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master's thesis
Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu
Zagreb: University of Zagreb, School of Medicine, 2016. urn:nbn:hr:105:594259
Sosa, Ivana
University of Zagreb
School of Medicine
Department of Pediatrics

Institutional repository: Dr Med - University of Zagreb School of Medicine Digital Repository

Cite this document

Sosa, I. (2016). Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu (Master's thesis). Zagreb: University of Zagreb, School of Medicine. Retrieved from https://urn.nsk.hr/urn:nbn:hr:105:594259

Sosa, Ivana. "Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu." Master's thesis, University of Zagreb, School of Medicine, 2016. https://urn.nsk.hr/urn:nbn:hr:105:594259

Sosa, Ivana. "Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu." Master's thesis, University of Zagreb, School of Medicine, 2016. https://urn.nsk.hr/urn:nbn:hr:105:594259

Sosa, I. (2016). 'Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu', Master's thesis, University of Zagreb, School of Medicine, accessed 24 December 2024, https://urn.nsk.hr/urn:nbn:hr:105:594259

Sosa I. Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu [Master's thesis]. Zagreb: University of Zagreb, School of Medicine; 2016 [cited 2024 December 24] Available at: https://urn.nsk.hr/urn:nbn:hr:105:594259

I. Sosa, "Epileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu", Master's thesis, University of Zagreb, School of Medicine, Zagreb, 2016. Available at: https://urn.nsk.hr/urn:nbn:hr:105:594259

Metadata
TitleEpileptičke encefalopatije s napadima senzitivnim na povišenu temperaturu
Title (english)Epileptic encephalopathy attacks sensitive to elevated temperature
AuthorIvana Sosa
MentorNina Barišić (mentor)
Committee memberDorian Tješić-Drinković (predsjednik povjerenstva)
Committee memberMarija Jelušić (član povjerenstva)
Committee memberNina Barišić (član povjerenstva)
GranterUniversity of Zagreb
School of Medicine
(Department of Pediatrics)
Zagreb
Defense date and country2016-07-15, Croatia
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Pediatrics
Abstract
Teška epileptička encefalopatija u djece školske dobi, koja su do tada bila zdrava opisuje se u literaturi s više različitih naziva, te je predložen naziv epileptičkog sindroma povezanog s febrilnom infekcijom (febrile infection related epilepsy syndrome, FIRES). Nakon febrilnog razdoblja započinje akutna faza u kojoj se javljaju učestali žarišni napadi koji progrediraju u farmakorezistentni epileptički status. Na kraju dolazi do razvoja kronične faze, koju obilježava farmakorezistentna epilepsija i kognitivni poremećaji. Etiologija ovoga sindroma nije poznata, ali postoje hipoteze o upalnom, genetskom, metaboličkom i autoimunom mehanizmu nastanka sindroma. Dijagnoza se postavlja na temelju kliničke slike nakon isključenja ostalih uzroka. Trenutno ne postoji specifična terapija. Odgovor na standardnu antikonvulzivnu terapiju ne zadovoljava, te se u terapiji koriste farmakološki uzrokovana koma, ketogena dijeta i imunoglobulini, ali učinkovitost ovih metoda nije dokazana. Prognoza ovoga sindroma je loša, te samo manji broj pacijenata preživi epizodu bez trajnih neurološko-psihičkih poremećaja. Dravetin sindrom, epileptička encefalopatija uzrokovana mutacijom gena za protokadherin 19 (PCDH19) te idiopatska hemikonvulzivna-hemiplegija i epilepsija (IHHE) predstavljaju epileptičke encefalopatije koje dijele karakteristike sa FIRES-om.
Abstract (english)
Severe epileptic encephalopathy in previously healthy school-aged children has been reported in literature under various names and the name febrile infection related epilepsy syndrome (FIRES) is proposed. A brief febrile episode is followed by an acute phase characterized by recurrent focal seizures that evolve into drug-resistant status epilepticus. Finally, patients enter a chronic phase consisting of drug-resistant epilepsy and cognitive impairment. The etiology of this syndrome is still unknown and inflammatory, genetic, metabolic and autoimmune mechanism has been hypothesized. The diagnosis is usually made on clinical grounds once most other causes have been excluded. There is currently no specific therapy for FIRES. Seizures do not respond well to high doses of conventional anti-convulsant medications. Burst suppression coma, ketogenic diet and immunoglobulins are used in therapy, although their efficacy has not been proved. The outcome of FIRES is bad and only a minority survive the episode without any permanent neuro-psychological impairment. Dravet syndrome, epileptic encephalopathy caused by mutation of the protocadherin gene PCDH19 and idiopathic hemiconvulsion hemiplegia and epilepsy (IHHE) are epileptic encephalopathies which share similarities with FIRES.
Keywords
Keywords (english)
Languagecroatian
URN:NBNurn:nbn:hr:105:594259
Study programmeTitle: Medicine
Study programme type: university
Study level: integrated undergraduate and graduate
Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
Type of resourceText
File originBorn digital
Access conditionsOpen access
Terms of useLicence In copyright icon
Created on2017-06-23 08:36:40