{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:12:42Z","timestamp":1760145162971,"version":"build-2065373602"},"reference-count":30,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T00:00:00Z","timestamp":1718841600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Faculty of Health Sciences, Jagiellonian University Medical College, Krakow, Poland"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and Objectives: Prolidase deficiency (PD) is a rare, life-threatening, genetically determined disease with an incidence of 1\u20132 cases per 1 million births. The disease inhibits collagen synthesis, which leads to organ and systems failure, including hepato- and splenomegaly, immune disorders, chronic ulcerative wounds, respiratory infections, and pulmonary fibrosis. The complexity of the problems associated with this disease necessitates a comprehensive approach and the involvement of an interdisciplinary team. The objective was to present the treatment and care plan, as well as complications of PD, in a young woman following admission to an intensive care unit (ICU). Materials and Methods: A retrospective observational single-case study. Results: A 26-year-old woman with PD was hospitalized in the ICU for acute respiratory failure. The presence of difficult-to-heal extensive leg ulcers and the patient\u2019s immunocompromised condition resulted in the development of sepsis with multiple organ failure (respiratory and circulatory, liver and kidney failure). Complex specialized treatment consisting of wound preparation, limb amputation, the minimization of neuropathic pain, mechanical ventilation, renal replacement therapy, circulatory stabilization, and the prevention of complications of the disease and of therapy were applied. On the 83rd day of hospitalization, the patient expired. Conclusions: Despite the use of complex treatment and care, due to the advanced nature of the disease and the lack of therapies with proven efficacy, treatment was unsuccessful. There is a need for evidence-based research to develop effective treatment guidelines for PD.<\/jats:p>","DOI":"10.3390\/medicina60061006","type":"journal-article","created":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T03:46:29Z","timestamp":1718855189000},"page":"1006","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Multiorgan Failure and Sepsis in an ICU Patient with Prolidase Enzyme Deficiency\u2014The Specificity of Treatment and Care: A Case Report"],"prefix":"10.3390","volume":"60","author":[{"given":"Katarzyna","family":"Wojnar-Gruszka","sequence":"first","affiliation":[{"name":"Department of Clinical Nursing, Faculty of Health Sciences, Jagiellonian University\u2014Medical College, 31-501 Krak\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2807-2500","authenticated-orcid":false,"given":"Ilona","family":"Nowak-K\u00f3zka","sequence":"additional","affiliation":[{"name":"Center for Innovative Medical Education, Jagiellonian University\u2014Medical College, 30-688 Krak\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2170-3760","authenticated-orcid":false,"given":"Jakub","family":"Cicho\u0144","sequence":"additional","affiliation":[{"name":"Department of Clinical Nursing, Faculty of Health Sciences, Jagiellonian University\u2014Medical College, 31-501 Krak\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aleksandra","family":"Ogryzek","sequence":"additional","affiliation":[{"name":"Clinical Department of Neurology, University Hospital in Krak\u00f3w, 30-688 Krak\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0767-6559","authenticated-orcid":false,"given":"Lucyna","family":"P\u0142aszewska-\u017bywko","sequence":"additional","affiliation":[{"name":"Department of Clinical Nursing, Faculty of Health Sciences, Jagiellonian University\u2014Medical College, 31-501 Krak\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3422","DOI":"10.1111\/febs.14620","article-title":"Structural basis for prolidase deficiency disease mechanisms","volume":"285","author":"Wilk","year":"2018","journal-title":"FEBS J."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Linhares, N.D., Wilk, P., Wator, E., Tostes, M.A., Weiss, M.S., and Pena, S.D.J. 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