-
2
-
-
24644507061
-
-
April 5
-
Universal Data Collection System national summary report. April 5, 2005. Available at www.cdc.gov/ncbdd/hdb/surveillance.htm. This database provides information on rates of joint disease in US patients with hemophilia.
-
(2005)
Universal Data Collection System National Summary Report
-
-
-
3
-
-
7444225852
-
Clinical and cost implications of target joints in Canadian boys with severe hemophilia A
-
Kern M, Blanchette V, Stain AM, et al. Clinical and cost implications of target joints in Canadian boys with severe hemophilia A. J Pediatr 2004; 145:628-634. This paper details the natural history of target joint development in boys with severe hemophilia A treated with on-demand therapy. Fifteen of 16 boys with severe hemophilia developed one or more target joints at a mean age of 53.5 months.
-
(2004)
J Pediatr
, vol.145
, pp. 628-634
-
-
Kern, M.1
Blanchette, V.2
Stain, A.M.3
-
4
-
-
12144290833
-
Joint range-of-motion limitations among young males with hemophilia: Prevalence and risk factors
-
Soucie JM, Cianfrini C, Janco RL, et al. Joint range-of-motion limitations among young males with hemophilia: prevalence and risk factors. Blood 2004; 103:2467-2473. This paper describes the association of body mass index and range-of-motion loss in persons with hemophilia. Elevated body mass index was the only factor positively correlated with joint disease in patients with mild, moderate, and severe hemophilia.
-
(2004)
Blood
, vol.103
, pp. 2467-2473
-
-
Soucie, J.M.1
Cianfrini, C.2
Janco, R.L.3
-
5
-
-
16544368532
-
Reduced bone density among children with severe hemophilia
-
Barnes C, Wong P, Egan B, et al. Reduced bone density among children with severe hemophilia. Pediatrics 2004; 114:e177-e181. This article reports the results of bone mineral density in boys with severe hemophilia A compared with age- and gender-matched control subjects. Boys with joint disease had statistically lower bone density compared with age-matched control subjects.
-
(2004)
Pediatrics
, vol.114
-
-
Barnes, C.1
Wong, P.2
Egan, B.3
-
6
-
-
2342538377
-
Experimental haemophilia synovitis: Rationale and development of a murine model of human factor VIII deficiency
-
Valentino LA, Hakobyan N, Kazarian T, et al. Experimental haemophilia synovitis: rationale and development of a murine model of human factor VIII deficiency. Haemophilia 2004; 10:280-287. This paper describes a reproducible method of inducing joint bleeding in a mouse model. Changes seen were similar to those seen in human disease. This will allow study of biochemical mechanisms of joint disease as well as efficacy of various treatment regimens.
-
(2004)
Haemophilia
, vol.10
, pp. 280-287
-
-
Valentino, L.A.1
Hakobyan, N.2
Kazarian, T.3
-
7
-
-
24644506142
-
Role for angiogenesis in hemophilic synovitis
-
Acharya SS, MacDonald DD, DiMichele DM, et al. Role for angiogenesis in hemophilic synovitis [abstract 42]. Blood 2004; 104:42. This abstract presents evidence of angiogenic factors on development of blood-induced synovitis. It also explores the link between joint disease and circulating angiogenesis-related cytokines.
-
(2004)
Blood
, vol.104
, pp. 42
-
-
Acharya, S.S.1
MacDonald, D.D.2
Dimichele, D.M.3
-
8
-
-
0031774695
-
Iron deposits and catabolic properties of synovial tissue from patients with haemophilia
-
Roosendaal G, Vianen ME, Wenting MJG, et al. Iron deposits and catabolic properties of synovial tissue from patients with haemophilia. J Bone Joint Surg Br 1998; 806:540-545.
-
(1998)
J Bone Joint Surg Br
, vol.806
, pp. 540-545
-
-
Roosendaal, G.1
Vianen, M.E.2
Wenting, M.J.G.3
-
9
-
-
0036682499
-
C-myc proto-oncogene expression in hemophilic synovitis: In vitro studies of the effects of iron and ceramide
-
Wen FQ, Jabbar AA, Chen YX, et al. C-myc proto-oncogene expression in hemophilic synovitis: in vitro studies of the effects of iron and ceramide. Blood 2002; 100:912-916.
-
(2002)
Blood
, vol.100
, pp. 912-916
-
-
Wen, F.Q.1
Jabbar, A.A.2
Chen, Y.X.3
-
10
-
-
4644248645
-
Pathobiology of hemophilic synovitis I: Overexpression of mdm2 oncogene
-
Hakobyan N, Kazarian T, Jabbar AA, et al. Pathobiology of hemophilic synovitis I: overexpression of mdm2 oncogene. Blood 2004; 104:2060-2064. This article describes the in-vitro, dose-dependent increase in synovial protooncogene c-myc expression after exposure to an iron source. It also shows that the synovial proliferation could be blocked by using apoptosis-inducing agents. This pioneering study paves the way for development of novel strategies to address synovial hypertrophy.
-
(2004)
Blood
, vol.104
, pp. 2060-2064
-
-
Hakobyan, N.1
Kazarian, T.2
Jabbar, A.A.3
-
11
-
-
0036529818
-
The effects of postponing prophylactic treatment on long-term outcome in patients with severe hemophilia
-
Fischer K, Van Der Bom JG, Mauser-Bunschoten EP, et al. The effects of postponing prophylactic treatment on long-term outcome in patients with severe hemophilia. Blood 2002; 99:2337-2341.
-
(2002)
Blood
, vol.99
, pp. 2337-2341
-
-
Fischer, K.1
Van Der Bom, J.G.2
Mauser-Bunschoten, E.P.3
-
12
-
-
0027219641
-
Experience with prophylaxis in Germany
-
Schramm W. Experience with prophylaxis in Germany. Semin Hematol 1993; 30(suppl 2):12-15.
-
(1993)
Semin Hematol
, vol.30
, Issue.SUPPL. 2
, pp. 12-15
-
-
Schramm, W.1
-
13
-
-
24644521465
-
Variability in bleeding pattern of severe hemophilia
-
Van Dijk K, Fischer K, Van Der Bom JG, et al. Variability in bleeding pattern of severe hemophilia [abstract 3094]. Blood 2004; 104:3094. This abstract highlights the variability in bleeding patterns in persons with severe hemophilia.
-
(2004)
Blood
, vol.104
, pp. 3094
-
-
Van Dijk, K.1
Fischer, K.2
Van Der Bom, J.G.3
-
14
-
-
0028004222
-
A longitudinal study of orthopaedic outcomes for severe factor VIII deficient haemophiliacs
-
Aledort LM, Haschmeyer RH, Pettersson H, et al. A longitudinal study of orthopaedic outcomes for severe factor VIII deficient haemophiliacs. J Intern Med. 1994; 236:391-399.
-
(1994)
J Intern Med
, vol.236
, pp. 391-399
-
-
Aledort, L.M.1
Haschmeyer, R.H.2
Pettersson, H.3
-
15
-
-
24644515299
-
The role of age at first exposure and therapy regimen on the development of neutralizing FVIII antibodies in hemophilia A
-
Ettingshausen CE, Zyschka A, Saguer IM, et al. The role of age at first exposure and therapy regimen on the development of neutralizing FVIII antibodies in hemophilia A [abstract 3082]. Blood 2004; 104:3082. This abstract suggests a link between early exposure (<6 months of age) to factor VIII and development of inhibitory antibodies.
-
(2004)
Blood
, vol.104
, pp. 3082
-
-
Ettingshausen, C.E.1
Zyschka, A.2
Saguer, I.M.3
-
16
-
-
22744458188
-
Impact of early factor VIII exposure, prophylaxis and prenatal/perinatal events on inhibitor risk in children with hemophilia A: A case-control study
-
Santagostino E, Rocino A, Mancuso ME, et al. Impact of early factor VIII exposure, prophylaxis and prenatal/perinatal events on inhibitor risk in children with hemophilia A: a case-control study [abstract 37]. Blood 2004; 104:37. This review found no link between early factor VIII exposure and development of inhibitory antibodies.
-
(2004)
Blood
, vol.104
, pp. 37
-
-
Santagostino, E.1
Rocino, A.2
Mancuso, M.E.3
-
17
-
-
4844222412
-
Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden
-
Steen Carlson K, Hojgard S, Lindgren A, et al. Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden. Haemophilia 2004; 10:515-526. This study calculated differences in hemophilia care between on-demand and prophylactic regimens. Not surprisingly, total cost for prophylactic care is higher, but patients on on-demand therapy spend more on hospitalization, reconstructive surgery, and lost productivity.
-
(2004)
Haemophilia
, vol.10
, pp. 515-526
-
-
Steen Carlson, K.1
Hojgard, S.2
Lindgren, A.3
-
18
-
-
4844226896
-
Willingness to pay for on-demand and prophylactic treatment for severe haemophilia in Sweden
-
Steen Carlson K, Hojgard S, Lethagen S, et al. Willingness to pay for on-demand and prophylactic treatment for severe haemophilia in Sweden. Haemophilia 2004; 10:527-541. This in an encouraging study in that, despite being educated about the higher cost of prophylaxis over on-demand therapy, Swedish citizens were still willing to pay for prophylactic care.
-
(2004)
Haemophilia
, vol.10
, pp. 527-541
-
-
Steen Carlson, K.1
Hojgard, S.2
Lethagen, S.3
-
19
-
-
24644484728
-
Long-term aspects of hemophilia B treatment: Part II
-
Gringeri A. Long-term aspects of hemophilia B treatment: part II. Blood Coagul Fibrinolysis 2004; 15(suppl 2):S15-S16. This article reports early results of a study comparing standard prophylaxis with on-demand therapy. It suggest that 13.8 bleeding episodes/person/year are avoided by prophylactic therapy.
-
(2004)
Blood Coagul Fibrinolysis
, vol.15
, Issue.SUPPL. 2
-
-
Gringeri, A.1
-
20
-
-
7044238659
-
Musculoskeletal results from the Canadian hemophilia dose-escalation prophylaxis trial
-
Blanchette VS, Rivard GFE, Israels SJ, et al. Musculoskeletal results from the Canadian hemophilia dose-escalation prophylaxis trial. In: Abstracts of the American Society of Hematology 45th Annual Meeting and Exposition. 2004; 102:171. This abstract describes the early results of the Canadian factor VIII dose escalation study. Boys with severe hemophilia A are started on once-a-week prophylactic infusions and only progress to twice or three times per week if they have recurrent bleeding. Thus far only 16% of patients have required thrice-weekly infusions to control bleeding. No patient has required a CVAD.
-
(2004)
Abstracts of the American Society of Hematology 45th Annual Meeting and Exposition
, vol.102
, pp. 171
-
-
Blanchette, V.S.1
Rivard, G.F.E.2
Israels, S.J.3
-
21
-
-
33645118243
-
Characterization of the pharmacokinetics of rVIII in young pre-school children with hemophilia, including an analysis of the influence of age and body weight
-
Blanchette V, Shapiro AD, Liesner R, et al. Characterization of the pharmacokinetics of rVIII in young pre-school children with hemophilia, including an analysis of the influence of age and body weight [abstract 3084]. Blood 2004; 104:3084. This abstract highlights the shorter half-life of infused recombinant factor VIII in young children compared with adults.
-
(2004)
Blood
, vol.104
, pp. 3084
-
-
Blanchette, V.1
Shapiro, A.D.2
Liesner, R.3
-
22
-
-
24644479576
-
Novel and clinically significant factors influencing the pharmacokinetic variability of recombinant factor VIII (Kogenate-FS) in children
-
Barnes C, Lillicrap D, Blanchette V, et al. Novel and clinically significant factors influencing the pharmacokinetic variability of recombinant factor VIII (Kogenate-FS) in children [abstract 3991]. Blood 2004; 104:3991. This abstract compares factor VIII recovery in children with adults and suggests that factor VII dosing based on body surface area may lead to more predictable recovery.
-
(2004)
Blood
, vol.104
, pp. 3991
-
-
Barnes, C.1
Lillicrap, D.2
Blanchette, V.3
-
23
-
-
0037331212
-
Immature articular cartilage is more susceptible to blood-induced damage than mature articular cartilage: An in vivo animal study
-
Hooiveld MJ, Roosendaal G, Vianen ME, et al. Immature articular cartilage is more susceptible to blood-induced damage than mature articular cartilage: an in vivo animal study. Arthritis Rheum 2003; 48:396-403.
-
(2003)
Arthritis Rheum
, vol.48
, pp. 396-403
-
-
Hooiveld, M.J.1
Roosendaal, G.2
Vianen, M.E.3
-
24
-
-
0035543814
-
Discontinuation of prophylactic therapy in severe haemophilia: Incidence and effects of outcome
-
Fischer K, Van Der Bom JG, Prejs R, et al. Discontinuation of prophylactic therapy in severe haemophilia: incidence and effects of outcome. Haemophilia 2001; 7:544-550.
-
(2001)
Haemophilia
, vol.7
, pp. 544-550
-
-
Fischer, K.1
Van Der Bom, J.G.2
Prejs, R.3
-
26
-
-
3042829631
-
Arthroscopic synovectomy for hemophilic joint disease in a pediatric population
-
Dunn AL, Busch MT, Wyly JB, et al. Arthroscopic synovectomy for hemophilic joint disease in a pediatric population. J Pediatr Orthop 2004; 24:414-426. This article reports the largest and longest experience to date using arthroscopic synovectomy to control hemophilic synovitis in a pediatric population. The procedure is highly effective in reducing recurrent joint bleeding, and rehabilitation was not problematic. Despite reduction in bleeding, arthropathy continued to worsen in patients, particularly if the joint disease was well established before surgery.
-
(2004)
J Pediatr Orthop
, vol.24
, pp. 414-426
-
-
Dunn, A.L.1
Busch, M.T.2
Wyly, J.B.3
-
27
-
-
0036125777
-
Radionuclide synovectomy for hemophilic arthropathy: A comprehensive review of safety and efficacy and recommendation for a standardized treatment protocol
-
Dunn AL, Busch MT, Wyly JB, et al. Radionuclide synovectomy for hemophilic arthropathy: a comprehensive review of safety and efficacy and recommendation for a standardized treatment protocol. Thromb Haemost 2002; 87:383-393.
-
(2002)
Thromb Haemost
, vol.87
, pp. 383-393
-
-
Dunn, A.L.1
Busch, M.T.2
Wyly, J.B.3
-
30
-
-
0035169021
-
Phosphate-32 colloid radiosynovectomy in hemophilia
-
Siegel HJ, Luck JV, Siegel ME, et al. Phosphate-32 colloid radiosynovectomy in hemophilia. Clin Orthop 2001; 392:409-417.
-
(2001)
Clin Orthop
, vol.392
, pp. 409-417
-
-
Siegel, H.J.1
Luck, J.V.2
Siegel, M.E.3
-
31
-
-
4844223869
-
Yttrium synoviorthesis of the elbow joints in persons with haemophilia
-
Heim M, Tiktinsky R, Amit Y, et al. Yttrium synoviorthesis of the elbow joints in persons with haemophilia. Haemophilia 2004; 10:590-592. This review of yttrium-90 synovectomy on elbows highlights an unacceptably high rate of loss of motion.
-
(2004)
Haemophilia
, vol.10
, pp. 590-592
-
-
Heim, M.1
Tiktinsky, R.2
Amit, Y.3
-
32
-
-
4243120812
-
Is corticosteroid coinjection necessary for radiosynoviorthesis of patients with hemophilia?
-
Gedik GK, Ugur O, Atilla B, et al. Is corticosteroid coinjection necessary for radiosynoviorthesis of patients with hemophilia? Clin Nucl Med 2004; 29: 538-541. This article questions the need for steroid coinjection to minimize leakage during yttrium-90 synovectomy of the knee. Without steroids, however, 1 of 12 patients had an excessive leakage of 70% of the radioactivity to the draining pelvic lymph nodes.
-
(2004)
Clin Nucl Med
, vol.29
, pp. 538-541
-
-
Gedik, G.K.1
Ugur, O.2
Atilla, B.3
-
34
-
-
4844229881
-
Functional consequences of haemophilia in adults: The development of the Haemophilia Activities List
-
Van Genderen FR, Van Meeteren NLU, Van Der Bom JG, et al. Functional consequences of haemophilia in adults: the development of the Haemophilia Activities List. Haemophilia 2004; 10:565-571. This paper describes the development of a QOL measure designed to assess functional health status of adults with hemophilia.
-
(2004)
Haemophilia
, vol.10
, pp. 565-571
-
-
Van Genderen, F.R.1
Van Meeteren, N.L.U.2
Van Der Bom, J.G.3
-
35
-
-
1542330950
-
Central venous access devises in hemophilia
-
Valentino LA, Ewenstein B, Navickis RJ, et al. Central venous access devises in hemophilia. Haemophilia 2004; 10:134-146. The authors reviewed existing literature regarding the use of CVADs in hemophilia care. They generated a meta-analysis outlining reasons for device use and rates of infection. The majority of lines were used for immune tolerance therapy and for difficult venous access. Infection was the major indication for line removal.
-
(2004)
Haemophilia
, vol.10
, pp. 134-146
-
-
Valentino, L.A.1
Ewenstein, B.2
Navickis, R.J.3
-
36
-
-
19944433826
-
A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia
-
Price VE, Carcao M, Connolly B, et al. A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia. J Thromb Haemost 2004; 2:737-742. This paper presents the longest and largest study to date of catheter-related thrombosis in hemophilia.
-
(2004)
J Thromb Haemost
, vol.2
, pp. 737-742
-
-
Price, V.E.1
Carcao, M.2
Connolly, B.3
-
37
-
-
4844224154
-
Consensus recommendations for use of central venous access devices in haemophilia
-
Ewenstein BM, Valentino LA, Jouneycake JM, et al. Consensus recommendations for use of central venous access devices in haemophilia. Haemophilia 2004; 10:629-648. An excellent and comprehensive review of the use of CVADs in persons with hemophilia. It provides evidence-based guidelines regarding patient selection, CVAD placement and care, caregiver education, and complications such as infection and thrombosis.
-
(2004)
Haemophilia
, vol.10
, pp. 629-648
-
-
Ewenstein, B.M.1
Valentino, L.A.2
Jouneycake, J.M.3
|