{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:14:50Z","timestamp":1760058890707,"version":"build-2065373602"},"reference-count":98,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2025,5,5]],"date-time":"2025-05-05T00:00:00Z","timestamp":1746403200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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\u2019s condition deteriorated, leading to respiratory failure and cardiac arrest. Emergency measures were taken to maintain airway patency\u2014the 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\u2019s complex nature and adapting emergency care accordingly to ensure timely and effective intervention in life-threatening situations.<\/jats:p>","DOI":"10.3390\/jcm14093187","type":"journal-article","created":{"date-parts":[[2025,5,5]],"date-time":"2025-05-05T04:31:36Z","timestamp":1746419496000},"page":"3187","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Disorders in Cystic Fibrosis That Affect Emergency Procedures\u2014A Case Report and Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9269-3494","authenticated-orcid":false,"given":"Sylwia","family":"Jarzynka","sequence":"first","affiliation":[{"name":"Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-6769-058X","authenticated-orcid":false,"given":"Mateusz","family":"Dobrosz","sequence":"additional","affiliation":[{"name":"Student Scientific Club Agar, Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"},{"name":"Emergency Department, Pediatric Teaching Clinical Hospital, University Clinical Centre of the Medical University of Warsaw, Zwirki and Wigury 63A, 02-091 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-4563-2374","authenticated-orcid":false,"given":"Sebastian","family":"Jaworski","sequence":"additional","affiliation":[{"name":"Student Scientific Club Agar, Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"},{"name":"Emergency Department, Pediatric Teaching Clinical Hospital, University Clinical Centre of the Medical University of Warsaw, Zwirki and Wigury 63A, 02-091 Warsaw, Poland"},{"name":"Medical Emergency Department, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kamil","family":"J\u00f3\u017awicki","sequence":"additional","affiliation":[{"name":"Student Scientific Club Agar, Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Wierzba","sequence":"additional","affiliation":[{"name":"Student Scientific Club Agar, Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8708-7625","authenticated-orcid":false,"given":"Olga","family":"Barbarska","sequence":"additional","affiliation":[{"name":"School of Medical & Health Sciences, University of Economics and Human Sciences in Warsaw, Okopowa 59, 01-043 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1249-7670","authenticated-orcid":false,"given":"Anna","family":"Minkiewicz-Zochniak","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Medical University of Warsaw, Litewska 14\/16, 00-575 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,5,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1126\/science.2570460","article-title":"Identification of the cystic fibrosis gene: Genetic analysis","volume":"245","author":"Kerem","year":"1989","journal-title":"Science"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1016\/j.jcf.2020.06.011","article-title":"Fibrocyte accumulation in the lungs of cystic fibrosis patients","volume":"19","author":"Kasam","year":"2020","journal-title":"J. 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