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    Wyświetlanie 1-7 z 7
    Tytuł:
    Demograficzno-społeczne uwarunkowania poziomu wiedzy o hemofilii u osób nie dotkniętych tym problemem zdrowotnym
    Demographic and social determinants of the level of knowledge about hemophilia in persons who do not suffer from this disease
    Autorzy:
    Walęcka-Matyja, Katarzyna
    Powiązania:
    https://bibliotekanauki.pl/articles/2139120.pdf
    Data publikacji:
    2004
    Wydawca:
    Uniwersytet Łódzki. Wydawnictwo Uniwersytetu Łódzkiego
    Tematy:
    hemophilia
    level of the knowledge of hemophilia
    family
    Opis:
    The group which has been examinated was composed of 82 persons (40 women and 42 men). The following method was questionnaire prepared by autor of this article. The purpose of the study was described the level of the knowledge of hemophilia in examined group of people, who were not suffernig from hemophilia nad comparison level of the knowledge of hemophilia between the women and the men. The results indicated the fact that few of the women and the men was able to explain correctly and enough the conceptions connected with hemophilia (definition, symptoms and kinds of this disease. There are statistically significant differences between the sexes regarded in range of the symptoms and kinds of hemophilia. The women often than men had more wide view of disease like hemophilia. Relatively more examinated persons were given properly answers, concerned social mtyhs of hemophilia.
    Źródło:
    Acta Universitatis Lodziensis. Folia Psychologica; 2004, 08; 91-101
    2353-4842
    Pojawia się w:
    Acta Universitatis Lodziensis. Folia Psychologica
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    COMPARISON OF PERSONALIZED AND STANDARD PROPHYLAXIS BASED ON OCTOCOG ALFA IN PEDIATRIC PATIENTS WITH HEMOPHILIA A
    Autorzy:
    Tatara, Tomasz
    Dąbrowska-Bender, Marta
    Duda-Zalewska, Aneta
    Staniszewska, Anna
    Kurek, Magdalena
    Powiązania:
    https://bibliotekanauki.pl/articles/895493.pdf
    Data publikacji:
    2020-04-29
    Wydawca:
    Polskie Towarzystwo Farmaceutyczne
    Tematy:
    Hemophilia A
    octocog alfa
    prophylaxis
    Opis:
    Comparison of personalized and standard prophylaxis prevention therapies based on octocog alfa in pediatric patients with hemophilia A. Studies on a general population (adults and children) demonstrate a statistically significant advantage of personalized prevention therapy over standard prophylaxis prevention therapy in terms of annual bleeding rate (ABR) and annual joint bleeding rate (AJBR) while studies on a pediatric population included insufficiently large populations, however, their results demonstrate a numerical advantage of personalized approach over standard prevention strategy prophylaxis regimen.
    Źródło:
    Acta Poloniae Pharmaceutica - Drug Research; 2020, 77, 2; 221-218
    0001-6837
    2353-5288
    Pojawia się w:
    Acta Poloniae Pharmaceutica - Drug Research
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    Sclerotherapy of esophageal varices in hemophilia patients with liver cirrhosis – a prospective, controlled clinical study
    Autorzy:
    Szczepanik, Andrzej B
    Pielaciński, Konrad
    Oses-Szczepanik, Anna M
    Huszcza, Sławomir
    Misiak, Andrzej
    Dąbrowski, Wojciech P
    Gajda, Sławomir
    Powiązania:
    https://bibliotekanauki.pl/articles/1392541.pdf
    Data publikacji:
    2018
    Wydawca:
    Index Copernicus International
    Tematy:
    hemophilia
    esophageal varices
    bleeding
    endoscopic sclerotherapy
    liver cirrhosis
    Opis:
    Introduction: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency or lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis in the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and safety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The aim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required to ensure proper hemostasis after sclerotherapy procedures. Material and methods: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding treated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified to repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group). A 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of normal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60-80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in terms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated group. Results: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients; 2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical differences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%), complete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent bleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication was 12-21 weeks (average 15.2). In the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences were found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U/kg b.w./day, in hemophilia A in a severe form with an inhibitor <5 BU – 95.2 U/kg b.w./day, in mild form – 64.2 U/kg b.w./day and in severe hemophilia B – 91.6 U/kg b.w./day. Conclusions: Sclerotherapy is an effective method in the management of esophageal variceal bleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis of hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid bleeding complications.
    Źródło:
    Polish Journal of Surgery; 2018, 90, 1; 29-34
    0032-373X
    2299-2847
    Pojawia się w:
    Polish Journal of Surgery
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    Sclerotherapy of esophageal varices in severe hemophilia a patient and high titer inhibitor – case report
    Autorzy:
    Szczepanik, Andrzej
    Dąbrwski, Wojciech
    Szczepaniak, Anna
    Pielaciński, Konrad
    Jaśkowiak, Wojciech
    Powiązania:
    https://bibliotekanauki.pl/articles/1394398.pdf
    Data publikacji:
    2015
    Wydawca:
    Index Copernicus International
    Tematy:
    hemophilia
    inhibitor
    esophageal varices
    injection sclerotherapy
    aPCC
    rFVIIa
    Opis:
    In cirrhotic hemophilia patients bleeding from esophageal varices is a serious clinical condition due to congenital deficiency of clotting factors VIII or IX, decreased prothrombin synthesis and hypersplenic thrombocytopenia. In hemophiliac with high-titer inhibitor bypassing therapy is required with activated prothrombin complex concentrates (aPCC) or recombinant activated coagulation factor VII (rFVIIa). Doses and duration treatment with these agents following endoscopic treatment of esophageal varices have not been yet established. Authors report the first case of a severe hemophilia A patient with high titer inhibitor (40 BU) treated with repeated injection sclerotherapy. The patient was admitted with symptoms of massive esophageal variceal hemorrhage ceased with emergency sclerotherapy. Bypassing therapy was administered with aPCC at initial dose of 72.5 U/kg and then with average daily dose of 162 U/kg through 5 days. To achieved a total eradication of esophageal varices the patient was then subjected to four elective sclerotherapy procedures. Two were covered by aPCC with daily dose of 120 U/kg and 145 U/kg for 4 and 3 days respectively and the following two procedures were covered by rFVIIa with the initial dose of 116 µg/kg and the next doses of 87 µg/kg administered every 3 hours in procedure day and every 4 hours on the next two days. During all procedures excellent hemostasis was achieved and no hemorrhagic or thromboembolic complications were observed. Bypassing regimen therapy with aPCC and rFVIIa we applied have been shown to be safe and effective in this patient subjected to sclerotherapy procedures.
    Źródło:
    Polish Journal of Surgery; 2015, 87, 9; 464-468
    0032-373X
    2299-2847
    Pojawia się w:
    Polish Journal of Surgery
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    The factor VIII protein and its function
    Autorzy:
    Mazurkiewicz-Pisarek, Anna
    Płucienniczak, Grażyna
    Ciach, Tomasz
    Płucienniczak, Andrzej
    Powiązania:
    https://bibliotekanauki.pl/articles/1038835.pdf
    Data publikacji:
    2016
    Wydawca:
    Polskie Towarzystwo Biochemiczne
    Tematy:
    Factor VIII (FVIII)
    molecular structure
    function
    hemophilia A
    bleeding disorders
    Opis:
    Factor VIII (FVIII), an essential blood coagulation protein, is a key component of the fluid phase blood coagulation system. Human factor VIII is a single chain of about 300 kDa consisting of domains described as A1-A2-B-A3-C1-C2. The protein undergoes processing prior to secretion into blood resulting in a heavy chain of 200 kDa (A1-A2-B) and a light chain of 80 kDa (A3-C1-C2) linked by metal ions. The role of factor VIII is to increase the catalytic efficiency of factor IXa in the activation of factor X. Variants of these factors lead frequently also to severe bleeding disorders.
    Źródło:
    Acta Biochimica Polonica; 2016, 63, 1; 11-16
    0001-527X
    Pojawia się w:
    Acta Biochimica Polonica
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    Piętno (nie)zawinione – historia dziecięcej stygmatyzacji
    Autorzy:
    Szubert, Mateusz
    Powiązania:
    https://bibliotekanauki.pl/articles/2028649.pdf
    Data publikacji:
    2020-09-13
    Wydawca:
    Polskie Towarzystwo Ludoznawcze
    Tematy:
    HIV/AIDS
    hemofilia
    tabu
    stygmatyzacja
    wykluczenie
    epidemia
    strach
    hemophilia
    taboo
    stigma
    illness
    exclusion
    epidemic
    fear
    Opis:
    Celem artykułu jest przybliżenie mało znanych polskiemu czytelnikowi przypadków stygmatyzacji najmłodszych ofiar epidemii AIDS. Hemofilicy od samego początku epidemii AIDS znaleźli się w tzw. najwyższej grupie ryzyka, obok homoseksualistów, narkomanów i prostytutek. W dobie histerycznego lęku budowanego wokół HIV, młodzi hemofilicy szybko stali się przedmiotem społecznych represji i prześladowań. Niezwykle cennym materiałem, ukazującym siłę społecznej stygmatyzacji, widzianej i doświadczanej z perspektywy młodego pacjenta, jest dziennik z czasu choroby Ryana White’a (My Own Story). Autobiografia Ryana ilustruje doskonale proces budowania negatywnej mitologii choroby, obnażając jednocześnie jej destrukcyjny potencjał. Wystąpienie młodego hemofilika to prawdziwe kompendium wiedzy na temat społecznych mechanizmów stygmatyzacji, w tym przede wszystkim dotyczących strategii zarządzania strachem. 
    The aim of the article is to shed light on cases of stigma among the youngest victims of the AIDS epidemic, a topic that Polish readers may not be familiar with. Since the very beginning of the AIDS epidemic, hemophiliacs have been classified as belonging to the so-called highest risk group, next to homosexuals, drug addicts, and prostitutes. In the age of hysterical anxiety centered around HIV, young hemophiliacs quickly became the subject of social repression and persecution. An extremely valuable material that shows the strength of the social stigma, as seen and experienced from the perspective of a young patient, is Ryan White’s diary documenting his illness. Ryan’s autobiography illustrates perfectly the development of a negative mythology concerning the disease and reveals its destructive potential. The portrayal of the young hemophiliac is a compendium of knowledge about the social mechanisms of stigmatisation, and in particular of strategies aimed to control fear.
    Źródło:
    Literatura Ludowa; 2020, 64, 1; 29-38
    2544-2872
    0024-4708
    Pojawia się w:
    Literatura Ludowa
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
    Tytuł:
    Expression of the gene encoding blood coagulation factor VIII without domain B in E. coli bacterial expression system
    Autorzy:
    Mazurkiewicz-Pisarek, Anna
    Mazurkiewicz, Alina
    Mikiewicz, Diana
    Baran, Piotr
    Ciach, Tomasz
    Powiązania:
    https://bibliotekanauki.pl/articles/16706242.pdf
    Data publikacji:
    2023
    Wydawca:
    Polska Akademia Nauk. Czasopisma i Monografie PAN
    Tematy:
    factor VIII
    hemophilia type A
    recombinant coagulation factor VIII
    prokaryotic expression system
    E. coli
    recombinant protein production system
    Opis:
    In this article, we have demonstrated the feasibility of generating an active form of recombinant blood coagulation factor VIII using an E. coli bacterial expression system as a potential treatment for hemophilia type A. Factor VIII (FVIII), an essential blood coagulation protein, is a key component of the fluid phase blood coagulation system. So far, all available recombinant FVIII formulations have been produced using eukaryotic expression systems. Mammalian cells can produce catalytically active proteins with all the necessary posttranslational modifications. However, cultivating such cells is time-consuming and highly expensive, and the amount of the obtained product is usually low. In contrast to eukaryotic cells, bacterial culture is inexpensive and allows the acquisition of large quantities of recombinant proteins in a short time. With this study, we aimed to obtain recombinant blood coagulation factor VIII using the E. coli bacterial expression system, a method not previously explored for this purpose. Our research encompasses the synthesis of blood coagulation factor VIII and its expression in a prokaryotic system. To achieve this, we constructed a prokaryotic expression vector containing a synthetic factor VIII gene, which was then used for the transformation of an E. coli bacterial strain. The protein expression was confirmed by mass spectrometry, and we assessed the stability of the gene construct while determining the optimal growth conditions. The production of blood coagulation factor VIII by the E. coli bacterial strain was carried out on a quarter-technical scale. We established the conditions for isolation, denaturation, and renaturation of the protein, and subsequently confirmed the activity of FVIII.
    Źródło:
    BioTechnologia. Journal of Biotechnology Computational Biology and Bionanotechnology; 2023, 104, 3; 247-262
    0860-7796
    Pojawia się w:
    BioTechnologia. Journal of Biotechnology Computational Biology and Bionanotechnology
    Dostawca treści:
    Biblioteka Nauki
    Artykuł
      Wyświetlanie 1-7 z 7

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