Document Detail

Effects of primary and secondary prophylaxis on the clinical expression of joint damage in children with severe haemophilia A. Results of a multicenter non-concurrent cohort study.
MedLine Citation:
PMID:  18217137     Owner:  NLM     Status:  MEDLINE    
Patients with severe haemophilia A (HA) can either be treated by regular FVIII infusions twice or three times per week (prophylaxis), or only in case of bleeding episodes (on-demand). Whereas prophylaxis reduces the number of bleeding episodes and may therefore prevent the development of haemophilic arthropathy, there is still a lot of controversy surrounding recommendations on age and dose at start of prophylactic regimens. The present database study was performed to investigate the role of primary versus secondary prophylaxis in HA children. The outcome variable was imaging-proven haemophilic joint damage. Forty-two children were initially treated with primary prophylaxis following the first bleeding episode, and were frequency-matched (year of birth, catchment area) to 67 patients receiving "on-demand" therapy with an early switch to "secondary prophylaxis". In multivariate analysis adjusted for the HA mutation type and the presence or absence of thrombophilia, the Pettersson score investigated at a median age of 12.5 years in joints with at least one documented bleeding episode was not significantly different between the two patient groups (p = 0.944), and no statistically significant differences were found in patients with target joints (p = 0.3), nor in children in whom synovitis had occurred (p = 0.77). No conclusion can be drawn from the data presented herein whether primary prophylaxis or an early start of secondary prophylaxis is superior with respect to joint outcome in children with severe HA.
Rosemarie Schobess; Karin Kurnik; Frauke Friedrichs; Susan Halimeh; Anne Krümpel; Christoph Bidlingmaier; Ulrike Nowak-Göttl
Related Documents :
1767077 - Immune abnormalities in the pathogenesis of juvenile rheumatoid arthritis.
12622307 - Psychiatric adjustment in chronic fatigue syndrome of childhood and in juvenile idiopat...
7257857 - Temporomandibular joint space in children without joint disease.
11771777 - Cellular immunity in pediatric uveitis.
24547827 - Teddy bears' quick knit.
12201917 - The role of pelvic-floor therapy in the treatment of lower urinary tract dysfunctions i...
Publication Detail:
Type:  Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Thrombosis and haemostasis     Volume:  99     ISSN:  0340-6245     ISO Abbreviation:  Thromb. Haemost.     Publication Date:  2008 Jan 
Date Detail:
Created Date:  2008-01-24     Completed Date:  2008-02-21     Revised Date:  2008-06-27    
Medline Journal Info:
Nlm Unique ID:  7608063     Medline TA:  Thromb Haemost     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  71-6     Citation Subset:  IM    
Department of Pediatrics, University Hospital Halle, Halle, Germany.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Child, Preschool
Coagulants / administration & dosage,  therapeutic use*
Cohort Studies
Drug Administration Schedule
Factor VIII / administration & dosage,  therapeutic use*
Follow-Up Studies
Hemarthrosis / etiology,  genetics,  pathology,  prevention & control*
Hemophilia A / complications,  drug therapy*,  genetics,  pathology
Infant, Newborn
Infusions, Parenteral
Retrospective Studies
Severity of Illness Index
Synovitis / etiology,  genetics,  pathology,  prevention & control*
Time Factors
Treatment Outcome
Reg. No./Substance:
0/Coagulants; 9001-27-8/Factor VIII
Comment In:
Thromb Haemost. 2008 May;99(5):965; author reply 966-8   [PMID:  18449430 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Increased thrombin generation and fibrinogen level after therapeutic plasma transfusion: relation to...
Next Document:  Thrombophilias and adverse pregnancy outcome - A confounded problem!