Atrioventrikularni blok srca trećeg stupnja u djece

Atrioventricular (AV) block is defined as a delay or interruption in the transmission of an impulse from the atria to the ventricles due to an anatomical or functional impairment in the conduction system. The conduction disturbance can be transient or permanent. In third degree AV block, also referred to as complete heart block, there is complete dissociation of the atrial and ventricular activity. Atrioventricular block is considered to be “congenital” when it occurs spontaneously in a fetus or young child. In children, the most common cause of permanent acquired complete AV block is surgery for congenital heart disease. Injury to fetal conduction tissues caused by transpla- cental exposure to maternal autoantibodies related to systemic lupus erythematosus or Sjogren’s syndrome is responsible for 60 to 90 percent of cases of congenital CHB overall1-3. As many as 40 percent of cases of congenital CHB do not present until later in childhood (mean age five to six years). Only rarely do these patients (5 percent) have proven autoimmune etiology. The increased risk of sudden death is associated with the onset of deep bradycardia or ventricular arrhythmia. A routine electrocardiogram is sufficient to diagnose the disease. A 15-year-old girl has been examined at the emergency pediatric outpatient clinic of the University Hospital Centre “Sestre Milosrdnice” for recurrent episodes of presyncope. Physical examination revealed no major devia- tions other than bradycardia. Her vital signs were within the reference range, with the exception of a pulse of about 44 beats per minute. The electrocardiogram showed atrioventricular dissociation consistent with third degree atrioventricular block. The echocardiogram showed a structurally normal heart except for sinus bradycardia. The girl underwent permanent epicardial pacemaker implantation after which there were no symptoms.Atrioventrikularni (AV) blok je definiran kao kašnjenje ili prekid u prijenosu impulsa iz atrija u ven- trikule zbog anatomskih ili funkcionalnih oštećenja u provodnom sustavu srca. Poremećaj provodl- jivosti može biti prolazan ili trajan. U AV bloku trećeg stupnja, koji se još naziva i kompletni srčani blok, dolazi do potpune disocijacije atrijske i ventrikularne aktivnosti. Atrioventrikularni blok se smatra “kongenitalnim” kada se pojavljuje spontano kod fetusa ili malog djeteta. Stečeni permanent- ni kompletni AV blok kod djece najčešće je posljedica kirurške korekcije prirođenih srčanih grešaka. Ozljeda fetalnog provodnog srčanog tkiva uzrokovana transplacentarnom izloženošću majčinim autoantitijelama povezanim sa sistemskim eritematoznim lupusom ili Sjogrenovim sindromom odgovorna je za 60 do 90 posto slučajeva kongenitalnog srčanog bloka1-3. Čak 40 posto slučajeva kongenitalnog srčanog bloka se javlja kasnije u djetinjstvu (prosječna dob od pet do šest godina). Od navedenih samo rijetki pacijenti (5 posto) imaju dokazanu autoimunu etiologiju. Povećan rizik iznenadne smrti se veže uz nastup duboke bradikardije ili ventrikulske aritmije. Za dijagnozu bolesti je dovoljan rutinski elektrokardiogram. Djevojčica od 15 godina je pregledana u hitnoj pedijatrijskoj ambulanti kliničkog bolničkog centra “Sestre Milosrdnice”, zbog ponavljajućih epizoda presinkopa. Tjelesnim pregledom se nije utvrdilo većih odstupanja, osim bradikardije. Njeni vitalni znakovi su bili unutar referentnog raspona, s izuzetkom pulsa koji je iznosio oko 44 otkucaja u minuti. Elektro- kardiogram je pokazao atrioventrikularnu disocijaciju u skladu s atrioventrikularnim blokom trećeg stupnja. Ehokardiogram je izuzev sinusne bradikardije pokazao strukturno normalno srce. Djevojka je podvrgnuta ugradnji trajnog epikardijalnog pacmakera nakon čega više nije imala simptoma i tegoba u budućnosti

Clinical manifestation of late neonatal sepsis – continuous differential diagnostic dilemma

Novorođenačka sepsa kao klinički sindrom jedan je od najčešćih dijagnostičko-terapijskih izazova u novorođenačkoj dobi s relativno visokom stopom smrtnosti. Prema dobi manifestacije, dijeli se na ranu i kasnu sepsu, ovisno o izvorima i mišljenjima raznih stručnjaka. Može se očitovati poremećajem svih organskih sustava i brojnim kasnim komplikacijama te znatnim morbiditetom i mortalitetom. Razdoblje od drugog tjedna života primarni je interes ovog rada jer je u tom periodu (dotad prividno zdrava) novorođenčad otpuštena kući iz rodilišta, adaptirana na svakodnevne zahtjeve ekstrauterinog života, s mogućnošću razvoja niza neinfektivnih stanja i bolesti koji klinički izgledaju kao septičko stanje. Široka diferencijalna dijagnoza takvog stanja može biti ozbiljan problem koji nalaže hitnu reakciju, pravilno i pravodobno liječenje te, naposljetku, zbrinjavanje u adekvatnoj ustanovi ovisno o osnovnoj bolesti. Cilj je ovog pregleda upozoriti na niz neinfektivnih stanja koja se mogu prezentirati kliničkom slikom kasne novorođenačke sepse.Neonatal sepsis as a clinical syndrome is one of the most common challenges in neonatal medicine with a relatively high mortality rate. At the time of clinical manifestation it can be distinguished into early onset and late onset sepsis, highly depending on sources and medical experts opinions.Its multiorgan affection can be associated with major complications and high late morbidity and mortality. The period of the second week of life is the primary interest of this paper, because children who were thought to be healthy are discharged home from the maternity ward, more or less successfully adapted to extrauterine life with the possibility of developing serial noninfectious conditions and diseases that clinically impress as sepsis. Such state can make serious differental diagnostic difficulty and requires quick reaction with timely and appropriate therapy that could be crucial in the management of these patients. Definitive care should be continued in an adequate institution, depending on diagnosis. The aim of this review is pointingb out the wide range of noninfectious conditions that can present as late onset neonatal sepsis

Karakterističan fenotip u djevojčice s Rettovim sindromom i delecijom 25 bp zbog nove mutacije u 4. eksonu (.881_905del25, nm_004992.3) gena MECP2

Rett syndrome is a pervasive developmental disorder with a variable clinical presentation, which is caused by point mutations or large deletions/duplications in the X-linked methyl-CpG-binding protein 2 (MECP2) gene. The aim is to describe variation in the clinical course related to the mutation identifi ed in exon 4 of the MECP2 gene. Retrospective review of data, electroencephalography and treatment was done in a 19-year-old girl previously diagnosed with a MECP2 gene mutation. Born after an uneventful pregnancy, the female patient’s growth and psychomotor development were normal, except for delayed speech. At the age of 3 years, tonic-clonic seizures started and at the age of 3.5 years autistic behavior was observed, followed by rapid mental deterioration, loss of speech and motor skills, with periods of hyperventilation. At the age of 5 years, she showed occasionally „hand-washing“ movements. Extensive neuro-metabolic investigation was nondiagnostic. Genetic analysis revealed a novel 25 bp deletion mutation in exon 4 (c.881_905del25) of the MECP2 gene. Until now, multiple epileptic seizure types, refractory to all antiepileptic polytherapy and with normal video EEG background, have occurred daily. She is spastic and ataxic, but still able to walk slowly with a wide based gait. In this female patient, the onset of symptoms manifested much later than encountered in typical cases of Rett syndrome. Epilepsy with daily frequency is however drug resistant. Unexpectedly, she is still able to walk at the age of 19 years. A genotype-phenotype correlation is suspected.Rettov sindrom (RTT) pervazivni je razvojni poremećaj s različitim kliničkim slikama, a uzrokovan najčešće točkastim mutacijama ili delecijom/duplikacijom metil-CpG-vezanog proteina 2 (MECP2) na genu X. Cilj je opisati različitosti kliničkog tijeka bolesti koje su vezane za nađenu mutaciju na 4. eksonu gena MECP2. Retrospektivna analiza anamnestičkih podataka, electroencefalograma i liječenja kod 19-godišnje djevojke s nalazom mutacije na genu MECP2. Djevojčica je rođena nakon uredne trudnoće kao 4. dijete u obitelji, rast i psihomotorni razvoj su bili uredni, osim zaostatka u razvoju govora. Sa 3 godine započeli su toničko-klonički grčevi, a sa 3,5 godine uočava se autistično ponašanje, nakon čega je uslijedila mentalna deterioracija s prestankon govora, smetnjama motorike i povremenim kratkotrajnim javljanjima hiperventilacije. Sa 5 godina povremeno su se uočavali pokreti „pranja ruku“. Velikim opsegom neurometaboličkih pretraga nije se našao uzrok smetnjama. Genetičkom analizom nađena je delecija 25 bp kao jedna od novih mutacija u 4. eksonu (c.881_905del25) gena MECP2. Uslijedili su razni oblici epileptičkih napadaja koji su bili tvrdokorni na antiepileptičku terapiju, uz urednu osnovnu aktivnost na video-elektroencefalogramu. Djevojka ima povišen mišićni tonus, ataktičan hod i može samostalno hodati na širokoj osnovi. U ove djevojke simptomi su počeli kasnije nego u tipičnim slučajevima Rettovog sindroma. Epileptički napadaji su svakodnevni i tvrdokorni na terapiju, a 19-godišnja djevojka još hoda, a najvjerojatniji razlog je međusobna povezanost genotipa i fenotipa

SATISFACTION OF YOUNG DOCTORS IN CROATIA: ARE WE HEADING IN THE RIGHT DIRECTION?

Uvod: Zadovoljstvo mladih liječnika vezano je ponajprije uz edukaciju tijekom specijalizacije i uvjetima rada. S obzirom na znatan odljev liječnika iz Republike Hrvatske, cilj istraživanja bio je evaluirati zadovoljstvo mladih liječnika. Metode: Povjerenstvo za mlade liječnike Hrvatske liječničke komore izradilo je anketu o zadovoljstvu mladih liječnika. Anketa je bila dostupna za ispunjavanje u razdoblju od 1. 2. do 20. 3. 2016. godine putem interneta. Rezultati: Anketu je ispunila 1531 osoba, srednje dobi 32 godine (raspon 29 – 35), 67% ženskog spola. Pedeset osam posto ispitanika napustilo bi Republiku Hrvatsku ako bi im se pružila prilika. Glavni razlozi odlaska jesu bolji uvjeti rada (74%), uređenost zdravstvenog sustava (64%) i bolja plaća (64%). U budućnosti svoj profesionalni status i razvoj ostankom ocjenjuju većinom nepromijenjen (44%). Zaključak: Rezultati pokazuju visoku razinu nezadovoljstva zdravstvenim sustavom, segmentima specijalističkog usavršavanja i ulogom mentora. Analizom čimbenika koji utječu na zadovoljstvo mladih liječnika omogućuju se razrada i donošenje mjera kojima će se navedeni trend umanjiti.Introduction: The satisfaction of junior medical doctors is primarily associated with the training they receive during the residency period and working conditions. Given a considerable brain-drain of medical doctors from the Republic of Croatia, the present research focused on the evaluation of the satisfaction of junior medical doctors. Methodology: The Junior Doctors Committee of the Croatian Medical Chamber prepared a questionnaire on the satisfaction of junior doctors. The relevant questionnaire was available for filling in online in the period from February 1 to March 20, 2016. Results: The questionnaire was completed by 1,531 persons aged between 29 and 35 (mean age 32) of which 67% were females. A 58% of respondents would leave the Republic of Croatia if offered an opportunity. The main reasons for leaving the country include better working conditions (74%), well-regulated healthcare system (64%) and higher wages (64%). In case of staying in the country, their future professional status and development would remain unaltered (44%). Conclusion: The questionnaire results show a high level of dissatisfaction with the healthcare system, some segments of the specialist training and the mentor role. The analysis of factors affecting the satisfaction of junior doctors will facilitate the preparation and adoption of measures aiming to mitigate the above-mentioned trend

Zadovoljstvo mladih liječnika u Republici Hrvatskoj: idemo li u pravom smjeru? [Satisfaction of young doctors in Croatia: are we heading in the right direction?]

Introduction: The satisfaction of junior medical doctors is primarily associated with the training they receive during the residency period and working conditions. Given a considerable brain-drain of medical doctors from the Republic of Croatia, the present research focused on the evaluation of the satisfaction of junior medical doctors. Methodology: The Junior Doctors Committee of the Croatian Medical Chamber prepared a questionnaire on the satisfaction of junior doctors. The relevant questionnaire was available for filling in online in the period from February 1 to March 20, 2016. Results: The questionnaire was completed by 1,531 persons aged between 29 and 35 (mean age 32) of which 67% were females. A 58% of respondents would leave the Republic of Croatia if offered an opportunity. The main reasons for leaving the country include better working conditions (74%), well-regulated healthcare system (64%) and higher wages (64%). In case of staying in the country, their future professional status and development would remain unaltered (44%). Conclusion: The questionnaire results show a high level of dissatisfaction with the healthcare system, some segments of the specialist training and the mentor role. The analysis of factors affecting the satisfaction of junior doctors will facilitate the preparation and adoption of measures aiming to mitigate the above-mentioned trend

Imunizacija djece s kongenitalnim srčanim grješkama – klinički i kritički osvrt [Immunisation of children with congenital heart defects – clinical and critical overview]

Numeruous scientific articles indicate the benefit of immunisation when dealing with children with congenital heart defects. High possibility of gaining infections can be related to compromised cardiopulmonary status, changed pressures in pulmonary circulation (including pulmonary hypertension) as well as ventilation-perfusion mismatch. Many children with heart anomalies within heterotaxy syndrome have functional hyposplenism with high susceptibilty to infections. Children with chromosomopathies and many other syndromes have a high frequency of immunodeficiency states of various intensity, alongside hemodynamically significant, often complex heart defects and numerous anomalies of other organs. Cardiopulmonary bypass and ECMO device are inevitably used for operative corrections and palliative procedures, however, main issue is their tendency to decrease the circulating antibodies that have occurred during active immunisation, thereby compromising the acquired immune system. Protecting such patients by systematic immunisation of children and their family members should be the main interest of scientific community. This paper gives a systematic overview of the above mentioned controversy

Frequent accelerated idioventricular rhythm in an otherwise healthy child: a case report and review of literature

Abstract Background Accelerated idioventricular rhythm (AIVR) is a wide QRS complex dysrhythmia that, as far as pediatric population is concerned, occurs mostly in children with underlying systemic or heart disease. Its clinical course is thought to be typically benign in otherwise healthy children and treatment to be completely needless. Existing guidelines/recommendations are based entirely on cases that had low daily burden of AIVR, and those referring to treatment itself are very unspecific. Pharmacologic therapy has been mostly unsuccessful and catheter ablation as a way of treatment has been only sporadically reported. This article is a case report with a literature review that aims to practically separate the age groups into newborn and older children and to emphasize the different clinical outcomes of children with occasional and frequent AIVR. There are only a few cases so far describing undesirable outcomes of this condition, and most of these patients had high daily burden of AIVR. To be more specific, among 38 healthy children older than 1 year reported in total, 6 had undesirable outcomes, short-term in terms of developing malignant arrhythmia or long-term in terms of developing cardiomyopathy/heart failure. Case presentation An 11-year-old boy had been referred to our center for a workup of incidentally discovered wide-complex arrhythmia. He was asymptomatic, with no underlying cardiac or systemic diseases. Continuous heart rate monitoring detected AIVR during most time of monitoring. In 24-h Holter-ECG, wide QRS complexes accounted for 73%. With parental consent, we conducted an electrophysiological study accompanied by radiofrequent ablation of ectopic focus, which lead to an instantaneous sinus rhythm that continued during the entire follow-up. Conclusion AIVR is a rare dysrhythmia in the pediatric population, typically considered benign. Nevertheless, more than a few cases evidence its harmful potential, short-term in terms of developing malignant arrhythmia or long-term in terms of developing cardiomyopathy. Gathering more knowledge and experience along with conducting further studies is essential for the enhancement of understanding this condition, and selecting potentially vulnerable patients as well as their treatment

Burnout among Croatian physicians: a cross- sectional national survey

Aim: To investigate the prevalence of burnout syndrome among physicians of all specialties, including residents and non-specialists, on a national level in Croatia. Methods: This cross- sectional study, conducted in October 2017, used anonymous online survey based on the Maslach Burnout Inventory Human Services Survey. The Croatian version of the inventory was assessed for acceptability, factorial validity, and reliability. Key dimensions of burnout – emotional exhaustion, depersonalization, and lack of personal accomplishment were assessed. Respodents scoring high for emotional exhaustion or depersonalization were defined as burned-out. Results: The response rate was 18% (2557/14 427). Respondents’ median age was 41 years (range 25-80), and 68% (1737/2557) were women. Good sampling adequacy and scale reliability were confirmed. Factorial validity suggested the presence of three overall factors, and no items were eliminated. Sixty-three percent of physicians were burned- out. High score on emotional exhaustion, depersonalization, and reduced personal accomplishment were found in 58%, 29%, and 52% of respondents, respectively. As many as 16% of the respondents simultaneously experienced high levels of all three burnout dimensions. Multivariate logistic regression analysis revealed that residents and physicians in tertiary or primary care were at an increased risk of burnout, while physicians working in institutes were at a decreased risk. Conclusion: Active national measures are needed to reduce the high prevalence of burnout among Croatian physicians