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    ACUTE MYELOBLASTIC LEUKEMIA IN CHILDREN, TREATMENT AND PROGNOSIS 125
    (Springer Science and Business Media LLC, 1997-05)
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    Zisovski, N
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    Glamocanin, S
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    PROCALCITONIN AS A PROMISING BIOCHEMICAL MARKER FOR EARLY DETECTION AND TREATMENT OF SEPSIS IN NEONATES AT INTENSIVE CARE UNIT AND ONCOLOGIC PATIENTS WITH FEBRILE NEUTROPENIA
    (Macedonian Association of Anatomists and Morphologists, 2020)
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    Tankoska Maja
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    Pandovska Bisera
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    Kimovska, Milica
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    Акривирана хемофилија - ретка но потенцијално живото-загрозувачка состојба за двата пола
    (Лекарска комора на Македонија, 2015-03)
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    Smilevska Tatjana
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    Cevreska Lidija
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    Improved survival in patients with TTP (Sy Moskowitz)
    (Medical Faculty, Ss. Cyril and Methodius University in Skopje, 2016)
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    Stankovic Vera
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    Procalcitonin-early biochemical marker for diagnosis, prognosis and treatment of sepsis in neonates and oncological patients with febile neutropenia
    (Macedonian Association of Anatomists, 2020)
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    Tankoska Maja
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    Kimovska Hristova, Mica
    Pediatric sepsis is a life - threatening condition, in which the immune system, instead of controlling the infection,causes damage to tissues and organs. The aim of this study was to determine the role of procalcitonin (PCT)in the early diagnosis of sepsis in high - risk infants and Oncological patients with febrile neutropenia,its prognostic value,and the role of PCT in the cho ice of antibiotic. The study is designed as retrospective - prospective, it is being worked at the PHI UC for Children Diseases Skopje. I t includes 60 critical newborns and 40 O ncology patients.The examined group were divided into two subgroups:30 critical i nfants with bacterial sepsis in the intensive care unit and 20 O ncological patients with sepsis and febrile neutropenia. PCT was determined the first 24h,3 - 5 days and 6 - 14 days of hospitalization. The value of PCT during the first 24 hours of admission was increased in all 50 patients .Most of them had signs of severe sepsis and few od them had signs of septic shock . In all of them, double parenteral antibiotic therapy was started .Seven patients has died in the first five days. After 3 - 5 days of the start of antibiotic therapy, PC T values decreased . After the third measurement, PCT values continued to decrease and in majority of the patients the antibiotic therapy was discontinued. By measuring the values of PCT, an early diagnosis of sepsis can be made. This is important to start with antibiotics to prevent sepsis and septic shock . T he dynamic in the values of PCT determine the duration of antibiotic,its modification,the rational uses of antibiotics and the emergence of resistance to it.
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    T-lymphoblastic leukemia/lymphoma in macedonian patients with Nijmegen breakage syndrome
    (Walter de Gruyter GmbH, 2016-07-01)
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    Antevska-Trajkova, Z
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    Coneska-Jovanova, B
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    Nijmegen breakage syndrome (NBS) is a rare autosomal recessive chromosomal instability disorder characterized by microcephaly, immunodeficiency, radiosensitivity and a very high predisposition to malignancy. The gene responsible for the disease, NBS1, is located on chromosome 8q21 and encodes a protein called nibrin. After identification of the gene, a truncating 5 bp deletion, 657-661delACAAA, was identified as the disease-causing mutation in patients with the NBS. In this report, we describe two patients with NBS and T-lymphoblastic leukemia/lymphoma in a Macedonian family. To the best of our knowledge, this is the first family with NBS reported from Macedonia. Both children presented with microcephaly, syndactyly and the development of T cell lymphoblastic lekemia/lymphoma at the age of 7 and 10 years, respectively. The molecular analysis of NBS1 genes in our patients showed homozygosity for the 657del5 mutation in the NBS1 gene. The parents were heterozygotes for the 657del5 mutation and they had no knowledge of a consanguineous relationship. The first child was treated with the International Berlin-Frankfurt-Münster (BFM)-Non Hodgkin lymphoma (NHL) protocol and achieved a complete remission that lasted for 21 months. Subsequently, he developed a medullar relapse with hyperleukocytosis and died due to lethal central nervous system (CNS) complications. The second child was treated according to the International Collaborative Treatment Protocol for Children and Adolescents with Acute Lymphoblastic Leukemia 2009 (AIOP-BFM ALL 2009) protocol. Unfortunately, remission was not achieved.
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    Case report of a rare coagulation disorder-Acquired hemophilia A
    (Medical Faculty, Ss. Cyril and Methodius University in Skopje, 2016)
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    Ivanovski Martin
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    First Cases of Hb Agrinio Described in Patients from the Republic of Macedonia
    (Taylor & Francis Online, 2017-12-08)
    Dimishkovska Marija,
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    Kuzmanovska Maja
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    Previous molecular analyses of α-thalassemia (α-thal) in the Republic of Macedonia have identified the following genetic defects: –α3.7 (rightward), –(α)20.5 and – –MED I deletions and Hb Icaria [α142, Term→Lys (α2), HBA2: c.427T>A] and polyadenylation signal (polyA) [AATAAA>AATGAA (α2), HBA2: c.*92A>G] point mutations. Here, we report two unrelated patients from the Romani population in the Republic of Macedonia, homozygotes for the α2-globin gene variant Hb Agrinio [α29(B10)Leu→Pro; HBA2: c.89T>C]. To date, Hb Agrinio has been described only in individuals of Greek, Cypriot and Spanish origin. Both of our patients had early presentation of the disease (3.5 years and 2 months, respectively) with frequent blood transfusions from early infancy. They have a severe intermediate phenotype of thalassemia (Hb H disease) with hemoglobin (Hb) levels of 7.8 and 7.7 g/dL, respectively. Although the HBA2: c.89T>C mutation results in an α+ allele, the severe phenotype of the homozygotes is due to the production of hyperunstable α chains that undergo post translational precipitation. This leads to a greater degree of red cell damage and hemolytic anemia. The detection of Hb Agrinio in two unrelated families of Romani ethnic origin, may suggest it is a founder mutation in this population living in the Republic of Macedonia. Considering the severity of the clinical presentation of the homozygotes or compound heterozygotes for this rare Hb variant, a targeted molecular screening for Hb Agrinio mutation carriers should be considered in all patients of Romani ethnic origin with manifested microcytosis.
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    Современ пристап во лекувањето на мултипниот миелом
    (Македонско лекарско друштво=Macedonian Medical Association, 2014)
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    Смилевска, Татјана
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    Попова, Марија
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