Cystic Fibrosis Lung Transplant Recipients Have Suppressed Airway Interferon Responses during Pseudomonas Infection.

Cell Rep Med. 2020 Jul 21;1(4):

Authors: Dugger DT, Fung M, Zlock L, Caldera S, Sharp L, Hays SR, Singer JP, Leard LE, Golden JA, Shah RJ, Kukreja J, Gordon E, Finkbeiner W, Kleinhenz ME, Langelier C, Greenland JR

Abstract
Lung transplantation can be lifesaving in end-stage cystic fibrosis (CF), but long-term survival is limited by chronic lung allograft dysfunction (CLAD). Persistent upper airway Pseudomonas aeruginosa (PsA) colonization can seed the allograft. While de novo PsA infection is associated with CLAD in non-CF recipients, this association is less clear for CF recipients experiencing PsA recolonization. Here, we evaluate host and pathogen contributions to this phenomenon. In the context of PsA infection, brushings from the airways of CF recipients demonstrate type 1 interferon gene suppression. Airway epithelial cell (AEC) cultures demonstrate similar findings in the absence of pathogens or immune cells, contrasting with the pre-transplant CF AEC phenotype. Type 1 interferon promoters are relatively hypermethylated in CF AECs. CF subjects in this cohort have more mucoid PsA, while non-CF PsA subjects have decreased microbiome α diversity. Peri-transplant protocols may benefit from consideration of this host and microbiome equilibrium.

PMID: 32754722 [PubMed]

Increased intracellular Cl- concentration improves airway epithelial migration by activating the RhoA/ROCK Pathway.

Theranostics. 2020;10(19):8528-8540

Authors: Huang W, Tan M, Wang Y, Liu L, Pan Y, Li J, Ouyang M, Long C, Qu X, Liu H, Liu C, Wang J, Deng L, Xiang Y, Qin X

Abstract
In the airway, Cl- is the most abundant anion and is critically involved in transepithelial transport. The correlation of the abnormal expression and activation of chloride channels (CLCs), such as cystic fibrosis transmembrane conductance regulators (CFTRs), anoctamin-1, and CLC-2, with cell migration capability suggests a relationship between defective Cl- transport and epithelial wound repair. However, whether a correlation exists between intracellular Cl- and airway wound repair capability has not been explored thus far, and the underlying mechanisms involved in this relationship are not fully defined. Methods: In this work, the alteration of intracellular chloride concentration ([Cl-]i) was measured by using a chloride-sensitive fluorescent probe (N-[ethoxycarbonylmethyl]-6-methoxyquinolium bromide). Results: We found that clamping with high [Cl-]i and 1 h of treatment with the CLC inhibitor CFTR blocker CFTRinh-172 and chloride intracellular channel inhibitor IAA94 increased intracellular Cl- concentration ([Cl-]i) in airway epithelial cells. This effect improved epithelial cell migration. In addition, increased [Cl-]i in cells promoted F-actin reorganization, decreased cell stiffness, and improved RhoA activation and LIMK1/2 phosphorylation. Treatment with the ROCK inhibitor of Y-27632 and ROCK1 siRNA significantly attenuated the effects of increased [Cl-]i on LIMK1/2 activation and cell migration. In addition, intracellular Ca2+ concentration was unaffected by [Cl-]i clamping buffers and CFTRinh-172 and IAA94. Conclusion: Taken together, these results suggested that Cl- accumulation in airway epithelial cells could activate the RhoA/ROCK/LIMK cascade to induce F-actin reorganization, down-regulate cell stiffness, and improve epithelial migration.

PMID: 32754261 [PubMed - in process]

Corrigendum: Pseudomonas aeruginosa Modulates the Antiviral Response of Bronchial Epithelial Cells.

Front Immunol. 2020;11:1453

Authors: Sörensen M, Kantorek J, Byrnes L, Boutin S, Mall MA, Lasitschka F, Zabeck H, Nguyen D, Dalpke AH

Abstract
[This corrects the article DOI: 10.3389/fimmu.2020.00096.].

PMID: 32754158 [PubMed - in process]

A putative enoyl-CoA hydratase contributes to biofilm formation and the antibiotic tolerance of Achromobacter xylosoxidans.

NPJ Biofilms Microbiomes. 2019 Aug 06;5(1):20

Authors: Cameron LC, Bonis B, Phan CQ, Kent LA, Lee AK, Hunter RC

Abstract
Achromobacter xylosoxidans has attracted increasing attention as an emerging pathogen in patients with cystic fibrosis. Intrinsic resistance to several classes of antimicrobials and the ability to form robust biofilms in vivo contribute to the clinical manifestations of persistent A. xylosoxidans infection. Still, much of A. xylosoxidans biofilm formation remains uncharacterized due to the scarcity of existing genetic tools. Here we demonstrate a promising genetic system for use in A. xylosoxidans; generating a transposon mutant library which was then used to identify genes involved in biofilm development in vitro. We further described the effects of one of the genes found in the mutagenesis screen, encoding a putative enoyl-CoA hydratase, on biofilm structure and tolerance to antimicrobials. Through additional analysis, we find that a fatty acid signaling compound is essential to A. xylosoxidans biofilm ultrastructure and maintenance. This work describes methods for the genetic manipulation of A. xylosoxidans and demonstrated their use to improve our understanding of A. xylosoxidans pathophysiology.

PMID: 32753610 [PubMed - in process]

Proteomics and Metabolomics for Cystic Fibrosis Research.

Int J Mol Sci. 2020 Jul 30;21(15):

Authors: Liessi N, Pedemonte N, Armirotti A, Braccia C

Abstract
The aim of this review article is to introduce the reader to the state-of-the-art of the contribution that proteomics and metabolomics sciences are currently providing for cystic fibrosis (CF) research: from the understanding of cystic fibrosis transmembrane conductance regulator (CFTR) biology to biomarker discovery for CF diagnosis. Our work particularly focuses on CFTR post-translational modifications and their role in cellular trafficking as well as on studies that allowed the identification of CFTR molecular interactors. We also show how metabolomics is currently helping biomarker discovery in CF. The most recent advances in these fields are covered by this review, as well as some considerations on possible future scenarios for new applications.

PMID: 32751630 [PubMed - in process]

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Cystic Fibrosis-Related Diabetes and Subclinical Hypothyroidism in Pregnancy.

Cureus. 2020 Jun 28;12(6):e8895

Authors: Kakoulidis I, Ilias I, Linardi A, Venaki E, Koukkou E

Abstract
Pregnancy in women with cystic fibrosis-related diabetes (CFRD) is rare and requires intensive monitoring and individualized treatment due to the pathophysiologic parameters of the disease in relation to insulin therapy and special nutritional needs. We present the case of a 33-year-old primigravida woman with CFRD (ΔF508 homozygote, with mild pulmonary involvement) on insulin therapy and treatment for exocrine pancreatic insufficiency, who developed subclinical hypothyroidism during gestation. Due to the complexity of the disease, major clinical challenges were glycemic variance, hypoglycemic episodes, and difficulty in weight gaining. In addition, the presence of malabsorption in the intestinal mucosa was an important aspect of difficulty in the treatment of subclinical hypothyroidism. Thus, the flexible approach in the timing of basal insulin administration, combined with the individualized medical nutrition therapy, and along with the progressive increase in levothyroxine dosage, all were proven to be key components in the effective management of our patient.

PMID: 32742862 [PubMed]

Concurrent and Longitudinal Associations among Parenting Style, Responsibility, and Adherence in Youth with Cystic Fibrosis.

Child Health Care. 2020;49(2):153-167

Authors: Murphy C, Miller VA

Abstract
In an effort to identify pathways for improvements in clinical monitoring and intervention, the current study investigated the role that parenting style plays in treatment adherence and responsibility for youth with CF. Participants (n = 50) completed questionnaires related to treatment adherence, treatment responsibility, and parenting style at baseline and at a two year follow up visit. Aspects of parenting style (e.g., warmth, autonomy support) were related to youth adherence and responsibility in cross-sectional and prospective analyses. These data suggest that aspects of parenting may be important targets of interventions to promote treatment adherence in youth with CF.

PMID: 32742054 [PubMed]

The Effects of Nutritional Status and Intervention on Pulmonary Functions in Pediatric Cystic Fibrosis Patients.

Pediatr Int. 2020 Aug 03;:

Authors: Kilinc AA, Beser OF, Ugur EP, Cokugras FC, Cokugras H

Abstract
OBJECTIVE: Cystic fibrosis (CF) causes malabsorption of nutrients that exacerbate pulmonary problems. Nutritional interventions can improve pulmonary functions. We aimed to evaluate the effects of nutritional intervention in CF patients with malnutrition , and to determine if there is a correlation between nutritional status and pulmonary functions.
MATERIALS AND METHODS: The study included 143 CF patients (67 females) with a mean 2 years follow-up time. Patients' sociodemographic data, presenting symptoms and history were recorded. Height-for-age (HFA); weight-for-age (WFA); weight-for-length/height (WFL/H); body mass index (BMI) were calculated in all patients. Patients were grouped as well nourished, mild malnutrition, moderate malnutrition, and severe malnutrition. These 4 groups were compared in terms of pulmonary function test results, lung infections, and the hospitalization rate.
RESULTS: Among the patients with a WFL/H or BMI z-scores that decreased, the frequency of lung infection was 74.1% and the hospitalization rate was 40.7%, versus 34% and 12.3%, respectively, among the patients with a WFL/H or BMI z-scores than increased; the difference was significant (P = 0.02 and P = 0.01, respectively). The difference in bacterial lung infections differed significantly between the 4 nutritional status groups (P = 0.002). Patients in the well-nourished group had significantly higher pulmonary function test scores than the other groups. FEV1 differed significantly between the patients with and without an increase in the WFL/H or BMI z-scores (P = 0.001).
CONCLUSION: The appropriate nutritional intervention to pediatric CF patients with malnutrition, decrease the frequency of lung infections, and maintain or improve respiratory function.

PMID: 32745357 [PubMed - as supplied by publisher]

Successful disinfection of trumpet mouthpieces using domestic steam disinfection.

Lett Appl Microbiol. 2020 Aug 03;:

Authors: Moore JE, Millar BC

Abstract
There have been numerous reports in the literature describing the diversity of microbial flora isolated from woodwind and brass instruments, with potential infection risks for players, especially when such instuments are shared. Steam disinfection has become established as a trusted method of decontamination, however, there have been no reports on the employment of this technology to disinfect parts of musical instruments, hence it was the aim of this study to examine the fate of bacterial and yeast pathogens on artificially contaminated trumpet mouthpieces and to evaluate whether such disinfection is an effective method of disinfection for such instrument parts. Trumpet mouthpieces were artificially contaminated with 18 microbial strains (17 bacteria from four genera [Enterococcus, Escherchia, Staphylococcus & Streptococcus] and one yeast [Candida]) each at an inoculum density of approximately 1.5 x 107 colony forming units and subjected to a disinfection cycle. The experiment was repeated including 50% [v/v] sterile sputum as soil. No bacteria or yeast organisms were recovered post disinfection, including following recovery and non-selective cultural enrichment techniques.

PMID: 32745274 [PubMed - as supplied by publisher]

The Uncertain Role of Corticosteroids in the Treatment of COVID-19.

JAMA Intern Med. 2020 Aug 03;:

Authors: Liou TG, Adler FR, Hatton ND

PMID: 32744622 [PubMed - as supplied by publisher]

Visualization of Pseudomonas aeruginosa within the Sputum of Cystic Fibrosis Patients.

J Vis Exp. 2020 Jul 16;(161):

Authors: Jackson L, DePas W, Morris AJ, Guttman K, Yau YCW, Waters V

Abstract
Early detection and eradication of Pseudomonas aeruginosa within the lungs of cystic fibrosis patients can reduce the chance of developing chronic infection. The development of chronic P. aeruginosa infections is associated with a decline in lung function and increased morbidity. Therefore, there is a great interest in elucidating the reasons for the failure to eradicate P. aeruginosa with antibiotic therapy which occurs in approximately 10-40% of pediatric patients. One of many factors that can affect host clearance of P. aeruginosa and antibiotic susceptibility is variations in spatial organization (such as aggregation or biofilm formation) and polysaccharide production. Therefore, we were interested in visualizing the in situ characteristics of P. aeruginosa within the sputum of CF patients. A tissue clearing technique was applied to sputum samples after embedding the samples into a hydrogel matrix to retain the 3D structures relative to host cells. After tissue clearing, fluorescent labels and dyes were added to allow visualization. Fluorescence in situ hybridization was performed for the visualization of bacterial cells, binding of fluorescently labeled anti-Psl-antibodies for the visualization of the exopolysaccharide and DAPI staining to stain host cells to obtain structural insight. These methods allowed for the high-resolution imaging of P. aeruginosa within the sputum of CF patients via confocal laser scanning microscopy.

PMID: 32744517 [PubMed - as supplied by publisher]

EDF1 coordinates cellular responses to ribosome collisions.

Elife. 2020 Aug 03;9:

Authors: Sinha NK, Ordureau A, Best K, Saba JA, Zinshteyn B, Sundaramoorthy E, Fulzele A, Garshott DM, Denk T, Thoms M, Paulo JA, Harper W, Bennett EJ, Beckmann R, Green R

Abstract
Translation of aberrant mRNAs induces ribosomal collisions, thereby triggering pathways for mRNA and nascent peptide degradation and ribosomal rescue. Here we use sucrose gradient fractionation combined with quantitative proteomics to systematically identify proteins associated with collided ribosomes. This approach identified Endothelial differentiation-related factor 1 (EDF1) as a novel protein recruited to collided ribosomes during translational distress. Cryo-electron microscopic analyses of EDF1 and its yeast homolog Mbf1 revealed a conserved 40S ribosomal subunit binding site at the mRNA entry channel near the collision interface. EDF1 recruits the translational repressors GIGYF2 and EIF4E2 to collided ribosomes to initiate a negative-feedback loop that prevents new ribosomes from translating defective mRNAs. Further, EDF1 regulates an immediate-early transcriptional response to ribosomal collisions. Our results uncover mechanisms through which EDF1 coordinates multiple responses of the ribosome-mediated quality control pathway and provide novel insights into the intersection of ribosome-mediated quality control with global transcriptional regulation.

PMID: 32744497 [PubMed - as supplied by publisher]

Cystic fibrosis - Ten promising therapeutic approaches in the current era of care.

Expert Opin Investig Drugs. 2020 Aug 03;:

Authors: Somayaji R, Nichols D, Bell SC

Abstract
INTRODUCTION: Cystic fibrosis (CF) is a genetic disease affecting multiple organ systems. Research and innovations in novel therapeutic agents and health care delivery have resulted in dramatic improvements in quality of life and survival for people with CF. Despite this, significant disease burden persists for many and this is compounded by disparities in treatment access and care which globally necessitates further work to improve outcomes. Because of the advent of a numerous therapies which includes gene-targeted modulators in parallel with specialized care delivery models, innovative efforts continue.
AREAS COVERED: In this review, we discuss the available data on investigational agents in clinical development and currently available treatments for CF. We also evaluate approaches to care delivery, consider treatment gaps and propose future directions for advancement.
EXPERT OPINION: Since the discovery of the CF gene, CFTR modulators have provided a hallmark of success, eventhough it was thought not previously possible. This has led to reinvigorated efforts and innovations in treatment approaches and care delivery. Numerous challenges remain because of genetic and phenotypic heterogeneity, access issues, and therapeutic costs, but the collaborative approach between stakeholders for continued innovation fuels optimism.

PMID: 32744089 [PubMed - as supplied by publisher]

Biosimilar infliximab in paediatric inflammatory bowel disease: Efficacy, immunogenicity and safety.

J Clin Pharm Ther. 2020 Aug 02;:

Authors: Dipasquale V, Romano C

Abstract
WHAT IS KNOWN AND OBJECTIVE: Based on extrapolation, biosimilar infliximab (IFX) was approved to treat inflammatory bowel disease (IBD). The first studies in adults have shown similar efficacy and safety in comparison with reference drug. The aim of this review was to collect and evaluate all the literature data regarding the use of biosimilar IFX in paediatric IBD.
METHODS: This article reviewed efficacy, immunogenicity and safety profile of biosimilar IFX in IBD paediatric patients through a comprehensive search of the published literature.
RESULTS AND DISCUSSION: Eight papers were extracted and critically reviewed. Four paediatric studies (prospective, n = 3; retrospective, n = 1) assessed the induction efficacy of the biosimilar IFX. Clinical response and remission rates reported were 86%-90% and 67%-68%, respectively. No significant difference in clinical response and remission rates between the reference and biosimilar IFX groups was found at follow-up (range: 3-13 months). Similar findings were shown in the prospective studies (n = 4) conducted on patients elected to switch from reference IFX to its biosimilar. The most frequently reported adverse events (AEs) of biosimilar IFX were mild upper respiratory tract infections. Taking into account of all AEs coming from published data, biosimilar IFX seems to be as safe as its originator. Immunogenicity has not been significantly impacted by the switch from the reference drug.
WHAT IS NEW AND CONCLUSION: To date, treatment with (or switch to) biosimilar IFX in paediatric patients with IBD have been successful, without affecting efficacy, immunogenicity or safety. However, further studies are warranted, including clinical trials and pharmacovigilance studies.

PMID: 32743840 [PubMed - as supplied by publisher]

Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants.

J Cyst Fibros. 2020 Jul 30;:

Authors: Laselva O, Bartlett C, Popa A, Ouyang H, Gunawardena TNA, Gonska T, Moraes TJ, Bear CE

Abstract
BACKGROUND: F508del is prototypical of Class 2 CFTR mutations associated with protein misprocessing and reduced function. Corrector compounds like lumacaftor partially rescue the processing defect of F508del-CFTR whereas potentiators like ivacaftor, enhance its channel activity once trafficked to the cell surface. We asked if emerging modulators developed for F508del-CFTR can rescue Class 2 mutations previously shown to be poorly responsive to lumacaftor and ivacaftor.
METHODS: Rescue of mutant CFTRs by the correctors: AC1, AC2-1 or AC2-2 and the potentiator, AP2, was studied in HEK-293 cells and in primary human nasal epithelial (HNE) cultures, using a membrane potential assay and Ussing chamber, respectively.
RESULTS: In HEK-293 cells, we found that a particular combination of corrector molecules (AC1 plus AC2-1) and a potentiator (AP2) was effective in rescuing both the misprocessing and reduced function of M1101K and G85E respectively. These findings were recapitulated in patient-derived nasal cultures, although another corrector combination, AC1 plus AC2-2 also improved misprocessing in these primary tissues. Interestingly, while this corrector combination only led to a modest increase in the abundance of mature N1303K-CFTR it did enable its functional expression in the presence of the potentiator, AP2, in part, because the nominal corrector, AC2-2 also exhibits potentiator activity.
CONCLUSIONS: Strategic combinations of novel modulators can potentially rescue Class 2 mutants thought to be relatively unresponsive to lumacaftor and ivacaftor.

PMID: 32741662 [PubMed - as supplied by publisher]

Intraoperative Implications of the Recipients' Disease for Double-Lung Transplantation.

J Cardiothorac Vasc Anesth. 2020 Jul 17;:

Authors: Fessler J, Davignon M, Sage E, Roux A, Cerf C, Feliot E, Gayat E, Parquin F, Fischler M, Guen ML

Abstract
OBJECTIVES: To compare intraoperative patterns among patients based on their primary pulmonary disease (cystic fibrosis [CF], chronic obstructive pulmonary disease [COPD]/emphysema [CE], and pulmonary fibrosis [PF]) during double- lung transplantation. The following 3 major outcomes were reported: blood transfusion, extracorporeal membrane oxygenation (ECMO) management, and the possibility of immediate extubation at the end of surgery.
DESIGN: Retrospective analysis of a prospectively maintained database, including donor and recipient characteristics and intraoperative variables.
SETTING: Foch Hospital, Suresnes, France (academic center performing 60-80 lung transplantations per year).
PARTICIPANTS: Patients who underwent double- lung transplantation from 2012-2019. Patients with retransplantation, multiorgan transplantation, or surgery performed with cardiopulmonary bypass were excluded.
INTERVENTIONS: None.
MEASUREMENTS AND MAIN RESULTS: Two hundred forty-six patients had CF, 117 had CE, and 66 had PF. No patient had primary pulmonary arterial hypertension. Blood transfusion was higher in the CF group than in the other 2 groups (red blood cells [p < 0.001], fresh frozen plasma [p = 0.004]). The CF and CE groups were characterized by a lower intraoperative requirement of ECMO (p = 0.002), and the PF group more frequently required postoperative ECMO (p < 0.001). CF and CE patients were more frequently extubated in the operating room than were PF patients (37.4%, 50.4%, and 13.6%, respectively; p < 0.001).
CONCLUSIONS: Intraoperative outcomes differed depending on the initial pathology. Such differences should be taken into account in specific clinical studies and in intraoperative management protocols.

PMID: 32741611 [PubMed - as supplied by publisher]

Correction of Airway Stem Cells: Genome Editing Approaches for the Treatment of Cystic Fibrosis.

Hum Gene Ther. 2020 Aug 01;:

Authors: King NE, Suzuki S, Barillà C, Hawkins FJ, Randell SH, Reynolds SD, Stripp BR, Davis BR

Abstract
Cystic fibrosis (CF) is an autosomal recessive disease caused by variations in the cystic fibrosis transmembrane conductance regulator (CFTR) ion channel gene. Although CF disease manifests in multiple organs, the primary cause of mortality is pulmonary complications which result from poor clearance of hyper-viscous secretions. Recently developed CFTR modulators provide significant therapeutic benefit to the vast majority of CF patients. However, alternative treatments directed at the underlying cause are needed for the ~7% of CF patients who produce little or no CFTR protein. Genome editing restores the native CFTR genetic sequence and function to mutant cells and represents a promising approach to establish durable, physiologic production of CFTR in patients with loss of function CFTR mutations. Although it is possible that editing of the CFTR gene in various cell types of the airway may transiently restore CFTR activity, significant focus is being given to editing of airway basal stem/progenitor cells, since their correction would allow appropriate and durable expression of CFTR in various stem cell-derived epithelial cell types. Substantial progress has recently been made to directly correct airway basal cells in vitro, which would enable transplanting autologous corrected cells to CF patients for regeneration of the airway with CFTR functional cells. A second potential approach for preparation of autologous, gene edited airway basal cells is the derivation of CF patient-specific induced pluripotent stem cells (iPSCs), correction of the CFTR gene, and subsequent differentiation into airway basal cells. Despite these advances, further work is needed to develop transplantation methodology. An alternative approach for establishing a functional CFTR airway would be to directly perform the editing in vivo. Achieving this objective will require several elements including robust delivery methods and ensuring that basal cells are efficiently targeted and corrected. Recent advances in these editing therapies give hope that the genetic underpinning of CF can be durably corrected and provide a cure for CF lung disease.

PMID: 32741223 [PubMed - as supplied by publisher]

A mouse model of asthma-chronic obstructive pulmonary disease overlap induced by intratracheal papain.

Allergy. 2020 Aug 02;:

Authors: Fukuda K, Matsuzaki H, Mikami Y, Makita K, Miyakawa K, Miyashita N, Hosoki K, Ishii T, Noguchi S, Urushiyama H, Horie M, Mitani A, Yamauchi Y, Shimura E, Nakae S, Saito A, Nagase T, Hiraishi Y

PMID: 32740929 [PubMed - as supplied by publisher]

Predictive value of the modified Bhalla score for assessment of pulmonary exacerbations in adults with cystic fibrosis.

Eur Radiol. 2020 Aug 01;:

Authors: Diab-Cáceres L, Girón-Moreno RM, García-Castillo E, Pastor-Sanz MT, Olveira C, García-Clemente MM, Nieto-Royo R, Prados-Sánchez C, Caballero-Sánchez P, Olivera-Serrano MJ, Padilla-Galo A, Nava-Tomas E, Esteban-Peris A, Fernández-Velilla M, Torres M, Gómez-Punter RM, Ancochea J

Abstract
OBJECTIVES: The objective of this study was to analyze the predictive value of the modified Bhalla score in high-resolution computed tomography (HRCT) for assessment of pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients. We also describe the relationship between this score and pulmonary function test results.
METHODS: We performed a multicenter and prospective study where adult patients with CF were included consecutively over 18 months. All patients underwent HRCT with acquisition in inspiration and expiration. The results were analyzed by an expert radiologist who assigned a modified Bhalla score value. Lung function was also assessed, and clinical variables were collected. Follow-up lasted approximately 1 year, and PEx were registered.
RESULTS: The study population comprised 160 subjects selected from 360 CF patients monitored in the participating CF units. The mean age was 28 years, 47.5% were women, and mean forced expiratory volume in 1 s (FEV1) was 67.5%. The mean global modified Bhalla score was 14.5 ± 0.31 points. Pulmonary function test (PFT) results and the modified Bhalla score correlated well, mainly forced vital capacity (FVC) and FEV1. We constructed a statistical model based on the overall Bhalla score to predict the number of PEx.
CONCLUSIONS: The overall modified Bhalla score can predict future PEx in CF patients. This useful tool can help to prevent PEx in higher risk patients.
KEY POINTS: • Pulmonary function test results and the modified Bhalla score correlated well with FVC and FEV1. • The total modified Bhalla score can predict the number of exacerbations in adult CF patients. • Our findings highlight the need to establish a unified protocol for chest HRCT during the follow-up of adult patients with CF in order to anticipate possible complications and determine their impact on pulmonary function.

PMID: 32740815 [PubMed - as supplied by publisher]