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Aim: To assess parents' attitudes toward childhood COVID-19 immunization in Croatia. Methods: In this multicenter cross-sectional study, we collected data from four tertiary care facilities in Zagreb, Split, and Osijek between December 2021 and February 2022. During the visit to the Pediatric Emergency Departments, parents were asked to fill out a highly-structured questionnaire about their attitudes toward COVID- 19 immunization in children. Results: The sample consisted of 872 respondents. A total of 46.3% of respondents were hesitant about vaccinating their child against COVID-19, 35.2% definitely did not intend to vaccinate their child, and 18.5% definitely intended to vaccinate their child. Parents who were themselves vaccinated against COVID-19 were more likely than unvaccinated parents (29.2% and 3.2%, P

Wilson’s disease (WD) is an autosomal recessive genetic disorder that leads to the impairment of cellular copper metabolism. Clinical presentation is heterogeneous, with predominantly hepatic, neurological and psychiatric manifestations. Acute decompensated WD presenting as fulminant liver failure is a life-threatening condition for which liver transplantation is the ultimate treatment. 14-year-old girl presented with acute abdominal pain and peripheral oedema lasting two weeks before onset of abdominal pain. On initial examination, patient was febrile, complaining of periumbilical pain, dyspnea, cough, presenting with anasarca, extensive limb oedema and ascites (gained 17 kg), without encephalopathy. Laboratory evaluation revealed Coombs-negative haemolytic anaemia (Hb 101 g/L, Rtc 125x109/L), thrombocytopenia (79x109/L), leukocytosis (19.27x109/L), mildly elevated inflammatory markers, hypergammaglobulinemia (IgG 21.7g/L) with reduced complement components (C3 0.24g/L, C42.5), marked hypoalbuminemia (>15 g/L), mildly elevated bilirubin (49 µmol/L), slightly elevated liver enzymes (AST 63 U/L, GGT 98 U/L), borderline serum ammonia (52.3 µmol/L), normal ALT and alkaline phosphatase. Abdominal ultrasound showed large amounts of ascites, with mildly enlarged, structurally altered liver (irregular surface, nodular parenchyma) and splenomegaly, with no signs of hepatic vein thrombosis on Color Doppler and MRI. Heart ultrasound was normal. Kayser- Fleischer ring was negative on slit lamp examination. Viral serology (HAV, HBV, HCV, HEV, EBV, CMV, and Parvo B19) was negative as well as autoantibodies (ANA, SMA, LKM, ANCA, anti dsDNA). Serum alpha-1 antitrypsin level was normal. Diagnosis of Wilson’s disease was practically confirmed after demonstrating pronounced cupriuria (17.8 µmol/24h) ; serum ceruloplasmin was low (0.13 g/L) and serum copper levels slightly reduced (10.1 umol/L). Initial treatment was supportive (albumin infusions, vitamin K, fresh frozen plasma, diuretics, lactulose, antibiotics). Liver transplant was considered (on admission MELD score 24, King’s Wilson index 12 ; on day 10 MELD score 18, King’s Wilson index 8). The decision was made to attempt plasma exchange over three days and introduce penicillamine. The patient improved clinically, tolerated penicillamine well and was released subsequently after 34 days of hospitalization and five plasma exchanges overall. Liver synthetic function completely normalized few months later. Genetic analysis of ATP7B gene (H1069Q mutation) confirmed presence of the H1069Q mutation on one allele. We presented a patient suffering from WD in whom the disease presented acutely with development of liver failure with near-normal values of liver enzymes, mild hyperbilirubinemia, markedly impaired synthetic liver function and Coombs-negative haemolytic anaemia. Acute liver failure in WD may be successfully managed with plasma exchange therapy.

Kreatin kinaza (CK) je enzim koji katalizira reverzibilnu reakciju kreatina (Cr) i adenozin trifosfata (ATP) u kojoj nastaju energijom bogat kreatin-fosfat (PCr) i adenozin difosfat (ADP), dok suprotna reakcija prijenosa fosfata između PCr i ADP-a omogućuje brzu regeneraciju ATP-a u tkivima s velikim i promjenjivim energetskim potrebama poput skeletnog i srčanog mišića i mozga. Laboratorijsko određivanje aktivnosti CK je jednostavna, neinvazivna i reproducibilna pretraga. U najvećem broju slučajeva, povišena aktivnost CK u djece popraćena je simptomima i znakovima oštećenja odnosno bolesti mišića. Velik broj različitih nasljednih mišićnih bolesti započinje u djetinjstvu, a poznavanje kliničke slike, obrazaca nasljeđivanja i vrijednosti laboratorijskih parametara poput aktivnosti CK može značajno doprinijeti pravovremenom postavljanju ispravne dijagnoze koja je jedini put k ispravnom zbrinjavanju bolesnika. Uz nasljedne, valja voditi računa i o drugim, stečenim uzrocima porasta aktivnosti CK. U radu je dan presjek autoimunih bolesti dječje dobi za koje je karakteristično zahvaćanje skeletnog mišića, ali i pregled spoznaja dobivenih u novijim istraživanjima koja ukazuju na kompleksnost mogućih uzroka rabdomiolize u djece koji obuhvaćaju infektivne agense, elektrolitne i endokrine poremećaje, nuspojave lijekova, učinke toksičnih tvari, traumu i brojne druge. Uz stanja koja dovode do „lažnog“ povišenja aktivnosti CK u serumu, opisano je i stanje u literaturi poznatije kao idiopatska hiperCKemija, u kojem podležeći uzrok nije moguće otkriti niti iscrpnom dijagnostičkom obradom., The creatine kinase (CK) is an enzyme catalizing the reversible reaction between creatine (Cr) and adenosine triphosphate (ATP) that results in the formation of phosphocreatine (PCr) and adenosine diphosphate. Reverse reaction, one in which creatine phosphate transfers a high-energy phosphate to ADP, allows a timely regeneration of ATP in tissues with high and fluctuating energy demands such as the skeletal and cardiac muscle, as well as the brain. Laboratory measurement of the creatine kinase activity is a simple, non-invasive and easily reproducible test. In most cases, increased activity of creatine kinase in children is accompanied with symptoms and signs indicating muscle damage or disease. A large number of various hereditary muscle diseases begin during childhood. Knowledge of the clinical features, patterns of inheritance and expected values of the laboratory parameters such as CK activity may greatly contribute to a timely and correct diagnosis of such diseases which is considered of the upmost importance for providing quality patient care. Clinicians should also take in account other, acquired causes of increased activity of creatine kinase. This thesis provides an overview of childhood autoimmune disease affecting the skeletal muscle, as well as an overview of scientific knowledge gained in recent studies concerning the complexity of possible causes of rhabdomyolysis in paediatric population. This pertains, but is not limited, to infective agents, electrolyte and endocrine disorders, adverse affects to medication, effects of toxins, trauma and more. Part of the thesis is dedicated to conditions which lead to falsely increased activity of creatine kinase, and there is also a mention of a condition widely known as idiopathic hyperCKemia, where the underlaying cause to elevated creatine kinase activity cannot be found despite extensive diagnostic evaluation.