{"title":"Effect of Prophylactic Management of Hemophilia on Bleeding Episodes.","authors":"Suman Roy, Arun Kumar De","doi":"10.1007/s12288-018-1054-6","DOIUrl":null,"url":null,"abstract":"<p><p>Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) are the most common and serious congenital coagulation factor deficiencies with repeated hemarthroses leading to development of target joints. Continuous prophylaxis is regular infusion of factor concentrates at fixed dose at regular interval to prevent hemorrhages. The study was designed to assess the outcome of continuous prophylaxis in hemophilia on bleeding episodes. It was conducted from November, 2017 to April, 2018 in the Paediatrics Department of Midnapore Medical College, Paschim Medinipur, West Bengal on 33 boys from 4 to 18 years of age suffering from Hemophilia with frequent bleeding episodes. Prior to starting continuous prophylaxis all the patients' target joint(s) were assessed based on Gilbert Score and bleeding episodes in the last 6 months were assessed based on Annualized Bleeding Rate and ISTH-BAT Score. All the children were provided prophylaxis therapy with plasma derived Factor concentrate twice per week at a dose of 20 + - 2 IU/Kg. All bleeding episodes of the children during prophylaxis were recorded. Bleeding episodes showed significant improvement only in children who could sufficiently adhere to continuous prophylactic therapy. Continuous prophylaxis reduces bleeding episodes in Hemophilia in twice weekly protocol provided the patients sufficiently adhere to continuous prophylaxis regimen.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.7000,"publicationDate":"2019-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s12288-018-1054-6","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Indian Journal of Hematology and Blood Transfusion","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s12288-018-1054-6","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2018/12/3 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) are the most common and serious congenital coagulation factor deficiencies with repeated hemarthroses leading to development of target joints. Continuous prophylaxis is regular infusion of factor concentrates at fixed dose at regular interval to prevent hemorrhages. The study was designed to assess the outcome of continuous prophylaxis in hemophilia on bleeding episodes. It was conducted from November, 2017 to April, 2018 in the Paediatrics Department of Midnapore Medical College, Paschim Medinipur, West Bengal on 33 boys from 4 to 18 years of age suffering from Hemophilia with frequent bleeding episodes. Prior to starting continuous prophylaxis all the patients' target joint(s) were assessed based on Gilbert Score and bleeding episodes in the last 6 months were assessed based on Annualized Bleeding Rate and ISTH-BAT Score. All the children were provided prophylaxis therapy with plasma derived Factor concentrate twice per week at a dose of 20 + - 2 IU/Kg. All bleeding episodes of the children during prophylaxis were recorded. Bleeding episodes showed significant improvement only in children who could sufficiently adhere to continuous prophylactic therapy. Continuous prophylaxis reduces bleeding episodes in Hemophilia in twice weekly protocol provided the patients sufficiently adhere to continuous prophylaxis regimen.
期刊介绍:
Indian Journal of Hematology and Blood Transfusion is a medium for propagating and exchanging ideas within the medical community. It publishes peer-reviewed articles on a variety of aspects of clinical hematology, laboratory hematology and hemato-oncology. The journal exists to encourage scientific investigation in the study of blood in health and in disease; to promote and foster the exchange and diffusion of knowledge relating to blood and blood-forming tissues; and to provide a forum for discussion of hematological subjects on a national scale.
The Journal is the official publication of The Indian Society of Hematology & Blood Transfusion.