Understanding haemophilic arthropathy: an exploration of current open issues

Jansen, N.W.; Roosendaal, G.; Lafeber, Floris P. J. G.

doi:10.1111/j.1365-2141.2008.07386.x

Cited by 122 publications

(120 citation statements)

References 57 publications

Supporting

Mentioning

115

Contrasting

Unclassified

Order By: Relevance

“…Our data suggest that the synovitis may play a crucial role in blood-induced arthropathy provoking an overreaction which subsequently becomes independent from bleeding, as postulated in other studies. 36,37 In addition, on the basis of our findings, the reduction in OPG seems to play a pivotal role in the progression of arthropathy and could even serve in the future as a biomarker of disease severity. Thus, an early clinical, instrumental and serological screening of all hemophiliacs may be recommended.…”

Section: Discussionmentioning

confidence: 71%

Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A

et al. 2015

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 71%

Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A

et al. 2015

View full text Add to dashboard Cite

“…They may suffer from hemarthrosis and severe arthropathy and need regular treatment with coagulation factor concentrates. The majority of hemophilia patients treated before the introduction of viral inactivation were infected with hepatitis C and/or HIV [31][32][33]. Previous studies found that HR-QoL is mainly dependent on severity of hemophilia, age, orthopedic status, hepatitis C infection and comorbidities [34][35][36][37].…”

Section: Quality Of Life With Von Willebrand Disease 1495mentioning

confidence: 99%

Health‐related quality of life among adult patients with moderate and severe von Willebrand disease

Wee

Mauser‐Bunschoten

Bom

et al. 2010

Journal of Thrombosis and Haemostasis

112

View full text Add to dashboard Cite

Summary. Background: von Willebrand Disease (VWD) is the most frequent inherited bleeding disorder. It is unknown how this disorder affects quality of life. Objectives: This nationwide multicenter cross-sectional study determined health-related quality of life (HR-QoL) in adult patients with moderate or severe VWD, and assessed whether bleeding severity and type of VWD are associated with HR-QoL. Methods: HR-QoL was assessed using the Short Form (SF)-36, and bleeding severity was measured using the Bleeding Score (BS). Results: Five hundred and nine patients participated; 192 males and 317 females, median age and range 45 (16-87) and 47 (16-84) years, respectively. Compared with the general population, HR-QoL in VWD patients was lower in the vitality domain (61 vs. 66 P < 0.001 for females, 67 vs. 72 P < 0.001 for males). Patients with the most severe bleeding phenotype (highest quartile BS, BS > 17) had a lower HR-QoL in eight domains than patients with a less severe bleeding type (lowest quartile BS, BS < 7) in the univariate analysis. After adjustment for age, gender, comorbidity and employment/educational status, a more severe bleeding phenotype was associated with lower scores on the domains of physical functioning, role limitations due to physical functioning, bodily pain, general health, social functioning and physical component summary. Conclusions: HRQoL is lower in VWD patients compared with the general population. HR-QoL is strongly associated with bleeding phenotype.

show abstract

“…Jansen et al [3] claimed that joint bleeding results in initially independent adverse changes in both synovial tissue and articular cartilage. These changes subsequently influence each other, with inflammatory changes in the synovium bringing about cartilage damage and vice versa.…”

Section: Pathophysiologymentioning

confidence: 98%

Cartilage damage in the haemophilic joints

Rodríguez-Merchán

2012

Blood Coagulation &Amp; Fibrinolysis

View full text Add to dashboard Cite

Intra-articular bleeding affects the metabolism and repair of articular cartilage. Biomechanical data have shown that blood causes harmful effects on overall cartilage function under loading conditions. Therefore, haemophilic patients suffering a haemarthrosis should be subjected to blood aspiration (arthrocentesis) to prevent cartilage damage. MRI and ultrasonography have shown themselves to be excellent noninvasive tools for the evaluation of early cartilage damage that remains undetectable by conventional radiography in haemophilic patients. Prophylaxis with the deficient factor can prevent cartilage deterioration and reduce the incidence of joint haemorrhage in children with haemophilia. Radiosynovectomy has proved to be a highly effective procedure to decrease both the frequency and the severity of recurrent intra-articular bleeding episodes. Nowadays, the most usual surgical procedures for treating articular cartilage defects (cartilage repair) include abrasion chondroplasty, microfracture, mosaicplasty, autologous chondrocyte implantation (ACI), and matrix-induced ACI. In small defects (<2-4 cm(2)), ostechondral autograft or microfracture are the recommended options. In large defects (>2-4 cm(2)), ACI or osteochondral allograft are indicated. However, these techniques have not been applied in haemophilic patients because inflammatory conditions and advanced degenerative change (>50% joint space narrowing) are contraindications for cartilage repair. Thus, prevention of cartilage damage is paramount in haemophilia. The definitive remedy for advanced cartilage damage is either (knee or hip) replacement or (ankle) arthrodesis. Primary prophylaxis and radiosynovectomy are the best alternatives at our disposal to protect our patients against cartilage damage and arthropathy in haemophilic joints.

show abstract

Understanding haemophilic arthropathy: an exploration of current open issues

Cited by 122 publications

References 57 publications

Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A

Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A

Health‐related quality of life among adult patients with moderate and severe von Willebrand disease

Cartilage damage in the haemophilic joints

Contact Info

Product

Resources

About