Hemophilia + orthopaedic

Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores...

INTRODUCTION: Patients with hemophilia (PWH) constantly suffer hemarthrosis, which leads to deformity of the hip joint. Therefore, PWH who are going to receive total hip arthroplasty (THA) should be exclusively treated before the surgery with careful measurement of their proximal femur. Hence, we conducted a retrospective study to explore the anatomical parameters of and differences in the proximal femur in hemophilic patients who underwent THA. METHODS: The retrospective study comprised data of adult patients who received total hip arthroplasty from 2020 to 2022 in the research center...

INTRODUCTION: In patients with haemophilia, repeated bleeding in large joints leads to chronic haemophilic arthropathy, a rare disease that can be managed surgically with ankle arthrodesis or with total ankle replacement (TAR). TAR has been reported to provide good surgical results in the medium/long-term and allow preservation of joint mobility but the medical therapeutic management of the patients has not been described. AIM: To describe the medical therapeutic management of TAR...

INTRODUCTION: Digital artery aneurysms are a rare event but cases have been reported in the literature. The hemostasis disorders make these aneurysms particularly dangerous with potentially irreversible hand complications: Compression of adjacent vascular and nervous structures, embolization of associated thrombi, finger ischemia, and necrosis. CASE REPORT: We reported a case of digital ischemia due to a ruptured aneurysm of a digital collateral artery, leading to the diagnosis of congenital hemophilia A...

BACKGROUND: Arthropathy is a common complication in patients with hemophilia. We examined the prevalence of this skeletal complication in patients with hemophilia who were registered at a Comprehensive Hemophilia Center in Shiraz, Southern Iran. MATERIALS AND METHODS: In this cross-sectional study, an orthopedic specialist visited 448 patients and conducted screenings for skeletal complications. The assessment included evaluating the type of hemophilia, disease severity, treatment modality, the presence of inhibitors, and the identification of skeletal complications...

PURPOSE: Total knee arthroplasty is the main method for the treatment of advanced haemophilic knee arthritis. Due to the particularity of hemophilia, the blood management plan is the focus of the perioperative period for haemophilia patients. This study aimed to investigate the clinical effect and safety of intra-articular injection of tranexamic acid in patients with haemophilia. METHODS: This is a retrospective study. According to whether tranexamic acid is used or not, patients are divided into tranexamic acid group (n=30) and non-tranexamic acid group (n=29)...

BACKGROUND: There is limited literature comparing the publications on ankle arthrodesis (AA) and total ankle arthroplasty (TAA) in the setting of hemophilic arthropathy. Our objective is to systematically review the existing literature and to assess ankle arthroplasty as an alternative to ankle arthrodesis in this patient population. METHODS: This systematic review was conducted and presented according to the PRISMA statement standards. A search was conducted on March 7-10, 2023, using MEDLINE (via PubMed), Embase, Scopus, ClinicalTrials...

OBJECTIVE: Although total joint replacement (TJR) procedures are efficacious, perioperative high-dose factors replacement therapy (FRT) to avoid catastrophic bleeding represents a significant hurdle, particularly for patients with multiple joint affection. Double simultaneous bilateral TJRs were reported as safe and cost-effective. However, little is known about multiple TJRs. The feasibility and effects remain debatable. Surgeons need to weigh the high cost of FRT against safety. Accordingly, we aimed to evaluate the clinical outcomes and cost-effectiveness of single-anesthetic multiple-joint procedures of lower limbs in end-stage hemophilic arthropathy...

BACKGROUND: Hemophilic arthropathy can cause recurrent hemarthroses and severe damage to the synovium and articular cartilage. Previous studies have shown that vascular endothelial growth factor (VEGF) plays an essential role in neoangiogenesis. Bevacizumab, a monoclonal VEGF inhibitor, is used clinically to prevent angiogenesis. However, its effects on hemophilic arthropathy are unknown. QUESTIONS/PURPOSES: Using a hemophilic arthropathy rabbit model, we asked: Does an intra-articular injection of bevacizumab (1) inhibit VEGF, (2) decrease signal intensity in dynamic contrast-enhanced MRI (DCE-MRI) as an assessment of capillary permeability and neoangiogenesis, (3) reduce cartilage damage, (4) reduce synovial changes, and (5) affect macroscopic changes during the development of hemophilic arthropathy? METHODS: Twenty-five male New Zealand rabbits were divided into four groups...

OBJECTIVE: To report the modified osteotomy and the short-term effectiveness of the total elbow joint replacement in patients of hemophilic elbow arthritis with severe flexion contracture deformity. METHODS: This study introduced the therapeutic approach of the total elbow joint replacement in patients of hemophilic elbow arthritis with severe flexion contracture deformity, and assessed the short-term effectiveness in three cases (three elbows) of end-stage hemophilic elbow arthritis admitted from October 2020 to December 2020...

BACKGROUND: Long-term prophylactic therapy is considered the standard of care for hemophilia A patients. This study models the long-term clinical and cost outcomes of two factor VIII (FVIII) products using a pharmacokinetic (PK) simulation model in a Chinese population. METHODS: Head-to-head PK profile data of BAY 81-8973 (KOVALTRY®) and antihemophilic factor (recombinant) plasma/albumin-free method (rAHF-PFM, ADVATE®) were applied to a two-state (alive and dead) Markov model to simulate blood FVIII concentrations at a steady state in prophylactically-treated patients with hemophilia A...

INTRODUCTION: Total knee arthroplasty (TKA) for a stiff knee of patients with hemophilia (PWH) represents a challenge for orthopaedic surgeons for the difficulties of exposing the knee and high complication rate compared to a flexible knee. AIM: To optimize the surgical exposure in primary TKA for PWH, and to propose a threshold angle of extension contracture in treating haemophilic knee joints, retrospectively. METHODS: 67 primary TKAs for PWH (mean age, 48 years) were performed, and incisional approaches to joint were standard (58 cases) and V-Y quadricepsplasty (V-Y) (9 cases)...

BACKGROUND: Abnormal epiphyseal growth plate development of the proximal tibia in hemophilia patients leads to notable morphological changes in the mature knee joint. This study aimed to compare the morphological characteristics of tibial component placement cut surface in patients with hemophilic arthritis (HA) and osteoarthritis (OA) and to determine the tibial component rotational alignment axis' best position for HA patients. METHODS: Preoperative computed tomography scans of 40 OA and 40 HA patients who underwent total knee arthroplasty were evaluated using a three-dimensional (3D) software...

BACKGROUND: Hemophilic knee arthritis is one of the most common presenting symptoms of hemophilia, and its management continues to be challenging to practitioners. Preliminary research has suggested that platelet-rich plasma (PRP) may have short-term efficacy in the treatment of hemophilic knee arthritis, but evidence for this treatment is limited. QUESTIONS/PURPOSES: What is the effectiveness of PRP compared with placebo in (1) reducing pain and improving knee joint function (as measured by WOMAC, VAS, and Hemophilia Joint Health Score [HJHS]) and (2) improving quality of life (as measured by SF-36 scores) in patients with hemophilic knee arthritis through 24 months of follow-up? METHODS: This was a prospective, parallel-group, double-blinded, single-center, placebo-controlled randomized clinical trial that included participants from a tertiary care center starting January 1, 2019, with follow-up completed on November 30, 2021...

Hemophilia A is a hereditary bleeding disorder due to coagulation factor VIII deficiency. Septic arthritis is in itself a dreaded infective condition of the joint. It is much more common in hemophilia patients than the general population and is associated with considerably higher morbidity. It presents as a difficult clinical and therapeutic problem, necessitating prompt diagnosis and intensive therapy. There was under-diagnosis of blood coagulation disorders in developing countries like India which has improved in the present time...

Introduction: We present a case of spontaneous compartment syndrome due to a very rare cause which is acquired hemophilia. Case Presentation: A 34-year-old patient presented with the right thigh swelling and features of acute compartment syndrome without history of trauma. He had no history of bleeding disorder. There were no features of infection. As his initial blood tests were within normal 16 g/dl, and his compartment syndrome worsened, he had decompression of the thigh...

OBJECTIVE: To evaluate the operative methods and clinical results of surgical treatment in a case series of 10 patients with hemophilic pseudotumors. METHODS: Ten patients with hemophilic pseudotumors who received surgical resection treatment in our hospital from October 2017 to June 2020 were retrospectively reviewed. All patients were hemophilia A (factor VIII deficiency).The age range was 20-51 years. Preoperative imaging examination revealed the size of irregular mass from 8...

INTRODUCTION: Joint damage in haemophilia often requires surgical correction. However, the surgery effect on bleeding rates and other clinical joint outcomes can be unclear. AIM: To investigate the effects of joint surgery on joint annualized bleeding rates (JABRs) and physical health outcomes in patients with haemophilia A undergoing N8-GP prophylaxis. METHODS: Patients in the pathfinder 2 trial received N8-GP prophylaxis, enrolling in the pathfinder 3 trial for indicated surgery...

In this observational study, we describe the medium-term outcomes of total joint arthroplasty (TJA) in human immunodeficiency virus (HIV)-positive patients in Malawi, a low-income country. With a high prevalence of HIV and increasing arthroplasty rates in low and middle-income countries, understanding the outcomes of TJA in this unique cohort of patients is essential to ensure that surgical practice is evidence-based. Methods: Data for all HIV-positive patients who had TJA from January 2005 to March 2020 were extracted from the National Arthroplasty Registry of the Malawi Orthopaedic Association (NARMOA)...

INTRODUCTION: Haemophilic arthropathy is a serious complication of haemophilia often requiring surgical intervention. It is unclear whether advances in comprehensive care are associated with a reduction in orthopaedic interventions and peri-procedural resource utilization. AIM: To determine temporal patterns of orthopaedic interventions in persons with haemophilia (PWH), and evaluate changes in healthcare utilization and outcomes. METHODS: In this Canadian multicentre retrospective cohort study, adult PWH from Northern Alberta and British Columbia who underwent orthopaedic procedures (1990-2018) were included...