scholarly journals Once‐weekly prophylactic treatment vs. on‐demand treatment with nonacog alfa in patients with moderately severe to severe haemophilia B

Haemophilia ◽  
2016 ◽  
Vol 22 (3) ◽  
pp. 381-388 ◽  
Author(s):  
K. Kavakli ◽  
L. Smith ◽  
K. Kuliczkowski ◽  
J. Korth‐Bradley ◽  
C. W. You ◽  
...  
Keyword(s):  
Prophylactic Treatment ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
On Demand

Epidemiology and Treatment of Patients with Haemophilia in Austria—Update from the Austrian Haemophilia Registry

2018 ◽  
Vol 39 (03) ◽  
pp. 284-293 ◽  
Author(s):  
Judit Rejtő ◽  
Sylvia Reitter-Pfoertner ◽  
Sylvia Kepa ◽  
Clemens Feistritzer ◽  
Michael Grundbichler ◽  
...  
Keyword(s):  
Prophylactic Treatment ◽  
Hiv Infections ◽  
Epidemiological Data ◽  
Treatment Modalities ◽  
Hiv Positive ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Immunodeficiency Virus ◽  
Mild Haemophilia

AbstractThe Austrian Haemophilia Registry collects epidemiological data on patients with haemophilia, on treatment modalities and potential side effects. The Registry covers more than 85% of the assumed total number of haemophilia patients in Austria. This report summarizes data on 753 patients: 84.3% (635) have haemophilia A and 15.7% (118) have haemophilia B. Patients' median age is 34 years (range: 1–93 years). Of the total cohort, 39.0% (294) patients have severe haemophilia, 11.3% (85) moderate haemophilia, and 49.4% (372) mild haemophilia. Of the patients with severe haemophilia, 38.4% (113) have been infected with hepatitis C virus (HCV) and 12.6% (37) are human immunodeficiency virus (HIV) positive. Overall, 10.6% (67) of patients with haemophilia A and 1.7% (2) of those with haemophilia B have had an inhibitor in their history. Among patients with severe haemophilia, 68.4% (201) receive prophylaxis and 28.6% (84) receive on-demand therapy. There are 65.0% (191) patients with severe haemophilia who are treated with recombinant products. In conclusion, most patients with severe haemophilia receive prophylactic treatment. HCV and HIV infections are still important issues in the Austrian haemophilia population.

Consequences of switching from prophylactic treatment to on-demand treatment in late teens and early adults with severe haemophilia A: the TEEN/TWEN study

Haemophilia ◽  
2013 ◽  
Vol 19 (5) ◽  
pp. 727-735 ◽  
Author(s):  
M. J. Manco-Johnson ◽  
J. Sanders ◽  
N. Ewing ◽  
N. Rodriguez ◽  
M. Tarantino ◽  
...  
Keyword(s):  
Prophylactic Treatment ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand

scholarly journals Willingness to pay for on-demand and prophylactic treatment for severe haemophilia in Sweden

Haemophilia ◽  
2004 ◽  
Vol 10 (5) ◽  
pp. 527-541 ◽  
Author(s):  
K. Steen Carlsson ◽  
S. Hojgard ◽  
S. Lethagen ◽  
A. Lindgren ◽  
E. Berntorp ◽  
...  
Keyword(s):  
Willingness To Pay ◽  
Prophylactic Treatment ◽  
Severe Haemophilia ◽  
On Demand

scholarly journals Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden

Haemophilia ◽  
2004 ◽  
Vol 10 (5) ◽  
pp. 515-526 ◽  
Author(s):  
K. STEEN Carlsson ◽  
S. Hojgard ◽  
A. Lindgren ◽  
S. Lethagen ◽  
S. Schulman ◽  
...  
Keyword(s):  
Prophylactic Treatment ◽  
Severe Haemophilia ◽  
On Demand

scholarly journals On-demand vs. prophylactic treatment for severe haemophilia in Norway and Sweden: differences in treatment characteristics and outcome

Haemophilia ◽  
2003 ◽  
Vol 9 (5) ◽  
pp. 555-566 ◽  
Author(s):  
K. Steen Carlsson ◽  
S. Höjgård ◽  
A. Glomstein ◽  
S. Lethagen ◽  
S. Schulman ◽  
...  
Keyword(s):  
Prophylactic Treatment ◽  
Severe Haemophilia ◽  
On Demand ◽  
Treatment Characteristics

Haemophilia registry of the Medical Committee of the Swiss Hemophilia Society

2012 ◽  
Vol 32 (S 01) ◽  
pp. S20-S24 ◽  
Author(s):  
N. von der Weid
Keyword(s):  
Quality Control ◽  
Young Adults ◽  
Prophylactic Treatment ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Coagulation Disorders ◽  
On Demand ◽  
Viii And Ix ◽  
Year 2000 ◽  
Low Rate

SummaryThe Haemophilia Registry of the Swiss Haemo philia Society was created in the year 2000. The latest records from October 31st 2011 are presented here. Included are all patients with haemophilia A or B and other inherited coagulation disorders (including VWD patients with R-Co activity below 10%) known and followed by the 11 paediatric and 12 adult haemophilia treatment or reference centers. Currently there are 950 patients registered, the majority of which (585) having haemophilia A. Disease severity is graded according to ISTH criteria and its distribution between mild, moderate and severe haemophilia is similar to data from other European and American registries. The majority (about two thirds) of Swiss patients with haemophilia A or B are treated on-demand, with only about 20% of patients being on prophylaxis. The figure is different in paediatrics and young adults (1st and 2nd decades), where 80 to 90% of patients with haemophilia A are under regular prophylaxis. Interestingly enough, use of factor concentrates, although readily available, is rather low in Switzerland, especially when taking the country’s GDP into account: The total amount of factor VIII and IX was 4.94 U pro capita, comparable to other European countries with distinctly lower incomes (Poland, Slovakia, Hungary). This finding is mainly due to the afore mentioned low rate of prophylactic treatment of haemophilia in our country. Our registry remains an important instrument of quality control of haemophilia therapy in Switzerland.

Performances of an Artificial Reagent for the One-stage Factor IX Assay

1975 ◽  
Vol 33 (03) ◽  
pp. 547-552 ◽  
Author(s):  
L Meunier ◽  
J. P Allain ◽  
D Frommel
Keyword(s):  
Human Plasma ◽  
Factor Ix ◽  
Severe Haemophilia ◽  
Normal Human Plasma ◽  
Haemophilia B ◽  
One Stage ◽  
Normal Human ◽  
The One

SummaryA mixture of adsorbed normal human plasma and chicken plasma was prepared as reagent for factor IX measurement using a one-stage method. The substrate was found to be specific for factor IX. Its performances tested on samples displaying factor IX activity ranging from <l%–2,500% compared favorably with those obtained when using the plasma of severe haemophilia B patients as substrate.

Management of Haemophilia in Sweden

1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson
Keyword(s):  
Factor Viii ◽  
Total Population ◽  
Age Groups ◽  
Factor Ix ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Human Fraction ◽  
Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

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