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26 records – page 1 of 3.

Alterations in T-lymphocyte subsets among Danish haemophiliacs: relation to source of factor VIII preparations and high dose factor VIII treatment.

https://arctichealth.org/en/permalink/ahliterature39856
Source
Scand J Haematol. 1984 May;32(5):544-51
Publication Type
Article
Date
May-1984
Author
J. Gerstoft
K. Bentsen
E. Scheibel
J. Dalsgård-Nielsen
J. Gormsen
E. Dickmeiss
Source
Scand J Haematol. 1984 May;32(5):544-51
Date
May-1984
Language
English
Publication Type
Article
Keywords
Adolescent
Adult
Alanine Transaminase - metabolism
Antibodies, Viral - analysis
Child
Child, Preschool
Cytomegalovirus - isolation & purification
Denmark
Drug Industry
Europe
Factor VIII - therapeutic use
Hemophilia A - immunology
Humans
Immunoglobulin G - analysis
Infant
Liver - enzymology
Middle Aged
T-Lymphocytes, Helper-Inducer - classification
T-Lymphocytes, Regulatory - classification
United States
Abstract
Screening of 43 healthy Danish haemophiliacs revealed a significantly lower helper/suppressor (H/S) ratio than in controls. 8 of the haemophiliacs had an H/S ratio less than or equal to 1.0. A significant negative correlation occurred between the total lifetime factor VIII treatment and the H/S ratio. However, high-dose factor VIII treatment given to patients with antibodies against factor VIII was not associated with immunological abnormalities. Children had a significantly higher H/S ratio than the adult haemophiliacs. Patients exclusively treated with Danish cryoprecipitate during the last year had a significantly higher H/S ratio than patients receiving preparations from other sources. This difference might, however, be explained by lower age and lower total lifetime dose in the group receiving Danish preparations. Haemophiliacs treated with American preparations did not differ immunologically from those treated with preparations of other origin. Total serum IgG was increased in 23% of the patients. This parameter was negatively correlated with the H/S ratio. The possible relation of the observed immunological alterations among otherwise healthy haemophiliacs to the acquired immune deficiency syndrome warrants further attention.
PubMed ID
6328639 View in PubMed
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A Canadian survey of self-infusion practices in persons with haemophilia A.

https://arctichealth.org/en/permalink/ahliterature119396
Source
Haemophilia. 2012 Nov;18(6):e403-5
Publication Type
Article
Date
Nov-2012

[Cases of hemophilia in the Karolinska Hospital Pediatric Clinic]

https://arctichealth.org/en/permalink/ahliterature44572
Source
Lakartidningen. 1968 Mar 6;65(10):1033-6
Publication Type
Article
Date
Mar-6-1968

Clinical experience of prophylactic treatment in von Willebrand disease.

https://arctichealth.org/en/permalink/ahliterature82463
Source
Thromb Res. 2006;118 Suppl 1:S9-11
Publication Type
Article
Date
2006
Author
Lethagen Stefan
Author Affiliation
Copenhagen Hemophilia Center, Rigshospitalet, Afs. 5002, Blegdamsvej 9, DK-2100 Copenhagen, Denmark. stefan.lethagen@rh.dk
Source
Thromb Res. 2006;118 Suppl 1:S9-11
Date
2006
Language
English
Publication Type
Article
Keywords
Adolescent
Adult
Aged
Child
Clinical Protocols
Factor VIII - therapeutic use
Female
Hemarthrosis - etiology - prevention & control
Hemorrhage - etiology - prevention & control
Humans
Male
Middle Aged
Premedication
Quality of Life
Retrospective Studies
Sweden
von Willebrand Disease - complications - drug therapy
Abstract
The prophylactic treatment of bleeding in von Willebrand disease (vWD) has a long history in Sweden, having been first initiated in the late 1950s. The clinical experience of the prophylaxis of vWD in Sweden is described in the current review of a retrospective study of 37 patients from three haemophilia centres receiving prophylaxis for vWD. Prophylaxis with a plasma concentrate (fraction I-0 or Factor VIII) effectively reduced the median number of bleeds per year, prevented joint arthropathy in those who started prophylaxis at a young age, and improved the quality of life of all patients with vWD, particularly those with type 3 disease. This study demonstrates that the long-term prophylactic treatment with von Willebrand Factor (vWF)/FVIII concentrate (Haemate-P, ZLB Behring) in vWD is warranted in the majority of patients with type 3 disease.
PubMed ID
16616322 View in PubMed
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Cryoprecipitate use in 25 Canadian hospitals: commonly used outside of the published guidelines.

https://arctichealth.org/en/permalink/ahliterature155364
Source
Transfusion. 2008 Oct;48(10):2122-7
Publication Type
Article
Date
Oct-2008
Author
Edward C Alport
Jeannie L Callum
Susan Nahirniak
Bernie Eurich
Heather A Hume
Author Affiliation
Canadian Blood Services, Regina, Saskatchewan, Canada. jeannie.callum@sunnybrook.ca
Source
Transfusion. 2008 Oct;48(10):2122-7
Date
Oct-2008
Language
English
Publication Type
Article
Keywords
Blood Banks - standards - statistics & numerical data
Blood Component Transfusion - standards - statistics & numerical data
Canada
Factor VIII - therapeutic use
Fibrinogen - therapeutic use
Guideline Adherence - standards - statistics & numerical data
Hospitals - standards - statistics & numerical data
Humans
Medical Audit
Practice Guidelines as Topic
Prospective Studies
Abstract
Canadian Blood Services' disposition reports suggested considerable variation in cryoprecipitate use and prompted this national audit.
Thirty-one institutions were invited to participate in a 2-month audit. Patient information and relevant laboratory and transfusion data were collected. Cryoprecipitate transfusions were categorized as appropriate if a fibrinogen level (taken 6 hr before/after transfusion) was not more than 1.0 g per L and inappropriate if the pretransfusion fibrinogen level was more than 1.0 g per L and posttransfusion fibrinogen level was more than 1.0 g per L or not performed. Appropriateness was categorized as undetermined if the pretransfusion fibrinogen level was not performed and the posttransfusion fibrinogen level was more than 1.0 g per L or not performed.
Overall, 25 of 31 invited hospitals agreed to participate. A total of 4370 units of cryoprecipitate were transfused in 603 events to 453 patients representing 62 percent of cryoprecipitate issued to hospitals during the time period. Comparison of the number of units of cryoprecipitate per 100 units of red blood cells (RBCs) transfused by each institution showed significant variation in practice (mean, 9 per 100 RBCs; range, 2 to 27 units). The single most common indication for cryoprecipitate was cardiac surgery (45.4% of events). Overall, 24 percent of cryoprecipitate transfusions were considered to be appropriate (pretransfusion fibrinogen level
PubMed ID
18764824 View in PubMed
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The diagnosis and management of von Willebrand disease in Canada.

https://arctichealth.org/en/permalink/ahliterature129499
Source
Semin Thromb Hemost. 2011 Jul;37(5):522-7
Publication Type
Article
Date
Jul-2011
Author
Paula D James
David P Lillicrap
Author Affiliation
Department of Medicine, Queen's University, Kingston, Ontario, Canada. jamesp@queensu.ca
Source
Semin Thromb Hemost. 2011 Jul;37(5):522-7
Date
Jul-2011
Language
English
Publication Type
Article
Keywords
Canada
Comprehensive Health Care - methods - organization & administration - standards
Deamino Arginine Vasopressin - therapeutic use
Factor VIII - therapeutic use
Genetic Testing
Hemorrhagic Disorders - diagnosis - drug therapy - genetics
Hemostatics - therapeutic use
Humans
Practice Guidelines as Topic
von Willebrand Diseases - diagnosis - drug therapy - genetics
von Willebrand Factor - therapeutic use
Abstract
In Canada, care for individuals with inherited bleeding disorders, including Von Willebrand disease, is provided by 26 tertiary care multidisciplinary Inherited Bleeding Disorders clinics geographically spread across the country. The Association of Hemophilia Clinic Directors of Canada, the Canadian Association of Nurses in Hemophilia Care, the Canadian Physiotherapists in Hemophilia Care, the Canadian Social Workers in Hemophilia Care, and the Canadian Hemophilia Society all collaborate to provide optimal management for patients with inherited bleeding disorders. The standards of care for these patients were explicitly laid out in a 2007 document published by the Canadian Hemophilia Standards Group (with representation from all of the groups just mentioned) entitled Canadian Comprehensive Care Standards for Hemophilia and Other Inherited Bleeding Disorders. Separate Canadian guidelines for the management of patients with Hemophilia and Von Willebrand disease also exist, focused on diagnosis, comprehensive care, assessment, and treatment.
PubMed ID
22102195 View in PubMed
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Effects of prophylactic treatment regimens in children with severe haemophilia: a comparison of different strategies.

https://arctichealth.org/en/permalink/ahliterature31603
Source
Haemophilia. 2002 Mar;8 Suppl 2:43-6
Publication Type
Article
Date
Mar-2002
Author
H M van den Berg
K. Fischer
J G van der Bom
G. Roosendaal
E P Mauser-Bunschoten
Author Affiliation
Van Creveldkliniek, Department of Paediatrics, Julius Centre for Patient Oriented Research, University Medical Centre, Utrecht, The Netherlands. h.m.vandeberg@digd.azu.nl
Source
Haemophilia. 2002 Mar;8 Suppl 2:43-6
Date
Mar-2002
Language
English
Publication Type
Article
Keywords
Adolescent
Child
Child, Preschool
Cohort Studies
Comparative Study
Factor IX - therapeutic use
Factor VIII - therapeutic use
Hemarthrosis - prevention & control
Hemophilia A - prevention & control
Hemophilia B - prevention & control
Humans
Infant
Netherlands
Sweden
Treatment Outcome
Abstract
Both Sweden and the Netherlands have a long experience with primary prophylaxis in children with severe haemophilia. In these countries it has been offered to all children for the last 3-4 decades. In Sweden prophylaxis is generally started at an earlier age with a higher dosage and frequency than in the Netherlands. Patients in the Netherlands receive a more individually tailored regimen, with prophylaxis now started after the first one or two joint bleeds and dosages are increased when breakthrough bleeds occur. The current study evaluated the effect prophylaxis on long-term outcomes and the consumption of clotting factor concentrates in Dutch and Swedish cohorts. Our results show that the orthopaedic outcome in the oldest groups of patients from Sweden and the Dutch cohorts were comparable, although the Swedish patients used twice as much clotting products per year. In the youngest patients, joint status is very good and further follow-up is necessary to demonstrate the benefits of either strategy. In conclusion, more individually tailored regimens aimed at preventing bleeding prevent joint damage in children with severe haemophilia, while clotting factor consumption is about half of that in previously described regimens.
PubMed ID
11966853 View in PubMed
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Haemophilia prophylaxis in young patients--a long-term follow-up.

https://arctichealth.org/en/permalink/ahliterature34288
Source
J Intern Med. 1997 May;241(5):395-400
Publication Type
Article
Date
May-1997
Author
T. Löfqvist
I M Nilsson
E. Berntorp
H. Pettersson
Author Affiliation
Department of Coagulation Disorders, University of Lund, Malmö, Sweden.
Source
J Intern Med. 1997 May;241(5):395-400
Date
May-1997
Language
English
Publication Type
Article
Keywords
Adolescent
Adult
Age Factors
Child
Factor IX - therapeutic use
Factor VIII - therapeutic use
Follow-Up Studies
Hemophilia A - complications - drug therapy
Hemophilia B - complications - drug therapy
Humans
Joint Diseases - etiology - prevention & control - radiography
Research Support, Non-U.S. Gov't
Severity of Illness Index
Treatment Outcome
Abstract
OBJECTIVES: To review long-term prophylactic factor treatment in young patients with severe haemophilia A and B, focusing on the orthopaedic and radiological outcome. DESIGN: We received 34 patients with severe haemophilia A (n = 29) and B (n = 5), aged 7-22 years. Age at start of treatment was 1-4.5 years. Dosages of factor concentrate (F VIII and F IX, respectively) were 25-40 IU/kg body weight, three times a week for haemophilia A and twice a week for haemophilia B. The patients had been checked annually over a 5-year period (1990-95). Orthopaedic and radiological joint scores were evaluated according to recommendations by the World Federation of Haemophilia. SETTING: All results were obtained at the Department for Coagulation Disorders, University of Lund, Malm? University Hospital, Malm?, Sweden. RESULTS: Orthopaedic and radiological joint scores were found to have remained unchanged during follow-up in almost all patients and to be still zero (i.e. no unaffected joints) in 79% (n = 27) of the patients. CONCLUSION: There is a growing international consensus haemophilic arthropathy can be prevented by administering early high-dose prophylaxis. The results of the present investigation strongly support this opinion.
PubMed ID
9183307 View in PubMed
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26 records – page 1 of 3.