Creutzfeldt-Jakobova bolest: prikaz slučaja i pregled literature

Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation and accumulation of pathologic cellular protein, occurring in 3 general forms: sporadic or spontaneous, genetic or familial, and acquired form including a variant form of CJD. Clinical presentation of CJD is characterized by progressive dementia, neurologic symptoms and visual impairment, development of akinetic mutism, and eventually death, usually from respiratory infection. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging findings. A case is presented of a 56-year-old woman with progressive dementia, typical neurologic symptoms, positive cerebrospinal fluid and typical magnetic resonance imaging findings. The clinical, pathologic and imaging findings of this rare condition are also discussed.Creutzfeldt-Jakobova bolest (CJD) je rijetka i smrtonosna neurodegenerativna bolest koju uzrokuje infektivna bjelančevina nazvana prion, a obilježena je spužvastim promjenama, gubitkom neurona, reaktivnom proliferacijom astrocita i nakupljanjem patološke stanične bjelančevine. Bolest se javlja u tri glavna oblika: sporadični ili spontani, genetski ili obiteljski, te stečeni oblik uključujući varijantni oblik CJD. Klinički je CJD obilježena progresivnom demencijom, neurološkim simptomima i poremećajem vida, razvojem akinetskog mutizma, te konačno smrću, najčešće zbog dišne infekcije. Dijagnoza se temelji na kliničkim značajkama, elektroencefalogramu, te tipičnim nalazima likvora i magnetske rezonancije. Prikazuje se slučaj 56-godišnje bolesnice s progresivnom demencijom, tipičnim neurološkim simptomima, te pozitivnim nalazima likvora i magnetske rezonancije. Također se raspravlja o kliničkim i patološkim nalazima, te nalazima slikovnog prikazivanja u ovoj rijetkoj bolesti

Inovativne terapije redefiniraju terapijske ciljeve u multiploj sklerozi

The treatment of multiple sclerosis (MS) is becoming more complex, especially with the expanding number of available therapies for relapsing forms of MS. Greater understanding of the degenerative aspects of MS pathogenesis is redefining treatment goals and creating new treatment strategies. The existing immunomodulation drugs (disease-modifying therapies, DMTs) used in MS treatment have shown only partial benefits in controlling disease progression, primarily by reducing the inflammation component. However, new therapies for MS have been shown to be effective in delaying disease progression by protecting against brain atrophy, which is considered the most important preindicator of future patient disability. The favorable effect on reducing brain atrophy suggests the potential neuroprotective or even neuroregenerative effects of new treatments, marking progress in the treatment of MS.Liječenje multiple skleroze (MS) postaje sve složenije, naročito zbog rastućeg broja dostupnih terapija za relapsni oblik MS. Sve bolje razumijevanje degenerativnog aspekta patogeneze MS redefinira terapijske ciljeve i stvara nove terapijske strategije. Dosadašnji imunomodulacijski lijekovi (disease-modifying therapies, DMTs) u terapiji MS pokazuju samo djelomičnu korist u kontroli progresije bolesti, prvenstveno smanjujući upalnu komponentu. Međutim, nove terapije u MS pokazuju djelotvoran učinak na odgađanje progresije bolesti štiteći od atrofije mozga koja se smatra najvažnijim predskazateljem budućeg invaliditeta bolesnika. Pozitivan utjecaj na smanjenje atrofije mozga ukazuje na potencijalno neuroprotektivno ili čak neuroregenerativno djelovanje novih terapija, što predstavlja korak naprijed u liječenju MS

Primarni vaskulitis središnjega živčanog sustava - dijagnostički izazov

Primary angiitis of the central nervous system (PACNS) is a rare and severe disease confined to the central nervous system, i.e., the brain and spinal cord. The etiology, pathogenesis and immune mechanism of PACNS have not yet been completely elucidated. The diagnosis is challenging; it is based upon constellation of clinical picture, cerebrospinal fluid analysis, imaging methods or tissue biopsy as the gold standard. In differential diagnosis of PACNS, it is necessary to rule out infectious, malignant or systemic inflammatory diseases, as well as reversible cerebral vasoconstriction syndrome. Immunosuppressants are cornerstone therapy for PACNS, although evidence-based strategies for the management are lacking so far. PACNS is an entity with considerable morbidity and mortality. Awareness of this rare and heterogeneous disease is crucial for establishing early diagnosis and treatment initiation.Primarni vaskulitis središnjega živčanog sustava (PVSŽS) je rijetka i teška bolest ograničena na središnji živčani sustav, tj. mozak i leđnu moždinu. Etiologija, patogeneza i imuni mehanizam PVSŽS-a još nisu u potpunosti razjašnjeni. Dijagnoza je zahtjevna i postavlja se na temelju kliničke slike, nalaza lumbalne punkcije, slikovnih metoda ili biopsije tkiva kao zlatnog standarda. U diferencijalnoj dijagnozi PVSŽS-a potrebno je isključiti infektivne, maligne ili sistemske upalne bolesti, kao i reverzibilni vazokonstrikcijski sindrom. Imunosupresivi su temelj terapije, iako zasad nema jasnih smjernica i preporuka za liječenje ove bolesti. PVSŽS je entitet sa značajnim pobolom i smrtnošću. Svijest o ovoj rijetkoj bolesti složene kliničke prezentacije ključna je za postavljanje rane dijagnoze i početak liječenja

Liječenje bolesnika oboljelih od mijastenije gravis s COVID-19: pregled literature

Coronavirus disease 2019 (COVID-19), caused by the late 2019 outbreak of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), causes a respiratory disease which could put myasthenia gravis (MG) patients at a greater risk of developing severe disease course, since infections and some drugs are a well-recognized trigger of symptom exacerbation in MG patients. Out of ten most commonly used past and present drugs used in COVID-19 treatment, two (quinolone derivatives and azithromycin) are known to worsen MG symptoms, whereas another two (tocilizumab and eculizumab) might have positive effect on MG symptoms. Colchicine, remdesivir, lopinavir, ritonavir and favipiravir seem to be safe to use, while data are insufficient for bamlanivimab, although it is also probably safe to use. Considering MG treatment options in patients infected with SARS-CoV-2, acetylcholine esterase inhibitors are generally safe to use with some preliminary studies even demonstrating therapeutic properties in regard to COVID-19. Corticosteroids are in general safe to use, even recommended in specific circumstances, whereas other immunosuppressive medications (mycophenolate mofetil, azathioprine, cyclosporine, methotrexate) are probably safe to use. The only exception is rituximab since the resulting B cell depletion can lead to more severe COVID-19 disease. Concerning plasmapheresis and intravenous immunoglobulins, both can be used in COVID- 19 while taking into consideration thromboembolic properties of the former and hemodynamic disturbances of the latter. As current data suggest, all known COVID-19 vaccines are safe to use in MG patients.Koronavirusna bolest 2019 (COVID-19) uzrokovana širenjem virusa SARS-CoV-2 izaziva respiracijsku bolest koja potencijalno može dodatno ugroziti pacijente koji boluju od mijastenije gravis. Uz infekcije, egzacerbaciju simptoma mijastenije gravis mogu uzrokovati i neki lijekovi. Od deset najčešće primjenjivanih skupina lijekova za liječenje COVID-19, dvije skupine lijekova (kinoloni i azitromicin) mogu uzrokovati pogoršanje simptoma mijastenije gravis, a dvije skupine lijekova (tocilizumab i ekulizumab) imaju pozitivne učinke na simptome mijastenije gravis. Kolhicin, remdesivir, lopinavir, ritonavir i favipiravir nemaju učinka na simptome mijastenije gravis, dok su podatci za bamlanivimab nedostatni, no isti je vjerojatno siguran za upotrebu u bolesnika s mijastenijom gravis. Što se tiče primjene lijekova za mijasteniju gravis kod bolesnika zaraženih virusom SARS-CoV-2, inhibitori acetilkolinestaraze su u pravilu sigurni za primjenu; neke studije su čak dokazale i terapijske mogućnosti navedenih lijekova u liječenju COVID-19. Kortikosteroidi su uglavnom sigurni za primjenu, a čak se i preporučaju u određenim okolnostima, dok su ostali imunosupresivi (mikofenolat mofetil, azatioprin, ciklosporin i metotreksat) sigurni za primjenu. Jedina iznimka je rituksimab učinak kojega na B-staničnu imunost može rezultirati težom kliničkom slikom bolesti COVID-19. Plazmafereza i intravenski imunoglobulini, dvije najčešće primjenjivane terapijske opcije liječenja akutnih egzacerbacija mijastenije gravis, mogu se primijeniti kod bolesnika s COVID-19 uzimajući u obzir tromboembolijske učinke intravenskih imunoglobulina, odnosno hemodinamske učinke plazmafereze. Sva cjepiva COVID-19, koliko je poznato, sigurna su za primjenu u bolesnika s mijastenijom gravis

IMPLEMENTATION OF QUALITY MANAGEMENT SYSTEMS AT DIFFERENT LEVELS OF HEALTH CARE

Osiguranje kvalitete postaje nužni preduvjet uspjeha u zdravstvenoj djelatnosti zbog rastućih potreba i očekivanja svih zainteresiranih strana. Hrvatski zdravstveni sustav teži povećanju razine kvalitete zdravstvenih usluga uvođenjem obvezne primjene sustava kvalitete zdravstvene zaštite te dobrovoljnog postupka akreditacije zdravstvenih ustanova i uvođenja certificiranih sustava za upravljanje kvalitetom ISO 9001:2008. Cilj ovog rada je ispitati poznavanje važnosti upravljanja kvalitetom u zdravstvu, svijesti menadžmenta zdravstvenih ustanova o kvaliteti te utjecaja primjene sustava upravljanja kvalitetom na zadovoljstvo pacijenta na različitim razinama zdravstvene djelatnosti. Rezultati istraživanja provedenim anketnim ispitivanjem na uzorku hrvatskih zdravstvenih ustanova primarne, sekundarne i tercijarne razine pokazali su da je poznavanje i primjena sustava upravljanja kvalitetom u hrvatskom zdravstvenom sustavu još uvijek nedovoljna te upućuju na nužnost promjene ponašanja i podizanja svijesti o važnosti primjene sustava upravljanja kvalitetom kao i povećanja izobrazbe menadžmenta i zaposlenih u hrvatskom zdravstvenom sustavu vezano uz pitanje kvalitete.Quality assurance has become the required precondition for successful provision of healthcare services due to the increasing needs and expectations of all interested parties. The Croatian healthcare system seeks to enhance the quality of healthcare services by introducing the mandatory system of quality standards, the accreditation process of health institutions and voluntary certifi ed quality management systems ISO 9001:2008. This paper attempts to address the issue of knowledge about the importance of quality management in healthcare, whether the management of medical institutions is aware of the issue of quality assessment and the impact of certifi ed quality management system implementation on patients’ satisfaction level at different levels of the healthcare system. Results of the survey carried out on a sample of Croatian healthcare institutions at primary, secondary and tertiary levels indicate that there is insuffi cient knowledge and implementation of the quality management system in the healthcare system. Moreover, they point to the necessity of change in behaviour and of raising awareness about the importance of implementing quality management systems, as well as the necessity of increased education on the issue of quality aimed at the management and healthcare workforce

ROLE OF QUALITY IN APPLICATION OF SURGICAL LABORATORY FOR BIOMEDICAL RESEARCH FOR INFRASTRUCTURAL PROJECTS OF EUROPEAN REGIONAL DEVELOPMENT FUND

Ovaj rad prikazuje prijavu Kirurškog laboratorija za biomedicinska istraživanja Zavoda za kardijalnu i transplantacijsku kirurgiju Kliničke bolnice Dubrava za nabavu opreme putem natječaja za infrastrukturalne projekte Europskog fonda za regionalni razvoj. Ministarstvo znanosti, obrazovanja i sporta Republike Hrvatske u veljači 2012. objavilo je Javni poziv za dostavu projektnih prijedloga kako bi se pripremila zaliha potencijalnih infrastrukturnih projekata čija bi se provedba mogla financirati iz Europskog fonda za regionalni razvoj u sklopu Operativnog programa za regionalnu konkurentnost 2014.- 2020. U radu je detaljno opisano od kuda uopće potreba za istraživačkim laboratorijem u sklopu jednog kliničkog odjela, te na koji se način nabavka opreme uklapa u strategije kako Republike Hrvatske, tako i Europske unije. Na kraju je istaknuto što će nabavom opreme dobiti sam Laboratorij, Republika Hrvatska i Europska unija.This paper presents an application of the Surgical Laboratory for Biomedical Research, Department of Cardiac and Transplantation Surgery, Dubrava University Hospital, for purchase of equipment through tenders for infrastructural projects of the European Fund for Regional Development. In February 2012, the Croatian Ministry of Science, Education and Sports announced a public call for proposals to prepare inventories of potential infrastructural projects, whose implementation could be financed from EFRD within the Operational Programme for Regional Competitiveness 2014 -2020. This paper describes in detail where the need for a research laboratory within a clinical department came from, and in what way this equipment purchase fi ts into the strategies of both Croatia and European Union. The paper emphasizes the benefits of this equipment purchase for the Laboratory, the Republic of Croatia and the European Union

Istraživanje percepcija studenata o kvaliteti usluge visokog obrazovanja

Changes in the higher education system resulted in higher education institutions having to pay more attention to their students. Keeping students and ensuring their satisfaction is the basis for sustainable success of higher education institutions. The aforementioned is the reason why this paper is focused on analyzing students’ perception of the quality of service in higher education. The main purpose of this paper is to analyze how students in the Croatian system of higher education perceive the quality of service and if there are differences with regard to individual characteristics of students. On the basis of HEdPERF instrument, data was collected for 1454 students from 93 higher education institutions in the Republic of Croatia. Principle component analysis was conducted to define key dimensions of quality. Based on the results of the analysis, five dimensions were extracted: access, non-academic dimension, academic dimension, space and study programs, and reputation of a higher education institution. The second part of the research was based on exploring the impacts of the control variables on the perception of quality. The results have indicated the existence of a statistically significant difference between students’ perceptions for the majority of the observed control variables.Promjene u visokoobrazovnom sustavu dovele su do situacije da visoka učilišta moraju sve više pažnje posvetiti svojim studentima. Zadržavanje studenata i osiguranje njihova zadovoljstva osnova su održivog uspjeha visokih učilišta. Stoga se u ovom radu analiziraju percepcije studenata o kvaliteti visokoobrazovne usluge. Osnovni je cilj rada istražiti kako studenti u hrvatskom sustavu visokog obrazovanja percipiraju kvalitetu usluge, te postoje li razlike s obzirom na pojedine osobine studenata. Na temelju HEdPERF instrumenta prikupljeni su podaci od 1454 studenta s 93 visoka učilišta u Republici Hrvatskoj. Provedena je analiza glavnih komponenti kako bi se definirale ključne dimenzije kvalitete. Na temelju rezultata analize izdvojeno je pet dimenzija: dostupnost, neakademska dimenzija, akademska dimenzija, prostor i studijski programi, ugled visokog učilišta. Drugi dio istraživanja temeljio se na ispitivanju utjecaja kontrolnih varijabli na percepcije kvalitete. Rezultati su pokazali postojanje statistički signifikantne razlike između percepcija studenata za većinu promatranih kontrolnih varijabli

Multipla skleroza: novi aspekt imunopatogeneze

Multiple sclerosis (MS) is a multicomponent disease characterized by inflammation, neurodegeneration, and cancellation of the central nervous system recovery mechanisms. The cause of MS is still unknown, but it is undeniable that genetic, environmental and immune factors are involved in the etiopathogenesis of this complex and heterogeneous disease. From the aspect of im-munopathogenesis, until recently the opinion prevailed that autoreactive T lymphocytes played a major role, the activation of which is a key step in MS. The knowledge of the effector and regulatory roles of B cells supports a new concept of MS immunopathogenesis that is based on the highly complex interaction of T and B cells, with B cells actively participating in cellular immunity by directing the intensity and quality of cellular immune response. The mechanisms of B cell activity in MS immunopathogenesis are multiple and include antigen presentation and T cell costimulation, cytokine secretion, antibody synthesis, and formation of ectopic lymphoid B cell aggregates in the intrameningeal spaces. The importance of B cells has been confirmed by modern therapeutic options for the treatment of MS.Multipla skleroza (MS) je multikomponentna bolest koju karakterizira upala, neurodegeneracija i otkazivanje mehanizama za obnavljanje središnjega živčanog sustava. Uzrok MS još uvijek je nepoznat, ali je nedvojbeno da su genetski, okolišni i imuni čimbenici uključeni u etiopatogenezu ove složene i heterogene bolesti. S aspekta imunopatogeneze, donedavno je prevladavalo mišljenje da glavnu ulogu imaju autoreaktivni T limfociti aktiviranje kojih predstavlja ključni korak u nastanku MS. Saznanja o efektorskim i regulatornim ulogama B stanica podupiru novi koncept imunopatogeneze MS koji se temelji na vrlo kompleksnoj interakciji T i B stanica, s tim da B stanice aktivno sudjeluju u staničnoj imunosti tako što usmjeravaju intenzitet i kvalitetu staničnog imunog odgovora. Mehanizmi djelovanja B stanica u imunopatogenezi MS su višestruki te uključuju prezentaciju antigena i kostimulaciju T stanica, lučenje citokina, sintezu protutijela i formiranje ektopičnih limfoidnih B staničnih agregata u intrameningealnim prostorima. Značenje B stanica u imunopatogenezi MS potvrđuju i suvremene terapijske opcije za liječenje MS