Soucie JM, Evatt B, Jackson D. Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators. Am. J. Hematol. 59(4), 288– 294(1998).
White GC 2nd, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J; Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb. Haemost. 85(3),560(2001).
Antonarakis SE. Molecular genetics of coagulation factor VIII gene and hemophilia A. Thromb. Haemost. 74(1),322– 328(1995).
Do H, Healey JF, Waller EK, Lollar P. Expression of factor VIII by murine liver sinusoidal endothelial cells. J. Biol. Chem. 274(28),19587– 19592(1999).
Hollestelle MJ, Thinnes T, Crain K et al. Tissue distribution of factor VIII gene expression in vivo – a closer look. Thromb. Haemost. 86(3), 855– 861(2001).
Antonarakis SE. The molecular genetics of hemophilia A and B in man. Factor VIII and factor IX deficiency. Adv. Hum. Genet. 17,27– 59(1988).
Antonarakis SE, Youssoufian H, Kazazian HH Jr. Molecular genetics of hemophilia A in man (factor VIII deficiency). Mol. Biol. Med. 4(2),81– 94(1987).
Geddes VA, MacGillivray RT. The molecular genetics of hemophilia B. Transfus. Med. Rev. 1(3), 161– 170(1987).
Lawn RM. The molecular genetics of hemophilia: blood clotting factors VIII and IX. Cell 42(2),405– 406(1985).
Howard TE, Yanover C, Mahlangu J et al. Haemophilia management: time to get personal? Haemophilia 17(5),721– 728(2011).
Rosner F. Hemophilia in the Talmud and rabbinic writings. Ann. Intern. Med. 70(4),833– 837(1969).
Lane S. Successful transfusion of blood. Lancet 1,185– 188(1840).
Feissly R. Etudes sur l’hemophilia Bulletin et emmoires de la Societe de Medicine de. Paris 47, 1778– 1783(1923).
Pool JG. Cryoprecipitate in the treatment of hemophilia. Calif. Med. 113(2),66– 67(1970).
Mannucci PM. Hemophilia and related bleeding disorders: a story of dismay and success. Hematology Am. Soc. Hematol. Educ. Program 1– 9(2002).
Nilsson IM, Hedner U, Ahlberg A. Haemophilia prophylaxis in Sweden. Acta Paediatr. Scand. 65(2), 129– 135(1976).
Rabiner SF, Telfer MC. Home transfusion for patients with hemophilia A. N. Engl. J. Med. 283(19), 1011– 1015(1970).
Evatt BL. Demographics of hemophilia in developing countries. Semin. Thromb. Hemost. 31(5), 489– 494(2005).
Goedert JJ, Kessler CM, Aledort LM et al. A prospective study of human immunodeficiency virus type 1 infection and the development of AIDS in subjects with hemophilia. N. Engl. J. Med. 321(17), 1141– 1148(1989).
Pergantou H, Matsinos G, Papadopoulos A, Platokouki H, Aronis S. Comparative study of validity of clinical, x-ray and magnetic resonance imaging scores in evaluation and management of haemophilic arthropathy in children. Haemophilia 12(3),241– 247(2006).
Arnold WD, Hilgartner MW. Hemophilic arthropathy. Current concepts of pathogenesis and management. J. Bone Joint Surg. Am. 59(3),287– 305(1977).
DePalma AF. Hemophilic arthropathy. Clin. Orthop. Relat. Res. 52,145– 165(1967).
Carcao MD, Aledort L. Prophylactic factor replacement in hemophilia. Blood Rev. 18(2),101– 113(2004).
Hoots WK. Pathogenesis of hemophilic arthropathy. Semin. Hematol. 43(1 Suppl. 1),S18– S22(2006).
Gringeri A, Lundin B, von Mackensen S, Mantovani L, Mannucci PM; ESPRIT Study Group. A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study). J. Thromb. Haemost. 9(4), 700– 710(2011).
Klamroth R, Pollmann H, Hermans C et al. The relative burden of haemophilia A and the impact of target joint development on health-related quality of life: results from the ADVATE Post-Authorization Safety Surveillance (PASS) study. Haemophilia 17(3),412– 421(2011).
Hoots WK, Rodriguez N, Boggio L, Valentino LA. Pathogenesis of haemophilic synovitis: clinical aspects. Haemophilia 13(Suppl. 3),4– 9(2007).
Valentino LA. Blood-induced joint disease: the pathophysiology of hemophilic arthropathy. J. Thromb. Haemost. 8(9), 1895– 1902(2010).
Valentino LA, Hakobyan N, Rodriguez N, Hoots WK. Pathogenesis of haemophilic synovitis: experimental studies on blood-induced joint damage. Haemophilia 13(Suppl. 3),10– 13(2007).
Mulder K, Llinás A. The target joint. Haemophilia 10(Suppl. 4),152– 156(2004).
Roosendaal G, Jansen NW, Schutgens R, Lafeber FP. Haemophilic arthropathy: the importance of the earliest haemarthroses and consequences for treatment. Haemophilia 14(Suppl. 6),4– 10(2008).
Roosendaal G, Mauser-Bunschoten EP, De Kleijn P et al. Synovium in haemophilic arthropathy. Haemophilia 4(4),502– 505(1998).
Roosendaal G, Vianen ME, Wenting MJ et al. Iron deposits and catabolic properties of synovial tissue from patients with haemophilia. J. Bone Joint Surg. Br. 80(3),540– 545(1998).
Paleolog EM, Miotla JM. Angiogenesis in arthritis: role in disease pathogenesis and as a potential therapeutic target. Angiogenesis 2(4),295– 307(1998).
Dunn AL. Pathophysiology, diagnosis and prevention of arthropathy in patients with haemophilia. Haemophilia 17(4),571– 578(2011).
Parsons JR, Zingler BM, McKeon JJ. Mechanical and histological studies of acute joint hemorrhage. Orthopedics 10(7),1019– 1026(1987).
Aledort LM, Haschmeyer RH, Pettersson H. A longitudinal study of orthopaedic outcomes for severe factor-VIII-deficient haemophiliacs. The Orthopaedic Outcome Study Group. J. Intern. Med. 236(4),391– 399(1994).
Fischer K, van Hout BA, van der Bom JG, Grobbee DE, van den Berg HM. Association between joint bleeds and Pettersson scores in severe haemophilia. Acta Radiol. 43(5),528– 532(2002).
Kreuz W, Escuriola-Ettingshausen C, Funk M, Schmidt H, Kornhuber B. When should prophylactic treatment in patients with haemophilia A and B start? – The German experience. Haemophilia 4(4),413– 417(1998).
Manco-Johnson MJ, Abshire TC, Shapiro AD et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N. Engl. J. Med. 357(6),535– 544(2007).
Nilsson IM, Berntorp E, Löfqvist T, Pettersson H. Twenty-five years’ experience of prophylactic treatment in severe haemophilia A and B. J. Intern. Med. 232(1),25– 32(1992).
Berntorp E, Astermark J, Björkman S et al. Consensus perspectives on prophylactic therapy for haemophilia: summary statement. Haemophilia 9(Suppl. 1),1– 4(2003).
Berntorp E. Pharmacoeconomics of factor dosing in the haemophilia population. Haemophilia 12(Suppl. 4),70– 73(2006).
Ragni MV, Fogarty PJ, Josephson NC, Neff AT, Raffini LJ, Kessler CM. Survey of current prophylaxis practices and bleeding characteristics of children with severe haemophilia A in US haemophilia treatment centres. Haemophilia 18(1),63– 68(2012).
Valentino LA. Secondary prophylaxis therapy: what are the benefits, limitations and unknowns? Haemophilia 10(2),147– 157(2004).
Manco-Johnson MJ, Nuss R, Geraghty S, Funk S, Kilcoyne R. Results of secondary prophylaxis in children with severe hemophilia. Am. J. Hematol. 47(2), 113– 117(1994).
Liesner RJ. Prophylaxis in haemophilic children. Blood Coagul. Fibrinolysis 8(Suppl. 1), S7– S10(1997).
Tagliaferri A, Di Perna C, Rivolta GF. Secondary prophylaxis in adolescent and adult haemophiliacs. Blood Transfus. 6(Suppl. 2),S17– S20(2008).
Pipe SW, Valentino LA. Optimizing outcomes for patients with severe haemophilia A. Haemophilia 13(Suppl. 4), 1– 16; quiz 3 p following 16(2007).
Rodríguez-Merchán EC. Pathogenesis, early diagnosis, and prophylaxis for chronic hemophilic synovitis. Clin. Orthop. Relat. Res. 343,6– 11(1997).
Srivastava A. Dose and response in haemophilia – optimization of factor replacement therapy. Br. J. Haematol. 127(1),12– 25(2004).
Ripa T, Scaraggi FA, Ciavarella N. Early treatment of hemophilia with minimal doses of factor VIII or factor IX. Blood 51(4),763(1978).
Allain JP. Dose requirement for replacement therapy in hemophilia A. Thromb. Haemost. 42(3), 825– 831(1979).
Hermans C, De Moerloose P, Fischer K et al. European Haemophilia Therapy Standardisation Board. Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations. Haemophilia 17(3),383– 392(2011).
Inwood MJ, Killackey B, Startup SJ. The use and safety of Ibuprofen in the hemophiliac. Blood 61(4), 709– 711(1983).
Thould AK, Keeling DH, Murrell JS. Gastric microbleeding studies in patients with haemophilia taking benoxaprofen. Eur. J. Rheumatol. Inflamm. 5(2), 165– 169(1982).
Quang-Cantagrel ND, Wallace MS, Magnuson SK. Opioid substitution to improve the effectiveness of chronic noncancer pain control: a chart review. Anesth. Analg. 90(4), 933– 937(2000).
Gooberman-Hill R, Heathcote C, Reid CM et al. Professional experience guides opioid prescribing for chronic joint pain in primary care. Fam. Pract. 28(1),102– 109(2011).
Shupak R, Teitel J, Garvey MB, Freedman J. Intra-articular methylprednisolone therapy in hemophilic arthropathy. Am. J. Hematol. 27(1),26– 29(1988).
Rodríguez-Merchán EC, Villar A, Orbe A, Magallón M. Intra-articular methylprednisolone therapy in chronic hemophilic synovitis of the knee. Rev. Clin. Esp. 194(6), 480– 482(1994).
Kisker CT, Burke C. Double-blind studies on the use of steroids in the treatment of acute hemarthrosis in patients with hemophilia. N. Engl. J. Med. 282(12), 639– 642(1970).
Rodriguez-Merchan EC. Articular bleeding (hemarthrosis) in hemophilia: an orthopedists point of view. World Federation of Hemophilia, Montreal, QC, Canada (2008).
Ribbans WJ, Giangrande P, Beeton K. Conservative treatment of hemarthrosis for prevention of hemophilic synovitis. Clin. Orthop. Relat. Res. 343, 12– 18(1997).
Bleakley C, McDonough S, MacAuley D. The use of ice in the treatment of acute soft-tissue injury: a systematic review of randomized controlled trials. Am. J. Sports Med. 32(1),251– 261(2004).
Collins NC. Is ice right? Does cryotherapy improve outcome for acute soft tissue injury? Emerg. Med. J. 25(2), 65– 68(2008).
Hubbard TJ, Denegar CR. Does cryotherapy improve outcomes with soft tissue injury? J. Athl. Train. 39(3), 278– 279(2004).
Beeton K, Buzzard B. Physiotherapy in the management of patients with inhibitors. In: Inhibitors in Patients with Hemophilia . Rodriguez-Merchan EC, Lee CA (Eds.). Blackwell Science Ltd., Oxford, UK, 160– 168(2002).
Rodriguez-Merchan EC, Jimenez-Yuste V, Aznar JA et al. Joint protection in haemophilia. Haemophilia 17(Suppl. 2),1– 23(2011).
Luck JV Jr, Silva M, Rodriguez-Merchan EC, Ghalambor N, Zahiri CA, Finn RS. Hemophilic arthropathy. J. Am. Acad. Orthop. Surg. 12(4),234– 245(2004).
Luck JV Jr, Kasper CK. Surgical management of advanced hemophilic arthropathy. An overview of 20 years’ experience. Clin. Orthop. Relat. Res. 242,60– 82(1989).
Silva M, Luck JV Jr, Siegel ME. 32P chromic phosphate radiosynovectomy for chronic haemophilic synovitis. Haemophilia 7(Suppl. 2), 40– 49(2001).
Ugur O, Gedik GK, Atilla B, Rubello D. Radiosynovectomy: current status in the management of arthritic conditions. Nucl. Med. Commun. 29(9),755– 758(2008).
Globe D, Young NL, Von Mackensen S, Bullinger M, Wasserman J; Health-related Quality Of Life Expert Working Group Of The International Prophylaxis Study Group. Measuring patient-reported outcomes in haemophilia clinical research. Haemophilia 15(4),843– 852(2009).
Rentz A, Flood E, Butler R et al. Psychometric evaluation of a patient-reported symptom assessment tool for adults with haemophilia (the HAEMO-SYM). Haemophilia 15(5),1039– 1047(2009).
Fischer K, van der Bom JG, van den Berg HM. Health-related quality of life as outcome parameter in haemophilia treatment. Haemophilia 9(Suppl. 1), 75– 81; discussion 82(2003).
Hilliard P, Funk S, Zourikian N et al. Hemophilia joint health score reliability study. Haemophilia 12(5),518– 525(2006).
Poonnoose PM, Thomas R, Keshava SN et al. Psychometric analysis of the Functional Independence Score in Haemophilia (FISH). Haemophilia 13(5),620– 626(2007).
van Genderen FR, van Meeteren NL, van der Bom JG et al. Functional consequences of haemophilia in adults: the development of the Haemophilia Activities List. Haemophilia 10(5),565– 571(2004).
Groen WG, van der Net J, Helders PJ, Fischer K. Development and preliminary testing of a Paediatric Version of the Haemophilia Activities List (pedhal). Haemophilia 16(2),281– 289(2010).
Sherlock E, O’Donnell JS, White B, Blake C. Physical activity levels and participation in sport in Irish people with haemophilia. Haemophilia 16(1),e202– e209(2010).
Beeton K, De Kleijn P, Hilliard P et al. Recent developments in clinimetric instruments. Haemophilia 12(Suppl. 3),102– 107(2006).
Doria AS, Lundin B, Kilcoyne RF et al. Reliability of progressive and additive MRI scoring systems for evaluation of haemophilic arthropathy in children: expert MRI Working Group of the International Prophylaxis Study Group. Haemophilia 11(3),245– 253(2005).
Doria AS. State-of-the-art imaging techniques for the evaluation of haemophilic arthropathy: present and future. Haemophilia 16(Suppl. 5),107– 114(2010).
Melchiorre D, Linari S, Innocenti M et al. Ultrasound detects joint damage and bleeding in haemophilic arthropathy: a proposal of a score. Haemophilia 17(1),112– 117(2011).
Lythgoe DJ, Williams SC, Cullinane M, Markus HS. Mapping of cerebrovascular reactivity using BOLD magnetic resonance imaging. Magn. Reson. Imaging 17(4), 495– 502(1999).
Amirabadi A, Miller E, Gahunia H et al. Functional MRI techniques as early diagnostic markers in hemophilic arthropathy. Proceedings of XXII Congress of the International Society on Thrombosis and Haemostasis. J. Thromb. Hemost. 5(Suppl. 2) (2009)(Abstract PP-MO-18).
Doria AS, Kiss MH, Lotito AP et al. Juvenile rheumatoid arthritis of the knee: evaluation with contrast-enhanced color Doppler ultrasound. Pediatr. Radiol. 31(7),524– 531(2001).
Nieminen MT, Rieppo J, Silvennoinen J et al. Spatial assessment of articular cartilage proteoglycans with Gd-DTPA-enhanced T1 imaging. Magn. Reson. Med. 48(4),640– 648(2002).
Dardzinski BJ, Mosher TJ, Li S, Van Slyke MA, Smith MB. Spatial variation of T2 in human articular cartilage. Radiology 205(2),546– 550(1997).
Patonay L, Nagy K, Engelke W. Real-time endoarticular ultrasound imaging of the TMJ – a new diagnostic possibility? A cadaver study. Int. J. Oral Maxillofac. Surg. 31(5),553– 557(2002).
Jansen NW, Roosendaal G, Lundin B et al. The combination of the biomarkers urinary C-terminal telopeptide of type II collagen, serum cartilage oligomeric matrix protein, and serum chondroitin sulfate 846 reflects cartilage damage in hemophilic arthropathy. Arthritis Rheum. 60(1), 290– 298(2009).
Valentino LA, Kawji M, Grygotis M. Venous access in the management of hemophilia. Blood Rev. 25(1), 11– 15(2011).
Valentino LA, Ismael Y, Grygotis M. Novel drugs to treat hemophilia. Expert Opin. Emerg. Drugs 15(4), 597– 613(2010).
High KA. Gene therapy for haemophilia: a long and winding road. J. Thromb. Haemost. 9(Suppl. 1),2– 11(2011).
Pipe S. Visions in haemophilia care. Thromb. Res. 124(Suppl. 2),S2– S5(2009).
Pipe SW. Recombinant clotting factors. Thromb. Haemost. 99(5), 840– 850(2008).
Pipe SW. Hemophilia: new protein therapeutics. Hematology Am. Soc. Hematol. Educ. Program 2010,203– 209(2010).
Valentino LA. Recombinant FIXFc: a novel therapy for the royal disease? Expert Opin. Biol. Ther. 11(10), 1361– 1368(2011).
Taylor PC. Antibody therapy for rheumatoid arthritis. Curr. Opin. Pharmacol. 3(3),323– 328(2003).
Choy EH, Kingsley GH, Panayi GS. Monoclonal antibody therapy in rheumatoid arthritis. Br. J. Rheumatol. 37(5),484– 490(1998).
Avastin^®, prescribing information. Genentech Inc., South San Francisco, CA, USA (2009).

Website
201. Srivastava A, Giangrande P, Poon MC, Chua M, McCraw A, Wiedel J. Guidelines for the management of hemophilia. World Federation of Hemophilia. www.wfh.org/index.asp?lang=EN (Accessed 12 December 2011)
202. National Hemophilia Foundation. MASAC (Medical and Scientific Advisory Council) Recommendation 179. MASAC Recommendation Concerning Prophylaxis (Regular Administration of Clotting Factor Concentrate to Prevent Bleeding). www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&content (Accessed 4 November 2007)
203. Centers for Disease Control and Prevention. Summary report of UDC national activity. Treatment/clinical characteristics (hemophilia). www2a.cdc.gov/ncbddd/htcweb/UDC_Report/UDC_Report.asp (Accessed 3 December 2011)

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Author(s)

Mindy L. Simpson, MD

Department of Pediatrics, Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, Illinois

Disclosures

Disclosure: Mindy L. Simpson, MD, has disclosed no relevant financial relationships.

Leonard A. Valentino, MD

Department of Pediatrics, Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, Illinois

Disclosures

Disclosure: Leonard A. Valentino, MD, has disclosed no relevant financial relationships.

Editor

Elisa Manzotti

Publisher, Future Science Group, London, United Kingdom

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Disclosure: Elisa Manzotti has disclosed no relevant financial relationships.

CME Author(s)

Laurie Barclay, MD

Freelance writer and reviewer, Medscape, LLC

Disclosures

Disclosure: Laurie Barclay, MD, has disclosed no relevant financial relationships.

CME Reviewer(s)

Nafeez Zawahir, MD

CME Clinical Director, Medscape, LLC

Disclosures

Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

CME

Management of Joint Bleeding in Hemophilia

Authors: Mindy L. Simpson, MD; Leonard A. Valentino, MD
CME Released: 9/20/2012
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 9/20/2013, 11:59 PM EST

Start Activity

Target Audience and Goal Statement

This activity is intended for hematologists, radiologists, pediatricians, and other clinicians caring for patients with joint bleeding due to hemophilia.

The goal of this activity is to review the clinical presentation and management of hemarthrosis in patients with hemophilia.

Upon completion of this activity, participants will be able to:

Describe the clinical characteristics of joint bleeding in hemophilia
Describe prophylaxis to prevent joint bleeding in hemophilia
Describe management of acute hemarthrosis in patients with hemophilia

Disclosures

Author(s)

Mindy L. Simpson, MD

Department of Pediatrics, Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, Illinois

Disclosures

Disclosure: Mindy L. Simpson, MD, has disclosed no relevant financial relationships.

Leonard A. Valentino, MD

Department of Pediatrics, Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, Illinois

Disclosures

Disclosure: Leonard A. Valentino, MD, has disclosed no relevant financial relationships.

Editor

Elisa Manzotti

Publisher, Future Science Group, London, United Kingdom

Disclosures

Disclosure: Elisa Manzotti has disclosed no relevant financial relationships.

CME Author(s)

Laurie Barclay, MD

Freelance writer and reviewer, Medscape, LLC

Disclosures

Disclosure: Laurie Barclay, MD, has disclosed no relevant financial relationships.

CME Reviewer(s)

Nafeez Zawahir, MD

CME Clinical Director, Medscape, LLC

Disclosures

Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

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Abstract and Introduction

Abstract

Hemarthrosis, the hallmark of severe hemophilia, is the major cause of serious bleeding events, disability and reduced quality of life in patients with factor VIII or factor IX deficiency. Joint bleeding is one of the greatest challenges confronting individuals treating hemophilia, and its economic impact is enormous. This article reviews the current management of hemophilic joint bleeding and discusses the potential impact of novel therapies.

Introduction

Hemophilia is an X-linked inherited disease that affects an estimated 400,000 persons worldwide and is characterized by the deficiency or absence of coagulation factor (F) VIII (hemophilia A) or FIX (hemophilia B) that predisposes to bleeding.^[201] Hemophilia A occurs in approximately one in 5000 male births, whereas hemophilia B occurs in one in 30,000 male births.^[1] Hemophilia is categorized as severe (factor activity level: <1 IU/dl or <1%), moderate (factor activity level: 1–5%) or mild (factor activity level: >5–40%).^[2] However, FVIII or FIX activity level alone does not fully represent an individual’s bleeding tendency and other disease-modifying factors are under evaluation.

Page 1 of 10

FVIII & FIX Genetics

CME Information

Abstract and Introduction
FVIII & FIX Genetics
Evolution of Hemophilia Therapy
Mechanisms of Blood-induced Joint Disease
Rationale for Prophylaxis
Management of Acute Hemarthrosis
Outcome Measurement
Expert Commentary
Five-year View
Key Issues

References

References

Disclaimer

The material presented here does not necessarily reflect the views of Medscape, LLC, or companies that support educational programming on medscape.org. These materials may discuss therapeutic products that have not been approved by the US Food and Drug Administration and off-label uses of approved products. A qualified healthcare professional should be consulted before using any therapeutic product discussed. Readers should verify all information and data before treating patients or employing any therapies described in this educational activity.

References

Faculty and Disclosures

Author(s)

Mindy L. Simpson, MD

Leonard A. Valentino, MD

Editor

Elisa Manzotti

CME Author(s)

Laurie Barclay, MD

CME Reviewer(s)

Nafeez Zawahir, MD

CME

Management of Joint Bleeding in Hemophilia

Target Audience and Goal Statement

Disclosures

Author(s)

Mindy L. Simpson, MD

Leonard A. Valentino, MD

Editor

Elisa Manzotti

CME Author(s)

Laurie Barclay, MD

CME Reviewer(s)

Nafeez Zawahir, MD

Accreditation Statements

For Physicians

Instructions for Participation and Credit

Management of Joint Bleeding in Hemophilia

Abstract and Introduction

Abstract

Introduction

Table of Contents