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Hemophilia + orthopaedic

Diane Nugent, Brian O'Mahony, Gerry Dolan
INTRODUCTION: Therapeutic advances over the past 30 years have led to longer life expectancy and improved quality of life (QOL) for persons with hemophilia. Access to innovative therapy may be compromised if treatment decisions are driven solely by cost. New strategies are needed to assess true therapeutic values, along with financial cost, as physicians, policymakers, payers and manufacturers work together to improve patient care. AIM: To provide an evidence-based assessment of the value of prophylaxis vs on-demand therapy for hemophilia, based on a widely recognized three-tiered value framework approach for assessing a range of therapeutic interventions...
September 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
P Tobase, H Lane, A-E-A Siddiqi, J M Soucie, R Ingram-Rich, S Ward, J C Gill
BACKGROUND: Invasive orthopaedic interventions (IOI) are often used to control recurrent haemarthrosis, pain and loss of joint function, in males with haemophilia (Factor VIII and Factor IX deficiency). AIM: Identify risk factors associated with IOIs in males with haemophilia enrolled in the Universal Data Collection (UDC) surveillance program from 2000 until 2010. METHODS: Data were collected on IOIs performed on patients receiving care in 130 haemophilia treatment centers in the United States annually by health care providers using standardized forms...
November 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Elcil Kaya Bicer, Kemal Kayaokay, Andac Alsina, Huseyin Gunay, Kaan Kavakli, Semih Aydoğdu
BACKGROUND: Arthroscopic debridement has been used in the treatment of early stages of hemophilic ankle arthropathy. The aim of this procedure is to alleviate pain, improve function, and diminish bleeding episodes. Evaluation of patient-reported outcomes of arthroscopic debridement for hemophilic ankle arthropathies was evaluated in this study. METHODS: Fifteen patients with hemophilia who had undergone arthroscopic debridement between 2008 and 2017 were included in this study...
October 2018: Foot & Ankle International
G F Pierce, A Haffar, G Ampartzidis, F Peyvandi, S Diop, M El-Ekiaby, H M van den Berg
INTRODUCTION: The gaps in haemophilia treatment around the world are enormous; approximately 60% of an estimated 475 000 individuals are not identified. Of the 187 000 diagnosed, 30% (57 000) access clotting factor replacement therapy. Since 1996, humanitarian aid distributed by the World Federation of Hemophilia (WFH) has played a minor, yet vital role providing life-saving clotting factor to countries in emergency situations. Donated amounts have been small and sporadic, often salvaging short-dated products, providing little opportunity to leverage donations with governments...
March 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
E C Merchan, E Galindo, M Magallon, J Gago, A Villar, M J Sanjurjo
Resection of the radial head and partial open synovectomy of the elbow have been used in the treatment of repeated haemarthroses of the elbows in 15 patients with haemophilia. The indications for the index operation were: severe pain and bleeding in the elbow that occurred in spite of appropriate, episodic, replacement therapy over a period of more than 6 months, associated with hypertrophy of the radial head and a significant loss of pronation-supination. The long-term results were assessed retrospectively according to the classification recommended by the Orthopaedic Advisory Committee of the World Federation of Hemophilia...
October 1995: Haemophilia: the Official Journal of the World Federation of Hemophilia
Juan Pretell-Mazzini, Ty Subhawong, Victor H Hernandez, Rafael Campo
➤Advances in combined antiretroviral therapy (cART) in recent years have transformed HIV infection into a chronic disease when treatment is available, increasing a patient's life expectancy and the chances that orthopaedic surgeons will encounter such patients in their clinical practice.➤Musculoskeletal manifestations in patients with HIV infection are common and sometimes are the initial presentation of the disease. Knowledge about neoplasms and associated conditions affecting muscle, bones, and joints is essential for successful management...
May 4, 2016: Journal of Bone and Joint Surgery. American Volume
Roberto Buda, Marco Cavallo, Francesco Castagnini, Annarita Cenacchi, Simone Natali, Francesca Vannini, Sandro Giannini
OBJECTIVE: Ankle arthropathy is a frequent and invalidating manifestation of hemophilia. Arthrodesis is the gold standard surgical procedure in end-stage disease, with many drawbacks in young patients. Recent literature has shown increase interest in regenerative procedures in hemophilic arthropathy, which may be desirable to delay or even avoid arthrodesis. The aim of this article is to present five cases of osteochondral lesions in ankle hemophilic arthropathy treated with a regenerative procedure: bone marrow-derived cells transplantation (BMDCT)...
July 2015: Cartilage
Elena Santagostino, Miguel Escobar, Margareth Ozelo, Luigi Solimeno, Per Arkhammar, Hye Youn Lee, Gabriela Rosu, Paul Giangrande
The availability of recombinant activated factor VII (rFVIIa, eptacog alfa activated) has greatly advanced the care of patients with haemophilia A or B who have developed inhibitors against the infused replacement factor. Recombinant FVIIa is licensed for the on-demand treatment of bleeding episodes and the prevention of bleeding in surgery or invasive procedures in patients with congenital haemophilia with inhibitors. This article attempts to review in detail the extensive evidence of rFVIIa in congenital haemophilia patients with inhibitors...
June 2015: Blood Reviews
A Barg, K Barg, M Wiewiorski, S W Schneider, M D Wimmer, D C Wirtz, V Valderrabano, G Pagenstert
BACKGROUND: Total ankle replacement (TAR) is a well-accepted treatment option in patients with end-stage ankle osteoarthritis. However, published literature on patients with bleeding disorders treated with TAR is limited. Therefore, we carried out this prospective study to analyze mid-term postoperative results in patients with bleeding disorders treated by TAR. METHODS: A total of 34 patients with end-stage ankle osteoarthritis--14 patients with hemophilia type A and 20 patients with von Willebrand disease (VWD)--treated by TAR were included in this prospective study...
August 2015: Der Orthopäde
Annarita Tagliaferri, Giulio Feola, Angelo Claudio Molinari, Cristina Santoro, Gianna Franca Rivolta, Dorina Bianca Cultrera, Fabio Gagliano, Ezio Zanon, Maria Elisa Mancuso, Lelia Valdré, Luciana Mameli, Susanna Amoresano, Prasad Mathew, Antonio Coppola
Rigorous evidence is lacking on long-term outcomes of factor VIII (FVIII) prophylaxis initiated in adolescent or adult patients with severe haemophilia A. The prospective, open-label Prophylaxis versus On-demand Therapy Through Economic Report (POTTER) study ( NCT01159587) compared long-term late secondary prophylaxis (recombinant FVIII-FS 20-30 IU/kg thrice weekly) with on-demand treatment in patients aged 12 to 55 years with severe haemophilia A. The annual number of joint bleeding episodes (primary endpoint), total bleeding episodes, orthopaedic and radiologic (Pettersson) scores, health-related quality of life (HRQoL), pharmacoeconomic impact, and safety were evaluated over a > 5-year period (2004-2010)...
July 2015: Thrombosis and Haemostasis
Nathalie Pireau, Laurence M Boon, Pascal Poilvache, Pierre-Louis Docquier
BACKGROUND: To assess the efficacy of surgical treatment of intra-articular knee venous malformations (VM). METHODS: Between 1998 and 2010, 8 children (mean age: 12.3 y) underwent surgical resection of their vascular malformation (7 venous and 1 capillary venous) involving the knee joint. The lesion was diffuse in 6 cases and well-demarcated in 2 cases. All children were suffering from knee pain and had recurrent hemarthroses. Color-Doppler ultrasonography, magnetic resonance imaging, computed tomography scan, and blood test were performed preoperatively...
April 2016: Journal of Pediatric Orthopedics
Christina Hart, Bernhard Heindl, Michael Spannagl, Susanne Lison
Recommendations on the administration of clotting factor concentrates in patients with severe haemophilia undergoing surgery are usually determined by monitoring target clotting factor levels. In this retrospective cohort study, we enrolled patients with severe haemophilia A who underwent major orthopaedic or trauma surgery. We wanted to evaluate the feasibility and the safety of a standardized medical treatment procedure. Further on, we wanted to assess whether our standardized treatment regimen enables surgical procedures in certain situations in which measuring clotting factor VIII (FVIII) activity is not available...
June 2015: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
K Meijer, S Rauchensteiner, E Santagostino, H Platokouki, R E G Schutgens, M Brunn, C Tueckmantel, F Valeri, P C Schinco
In haemophilia A, continuous infusion (CI) of FVIII perioperatively provides a more constant FVIII level than conventional bolus injections, avoiding low trough levels that could increase bleeding risk. Due to the low number of surgical cases in clinical trials, especially in haemophilia, more information on the clinical practice of CI from observational studies is helpful. We aimed to evaluate the effectiveness and safety of CI with recombinant factor VIII formulated with sucrose (rFVIII-FS) in a typical surgery practice setting...
January 2015: Haemophilia: the Official Journal of the World Federation of Hemophilia
J G Asencio, C Leonardi, C Biron-Andreani, J F Schved
BACKGROUND: Ankle arthropathy is very frequent in haemophilic patients. Prostheses are valuable alternatives to arthrodesis in non-haemophilic patients. We report the experience of a single centre in France on the use of prostheses in haemophilic patients. METHODS: Retrospective study of 21 patients with haemarthropathy who underwent ankle arthroplasty (32 ankles), with additional surgery, if needed, from July 2002 to September 2009 (mean follow-up 4.4±1.7 years)...
December 2014: Foot and Ankle Surgery: Official Journal of the European Society of Foot and Ankle Surgeons
E Carlos Rodriguez-Merchan, Primitivo Gomez-Cardero, Ángel Martinez-Lloreda, Hortensia De La Corte-Rodriguez, Victor Jimenez-Yuste
The purpose of this study is to describe the results of arthroscopic ankle debridement (AAD) with the aim of determining whether it is possible to avoid or delay ankle fusion or total ankle replacement for advanced haemophilic arthropathy of the ankle in young patients. In a 12-year period (2000-2011), 24 AADs (one bilateral in two stages) were performed for advanced haemophilic arthropathy of the ankle in 23 patients (22 had haemophilia A, 1 haemophilia B, 0 with inhibitors against the deficient factor). Their average age at operation was 25...
April 2015: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
M Sammels, J Vandesande, E Vlaeyen, K Peerlinck, K Milisen
Falls are a particular risk in persons with haemophilia (PWH) because of damaged joints, high risk of bleeding, possible impact on the musculoskeletal system and functioning and costs associated with treatment for these fall-related injuries. In addition, fall risk increases with age and PWH are increasingly entering the over 65 age group. The aim of this study was to determine the occurrence of falls during the past year and to explore which fall risk factors are present in community-dwelling PWH. Dutch speaking community-dwelling adults were included from the age of 40 years with severe or moderate haemophilia A or B, independent in their mobility and registered at the University Hospitals Leuven...
November 2014: Haemophilia: the Official Journal of the World Federation of Hemophilia
E Santagostino, S R Lentz, M Misgav, B Brand, P Chowdary, A Savic, Y Kilinc, Y Amit, A Amendola, L P Solimeno, T Saugstrup, I Matytsina
Recombinant factor VIII (rFVIII) products provide a safe and efficacious replacement therapy for prevention and treatment of bleeding episodes in patients with haemophilia A. The present investigations from the multinational, open-label guardian(™) clinical trials assessed the haemostatic response of turoctocog alfa (NovoEight(®)), a rFVIII product, in patients with severe haemophilia A (FVIII ≤ 1%) undergoing surgery. All patients had a minimum of 50 exposure days to any FVIII product prior to surgery and no history of inhibitors...
January 2015: Haemophilia: the Official Journal of the World Federation of Hemophilia
J E Asuquo, E A Orimolade, L Salawu, A B Oladiran
AIMS AND OBJECTIVES: The objective of this article is to report a rarely encountered case of haemophilic arthropathy which should be considered in the differential diagnosis of knee swelling. PATIENTS AND METHODS: The history. physical findings and results of investigations of a patient presenting with haemophilic arthropathy and the past medical records of the patient were reviewed with relevant literature. RESULTS: This is a report of the existence of this condition in this environment and the management of this rarely encountered condition in a I0 year old boy, known haemophiliac who presented at the orthopaedic clinic with features of right knee arthropathy...
June 2014: Nigerian Postgraduate Medical Journal
H I Balci, M Kocaoglu, L Eralp, F E Bilen
Haemophilia, a bleeding disorder, causes recurrent intra-articular bleeding of the joints result-ing in chronic haemophilic arthropathy with fixed knee flexion deformity. Mid-long-term results (between 2002 and 2006) of deformity correction in haemophilic patients with Ilizarov type circular external fixators were retrospectively evaluated. There were six patients (five haemophilia A and one haemophilia B). The mean age was 14.7 years (range, 8-22 years) at the time of initial surgery. The mean knee flexion contracture was 45 degrees (range, 30-75 degrees)...
November 2014: Haemophilia: the Official Journal of the World Federation of Hemophilia
E C Rodriguez-Merchan
Close collaboration between haematologists, orthopaedic surgeons, rehabilitation physicians, pediatricians, pharmacist, radiologist, pain specialist, psychologists, physiotherapists and nurses is essential for the proper handling of knee problems in haemophiliac patients. The ideal scenario would be for the primary prophylaxis (prevention of the disease) to prevent major degenerative changes that we still see so frequently in this type of patients. Until we achieve this we will continue having to perform multiple invasive orthopaedic procedures, such as articular punctures (joint aspiration) to drain haemarthrosis, radiosynovectomies, arthroscopic synovectomies, tendon lenghtenings, realignment osteotomies and total knee replacements (primary and revision) on the knees of people with haemophilia...
September 2014: Thrombosis Research
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