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Cystic Fibrosis

Leander Jonckheere, Petra Schelstraete, Leen Van Simaey, Eva Van Braeckel, Julie Willekens, Sabine Van Daele, Frans De Baets, Mario Vaneechoutte
After antibiotic eradication treatment for a first ever Pseudomonas aeruginosa isolation, the European consensus criteria (ECC) are widely used to assess colonization status with P. aeruginosa in CF-patients. We evaluated to what extent genotyping (GT) of subsequent P. aeruginosa isolates could predict/assess chronic colonization (CC), in comparison with the ECC. METHODS: Over a 14-year period, sputa were cultured from 80 CF-patients (age range: 2-51 years), from a first ever isolation of P...
April 11, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Lisa Saiman, Juyan J Zhou, Xiaotong Jiang, Michael R Kosorok, Marianne S Muhlebach
OBJECTIVEIn 2013, the Cystic Fibrosis (CF) Foundation developed an updated guideline for infection prevention and control (IP&C) practices for CF. We sought to assess the adoption of specific recommendations by CF care centers.METHODSDirectors of the 277 CF care centers in the United States were asked to complete a confidential online survey regarding the adoption of selected IP&C recommendations. Selected recommendations were those we considered less likely to be incorporated into a center's written IP&C policies...
April 15, 2018: Infection Control and Hospital Epidemiology
Stephen P Newman
The repurposing of drug delivery by the pulmonary route has been applied to treatment and prophylaxis of an increasingly wide range of respiratory diseases. Repurposing has been most successful for the delivery of inhaled bronchodilators and corticosteroids in patients with asthma and chronic obstructive pulmonary disease (COPD). Repurposing utilizes the advantages that the pulmonary route offers in terms of more targeted delivery to the site of action, the use of smaller doses, and a lower incidence of side-effects...
April 10, 2018: Advanced Drug Delivery Reviews
Shan F Brunel, Janet A Willment, Gordon D Brown, Graham Devereux, Adilia Warris
Aspergillus fumigatus infects up to 50% of cystic fibrosis (CF) patients and may play a role in progressive lung disease. As cystic fibrosis transmembrane conductance regulator is expressed in cells of the innate immune system, we hypothesised that impaired antifungal immune responses play a role in CF-related Aspergillus lung disease. Peripheral blood mononuclear cells, polymorphonuclear cells (PMN) and monocytes were isolated from blood samples taken from CF patients and healthy volunteers. Live-cell imaging and colorimetric assays were used to assess antifungal activity in vitro ...
April 2018: ERJ Open Research
Graciela Rodríguez-Sevilla, Charlotte Rigauts, Eva Vandeplassche, Lisa Ostyn, Ignacio Mahíllo-Fernández, Jaime Esteban, Concepción Pérez-Jorge Peremarch, Tom Coenye, Aurélie Crabbé
Mycobacterium abscessus lung infection is a major health problem for cystic fibrosis (CF) patients. Understanding the in vivo factors that influence the outcome of therapy may help addressing the poor correlation between in vitro and in vivo antibiotic efficacy. We evaluated the influence of interspecies interactions and lung epithelial cells on antibiotic efficacy. Therefore, single and dual species biofilms of M. abscessus and a major CF pathogen (Pseudomonas aeruginosa) were cultured on a plastic surface or on in vivo-like three-dimensional (3-D) lung epithelial cells, and the activity of antibiotics (colistin, amikacin, clarithromycin, ceftazidime) in inhibiting biofilm formation was evaluated...
April 10, 2018: Pathogens and Disease
Ashley L Heilingoetter, Bobby Tajudeen, Hannah N Kuhar, Paolo Gattuso, Ritu Ghai, Mahboobeh Mahdavinia, Pete S Batra
Background Structured histopathology reporting facilitates better understanding of the underlying pathophysiologic mechanisms of chronic rhinosinusitis. The microbiology of chronic rhinosinusitis has been studied extensively; however, distinct histopathologic changes associated with bacteria isolated in chronic rhinosinusitis are largely unknown. Objective The goal of this study is to better understand the relationship between culturable bacteria and histopathology in chronic rhinosinusitis. Methods A structured histopathology report was utilized to analyze sinus tissue removed during functional endoscopic sinus surgery in a group of patients with chronic rhinosinusitis refractory to medical therapy...
March 2018: American Journal of Rhinology & Allergy
X Y Yang, L P Zhu, X Q Liu, C Y Zhang, Y Yao, Y Wu
This case report is about one genetically specified diagnosed infant case of Caroli syndrome with autosomal recessive polycystic kidney disease (ARPKD) in China. The patient in this case report was an eight-month infant boy with an atypical onset and the main clinical manifestation was non-symptomatic enlargement of the liver and kidneys. The imaging study demonstrated a diffused cystic dilatation of intrahepatic bile ducts as well as polycystic changes in bilateral kidneys. The basic blood biochemical tests indicated a normal hepatorenal function...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Oliver W Meldrum, Gleb E Yakubov, Mauricio R Bonilla, Omkar Deshmukh, Michael A McGuckin, Michael J Gidley
Mucus is characterized by multiple levels of assembly at different length scales which result in a unique set of rheological (flow) and mechanical properties. These physical properties determine its biological function as a highly selective barrier for transport of water and nutrients, while blocking penetration of pathogens and foreign particles. Altered integrity of the mucus layer in the small intestine has been associated with a number of gastrointestinal tract pathologies such as Crohn's disease and cystic fibrosis...
April 11, 2018: Scientific Reports
Michelle E Wood, Rebecca E Stockwell, Graham R Johnson, Kay A Ramsay, Laura J Sherrard, Timothy J Kidd, Joyce Cheney, Emma L Ballard, Peter O'Rourke, Nassib Jabbour, Claire E Wainwright, Luke D Knibbs, Peter D Sly, Lidia Morawska, Scott C Bell
The airborne route is a potential pathway in the person-to-person transmission of bacterial strains among cystic fibrosis (CF) populations. In this cross-sectional study, we investigate the physical properties and survival of common non- Pseudomonas aeruginosa CF pathogens generated during coughing. We conclude that Gram-negative bacteria and Staphylococcus aureus are aerosolised during coughing, can travel up to 4 m and remain viable within droplet nuclei for up to 45 min. These results suggest that airborne person-to-person transmission is plausible for the CF pathogens we measured...
April 7, 2018: Thorax
Giovanni Marzaro, Ilaria Lampronti, Elisabetta D'Aversa, Gianni Sacchetti, Giorgia Miolo, Christian Vaccarin, Giulio Cabrini, Maria Cristina Dechecchi, Roberto Gambari, Adriana Chilin
A series of trimethylangelicin (TMA) derivatives were designed and synthesized to overcome the unwanted effects of TMA, promising agent for treatment of inflammation-related diseases and other pathologies, such as cystic fibrosis. The new generation TMA analogues bore hindered substituents at the 4 position in order to minimize or avoid the photoreactions with DNA. Among them, the 4-isopropyl-6-ethyl derivative 23 exhibited TMA-like inhibitory activity on NF-κB/DNA interactions but it proved unable to photoreact with pyrimidine bases of DNA, nor to induce any other DNA damage...
March 31, 2018: European Journal of Medicinal Chemistry
Iolo Doull
The number of published articles on Cystic Fibrosis (CF) continues to increase year on year. The evidence base for small molecule therapies in CF has continued to expand, with evidence for lumacaftor/ivacaftor in younger patients and longer-term evidence in adults, and pivotal studies on tezacaftor/ivacaftor. There were reports on emerging CFTR mutation agnostic therapies, and new evidence for long standing therapies.
March 15, 2018: Paediatric Respiratory Reviews
Sandra D Anderson, Evangelia Daviskas, John D Brannan, Hak Kim Chan
The story of how we came to use inhaled mannitol to diagnose asthma and to treat cystic fibrosis began when we were looking for a surrogate for exercise as a stimulus to identify asthma. We had proposed that exercise-induced asthma was caused by an increase in osmolarity of the periciliary fluid. We found hypertonic saline to be a surrogate for exercise but an ultrasonic nebuliser was required. We produced a dry powder of sodium chloride but it proved unstable. We developed a spray dried preparation of mannitol and found that bronchial responsiveness to inhaling mannitol identified people with currently active asthma...
April 4, 2018: Advanced Drug Delivery Reviews
Sophia Karandashova, Apparao Kummarapurugu, Shuo Zheng, Le Kang, Shumei Sun, Bruce K Rubin, Judith A Voynow
INTRODUCTION: Sphingolipids are associated with the regulation of pulmonary inflammation. Although sphingolipids have been investigated in the context of cystic fibrosis (CF), the focus has been on loss of CF transmembrane conductance regulator (CFTR) function in mice, and in CF human lung epithelial cell lines. The sphingolipid content of CF sputum and the potential link between ceramide and airway inflammation in CF remain relatively unexplored. METHODS: Fifteen patients with CF provided two spontaneously expectorated sputum samples, one collected during a hospitalization for an acute pulmonary exacerbation and one from an outpatient visit at a time of clinical stability...
April 6, 2018: Pediatric Pulmonology
Anke Jaudszus, Christin Arnold, Julia Hentschel, Kerstin Hünniger, Michael Baier, Jochen G Mainz
OBJECTIVES: Previously, we found linkages of inflammatory mediator levels in CF upper airways (UAW) sampled by nasal lavage (NL) to disease severity and to chronic pathogen colonization such as Pseudomonas aeruginosa (PsA). Here, we assess UAW cytokine dynamics in CF patients with a new PsA colonization. METHODS: We measured cytokines in 149 longitudinally obtained NL samples from 34 CF patients. Cytokine concentrations determined prior to, at the time of de novo PsA detection in either UAW or lower airways (LAW), and in a subsequent PsA free period in newly colonized patients (PsA-new/n = 7) were compared to levels of not- (PsA-free/n = 13) and chronically colonized patients (PsA-chron/n = 14)...
April 6, 2018: Pediatric Pulmonology
Pamela J McShane, Jeffry G Weers, Thomas E Tarara, Alfred Haynes, Preeti Durbha, Danforth P Miller, Tobias Mundry, Elisabeth Operschall, J Stuart Elborn
Bronchiectasis is a chronic respiratory disease with heterogeneous etiology, characterized by a cycle of bacterial infection and inflammation, resulting in increasing airway damage. Exacerbations are an important cause of morbidity and are strongly associated with disease progression. Many patients with bronchiectasis suffer from two or more exacerbations per year. However, there are no approved therapies to reduce or delay exacerbations in this patient population. Ciprofloxacin DPI is in development as a long-term, intermittent therapy to reduce exacerbations in patients with non-cystic fibrosis (CF) bronchiectasis and evidence of respiratory pathogens...
April 2, 2018: Pulmonary Pharmacology & Therapeutics
Diego P Peralta, Aymara Y Chang, Angie Ariza-Hutchinson, Catherine A Ho
Burkholderia multivorans is a member of the Burkholderia cepacia complex. Although it is usually associated with infections in patients with cystic fibrosis, chronic granulomatous disease, and immunosuppression, central nervous infections are not commonly reported. Moreover, management of these infections is difficult due to multiple mechanisms of bacterial resistance to antimicrobial agents. We report a 55-year-old-man who developed Burkholderia multivorans meningitis after two episodes of central line-associated bloodstream infections...
2018: IDCases
Jamie Wood, Abbey Sawyer, Siobhain Mulrennan, Andrew Bullock
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures...
July 2018: Respirology Case Reports
Onofrio Laselva, Steven Molinski, Valeria Casavola, Christine E Bear
The most common Cystic Fibrosis causing mutation is deletion of phenylalanine 508 (F508del), a mutation that leads to protein misassembly with defective processing. Small molecule correctors compounds: VX-809 or Corr-4a (C4) partially restore processing of the major mutant. These two prototypical corrector compounds cause an additive effect on F508del-CFTR processing and hence were proposed to act through distinct mechanisms: VX-809 stabilizing the first membrane spanning domain 1 (MSD1) and C4, acting on the second half of the molecule (consisting of MSD2 and/or nucleotide binding domain 2 (NBD2))...
April 4, 2018: Molecular Pharmacology
Mark D Schluchter, Annalisa V Piccorelli
Many longitudinal studies observe time to occurrence of a clinical event such as death, while also collecting serial measurements of one or more biomarkers that are predictive of the event, or are surrogate outcomes of interest. Joint modeling can be used to examine the relationship between the biomarker and the event, and also as a way of adjusting analyses of the biomarker for non-ignorable dropout. In settings such as registry studies, an additional complexity is caused when follow-up of subjects is delayed, referred to as left-truncation of follow-up in the survival analysis setting...
January 1, 2018: Statistical Methods in Medical Research
Imelda Coyne, Helen Malone, Emma Chubb, Alison E While
Parents of young people with cystic fibrosis (YPWCF) play an important role during the transition from paediatric to adult health services. There is limited evidence on parental information needs and the extent to which they are met. An online survey was conducted targeting a finite population of 190 parents of YPWCF in Ireland. Fifty-nine parents responded (31% response rate). Parents reported the need for more general preparation and timing of the transfer, more information regarding the differences between adult and child health services and how their child will self-manage his/her illness in the future...
January 1, 2018: Journal of Child Health Care: for Professionals Working with Children in the Hospital and Community
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