2001 Fiscal Year Final Research Report Summary
Clinical and Biomolecular Studies on Thrombophilia in Children.
Project/Area Number |
10470212
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Hematology
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Research Institution | Nara Medical University |
Principal Investigator |
YOSHIOKA Akira Nara Medical Univ, Professor, 医学部, 教授 (40106498)
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Co-Investigator(Kenkyū-buntansha) |
TANAKA Ichiro Nara Medical Univ, Assistant Professor, 医学部, 講師 (00201616)
SUGIMOTO Mitsuhiko Nara Medical Univ, Assistant Professor, 医学部, 講師 (80192128)
TAKAHASHI Yukihiro Nara Medical Univ, Associate Professor, 医学部, 助教授 (60142379)
TAKAMIYA Osamu Shinshu University, Professor, 医療短期大学部, 教授 (50216785)
NAKA Hiroyuki Nara Medical Univ, Assistant, 医学部, 助手 (40281761)
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Project Period (FY) |
1998 – 2000
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Keywords | thrombophilia / antithrombin / protein C / protein S / Heparin cofactor-II / Upshaw-Schulman Syndome / VWF-cleaving protease / children |
Research Abstract |
In order to know the patients' number and its clinical conditions, we conducted a nation-wide survey of thrombophilia in children using questionnaire. We sent the first questionnaire to 1, 319 medical doctors and got 806 answers (61.1 %). They are as follows; congenital antithrombin(AT) deficiency 137 families/190 cases (15 children), protein C (PC) deficiency 146/182(27), protein S (PS) deficiency 62/84(16), heparin cofactor-Iiciency (HC-II2/3(0), abnormal plasminogenemia 65/65+α (7), abnormal fibrinogenemia 47/57 (13), others 16/20 (4), and totally 475/601 (82). We got answers of 36 cases (43.9 %) from 82 cases by the 2nd questionnaire. It was divided into 2 groups, positive history of thromboembolic events in childhood, 21 cases (AT 4, PC10, PS7) and negative history of those in childhood, 15 cases (AT3, PC4, PS8). Sex ratio of theformergroup was M11: F10. Age at onset of the events was ranged between newborn period and adlescence. Main initial events of thromboembolism were deep vein thrombosis in lower extremity (DVT), pulmonary thromboembolism (PTE), brain embolism and etc. Laboratory examinations showed medium low levels of activity and antigen of AT, PC and PS (Type I, heterozygote) in the majority of the cases and extremely low levels of those of PC and PS in only 3 cases, gene analysis was performed in 8 cases. Several kinds of antithrombotic agents were used at the in itial events of thromboembolism in these cases and warfarin was mostly applied for prophylaxis treatment after establishment of each diagnosis. Prothrombin time-INR and/or Thrombotest were used as a monitor to evalute good hemostatic control. It is suggested that careful and continuous observation is most important in children with thrombophilia. Other clinical and biomolecular studies on thrombophilia in children were also performed.
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Research Products
(9 results)