Twenty-five years of Respirology: Advances in paediatric lung disease.

Respirology. 2020 Jan;25(1):35-37

Authors: Bush A, Fitzgerald D

PMID: 31840887 [PubMed - in process]

High-Efficiency, Selection-free Gene Repair in Airway Stem Cells from Cystic Fibrosis Patients Rescues CFTR Function in Differentiated Epithelia.

Cell Stem Cell. 2019 Dec 11;:

Authors: Vaidyanathan S, Salahudeen AA, Sellers ZM, Bravo DT, Choi SS, Batish A, Le W, Baik R, de la O S, Kaushik MP, Galper N, Lee CM, Teran CA, Yoo JH, Bao G, Chang EH, Patel ZM, Hwang PH, Wine JJ, Milla CE, Desai TJ, Nayak JV, Kuo CJ, Porteus MH

Abstract
Cystic fibrosis (CF) is a monogenic disorder caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Mortality in CF patients is mostly due to respiratory sequelae. Challenges with gene delivery have limited attempts to treat CF using in vivo gene therapy, and low correction levels have hindered ex vivo gene therapy efforts. We have used Cas9 and adeno-associated virus 6 to correct the ΔF508 mutation in readily accessible upper-airway basal stem cells (UABCs) obtained from CF patients. On average, we achieved 30%-50% allelic correction in UABCs and bronchial epithelial cells (HBECs) from 10 CF patients and observed 20%-50% CFTR function relative to non-CF controls in differentiated epithelia. Furthermore, we successfully embedded the corrected UABCs on an FDA-approved porcine small intestinal submucosal membrane (pSIS), and they retained differentiation capacity. This study supports further development of genetically corrected autologous airway stem cell transplant as a treatment for CF.

PMID: 31839569 [PubMed - as supplied by publisher]

Use of CT-SCAN score and volume measures to early identify restrictive allograft syndrome in single lung transplant recipients.

J Heart Lung Transplant. 2019 Nov 25;:

Authors: Philippot Q, Debray MP, Bun R, Frija-Masson J, Bunel V, Morer L, Roux A, Picard C, Jebrak G, Dauriat G, Castier Y, Cazes A, Mal H, Taupin JL, Couffignal C, Brugière O

Abstract
BACKGROUND: Restrictive allograft syndrome (RAS) after lung transplantation (LTx) is associated with the poorer graft survival in patients with chronic lung allograft dysfunction (CLAD). Nevertheless, its diagnostic criteria have not been clearly defined after single-LTx (SLTx). Hence, we studied an SLTx cohort with CLAD to investigate the utility of both computed tomography (CT)-score/volume measures and functional spirometric criteria for the early identification of RAS in this population.
METHODS: We included 51 patients with SLTx (17 RAS, 17 bronchiolitis obliterans syndrome [BOS], and 17 stable condition). The criteria for RAS diagnosis in SLTx included forced vital capacity (FVC) <80% baseline (BL) or forced expiratory volume in 1 second (FEV1) <80% BL with an FEV1/FVC ratiounchanged or >0.7 and persistent CT-scan-lung opacities. We defined 4 time points (T): T-baseline, T-onset (first CT-scan-opacities), T-follow-up, and T-last.
RESULTS: In patients with RAS, the spirometric criteria for RAS at T-onset were reached in only 47% (FVC decline <80% BL [(29%] or FEV1 <80% BL/ratiounchanged or >0.7 [41%]), whereas at the same T-onset date, the graft CT-score increased to 5 (4-6) vs 1 (0-2) at baseline (p < 0.001) (CT - score ≥2 at T-onset in 100% and ΔCT - score ≥2 in 74% of patients with RAS), and the median CT-scan graft volume decreased to 1,722 ml (vs 1,796 ml at T-baseline, p = 0.003) (decreased CT-graft - volume <90% BL in 50% of patients). In contrast, in patients with BOS, CT-score/volume were unchanged at T-onset vs T-baseline (p = 0.8, p = 0.68, respectively).
CONCLUSION: Our results suggest that the use of a simple CT-score and to a lesser extent, CT-volume measures, might allow for the early identification and/or prediction of RAS in SLTx rather than functional criteria.

PMID: 31836403 [PubMed - as supplied by publisher]

Disruption in research publishing - the open access revolution.

J Cyst Fibros. 2019 Nov;18(6):747-749

Authors: Bell SC, Castellani C, Flume PA

PMID: 31836188 [PubMed - in process]

Sinonasal quality-of-life declines in cystic fibrosis patients with pulmonary exacerbations.

Int Forum Allergy Rhinol. 2019 Dec 13;:

Authors: Safi C, DiMango E, Keating C, Zhou Z, Gudis DA

Abstract
BACKGROUND: In cystic fibrosis (CF), the relationship between chronic rhinosinusitis (CRS) and pulmonary disease is poorly understood. The purpose of this study was to evaluate the relationship between scores on the 22-item Sino-Nasal Outcome Test (SNOT-22) and CF Questionnaire-revised for adolescents and adults over 14 (CFQ-R 14+), and pulmonary function tests in 2 cohorts of CF patients: those at their baseline health and those with a pulmonary exacerbation.
METHODS: Patients >18 years old seen in a Cystic Fibrosis Foundation-accredited clinic completed the SNOT-22 and CFQ-R 14+ instruments. Patients presenting for routine care represented the baseline cohort. Patients diagnosed with a pulmonary exacerbation represented the exacerbation cohort. Average SNOT-22 and CFQ-R 14+ scores for both groups were compared using a 2-sample t test, and correlation coefficient was calculated.
RESULTS: One hundred three patients were enrolled over 3 months (30 exacerbations and 73 baseline). Patients' mean age was 32 years (56% female and 44% male). Average SNOT-22 and CFQ-R 14+ scores were significantly worse for exacerbation patients (p = 0.001 and p = 0.0003, respectively). Percent predicted forced expiratory volume in 1 second and forced vital capacity were both higher for baseline patients (p = 0.002 and p = 0.001, respectively). Average SNOT-22 score for all patients was worse than the average score for non-CF, non-CRS patients.
CONCLUSION: CF patients with pulmonary exacerbations have worse SNOT-22 and CFQ-R 14+ scores than CF patients at their baseline health. This finding suggests a temporal relationship between sinonasal and pulmonary quality of life, and that worsening of both is associated with reduced pulmonary function.

PMID: 31834674 [PubMed - as supplied by publisher]

Gastro-oesophageal reflux disease in infancy: a review based on international guidelines.

Med J Aust. 2019 Dec 13;:

Authors: Lopez RN, Lemberg DA

Abstract
Gastro-oesophageal reflux (GOR) in infancy is common, physiological and self-limiting; it is distinguished from gastro-oesophageal reflux disease (GORD) by the presence of organic complications and/or troublesome symptomatology. GORD is more common in infants with certain comorbidities, including history of prematurity, neurological impairment, repaired oesophageal atresia, repaired diaphragmatic hernia, and cystic fibrosis. The diagnosis of GORD in infants relies almost exclusively on clinical history and examination findings; the role of invasive testing and empirical trials of therapy remains unclear. The assessment of infants with vomiting and regurgitation should seek out red flags and not be attributed to GOR or GORD without considered evaluation. Investigations should be considered to exclude other pathology in infants referred with suspected GORD, and occasionally to confirm the diagnosis. Management of GORD should follow a step-wise approach that uses non-pharmacological options where possible and pharmacological interventions only where necessary.

PMID: 31834639 [PubMed - as supplied by publisher]

Impact of Achromobacter xylosoxidans isolation on the respiratory function of adult patients with cystic fibrosis.

ERJ Open Res. 2019 Oct;5(4):

Authors: Tetart M, Wallet F, Kyheng M, Leroy S, Perez T, Le Rouzic O, Wallaert B, Prevotat A

Abstract
Background: The prevalence of Achromobacter xylosoxidans lung isolation in cystic fibrosis (CF) patients has increased, but the impact on lung function is controversial. The aim of this study was to evaluate the long-term effects of A. xylosoxidans isolation on respiratory function of adult patients with CF in the first 3 years after identification of A. xylosoxidans isolation.
Methods: This was a case-control retrospective study performed at a single CF centre in Lille, France. Data for 36 patients with CF who had at least one sputum culture positive for A. xylosoxidans (Ax+) were evaluated and compared with control CF patients uninfected by A. xylosoxidans (Ax-). Respiratory function and exacerbation frequency were evaluated between 1 year prior to and 3 years after A. xylosoxidans isolation.
Results: Compared with the Ax- group, the Ax+ group had a lower forced expiratory volume in 1 s (FEV1) at baseline (median (interquartile range): 55.2% (50.6-59.8%) versus 73.8% (67.2-80.4%); p=0.005), a greater decline in FEV1 (±se) in the first year after A. xylosoxidans identification (-153.6±16.1 mL·year-1 versus -63.8±18.5 mL·year-1; p=0.0003), and more exacerbations in the first 3 years after A. xylosoxidans identification (9 (7-12) versus 7 (5-10); p=0.03). Ax+ patients co-colonised with Pseudomonas aeruginosa (n=27, 75%) had a greater FEV1 decline (p=0.003) and more exacerbations in the year after A. xylosoxidans identification (p=0.037) compared with patients colonised with A. xylosoxidans alone. Patients with chronic A. xylosoxidans isolation (n=23, 64%) had more exacerbations than intermittently colonised patients in the 3 years after A. xylosoxidans identification (p=0.012).
Conclusion: A. xylosoxidans isolation is associated with a decline in respiratory function in patients with CF. Chronic A. xylosoxidans isolation and P. aeruginosa co-isolation may be markers of more severe respiratory disease in Ax+ patients.

PMID: 31832429 [PubMed]

Bronchial epithelium repair by Esculentin-1a-derived antimicrobial peptides: involvement of metalloproteinase-9 and interleukin-8, and evaluation of peptides' immunogenicity.

Sci Rep. 2019 Dec 12;9(1):18988

Authors: Cappiello F, Ranieri D, Carnicelli V, Casciaro B, Chen HT, Ferrera L, Di YP, Mangoni ML

Abstract
The airway epithelium is seriously damaged upon pulmonary Pseudomonas aeruginosa infection, especially in cystic fibrosis (CF) sufferers. Therefore, the discovery of novel anti-infective agents accelerating healing of infected injured tissues is crucial. The antipseudomonal peptides esculentin-1a(1-21)NH2 and its diastereomer Esc(1-21)-1c (Esc peptides) hold promise in this respect. In fact, they stimulate airway epithelial wound repair, but no mechanistic insights are available. Here we demonstrated that this process occurs through promotion of cell migration by an indirect activation of epidermal growth factor receptor mediated by metalloproteinases. Furthermore, we showed an increased expression of metalloproteinase 9, at both gene and protein levels, in peptide-treated bronchial epithelial cells with a functional or mutated form of CF transmembrane conductance regulator. In addition, the two peptides counteracted the inhibitory effect of Pseudomonas lipopolysaccharide (mimicking an infection condition) on the wound healing activity of the airway epithelium, and they enhanced the production of interleukin-8 from both types of cells. Finally, no immunogenicity was discovered for Esc peptides, suggesting their potential safety for clinical usage. Besides representing a step forward in understanding the molecular mechanism underlying the peptide-induced wound healing activity, these studies have contributed to highlight Esc peptides as valuable therapeutics with multiple functions.

PMID: 31831857 [PubMed - in process]

A novel application of Gini coefficient for the quantitative measurement of bacterial aggregation.

Sci Rep. 2019 Dec 12;9(1):19002

Authors: Cai YM, Chatelet DS, Howlin RP, Wang ZZ, Webb JS

Abstract
Non-surface attached bacterial aggregates are frequently found in clinical settings associated with chronic infections. Current methods quantifying the extent to which a suspended bacterial population is aggregated mainly rely on: (1) cell size distribution curves that are difficult to be compared numerically among large-scale samples; (2) the average size/proportion of aggregates in a population that do not specify the aggregation patterns. Here we introduce a novel application of Gini coefficient, herein named Aggregation Coefficient (AC), to quantify the aggregation levels of cystic fibrosis Pseudomonas aeruginosa (CF-PA) isolates in vitro using 3D micrographs, Fiji and MATLAB. Different aggregation patterns of five strains were compared statistically using the numerical AC indexes, which correlated well with the size distribution curves plotted by different biovolumes of aggregates. To test the sensitivity of AC, aggregates of the same strains were treated with nitric oxide (NO), a dispersal agent that reduces the biomass of surface attached biofilms. Strains unresponsive to NO were reflected by comparable AC indexes, while those undergoing dispersal showed a significant reduction in AC index, mirroring the changes in average aggregate sizes and proportions. Therefore, AC provides simpler and more descriptive numerical outputs for measuring different aggregation patterns compared to current approaches.

PMID: 31831832 [PubMed - in process]

The efficacy of lyticase and β-glucosidase enzymes on biofilm degradation of Pseudomonas aeruginosa strains with different gene profiles.

BMC Microbiol. 2019 Dec 12;19(1):291

Authors: Banar M, Emaneini M, Beigverdi R, Fanaei Pirlar R, Node Farahani N, van Leeuwen WB, Jabalameli F

Abstract
BACKGROUND: Pseudomonas aeruginosa is a nosocomial pathogen that causes severe infections in immunocompromised patients. Biofilm plays a significant role in the resistance of this bacterium and complicates the treatment of its infections. In this study, the effect of lyticase and β-glucosidase enzymes on the degradation of biofilms of P. aeruginosa strains isolated from cystic fibrosis and burn wound infections were assessed. Moreover, the decrease of ceftazidime minimum biofilm eliminating concentrations (MBEC) after enzymatic treatment was evaluated.
RESULTS: This study demonstrated the effectiveness of both enzymes in degrading the biofilms of P. aeruginosa. In contrast to the lyticase enzyme, β-glucosidase reduced the ceftazidime MBECs significantly (P < 0.05). Both enzymes had no cytotoxic effect on the A-549 human lung carcinoma epithelial cell lines and A-431 human epidermoid carcinoma cell lines.
CONCLUSION: Considering the characteristics of the β-glucosidase enzyme, which includes the notable degradation of P. aeruginosa biofilms and a significant decrease in the ceftazidime MBECs and non-toxicity for eukaryotic cells, this enzyme can be a promising therapeutic candidate for degradation of biofilms in burn wound patients, but further studies are needed.

PMID: 31830915 [PubMed - in process]

Universal strategy for preimplantation genetic testing for cystic fibrosis based on next generation sequencing.

J Assist Reprod Genet. 2019 Dec 11;:

Authors: Chamayou S, Sicali M, Lombardo D, Alecci C, Ragolia C, Maglia E, Liprino A, Cardea C, Storaci G, Romano S, Guglielmino A

Abstract
PURPOSE: We developed and applied a universal strategy for preimplantation genetic testing for all cystic fibrosis gene mutations (PGT-CF) based on next-generation sequencing (NGS).
METHODS: A molecular protocol was designed to diagnose all CF mutations at preimplantation stage. The detection of CF mutations was performed by direct gene sequencing and linkage strategy testing 38 specific SNPs located upstream and inside the gene for PGT-CF. Seventeen couples at risk of CF transmission decided to undergo PGT-CF. Trophectoderm cell biopsies were performed on day 5-6 blastocysts. PGT for aneuploidy (PGT-A) was performed from the same samples. Tested embryos were transferred on further natural cycles.
RESULTS: PGT was performed on 109 embryos. Fifteen CF mutations were tested. PGT-CF and PGT-A were conclusive for respectively 92.7% and 95.3% of the samples. A mean of 24.1 SNPs was informative per couple. After a single embryo transfer on natural cycle, 81.3% of the transferred tested embryos were implanted.
CONCLUSIONS: The present protocol based on the entire CFTR gene together with informative SNPs outside and inside the gene can be applied to diagnose all CF mutations at preimplantation stage.

PMID: 31828483 [PubMed - as supplied by publisher]

Editorial: Secondary Respiratory Infections in the Context of Acute and Chronic Pulmonary Diseases.

Front Immunol. 2019;10:2764

Authors: Trottein F, Alcorn JF

PMID: 31827474 [PubMed - in process]

Pseudomonas Prolyl Hydroxylase (PPHD), a putative bacterial oxygen sensor suppresses antibiotic resistance and pathogenicity in Pseudomonas aeruginosa.

J Biol Chem. 2019 Dec 11;:

Authors: Schaible B, Crifo B, Schaffer K, Taylor CT

Abstract
Pseudomonas aeruginosa is an extracellular opportunistic pathogen commonly associated with infectious complications in susceptible patients such as those with underlying diseases including HIV/AIDS and cystic fibrosis. Antibiotic resistance in multiple strains of P. aeruginosa is a rapidly developing clinical problem. We have previously demonstrated that the oxygen levels at the site of P. aeruginosa infection can strongly influence virulence and antibiotic resistance in the pathogen, although the oxygen sensing and signaling mechanisms underpinning this response have remained unknown. In this study, we have investigated the potential role of the putative oxygen sensor PPHD in the control of virulence and antibiotic resistance in P. aeruginosa. We found that a P. aeruginosa strain lacking PPHD (PAO310) demonstrates increased virulence associated with increased bacterial motility. Furthermore, PPHD-deficient P. aeruginosa displayed enhanced antibiotic resistance against tetracycline and related antibiotics through increased expression of xenobiotic transporters. The effect of PPHD knockout on antibiotic resistance was phenocopied in bacteria exposed to atmospheric hypoxia. In summary, PPHD is a putative bacterial oxygen sensor which may link microenvironmental oxygen levels to virulence and antibiotic resistance in P. aeruginosa.

PMID: 31826919 [PubMed - as supplied by publisher]

New Drug Hailed as Major Breakthrough in Cystic Fibrosis.

Am J Med Genet A. 2020 Jan;182(1):8-9

Authors:

PMID: 31825178 [PubMed - in process]

Update on the diagnosis and management of exocrine pancreatic insufficiency.

F1000Res. 2019;8:

Authors: Perbtani Y, Forsmark CE

Abstract
Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine, resulting in malabsorption. Clinical manifestations of EPI are often nonspecific and can lead to lack of timely recognition and diagnosis. Central to this clinical dilemma is the lack of highly accurate or specific testing which leads to misdiagnosis and suboptimal treatment. Identification of high-risk patients is key in the diagnosis of EPI and this includes patients with pancreatic parenchyma disorders such as chronic pancreatitis, pancreatic malignancy, cystic fibrosis, and those undergoing pancreatic resection for benign and malignant disease. Less recognized are the number of additional conditions which may also have EPI as a consequence. Owing to an increase in morbidity and impaired quality of life associated with this condition, goals of treatment have been aimed at repleting exocrine enzyme deficiency by oral pancreatic enzyme replacement therapy (PERT). The basis of PERT is to provide activated digestive enzymes to the small bowel during the prandial period, mainly, leading to sufficient absorption of fat and fat-soluble vitamins. The benefits of PERT have been shown to go beyond the improvement in signs and symptoms associated with EPI and include decreasing prevalence of osteopathy and improving survival outcomes in subsets of patients with this condition. However, despite the overall benefits in treatment, the diagnosis and management of EPI are suboptimal. Current literature suggests patients at high risk of developing EPI are not tested and those who are diagnosed are not treated with adequate dosages. In this review, we highlight patients who are at high risk for the development of EPI, analyze consequences and treatment of this disorder, review rationale for enzyme replacement therapy, and examine current evidence for treatment optimization.

PMID: 31824646 [PubMed - in process]

A Continuous-Flow Model for in vitro Cultivation of Mixed Microbial Populations Associated With Cystic Fibrosis Airway Infections.

Front Microbiol. 2019;10:2713

Authors: O'Brien TJ, Welch M

Abstract
The airways of people with cystic fibrosis (CF) provide a nutrient-rich environment which favours colonisation by a variety of bacteria and fungi. Although the dominant pathogen associated with CF airway infections is Pseudomonas aeruginosa, it is becoming increasingly clear that inter-species interactions between P. aeruginosa and other colonists in the airways may have a large impact on microbial physiology and virulence. However, there are currently no suitable experimental models that permit long-term co-culture of P. aeruginosa with other CF-associated pathogens. Here, we redress this problem by describing a "3R's-compliant" continuous-flow in vitro culture model which enables long-term co-culture of three representative CF-associated microbes: P. aeruginosa, Staphylococcus aureus and Candida albicans. Although these species rapidly out-compete one another when grown together or in pairs in batch culture, we show that in a continuously-fed setup, they can be maintained in a very stable, steady-state community. We use our system to show that even numerically (0.1%) minor species can have a major impact on intercellular signalling by P. aeruginosa. Importantly, we also show that co-culturing does not appear to influence species mutation rates, further reinforcing the notion that the system favours stability rather than divergence. The model is experimentally tractable and offers an inexpensive yet robust means of investigating inter-species interactions between CF pathogens.

PMID: 31824471 [PubMed]

Congenital bilateral absence of the vas deferens (CBAVD): do genetic disorders modify assisted reproductive technologies outcomes?

Arch Esp Urol. 2019 Dec;72(10):1038-1042

Authors: Gallego Á, Rogel R, Pérez-Ardavín J, Lorenzo L, Lujan S, Oltra S, Molina I, Broseta E

Abstract
OBJECTIVES: To evaluate the impact of common Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene mutations, 5T polymorphism and presence of severe Cystic Fibrosis (CF) on fertility outcomes with Assisted Reproductive Techniques (ART) in patients presenting Congenital Bilateral Absence of Vas Deferens (CBAVD).
METHODS: A comparative observational cohort study was performed from 2002 to 2018 with 51 patients with diagnosis of CBAVD. Presence of CFTR mutations and 5T, CF, pregnancy and newborn rates were analyzed.
RESULTS: 80.4% percent had some mutation of CFTR gene being ΔF508 the most common (51%). The most frequently described genotype was the 7T/9T (31.4%) with the presence of 5T polymorphism in up to 25.5% of cases. Global newborn rates were 34% in the group using partner spermatozoa. When comparing 5T presence, we observed a decrease in newborn rates when carrying this mutation, without obtaining statistical significance (newborn rate: 5T/non-5T: 7.1/28%, p 0.45). No differences were found when comparing presence of severe CF, common CFTR gene mutations and ICSI-related parameters.
CONCLUSION: The analysis of the presence of 5T polymporphism in CBAVD patients may add information when predicting the outcome of assisted reproductive techniques.

PMID: 31823853 [PubMed - in process]

Developing Future Clinical Pharmacy Leaders in the Interprofessional Care of Children with Special Health Care Needs and Medical Complexity (CSHCN-CMC) in a Pediatric Pulmonary Center.

Children (Basel). 2019 Dec 09;6(12):

Authors: Hobart CB, Daines CL, Phan H

Abstract
The health care needs of children with special health care needs and medical complexity (CSHCN-CMC) are multifaceted and often require the expertise of various disciplines. The medication-related needs of this population can be further complicated with off-label medication use, polypharmacy, and vulnerability to medication errors. Although clinical pharmacists are increasingly becoming a common part of inpatient, pediatric interprofessional patient care teams, their presence remains lacking in the outpatient or ambulatory care realm. Pediatric clinical pharmacists in the ambulatory care setting have the potential to help optimize medication use and safety through collaborative efforts as part of the interprofessional team. Since the late 1960s, Pediatric Pulmonary Centers (PPCs) provide training programs designed to develop interprofessional leaders who will improve the health status of CSHCN-CMC, specifically those with chronic respiratory and sleep-related conditions. The addition of pharmacists not only provides a more comprehensive care model for CSHCN-CMC, it creates an avenue to encourage the career paths of pediatric pharmacists in the ambulatory care setting. Here, we describe the addition of clinical pharmacy as part of an interprofessional patient care team and the development and implementation of a maternal child health (MCH) pharmacy discipline training model designed to mentor future pharmacist leaders in the care of CSHCN-CMC.

PMID: 31818043 [PubMed]

Novel Therapeutic Strategies Applied to Pseudomonas aeruginosa Infections in Cystic Fibrosis.

Materials (Basel). 2019 Dec 07;12(24):

Authors: Chirgwin ME, Dedloff MR, Holban AM, Gestal MC

Abstract
Cystic fibrosis (CF) is one of the most prevalent genetic diseases and a total of 1700 different genetic mutations can cause this condition. Patients that suffer this disease have a thickening of the mucus, creating an environment that promotes bacterial infections. Pseudomonas aeruginosa is a ubiquitous bacterium, which is frequently found in the lungs of CF patients. P. aeruginosa is known for its high level of antibiotic resistance as well as its high rate of mutation that allows it to rapidly evolve and adapt to a multitude of conditions. When a CF lung is infected with P. aeruginosa, the decay of the patient is accelerated, but there is little that can be done apart from controlling the infection with antibiotics. Novel strategies to control P. aeruginosa infection are imperative, and nanotechnology provides novel approaches to drug delivery that are more efficient than classic antibiotic treatments. These drug delivery systems are offering new prospects, especially for these patients with special mucus conditions and bacterial characteristics that limit antibiotic use.

PMID: 31817881 [PubMed]

Hemoptysis from the Perspective of People with Cystic Fibrosis.

Clin Respir J. 2019 Dec 10;:

Authors: Roman C, Loughlin H, Aliaj E, Fay R, Tran Q, Borowitz D

Abstract
INTRODUCTION: People with cystic fibrosis (CF) are living longer, thus complications associated with age, such as hemoptysis, are increasing. The Institute of Medicine has emphasized the importance of patient-centeredness. Although guidelines about hemoptysis in people with CF are available, these focus on management of the complication and not the patient perspective.
OBJECTIVE: We sought to understand hemoptysis from the point of view of those who have experienced it.
METHODS: We fielded an 11-question survey to adults with CF and asked those who had hemoptysis to respond. Four questions had open-ended options: 1) the person's first experience with hemoptysis, 2) how that experience affected the way they approach their CF, 3) how they deal with hemoptysis when it occurs outside the home, and 4) a free text box for general comments.
RESULTS: Thirty-one of 132 adults with CF who were sent a survey completed it (23% response rate); 63% F), indicated that they had experienced hemoptysis and described their triggers. In response to open questioning, 77% of respondents found their first experience with hemoptysis to be "scary", "frightening", "worrying" or "jarring". Half of respondents reported quality of life being negatively affected by worsening stress or anxiety, fear of bleeding in public, or other life impacts.
CONCLUSIONS: Focusing on how to cope with future episodes of hemoptysis and the associated anxiety can be helpful to patients. Proactive communication and sensitivity to patient experience may deepen physician-patient rapport, increase self-efficacy to cope with future episodes and lead to more comprehensive care of hemoptysis.

PMID: 31821725 [PubMed - as supplied by publisher]