3 research outputs found
Autoimmune encephalopathies in children: clasifi cation, diagnosis and treatment
Autoimune encefalopatije (AE) kliniÄki se manifestiraju znakovima limbiÄkog ili difuznog encefalitisa, a prema etiologiji se mogu
podijeliti u paraneoplastiÄne i neparaneoplastiÄne. Simptomi i znakovi autoimunog encefalitisa su vrlo varijabilni i najÄeÅ”Äe uklju-
Äuju glavobolju, epileptiÄke napadaje i nerijetko epileptiÄki status, poremeÄaje kretanja (ataksiju, diskinezije, koreju, distoniju i tremor),
poremeÄaje pamÄenja, ponaÅ”anja, psihoze te razliÄite stupnjeve poremeÄaja svijesti. Autonomna disfunkcija, poremeÄaji
spavanja i hipoventilacija Äesto su dio kliniÄke slike. Razvoj autoimunih encefalopatija mogu osim tumorskih potaknuti i virusni
antigeni, no najÄeÅ”Äe okidaÄi ostaju neidentifi cirani. Prema lokalizaciji antigena na koje je autoimunosni proces usmjeren, autoimune
se encefalopatije dijele u one uzrokovane protutijelima na intracelularne antigene (Ma2, Hu), a koje su ÄeÅ”Äe u odraslih,
paraneoplastiÄke su etiologije i slabo reagiraju na imunoterapiju te na one uzrokovane protutijelima na povrÅ”inske, odnosno
sinaptiÄke antigene (N-metil-diaspartatni receptor (NMDAR), kompleks naponom reguliranih kalijevih kanala VGKC /LGI1) koje
dobro reagiraju na imunoterapiju. Protutijela u autoimunim encefalopatijama dokazuju se u serumu i cerebrospinalnom likvoru. U
cerebrospinalnom se likvoru nalazi blaga pleocitoza i/ili oligoklonske vrpce, a nerijetko je nalaz u likvoru normalan. EEG pokazuje
difuzne encefalopatske spore disritmiÄke promjene ili tzv. āekstremne delta Äetkeā te žariÅ”ne epileptogene promjene, odnosno
paroksizmalna izbijanja u limbiÄkom encefalitisu. MR mozga u bolesnika s NMDAR protutijelima u pravilu je normalan ili pokazuje
prolazne subkortikalne T2 hiperintenzitete, no posebno je znaÄajan za dijagnozu limbiÄkog encefalitisa udruženog s kompleksom
protutijela VGKC/LGI1. Rano prepoznavanje AE-a, kao i poznavanje njegove potencijalne etiologije od izuzetnog je znaÄenja zbog
poduzimanja cjelovitog dijagnostiÄkog postupka i pravodobne primjene odgovarajuÄe terapije.Autoimmune encephalopathies are clinically manifested as limbic or diff use encephalitis. According to the etiology, they are classifi
ed as paraneoplastic and non-paraneoplastic. Signs and symptoms of autoimmune encephalitis are variable. The symptomatology
commonly includes headache and epileptic attacks often progressing to epileptic status, movement disorders (ataxia, dyskinesias,
chorea, dystonia and tremor), behavior changes, cognitive impairments, psychoses and various degrees of disorders of
consciousness. Faciobrachial dystonic seizures can precede the development of limbic encephalitis. Autonomic dysfunction, sleep
disorders and hypoventilation are often present. The development of autoimmune encephalopathies can be induced by either
tumor or viral antigens. However, in a signifi cant number of cases, disease triggers remain unidentifi ed. According to the localization
of target antigens, autoimmune encephalopathies can be divided into those caused by antibodies against intracellular antigens
(Ma2,Hu) and those caused by antibodies against cell surface antigens, i.e. synaptic antigens (N-methyl-D-aspartate receptor
(NMDAR), voltage-gated potassium channel complex/LGI1). The former are paraneoplastic in origin, more often in adults and
respond poorly to immunotherapy. The latter ones can aff ect children as well, and are usually-responsive to immunotherapy. Antibodies
can be detected in both cerebrospinal fl uid and serum. Mild pleocytosis and/or oligoclonal bands can be found in cerebrospinal
fl uid but in some patients the cerebrospinal examination fi ndings can be completely normal. The electroencephalography fi nding
consists of diff use, slow dysrhythmic encephalopathic changes or so-called extreme delta brushes and focal epileptogenic
changes, i.e. paroxysmal bursts in case of limbic encephalitis. Magnetic resonance image fi nding of the brain is usually normal or
presents transient sub/cortical hyperintensities in T2-weighted images but is signifi cant for the diagnosis of limbic encephalitis.
Early recognition of autoimmune encephalopathy is of utmost importance because of the need of proper diagnostic procedure and
timely introduction of appropriate therapy
Report on the work of the Reference center for pediatric cardiology Ministry of Health of the Republic of Croatia
Cilj je ovog izvjeÅ”Äa u proteklom trogodiÅ”njem razdoblju (2019ā2022) prikazati:
1) aktivnosti Referentnog centra,
2) postignute struÄne rezultate i primjenu novih metoda, postupaka i unaprjeÄenje struke, i
3) znanstvenu i struÄnu suradnju s inozemnim ustanovama visoke razine. Rezultati: Referentni je centar jedino mjesto u Republici Hrvatskoj koje kontinuirano zbrinjava populaciju najugroženijih i najtežih bolesnika pedijatrijske dobi sa srÄanom patologijom. Ima kontinuirano, 24 sata dostupnu kardioloÅ”ku, kardiokirurÅ”ku, anestezioloÅ”ku, neonatalnu i intenzivnu skrb za djecu sa složenim priroÄenim i steÄenim srÄanim bolestima. TakoÄer su dostupne metode nadomjeÅ”tanja funkcije organa u zatajivanju (ECMO potpora, LVAD-BiVAD, Berlin-Heart pumpa, hemodijaliza, program transplantacije srca / drugih organa), a sve zahvaljujuÄi timskom radu i suradnji tima nekoliko Zavoda. Izvode se složene kardiokirurÅ”ke operacije u djece s priroÄenim srÄanim greÅ”kama uz jasan trend poveÄanja broja i složenosti operacija, te uz i dalje prihvatljivo nisku smrtnost. Danas smo u moguÄnosti samostalno lijeÄiti gotovo sve srÄane bolesti u djece. GodiÅ”nje se izvede oko 200 kateterizacija srca u djece. ViÅ”e od 40% Äine intervencijske procedure, a viÅ”e od 50% tih intervencija izvodi se u dojenaÄkom periodu. Tijekom protekle tri godine uvedeno je pet novih perkutanih intervencijskih metoda: lijeÄenje nativne koarktacije i rekoarktacije umetanjem stenta, lijeÄenje stenoze pulmonalnih grana umetanjem stenta, perkutano umetanje valvule na pulmonalnu poziciju, dilatacija postojeÄeg stenta te zatvaranje aortopulmonalnih kolaterala u djece s univentrikulskim srcem. Navedeni iskoraci uÄinjeni su kontinuiranim zalaganjem Älanova tima uz potporu i mentorstvo, odnosno kontinuiranu suradnju s inozemnim struÄnjacima iz triju inozemnih ustanova (DeutschesHerzZentrum Muenchen, KinderherzZentrum Linz, Kids Heart Center Budapest). ZakljuÄak: NaÅ” centar stoji uz bok rijetkih centara u Europi koji su u moguÄnosti izvesti navedene procedure. Navedene su aktivnosti rezultirale unaprjeÄenjem kvalitete skrbi na razini RH i temelj su za daljnji planirani rast i razvoj struke u okvirima naÅ”e zemlje.The aim of this report is to show in the past three-year period (2019ā2022):
1)activities of the Reference Center,
2)achieved professional results and the application of new methods, procedures, and improvement of the profession, and
3)scientific and professional cooperation with high-level foreign institutions. Results: The reference center is the only place in the Republic of Croatia that continuously cares for the population of the most vulnerable children with cardiac pathology. It has continuous, 24-hour cardiology, cardiac surgery, anesthesiology, neonatal and intensive care for children with complex congenital and acquired heart diseases. Organ function replacement are also available (ECMO support, LVAD-BiVAD, Berlin-Heart pump, hemodialysis, heart/other organ transplant program) thanks to the teamwork and cooperation of different Departments. Complex cardiac surgeries are performed in children with a clear trend of increasing the number and complexity of surgeries, with low mortality. Today, we can independently treat almost all congenital heart defects in children. About 200 cardiac catheterizations are performed in children annually. More than 40% are interventional procedures with more than 50% of these interventions performed in infancy. In the past three years, five new percutaneous intervention methods have been introduced: stent insertion in native coarctation and in recoarctation, stent insertion in stenosis of the pulmonary branches, percutaneous valve insertion in the pulmonary position, dilatation of the existing stent, and closure of aortopulmonary collaterals in children with a univentricular heart. The steps were made by the continuous efforts of team members with support, mentoring, and continuous cooperation with foreign experts from three foreign institutions (DeutschesHerzZentrum Muenchen, KinderherzZentrum Linz, KidsHeart- Center Budapest). Conclusion: Our center stands alongside the rare centers in Europe that can perform the abovementioned procedures. The activities resulted in the improvement of the quality of care and form the basis for further development of the profession within the framework of our country
Report on the work of the Reference center for pediatric cardiology Ministry of Health of the Republic of Croatia
Cilj je ovog izvjeÅ”Äa u proteklom trogodiÅ”njem razdoblju (2019ā2022) prikazati:
1) aktivnosti Referentnog centra,
2) postignute struÄne rezultate i primjenu novih metoda, postupaka i unaprjeÄenje struke, i
3) znanstvenu i struÄnu suradnju s inozemnim ustanovama visoke razine. Rezultati: Referentni je centar jedino mjesto u Republici Hrvatskoj koje kontinuirano zbrinjava populaciju najugroženijih i najtežih bolesnika pedijatrijske dobi sa srÄanom patologijom. Ima kontinuirano, 24 sata dostupnu kardioloÅ”ku, kardiokirurÅ”ku, anestezioloÅ”ku, neonatalnu i intenzivnu skrb za djecu sa složenim priroÄenim i steÄenim srÄanim bolestima. TakoÄer su dostupne metode nadomjeÅ”tanja funkcije organa u zatajivanju (ECMO potpora, LVAD-BiVAD, Berlin-Heart pumpa, hemodijaliza, program transplantacije srca / drugih organa), a sve zahvaljujuÄi timskom radu i suradnji tima nekoliko Zavoda. Izvode se složene kardiokirurÅ”ke operacije u djece s priroÄenim srÄanim greÅ”kama uz jasan trend poveÄanja broja i složenosti operacija, te uz i dalje prihvatljivo nisku smrtnost. Danas smo u moguÄnosti samostalno lijeÄiti gotovo sve srÄane bolesti u djece. GodiÅ”nje se izvede oko 200 kateterizacija srca u djece. ViÅ”e od 40% Äine intervencijske procedure, a viÅ”e od 50% tih intervencija izvodi se u dojenaÄkom periodu. Tijekom protekle tri godine uvedeno je pet novih perkutanih intervencijskih metoda: lijeÄenje nativne koarktacije i rekoarktacije umetanjem stenta, lijeÄenje stenoze pulmonalnih grana umetanjem stenta, perkutano umetanje valvule na pulmonalnu poziciju, dilatacija postojeÄeg stenta te zatvaranje aortopulmonalnih kolaterala u djece s univentrikulskim srcem. Navedeni iskoraci uÄinjeni su kontinuiranim zalaganjem Älanova tima uz potporu i mentorstvo, odnosno kontinuiranu suradnju s inozemnim struÄnjacima iz triju inozemnih ustanova (DeutschesHerzZentrum Muenchen, KinderherzZentrum Linz, Kids Heart Center Budapest). ZakljuÄak: NaÅ” centar stoji uz bok rijetkih centara u Europi koji su u moguÄnosti izvesti navedene procedure. Navedene su aktivnosti rezultirale unaprjeÄenjem kvalitete skrbi na razini RH i temelj su za daljnji planirani rast i razvoj struke u okvirima naÅ”e zemlje.The aim of this report is to show in the past three-year period (2019ā2022):
1)activities of the Reference Center,
2)achieved professional results and the application of new methods, procedures, and improvement of the profession, and
3)scientific and professional cooperation with high-level foreign institutions. Results: The reference center is the only place in the Republic of Croatia that continuously cares for the population of the most vulnerable children with cardiac pathology. It has continuous, 24-hour cardiology, cardiac surgery, anesthesiology, neonatal and intensive care for children with complex congenital and acquired heart diseases. Organ function replacement are also available (ECMO support, LVAD-BiVAD, Berlin-Heart pump, hemodialysis, heart/other organ transplant program) thanks to the teamwork and cooperation of different Departments. Complex cardiac surgeries are performed in children with a clear trend of increasing the number and complexity of surgeries, with low mortality. Today, we can independently treat almost all congenital heart defects in children. About 200 cardiac catheterizations are performed in children annually. More than 40% are interventional procedures with more than 50% of these interventions performed in infancy. In the past three years, five new percutaneous intervention methods have been introduced: stent insertion in native coarctation and in recoarctation, stent insertion in stenosis of the pulmonary branches, percutaneous valve insertion in the pulmonary position, dilatation of the existing stent, and closure of aortopulmonary collaterals in children with a univentricular heart. The steps were made by the continuous efforts of team members with support, mentoring, and continuous cooperation with foreign experts from three foreign institutions (DeutschesHerzZentrum Muenchen, KinderherzZentrum Linz, KidsHeart- Center Budapest). Conclusion: Our center stands alongside the rare centers in Europe that can perform the abovementioned procedures. The activities resulted in the improvement of the quality of care and form the basis for further development of the profession within the framework of our country