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Publikacije (40)

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Emina Vukas-Salihbegović, Tarik Jarkoč, S. Užičanin, Lejla Prnjavorac, O. Abud, Berina Omanović Kulović

Background: Guillain-Barré syndrome (GBS) is an acute, immune-mediated post-infectious polyradiculoneuropathy usually presenting with symmetrical ascending weakness, diminished deep tendon reflexes, and non-specific sensory symptoms. GBS is in essence an autoimmune disorder, and the underlying mechanism is thought to result from so-called molecular mimicry. This hypothesis is further supported by approximately 2/3 of the patients having a preceding infection. In most cases, the infectious trigger occurs in the gastrointestinal or respiratory tract, with the disease manifesting within four weeks. Even though it most commonly affects children aged 1 to 5 years old, there are rare cases reported in neonates and infants. Case Presentation: We report a case of a 6-month-old infant with Guillain-Barré syndrome following a respiratory infection. The diagnosis was confirmed through cerebrospinal fluid analysis, EMNG, spine MRI, and clinical assessment. Positive human herpes virus 6 (HHV-6) in cerebrospinal fluid suggested a potential infectious trigger. The infant was treated with intravenous immunoglobulin and ganciclovir, requiring intensive care and mechanical ventilation. Recovery involved gradual neurological improvement and restored motor function over 30 days. Conclusion: GBS is a rare disorder, especially in children and requires multidisciplinary management to prevent complications from occurring and thereby improve the prognosis of patients. Upon arrival at the emergency department, all patients should be carefully evaluated, looking for autonomic and respiratory dysfunction signs. Generally, pediatric patients have a better prognosis compared to adults. Initiation of treatment in the early stages of the disease leads to a faster recovery and consequently fewer sequelae.

Tarik Jarkoč, A. Čengić, V. Selmanović, Amila Hadžimuratović, E. Vukas, S. Užičanin, Zinka Huseinbegovic, Mirna Sarajlic

Background: Benign acute childhood myositis (BACM) is a rare complication of viral URTIs, usually occurring in winter. It is characterized by acute onset of bilateral calf pain and difficulty/refusal to walk. A prodromal phase precedes these manifestations, consisting of catarrhal and constitutional symptoms. These are associated with increased muscle-specific enzymes, usually normal inflammatory parameters, and leukopenia. Objective: Our study aimed to define the demographic, clinical, and laboratory characteristics of BACM patients and determine the etiology leading to their development. Methods: Medical charts for patients diagnosed with BACM from October to April 2023/2024 at the Pediatric Clinic Clinical Center University of Sarajevo, Department of Allergology, Rheumatology, and Immunology were reviewed retrospectively. Relevant medical information was collected for 20 patients. Statistical analysis was done in Microsoft Excel 2013. Results: Demographic analysis demonstrated male predominance (75%), with primarily school-aged children affected (median age 8.36). Most cases occurred in winter (60%). The majority of patients presented with bilateral calf pain (100%), difficulty walking (90%), and fever (100%). All cases demonstrated increased CK levels, with median values of 3779 U/L; a notable number had leukopenia (70%). The most commonly isolated pathogen was Influenza B virus (75%). Conclusion: BACM is relatively rare but presents acutely and leads to plenty of distress for both patients and their parents. A child with coryzal symptoms complicated by bilateral calf pain or difficulty/refusal to walk and an increase in CK levels should raise suspicion of BACM. The condition is self-limiting and usually resolves without complications.

Background: Tuberous Sclerosis Complex (TSC) is an autosomal dominant genetic disorder and involves multiple organs, intellectual disability and epilepsy. Mutations in TSC1 and TSC2 genes are responsible for the molecular disease mechanism. Objective: The aim is to determine molecular background of a patient with a suspicion of TSC. Case presentation: In this case report, we describe a seven year old patient with the clinical manifestation of TSC that includes supratentorial changes, subependymal hamartomas and angifibromas in the facial area. Besides the brain and skin changes, no other TSC characteristics were observed. The patient was referred to molecular genetic testing using Next Generation Sequencing (NGS). Results: Clinical exome sequencing revealed intronic TSC2 c.4849+2T>G variant. The variant was confirmed using Sanger sequencing on the subject. However, the variant was not detected in the parents, which indicated that it arose de-novo. The RegSNP-intron, Mutation Taster and Human Splicing Finder were used as a bioinformatic tools to predict the possible effect on protein. Using bioinformatic tools, it was determined that the variant is possibly damaging to protein. Conclusion: This data suggest that observed splicing intronic variant could be the cause of TSC in this pediatric patient.

F. Catibusic, S. Užičanin, Emina Salihbegovic, Zinka Huseinbegovic

Background: Levetiracetam (LEV) is a broad spectrum second-generation antiepileptic drug (AED). Objective: The objective of the study was to investigate the efficacy and safety of levetiracetam for childhood epilepsies. Methods: This is single, tertiary centre observational, prospective study, that included paediatric patients who were treated with levetiracetam at Paediatric hospital University Clinical Centre Sarajevo, during the period of 15 years (2008-2022). Inclusion criteria were: paediatric patients age > 1 month, diagnosed with epilepsy according to International League Against Epilepsy. After the introduction of levetiracetam, each patient has been followed up at least 12 months. According to the outcome the patients were divided into 5 groups: seizure reduction >50%, seizure reduction <50%, complete seizure freedom, the same number of seizures and increased number of seizures. From these groups two intergroups have been formed: responders (seizure reduction >50% and complete seizure freedom) and non-responders (seizure reduction <50%, the same number of seizures and increased number of seizures). Results The study enrolled 259 patients (141 female and 118 male), with mean age 7 years (3,0–12.0). Comorbidities were present at 129/259 (49.8%) patients. After 12 months of treatment, 25/259 (9.7%) patients had seizure reduction >50%, 30/259 (11.6%) patients had seizure reduction <50%, 154/259 (56.5%) patients had achieved seizure freedom, 31/259 (12%) patients had same number of seizures, while 19/259 (7.3%) patients had increased number of seizures. Seizure frequency between responders and non-responders, before treatment and after 12 months of treatment was statistically significant (p<0.001). Discussion: Non responders had the best outcome with ditherapy (30/79; 38%), while responders had the best outcome with monotherapy (161/180;89.4%). Conclusion: Levetiracetam is efficient antiepileptic drug for different types of epilepsies in childhood, used as mono, di or polytherapy.

Background: Acute kidney injury (AKI) is the result of various causes and is associated with significant morbidity and mortality as well as long-term renal sequelae in pediatric patients. Objectives: The aim of the study is to determine the causes of AKI in pediatric patients who needed renal replacement therapy (RRT) and were admitted to the Pediatric and Neonatal Intensive Care Unit (PICU and NICU) at the Pediatric Clinic, University Clinical Center Sarajevo (UCCS). Methods: Our research included 81 children with AKI who needed RRT. We used the Kidney Disease: Improving Global Outcomes (KDIGO) criteria to define AKI. Severe acute kidney injury was defined as stage 2 or 3 of AKI when plasma creatinine level ≥2 times the baseline level or urine output <0.5 ml per kilogram of body weight per hour for ≥12 hours. Other laboratory findings and imaging tests were made depending on their primary disease that led to the AKI and its complications. Results: Our research analyzed 81 children with AKI who needed RRT 38 girls and 43 boys ages from birth to 18 years. Mean age of presentation was 6.28 years. Male female ratio in this study was 1.1:1. Non-olyguric AKI was diagnosed in 12 (14.8%) of children with AKI, while the rest 69 (85.2%) had the olyguric type. Patients with AKI were analyzed after a rough division on prerenal in 57 (70.4%) children, intrarenal in 23 (28.4%) and post-renal in 1 (1.2%) patient. Conclusion: As the AKI plays a key role in the mortality and morbidity in pediatric patients, especially in infants, it is important to recognise and treatment on time different etiologies of this serious condition. Some causes of AKI in our country can be prevented by better organization of primary and secondary health care, which would also reduce mortality and morbidity from AKI.

Feriha Hadžagić Ćatibušić, S. Užičanin, D. Bulja, Emira Gasal Gvozdenović

Aim To assess hand function and explore the relationship between hand function and neuroimaging findings in children with unilateral spastic cerebral palsy (US CP). Methods Hand function was assessed using Manual Ability Classification System (MACS, I-V). Brain lesions were divided into five groups: brain maldevelopment (MAL), periventricular white matter lesions (PV WM), cortical/subcortical gray matter lesions (C/SC GM), nonspecific and normal findings. Results Of 114 children with US CP (77 boys and 37 girls), 56 were with right-sided and 58 with left-sided involvement. MACS I was found in 49 (42.9%), MACS II in 19 (16.7%), MACS III in 19 (16.7 %), MACS IV in 9 (7.9%) and MACS V in 18 (15.8%) children (p=0.002). Computed tomography (CT) as the only neuroimaging has been done in 18 (15.8%), magnetic resonance imaging (MRI) at 94 (82.5%) children, whereas 2 (1.7%) children had neither CT nor MRI. The CT showed PV WM in eight (44.4%), C/SC GM lesions in 6 (33.3%), and normal findings in 4 (22.2%) children (p=0.709). The MRI showed MAL in 8 ( 8.5%), PV WM in 46 (48.9%), C/SC GM in 28 (29.8%), miscellaneous in 2 (2.1%), and normal finding in 10 (10.7%) children (p=0.0001). Mild hand dysfunction (MACS I and II) was assessed in 68 (59.7%) children, of which 33 had PV WM lesions (p=0.001). Conclusion Mild hand dysfunction in children with US CP has been significantly associated with PV WM lesions. The type of brain lesion may help to identify its timing and predict the level of hand dysfunction.

Introduction: Epilepsy is one of the most common neurological diseases in childhood and adolescence. Carbamazepine (CBZ) and valproate (VPA) have been widely used as the first generation of antiepileptic drugs (AED). Aim: The aim of the study has been to evaluate and compare the effect of CBZ and VPA monotherapy on aspartate aminotransferase (AST), alanine aminotransferase (ALT) and gamma-glutamyltransferase (GGT) serum levels in children. Material and methods: The study has included 100 patients (boys 57/girls 43, age range 1 to 18 years), who have been treated with CBZ or VPA, as initial monotherapy, for at least 12 months. Patients with liver lesions or patients who have been treated with other drugs have been excluded from the study. The initial serum enzyme levels (AST, ALT and GGT) and after 12 months of treatment have been compared. Results: 53/100 (53%) patients have been treated with CBZ and 47/100 (47%) patients have been treated with VPA.The initial level of enzymes were within the referece range. After one year-long treatment AST was elevated at 4/53 (7.5%) CBZ patients and 9/47 (19.15%) VPA patients (x2 test =3.965, p<0.05). ALT was elevated at 5/53 (9.4%) CBZ patients and 9/47 (19.15%) VPA patients (x2 test =6.953, p<0.05). GGT was elevated at 18/53 (34%) CBZ patients and 7/47 (14.9%) VPA patients (x2 test =4.831, p<0.05). Conclusion: The levels of enzymes AST and ALT have been elevated statistically significant in VPA group and GGT in CBZ group.

Introduction: Unilateral spastic cerebral palsy (US CP) is the second most common subtype of cerebral palsy. Aim: The aim of the study was to analyze neuroimaging findings in children with unilateral spastic cerebral palsy. Material and methods: The study was hospital based, which has included 106 patients with US CP (boys 72/girls 34, term 82/preterm 24). Neuroimaging findings were classified into 5 groups: Brain maldevelopment, predominant white matter injury, predominant gray matter injury, non specific findings and normal neuroimaging findings. Results: Predominant white matter lesions where the most frequent (48/106,45.28%; term 35/preterm 13), without statistically significant difference between term and preterm born children (x2=0.4357; p=0.490517). Predominant gray matter lesions had 32/106 children, 30.19%; (term 25/preterm 7, without statistically significant difference between term and preterm born children (x2=0.902; p=0.9862). Brain malformations had 10/106 children, 9.43%, and all of them were term born. Other finding had 2/106 children, 1.89%, both of them were term born. Normal neuroimaging findings were present in14/106 patients (13.21%). Conclusion: Neuroimaging may help to understand morphological background of motor impairment in children with US CP. Periventricular white matter lesions were the most frequent, then gray matter lesions.

Objective: The aim of this study was to analyze prognostic indicators for mortality in neonates with seizures in a level III Neonatal Intensive Care Unit (NICU). Patients and methods: A cohort of 100 neonates with clinically manifested seizures hospitalized in the NICU during 4 years period was prospectively monitored for the first year of life. The cohort consisted of 33 preterm and 67 full-term babies with 60 male and 40 female infants. Results: The mortality rate in the first year of life of infants with seizures in the neonatal period was 23%. The most common cause of seizures was birth asphyxia for full-term infants and intra-periventricular hemorrhage for preterm infants. Death was more common in pre-term than term infants (p <0,005). Simple regression demonstrated statistically significant associations between death in the first year of life and a cluster of highly associated variables: resuscitation (p<0, 01), mechanical ventilation (p<0,01) and asphyxia (p<0,05). This cluster of variables significantly correlates with: gestational age (p<0, 05), birth weight (p<0, 05) and intracranial hemorrhage (p<0, 05). Conclusion: In this cohort of neonates with seizures asphyxia requiring neonatal resuscitation was the primary risk factor for death.

Introduction: Neuroimaging procedures and electroencephalography (EEG) are basic parts of investigation of patients with epilepsies. Aim: The aim is to try to assess relationship between bilaterally localized brain lesions found in routine management of children with newly diagnosed epilepsy and their interictal EEG findings. Patients and methods: Total amount of 68 patients filled criteria for inclusion in the study that was performed at Neuropediatrics Department, Pediatric Hospital, University Clinical Center Sarajevo, or its outpatient clinic. There were 33 girls (48,5%) and 35 boys (51,5%). Average age at diagnosis of epilepsy was 3,5 years. Results: Both neurological and neuropsychological examination in the moment of making diagnosis of epilepsy was normal in 27 (39,7%) patients, and showed some kind of delay or other neurological finding in 41 (60,3%). Brain MRI showed lesions that can be related to antenatal or perinatal events in most of the patients (ventricular dilation in 30,9%, delayed myelination and post-hypoxic changes in 27,9%). More than half of patients (55,9%) showed bilateral interictal epileptiform discharges on their EEGs, and further 14,7% had other kinds of bilateral abnormalities. Frequency of bilateral epileptic discharges showed statistically significant predominance on level of p<0,05. Cross tabulation between specific types of bilateral brain MRI lesions and EEG finding did not reveal significant type of EEG for assessed brain lesions. Conclusion: We conclude that there exists relationship between bilaterally localized brain MRI lesions and interictal bilateral epileptiform or nonspecific EEG findings in children with newly diagnosed epilepsies. These data are suggesting that in cases when they do not correlate there is a need for further investigation of seizure etiology.

Introduction: Neuroprotective benefit of therapeutic hypothermia in term newborns with hypoxic-ischemic encephalopathy (HIE) was assessed by analyzing survival and neurodevelopmental outcome of neonates subjected to this procedure. Material and methods: Newborns with gestational age > 36 weeks and < 6 hours of age with moderate to severe asphyxial encephalopathy underwent cooling protocol at a temperature of 33.5 °C for 72 hours and rewarming period of 6 hours. Outcome measures assessed were death and neurodevelopmental characteristics, which were compared at the different age using ASQ-3. Twenty-five children were assessed at age 3-6, 12-18 and 24-36 months. Median gestational age was 40 weeks, birth weight 3470 g, Apgar score 2/4 and pH on admission to the hospital 7.02. Four (16%) children died. Results: At the first assessment developmental categories of communication were normal in 78.9%, problem solving in 63.2%, personal-social in 68.4%, gross motor in 68.4%, and fine motor in 42.1% with a high need of retesting in this area. Second assessment was done in 17 patients: developmental categories of communication normal in 58.8%, problem solving in 70.6%, personal-social in 64.7%, gross motor in 64.7%, and fine motor in 35.3%. Third evaluation was done in 14 patients: developmental categories of communication were normal in 64.3%, problem solving in 71.4%, personal-social in 57.1%, gross motor in 64.3%, and fine motor in 42.9%. Conclusion: There was no correlation between baseline parameters and outcome. Results of the study are showing that therapeutic hypothermia in term newborns can provide better survival and less neurologic sequels in HIE patients.

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