rVIIa therapy to secure haemostasis during central line insertion in children with high‐responding FVIII inhibitors

Smith, Owen; Hann, Ian

doi:10.1046/j.1365-2141.1996.435968.x

Cited by 35 publications

(31 citation statements)

References 7 publications

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“…Details of the procedures used in patients with and without inhibitors to factor VIII have been reported previously (Liesner et al, 1995;Smith & Hann, 1996). Parents were taught sterile technique and how to use the line, and were instructed to withdraw and discard 1 ml of blood each time the line was used.…”

Section: Methodsmentioning

confidence: 99%

Complications experienced with central venous catheters in children with congenital bleeding disorders

et al. 1997

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Summary. The use of central venous catheters (CVC) in children with coagulation disorders allows home treatment, the use of prophylactic blood product replacement and induction of immune tolerance. Previous reports have suggested an almost complete lack of infective complications in this patient group. We reviewed 23 patients with bleeding disorders who have had 32 CVC inserted at this institution with a median follow-up of 27 months (range 1-92 months). There were 25 documented line-associated infections, including two subcutaneous infections at the port site, and 23 bacteraemias (one episode per 26 patient months at risk). There were 15 Gram-positive, nine Gram-negative and one mixed infection. Infections occurred in 48% of the patients. 15 CVCs were removed: one for erosion through the skin, two for line blockage and 12 for infection. Five patients with inhibitors to factor VIII suffered 14 infections in 12 lines (one per 8 . 3 months) whereas the 18 without inhibitors suffered 11 infections in 20 lines (one per 50 months) (P < 0 . 03). The use of CVCs is favoured by families of children with bleeding disorders in spite of these complications, but close liaison between families and experienced staff at a Haemophilia Centre is essential to ensure that patients gain the benefits of a CVC as safely as possible.

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Section: Methodsmentioning

confidence: 99%

Complications experienced with central venous catheters in children with congenital bleeding disorders

et al. 1997

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“…Most published reports on the use of bypass therapy specifically for surgical prophylaxis in infants and young children with haemophilia and inhibitors confirm the safety and efficacy of activated recombinant FVII (rFVIIa) when infused at a dose of 90 lg kg )1 every 2-4 h [25][26][27]29]. The safety and efficacy of a limited number of doses of 90-100 lg kg )1 in neonates have been reported only within the context of off-label use of this product [30,31].…”

Section: Surgery In Haemophilic Infants With Inhibitorsmentioning

confidence: 99%

“…A review of the literature suggests that CVAD placement and removal represent the most common procedures in children with inhibitors [25][26][27]. Furthermore, although few bleeding complications are reported in the absence of an antibody, bleeding can occur with as many as 20% of CVAD placements in inhibitor patients [28].…”

Section: Surgery In Haemophilic Infants With Inhibitorsmentioning

confidence: 99%

Haemophilia in the first years of life

et al. 2008

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Summary. Surgery in infants and young children with haemophilia, when preceded by accurate diagnosis and accompanied by safe and effective factor prophylaxis, is not associated with a significant risk of haemorrhage. Haemophilic newborns undergoing circumcision or major surgery prior to diagnosis and in the absence of appropriate haemostatic prophylaxis remain as a concern. Inhibitor development has replaced haemorrhage as the major surgical complication in the developed world, largely because of the intensity of treatment used to secure haemostasis. For that reason only, essential surgery should be performed. Intracranial haemorrhage (ICH) during the neonatal period affects 3.5-4.0% of all haemophilia boys in countries with a good standard of health care, which is considerably (40-80 times) higher than expected in the normal population. Because of the high frequency of sporadic cases, ICH in the neonatal period can only be partially prevented by improved carrier diagnosis and counselling. Infections and thrombosis are the major serious complications of central venous lines. Large differences are seen in the frequency of these complications, the most plausible explanations are probably related to the protocol used for device care, the quality of education and the compliance of the users, an issue addressed in an on-going study.

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“…High-dose recombinant factor VIIa (NovoSeven ® ) has been used very successfully in haemophiliacs with inhibitors who have uncontrollable bleeding including synovectomy (Hedner et al, 1988), retropharyngeal haemorrhage , joint haemorrhage (Macik et al, 1993), orthopaedic surgery (O'Marcaigh et al, 1994), intracranial haemorrhage (Schmidt et al, 1994) and for central line insertion (Smith & Hann, 1996). This therapy has had remarkably few side-effects, including little or no evidence of thrombotic complications (Macik et al, 1993;O'Marcaigh et al, 1994;Schmidt et al, 1994).…”

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confidence: 99%

Platelet activity of high‐dose factor VIIa is independent of tissue factor

et al. 1997

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High‐dose recombinant factor VIIa has been successfully used as therapy for haemophiliacs with inhibitors. The mechanism by which high‐dose factor VIIa supports haemostasis is the subject of some controversy. Postulating a mechanism in which activity is dependent on tissue factor at the site of injury explains the localization of activity but not the requirement for high doses. Postulating a mechanism in which factor VIIa acts on available lipid independently of tissue factor explains the requirement for high doses but not the lack of systemic procoagulant activity. We report that factor VIIa bound weakly to activated platelets (Kd ∼ 90 nm). This factor VIIa was functionally active and could initiate thrombin generation in the presence of plasma concentrations of prothrombin, factor X, factor V, antithrombin III and tissue factor pathway inhibitor. The activity was not dependent on tissue factor. The concentration of factor VIIa required for detectable thrombin generation agreed well with the lowest concentration of factor VIIa required for efficacy in patients. High‐dose factor VIIa may function on the activated platelets that form the initial haemostatic plug in haemophilic patients. These observations are in agreement with clinical trials which have shown that high‐dose factor VIIa was haemostatically effective without causing systemic activation of coagulation.

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rVIIa therapy to secure haemostasis during central line insertion in children with high‐responding FVIII inhibitors

Cited by 35 publications

References 7 publications

Complications experienced with central venous catheters in children with congenital bleeding disorders

Complications experienced with central venous catheters in children with congenital bleeding disorders

Haemophilia in the first years of life

Platelet activity of high‐dose factor VIIa is independent of tissue factor

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