Children's Hospital Zagreb

Ovaj rad analizira utjecaj korištenja digitalne tehnologije na mentalno zdravlje djece i adolescenata, s naglaskom na razvojne, emocionalne i bihevioralne posljedice pretjeranog izlaganja ekranima. Istraživanja pokazuju da kod predškolske djece vrijeme provedeno pred ekranom može usporiti sazrijevanje bijele moždane tvari, te dovesti do kašnjenja u socijalnoj komunikaciji, regulaciji emocija i interakciji s vršnjacima. Kod školske djece pretjerano korištenje tehnologije povezano je s problemima pažnje i koncentracije, slabijim školskim uspjehom, smanjenom tjelesnom aktivnošću te povećanim rizikom od anksioznosti, depresivnih simptoma i narušenog samopoštovanja. Korištenje društvenih mreža nosi dodatne rizike, uključujući izloženost neprimjerenom sadržaju, cyberbullying i prijetnje sigurnosti na internetu. Kod adolescenata intenzivna upotreba digitalnih platformi povezana je s nižom životnom zadovoljstvom, razvojem ovisničkog ponašanja i simptomima psihijatrijskih poremećaja. Pretjerano korištenje digitalnih uređaja aktivira dopaminergički sustav nagrade, što može pridonijeti razvoju bihevioralnih ovisnosti, uključujući poremećaj igranja videoigara. Prisutan je i negativan utjecaj roditeljskog korištenja tehnologije na formiranje privrženosti i emocionalnu sigurnost djece. Postoji potreba za preventivnim mjerama, ograničenjem vremena koje djeca provode pred ekranom i promicanjem zdravih razvojnih aktivnosti, radi zaštite mentalnog zdravlja djece i adolescenata.

We present the report of disease in a child with severe restrictive cardiomyopathy (RCM) resulting in the development of veno-occlusive pulmonary arterial hypertension (PAH) and cardiopulmonary insufficiency with NYHA II-III. Due to borderline criteria for heart transplantation (HT), it was difficult to find justification for the procedure, although it was the only way to save the child’s life. The boy was monitored for hepatomegaly one year before admission. At the age of 11, significant dilatation in both atria, accompanied by a reduced cavity of both ventricles led to the recognition that the clinical symptoms were attributed to restrictive cardiomyopathy (RCM) with preserved left ventricular ejection fraction (LVpEF – LV with preserved EF). Because of the evidence suggesting that it is a secondary development of veno-occlusive PAH, before the HT decision it was required to clarify distinguish from reactive PH, in accordance with the latest 6WSPH criteria (6th World Symposium on pulmonary hypertension) [1]. Pulmonary hypertension is considered when the mean pulmonary artery pressure (mPA) is >20 mmHg, transcapillary gradient (PAWP) > 15 mmHg and pulmonary vascular resistance (PVR) ≥ 3 iWJ. Since the patient did not meet the criteria for passive (isolated) precapillary PAH (mPA>20 mmHg, PAWP >15 mmHg and PVR < 3iWJ) with preserved LVEF /IpcPH/LVpEF=Isolated precapillary PH with LVpEF/, but according to the same criteria, he had reactive, or combined veno-occlusive pulmonary hypertension (PVR ≥ 3iWJ) /CpcPH/LVpEF= Combined precapillary PH with LV preserved EF)/. Diagnosis indicated that it was a contraindication for HT. This position is valid only if the discussion about possible reversible changes in PAS (Pulmonary artery small – the former name of arteriole) are ignored. Aware of the fact that HT is risky, but also of the probability that changes caused by the Kitajew phenomenon are reversible [2] contrary to precapillary PH caused by CHD (Euler Ljiljestrand fenomen) [3], we decided to proceed with HT. We are predicting a difficult postoperative course but which can be overcome with adequate measures (strict supervision of the Frank-Starling law) [4] with mechanical and pharmacology support. Encouraged by reports from the literature indicating a significantly higher incidence of passive postcapillary than reactive pulmonary hypertension as well as rapid regression of pulmonary hypertension after elimination of the cause of PAS self perpetuation [5-7], we decided on HT as the only perspective for the survival of a severely ill child. Our arguments also included a discussion on the irreversibility of PAH before the development of Eisenman’s syndrome (ES) (“gray area”, 6-8 iWJ) [8,9]. HT decision despite the inconsistency of the criteria for defining pulmonary hypertension and the type of pulmonary vascular resistance according to the previous international criteria’s [10-13], including the 6WSPH criteria [1,8,14,15]. Our arguments were accepted by the EUROTRANSPLANT committee. A HT was successfully performed and boy was discharged home with good follow up more than > 10 years in good condition (NYHA I).

Uvod: Wilsonova bolest je autosomno recesivni poremećaj metabolizma bakra uzrokovan mutacijama gena ATP7B koji dovodi do smanjenog izlučivanja bakra iz hepatocita u žuč i njegova nakupljanja, prvenstveno u jetri i središnjem živčanom sustavu. Klinička slika uključuje jetrene, neurološke i psihijatrijske manifestacije. U dječjoj dobi je najčešća jetrena prezentacija, često u obliku asimptomatskog povišenja aminotransferaza. Prikaz bolesnika: Djevojčica u dobi od šest godina, urednog rasta i razvoja, bez tegoba, dolazi na obradu povišenih aminotransferaza. Povišene vrijednosti jetrenih enzima prvi su put zabilježene u tijeku respiratorne infekcije, a u kontrolnim nalazima tijekom 7 mjeseci se prati porast alanin aminotransferaze (ALT) (maksimalno 12 puta iznad gornje granice referentnog intervala) i blaže povišene aspartat aminotransferaza (AST) i gama-glutamil transferaza (GGT) uz uredne parametre sintetske funkcije jetre i bilirubin. Diferencijalno-dijagnostičkom obradom isključeni su infektivni i autoimuni uzroci, a vrijednosti lipidograma, alfa-1 antitripsinai i hormona štitnjače bili su uredni. Ceruloplazmin je bio uredan, ali je izlučivanje bakra u 24-satnom urinu bilo povišeno, a zatim je i nalaz penicilaminskog testa bio patološki pa je učinjena biopsija jetre čiji nalaz je utvrdio makrovezikularnu steatozu i blagu fibrozu uz značajno povišenu koncentraciju bakra u suhoj tvari jetrenog tkiva (399 µg/g). Zaključno je postavljena dijagnoza Wilsonove bolesti i liječenje je započeto penicilaminom u postupno rastućim dozama, uz vitamin B6 i smanjeni unos bakra. Prati se povoljan biokemijski odgovor. Molekularno-genetskom analizom utvrđene su 2 heterozigotne mutacije gena ATP7B te je potvrđena dijagnoza. Zaključak: Ovaj prikaz naglašava važnost sustavne obrade perzistentno povišenih aminotransferaza čak i u djece bez simptoma kako bi se na vrijeme dijagnosticirale lječive bolesti kao što je Wilsonova bolest.

Introduction: Acute cerebellitis is a rare inflammatory disease with a variable course, from a benign self-limiting condition with minimal symptoms to fulminant cerebellitis associated with a high risk of morbidity and mortality. The etiology of the disease is most often infectious or post-infectious, and rotavirus has been described as one of the possible causes. Complications of the disease are the result of acute cerebellar edema that compresses the structures of the posterior cranial fossa and brainstem and leads to the development of obstructive hydrocephalus, intracranial hypertension with impaired consciousness up to status epilepticus, and a fatal outcome due to herniation of the cerebellar tonsils. Children usually have generalized symptoms with nausea, headache, rapid impairment of consciousness, and later mutism. Pleocytosis is present in the cerebrospinal fluid, and brain imaging studies show progressive, significant cerebellar atrophy. If not treated promptly, death can occur. Case report: We present a 6-year-old girl admitted to the Children's Hospital Zagreb with acute ataxia and somnolence. On admission, the Glasgow Coma Scale (GCS) was 13/15. The evening before admission, she vomited 3-4 times, had dyskinesias, was irritable, and made unusual noises. Initial laboratory findings, electroencephalogram, and magnetic resonance imaging (MRI) were normal. Within 12 hours, she developed progressive impairment of consciousness to coma (GCS 5), was intubated, and transferred to the Intensive Care Unit. Repeated MRI scans showed the development of diffuse cerebellitis, hypertensive hydrocephalus, and encephalitis, which were treated with acyclovir, ceftriaxone, intravenous immunoglobulins, and corticosteroids, and external cerebrospinal fluid drainage. The clinical picture was further complicated by the occurrence of recurrent epileptic seizures and the development of status epilepticus, which were treated with levetiracetam and midazolam. After three weeks, all of the above changes regressed. After two months, she was discharged from the hospital for inpatient rehabilitation with a very slow recovery and dominant mutism. A slight improvement in neurological status was gradually noted (GCS 9). Five months after the onset of the disease, with home physical therapy, the girl made significant progress, walking and speaking (GCS 14). She recovered completely. Conclusion: The presented case of fulminant cerebellitis as a rare complication of a common infection emphasizes the importance of early recognition of the disease, repeated neuroradiological examinations, and timely and intensive therapy. Despite the severe initial clinical picture and numerous complications, with adequate treatment and rehabilitation, a full recovery is possible.

Introduction. Good preoperative preparation as one of the most important predictors of overall patient satisfaction increases cooperation and their desire to continue treatment.Aim. The aim of the research was to examine the respondents’ satisfaction with the pre-operative preparation and post-operative health care of patients treated at the Surgery Clinic of the “Sveti Duh” Clinical Hospital in Zagreb, Croatia.Methods. The measuring instrument of this research was an anonymous questionnaire created for the purposes of the research itself. The survey included 60 respondents with an average age of 51.Results. The results of the conducted research showed that the majority of respondents were satisfied with the healthcare provided, with 53 (88.3%) responding that they were satisfied, and 54 (90%) of the respondents answered that they could confidently to contact the health workers of the clinic, who showed concern and understanding for their problem. An important factor in postoperative satisfaction was postoperative pain. After the procedure, the median pain (on a scale of 0 to 5) was 4, and immediately after the subjects started to feel pain and asked the nurse/technician for analgesic, 35 (58%) patients received it. A total of 44 (73%) patients expressed satisfaction with the speed of the nurses’ intervention after they reported feeling pain.Conclusion. A survey conducted at the Surgery Clinic of the “Sveti Duh” Clinical Hospital showed that the largest number of respondents, a total of 53 (88.3%) were satisfied with the healthcare provided during hospitalization. It was observed that women were more satisfied than men, and that respondents aged 46 to 60 reported significantly higher levels of satisfaction compared to those aged up to 45.

The resilience of sarcomas, tumors characterized by resistance to therapy and high metastatic potential, is largely driven by the unique characteristics of a small population known as cancer stem cells (CSC). Although ascorbic acid (AA) and its oxidized form, dehydroascorbic acid (DHA), have shown potential for selectively targeting cancer cells, their effects on sarcoma CSCs remain insufficiently explored. Still, recent research indicates that AA can affect the specific characteristics of CSC and lead to their cytotoxicity. To investigate the sensitivity of sarcoma CSCs to ascorbate, CSCs were isolated from six sarcoma patient-derived samples using a sphere assay, and their stem identity was evaluated through gene expression profiling and dye-efflux assays. Cytotoxicity testing of AA and DHA showed that DHA has a selective cytotoxic effect on cancer stem cells. The presence of basic fibroblast growth factor (bFGF), which is commonly used to support the self-renewal of CSCs, had an influence on the cytotoxic effect of DHA. To evaluate the difference in the effect of AA and DHA, a seven-day treatment of CSCs with these forms of ascorbate was performed. The gene expression analysis revealed that DHA in the presence of bFGF had a stronger impact on response to oxidative stress and cellular metabolism. Also, investigation of somatic mutations of oncogenes and tumor suppressors revealed that in liposarcoma and rhabdomyosarcoma, there are mutations that induce proliferative signals. These proliferative signals, joined with bFGF in the presence of DHA, do not lead to proliferation but instead cause cell death.