Literature DB >> 25112898

Current management of hemophilia B: recommendations, complications and emerging issues.

Massimo Franchini1.   

Abstract

Hemophilia B is a rare hereditary hemorrhagic disorder characterized by deficiency of the clotting factor IX (FIX). Hemophilia B patients experience mild-to-severe bleeding complications according to the degree of FIX defect. The mainstay of treatment of hemophilia B consists of replacement therapy and nowadays several plasma-derived and recombinant FIX products are commercially available. This article reviews the current management of hemophilia B patients analyzing the results of the most important clinical trials. In addition, it will focus on the more recent advances in the production of new FIX molecules aimed at the improvement of the clinical management of such patients.

Entities:  

Keywords:  hemophilia B; inhibitors; long-acting FIX products; plasma-derived FIX concentrates; recombinant FIX products

Mesh:

Substances:

Year:  2014        PMID: 25112898     DOI: 10.1586/17474086.2014.947955

Source DB:  PubMed          Journal:  Expert Rev Hematol        ISSN: 1747-4094            Impact factor:   2.929


  7 in total

Review 1.  Practical aspects of extended half-life products for the treatment of haemophilia.

Authors:  Thierry Lambert; Gary Benson; Gerry Dolan; Cedric Hermans; Victor Jiménez-Yuste; Rolf Ljung; Massimo Morfini; Silva Zupančić-Šalek; Elena Santagostino
Journal:  Ther Adv Hematol       Date:  2018-09-06

Review 2.  Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management.

Authors:  Gary Benson; Günter Auerswald; Gerry Dolan; Anne Duffy; Cedric Hermans; Rolf Ljung; Massimo Morfini; Silva Zupančić Šalek
Journal:  Blood Transfus       Date:  2017-11-14       Impact factor: 3.443

Review 3.  Tolerogenic properties of the Fc portion of IgG and its relevance to the treatment and management of hemophilia

Authors:  Richard S Blumberg; David Lillicrap
Journal:  Blood       Date:  2018-03-27       Impact factor: 22.113

4.  Management of Odontogenic Fascial Space Infection in Hemophilia Patients: A Proposed Protocol.

Authors:  Md Kalim Ansari; Ghulam Sarwar Hashmi; Syed Saeed Ahmed; Sajjad Abdur Rahman; Tabishur Rahman
Journal:  J Maxillofac Oral Surg       Date:  2018-05-09

5.  First open-label, single-arm, prospective study of real-world use of FIX replacement therapy in a predominantly pediatric hemophilia B population in China.

Authors:  Renchi Yang; Runhui Wu; Jing Sun; Feifei Sun; Jeremy Rupon; Francois Huard; Joan M Korth-Bradley; Lihong Xu; Binyu Luo; Yingxue Cathy Liu; Pablo Rendo
Journal:  Medicine (Baltimore)       Date:  2021-05-28       Impact factor: 1.817

6.  Thrombogenicity evaluation in 221 patients with haemophilia B treated with nonacog alfa.

Authors:  Pablo Rendo; Janice Lamb; Lynne Smith; Joanne Fuiman; Joan M Korth-Bradley
Journal:  Blood Coagul Fibrinolysis       Date:  2018-01       Impact factor: 1.276

7.  Health-related quality of life in paediatric haemophilia B patients treated with rIX-FP.

Authors:  Sylvia von Mackensen; Jinesh Shah; Wilfried Seifert; Gili Kenet
Journal:  Haemophilia       Date:  2018-11-14       Impact factor: 4.287

  7 in total

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