Guidelines for the management of hemophilia.

doi:10.1111/J.1365-2516.2012.02909.X

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Guidelines for the management of hemophilia.

Journal Article•DOI•

Guidelines for the management of hemophilia.

A. Srivastava¹, A. K. Brewer, Evelien P. Mauser-Bunschoten², Nigel S. Key³, Steve Kitchen⁴, Adolfo Llinás⁵, C. A. Ludlam, Johnny Mahlangu⁶, K. Mulder, M.-C. Poon⁷, Alison Street⁸ - Show less +7 more•Institutions (8)

Christian Medical College & Hospital¹, Utrecht University², University of North Carolina at Chapel Hill³, Royal Hallamshire Hospital⁴, University of Los Andes⁵, University of the Witwatersrand⁶, University of Calgary⁷, Alfred Hospital⁸

01 Jan 2013-Haemophilia (Haemophilia)-Vol. 19, Iss: 1

TL;DR: These evidence‐based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion‐transmitted infections.

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Abstract: Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.

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Journal Article•DOI•

Management of severe perioperative bleeding Guidelines from the European Society of Anaesthesiology

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Sibylle A. Kozek-Langenecker, Arash Afshari¹, Pierre Albaladejo², Cesar Aldecoa Alvarez Santullano³, Edoardo De Robertis⁴, Daniela Filipescu, Dietmar Fries⁵, Thorsten Haas, Georgina Imberger, Matthias Jacob⁶, Marcus D. Lancé⁷, Juan V. Llau⁸, Susan Mallett, Jens Meier, Niels Rahe-Meyer, Charles Marc Samama, Andrew Smith⁹, Cristina Solomon¹⁰, Philippe Van der Linden, Anne Wikkelsø¹, Patrick Wouters, Piet Wyffels - Show less +18 more•Institutions (10)

University of Copenhagen¹, Centre Hospitalier Universitaire de Grenoble², University of Valladolid³, University of Naples Federico II⁴, Innsbruck Medical University⁵, Ludwig Maximilian University of Munich⁶, Maastricht University⁷, University of Valencia⁸, Royal Lancaster Infirmary⁹, University of Salzburg¹⁰

01 Jun 2013-European Journal of Anaesthesiology

TL;DR: These guidelines are intended to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthetists throughout Europe to integrate this knowledge into daily patient care wherever possible.

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Abstract: The aims of severe perioperative bleeding management are three-fold. First, preoperative identification by anamesis and laboratory testing of those patients for whom the perioperative bleeding risk may be increased. Second, implementation of strategies for correcting preoperative anaemia and stabilisation of the macro- and microcirculations in order to optimise the patient’s tolerance to bleeding. Third, targeted procoagulant interventions to reduce the amount of bleeding, morbidity, mortality and costs. The purpose of these guidelines is to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthetists throughout Europe to integrate this knowledge into daily patient care wherever possible. The Guidelines Committee of the European Society of Anaesthesiology (ESA) formed a task force with members of scientific subcommittees and individual expert members of the ESA. Electronic databases were searched without language restrictions from the year 2000 until 2012. These searches produced 20 664 abstracts. Relevant systematic reviews with meta-analyses, randomised controlled trials, cohort studies, case-control studies and cross-sectional surveys were selected. At the suggestion of the ESA Guideline Committee, the Scottish Intercollegiate Guidelines Network (SIGN) grading system was initially used to assess the level of evidence and to grade recommendations. During the process of guideline development, the official position of the ESA changed to favour the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. This report includes general recommendations as well as specific recommendations in various fields of surgical interventions. The final draft guideline was posted on the ESA website for four weeks and the link was sent to all ESA members. Comments were collated and the guidelines amended as appropriate. When the final draft was complete, the Guidelines Committee and ESA Board ratified the guidelines.

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883 citations

Journal Article•DOI•

WFH Guidelines for the Management of Hemophilia, 3rd edition

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Alok Srivastava¹, Elena Santagostino, Alison Dougall², Steve Kitchen, Megan Sutherland³, Steven W. Pipe⁴, Manuel Carcao⁵, Johnny Mahlangu⁶, Margaret V. Ragni⁷, Jerzy Windyga, Adolfo Llinás⁸, Nicholas J. Goddard⁹, Richa Mohan, Pradeep M. Poonnoose¹, Brian M. Feldman⁵, Sandra Zelman Lewis, H. Marijke van den Berg, Glenn F. Pierce - Show less +14 more•Institutions (9)

Christian Medical College & Hospital¹, Trinity College, Dublin², University of Manchester³, University of Michigan⁴, University of Toronto⁵, National Health Laboratory Service⁶, University of Pittsburgh⁷, University of Los Andes⁸, Royal Free Hospital⁹

03 Aug 2020-Haemophilia

TL;DR: The WFH Guidelines for the Management of Hemophilia panelists and co-authors thank the panelists for their time and share their views on how to better understand and treat hemophilia.

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751 citations

Journal Article•DOI•

Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology: First update 2016.

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Sibylle A. Kozek-Langenecker, Aamer B Ahmed¹, Arash Afshari², Pierre Albaladejo³, Cesar Aldecoa⁴, Guidrius Barauskas, Edoardo De Robertis⁵, David Faraoni⁶, Daniela Filipescu, Dietmar Fries, Thorsten Haas, Matthias Jacob, Marcus D. Lancé⁷, Juan V.L. Pitarch, Susan Mallett, Jens Meier, Zsolt Molnár⁸, Niels Rahe-Meyer, Charles Marc Samama, Jakob Stensballe², Philippe Van der Linden, Anne Wikkelsø, Patrick Wouters, Piet Wyffels, Kai Zacharowski⁹ - Show less +21 more•Institutions (9)

Glenfield Hospital¹, Copenhagen University Hospital², Centre Hospitalier Universitaire de Grenoble³, University of Valladolid⁴, University of Naples Federico II⁵, Boston Children's Hospital⁶, Maastricht University⁷, University of Szeged⁸, Goethe University Frankfurt⁹

01 Jun 2017-European Journal of Anaesthesiology

TL;DR: This update includes revisions to existing recommendations with respect to the wording, or changes in the grade of recommendation, and also the addition of new recommendations.

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Abstract: The management of perioperative bleeding involves multiple assessments and strategies to ensure appropriate patient care. Initially, it is important to identify those patients with an increased risk of perioperative bleeding. Next, strategies should be employed to correct preoperative anaemia and to

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613 citations

Journal Article•DOI•

Hemophilia B Gene Therapy with a High-Specific-Activity Factor IX Variant.

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Lindsey A. George¹, Spencer K. Sullivan², Adam Giermasz³, John E.J. Rasko⁴, John E.J. Rasko⁵, Benjamin J. Samelson-Jones⁶, Jonathan M. Ducore⁷, Adam Cuker⁸, Lisa M. Sullivan, Suvankar Majumdar², Jerome M. Teitel⁶, Catherine E. McGuinn⁷, Margaret V. Ragni⁸, Alvin Luk, Daniel Hui, J. Fraser Wright, Yifeng Chen, Yun Liu, Katie Wachtel, Angela Winters¹, Stefan Tiefenbacher, Valder R. Arruda¹, Johannes C.M. Van der Loo¹, Olga Zelenaia¹, Daniel Takefman, Marcus E. Carr, Linda B. Couto, Xavier M. Anguela, Katherine A. High - Show less +25 more•Institutions (8)

University of Pennsylvania¹, University of Mississippi², University of California, Davis³, Centenary Institute⁴, University of Sydney⁵, University of Toronto⁶, Cornell University⁷, University of Pittsburgh⁸

06 Dec 2017-The New England Journal of Medicine

TL;DR: Transgene‐derived factor IX coagulant activity enabled the termination of baseline prophylaxis and the near elimination of bleeding and factor use in 10 participants with hemophilia who received the same vectors.

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Abstract: BackgroundThe prevention of bleeding with adequately sustained levels of clotting factor, after a single therapeutic intervention and without the need for further medical intervention, represents an important goal in the treatment of hemophilia. MethodsWe infused a single-stranded adeno-associated viral (AAV) vector consisting of a bioengineered capsid, liver-specific promoter and factor IX Padua (factor IX–R338L) transgene at a dose of 5×1011 vector genomes per kilogram of body weight in 10 men with hemophilia B who had factor IX coagulant activity of 2% or less of the normal value. Laboratory values, bleeding frequency, and consumption of factor IX concentrate were prospectively evaluated after vector infusion and were compared with baseline values. ResultsNo serious adverse events occurred during or after vector infusion. Vector-derived factor IX coagulant activity was sustained in all the participants, with a mean (±SD) steady-state factor IX coagulant activity of 33.7±18.5% (range, 14 to 81). On cumu...

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491 citations

Journal Article•DOI•

AAV5–Factor VIII Gene Transfer in Severe Hemophilia A

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Savita Rangarajan, Liron Walsh, Will Lester, David Perry, Bella Madan, Michael Laffan, Hua Yu, Christian Vettermann, Glenn F. Pierce, Wing Yen Wong, K. John Pasi - Show less +7 more

09 Dec 2017-The New England Journal of Medicine

TL;DR: The infusion of AAV5‐hFVIII‐SQ was associated with the sustained normalization of factor VIII activity level over a period of 1 year in six of seven participants who received a high dose, with stabilization of hemostasis and a profound reduction in factor VIII use in all seven participants.

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Abstract: BackgroundPatients with hemophilia A rely on exogenous factor VIII to prevent bleeding in joints, soft tissue, and the central nervous system. Although successful gene transfer has been reported in patients with hemophilia B, the large size of the factor VIII coding region has precluded improved outcomes with gene therapy in patients with hemophilia A. MethodsWe infused a single intravenous dose of a codon-optimized adeno-associated virus serotype 5 (AAV5) vector encoding a B-domain–deleted human factor VIII (AAV5-hFVIII-SQ) in nine men with severe hemophilia A. Participants were enrolled sequentially into one of three dose cohorts (low dose [one participant], intermediate dose [one participant], and high dose [seven participants]) and were followed through 52 weeks. ResultsFactor VIII activity levels remained at 3 IU or less per deciliter in the recipients of the low or intermediate dose. In the high-dose cohort, the factor VIII activity level was more than 5 IU per deciliter between weeks 2 and 9 after ...

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452 citations

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Clinical and Laboratory Standards Institute.

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Usa Pennsylvania

01 Jan 2013

4,737 citations

Journal Article•DOI•

Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.

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Marilyn J. Manco-Johnson¹, Marilyn J. Manco-Johnson², Thomas C. Abshire³, Amy D. Shapiro, Brenda Riske¹, Michele R. Hacker⁴, Ray F. Kilcoyne¹, J. David Ingram², M L Manco-Johnson¹, Sharon Funk¹, Linda J. Jacobson¹, Leonard A. Valentino², W. Keith Hoots⁵, George R. Buchanan⁶, Donna DiMichele⁷, Michael Recht², Deborah L Brown⁵, Cindy A. Leissinger⁸, Shirley Bleak⁹, Alan R. Cohen¹⁰, Prasad Mathew¹¹, Alison Matsunaga¹², Desiree Medeiros¹³, Diane J. Nugent¹⁴, Gregory Thomas¹⁵, Alexis A. Thompson¹⁶, Kevin McRedmond¹⁷, J. Michael Soucie¹⁸, Harlan Austin¹⁸, Bruce L. Evatt¹⁸ - Show less +26 more•Institutions (18)

University of Colorado Denver¹, Boston Children's Hospital², Emory University³, Harvard University⁴, University of Texas at Austin⁵, University of Texas Southwestern Medical Center⁶, Cornell University⁷, Tulane University⁸, Primary Children's Hospital⁹, University of Pennsylvania¹⁰, University of New Mexico¹¹, Children's Hospital Oakland Research Institute¹², University of Hawaii at Manoa¹³, Children's Hospital of Orange County¹⁴, Oregon Health & Science University¹⁵, Children's Memorial Hospital¹⁶, Palmetto Health Richland¹⁷, Centers for Disease Control and Prevention¹⁸

09 Aug 2007-The New England Journal of Medicine

TL;DR: Prophylaxis with recombinant factor VIII can prevent joint damage and decrease the frequency of joint and other hemorrhages in young boys with severe hemophilia A.

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Abstract: Sixty-five boys younger than 30 months of age were randomly assigned to prophylaxis (32 boys) or enhanced episodic therapy (33 boys). When the boys reached 6 years of age, 93% of those in the prophylaxis group and 55% of those in the episodic-therapy group were considered to have normal index-joint structure on MRI (P = 0.006). The relative risk of MRI-detected joint damage with episodic therapy as compared with prophylaxis was 6.1 (95% confidence interval, 1.5 to 24.4). The mean annual numbers of joint and total hemorrhages were higher at study exit in the episodic-therapy group than in the prophylaxis group (P<0.001 for both comparisons). High titers of inhibitors of factor VIII developed in two boys who received prophylaxis; three boys in the episodic-therapy group had a life-threatening hemorrhage. Hospitalizations and infections associated with central-catheter placement did not differ significantly between the two groups. Conclusions Prophylaxis with recombinant factor VIII can prevent joint damage and decrease the frequency of joint and other hemorrhages in young boys with severe hemophilia A. (ClinicalTrials.gov number, NCT00207597.)

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1,613 citations

"Guidelines for the management of he..." refers background in this paper

...A dental evaluation is advisable before initiating long-term bisphosphonate therapy [28,29]....
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...(Level 2) [24-29]...
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...[26,29,30]...
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Journal Article•DOI•

Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B

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Inga Marie Nilsson¹, Erik Berntorp¹, Thomas Löfqvist¹, Holger Pettersson•Institutions (1)

Lund University¹

01 Jul 1992-Journal of Internal Medicine

TL;DR: It appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1% of normal.

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Abstract: In Sweden, prophylactic treatment of boys with severe haemophilia has been practised since 1958 in an attempt to convert the disease from a severe to a milder form. The present study population consisted of 60 severe haemophiliacs (52 A, 8 B), aged 3-32 years. Treatment is started when the boys are 1-2 years of age, the regimens used being 24-40 IU F VIII kg-1 three times weekly in haemophilia-A cases (i.e. greater than 2000 IU kg-1 annually) and 25-40 IU F IX kg-1 twice weekly in haemophilia-B cases. The orthopaedic and radiological joint scores (maximum scores of 90 and 78, respectively) are evaluated as recommended by the World Federation of Haemophilia. Of those subjects aged 3-17 years, 29 out of 35 individuals had joint scores of zero. The oldest group had only minor joint defects. The VIII:C and IX:C concentrations had usually not fallen below 1% of normal. All 60 patients are able to lead normal lives. In conclusion, it appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1% of normal.

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928 citations

Journal Article•DOI•

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.

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Gilbert C. White¹, Frits R. Rosendaal, Louis M. Aledort, Jeanne M. Lusher, Chantal Rothschild, Jørgen Ingerslev - Show less +2 more•Institutions (1)

University of North Carolina at Chapel Hill¹

01 Mar 2001-Thrombosis and Haemostasis

TL;DR: Definitions in Hemophilia - Recommendation of the Scientific Subcommittee on Factor VIII and Factor IX and the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis.

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Abstract: 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

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797 citations

"Guidelines for the management of he..." refers background in this paper

...5 Bleeding manifestations 5 1.2 PRINCIPLES OF CARE 5 1.3 COMPREHENSIVE CARE 6 Comprehensive care team 6 Functions of a comprehensive care program 7 1.4 FITNESS AND PHYSICAL ACTIVITY 8 1.5 ADJUNCTIVE MANAGEMENT 8 1.6 PROPHYLACTIC FACTOR REPLACEMENT THERAPY 8 Administration and dosing schedules 9 1.7 HOME THERAPY 9 1.8 MONITORING HEALTH STATUS AND OUTCOME 10 1.9 PAIN MANAGEMENT 10 Pain caused by venous access 10 Pain caused by joint or muscle bleeding 10 Postoperative pain 10 Pain due to chronic hemophilic arthropathy 10 1.10 SURGERY AND INVASIVE PROCEDURES 11 1.11 DENTAL CARE AND MANAGEMENT 11 REFERENCES 12 2 SPECIAL MANAGEMENT ISSUES 14 2.1 CARRIERS 14 2.2 GENETIC TESTING/COUNSELING AND PRENATAL DIAGNOSIS 14 2.3 DELIVERY OF INFANTS WITH KNOWN OR SUSPECTED HEMOPHILIA 14 2.4 VACCINATIONS 15 2.5 PSYCHOSOCIAL ISSUES 15 2.6 SEXUALITY 15 2.7 AGING HEMOPHILIA PATIENTS 15 Osteoporosis 16 Obesity 16 Hypertension 16 Diabetes Mellitus (DM) 16 Hypercholesterolemia 16 Cardiovascular disease 16 Psychosocial Impact 17 2.8 VON WILLEBRAND DISEASE/RARE BLEEDING DISORDERS 17 REFERENCES 17 3 LABORATORY DIAGNOSIS 19 3.1 KNOWLEDGE AND EXPERTISE IN COAGULATION LABORATORY TESTING 19 Principles of diagnosis 19 Technical aspects 19 3.2 USE OF THE CORRECT EQUIPMENT AND REAGENTS 21 Equipment 21 Reagents 22 3.3 QUALITY ASSURANCE 22 Internal quality control (IQC) 22 External quality assessment (EQA) 22 REFERENCES 23 4 HEMOSTATIC AGENTS 24 4.1 CLOTTING FACTOR CONCENTRATES 24 Product selection 24 FVIII concentrates 25 FIX concentrates 25 4.2 OTHER PLASMA PRODUCTS 26 Fresh frozen plasma (FFP) 26 Cryoprecipitate 27 4.3 OTHER PHARMACOLOGICAL OPTIONS 27 Desmopressin (DDAVP) 27 Tranexamic acid 28 Epsilon aminocaproic acid 28 REFERENCES 29 5 TREATMENT OF SPECIFIC HEMORRHAGES 30 5.1 JOINT HEMORRHAGE (HEMARTHROSIS) 30 Arthrocentesis 31 5.2 MUSCLE HEMORRHAGE 31 Iliopsoas hemorrhage 32 5.3 CENTRAL NERVOUS SYSTEM HEMORRHAGE/HEAD TRAUMA 32 (continued) Haemophilia (2013), 19, e1–e47 © 2012 Blackwell Publishing Ltd 5.4 THROAT AND NECK HEMORRHAGE 32 5.5 ACUTE GASTROINTESTINAL (GI) HEMORRHAGE 32 5.6 ACUTE ABDOMINAL HEMORRHAGE 32 5.7 OPHTHALMIC HEMORRHAGE 33 5.8 RENAL HEMORRHAGE 33 5.9 ORAL HEMORRHAGE 33 5.10 EPISTAXIS 33 5.11 SOFT TISSUE HEMORRHAGE 33 5.12 LACERATIONS AND ABRASIONS 34 REFERENCES 34 6 COMPLICATIONS OF HEMOPHILIA 35 6.1 MUSCULOSKELETAL COMPLICATIONS 35 Synovitis 35 Chronic hemophilic arthropathy 36 Principles of physiotherapy/physical medicine in hemophilia 36 Pseudotumors 37 Fractures 37 Principles of orthopedic surgery in hemophilia 37 6.2 INHIBITORS 38 Management of bleeding 39 Allergic reactions in patients with hemophilia B 39 Immune tolerance induction 39 Patients switching to new concentrates 39 6.3 TRANSFUSION-TRANSMITTED AND OTHER INFECTION-RELATED COMPLICATIONS 40 Principles of management of HIV infection in hemophilia 40 Principles of management of HCV infection in hemophilia 40 Principles of management of HBV infection in hemophilia 40 Principles of management of bacterial infection in hemophilia 41 REFERENCES 41 7 PLASMA FACTOR LEVEL AND DURATION OF ADMINISTRATION 44 7.1 CHOICE OF FACTOR REPLACEMENT THERAPY PROTOCOLS 44 REFERENCES 44 Tables 1-1 RELATIONSHIP OF BLEEDING SEVERITY WITH CLOTTING FACTOR LEVEL 5 1-2 SITES OF BLEEDING IN HEMOPHILIA 5 1-3 APPROXIMATE FREQUENCY OF BLEEDING AT DIFFERENT SITES 5 1-4 DEFINITIONS OF FACTOR REPLACEMENT THERAPY PROTOCOLS 8 1-5 STRATEGIES FOR PAIN MANAGEMENT IN PATIENTS WITH HEMOPHILIA 11 1-6 DEFINITION OF ADEQUACY OF HEMOSTASIS FOR SURGICAL PROCEDURES 11 3-1 INTERPRETATION OF SCREENING TESTS 20 5-1 DEFINITION OF RESPONSE TO TREATMENT OF ACUTE HEMARTHROSIS 30 7-1 SUGGESTED PLASMA FACTOR PEAK LEVEL AND DURATION OF ADMINISTRATION (WHEN THERE IS NO SIGNIFICANT RESOURCE CONSTRAINT) 45 7-2 SUGGESTED PLASMA FACTOR PEAK LEVEL AND DURATION OF ADMINISTRATION (WHEN THERE IS SIGNIFICANT RESOURCE CONSTRAINT) 45 © 2012 Blackwell Publishing Ltd Haemophilia (2013), 19, e1–e47 Introduction The first edition of these guidelines, published in 2005 by the World Federation of Hemophilia (WFH), served its purpose of being a useful document for those looking for basic information on the comprehensive management of hemophilia....
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...(Level 3) [4-6] TABLE 1-1: RELATIONSHIP OF BLEEDING SEVERITY TO CLOTTING FACTOR LEVEL [62]...
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Journal Article•DOI•

Guidelines for the use of fresh-frozen plasma, cryoprecipitate and cryosupernatant.

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D F O'Shaughnessy¹, C Atterbury², P. Bolton Maggs, Michael F. Murphy, Dafydd Thomas³, S Yates¹, Lorna M. Williamson - Show less +3 more•Institutions (3)

University of Southampton¹, Lynn University², Morriston Hospital³

01 Jul 2004-British Journal of Haematology

TL;DR: Fresh‐frozen plasma (FFP), cryoprecipitate and cryosupernatant plasma are very limited and should not be used to reverse warfarin anticoagulation in the absence of severe bleeding, and PRP may be used as an alternative to FFP.

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Abstract: The indications for transfusing fresh-frozen plasma (FFP), cryoprecipitate and cryosupernatant plasma are very limited. When transfused they can have unpredictable adverse effects. The risks of transmitting infection are similar to those of other blood components unless a pathogen-reduced plasma (PRP) is used. Of particular concern are allergic reactions and anaphylaxis, transfusion-related acute lung injury, and haemolysis from transfused antibodies to blood group antigens, especially A and B. FFP is not indicated in disseminated intravascular coagulation without bleeding, is only recommended as a plasma exchange medium for thrombotic thrombocytopenic purpura (for which cryosupernatant is a possible alternative), should never be used to reverse warfarin anticoagulation in the absence of severe bleeding, and has only a very limited place in prophylaxis prior to liver biopsy. When used for surgical or traumatic bleeding, FFP and cryoprecipitate doses should be guided by coagulation studies, which may include near-patient testing. FFP is not indicated to reverse vitamin K deficiency for neonates or patients in intensive care units. PRP may be used as an alternative to FFP. In the UK, PRP from countries with a low bovine spongiform encephalopathy incidence is recommended by the Departments of Health for children born after 1 January 1996. Arrangements for limited supplies of single donor PRP of non-UK origin are expected to be completed in 2004. Batched pooled commercially prepared PRP from donors in the USA (Octaplas) is licensed and available in the UK. FFP must be thawed using a technique that avoids risk of bacterial contamination. Plastic packs containing any of these plasma products are brittle in the frozen state and must be handled with care.

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786 citations

"Guidelines for the management of he..." refers background in this paper

...Patients and their families should be provided with psychological and social support [21,22]....
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...Prophylaxis was conceived from the observation that moderate hemophilia patients with clotting factor level >1 IU/dl seldom experience spontaneous bleeding and have much better preservation of joint function [21-24]....
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