Joint range-of-motion limitations among young males with hemophilia: prevalence and risk factors

Soucie, J. Michael; Cianfrini, Christy; Janco, Robert L.; Kulkarni, Roshni; Hambleton, Julie; Evatt, Bruce L.; Forsyth, Angela; Geraghty, Sue; Hoots, Keith; Abshire, Tom; Curtis, Randall; Forsberg, Ann D.; Huszti, Heather C.; Wagner, M.; White, Gilbert

doi:10.1182/blood-2003-05-1457

Cited by 231 publications

(272 citation statements)

References 13 publications

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“…However, joint damage occurs even though the efficacy of prophylaxis with recombinant factor reduce the incidence of joint hemorrhages. Soucie et al [14] reported that the overall joint range of motion is affected in almost 85% of the patients with severe hemophilia. The assessment of joint health has been one of the most important goals in hemophilia care.…”

Section: Discussionmentioning

confidence: 99%

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Tlacuilo‐Parra¹,

Villela-Rodriguez²,

Garibaldi-Covarrubias³

et al. 2009

Pediatric Blood & Cancer

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BackgroundThe Functional Independence Score in Hemophilia (FISH) is a performance‐based assessment tool used to measure the patients functional ability so far only used in patients with severe hemophilia. Its aim is to determine if FISH is useful in patients with mild and moderate disease.ProcedureIn a cross‐sectional study 90 children 60 hemophilic and 30 sex‐, race‐, and age‐matched healthy males were assessed. Patients between 5 and 16 years of age were selected each patient was evaluated in seven activities under three categories: self‐care (grooming and eating, bathing, and dressing), transfers (chair and squat), and locomotion (walking and step climbing). Each activity was graded from 1 to 4 according to the amount of assistance required to perform the activity with total scores ranging from 7 to 28.ResultsAs a whole, the mean age of the patients was 10.0 ± 3.4 years with a mean FISH of 25.8 ± 3.6 (range 15–28). There were no differences in the FISH between healthy males and patients with mild hemophilia; however, the score was significantly higher in patients with mild hemophilia (28 ± 0) than patients with moderate (26.2 ± 2.5; P = 0.004) or severe hemophilia (24.0 ± 4.7; P = 0.0006). The most affected activities were squatting, walking, and step climbing.ConclusionsA significant decrease in functional ability was demonstrated according to the severity of hemophilia, especially for those activities involving weight‐bearing demands like locomotion and step climbing. Of seven activities evaluated, changes were observed in the group with moderate and severe hemophilia, but no changes detected in patients with mild disease. Pediatr Blood Cancer 2010;54:394–397. © 2009 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Tlacuilo‐Parra¹,

Villela-Rodriguez²,

Garibaldi-Covarrubias³

et al. 2009

Pediatric Blood & Cancer

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“…3 Although rates of detection are low with routine surveillance in those with greater than 150 treatment days, we recommend annual testing to facilitate postmarketing surveillance by the Centers for Disease Control and Prevention Universal Data Collection (UDC) Project and other national surveillance programs. 4 The BIA consists of incubating a 1:1 mixture of a dilution of the patient's plasma with undiluted normal plasma for 2 hours, followed by assay of residual fVIII activity. 5 The control incubation consists of a 1:1 mixture of the buffer diluent and undiluted normal plasma.…”

Section: How Are Inhibitors Detected?mentioning

confidence: 99%

How we treat a hemophilia A patient with a factor VIII inhibitor

Kempton

White

2009

Blood

150

144

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The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patientspecific and treatment-related factors work together to influence anti-factor VIII antibody production. (Blood. 2009;113: 11-17)

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“…A patient with moderate haemophilia may bleed after trauma and mild haemophilia usually bleeds after surgery 7 . One of the most severe complications of haemophilia is the development of a characteristic chronic arthropathy that results from repeated bleeding into joints 8 In haemophilia, arthropathy is secondary to recurrent haemarthrosis and chronic synovitis 9 . The chronic arthropathy affects only a few joints, most notably the knees and elbows.…”

Section: Discussionmentioning

confidence: 99%