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Cystic Fibrosis: Diagnosis and Treatment
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Cystic Fibrosis: Diagnosis and Treatment

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

Deadline for manuscript submissions: 30 August 2025 | Viewed by 3142

Special Issue Editors

Dr. Corrado Tagliati

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Guest Editor
Radiologia AST Pesaro Urbino, 61121 Pesaro, Italy
Interests: cystic fibrosis; ultrasound; CT
Prof. Dr. Gian Marco Giuseppetti

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Guest Editor
Department of Radiological Sciences, Azienda Ospedaliero Universitaria Ospedali Riuniti, Università Politecnica delle Marche, 60121 Ancona, Italy
Interests: breast imaging; ultrasound; CT

Special Issue Information

Dear Colleagues,

You know better than us that cystic fibrosis is an important genetic disease caused by a myriad of CFTR gene mutations and that the sweat chloride test, genetic testing, prenatal/preconception testing, and newborn screening are different ways to obtain a diagnosis. In recent years, novel drugs have dramatically changed the symptoms and, therefore, the lives of many cystic fibrosis patients who are allowed to take them. Moreover, the median age of treated patients is reducing over time thanks to new legislations. What's more, a lot of patients without F508del will hope to be allowed to take these new medications.

In this Special Issue, we welcome original articles, reviews, brief reports about the diagnosis, treatment, and management of cystic fibrosis.

Dr. Corrado Tagliati
Prof. Dr. Gian Marco Giuseppetti
Guest Editors

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
  • diagnosis
  • treatment
  • management

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Published Papers (3 papers)

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Research

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14 pages, 674 KiB  
Article
Exploration of Olfaction and ChiPSO in Pediatric Cystic Fibrosis
by Tiana M. Saak, Jeremy P. Tervo, Brandon M. Moore, Alicia S. Wang, Emily DiMango, Hossein Sadeghi, David A. Gudis and Jonathan B. Overdevest
J. Clin. Med. 2025, 14(8), 2583; https://doi.org/10.3390/jcm14082583 - 9 Apr 2025
Viewed by 319
Abstract
Background/Objectives: Olfactory dysfunction (OD) is a common symptom among people with cystic fibrosis (PwCF) and contributes to environmental safety concerns, nutritional challenges, and an overall diminished quality of life. OD is perceived to progress along the lifespan in PwCF, often due to [...] Read more.
Background/Objectives: Olfactory dysfunction (OD) is a common symptom among people with cystic fibrosis (PwCF) and contributes to environmental safety concerns, nutritional challenges, and an overall diminished quality of life. OD is perceived to progress along the lifespan in PwCF, often due to worsening sinonasal disease. Among children with cystic fibrosis (CwCF), OD is poorly characterized as limited resources and tolerance contribute to challenges in psychophysical olfactory evaluation among pediatric populations. The Children’s Personal Significance of Olfaction (ChiPSO) questionnaire was recently proposed as a tool to assess olfaction and the importance of olfactory stimulation among children. This pilot study aimed to evaluate the utility of ChiPSO among a cohort of ethnically diverse CwCF. Methods: Individuals aged 7–17 with physician-diagnosed CF were asked to complete questionnaires, including ChiPSO and the brief questionnaire on olfactory dysfunction (bQOD-NS), prior to undergoing psychophysical olfactory evaluation with the U-Sniff Identification test. Potential associations between questionnaires and olfactory performance, pulmonary function, and demographic characteristics were evaluated using Pearson and Spearman correlations, independent-sample t-tests, Wilcoxon rank sum tests, and multiple linear regression. Results: U-Sniff Identification score positively correlated with the overall ChiPSO total score [r(13) = 0.640, p = 0.010] and its environmental subdomain score [r(13) = 0.774, p < 0.001], though not with the food subdomain [r(13) = 0.450, p = 0.093], the social subdomain [r(13) = 0.343, p = 0.2], or bQOD-NS score [r(11) = −0.125, p = 0.7]. Hispanic ethnicity is associated with ChiPSO (p = 0.041). Conclusions: In this preliminary study, olfactory importance increases with olfactory function among an ethnically diverse sample of CwCF, with a preferential influence of olfactory function on personal importance of environmental olfactory information. While these results should be interpreted with limitations imposed by the pilot nature of our sample size, our pilot data highlights associations with early adolescent development of importance of olfaction that can be disrupted in the setting of progressive disease among CwCF. Full article
(This article belongs to the Special Issue Cystic Fibrosis: Diagnosis and Treatment)
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Review

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18 pages, 1422 KiB  
Review
Clinical Disorders in Cystic Fibrosis That Affect Emergency Procedures—A Case Report and Review
by Sylwia Jarzynka, Mateusz Dobrosz, Sebastian Jaworski, Kamil Jóźwicki, Sebastian Wierzba, Olga Barbarska and Anna Minkiewicz-Zochniak
J. Clin. Med. 2025, 14(9), 3187; https://doi.org/10.3390/jcm14093187 - 5 May 2025
Abstract
Cystic fibrosis (CF) is a multisystemic disease caused by a genetic defect, namely a mutation in the CFTR gene, that results in the production of an abnormal protein that regulates the flow of chloride ions through epithelial cells, leading to the dehydration of [...] Read more.
Cystic fibrosis (CF) is a multisystemic disease caused by a genetic defect, namely a mutation in the CFTR gene, that results in the production of an abnormal protein that regulates the flow of chloride ions through epithelial cells, leading to the dehydration of secreted mucus and changes in its biological properties. Chronic inflammation and recurrent respiratory infections progressively damage lung tissue, leading to respiratory and cardiorespiratory failure. This study aims to present a clinical case and explore the clinical changes in CF that may influence the provision of pre-hospital first aid. The study presents a case report of a 23-year-old CF patient undergoing evaluation for lung transplantation, infected with Pseudomonas aeruginosa and Staphylococcus aureus with the MSSA phenotype, and in a severe condition due to infectious exacerbation. Despite antibiotic treatment, the patient’s condition deteriorated, leading to respiratory failure and cardiac arrest. Emergency measures were taken to maintain airway patency—the patient was sedated, intubated, and connected to a ventilator. CF involves systemic complications that, during exacerbations, may require urgent interventions. Cystic fibrosis is associated with multiple systemic complications, some of which may, during exacerbations, require emergency medical interventions. Providing care to this patient group involves specific procedures addressing the consequences of the underlying disease. Due to increasing survival rates and the emergence of new phenotypes, there is a need for the continuous education of medical personnel, including emergency responders, regarding the management of genetically determined diseases. This study underscores the importance of recognizing CF’s complex nature and adapting emergency care accordingly to ensure timely and effective intervention in life-threatening situations. Full article
(This article belongs to the Special Issue Cystic Fibrosis: Diagnosis and Treatment)
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19 pages, 325 KiB  
Review
Treatment of Psychological Symptoms in Patients with Cystic Fibrosis
by Giovanna Campagna, Corrado Tagliati, Gian Marco Giuseppetti and Pietro Ripani
J. Clin. Med. 2024, 13(19), 5806; https://doi.org/10.3390/jcm13195806 - 28 Sep 2024
Cited by 3 | Viewed by 2159
Abstract
The aim of this article is to identify and illustrate the most used psychological techniques in the field of cystic fibrosis (CF) and to help clinicians choose the most appropriate strategy among various possibilities. The disease and its medical treatments can be difficult [...] Read more.
The aim of this article is to identify and illustrate the most used psychological techniques in the field of cystic fibrosis (CF) and to help clinicians choose the most appropriate strategy among various possibilities. The disease and its medical treatments can be difficult to tolerate and can cause anxiety about health status or feelings of hopelessness and stress. The prevalence of depression and anxiety is 2.3 times higher in adults with CF than in community samples. A strong correlation has been identified between elevated psychological distress and unfavorable health outcomes, including, among others, impaired lung function, reduced BMI, an increased incidence of pulmonary exacerbations, and an elevated risk of transplantation. The use of psychological interventions is useful in addressing these common distresses in CF patients. Aware of the necessity of identifying efficacious interventions for all levels of depression and anxiety in CF patients, this study presents an overview of the research on psychological interventions for patients with CF, in order to complement the treatments suggested by the international guidelines on mental health in CF cases. In fact, the aim of this study is to conduct a review and quantitative synthesis of the psychological intervention techniques that are currently available for individuals with CF. Full article
(This article belongs to the Special Issue Cystic Fibrosis: Diagnosis and Treatment)
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