Association of bone mineral density with lung function in a Chinese general population: the Xinxiang rural cohort study
Bone mineral density (BMD) has been positively associated with lung function in patients diagnosed with respiratory diseases such as chronic obstructive pulmonary disease (COPD) and cystic fibrosis. However, t... (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - December 9, 2019 Category: Respiratory Medicine Authors: Xiang Zeng, Dongling Liu, Xiangmei Zhao, Ling Chao, Yuchun Li, Huijun Li, Wen Li, Lihui Gui and Weidong Wu Tags: Research article Source Type: research

Functional rescue of c.3846G > A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action
Cystic Fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR is an ATP- and PKA-dependent chloride channel, regulating chloride and bicarbonate ion flux across apical membranes of polarized epithelial cells [1 –3]. To date, over 2000 mutations have been reported in the CFTR gene (CFTR1 database, http://www.genet.sickkids.on.ca). Around 10% of these mutations result in the formation of premature termination codons (PTCs) and prevent synthesis of full length CFTR mRNA and protein. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - December 9, 2019 Category: Respiratory Medicine Authors: Onofrio Laselva, Paul DW Eckford, Claire Bartlett, Hong Ouyang, Tarini NA Gunawardena, Tanja Gonska, Theo J Moraes, Christine E Bear Source Type: research

Congenital bilateral absence of the vas deferens (CBAVD): do genetic disorders modify assisted reproductive technologies outcomes?
CONCLUSION: The analysis of the presence of 5T polymporphism in CBAVD patients may add information when predicting the outcome of assisted reproductive techniques. PMID: 31797807 [PubMed - in process] (Source: Archivos Espanoles de Urologia)
Source: Archivos Espanoles de Urologia - December 6, 2019 Category: Urology & Nephrology Tags: Arch Esp Urol Source Type: research

Lung T-Cell Profile Alterations are Associated with Bronchiolitis Obliterans Syndrome in Cystic Fibrosis Lung Transplant Recipients
AbstractThe contribution of T-cells after lung transplant (LTx) remains controversial with no current consensus of their role concerning chronic lung allograft dysfunction. Using flow cytometry to assess T-cell subsets of bronchoalveolar lavage fluid (BALF) in 16 cystic fibrosis (CF) LTx recipients, we identified a decline in CD4+ T-cell frequency and an increase in CD8+ T-cell frequency in patients who developed severe bronchiolitis obliterans syndrome (BOS) (N = 10) when comparing baseline (6 months post-LTx) and follow-up (most recent bronchoscopy—clinical or surveillance per protocol). Comparin...
Source: Lung - December 5, 2019 Category: Respiratory Medicine Source Type: research

Strong toll-like receptor responses in cystic fibrosis patients are associated with higher lung function
Cystic Fibrosis pulmonary disease is characterized by chronic airway infections and concomitant non-resolving inflammation that usually leads to respiratory failure within the fourth decade of life. The disease results from patients bearing two mutant copies of the cystic fibrosis transmembrane conductance regulator (CFTR), a channel that transports chloride and bicarbonate [1]. Over 2000 different mutations in CFTR have been identified, and these mutations are associated with varying severity of disease based on how much residual CFTR activity remains. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - December 5, 2019 Category: Respiratory Medicine Authors: Susanna Kosamo, Katherine B. Hisert, Victoria Dmyterko, Catherine Nguyen, R. Anthony Black, Tarah D. Holden, Frank Radella, Pauline A. Cotten, Christopher H. Goss, Moira L. Aitken, Mark M. Wurfel Tags: Original Article Source Type: research

Treatment for avascular necrosis of bone in people with sickle cell disease.
CONCLUSIONS: We found no evidence that adding hip core decompression to physical therapy achieves clinical improvement in people with sickle cell disease with avascular necrosis of bone compared to physical therapy alone. However, we highlight that our conclusion is based on one trial with high attrition rates. Further randomized controlled trials are necessary to evaluate the role of hip-core depression for this clinical condition. Endpoints should focus on participants' subjective experience (e.g. quality of life and pain) as well as more objective 'time-to-event' measures (e.g. mortality, survival, hip longevity). The a...
Source: Cochrane Database of Systematic Reviews - December 5, 2019 Category: General Medicine Authors: Martí-Carvajal AJ, Solà I, Agreda-Pérez LH Tags: Cochrane Database Syst Rev Source Type: research

Effects of synbiotic supplementation on the pulmonary manifestations and anthropometric measurements in children with cystic fibrosis- a randomized clinical trial
ConclusionsThe synbiotic had no significant effect on pulmonary and anthropometric outcomes in children with CF. Further studies are necessary to confirm these findings. (Source: European Journal of Integrative Medicine)
Source: European Journal of Integrative Medicine - December 4, 2019 Category: Complementary Medicine Source Type: research

Call for papers on cystic fibrosis: a collaborative research session featuring the European Respiratory Journal, The Lancet Respiratory Medicine and the Journal of Cystic Fibrosis
For the past four years, a research session at the annual European Cystic Fibrosis Society (ECFS) meeting has been co-hosted by The Lancet Respiratory Medicine and the Journal of Cystic Fibrosis. At the 2020 ECFS meeting in June in Lyon, France, we are pleased to announce that the European Respiratory Journal will join this session for the first time. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 4, 2019 Category: Respiratory Medicine Tags: Call for papers Source Type: research

Inhibition of ATP hydrolysis restores airway surface liquid production in cystic fibrosis airway epithelia.
Abstract Airway surface dehydration is a pathological feature of cystic fibrosis (CF) lung disease. CF is caused by mutations in the CF transmembrane conductance regulator (CFTR), a cyclic AMP-regulated Cl- channel controlled in part by the adenosine A2B receptor. An alternative, CFTR-independent mechanism of fluid secretion is regulated by ATP, via the P2Y2 receptor (P2Y2R) that activates Ca2+-regulated Cl- channels (CaCC/TMEM16) and inhibits Na+ absorption. However, due to rapid ATP hydrolysis, steady-state ATP levels in CF airway surface liquid (ASL) are inadequate to maintain P2Y2R-mediated fluid secretion. Th...
Source: American Journal of Physiology. Lung Cellular and Molecular Physiology - December 4, 2019 Category: Cytology Authors: van Heusden C, Button B, Anderson WH, Ceppe A, Morton LC, O'Neal WK, Dang H, Alexis NE, Donaldson SH, Stephan H, Boucher RC, Lazarowski ER Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research

[ASAP] Multitarget CFTR Modulators Endowed with Multiple Beneficial Side Effects for Cystic Fibrosis Patients: Toward a Simplified Therapeutic Approach < xref rid="notes-1" ref-type="statement" > † < /xref >
Journal of Medicinal ChemistryDOI: 10.1021/acs.jmedchem.9b01416 (Source: Journal of Medicinal Chemistry)
Source: Journal of Medicinal Chemistry - December 3, 2019 Category: Chemistry Authors: Sabrina Tassini ‡, Emily Langron?, Leen Delang§, Carmen Mirabelli§, Kristina Lanko§, Emmanuele Crespan?, Miroslava Kissova?, Giulia Tagliavini?, Greta Fonto`‡, Simona Bertoni‡, Simone Palese‡, Carmine Giorgio‡, Francesca Ravanetti#, Luisa Rag Source Type: research

Mortality risk and causes of death in patients with non-cystic fibrosis bronchiectasis
All-cause mortality risk and causes of death in bronchiectasis patients have not been fully investigated. The aim of this study was to compare the mortality risk and causes of death between individuals with br... (Source: Respiratory Research)
Source: Respiratory Research - December 3, 2019 Category: Respiratory Medicine Authors: Sooim Sin, Seo Young Yun, Jee Min Kim, Chang Min Park, Jaeyoung Cho, Sun Mi Choi, Jinwoo Lee, Young Sik Park, Sang-Min Lee, Chul-Gyu Yoo, Young Whan Kim, Sung Koo Han and Chang-Hoon Lee Tags: Research Source Type: research

Proof of concept for identifying cystic fibrosis from perspiration samples [Medical Sciences]
We present an alternative method for the identification of CF by combining desorption electrospray ionization mass... (Source: Proceedings of the National Academy of Sciences)
Source: Proceedings of the National Academy of Sciences - December 3, 2019 Category: Science Authors: Zhenpeng Zhou, Daniel Alvarez, Carlos Milla, Richard N. Zare Tags: Physical Sciences Source Type: research

A quieter year for new drug approvals
FDA approved fewer drugs, but therapies for cystic fibrosis, postpartum depression stand out (Source: Chemical and Engineering News)
Source: Chemical and Engineering News - December 2, 2019 Category: Chemistry Authors: Megha Satyanarayana Source Type: research

Colonisation of Staphylococcus aureus in patients with Nasal Polyposis.
CONCLUSION: Our results did not show that S. aureus found in nasal mucosa membrane is significantly different in patients with or without NP. However, association of the presence of S. aureus in patients with nasal polyposis with asthma, allergy and inflammation has been shown. PMID: 31785225 [PubMed - as supplied by publisher] (Source: Neuroendocrinology Letters)
Source: Neuroendocrinology Letters - December 1, 2019 Category: Endocrinology Tags: Neuro Endocrinol Lett Source Type: research

The Family of Chloride Channel Regulator, Calcium-activated Proteins in the Feline Respiratory Tract: A Comparative Perspective on Airway Diseases in Man and Animal Models
Publication date: January 2020Source: Journal of Comparative Pathology, Volume 174Author(s): N.A. Erickson, A.D. Gruber, L. MundhenkSummaryMembers of the chloride channel regulator, calcium-activated (CLCA) family are considered to be modifiers in inflammatory, mucus-based respiratory conditions such as asthma and cystic fibrosis. Previous work has shown substantial differences between human and murine CLCA orthologues that limit the value of mouse models. As an alternative, the cat is an unfamiliar but powerful model of human asthma. We therefore characterized the expression profiles of CLCA proteins in the feline respira...
Source: Journal of Comparative Pathology - November 30, 2019 Category: Pathology Source Type: research

Presentation of an H-type tracheoesophageal fistula in an adolescent male with cystic fibrosis: A case report and review of literature
Congenital TEFs without esophageal atresia are rare but may occur more frequently than previously documented in literature. Careful history is required to suspect the diagnoses, as most patients will present with coughing associated with solid or liquids, recurrent unexplained pulmonary infections and complaints with eating. Some patients may show signs of chronic airway changes from recurrent aspiration pneumonia at the time of presentation. Diagnosis is challenging, with multiple imaging modalities including x ray, CT scan and esophogram able to identify a fistula. (Source: Clinical Imaging)
Source: Clinical Imaging - November 30, 2019 Category: Radiology Authors: Timothy M. Klouda, Erika Lindholm, Erica Poletto, Seema Rani, Laurie Varlotta, Jeanne Velasco Tags: Pediatric Radiology Source Type: research

Recapitulation of polymicrobial communities associated with cystic fibrosis airway infections: a perspective
Future Microbiology, Ahead of Print. (Source: Future Microbiology)
Source: Future Microbiology - November 29, 2019 Category: Microbiology Authors: Thomas J O'Brien Martin Welch Source Type: research

Perspiration interventions for conservative management of kidney disease and uremia
Purpose of review There has been an increasing interest in developing novel technologies to treat patients with chronic kidney disease as evidenced by KidneyX, the public–private partnership between government and industry. Perhaps a simple technology for treating kidney failure would be to utilize perspiration. It is a physiological process, and when used properly it might not be an unpleasant experience. This review will explore the current state of knowledge regarding perspiration therapy in the setting of far advanced kidney failure. Recent findings A literature review using the PubMed database was conducted...
Source: Current Opinion in Nephrology and Hypertension - November 29, 2019 Category: Urology & Nephrology Tags: NOVEL THERAPEUTIC APPROACHES IN NEPHROLOGY AND HYPERTENSION: Edited by Kamyar Kalantar-Zadeh and Ekamol Tantisattamo Source Type: research

Obstructive lung diseases and risk of rheumatoid arthritis.
Authors: Friedlander HM, Ford JA, Zaccardelli A, Terrio AV, Cho MH, Sparks JA Abstract Introduction: Smoking is an established risk factor for both lung diseases and rheumatoid arthritis (RA). Chronic mucosal airway inflammation may result in immune tolerance loss, neoantigen formation, and production of RA-related autoantibodies that increase the subsequent risk of RA. In this review, we aimed to summarize the current evidence supporting the role of obstructive lung diseases and subsequent risk of RA.Areas covered: We identified scientific articles discussing the biologic mechanisms linking mucosal airway inflamma...
Source: Expert Review of Clinical Immunology - November 29, 2019 Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research

Editorial: Emerging Therapeutic Approaches for Cystic Fibrosis
(Source: Frontiers in Pharmacology)
Source: Frontiers in Pharmacology - November 29, 2019 Category: Drugs & Pharmacology Source Type: research

Elexacaftor/Ivacaftor/Tezacaftor: First Approval
This article summarizes the milestones in the development of elexacaftor/ivacaftor/tezacaftor leading to this first approval for the treatment of cystic fibrosis in patients aged  ≥ 12 years who have ≥ 1F508del mutation in theCFTR gene. (Source: Drugs)
Source: Drugs - November 29, 2019 Category: Drugs & Pharmacology Source Type: research

The KDEL receptor has a role in the biogenesis and trafficking of the epithelial sodium channel (ENaC) [Protein Synthesis and Degradation]
Endoplasmic reticulum protein of 29 kDa (ERp29) is a thioredoxin-homologous endoplasmic reticulum (ER) protein that regulates the biogenesis of cystic fibrosis transmembrane conductance regulator (CFTR) and the epithelial sodium channel (ENaC). ERp29 may promote ENaC cleavage and increased open probability by directing ENaC to the Golgi via coat complex II (COP II) during biogenesis. We hypothesized that ERp29's C-terminal KEEL ER retention motif, a KDEL variant that is associated with less robust ER retention, strongly influences its regulation of ENaC biogenesis. As predicted by our previous work, depletion of Sec24D, th...
Source: Journal of Biological Chemistry - November 29, 2019 Category: Chemistry Authors: Yann Bikard, Jeffrey Viviano, Melissa N. Orr, Lauren Brown, Margaret Brecker, Jonathan Litvak Jeger, Daniel Grits, Laurence Suaud, Ronald C. Rubenstein Tags: Cell Biology Source Type: research

The anion transporter SLC26A9 localizes to tight ȷunctions and is degraded by the proteasome when co-expressed with F508del-CFTR [Molecular Bases of Disease]
Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) disrupt epithelial secretion and cause cystic fibrosis (CF). Available CFTR modulators provide only modest clinical benefits, so alternative therapeutic targets are being explored. The anion-conducting transporter solute carrier family 26 member 9 (SLC26A9) is a promising candidate, but its functional expression is drastically reduced in cells that express the most common CF-associated CFTR variant, F508del–CFTR, through mechanisms that remain incompletely understood. Here, we examined the metabolic stability and location of...
Source: Journal of Biological Chemistry - November 29, 2019 Category: Chemistry Authors: Yukiko Sato, David Y. Thomas, John W. Hanrahan Tags: Membrane Biology Source Type: research

Multidrug-Resistant and Clinically Relevant Gram-Negative Bacteria Are Present in German Surface Waters
Water is considered to play a role in the dissemination of antibiotic-resistant Gram-negative bacteria including those encoding Extended-spectrum beta-lactamases (ESBL) and carbapenemases. To investigate the role of water for their spread in more detail, we characterized ESBL/Carbapenemase-producing bacteria from surface water and sediment samples using phenotypic and genotypic approaches. ESBL/Carbapenemase-producing isolates were obtained from water/sediment samples. Species and antibiotic resistance were determined. A subset of these isolates (n = 33) was whole-genome-sequenced and analyzed for the presence of antibioti...
Source: Frontiers in Microbiology - November 29, 2019 Category: Microbiology Source Type: research

Measures of Cystic Fibrosis Airway Microbiota during Periods of Clinical Stability
Annals of the American Thoracic Society,Volume 16, Issue 12, Page 1534-1542, December 2019. (Source: Annals of the American Thoracic Society)
Source: Annals of the American Thoracic Society - November 28, 2019 Category: Respiratory Medicine Authors: Lindsay J. Caverly Junnan Lu Lisa A. Carmody Linda M. Kalikin Kerby Shedden Kristopher Opron Michelle Azar Shannon Cahalan Bridget Foster Donald R. VanDevanter Richard H. Simon John J. LiPuma Source Type: research

Improvement in contraceptive coverage and gynecological care of adult women with cystic fibrosis following the implementation of an on-site gynecological consultation
Our study aimed to evaluate the impact of the introduction of a new gynecologic referral service in our adult Cystic Fibrosis (CF) center on contraceptive coverage, gynecological follow-up regularity, and cervical cancer screening coverage. (Source: Contraception)
Source: Contraception - November 28, 2019 Category: OBGYN Authors: C. Rousset-Jablonski, Q. Reynaud, M. Perceval, R. Nove-Josserand, S. Durupt, I Ray-Coquard, F. Golfier, I. Durieu Tags: Original Research Article Source Type: research

Intestinal organoids for Cystic Fibrosis research
Significant progress has been made in the development of CFTR modulator therapy; however, current CFTR modulator therapies are only available for a minority of the CF-patient population. Additionally, heterogeneity in in vivo modulator response has been reported among individuals carrying homozygous F508del-CFTR, adding to the desire for an optimal prediction of response-to-therapy on an individual level. In the last decade, a lot of progress has been made in the development of primary cell cultures into 3D patient-derived disease models. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - November 28, 2019 Category: Respiratory Medicine Authors: E. de Poel, J.W. Lefferts, J.M. Beekman Tags: Review Source Type: research

CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth
Cystic fibrosis (CF) disease, which is caused by mutations of the CF transmembrane conductance regulator (CFTR) gene, is characterized by multiorgan deficiencies that begin early in life [1,2]. With the increasing life expectancy due to improvements in the treatment of the disease's pulmonary and gastrointestinal disorders, other CF complications and comorbidities have become more prevalent, such as diabetes and CF-related bone disease (CFBD) with 55 –65% of affected patients being older than 45 [3–5]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - November 28, 2019 Category: Respiratory Medicine Authors: Julien Braux, Marie-Laure Jourdain, Christine Guillaume, Val érie Untereiner, Olivier Piot, Andrea Baehr, Nikolai Klymiuk, Nathalie Winter, Mustapha Berri, Dominique Buzoni-Gatel, Ignaccio Caballero, Antoine Guillon, Mustapha Si-Tahar, Jacky Jacquot, Fr Tags: Original Article Source Type: research

Novel intermicrobial molecular interaction: Pseudomonas aeruginosa Quinolone Signal (PQS) modulates Aspergillus fumigatus response to iron.
ns DA Abstract Pseudomonas aeruginosa (Pa) and Aspergillus fumigatus (Af), the commonest bacterium and fungus in compromised host airways, compete for iron (Fe). The Pseudomonas quinolone signal (PQS), a Pa quorum sensing molecule, also chelates Fe, and delivers Fe to the Pa cell membrane using Pa siderophores. In models of Af biofilm formation or preformed biofilms, PQS inhibited Af in a low Fe environment. AfΔsidA (mutant unable to produce siderophores) biofilm was more sensitive to PQS inhibition than wild-type (WT), as was planktonic AfΔsidA growth. PQS decreased WT Af growth on agar. All these inh...
Source: Microbiology - November 28, 2019 Category: Microbiology Authors: Nazik H, Sass G, Ansari SR, Ertekin R, Haas H, Déziel E, Stevens DA Tags: Microbiology Source Type: research

Ramachandran Conformational Energy Maps for Disaccharide Linkages found in Burkholderia multivorans Biofilm Polysaccharides.
Abstract Ramachandran conformational energy maps have been prepared for all of the glycosidic linkages found in the C1576 exopolysaccharide that constitutes the biofilms of the bacterial species Burkholderia multivorans, a member of the Burkholderia cepacian complex that was isolated from a cystic fibrosis patient. This polysaccharide is a rhamnomannan with a tetrasaccharide repeat unit containing two mannose residues and two rhamnose residues, -[3-α-D-Man-(1→2)- α -D-Man-(1→2)- α -D-Rha-(1→3)- α -D-Rha-(1→]n-, where approximately 50% of the rhamnoses are randomly methyl...
Source: International Journal of Biological Macromolecules - November 28, 2019 Category: Biochemistry Authors: Jou IA, Caterino M, Schnupf U, Rizzo R, Cescutti P, Brady JW Tags: Int J Biol Macromol Source Type: research

Positive Newborn Screening for Cystic Fibrosis, What to Do Next?
(Source: Indian Journal of Pediatrics)
Source: Indian Journal of Pediatrics - November 27, 2019 Category: Pediatrics Source Type: research

What She Leaves Behind.
Abstract Cystic fibrosis and an unfinished life. PMID: 31764059 [PubMed - in process] (Source: The American Journal of Nursing)
Source: The American Journal of Nursing - November 27, 2019 Category: Nursing Authors: Brown T Tags: Am J Nurs Source Type: research

Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis.
CONCLUSIONS: The evidence provided by this review is of variable quality, but suggests that all techniques and devices described may have a place in the clinical treatment of people with CF. Following meta-analyses of the effects of PEP versus other airway clearance techniques on lung function and patient preference, this Cochrane Review demonstrated that there was high-quality evidence that showed a significant reduction in pulmonary exacerbations when PEP using a mask was compared with HFCWO. It is important to note that airway clearance techniques should be individualised throughout life according to developmental stage...
Source: Cochrane Database of Systematic Reviews - November 27, 2019 Category: General Medicine Authors: McIlwaine M, Button B, Nevitt SJ Tags: Cochrane Database Syst Rev Source Type: research

Cystic fibrosis: a call for papers for ECFS 2020
Publication date: December 2019Source: The Lancet Respiratory Medicine, Volume 7, Issue 12Author(s): Emma Grainger (Source: The Lancet Respiratory Medicine)
Source: The Lancet Respiratory Medicine - November 26, 2019 Category: Respiratory Medicine Source Type: research

Increased expression of anion transporter SLC26A9 delays diabetes onset in cystic fibrosis
Diabetes is a common complication of cystic fibrosis (CF) that affects approximately 20% of adolescents and 40%–50% of adults with CF. The age at onset of CF-related diabetes (CFRD) (marked by clinical diagnosis and treatment initiation) is an important measure of the disease process. DNA variants associated with age at onset of CFRD reside in and near SLC26A9. Deep sequencing of the SLC26A9 gene in 762 individuals with CF revealed that 2 common DNA haplotypes formed by the risk variants account for the association with diabetes. Single-cell RNA sequencing (scRNA-Seq) indicated that SLC26A9 is predominantly expressed...
Source: Journal of Clinical Investigation - November 26, 2019 Category: Biomedical Science Authors: Anh-Thu N. Lam, Melis A. Aksit, Briana Vecchio-Pagan, Celeste A. Shelton, Derek L. Osorio, Arianna F. Anzmann, Loyal A. Goff, David C. Whitcomb, Scott M. Blackman, Garry R. Cutting Source Type: research

Adherence of adolescents with cystic fibrosis to enzyme replacement therapy: associated factors
This article sets out to evaluate the prevalence and factors associated with adherence to enzyme replacement therapy among adolescents with cystic fibrosis. It is a cross-sectional, descriptive and observational study. Sociodemographic and clinical data were collected. The instruments used to assess adherence were: the Morisky-Green questionnaire and medication dispensation at the pharmacy, and interviews with structured questionnaires for the associated factors. Forty-four adolescents were interviewed. According to the method of the pharmacy medication dispensation analysis and the Morisky-Green questionnaire, the adheren...
Source: Ciencia e Saude Coletiva - November 26, 2019 Category: Occupational Health Source Type: research

Using photo-elicitation to explore perceptions of physical activity among young people with cystic fibrosis
Physical activity is recommended in the management of cystic fibrosis (CF). The aim of this study was to explore motives, barriers and enablers to physical activity among this population. Methods: Twelve parti... (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - November 26, 2019 Category: Respiratory Medicine Authors: S. Denford, D. M. Hill, K. A. Mackintosh, M. A. McNarry, A. R. Barker and C. A. Williams Tags: Research article Source Type: research

Duration of Antibiotic Therapy in Non-cystic Fibrosis Bronchiectasis
AbstractPurpose of ReviewWe conducted a review of the current evidence relating to antibiotic duration in the short- and long-term management of non-cystic fibrosis bronchiectasis.Recent FindingsIn non-cystic fibrosis pulmonary exacerbations, evidence is primarily based on expert consensus and recent guidelines recommend antibiotic durations of approximately 14  days. Chronic antibiotics (oral or inhaled) are recommended in patients with frequent exacerbations or with chronicPseudomonas aeruginosa airways infection. Macrolides are the best studied therapies for long-term use with evidence for effect limited to a 12-mo...
Source: Current Respiratory Care Reports - November 26, 2019 Category: Respiratory Medicine Source Type: research

Ivacaftor in cystic fibrosis with residual function: Lung function results from an N-of-1 study
Ivacaftor (Kalydeco ®) is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that enhances chloride transport in multiple mutant CFTR forms in vitro, including the G551D-CFTR mutation, other severe gating mutations, and certain mutations associated with residual CFTR function [1–3]. Clinical st udies first established the efficacy and safety of ivacaftor in patients with CF and a G551D mutation or other severe gating mutations (ie, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R) [4–9]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - November 26, 2019 Category: Respiratory Medicine Authors: Jerry A. Nick, Connie St. Clair, Marion C. Jones, Lan Lan, Mark Higgins, VX12-770-113 Study Team Tags: Original Article Source Type: research

Lower Airway Infection in Preschool Children with Cystic Fibrosis - An International Comparison.
PMID: 31769996 [PubMed - as supplied by publisher] (Source: American Journal of Respiratory and Critical Care Medicine)
Source: American Journal of Respiratory and Critical Care Medicine - November 26, 2019 Category: Respiratory Medicine Authors: Hulme KM, Linnane B, McNally PG Tags: Am J Respir Crit Care Med Source Type: research

Characterization of gallium resistance induced in a Pseudomonas aeruginosa cystic fibrosis isolate.
Maeda T, García-Contreras R Abstract The repurposing of gallium nitrate as an antibacterial, a drug used previously for the treatment of hypercalcemia, is a plausible alternative to combat infections by Pseudomonas aeruginosa, since it has antipseudomonal properties in vitro and in vivo in animal models and in human lung infections. Furthermore, gallium nitrate tolerance in clinical isolates is very rare. Nevertheless, studies on the reference strains PA14 and PAO1 show that resistance against gallium nitrate is achieved by decreasing gallium intracellular levels by increasing the production of pyocyanin. ...
Source: Archives of Microbiology - November 26, 2019 Category: Microbiology Authors: Tovar-García A, Angarita-Zapata V, Cazares A, Jasso-Chávez R, Belmont-Díaz J, Sanchez-Torres V, López-Jacome LE, Coria-Jiménez R, Maeda T, García-Contreras R Tags: Arch Microbiol Source Type: research

Whole genome analysis of Pandoraea species strains from cystic fibrosis patients
Future Microbiology, Ahead of Print. (Source: Future Microbiology)
Source: Future Microbiology - November 25, 2019 Category: Microbiology Authors: Jumamurat R Bayjanov Miquel B Ekkelenkamp Malbert RC Rogers Rafael Cant ón Barry J Benaissa-Trouw Mar ía Díez-Aguilar Michael Tunney Ad C Fluit Source Type: research

What She Leaves Behind
Cystic fibrosis and an unfinished life. (Source: AJN)
Source: AJN - November 25, 2019 Category: Nursing Tags: What I'm Reading Source Type: research

Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis
Cystic fibrosis (CF) is one of the most frequent genetic diseases and the median survival of these patients has improved in the last few decades, therefore it becomes necessary to evaluate the long-term compli... (Source: Journal of Translational Medicine)
Source: Journal of Translational Medicine - November 25, 2019 Category: Research Authors: Silvia Lai, Sandro Mazzaferro, Anna Paola Mitterhofer, Enea Bonci, Paolo Giangreco Marotta, Francesco Pelligra, Manuel Murciano, Camilla Celani, Patrizia Troiani, Giuseppe Cimino and Paolo Palange Tags: Research Source Type: research

A Novel Technique for Thoracic Duct Access through MR Imaging/Ultrasound Fusion: Successful Percutaneous Embolization of Pulmonary Lymphatic Vessels
Plastic bronchitis is a potentially fatal disorder characterized by the exudation of proteinaceous material and cells in the airways that leads to branching cast formation, which can be expectorated or lead to asphyxiation. Plastic bronchitis can appear as a complication after single-ventricle palliation. Other causes of plastic bronchitis that have been described include cystic fibrosis, sickle-cell anemia, asthma, and lymphangiomatosis. The underlying pathophysiology of plastic bronchitis seems to be abnormal pulmonary lymphatic perfusion, and the preferred treatment is embolization of the abnormal pulmonary lymphatic ve...
Source: Journal of Vascular and Interventional Radiology : JVIR - November 25, 2019 Category: Radiology Authors: Patricio Vargas, Benjamin Horwitz, Gian Paolo Zamboni, Daniel Hasson, Maria Faure, Pablo Soffia, Cesar Salinas Tags: Letter to the Editor Source Type: research

Airway Mucus Hyperconcentration in Non-Cystic Fibrosis Bronchiectasis.
CONCLUSIONS: Hyper-concentrated airway mucus is characteristic of bronchiectasis subjects, likely contributes to disease pathophysiology, and may be a target for pharmacotherapy. PMID: 31765597 [PubMed - as supplied by publisher] (Source: American Journal of Respiratory and Critical Care Medicine)
Source: American Journal of Respiratory and Critical Care Medicine - November 25, 2019 Category: Respiratory Medicine Authors: Ramsey KA, Chen ACH, Radicioni G, Lourie R, Martin M, Broomfield A, Sheng YH, Hasnain SZ, Radford-Smith G, Simms LA, Burr L, Thornton DJ, Bowler SD, Livengood S, Ceppe A, Knowles MR, Donaldson SH, Hill DB, Ehre C, Button B, Alexis NE, Kesimer M, Boucher R Tags: Am J Respir Crit Care Med Source Type: research

Discovery of ubonodin, an antimicrobial lasso peptide active against members of the Burkholderia cepacia complex.
We report the heterologous expression, structure, and antimicrobial activity of a lasso peptide, ubonodin, encoded in the genome of Burkholderia ubonensis . The topology of ubonodin is unprecedented amongst lasso peptides with 18 of its 28 amino acids found in the mechanically-bonded loop segment. Ubonodin inhibits RNA polymerase in vitro and has potent antimicrobial activity against several pathogenic members of the Burkholderia genus, most notably B. cepacia and B. multivorans , causative agents of lung infections in cystic fibrosis patients. PMID: 31765515 [PubMed - as supplied by publisher] (Source: Chembiochem)
Source: Chembiochem - November 25, 2019 Category: Biochemistry Authors: Cheung-Lee WL, Parry M, Zong C, Jaramillo Cartagena A, Darst S, Connell N, Russo R, Link AJ Tags: Chembiochem Source Type: research

Susceptibility of Pseudomonas aeruginosa recovered from cystic fibrosis patients to murepavadin and thirteen comparator antibiotics.
Conclusions: P. aeruginosa CF isolates exhibited the lowest resistance rates against ceftolozane-tazobactam, ceftazidime-avibactam and colistin. Murepavadin demonstrated the highest activity on a per weight basis and may therefore become a valuable addition to the currently available antibiotics for treatment of respiratory infection in people with CF. PMID: 31767727 [PubMed - as supplied by publisher] (Source: Antimicrobial Agents and Chemotherapy)
Source: Antimicrobial Agents and Chemotherapy - November 25, 2019 Category: Microbiology Authors: Ekkelenkamp MB, Cantón R, Díez-Aguilar M, Tunney MM, Gilpin DF, Bernardini F, Dale GE, Elborn JS, Bayjanov JR, Fluit A Tags: Antimicrob Agents Chemother Source Type: research

Integrating the multiple breath washout test into international multicentre trials
Forced expiratory volume in 1  s (FEV1), measured by spirometry, has been a primary endpoint in cystic fibrosis (CF) clinical trials for many decades. However, as lung function improves for patients with CF, FEV1 now remains in the normal range for the majority of paediatric patients and a significant proportion of adults [1,2] . The insensitivity of FEV1 to detect early lung disease and the beneficial effect of interventions has been highlighted by studies comparing spirometry with more sensitive lung function tests and imaging-based assessments of structural disease [2–5]. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - November 24, 2019 Category: Respiratory Medicine Authors: Clare Saunders, Renee Jensen, Paul D. Robinson, Sanja Stanojevic, Michelle Klingel, Christopher Short, Jane C. Davies, Felix Ratjen Tags: Original Article Source Type: research

Positive clinical outcomes following ivacaftor treatment in a cystic fibrosis patient with the genotype 3272 –26A  >  G/Q493X
We present a case of sustained improvements in several different clinical domains following ivacaftor treatment in a cystic fibrosis patient with the functional class 5 CFTR mutation 3272 –26A > G and a functional class 1 mutation in compound heterozygosity. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - November 23, 2019 Category: Respiratory Medicine Authors: M. Pallin, C.P. Daley Tags: Letter to the Editor Source Type: research