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Recent Publications

  • Opportunities to Improve Utilization of Palliative Care among Adults with Cystic Fibrosis: A Systematic Review.

    Related ArticlesOpportunities to Improve Utilization of Palliative Care among Adults with Cystic Fibrosis: A Systematic Review. J Pain Symptom Manage. 2019 Aug 19;: Authors: Marmor M, Jonas A, Mirza A, Rad E, Wong H, Aslakson RA Abstract CONTEXT: Individuals with Cystic Fibrosis (CF) frequently survive into adulthood and many have multi-faceted symptoms that impair quality of life. OBJECTIVE: We conducted a systematic review to investigate opportunities to improve utilization of palliative care among adults with CF. METHODS: We searched PubMed, Embase, Scopus, Web of Science and CINAHL databases from inception until September 27, 2018, and reviewed references manually. Eligible articles were published in English, involved adults age 18 years and older with CF and contained original data regarding patient outcomes related to presence of advanced care planning (ACP), symptom experience, and preferred and/or received end-of-life care. RESULTS: We screened 652 article abstracts and 32 full text articles; 12 studies met inclusion criteria. All studies were published between 2000 and 2018. Pertinent findings include that while 43% to 65% of adults with CF had contemplated completing ACP, the majority only completed ACP during their terminal hospital admission. Patients also reported high prevalence of untreated symptoms, with adequate symptom control reported in 45% among those with dyspnea, 22% among those with pain and 51% among those with anxiety and/or depression. Prevalence of in-hospital death ranged from 62% to 100%, with a third dying in the intensive care unit (ICU). The majority received antibiotics and preventative treatments during their terminal hospitalization. Finally, treatment from a palliative care specialist was associated with a higher prevalence of patient completion of advanced directives, decreased likelihood of in-ICU death and decreased use of mechanical ventilation at end-of-life. CONCLUSION: Adults with CF often have untreated symptoms and many opportunities exist for palliative care specialists to improve ACP completion and quality of end-of-life care.PMID: 31437475 [PubMed - as supplied by publisher […]

  • Prevention of transmission of Mycobacterium abscessus among patients with cystic fibrosis.

    Related ArticlesPrevention of transmission of Mycobacterium abscessus among patients with cystic fibrosis. Curr Opin Pulm Med. 2019 Aug 20;: Authors: Gross JE, Nick JA, Martiniano SL Abstract PURPOSE OF REVIEW: Pulmonary nontuberculous mycobacterial (NTM) infection is recognized as one of the most challenging infections to treat among cystic fibrosis patients. The source of NTM infection, modes of transmission, and exposure risks are poorly understood. Healthcare-associated transmission of Mycobacterium abscessus among cystic fibrosis patients has been suspected and is a growing concern for cystic fibrosis centers worldwide. This review describes our current understanding of prevention of healthcare-associated transmission of M. abscessus among patients with cystic fibrosis. RECENT FINDINGS: Multiple healthcare-associated outbreaks of M. abscessus among cystic fibrosis patients within cystic fibrosis care centers have been reported. The percentage of patients involved in the reported outbreaks, as well as the perceived impact of patient-to-patient transmission varies dramatically between the reporting centers and population surveys. Several groups have now proposed M. abscessus-specific measures to limit future outbreaks. SUMMARY: Improved NTM surveillance combined with a standardized, systematic approach to epidemiologic investigation of potential episodes of healthcare-associated transmission will help to reveal risk factors for NTM acquisition and inform future evidence-based infection prevention and control measures for patients with cystic fibrosis.PMID: 31436542 [PubMed - as supplied by publisher […]

  • Efficacy and safety of tobramycin inhalation powder in bronchiectasis patients with P. aeruginosa infection: Design of a dose-finding study (iBEST-1).

    Related ArticlesEfficacy and safety of tobramycin inhalation powder in bronchiectasis patients with P. aeruginosa infection: Design of a dose-finding study (iBEST-1). Pulm Pharmacol Ther. 2019 Aug 18;:101834 Authors: Loebinger MR, Polverino E, Blasi F, Elborn SJ, Chalmers JD, Tiddens HA, Goossens H, Tunney M, Zhou W, Angyalosi G, Hill AT, Haworth CS, iBEST-1 Trial Team Abstract In patients with bronchiectasis (BE), infection with Pseudomonas aeruginosa (Pa) results in disease progression, frequent pulmonary exacerbations and lung function decline. However, at present, no inhaled antibiotics have been approved for the treatment of these patients. Tobramycin inhalation powder (TIP), approved for treatment of Pa infection in cystic fibrosis, could be a promising candidate. We aimed to assess effective and well-tolerated doses and regimens of TIP in BE patients with Pa infection. In this phase II, double-blind, placebo-controlled, randomised study, three different daily doses of TIP are administered either as continuous or cyclical regimens. The study protocol comprises 7-28 days of screening, 112 days of double-blind treatment and 56 days of follow-up. The plan was to enrol 180 patients (aged ≥18 years) with BE, documented Pa infection and a history of exacerbations. The primary outcome is change in sputum Pa density from baseline. Key secondary outcomes include number of pulmonary exacerbations, use of antipseudomonal antibiotics, serum and sputum tobramycin concentrations, quality of life and safety. Exploratory endpoints include lung clearance index, sputum inflammatory markers and microbiome analysis. As of October 2018, 107/180 patients were enrolled at 34 sites (six countries) following which recruitment was closed for administrative reasons unrelated to safety findings. Despite a reduced sample size from initially planned enrolment, the unique design may inform the benefit-risk profile of TIP in BE patients with chronic Pa infection. Moreover, several novel and exploratory endpoints (lung clearance index, inflammatory biomarkers, lung microbiome), will contribute to the advancement of research in this area.PMID: 31433997 [PubMed - as supplied by publisher […]

  • Phosphatidylcholine Passes by Paracellular Transport to the Apical Side of the Polarized Biliary Tumor Cell Line Mz-ChA-1.

    Related ArticlesPhosphatidylcholine Passes by Paracellular Transport to the Apical Side of the Polarized Biliary Tumor Cell Line Mz-ChA-1. Int J Mol Sci. 2019 Aug 19;20(16): Authors: Stremmel W, Staffer S, Weiskirchen R Abstract Phosphatidylcholine (PC) translocation into mucus of the intestine was shown to occur via a paracellular transport across the apical/lateral tight junction (TJ) barrier. In case this could also be operative in biliary epithelial cells, this may have implication for the pathogenesis of primary sclerosing cholangitis (PSC). We here evaluated the transport of PC across polarized cholangiocytes. Therefore, the biliary tumor cell line Mz-ChA-1 was grown to confluency. In transwell culture systems the translocation of PC to the apical compartment was analyzed. After 21 days in culture, polarized Mz-ChA-1 cells revealed a predominant apical translocation of choline containing phospholipids including PC with minimal intracellular accumulation. Transport was suppressed by TJ destruction employing chemical inhibitors and pretreatment with siRNA to TJ forming proteins as well as the apical transmembrane mucin 3 as PC acceptor. Apical translocation was dependent on a negative apical electrical potential created by the cystic fibrosis transmembrane conductance regulator (CFTR) and the anion exchange protein 2 (AE2). It was stimulated by apical application of secretory mucins. The results indicated the existence of a paracellular PC passage across apical/lateral TJ of the polarized biliary epithelial tumor cell line Mz-ChA-1. This has implication for the generation of a protective mucus barrier in the biliary tree.PMID: 31430850 [PubMed - in process […]