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    TROPONIN – OUR EXPERIENCE IN DETERMINATION OF MYOCARDIAL ISCHEMIC DAMAGE IN POSTOPERATIVE PERIOD OF CARDIAC SURGERY IN PEDIATRIC POPULATION
    (2017)
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    Lj. Kojik
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    Radica Muratovska-Delimitova
    Background: Troponin is an important biomarker for early evidence of ischemic damage to the heart tissue after a cardiac surgery conducted in the pediatric and adult populations. Elevated values correlate with perioperative and postoperative procedures and practices and are a significant factor for possible later complications. Methods: The study included 30 operated children divided into two groups, the first group of operated children without a cardiopulmonary bypass (CPB), and the second group of operated children with a cardiopulmonary bypass. The correlation between elevated troponin and perioperative and postoperative parameters was monitored (duration of CPB and aortic crossclamping time, stay in the intensive care and therapy during respiratory support, during inotropic support, the presence of renal or hepatic failure, postoperative complications). Results: In both groups of operated children troponin was elevated. In the first group of children operated without cardiopulmonary bypass, the average value of troponin was 9.5 ng/ml (range 6.5- 16.8 ng/l). In the second group of operated children (27 children) with cardiopulmonary bypass, the mean value of duration was 81.5 minutes (range 18 to 296 minutes), and X-cross time (aortic crossclamping time) in the same group of children was with a mean value of 28.2 minutes (range of 0-86 min.). In the first group of children the mean value of troponin was 9.5 ng/ml and in the second group 23.0 ng/ml. The obtained values of troponin have confirmed a highly significant correlation with perioperative and postoperative procedures. Conclusions: Troponin is a prognostic marker for early evidence of ischemic and necrotic changes of cardiac infarction in the pediatric population in cardiac surgery. Elevated values in the first 24-48 hours are significantly correlated with perioperative and postoperative procedures and are an important indicator of the extent of damage to the heart tissue. But its prognostic significance of myocardial ischemic changes is lost in a period between 2-6 months after cardiac surgery.
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    ASSOCIATION OF POLAND SYNDROME AND HYPERTRICHOSIS IN A PUBERTAL GIRL - Case report
    (Institute of Public Health of the Republic of North Macedonia = Институт за јавно здравје на Република Северна Македонија, 2021-06-30)
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    Hristijan Nestorov
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    Poland syndrome (PS) is a rare congenital malformation, most commonly characterized by absence of chest wall muscles on one side of the body. It may be accompanied with other deformities of the extremities. We present the case of a 10-year-old girl with Poland syndrome and hypertrichosis of the back of the trunk and extremities. The clinical examination did not reveal the etiology of the syndrome, such as familial predisposition or some event that led to interrupted blood flow during the early embionic growth. The pregnancy was concieved with in vitro fertilization (IVF); triplets were born and our patient is one of these three girls. The hypertrychosis appeared at 8 years of life, without evidence of previous familial occurance, medications or hormonal disbalance. Other malformations that were found were: a mild form of kyphoscoliosis and mitral valve prolapse. The child was evaluated using a multidisciplinary approach, with further follow-up planned with surgical correction of the chest wall and breast augmentation.
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    EVALUATION OF PATIENTS WITH CONGENITAL LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
    (Medical Faculty, Ss. Cyril and Methodius University in Skopje, 2020)
    Gjurkova-Angelovska B
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    Jovanovska V
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    Neshkovska-Shumenkovska M
    Left ventricular outflow tract obstruction is a relatively common form of congenital heart disease accounting for 6%-10% of congenital heart defects and is a significant problem in pediatric cardiology. Obstruction may involve the arch of aorta, aortic valve and subaortic portion of the left ventriclе. The degree of stenosis and hemodynamic consequences may vary from hypoplastic left heart syndrome to almost normal hearts. The study included patients aged 0-14 years, examined at the University Clinic of Pediatric Diseases in Skopje during the period of March 2013 and March 2019. Echocardiography was the main diagnostic method applied. A total of 1100 examinations on selected patients were preformed in order to determine the timing for surgery. Some types of congenital left ventricular outflow tract obstruction were detected in 43 patients.
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    Cardio toxic effects of anthracycline therapy in children with acute lymphoblastic leukemia
    (Elsevier BV, 2009-09)
    B. Coneska Jovanova
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    O. Muratovska
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    S. Glamocanin
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    K. Martinova
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    Z. Trajkova-Antevska
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    Two cases of non-syndromic congenital unilateral breast hypoplasia in one family
    (Institute of Public Health of the Republic of North Macedonia = Институт за јавно здравје на Република Северна Македонија, 2020-02-20)
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    Ana Stamatova
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    Micromastia or breast hypoplasia is described as underdevelopment of a woman’s mammary tissue. We present the case of a 15-year-old girl with unilateral micromastia, with familial predisposition. Ultrasound, hormonal, dysmorphic, cardiologic, genetic examinations and testing were performed. No mutation in the whole- exome sequencing was found, nor novel mutation. Some of these cases have been reported to be related to breаst cancer so further follow-up is mandatory. Therapy consists of surgical reconstruction of the affected breast. This is a rare condition and it requires a multidisciplinary approach.
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    Arrhythmias and conduction abnormalities in children after repair of tetralogy of Fallot
    (Military Health Department, Ministry of Defense of the Republic of Serbia, 2005)
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    Gurkova, Beti
    Aim. To find out types and frequency of cardiac arrhythmias and conduction abnormalities in the group of children who underwent surgery for tetralogy of Fallot (TOF). Methods. Forty-six pedicatric patients who underwent a complete repair of TOF at the age of 1 to 13 (mean 2.89 ? 2.36) were studied. Thirty-eight (82.60%) had total correction and 8 (17.40%) had palliative operation first, and total correction afterwards. Twenty-four-hour Holter ECG monitoring was performed in all 46 pediatric patients aged from 1 to 16 yrs (mean 6.48 ? 4.04) after surgery as follows: in 1 patient (2.17%) after a year, in 20 patients (43.47%) after 2 to 5 years and in 25 patients (54.34%) after 5 years. Mean age of patients on Holter monitoring was 9.25 ? 4.39 (range 4?19). Twenty of them (43.47%) were girls and 28 (56.53%) were boys. All the patients were evaluated by standard methods (clinical signs, clinical findings, ECG before surgery, ECG before Holter monitoring and 2D Doppler echocardiography. Results. Types of heart rhythm found out by Holter monitoring were: sinus nodus dysfunction in 1 child (2.17%), significant premature atrial contraction (PAC) in 8 (17.39%), supraventricular paroxysmal tachycardia in 3 (6.53%), transient nodal rhythm in 2 (4.34%), premature ventricular contraction (PVC) Lown grade I-III in 9 (19.56%) and Lown grade IV in 2 (4.34), atrioventricular (AV) block grade I in 2, right bundle branch block (RBBB) in all 46 (100%) and RBBB + left anterior hemiblock (LAH) in 4 (8.96%). There was no presence of atrial flutter, ventricular tachycardia or complete AV block. None of them experienced sudden death. Using cross procedure statistical methods, it was found that all the patients with PVC had right ventricular dilatation. There was no relation of other types of arrhythmia found on Holter monitoring to the other parameters from echocardiography, neither to the other standard methods. Children did not need the pace-maker, but 36.95% of the them required antiarrhythmic drugs. Conclusion. Twenty-four hour Holter ECG is a noninvasive and very sensitive method for discovering heart rhythm disturbances in children after the repair of tetralogy of Fallot, especially in asymptomatic patients. The patients after the repair of this congenital heart disease needed a long-term follow-up for early recognition of serious heart rhythm disturbances and their treatment.
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    Autoimmune thyroiditis after long term gonadotropin- releasing hormone agonist treatment for central precocious puberty: case report
    (Институт за јавно здравје на Р Северна Македонија = Institute of Public Health of R North Macedonia, 2020-02-20)
    Maja Tankoska
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    Avdi Murtezani
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    There is a small number of studies that have reported abnormalities in endocrine function after a long-term gonadotropin-releasing hormone agonist (GnRHa) treatment in girls. This treatment is considered as safe and effective by most authors. We report our second case of unusual outcome of long-term GnRHa therapy in a girl with central precocious puberty (CPP) of idiopathic or familial etiology. She has received monthly depot of injections of triptorelin for a time period of 4 years. We have examined thyroid function by measuring serum levels of thyrotropin (TSH), thyroxine (T4), thyroid antibodies and ultrasound of thyroid gland. At the age of 11 years she developed a mild goiter and presented with autoimmune thyroiditis, having elevated thyroid antibodies and ultrasound of thyroid gland typical for Hashimoto thyroiditis. Having in mind these two cases, we suggest a closer monitoring of thyroid function in girls with CPP, before and during therapy with GnRH agonist.
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    Brugada syndrome: A case report
    (Association of Medical Doctors "Sanamed" Novi Pazar, 2016)
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    Gjurkova-Angelovska, Beti
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    Cor triatriatum sinister - ретка вродена срцева мана - наши искуства во дијагнозата и третманот во детска возраст
    (Institute of Public Health of the Republic of North Macedonia = Институт за јавно здравје на Република Северна Македонија, 2019-04-06)
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    Beti Gjurkova- Angelovska
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    Valentina Jovanovska
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    Marija Neshkovska-Shumenkovska
    Cor triatriatum sinister е ретка срцева мана кај која левата преткомора е поделена на два дела со фибромускулна мембрана. Проксималната преткомора ги прима пулмоналните вени заедно со крвта која тие ја носат, а дисталната или (вистинска) преткомора е вообичаено празна и таа е поделена од комората со митралната валвула. Има постојана комуникација помеѓу двата дела на поделената преткомора преку која крвта се пренасочува кон левата комора. Маната е многу ретка, најчесто е изолирана, но може да оди во комбинација со други срцеви мани. Презентираме случаи на две деца на возраст од 8 и 3 години во времето кога е поставена дијагнозата. Кај обете причина за дијагнозата бил замор и чуен срцев шум. За поставување на дијагнозата беа користени анамнезата, клиничките знаци и симптоми, ЕКГ, рендген на срце и бели дробови и златниот стандард - ехокардиографијата. Дефинитивно излекување беше направено со изведување на кардиохируршка ресекција на фибромускулната мембрана со користење на екстракорпорална циркулација. Потребно е регуларно периодично следење на децата со цел откривање на доцни компликации од типот на нарушувања на срцевиот  ритам.
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    Evaluation of pediatric patients with juvenile idiopathic arthritis treated with biological therapy tocilizumab (actemra)
    (Институт за јавно здравје на Република Македонија = Institute of public health of Republic of Macedonia, 2021-10-06)
    Neshkovska Shumenkovska Marija
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    Gjurkova-Angelovska Beti
    Juvenile idiopathic arthritis (JIA) is the most common chronic disease in childhood. It manifests a heterogenic group of symptoms of arthritis, lasting at least 6 weeks and it appears before the age of 16. Patients who had no good therapeutic response to conventional therapy with Methotrexate were treated with biological therapy. The aim of this paper was to evaluate 9 patients who were receiving Tocilizumab at the Department of Rheumocariology, University Clinic of Pediatric Diseases in Skopje. Materials and methods: Our study included 9 patients treated at our Department with biological therapy with Tocilizumab. Prior to initiation of the biological therapy, all patients underwent laboratory investigations, purified protein derivate (PPD) skin test for tuberculosis, X ray of the lungs and heart, and analysis of hepatitis markers. All patients were treated with amp. Actemra( Tocilizumab) 8mg/kg /tt i.v. Two of the patients had a severe form of the disease (one with severe systemic form and one with severe oligoarticular form of JIA). All presented patients had clinical remission of the disease. Conclusion: Therapy with tocilizumab in patients with juvenile idiopathic arthritis is a good therapeutic choice. The results obtained in our study have shown a significant therapeutic effect of tocilizumab even in severe forms of the disease