keyword
https://read.qxmd.com/read/34587640/the-bone-microarchitecture-deficit-in-patients-with-hemophilia-is-influenced-by-arthropathy-hepatitis-c-infection-and-physical-activity
#21
JOURNAL ARTICLE
Katharina Holstein, Leonora Witt, Anna Matysiak, Constantin Schmidt, Florian Barvencik, Michael Amling, Tim Rolvien, Florian Langer
Low bone mineral density (BMD) is common in patients with hemophilia (PWHs). The aim of the present study was to describe BMD and microarchitecture in PWHs in Northern Germany and to determine factors contributing to possible skeletal alterations. Demographic characteristics, BMD and microarchitecture, bone metabolism markers, and orthopaedic joint score (OJS) were assessed during routine check-ups. Areal BMD was assessed by dual-energy X-ray absorptiometry (DXA) at the hip and lumbar spine. Volumetric BMD and microarchitecture were quantified by high-resolution peripheral quantitative computed tomography at the distal radius and tibia...
September 29, 2021: Thrombosis and Haemostasis
https://read.qxmd.com/read/34569205/-progress-on-surgical-treatment-for-hemophiliac-arthropathy
#22
JOURNAL ARTICLE
Shao-Ning Shen, Dong-Xiao Wu, Pei-Jian Tong
Surgical treatment is the main treatment for hemophilia arthritis, including synoviectomy, joint replacement and joint fusion. Synoviectomy is suitable for early hemophilia synovitis, and is divided into radiation, chemical, arthroscopy, and open operation. Radionuclides were recommended as the first choice due to its positive efficacy and less side effects, but exsit some problems such as scarcity of nuclides. Chemical synoviectomy is cheap and easy to operate, which is suitable for developing countriesm, while mutiple doses and pain after injection are main fault...
September 25, 2021: Zhongguo Gu Shang, China Journal of Orthopaedics and Traumatology
https://read.qxmd.com/read/34106506/bleeding-phenotype-of-patients-with-moderate-haemophilia-a-and-b-assessed-by-thromboelastometry-and-thrombin-generation
#23
MULTICENTER STUDY
Ragnhild J Måseide, Erik Berntorp, Vuokko Nummi, Riitta Lassila, Geir E Tjønnfjord, Pål A Holme
INTRODUCTION: Predicting the bleeding phenotype is crucial for the management of patients with moderate haemophilia. Global coagulation assays evaluate haemostasis more comprehensively than conventional methods. AIM: To explore global coagulation assays and the bleeding phenotype of patients with moderate haemophilia A (MHA) and B (MHB). METHODS: The MoHem study is a cross-sectional, multicentre study covering Nordic patients with MHA and MHB...
September 2021: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/34096536/a-foot-to-last-a-lifetime-is-hindfoot-fusion-ever-appropriate-for-pediatric-orthopaedic-conditions
#24
JOURNAL ARTICLE
Derek M Kelly
INTRODUCTION: Hindfoot deformities in the pediatric population can be painful and result in severe limitations. Although arthrodesis is known to relieve pain, there are concerns over its use because of the risk that adjacent joint degenerative disease could result, leading to a new source of pain, dysfunction, and additional surgical procedures later in life. METHODS: A literature review of hindfoot fusions in children focused on articles with the highest levels of evidence and those of particular historical significance...
July 1, 2021: Journal of Pediatric Orthopedics
https://read.qxmd.com/read/34017743/clinicopathological-features-of-hemophilia-in-a-tertiary-care-centre-of-india
#25
JOURNAL ARTICLE
Pandey K Pawan, Yadav Mahima, Tilak Vijai, Lader Manjula
CONTEXT: Inherited bleeding disorders are common in India and hemophila and von Willebrand diseases are the most common among them. These patients can present in any department including paediatrics, medicine, orthopaedics and even gynaecology so knowledge about hemophilias and facilities for specialized tests for diagnosis are required. Few centres of north-eastern part of India perform these tests so hemophilias remain an underdiagnosed and underreported disease. AIMS: The objective of this study was to estimate the prevalence of hemophilia in patients referred to this tertiary care centre and study the clinicopathological profile of these patients...
January 2021: Journal of Family Medicine and Primary Care
https://read.qxmd.com/read/33882996/surgical-treatment-for-patients-with-hemophilic-pseudotumor-related-femoral-fracture-a-retrospective-study
#26
JOURNAL ARTICLE
Keyu Chen, Guiyong Jiang, Yaowen Xu, Yunping Yang, Zexiong Mao, Jiaxin Lv, Fei Liu, Bin Chen
BACKGROUND: Hemophilic pseudotumor (HPT)-related fracture is a rare but severe complication in patients with HPTs. These fractures often occur in femurs. There is no consensus on the standard surgical protocol for HPT-related femoral fracture. The present retrospective study evaluated the outcomes of these patients treated with surgical interventions. METHODS: Ten patients with HPT-related femoral fractures who were treated with 14 surgical procedures due to 11 fractures in our hospital from January 2014 to April 2020 were evaluated retrospectively...
April 21, 2021: Journal of Orthopaedic Surgery and Research
https://read.qxmd.com/read/33772963/results-of-a-randomized-phase-iii-iv-trial-comparing-intermittent-bolus-versus-continuous-infusion-of-antihaemophilic-factor-recombinant-in-adults-with-severe-or-moderately-severe-haemophilia-a-undergoing-major-orthopaedic-surgery
#27
RANDOMIZED CONTROLLED TRIAL
Ingrid Pabinger, Vasily Mamonov, Jerzy Windyga, Werner Engl, Jennifer Doralt, Srilatha Tangada, Gerald Spotts, Bruce Ewenstein
INTRODUCTION: In patients with haemophilia A undergoing surgery, factor VIII (FVIII) replacement therapy by continuous infusion (CI) may offer an alternative to bolus infusion (BI). AIM: To compare the perioperative haemostatic efficacy and safety of antihaemophilic factor (recombinant) (ADVATE® ; Baxalta US Inc., a Takeda company, Lexington, MA, USA) CI or BI administration. METHODS: In this multicentre, phase III/IV, controlled study (NCT00357656), 60 previously treated adult patients with severe or moderately severe disease undergoing elective unilateral major orthopaedic surgery (knee replacement, n = 48; hip surgery, n = 4; other, n = 8) requiring drain placement were randomized to receive antihaemophilic factor (recombinant) CI (n = 29) or BI (n = 31) through postoperative day 7...
May 2021: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/33751769/recombinant-fviia-in-elective-non-orthopaedic-surgery-of-adults-with-haemophilia-and-inhibitors-a-systematic-literature-review
#28
REVIEW
Gerard Dolan, Jan Astermark, Cedric Hermans
AIM: To assess available evidence on the use of rFVIIa in non-orthopaedic surgery including dental surgery in adult patients with congenital haemophilia with inhibitors (PWHI). METHODS: A systematic literature search was performed according to a prespecified search string; prespecified criteria were used to select applicable studies including PWHI ≥18 years of age who underwent any non-orthopaedic surgery using rFVIIa. RESULTS: Thirty-three publications met the eligibility criteria, of which 26 publications - including 46 procedures in 44 patients - were selected for the qualitative analysis...
May 2021: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/33339074/low-rate-of-subclinical-venous-thrombosis-in-patients-with-haemophilia-undergoing-major-orthopaedic-surgery-in-the-absence-of-pharmacological-thromboprophylaxis
#29
JOURNAL ARTICLE
Géraldine Verstraete, Catherine Lambert, Frank Hammer, Cedric Hermans
BACKGROUND: Deep venous thrombosis (DVT) is a common postoperative complication in patients undergoing major orthopaedic surgery of the lower limbs, such as total hip or knee replacement (THR, TKR). Routine pharmacological thromboprophylaxis with low-molecular-weight heparin (LMWH) or a direct oral anticoagulant agent is strongly recommended in this setting. THR and TKR as well as ankle arthrodesis are frequently performed in people with haemophilia (PWH) and chronic haemophilic arthropathy...
November 2020: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/32211475/total-knee-arthroplasty-in-hemophilia-a
#30
Neil Pathak, Alana M Munger, Ahmad Charifa, William B Laskin, Emily Bisson, Gary M Kupfer, Lee E Rubin
Hemophilia A is a rare genetic disorder involving a deficiency of clotting factor VIII. Coagulation factor replacement therapy has prolonged the life expectancy of patients with hemophilia, but recurrent hemarthrosis of major joints is often a common occurrence. Therefore, orthopaedic adult reconstructive surgeons increasingly encounter hemophilic arthropathy in young adults and consider treating with total joint arthroplasty. In this report, the authors describe a patient with hemophilia A and severe knee osteoarthritis, who was subsequently treated with primary total knee arthroplasty...
March 2020: Arthroplasty Today
https://read.qxmd.com/read/31970256/various-surgical-treatment-of-hemophilic-pseudotumor-a-case-series
#31
JOURNAL ARTICLE
Achmad Fauzi Kamal, Agus Waryudi, Aryadi Kurniawan, Anna Mira Lubis, Djayadiman Gatot
BACKGROUND: Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild trauma or daily activities. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor, usually in the muscle adjacent to the bone. We reported haemophilic pseudotumor treated with various surgical interventions. METHODS: This study was conducted inthe Department of Orthopaedic and Traumatology at a government hospital over a period of 7 years(2010 -2017)...
November 2019: Archives of Bone and Joint Surgery
https://read.qxmd.com/read/31184581/physical-activity-improved-by-adherence-to-prophylaxis-in-an-italian-population-of-children-adolescents-and-adults-with-severe-haemophilia-a-the-shape-study
#32
JOURNAL ARTICLE
Ezio Zanon, Annarita Tagliaferri, Samantha Pasca, Cosimo P Ettorre, Lucia D Notarangelo, Chiara Biasioli, Anna B Aru, Marta Milan, Silvia Linari, Angiola Rocino, Fabio Gagliano, Giovanni Di Minno, Gabriella Gamba, Rita C Santoro, Piercarla Schinco, Marco Marietta, Axel Seuser, Sylvia von Mackensen
BACKGROUND: Physical activity in people with haemophilia (PWH) reduces the development of severe arthropathy, but it must be performed after regular, proper prophylaxis. Strict adherence to treatment is crucial to achieving effectiveness and established outcomes. The primary aim of this study was to collect prospective data on adherence to prophylaxis for over 36 months. A secondary aim was to verify whether adherence correlates with physical activity. MATERIALS AND METHODS: Italian patients with severe haemophilia A treated on prophylaxis with octocog alfa were included in the study...
June 5, 2019: Blood Transfusion
https://read.qxmd.com/read/30088692/value-of-prophylaxis-vs-on-demand-treatment-application-of-a-value-framework-in-hemophilia
#33
JOURNAL ARTICLE
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
https://read.qxmd.com/read/29957840/risk-factors-associated-with-invasive-orthopaedic-interventions-in-males-with-haemophilia-enrolled-in-the-universal-data-collection-program-from-2000-to-2010
#34
JOURNAL ARTICLE
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
https://read.qxmd.com/read/29860878/role-of-arthroscopic-debridement-of-hemophilic-ankles
#35
JOURNAL ARTICLE
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
https://read.qxmd.com/read/29537123/first-year-results-of-an-expanded-humanitarian-aid-programme-for-haemophilia-in-resource-constrained-countries
#36
JOURNAL ARTICLE
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
https://read.qxmd.com/read/27214634/resection-of-the-radial-head-and-partial-open-synovectomy-of-the-elbow-in-the-young-adult-with-haemophilia-long-term-results
#37
JOURNAL ARTICLE
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
https://read.qxmd.com/read/27147691/hiv-and-orthopaedics-musculoskeletal-manifestations-and-outcomes
#38
REVIEW
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
https://read.qxmd.com/read/26175860/treatment-of-hemophilic-ankle-arthropathy-with-one-step-arthroscopic-bone-marrow-derived-cells-transplantation
#39
JOURNAL ARTICLE
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
https://read.qxmd.com/read/26073369/recombinant-activated-factor-vii-in-the-treatment-of-bleeds-and-for-the-prevention-of-surgery-related-bleeding-in-congenital-haemophilia-with-inhibitors
#40
REVIEW
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
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