria can survive antibiotic treatment both by acquiring antibiotic resistance genes, and through mechanisms of tolerance that are based on phenotypic changes and the formation of metabolically inactive cells. Here, we report an Enterococcus faecalis strain (E. faecalis UM001B) isolated from a cystic fibrosis patient that has no increase in resistance but extremely high-level tolerance to ampicillin, vancomycin, and tetracycline. Specifically, the percentage of cells that survive 3.5-h antibiotic treatment (at 100 μg ⋅mL-1) is 25.4 ± 4.3% and 51.9 ± 4.0% for ampicillin and tetracy...
Source: Applied and Environmental Microbiology - October 23, 2020 Category: Microbiology Authors: Gu H, Roy S, Zheng X, Gao T, Ma H, Soultan Z, Fortner C, Nangia S, Ren D Tags: Appl Environ Microbiol Source Type: research

Phenotypic and transcriptomic analysis of seven clinical Stenotrophomonas maltophilia isolates identify a small set of shared and commonly regulated genes involved in biofilm lifestyle.
This study will lay the foundation for the identification of strategies for fighting S. maltophilia biofilms in clinical and industrial settings.IMPORTANCEMicroorganisms living in a biofilm are much more tolerant to antibiotics and antimicrobial substances than planktonic cells. Thus, the treatment of infections caused by microorganisms living in biofilms is extremely difficult. The nosocomial infections among others caused by S. maltophilia, particularly lung infection among CF patients, increased in prevalence in the last years. The intrinsic multidrug resistance of S. maltophilia and the increased tolerance to antimicro...
Source: Applied and Environmental Microbiology - October 23, 2020 Category: Microbiology Authors: Alio I, Gudzuhn M, Pérez García P, Danso D, Schoelmerich M, Mamat U, Schaible UE, Steinmann J, Yero D, Gibert I, Kohl TA, Niemann S, Gröschel MI, Haerdter J, Hackl T, Vollstedt C, Bömeke M, Egelkamp R, Daniel R, Poehlein A, Streit WR Tags: Appl Environ Microbiol Source Type: research

Identification of AAV serotypes for lung gene therapy in human embryonic stem cell-derived lung organoids
Gene therapy is being investigated for a range of serious lung diseases, such as cystic fibrosis and emphysema. Recombinant adeno-associated virus (rAAV) is a well-established, safe, viral vector for gene deli... (Source: Stem Cell Research and Therapy)
Source: Stem Cell Research and Therapy - October 23, 2020 Category: Stem Cells Authors: Helena Meyer-Berg, Lucia Zhou Yang, Mar ía Pilar de Lucas, Alberto Zambrano, Stephen C. Hyde and Deborah R. Gill Tags: Short report Source Type: research

[ASAP] Lipidome Alterations Induced by Cystic Fibrosis, CFTR Mutation, and Lung Function
Journal of Proteome ResearchDOI: 10.1021/acs.jproteome.0c00556 (Source: Journal of Proteome Research)
Source: Journal of Proteome Research - October 22, 2020 Category: Biochemistry Authors: Adriana Zardini Buzatto, Mai Abdel Jabar, Imran Nizami, Majed Dasouki, Liang Li, and Anas M. Abdel Rahman Source Type: research

LocusFocus: Web-based colocalization for the annotation and functional follow-up of GWAS
by Naim Panjwani, Fan Wang, Scott Mastromatteo, Allen Bao, Cheng Wang, Gengming He, Jiafen Gong, Johanna M. Rommens, Lei Sun, Lisa J. Strug Genome-wide association studies (GWAS) have primarily identified trait-associated loci in the non-coding genome. Colocalization analyses of SNP associations from GWAS with expression quantitative trait loci (eQTL) evidence enable the generation of hypotheses about responsible mechanism, genes and tissues of origin to guide functional characterization. Here, we present a web-based colocalization browsing and testing tool namedLocusFocus (https://locusfocus.research.sickkids.ca). LocusF...
Source: PLoS Computational Biology - October 22, 2020 Category: Biology Authors: Naim Panjwani Source Type: research

Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiences
The findings of early disease surveillance collaborations, such as the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF), and the London Cystic Fibrosis Collaboration (LCFC), have transformed our understanding of early CF lung disease and the need to intervene early in life to improve health outcomes [1 –5]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 22, 2020 Category: Respiratory Medicine Authors: Tonia A Douglas, Julie Ann Pooley, Linda Shields, Stephen M Stick, Cindy Branch-Smith, Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) Tags: Original Article Source Type: research

Pseudomonas aeruginosa Type III Secretion System Virulotypes and Their Association with Clinical Features of Cystic Fibrosis Patients
(Source: Infection and Drug Resistance)
Source: Infection and Drug Resistance - October 21, 2020 Category: Infectious Diseases Tags: Infection and Drug Resistance Source Type: research

Cystic fibrosis transmembrane conductance regulator (CFTR) regulates embryonic organizer formation during zebrafish early embryogenesis.
Authors: Liu Y, Lin Z, Sun H Abstract Cystic fibrosis (CF) is associated with the manifestation of a number of medical conditions throughout the body. This prompted us to investigate the etiology of CF from the viewpoint of the embryonic organizer, which is responsible for steering the movement of surrounding cells into specific organs and tissues. In our previous work, we found that a cftr mutant had decreased nuclear β-catenin levels in the early embryo at 5 hours post-fertilization (hpf), when the organizer forms. It is known that nuclear β-catenin signaling is essential for the induction of the dorsal...
Source: International Journal of Developmental Biology - October 21, 2020 Category: Biology Tags: Int J Dev Biol Source Type: research

A Phylogeny-Informed Proteomics Approach for Species Identification within the Burkholderia cepacia Complex [Bacteriology]
Ancestral genetic exchange between members of many important bacterial pathogen groups has resulted in phylogenetic relationships better described as networks than as bifurcating trees. In certain cases, these reticulated phylogenies have resulted in phenotypic and molecular overlap that challenges the construction of practical approaches for species identification in the clinical microbiology laboratory. Burkholderia cepacia complex (Bcc), a betaproteobacteria species group responsible for significant morbidity in persons with cystic fibrosis and chronic granulomatous disease, represents one such group where network-struc...
Source: Journal of Clinical Microbiology - October 21, 2020 Category: Microbiology Authors: Wang, H., Cisse, O. H., Bolig, T., Drake, S. K., Chen, Y., Strich, J. R., Youn, J.-H., Okoro, U., Rosenberg, A. Z., Sun, J., LiPuma, J. J., Suffredini, A. F., Dekker, J. P. Tags: Bacteriology Source Type: research

Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis
Cystic fibrosis (CF)-related bone disease (CFBD) is characterized by low bone mass, impaired bone quality and increased incidence of thoracic fractures with prevalence rates reaching 96% in end-stage patients awaiting lung transplant (LTx) [1]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - October 21, 2020 Category: Respiratory Medicine Authors: Gabrielle Durette, Val érie Jomphe, Nathalie J Bureau, Charles Poirier, Pasquale Ferraro, Larry C. Lands, Geneviève Mailhot Tags: Original Article Source Type: research

Evaluation of both exonic and intronic variants for effects on RNA splicing allows for accurate assessment of the effectiveness of precision therapies
by Anya T. Joynt, Taylor A. Evans, Matthew J. Pellicore, Emily F. Davis-Marcisak, Melis A. Aksit, Alice C. Eastman, Shivani U. Patel, Kathleen C. Paul, Derek L. Osorio, Alyssa D. Bowling, Calvin U. Cotton, Karen S. Raraigh, Natalie E. West, Christian A. Merlo, Garry R. Cutting, Neeraj Sharma Elucidating the functional consequence of molecular defects underlying genetic diseases enables appropriate design of therapeutic options. Treatment of cystic fibrosis (CF) is an exemplar of this paradigm as the development of CFTR modulator therapies has allowed for targeted and effective treatme nt of individuals harboring specific ...
Source: PLoS Genetics - October 21, 2020 Category: Genetics & Stem Cells Authors: Anya T. Joynt Source Type: research

Cystic Fibrosis: A Pocket Guide.
Authors: Roughley M, MacDougall L, Erolin C Abstract In 2010, an iPad app titled 'A Patient's Guide to Cystic Fibrosis' was developed for use by Cystic Fibrosis (CF) clinical nurse specialists during a patient's annual review. Feedback from pseudo-CF patients and NHS staff including CF clinical nurse specialists and respiratory consultants about the appearance, usability and content of the app and iPad interface, and the appropriateness of the app for CF patients was positive. The visual images, animations and interactive elements of the app were sufficient in providing an engaging experience for the user. The app ...
Source: Journal of Visual Communication in Medicine - October 20, 2020 Category: Information Technology Tags: J Vis Commun Med Source Type: research

IJERPH, Vol. 17, Pages 7630: Managing Cystic Fibrosis in Polish Healthcare
liniak The quality and length of life of patients with cystic fibrosis (CF) are determined by a number of factors including the quality of healthcare received by patients, as well as access to drug programs dedicated to this particular disease. The purpose of this paper is to present an overview of changes in the average life expectancy and mortality rate of the CF population in Poland between 2000 and 2018. Furthermore, we would like to evaluate access to healthcare services, including the drug program, guaranteed by public healthcare system, and funded by National Health Fund (NHF). The average life expectancy of pat...
Source: International Journal of Environmental Research and Public Health - October 20, 2020 Category: Environmental Health Authors: Marta Rachel Stanis ław Topolewicz Andrzej Śliwczyński Sabina Galiniak Tags: Article Source Type: research

Drug treatments for managing cystic fibrosis-related diabetes.
CONCLUSIONS: This review has not found any conclusive evidence that any agent has a distinct advantage over another in controlling hyperglycemia or the clinical outcomes associated with CFRD. Given the treatment burden already experienced by people with cystic fibrosis, oral therapy may be a viable treatment option. While some cystic fibrosis centers use oral medications to help control diabetes, the Cystic Fibrosis Foundation (USA) clinical practice guidelines support the use of insulin therapy and this remains the most widely-used treatment method. Randomized controlled trials specifically related to controlling diabetes...
Source: Cochrane Database of Systematic Reviews - October 19, 2020 Category: General Medicine Authors: Onady GM, Stolfi A Tags: Cochrane Database Syst Rev Source Type: research

Utility of Mid-Upper Arm Circumference in Diagnosing Malnutrition in Children With Cystic Fibrosis.
CONCLUSIONS: Our small-scale data suggest a promising role for MUAC z-scores in classifying malnutrition and in measuring changes in nutrition status over time in pediatric CF. PMID: 33078441 [PubMed - as supplied by publisher] (Source: Nutrition in Clinical Practice)
Source: Nutrition in Clinical Practice - October 19, 2020 Category: Nutrition Authors: Phong RY, Taylor SL, Robinson BA, Jhawar S, Nandalike K Tags: Nutr Clin Pract Source Type: research

Derivation of Airway Basal Stem Cells from Human Pluripotent Stem Cells.
er EJ, Horani A, Brody SL, Davis BR, Kotton DN Abstract The derivation of tissue-specific stem cells from human induced pluripotent stem cells (iPSCs) would have broad reaching implications for regenerative medicine. Here, we report the directed differentiation of human iPSCs into airway basal cells ("iBCs"), a population resembling the stem cell of the airway epithelium. Using a dual fluorescent reporter system (NKX2-1GFP;TP63tdTomato), we track and purify these cells as they first emerge as developmentally immature NKX2-1GFP+ lung progenitors and subsequently augment a TP63 program during proximal airw...
Source: Cell Stem Cell - October 19, 2020 Category: Stem Cells Authors: Hawkins FJ, Suzuki S, Beermann ML, Barillà C, Wang R, Villacorta-Martin C, Berical A, Jean JC, Le Suer J, Matte T, Simone-Roach C, Tang Y, Schlaeger TM, Crane AM, Matthias N, Huang SXL, Randell SH, Wu J, Spence JR, Carraro G, Stripp BR, Rab A, Sorsher EJ Tags: Cell Stem Cell Source Type: research

Impact of novel CFTR modulator on sinonasal quality of life in adult patients with cystic fibrosis
International Forum of Allergy& Rhinology, EarlyView. (Source: International Forum of Allergy and Rhinology)
Source: International Forum of Allergy and Rhinology - October 18, 2020 Category: Allergy & Immunology Authors: Jennifer E. Douglas, Alyssa M. Civantos, Tran B. Locke, Auddie M. Sweis, Denis Hadjiliadis, Gina Hong, Daniel J. Dorgan, Michael A. Kohanski, James N. Palmer, Nithin D. Adappa Tags: RESEARCH NOTE Source Type: research

Cystic fibrosis: On the cusp of cure?
International Forum of Allergy& Rhinology, EarlyView. (Source: International Forum of Allergy and Rhinology)
Source: International Forum of Allergy and Rhinology - October 18, 2020 Category: Allergy & Immunology Authors: Brent A. Senior Tags: EDITORIAL Source Type: research

Influence of Cystic Fibrosis-Related Diabetes on Mental Health in Adults: A Single-Center Study
ConclusionWe show a significant association between CFRD and selected diagnoses of depression in a cohort of adult patients. (Source: Lung)
Source: Lung - October 16, 2020 Category: Respiratory Medicine Source Type: research

Aquagenic (pseudo) keratoderma (aquagenic palmoplantar keratoderma, aquagenic wrinkling of palms)
Aquagenic (palmoplantar) keratoderma (APK) is an uncommon hereditary or sporadic condition which is characterized by edematous flat-topped papules appearing on palmar skin with wrinkling after brief water exposure. APK has been associated with cystic fibrosis (CF), presenting with the same mutations found in CF (usually ΔF508 of the CFTR gene), either homozygous or heterozygous. APK may be idiopathic or drug-induced. The diagnosis is easily made, if one is aware of this entity. Topical aluminum hydroxide and botulinum toxin injections are the most commonly used treatments. (Source: Clinics in Dermatology)
Source: Clinics in Dermatology - October 16, 2020 Category: Dermatology Authors: Laura Atzori, Caterina Ferreli, Franco Rongioletti Source Type: research

Ambient air pollution and respiratory bacterial infections, a troubling association: epidemiology, underlying mechanisms, and future challenges.
Abstract The World Health Organization attributed more than four million premature deaths to ambient air pollution in 2016. Numerous epidemiologic studies demonstrate that acute respiratory tract infections and exacerbations of pre-existing chronic airway diseases can result from exposure to ambient (outdoor) air pollution. In this context, the atmosphere contains both chemical and microbial pollutants (bioaerosols), whose impact on human health remains unclear. Therefore, this review: summarises the findings from recent studies on the association between exposure to air pollutants-especially particulate matter an...
Source: Critical Reviews in Microbiology - October 16, 2020 Category: Microbiology Authors: Pompilio A, Di Bonaventura G Tags: Crit Rev Microbiol Source Type: research

Management and outcomes of Burkholderia cepacia complex bacteremia in patients without cystic fibrosis: a retrospective observational study
The objective was to evaluate the management and outcomes of BCC bacteremia in patients without CF. We conducted a retrospective study of non-CF adult patients with BCC bacteremia between January 1997 and December 2016 at 4 tertiary hospitals in South Korea. A total of 216 non-CF patients with BCC bacteremia were identified. Most cases were hospital-acquired (79.2%), and the most common source was a central venous catheter (CVC) (42.1%). The rates of susceptibility to trimethoprim-sulfamethoxazole and piperacillin-tazobactam of BCC isolates were high as 92.8% and 90.3%, respectively. The rates of susceptibility to ceftazid...
Source: European Journal of Clinical Microbiology and Infectious Diseases - October 15, 2020 Category: Microbiology Source Type: research

Biological applications of synthetic anion transporters.
Abstract The use of synthetic ion transporters for alteration of the concentration of ions across cell membranes has drawn attention from scientists over the last two decades. This ion transport property has been sensibly used to reduce the viability of cancer cells mainly due to the disruption of their ion homeostasis, leading to the perturbation of their abnormal pH gradient. The use of the proanionophore strategy has been recently adopted to increase cellular deliverability and reduce unwanted cytotoxicity towards normal cells. Meanwhile, various anionophores exhibiting non-toxic behavior in epithelial cells ha...
Source: Chemical Communications - October 15, 2020 Category: Chemistry Authors: Akhtar N, Biswas O, Manna D Tags: Chem Commun (Camb) Source Type: research

Exploring the reproductive decision support needs and preferences of women with cystic fibrosis
As women with cystic fibrosis (CF) live longer, healthier lives, they increasingly face decisions related to their reproductive health. This qualitative study explores their unique decision support needs and preferences to aid in the development of a CF-specific reproductive goals decision aid.Study DesignWomenwith CF age 18-44 years participated in individual, semi-structured, telephone-based interviews, and women with CF age 18 years and older participated in semi-structured focus group discussions (FGDs). (Source: Contraception)
Source: Contraception - October 14, 2020 Category: OBGYN Authors: Mary M. Leech, Olivia M. Stransky, Mehret Birru Talabi, Sonya Borrero, Andrea H. Roe, Traci M. Kazmerski Source Type: research

IJERPH, Vol. 17, Pages 7483: Prevalence and Economic Burden of Respiratory Diseases in Central Asia and Russia: A Systematic Review
van Boven Prevalence data of respiratory diseases (RDs) in Central Asia (CA) and Russia are contrasting. To inform future research needs and assist government and clinical policy on RDs, an up-to-date overview is required. We aimed to review the prevalence and economic burden of RDs in CA and Russia. PubMed and EMBASE databases were searched for studies that reported prevalence and/or economic burden of RDs (asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis, interstitial lung diseases (ILD), lung cancer, pulmonary hypertension, and tuberculosis (TB)) in CA (Kyrgyzstan, Uzbekistan, Tajikistan, Kaz...
Source: International Journal of Environmental Research and Public Health - October 14, 2020 Category: Environmental Health Authors: Tabyshova Emilov Postma Chavannes Sooronbaev van Boven Tags: Review Source Type: research

Overview of Atypical Diabetes
Although type 1 diabetes mellitus and, to a lesser extent, type 2 diabetes mellitus, are the prevailing forms of diabetes in youth, atypical forms of diabetes are not uncommon and may require etiology-specific therapies. By some estimates, up to 6.5% of children with diabetes have monogenic forms. Mitochondrial diabetes and cystic fibrosis related diabetes are less common but often noted in the underlying disease. Atypical diabetes should be considered in patients with a known disorder associated with diabetes, aged less than 25  years with nonautoimmune diabetes and without typical characteristics of type 2 diabetes ...
Source: Endocrinology and Metabolism Clinics of North America - October 14, 2020 Category: Endocrinology Authors: Jaclyn Tamaroff, Marissa Kilberg, Sara E. Pinney, Shana McCormack Source Type: research

A chronic strain of the cystic fibrosis pathogen Pandoraea pulmonicola expresses a heterogenous hypo-acylated lipid A
AbstractPandoraea sp. is an emerging Gram-negative pathogen in cystic fibrosis causing severe and persistent inflammation and damage of the lungs. The molecular mechanisms underlying the high pathogenicity ofPandoraea species are still largely unknown. As Gram-negatives,Pandoraea sp. express lipopolysaccharides (LPS) whose recognition by the host immune system triggers an inflammatory response aimed at the bacterial eradication from the infected tissues. The degree of the inflammatory response strongly relies on the fine structure of the LPS and, in particular, of its glycolipid moiety, i.e. the lipid A. Here we report the...
Source: Glycoconjugate Journal - October 13, 2020 Category: Biochemistry Source Type: research

Mucin secretion in cystic fibrosis – a systematic review
Conclusions Increased expression of MUC5AC in the surface epithelium and of MUC5B in the sub-epithelial glands may be the result of higher secretion rate of mucin into the lumen of the respiratory tract, causing mucus plaque, infection and inflammation. (Source: Digestive Diseases)
Source: Digestive Diseases - October 12, 2020 Category: Gastroenterology Source Type: research

Lipolytic enzymes inhibitors: A new way for antibacterial drugs discovery.
Abstract Tuberculosis (TB) caused by Mycobacterium tuberculosis (M. tb) still remains the deadliest infectious disease worldwide with 1.5 million deaths in 2018, of which about 15% are attributed to resistant strains. Another significant example is Mycobacterium abscessus (M. abscessus), a nontuberculous mycobacteria (NTM) responsible for cutaneous and pulmonary infections, representing up to 95% of NTM infections in cystic fibrosis (CF) patients. M. abscessus is a new clinically relevant pathogen and is considered one of the most drug-resistant mycobacteria for which standardized chemotherapeutic r...
Source: European Journal of Medicinal Chemistry - October 12, 2020 Category: Chemistry Authors: Cavalier JF, Spilling CD, Durand T, Camoin L, Canaan S Tags: Eur J Med Chem Source Type: research

CRISPR/Cas9 genome editing tool: A Promising Tool for Therapeutic Applications on respiratory diseases.
Abstract Respiratory diseases are one of the prime topics of concern in the current era due to improper diagnostics tools. Gene-editing therapy like Clustered regularly interspaced palindromic repeats-associated nuclease 9 (CRISPR/Cas9) is gaining popularity in pulmonary research opening up doors to invaluable insights on underlying mechanisms. CRISPR/Cas9 can be considered as a potential gene editing tool with a scientific community that is helping in the advancement of knowledge in respiratory health and therapy. As an appealing therapeutic tool, we hereby explore the advanced research on the application of CRIS...
Source: Current Gene Therapy - October 12, 2020 Category: Genetics & Stem Cells Authors: Shaikh SB, Bhandary YP Tags: Curr Gene Ther Source Type: research

Nutritional Risk Index: A Predictive Metric for Mortality Following Lung Transplant.
Abstract BACKGROUND: Malnourishment is associated with poor outcomes following Lung Transplant (LTx). Validated screening tools for patients awaiting lung transplants are needed. We assessed the association of Nutritional Risk Index (NRI) with outcomes following LTx. METHODS: We categorized adult patients (age>18) undergoing incident LTx in the Scientific Registry of Transplant Recipients between 2005-2018 (N=13,392) according to NRI categories of malnutrition: none:≥100, mild:97.5 -
Source: The Annals of Thoracic Surgery - October 12, 2020 Category: Cardiovascular & Thoracic Surgery Authors: Bigelow B, Toci G, Etchill E, Krishnan A, Merlo C, Bush EL Tags: Ann Thorac Surg Source Type: research

Primary Lung Carcinoma in Cystic Fibrosis: A Case Report and Literature Review
Publication date: Available online 10 October 2020Source: Respiratory Medicine Case ReportsAuthor(s): Vipul Patel, Tilottama Majumdar, Isha Samreen, Harpreet Grewal, Thomas Kaleekal (Source: Respiratory Medicine Case Reports)
Source: Respiratory Medicine Case Reports - October 10, 2020 Category: Respiratory Medicine Source Type: research

Thymosin alpha 1 exerts beneficial extrapulmonary effects in cystic fibrosis.
Abstract Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene encoding for the ion channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Long considered a lung disease for the devastating impact on the respiratory function, the recent diagnostic and therapeutic advances have shed the light on the extra-pulmonary manifestations of CF, including gastrointestinal, hepatobiliary and pancreatic symptoms. We have previously demonstrated that thymosin alpha1 (Tα1), a naturally occurring immunomodulatory peptide, displays multi-sided beneficial effects in CF that conc...
Source: European Journal of Medicinal Chemistry - October 9, 2020 Category: Chemistry Authors: Bellet MM, Borghi M, Pariano M, Renga G, Stincardini C, D'Onofrio F, Brancorsini S, Garaci E, Costantini C, Romani L Tags: Eur J Med Chem Source Type: research

[Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen].
e-Vicente C Abstract INTRODUCTION: Neonatal cystic fibrosis (CF) screening has enabled the disease to be diagnosed early, and is a determining factor in the increase in survival of these patients. Its main disadvantage is its low specificity and elevated number of false positives. The aim of this study is to analyse the differences in immunoreactive trypsin (IRT) between the different groups of newborns (NB) with a positive neonatal screen depending on whether they were healthy, healthy carriers, affected by CF, or CFSPID (Cystic Fibrosis Screen Positive, Inconclusive Diagnosis). MATERIAL: Retrospective analy...
Source: Anales de Pediatria - October 9, 2020 Category: Pediatrics Authors: Arrudi-Moreno M, García-Romero R, Samper-Villagrasa P, Sánchez-Malo MJ, Martin-de-Vicente C Tags: An Pediatr (Barc) Source Type: research

Editorial: The changing landscape of cystic fibrosis: new therapies, challenges and a global pandemic
No abstract available (Source: Current Opinion in Pulmonary Medicine)
Source: Current Opinion in Pulmonary Medicine - October 8, 2020 Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

Sexual and reproductive health in cystic fibrosis
Purpose of review With improving life expectancy and quality of life, sexual and reproductive health (SRH) has become an increasingly important aspect of patient-centered cystic fibrosis care. This review aims to describe advances in cystic fibrosis-related SRH and highlight optimal practices. Recent findings Recent publications suggest that people with cystic fibrosis follow a similar trajectory of sexual development and activity as their noncystic fibrosis peers, although contraception use is lower. Although fertility is reduced in patients with cystic fibrosis, improved survival and assisted reproductive technologi...
Source: Current Opinion in Pulmonary Medicine - October 8, 2020 Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators
Purpose of review The current review provides an overview of key psychological issues and challenges for the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator era of care. It discusses research from diagnosis and beyond, to patient-team communication with a particular focus on medical trials, adherence and living with CFTR modulators. Recent findings The impact of the diagnosis on parents is immense and the complexity of treatment now and in the future, are a challenge for both parents and teams. Communicating digitally is starting to become daily practice for many in CF care, with coronavirus ...
Source: Current Opinion in Pulmonary Medicine - October 8, 2020 Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

Monitoring early stage lung disease in cystic fibrosis
Purpose of review Early stage lung disease has long been synonymous with infancy and childhood. As diagnosis happens earlier and conventional management improves, we are seeing larger proportions of people with cystic fibrosis (CF) in adolescence and even adulthood with well preserved lung health. The availability of highly effective cystic fibrosis transmembrane conductance regulator modulator drugs for a large proportion of the CF population will impact even further. Transitioning into adult care with ‘normal’ lung function will become more common. However, it is crucial that we are not blasé about t...
Source: Current Opinion in Pulmonary Medicine - October 8, 2020 Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

Treatment of pulmonary exacerbations in cystic fibrosis
Purpose of review This review will discuss the challenges of defining a pulmonary exacerbations in cystic fibrosis and the key pathogens, which contribute. It will discuss the treatment options currently available and the importance of preventing pulmonary exacerbations. Recent findings The basis for treatment of pulmonary exacerbations remains unchanged over the past 15 years and whilst there have been trials exploring alternative antibiotics, there has been little change. However, there are ongoing studies that are expected to establish a platform for identifying best practices. Chronic cystic fibrosis therapies hav...
Source: Current Opinion in Pulmonary Medicine - October 8, 2020 Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

Lung Clearance Index to Track Acute Respiratory Events in School-age Children with Cystic Fibrosis.
CONCLUSIONS: In school-age children with CF, LCI is a sensitive measure to assess lung function worsening with acute respiratory events and incomplete recovery at follow-up. LCI and FEV1 in combination capture a higher proportion of events with functional impairment. PMID: 33030967 [PubMed - as supplied by publisher] (Source: American Journal of Respiratory and Critical Care Medicine)
Source: American Journal of Respiratory and Critical Care Medicine - October 8, 2020 Category: Respiratory Medicine Authors: Perrem L, Stanojevic S, Shaw M, Jensen R, McDonald N, Isaac SM, Davis M, Clem C, Guido J, Jara S, France L, Solomon M, Grasemann H, Waters V, Sweezey N, Sanders DB, Davis SD, Ratjen F Tags: Am J Respir Crit Care Med Source Type: research

The Enemy of my Enemy: Bacterial Competition in the Cystic Fibrosis Lung.
Abstract Bacterial competition within host-associated polymicrobial communities shapes their composition, often with far-reaching consequences for human health. In this issue of Cell Host & Microbe, Perault et al. reveal how competition between two opportunistic pathogens could account for the epidemiology of chronic lung infections in people with cystic fibrosis. PMID: 33031766 [PubMed - in process] (Source: Cell Host and Microbe)
Source: Cell Host and Microbe - October 7, 2020 Category: Microbiology Authors: Kazmierczak BI Tags: Cell Host Microbe Source Type: research