Liver transplantation in Spanish haemophiliacs

Aznar, José A.; Marco, A.; Parra, Rafael; Jiménez‐Yuste, Víctor; Lucia, F.; Balda, I.; Soto, Inmaculada

doi:10.1111/j.1365-2516.2011.02658.x

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…However, the long-term outcome of on-demand treatment is worse in terms of joint status, serious hemorrhages, quality of life, and labor force participation. 4,37,39 Previous health and economic evaluations have addressed issues of cost-effectiveness in hemophilia care. 9,40 One cost-benefit analysis in a Swedish setting reported that the average willingness to pay for prophylaxis in the general population exceeded the average cost of provision of prophylaxis per taxpayer.…”

Section: Comparison With Other Studiesmentioning

confidence: 99%

Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s

Fischer

Carlsson

Petrini

et al. 2013

Blood

202

248

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Section: Comparison With Other Studiesmentioning

confidence: 99%

Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s

Fischer

Carlsson

Petrini

et al. 2013

Blood

202

248

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show abstract

“…1,2 Current efforts to prevent HA are mainly focused on management of acute bleeds and optimizing the schedule for prophylactic factor replacement, 5 which has significant advantages over episodic factor replacement following bleeding. [6][7][8][9] However, breakthrough bleeds occur even on the best prophylaxis protocols, 10 and HA remains a persistent problem and challenge for hemophilia patients. Therefore, improving the understanding of its pathogenesis and devising novel treatments remain major unmet needs in this area.…”

Section: Introductionmentioning

confidence: 99%

Blood-induced bone loss in murine hemophilic arthropathy is prevented by blocking the iRhom2/ADAM17/TNF-α pathway

Haxaire

Hakobyan

Pannellini³

et al. 2018

Blood

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Hemophilic arthropathy (HA) is a debilitating degenerative joint disease that is a major manifestation of the bleeding disorder hemophilia A. HA typically begins with hemophilic synovitis that resembles inflammatory arthritides, such as rheumatoid arthritis, and frequently results in bone loss in patients. A major cause of rheumatoid arthritis is inappropriate release of the proinflammatory cytokine tumor necrosis factor-α (TNF-α) by the TNF-α convertase (TACE; also referred to as ADAM17) and its regulator, iRhom2. Therefore, we hypothesized that iRhom2/ADAM17-dependent shedding of TNF-α also has a pivotal role in mediating HA. Here, we show that addition of blood or its components to macrophages activates iRhom2/ADAM17-dependent TNF-α shedding, providing the premise to study the activation of this pathway by blood in the joint in vivo. For this, we turned to hemophilic deficient mice ( mice), which develop a hemarthrosis following needle puncture injury with synovial inflammation and significant osteopenia adjacent to the affected joint. We found that needle puncture-induced bleeding leads to increased TNF-α levels in the affected joint of mice. Moreover, inactivation of TNF-α or iRhom2 in mice reduced the osteopenia and synovial inflammation that develops in this mouse model for HA. Taken together, our results suggest that blood entering the joint activates the iRhom2/ADAM17/TNF-α pathway, thereby contributing to osteopenia and synovitis in mice. Therefore, this proinflammatory signaling pathway could emerge as an attractive new target to prevent osteoporosis and joint damage in HA patients.

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“…The same group achieved the first successful OLT three years later (5). At the time of writing, only about 115 haemophilia patients worldwide have undergone liver transplantation for HCV associated liver disease (1,2,4,6,7).…”

Section: Zusammenfassungmentioning

confidence: 99%

Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients

Goldmann¹,

Zeitler²,

Marquardt³

et al. 2015

Hamostaseologie

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Liver transplantation in Spanish haemophiliacs

Cited by 5 publications

References 10 publications

Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s

Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s

Blood-induced bone loss in murine hemophilic arthropathy is prevented by blocking the iRhom2/ADAM17/TNF-α pathway

Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients

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