Pathogenesis of hemophilic arthropathy, Semin Hematol, vol.43, pp.18-22, 2006. ,
HTCN Study Investigators, Bleeding symptoms and laboratory correlation in patients with severe von Willebrand disease, Haemophilia, vol.15, pp.918-925, 2009. ,
Joint bleeding in factor VIII deficient mice causes an acute loss of trabecular bone and calcification of joint soft tissues which is prevented with aggressive factor replacement, Haemophilia, vol.20, pp.716-722, 2014. ,
Prevalence and risk factors associated with decreased bone mineral density in patients with haemophilia, Haemophilia, vol.15, pp.559-565, 2009. ,
Long-term prophylaxis in severe haemophilia seems to preserve bone mineral density, Haemophilia, vol.15, pp.261-266, 2009. ,
low bone mass, and osteopenia/osteoporosis, Transfus Apher Sci, vol.38, pp.33-40, 2008. ,
Reduced bone density in individuals with severe hemophilia B, Int J Rheum Dis, vol.12, pp.125-129, 2009. ,
Premature changes in trabecular and cortical microarchitecture result in decreased bone strength in hemophilia, Blood, vol.125, pp.2160-2163, 2015. ,
The bone disease associated with factor VIII deficiency in mice is secondary to increased bone resorption, Haemophilia, vol.19, pp.908-912, 2013. ,
A novel role for factor VIII and thrombin/PAR1 in regulating hematopoiesis and its interplay with the bone structure, Blood, vol.122, pp.2562-2571, 2013. ,
RANKL, RANK, osteoprotegerin: key partners of osteoimmunology and vascular diseases, Cell Mol Life Sci, vol.64, pp.2334-2350, 2007. ,
Mechanisms of bone repair and regeneration, Trends Mol Med, vol.15, pp.417-429, 2009. ,
Characterization of the interaction between von Willebrand factor and osteoprotegerin, J Thromb Haemost, vol.5, pp.1956-1962, 2007. ,
Osteoprotegerin (OPG) is localized to the Weibel-Palade bodies of human vascular endothelial cells and is physically associated with von Willebrand factor, J Cell Physiol, vol.204, pp.714-723, 2005. ,
Factor VIII-von Willebrand factor complex inhibits osteoclastogenesis and controls cell survival, J Biol Chem, vol.284, pp.31704-31713, 2009. ,
Histological changes in murine haemophilic synovitis: a quantitative grading system to assess blood-induced synovitis, Haemophilia, vol.12, pp.654-662, 2006. ,
Intraarticular factor IX protein or gene replacement protects against development of hemophilic synovitis in the absence of circulating, Blood, vol.112, pp.4532-4541, 2008. ,
Employing a gain-of-function factor IX variant R338L to advance the efficacy and safety of hemophilia B human gene therapy: preclinical evaluation supporting an ongoing adeno-associated virus clinical trial, Hum Gene Ther, vol.26, pp.69-81, 2015. ,
Cytokine family and bone cells, Cytokine, vol.12, pp.1455-1468, 2000. ,
IL-6, RANKL, TNF-alpha/IL-1: interrelations in bone resorption pathophysiology, Cytokine Growth Factor Rev, vol.15, pp.49-60, 2004. ,
Inhibition of osteoblast apoptosis by thrombin, Bone, vol.33, pp.733-743, 2003. ,
Thrombin-stimulated growth factor and cytokine expression in osteoblasts is mediated by protease-activated receptor-1 and prostanoids, Bone, vol.44, pp.813-821, 2009. ,
Altered osteoprotegerin/RANKL ratio and low bone mineral density in celiac patients on long-term treatment with gluten-free diet, Horm Metab Res, vol.38, pp.417-422, 2006. ,
The RANKL/OPG system and bone mineral density in patients with chronic liver disease, J Hepatol, vol.43, pp.973-983, 2005. ,
IL-6 negatively regulates osteoblast differentiation through the SHP2/MEK2 and SHP2/Akt2 pathways in vitro, J Bone Miner Metab, vol.32, pp.378-392, 2014. ,
Interleukin-6 and soluble interleukin-6 receptors in the synovial fluids from rheumatoid arthritis patients are responsible for osteoclast-like cell formation, J Bone Miner Res, vol.11, pp.88-95, 1996. ,
Glycoprotein 130 regulates bone turnover and bone size by distinct downstream signaling pathways, J Clin Invest, vol.113, pp.379-389, 2004. ,
Bone mineral density in men and children with haemophilia A and B: a systematic review and meta-analysis, Osteoporosis Int, vol.25, pp.2399-2407, 2014. ,
Bone mineral density in haemophilia patients. A meta-analysis ,
, Thromb Haemost, vol.103, pp.596-603, 2010.
Reduced bone density among children with severe hemophilia, Pediatrics, vol.114, pp.177-181, 2004. ,
Are individuals with severe haemophilia A prone to reduced bone density?, Rheumatol Int, vol.28, pp.1079-1083, 2008. ,
Reduced bone density in individuals with combined factor V and VIII deficiency, Haemophilia, vol.13, pp.340-343, 2007. ,
Decreased bone density and bone strength in a mouse model of severe factor VIII deficiency, Br J Haematol, vol.158, pp.140-143, 2012. ,
Molecular mechanisms of platelet adhesion and activation, Int J Biochem Cell Biol, vol.29, pp.91-105, 1997. ,
Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease, J Clin Invest, vol.60, pp.390-404, 1977. ,
Human blood platelet adhesion to artery subendothelium is mediated by factor VIII-Von Willebrand factor bound to the subendothelium, Nature, vol.279, pp.636-638, 1979. ,
Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease, Br J Haematol, vol.111, pp.1236-1239, 2000. ,
Factor VIII:C increases after desmopressin in a subgroup of patients with autosomal recessive severe von Willebrand disease, Br J Haematol, vol.89, pp.147-151, 1995. ,
, The Diagnosis, Evaluation, and Management of von Willebrand Disease, pp.8-5832, 2007.
IL-6 receptor antagonist as adjunctive therapy with clotting factor replacement to protect against bleeding-induced arthropathy in hemophilia, J Thromb Haemost, vol.11, pp.881-893, 2013. ,
Heterotropic bone formation as a complication of elective joint replacement in haemophilic patients -a case report and literature review, Haemophilia, vol.12, pp.672-675, 2006. ,
Association of severe haemophilia A with osteoporosis: a densitometric and biochemical study, Q J Med, vol.87, pp.181-186, 1994. ,
The diagnosis of osteoporosis, J Bone Miner Res, vol.9, pp.1137-1141, 1994. ,
Guidelines for the management of hemophilia, Haemophilia, vol.19, pp.1-47, 2013. ,
Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia: A United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) guideline, Haemophilia, vol.23, pp.511-520, 2017. ,
In collaboration with the national patient member organizations in the Nordic countries, Nordic Hemophilia Guidelines, 2015. ,
Bone health in persons with haemophilia, Haemophilia, vol.21, pp.568-577, 2015. ,
, Australian Hemophilia Centre Directors' Organisation. Guidelines for the management of hemophilia in Australia, 2016.
Hypercalciuria in children with haemophilia suggests primary skeletal pathology, Br J Haematol, vol.153, pp.364-371, 2011. ,
URL : https://hal.archives-ouvertes.fr/hal-00621305
Targeted disruption of the mouse factor VIII gene produces a model of haemophilia A, Nat Genet, vol.10, pp.119-121, 1995. ,
A coagulation factor IX-deficient mouse model for human hemophilia B, Blood, vol.90, pp.3962-3966, 1997. ,
A mouse model of severe von Willebrand disease: defects in hemostasis and thrombosis, Proc Natl Acad Sci, vol.95, pp.9524-9529, 1998. ,
, day three and peaked at day seven. C: cortical bone; P: periosteum; M: skeletal muscle. Original magnification: X200. n=5-8 per group
, µCT measurements were compared using t-test comparison between WT and KO of each line. FVIII WT n=16, KO n=14; FIX WT n=14, KO n=14; VWF WT n=10, KO n=17. DXA measurements BMD and BMC were compared using 2-way ANOVA with multiple comparisons