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Clinical Advances in Cystic Fibrosis
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Clinical Advances in Cystic Fibrosis

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (22 November 2023) | Viewed by 6312

Special Issue Editor

Dr. Rosanna Papa

E-Mail Website
Guest Editor
Department of Public Health and Infectious Diseases, Sapienza University, Rome, Italy
Interests: Pseudomonas aeruginosa; Staphylococcus aureus; virulence; biofilm; anti-virulence; natural compounds

Special Issue Information

Dear Colleagues,

This issue aims to gather a collection of review, perspective and original research articles that provide current efforts at basic, translational and clinical levels for the development of innovative therapies for cystic fibrosis disease. Furthermore, this topic aims to shed light on advances regarding the diagnosis, epidemiology, clinical management, and pathological mechanisms of this devastating disease.

Cystic fibrosis (CF) is a genetic disease, where a mutation of a protein called cystic fibrosis transmembrane conductance regulator leads to an alteration of ion transport across the cellular membrane, and the to the production of thick, sticky mucus that clogs the airways and traps microorganisms. This condition leads to inflammation and persistent bacterial infections, which principally cause respiratory malfunction.

The main problem associated with CF disease is represented by recurrent infections due to multi-resistant bacteria, including Pseudomonas aeruginosa. Recently, the WHO included P. aeruginosa in the ESKAPE pathogens, multi-resistant bacteria capable of ‘escaping’ the biocidal action of antibiotics and mutually representing new paradigms in pathogenesis, transmission and resistance.

Despite progress in understanding the pathological mechanisms involved in this genetic disorder, there is still no effective therapy able to halt its natural history or reverse the morphological and functional injury already established. At end-stage lung disease, lung transplantation remains the only feasible intervention.

This Special Issue aims to publish contributions from distinguished authors who actively experience innovations in the field of CF and want to provide more solid scientific evidence. All researchers are invited to contribute original works and reviews.

Dr. Rosanna Papa
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cystic fibrosis
  • lung disease
  • respiratory diseases
  • infection diseases
  • multi-drug resistant bacteria
  • innovative anti-virulence strategies

Published Papers (5 papers)

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Editorial

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4 pages, 199 KiB  
Editorial
Clinical Advances in Cystic Fibrosis
by Esther Imperlini and Rosanna Papa
J. Clin. Med. 2022, 11(21), 6306; https://doi.org/10.3390/jcm11216306 - 26 Oct 2022
Cited by 1 | Viewed by 1131
Abstract
Over recent decades, significant advances have been achieved in ameliorating clinical outcomes for patients with cystic fibrosis (CF) [...] Full article
(This article belongs to the Special Issue Clinical Advances in Cystic Fibrosis)

Research

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13 pages, 1317 KiB  
Article
Cystic Fibrosis in Adults: A Paradigm of Frailty Syndrome? An Observational Study
by Paola Iacotucci, Vincenzo Carnovale, Lorenza Ferrillo, Jolanda Somma, Marialuisa Bocchino, Marcella D’Ippolito, Alessandro Sanduzzi Zamparelli, Giuseppe Rengo, Nicola Ferrara, Valeria Conti and Graziamaria Corbi
J. Clin. Med. 2024, 13(2), 585; https://doi.org/10.3390/jcm13020585 - 19 Jan 2024
Viewed by 867
Abstract
This study aimed to assess the main clinical and anamnestic characteristics of adult Cystic Fibrosis (CF) patients and to evaluate the association of frailty with the CF genotyping classification. In an observational cross-sectional study, all ambulatory CF patients over 18 years old who [...] Read more.
This study aimed to assess the main clinical and anamnestic characteristics of adult Cystic Fibrosis (CF) patients and to evaluate the association of frailty with the CF genotyping classification. In an observational cross-sectional study, all ambulatory CF patients over 18 years old who received a diagnosis at the Regional Cystic Fibrosis Center for adults were enrolled and assessed by spirometry for respiratory function, by ADL and IADL for functional status, and by the Study of Osteoporotic Fractures (SOF) Index for frailty. The study population consisted of 139 CF patients (mean age 32.89 ± 10.94 years old, 46% women). Most of the subjects were robust (60.4%). The pre-frail/frail group was more frequently females (p = 0.020), had a lower BMI (p = 0.001), worse respiratory function, a higher number of pulmonary exacerbations/years, cycles of antibiotic therapy, and hospitalization (all p < 0.001) with respect to robust patients. The pre-frail/frail subjects used more drugs and were affected by more CF-related diseases (all p < 0.001). In relation to logistic regression, the best predictor of the pre-frail/frail status was a low FEV1 level. The CF patients show similarities to older pre-frail/frail subjects, suggesting that CF might be considered an early expression of this geriatric syndrome. This finding could help to better define the possible progression of CF, but overall, it could also suggest the usefulness employing of some tools used in the management and therapy of frailty subjects to identify the more severe CF subjects. Full article
(This article belongs to the Special Issue Clinical Advances in Cystic Fibrosis)
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16 pages, 965 KiB  
Article
Changes of Erythrocyte Fatty Acids after Supplementation with Highly Concentrated Docosahexaenoic Acid (DHA) in Pediatric Cystic Fibrosis: A Randomized Double-Blind Controlled Trial
by Roser Ayats-Vidal, Montserrat Bosque-García, Begoña Cordobilla, Oscar Asensio-De la Cruz, Miguel García-González, Jesús Castro-Marrero, Irene López-Rico and Joan Carles Domingo
J. Clin. Med. 2023, 12(11), 3704; https://doi.org/10.3390/jcm12113704 - 26 May 2023
Cited by 2 | Viewed by 1243
Abstract
We characterized the fatty acid profiles in the erythrocyte membrane of pediatric patients with cystic fibrosis (CF) receiving highly concentrated docosahexaenoic acid (DHA) supplementation (Tridocosahexanoin-AOX® 70%) at 50 mg/kg/day (n = 11) or matching placebo (n = 11) for 12 [...] Read more.
We characterized the fatty acid profiles in the erythrocyte membrane of pediatric patients with cystic fibrosis (CF) receiving highly concentrated docosahexaenoic acid (DHA) supplementation (Tridocosahexanoin-AOX® 70%) at 50 mg/kg/day (n = 11) or matching placebo (n = 11) for 12 months. The mean age was 11.7 years. The DHA group showed a statistically significant improvement in n-3 polyunsaturated fatty acids (PUFAs), which was observed as early as 6 months and further increased at 12 months. Among the n-3 PUFAs, there was a significant increase in DHA and eicosapentaenoic acid (EPA). Additionally, a statistically significant decrease in n-6 PUFAs was found, primarily due to a decrease in arachidonic acid (AA) levels and elongase 5 activity. However, we did not observe any changes in linoleic acid levels. The long-term administration of DHA over one year was safe and well tolerated. In summary, the administration of a high-rich DHA supplement at a dose of 50 mg/kg/day for one year can correct erythrocyte AA/DHA imbalance and reduce fatty acid inflammatory markers. However, it is important to note that essential fatty acid alterations cannot be fully normalized with this treatment. These data provide timely information of essential fatty acid profile for future comparative research. Full article
(This article belongs to the Special Issue Clinical Advances in Cystic Fibrosis)
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Other

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8 pages, 1120 KiB  
Case Report
Identification of the blaOXA-23 Gene in the First Mucoid XDR Acinetobacter baumannii Isolated from a Patient with Cystic Fibrosis
by Martina Rossitto, Gianluca Vrenna, Vanessa Tuccio Guarna Assanti, Nour Essa, Maria Luisa De Santis, Annarita Granaglia, Vanessa Fini, Valentino Costabile, Manuela Onori, Luca Cristiani, Alessandra Boni, Renato Cutrera, Carlo Federico Perno and Paola Bernaschi
J. Clin. Med. 2023, 12(20), 6582; https://doi.org/10.3390/jcm12206582 - 18 Oct 2023
Cited by 1 | Viewed by 1263
Abstract
Acinetobacter baumannii is one of the pathogens most involved in health care-associated infections in recent decades. Known for its ability to accumulate several antimicrobial resistance mechanisms, it possesses the oxacillinase blaoxa-23, a carbapenemase now endemic in Italy. Acinetobacter species are not [...] Read more.
Acinetobacter baumannii is one of the pathogens most involved in health care-associated infections in recent decades. Known for its ability to accumulate several antimicrobial resistance mechanisms, it possesses the oxacillinase blaoxa-23, a carbapenemase now endemic in Italy. Acinetobacter species are not frequently observed in patients with cystic fibrosis, and multidrug-resistant A. baumannii is a rare event in these patients. Non-mucoid A. baumannii carrying the blaoxa-23 gene has been sporadically detected. Here, we describe the methods used to detect blaoxa-23 in the first established case of pulmonary infection via a mucoid strain of A. baumannii producing carbapenemase in a 24-year-old cystic fibrosis patient admitted to Bambino Gesù Children’s Hospital in Rome, Italy. This strain, which exhibited an extensively drug-resistant antibiotype, also showed a great ability to further increase its resistance in a short time. Full article
(This article belongs to the Special Issue Clinical Advances in Cystic Fibrosis)
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5 pages, 214 KiB  
Case Report
Concomitant Use of Elexacaftor/Tezacaftor/Ivacaftor and Etanercept in a Cystic Fibrosis Patient with Juvenile Idiopathic Arthritis
by Sara Immacolata Orsini, Edoardo Marrani, Ilaria Pagnini, Giovanni Taccetti, Vito Terlizzi and Gabriele Simonini
J. Clin. Med. 2023, 12(5), 1730; https://doi.org/10.3390/jcm12051730 - 21 Feb 2023
Cited by 1 | Viewed by 1178
Abstract
Patients with cystic fibrosis often complain of joint manifestations. However, only a few studies have reported the association between cystic fibrosis and juvenile idiopathic arthritis and addressed the therapeutic challenges of these patients. We describe the first paediatric case of a patient affected [...] Read more.
Patients with cystic fibrosis often complain of joint manifestations. However, only a few studies have reported the association between cystic fibrosis and juvenile idiopathic arthritis and addressed the therapeutic challenges of these patients. We describe the first paediatric case of a patient affected by cystic fibrosis, Basedow’s disease and juvenile idiopathic arthritis who was contemporarily treated with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and anti-tumor necrosis factor α (anti-TNFα). This report seems to reassure regarding the potential side effects of these associations. Moreover, our experience suggests that anti-TNFα is an effective option in CF patients affected by juvenile idiopathic arthritis, and is even safe for children receiving a triple CFTR modulator. Full article
(This article belongs to the Special Issue Clinical Advances in Cystic Fibrosis)
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