Terapijska primjena koncentrata koagulacijskog faktora XIII

master's thesis

• Title (english): Therapeutic administration of coagulation factor XIII
• Author: Ema Šćulac
• Mentor: Ana Boban (mentor)
• Committee member: Dražen Pulanić (predsjednik povjerenstva)
• Committee member: Jasenka Markeljević (član povjerenstva)
• Committee member: Ana Boban (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Internal Medicine); Zagreb
• Defense date and country: 2022-07-15, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

Koagulacijski faktor XIII (FXIII) je faktor u koagulacijskoj kaskadi čija aktivacija dovodi do povezivanja fibrina i inhibitora fibrinolize u fibrinski ugrušak koji je manje podložan fibrinolizi. Deficijencija FXIII može biti posljedica nasljednog ili stečenog poremećaja te dovodi do produljenog krvarenja. Nasljedni oblik je rijetka koagulacijska bolest s prevalencijom 1 na 2 milijuna osoba. Stečeni manjak je češći oblik i karakteriziran je autoimunošću ili povećanom potrošnjom FXIII. Pacijenti s manjkom FXIII liječe se nadoknadom FXIII čime se sprječava ili zaustavlja krvarenje uzrokovano njegovim manjkom. U ovom radu smo retrospektivno analizirali indikaciju za primjenu koncentrata FXIII u KBC-u Zagreb u periodu od siječnja 2020. do ožujka 2021. godine. Prikupljeni su demografski podaci, dijagnoza prijema u bolnicu, indikacija za primjenu koncentrata FXIII, ishod liječenja, te klinika na kojoj su ispitanici liječeni. U navedenom razdoblju, ukupno je 24 ispitanika primilo koncentrat FXIII. Najčešće dijagnoze bile su aneurizma aorte, leukemije i limfomi, diseminirana intravaskularna koagulacija, ciroza jetre i zatajenje bubrega. Njih 21 (87,5%) imalo je kao indikaciju za terapiju koncentratom FXIII neki oblik krvarenja, dok je 10 (41,67%) zadovoljilo kriterije za masovno krvarenje. Postotak smrtnosti iznosio je 54,2%. Prije primjene terapije, aktivnost FXIII određena je u 16 (66,7%) ispitanika, a bila je snižena kod svih ispitanika s određenim FXIII. Manjak FXIII klinički je entitet koji je nedovoljno dijagnosticiran, često zbog normalnih koagulacijskih parametara, a njegova nadoknada može poboljšati kliničku sliku i ishod pacijenta.

Abstract (english)

Coagulation factor XIII (FXIII) is a factor in the coagulation cascade whose activation leads to the binding of fibrin and fibrinolysis inhibitors into a fibrin clot that is less susceptible to fibrinolysis. FXIII deficiency can be the result of a hereditary or acquired disorder and leads to prolonged bleeding. The hereditary form is a rare coagulation disease with a prevalence of 1 in 2 million people. Acquired deficiency is the more common form and is characterized by autoimmunity or increased consumption of FXIII. Patients with FXIII deficiency are treated with FXIII replacement therapy, which prevents or stops bleeding. In this paper, we retrospectively analyzed the indication for the use of FXIII concentrate at the University Hospital Centre Zagreb, in the period from January 2020 to March 2021. We acquired demographic data, the diagnosis of admission to the hospital, the indication for the use of FXIII concentrate, the treatment outcome, and the clinic where patients were treated. A total of 24 patients received FXIII concentrate in the mentioned period. The most common diagnoses were aortic aneurysm, leukemia, and lymphoma, disseminated intravascular coagulopathy, liver cirrhosis and kidney failure. As an indication for FXIII concentrate therapy, 21 of them (87.5%) had some form of bleeding, while 10 (41.67%) met the criteria for massive bleeding. The percentage of mortality was 54.2%. FXIII activity was determined in 16 (66.7%) patients before therapy and was decreased in all patients with determined FXIII activity. FXIII deficiency is an underdiagnosed clinical entity, often due to normal coagulation parameters, and its replacement can improve the clinical picture and patient outcome.

• Keywords: faktor XIII; terapija; koagulacija; poremećaji krvarenja
• Keywords (english): factor XIII; therapy; coagulation; bleeding disorders
• Language: croatian
• URN:NBN: urn:nbn:hr:105:059720
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2023-02-28 10:02:30