Management patients with and prevention of recurrent hemarthrosis in hemophilia

被引:17
作者
Dunn, AL
机构
[1] Emory Univ, AFLAC, Ctr Canc, Atlanta, GA 30322 USA
[2] Emory Univ, Blood Disorders Serv, Dept Pediat, Atlanta, GA 30322 USA
[3] Childrens Healthcare Atlanta, Atlanta, GA USA
关键词
arthropathy; hemarthrosis; hemophilia;
D O I
10.1097/01.moh.0000169285.66841.c8
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Purpose of review Degenerative arthopathy resulting from recurrent hemarthrosis remains the largest source of noninfections morbidity for persons with hemophilia, Optimal treatment to prevent or reduce joint destruction remains controversial. Recent findings The most exciting developments in the past year have contributed to our understanding of the pathophysiology behind this destructive process. Particularly interesting is the possible role of the protooncogenes and angiogenic factors factors in the development of hypertrophic synovium and resultant bleeding, and can be used safely even in very young patients. Prophylactic factor replacements remains the mainstay to reduce or prevent joint damage. New approaches, chiefly dose escalation strategies, have shown promise instead of standard prophylactic regimens. Additionally aggressive on-demand approaches may be feasible. These techniques allow for fewer indwelling venous access devices in small children, as rates of complications with these devices in small children, as rates of complications with these devices are shown to be increasingly high. Whether prophylactic therapy can be safely stopped in certain patients is an evolving area of interest. Outcome measures are being validated to assess the impact of interventions on patient quality of life. Summary As hemophilia care evolves, current on-demand and prophylactic regimens will be optimized. Synovectomy will likely play a larger role in the control of synovitis, and validated quality of life scores will allow more consistent communication of outcomes. New understanding of the molecular mechanism of joint destruction will pave the way for new therapeutic options for persons with hemophilia.
引用
收藏
页码:390 / 394
页数:5
相关论文
共 36 条
[1]  
Acharya SS, 2004, BLOOD, V104, p16A
[2]   A LONGITUDINAL-STUDY OF ORTHOPEDIC OUTCOMES FOR SEVERE FACTOR-VIII-DEFICIENT HEMOPHILIACS [J].
ALEDORT, LM ;
HASCHMEYER, RH ;
PETTERSSON, H ;
EIBL, H ;
GILBERT, M ;
HILGARTNER, M ;
KUNSHACK, M ;
LARRIEU, MJ ;
LEVINE, P .
JOURNAL OF INTERNAL MEDICINE, 1994, 236 (04) :391-399
[3]   Novel and clinically significant factors influencing the pharmacokinetic, variability of recombinant factor VIII (Kogenate-FS) in children. [J].
Barnes, C ;
Lillicrap, D ;
Blanchette, V ;
Stain, AM ;
Pazmino-Canizares, J ;
Clark, D ;
Hensmen, C ;
Carcao, M .
BLOOD, 2004, 104 (11) :83B-84B
[4]   Reduced bone density among children with severe hemophilia [J].
Barnes, C ;
Wong, P ;
Egan, B ;
Speller, T ;
Cameron, F ;
Jones, G ;
Ekert, H ;
Monagle, P .
PEDIATRICS, 2004, 114 (02) :177-181
[5]  
Blanchette V, 2004, BLOOD, V104, p842A
[6]  
BLANCHETTE VS, 2004, AM SOC HEM 45 ANN M, V102, P171
[7]   Willingness to pay for on-demand and prophylactic treatment for severe haemophilia in Sweden [J].
Carlsson, KS ;
Höjgård, S ;
Lethagen, S ;
Lindgren, A ;
Berntorp, E ;
Lindgren, B .
HAEMOPHILIA, 2004, 10 (05) :527-541
[8]   Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden [J].
Carlsson, KS ;
Höjgård, S ;
Lindgren, A ;
Lethagen, S ;
Schulman, S ;
Glomstein, A ;
Tengborn, L ;
Berntorp, E ;
Lindgren, B .
HAEMOPHILIA, 2004, 10 (05) :515-526
[9]   Arthroscopic synovectomy for hemophilic joint disease in a pediatric population [J].
Dunn, AL ;
Busch, MT ;
Wyly, JB ;
Sullivan, KM ;
Abshire, TC .
JOURNAL OF PEDIATRIC ORTHOPAEDICS, 2004, 24 (04) :414-426
[10]  
Dunn AL, 2002, THROMB HAEMOSTASIS, V87, P383