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Int J Environ Res Public Health 2022 Jun 20;19(12). Epub 2022 Jun 20.

Inserm, UMR 1229, RMeS, Regenerative Medicine and Skeleton, Nantes Université, ONIRIS, 44042 Nantes, France.

Some patients with moderate haemophilia (PWMH) report joint damage potentially responsible for gait disorders. Three-dimensional gait analysis (3DGA) is a relevant tool for the identification of complex musculoskeletal impairment. We performed an evaluation with 3DGA of 24 PWMH aged 44. Read More

J Orthop Surg Res 2022 Jun 14;17(1):315. Epub 2022 Jun 14.

Department of Orthopedic Surgery, Second Affiliated Hospital of Anhui Medical University, Heifei, 230601, China.

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. Read More

Acta Orthop Belg 2022 Mar;88(1):121-125

Management of symptomatic osteoarthritis (OA) of the ankle in patients with haemophilia can be challenging. Arthroscopic ankle arthrodesis has been shown in non-haemophiliac patients to provide similar or superior rates of fusion to open ankle fusion. However, the literature regarding ankle arthrodesis in patients with haemophilia is limited. Read More

Gac Med Mex 2022 ;158(1):11-15

Hematology Department, "Dr. Gaudencio González Garza" General Hospital, High Specialty Medical Unit, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Mexico City, Mexico.

Background: Joint ultrasound examination using the HEAD-US method in the detection of early arthropathy is poorly studied in our country.

Objective: To compare the clinical and ultrasound evaluation of the joints in haemophilia.

Method: Longitudinal, prospective and descriptive study with paediatric patients with haemophilia A and B evaluated with the HJHS 2. Read More

Haemophilia 2022 May 16;28(3):480-490. Epub 2022 Mar 16.

Haemostasis and Thrombosis Unit, Division of Haematology, Cliniques universitaires Saint-Luc, Brussels, Belgium.

Introduction: Ankle arthropathy is highly prevalent among people with haemophilia (PwH), even with prophylaxis, and leads to pain and disability. Mechanisms and consequences of painful symptoms related to ankle arthropathy have not been extensively studied.

Methods: A consecutive sample of 30 adult PwH was included (60 ankles). Read More

Br J Haematol 2022 02 19;196(4):871-883. Epub 2021 Dec 19.

IRCCS San Matteo Hospital, Pavia, Italy.

Although synovitis is recognized as a marker of joint disease activity, its periodic assessment is not included in routine clinical surveillance of patients with haemophilia (PwH). In order to evaluate the current knowledge and to identify controversial issues, a preliminary literature search by the Musculoskeletal Committee of the Italian Association of Haemophilia Centres (AICE) has been conducted. Statements have been established and sent to the Italian AICE members to collect their level of agreement or disagreement by a Delphi process. Read More

Haemophilia 2021 Sep 25;27(5):866-875. Epub 2021 Jun 25.

Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, California, USA.

Background: Painful arthropathy is a long-term complication in patients with hemophilia (PWH), affecting mobility and quality of life. A major barrier for the appraisal of joint health is the absence of point-of-care (POC) imaging modalities to promptly identify and manage arthropathic changes. Accordingly, we developed the Joint tissue Activity and Damage Exam (JADE) POC musculoskeletal ultrasound (MSKUS) protocol. Read More

Clin Anat 2021 Sep 25;34(6):941-947. Epub 2021 May 25.

Institute of Medical and Biological Engineering, School of Mechanical Engineering, University of Leeds, Leeds, UK.

Flattening of the trochlear tali is clinically observed as structural and functional changes advance in patients with hemarthropathy of the ankle. However, the degree of this flattening has not yet been quantified, and distribution of the morphological changes across the talus not yet defined. Chronologically sequential MR images of both a hemophilic patient group (N = 5) and a single scan from a nondiseased, sex-matched, control group (N = 11) were used to take four measurements of the trochlear talus morphology at three locations (medial, central and lateral) along the sagittal plane. Read More

Haemophilia 2021 Jul 10;27(4):641-647. Epub 2021 May 10.

Haemophilia Foundation of Argentina, Buenos Aires, Argentina.

Introduction: Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration and subsequent disability. The aim of this study is to evaluate the level of agreement between the Haemophilia Joint Health Score and the Haemophilia Early Arthropathy Detection with Ultrasound tools in children with severe haemophilia on primary and secondary prophylaxis. Read More

JBJS Case Connect 2020 12 4;10(4):e20.00351. Epub 2020 Dec 4.

Department of Orthopedic Surgery, Hasbro Children's Hospital, Warren Alpert Medical School, Brown University, Providence, Rhode Island.

Case: We report the case of an active 8-year-old boy with a history of hemophilia and with a 1-year history of knee pain and limited range of motion. Magnetic resonance imaging of the knee demonstrated a ganglion cyst arising from the posterior cruciate ligament (PCL). Arthroscopic decompression of the cyst was performed, and he was asymptomatic at the 1-year follow-up. Read More

Folia Med (Plovdiv) 2020 Dec;62(4):762-768

Medical University of Plovdiv, Plovdiv, Bulgaria.

Introduction: Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. Pre-cise assessment and follow-up of joint status are crucial for tailoring their treatment.

Aim: To study the correlation between the bleeding phenotype, the functional joint status, and the magnetic resonance imaging score in pediatric patients with haemophilia. Read More

Medicine (Baltimore) 2020 Nov;99(47):e22986

Department of Orthopaedic Surgery, Second Affiliated Hospital of Anhui Medical University, Heifei, China.

This retrospective study aimed to define the morphological characteristics of the distal femur in patients with hemophilia-related knee arthritis (HA) and develop precise femoral component installation during total knee arthroplasty (TKA) using a reference axis.Computed tomography (CT) was performed in 75 patients [HA group: 34 patients, 48 knees; osteoarthritis (OA group): 41 patients, 48 knees] during 2017-2019. CT scans were constructed into three-dimensional models. Read More

Haemophilia 2020 Nov 3;26(6):e323-e333. Epub 2020 Oct 3.

Clinical Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.

Introduction: 3D gait analysis has been proposed as a reproducible and valid method to assess abnormal gait patterns and to monitor disease progression in patients with haemophilia (PWH).

Aim: This study aimed at comparing Gait Deviation Index (GDI) between adult PWH and healthy controls, and at assessing the agreement between outcome measures of haemophilic arthropathy.

Methods: Male PWH aged 18-49 years (prespecified subgroups: 18-25 vs 26-49 years) on prophylactic replacement therapy, and male healthy age-matched controls passed through a cross-sectional assessment panel. Read More

Zhonghua Yi Xue Za Zhi 2020 Aug;100(31):2429-2434

Department of Orthopedics, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, China.

To investigate the application and efficacy of the one-stage total knee arthroplasty (TKA) of intra-articular compensation osteotomy in knee osteoarthritis(KOA) patients with extra-articular deformity (EAD). A retrospective study of 9 patients with end-stage KOA and EAD undergoing one-stage TKA from January 2014 to December 2017 in the First Affiliated Hospital of Zhejiang Chinese Medical University was performed. There were 3 males and 6 females with an average age of 56 years(range, 19-77 years);5 cases of simple coronal deformity (varus 10°-27°, mean 18. Read More

Expert Rev Hematol 2020 09 23;13(9):991-1001. Epub 2020 Aug 23.

Department of Physical Medicine and Rehabilitation "La Paz" University Hospital-IdiPaz , Madrid, Spain.

Introduction: After the ankle and knee, the elbow is the most commonly impaired joint in patients with hemophilia.

Areas Covered: A Cochrane Library and PubMed (MEDLINE) search related to elbow problems in hemophilia was conducted. Early and continuous primary hematological prophylaxis (ideally starting in infancy) is essential, given that the juvenile elbow is highly prone to the musculoskeletal complications of hemophilia. Read More

Haemophilia 2020 Jul 25;26(4):701-710. Epub 2020 Jun 25.

Department of Rehabilitation Sciences-Musculoskeletal Rehabilitation Research Group, KULeuven, Brugge, Belgium.

Introduction And Aim: The ankle joint remains vulnerable in children with haemophilia and is the primary joint affected. The purpose of this study was to dynamically characterize the segmental foot and ankle kinematics of male children, adolescents and young adults with or without ankle arthropathy.

Methods: The barefoot multi-segment foot kinematics of 70 ankles from 35 haemophilia subjects between 6 and 20 years old were captured with the Rizzoli Multi-Segment Foot Model. Read More

Expert Rev Hematol 2020 08 5;13(8):803-809. Epub 2020 Jul 5.

Department of Physical Medicine and Rehabilitation, "La Paz" University Hospital-IdiPaz , Madrid, Spain.

Introduction: Iliopsoas muscle hemorrhage in patients with hemophilia is a serious complication with relevant related morbidity.

Areas Covered: A Cochrane Library and PubMed (MEDLINE) search of studies related to iliopsoas hematomas in hemophilia was analyzed. Pain in the hip joint area and femoral nerve involvement in people with hemophilia should alert physicians to the likelihood of a hemorrhage of the iliopsoas muscle. Read More

Haemophilia 2020 Jul 4;26(4):565-574. Epub 2020 Jun 4.

Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Canada.

A goal of the International Prophylaxis Study Group (IPSG) is to provide an accurate instrument to measure MRI-based disease severity of haemophilic arthropathy at various time points, so that longitudinal changes in disease severity can be identified to support decisions on treatment management. We review and discuss in this paper the evaluative purpose of the IPSG MRI scale in relation to its development and validation processes so far. We also critically appraise the validity, reliability and responsiveness of using the IPSG MRI scale in different clinical and research settings, and whenever applicable, compare these clinimetric properties of the IPSG MRI scale with those of its precursors, the compatible additive and progressive MRI scales. Read More

Haemophilia 2020 Jul 22;26(4):685-693. Epub 2020 May 22.

Department of Medical Imaging, University of Saskatchewan and Saskatchewan Health Authority Saskatoon City Hospital, Saskatoon, SK, Canada.

Aim: The objective of this survey was to understand the global trends of imaging assessments in persons with haemophilia, focusing on point-of-care ultrasound (POCUS). Insights into the barriers impeding its widespread proliferation as a frontline imaging modality were obtained.

Methods: The survey opened in September of 2017 and closed in May of 2018. Read More

Appl Radiat Isot 2020 Sep 11;163:109177. Epub 2020 Apr 11.

Department of Nuclear Engineering, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

Radiosynoviorthesis (RSO) is a minimally invasive treatment aiming for the necrosis of the pannus tissue by the use of radionuclide. The method suggested here starts with the segmentation of the joint effusion, synovial thickness, and area of the synovial membrane using the 3D Slicer software. The last step is the estimated value of the activity to be injected without considering the leakage of the radiopharmaceutical into the articular cavity. Read More

Br J Haematol 2020 08 7;190(4):484. Epub 2020 May 7.

Hematology, Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.

Haemophilia 2020 Jul 4;26(4):726-734. Epub 2020 May 4.

Clinical Motion Analysis Laboratorium (CMAL), UZ Leuven, Pellenberg, Belgium.

Introduction: Patients with haemophilia (PwH) often prefer shod walking over barefoot walking as footwear offers ankle joint stability and comfort during gait. Yet, the biomechanical mechanisms contributing to the latter remain poorly understood.

Aim: To explore the effect of shoes on the biomechanical functioning of the ankle and foot complex in PwH with and without haemophilic ankle arthropathy and to determine the amount of ankle joint loading during shod walking. Read More

BMC Musculoskelet Disord 2020 Apr 14;21(1):241. Epub 2020 Apr 14.

Global Drug Discovery, Novo Nordisk A/S, Novo Nordisk Park 1, 2760, Maaloev, Denmark.

Background: Haemophilic arthropathy is a chronic and debilitating joint disease caused by recurrent spontaneous joint bleeds in patients with haemophilia. Understanding how characteristics of individual joint bleeds relate to the subsequent development of arthropathy could improve management and prevention of this joint disease. Here, we aimed to explore relations between joint bleed characteristics and development of bone pathology in a mouse model of haemophilic arthropathy by using novel in vivo imaging methodology. Read More

Medicine (Baltimore) 2020 Apr;99(15):e19788

Department of Radiology, The Third Hospital of Hebei Medical University, Shijiazhuang Hebei Province, China.

Rationale: Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count of unknown causes and is a poorly understood acquired hemorrhagic disease involving destruction of platelets in the reticuloendothelial system induced by antiplatelet antibodies. Patients with ITP can have traumatic intra-articular, intraosseous or soft tissue hemorrhage which may present as a rare intraosseous pseudotumor on medical imaging.

Patient Concerns: A 30-year old male patient had complaint of pain in the right leg for 1 year. Read More

Haemophilia 2020 Mar 15;26(2):333-339. Epub 2020 Jan 15.

Clinical Motion Analysis Laboratorium (CMAL), UZ Leuven, Pellenberg, Belgium.

Introduction: Haemophilic ankle arthropathy due to repeated joint bleeds often leads to altered gait in adult patients with haemophilia.

Aim: To investigate the association between clinical gait features and blood-induced ankle joint damage scored using MRI findings in patients with haemophilic ankle arthropathy.

Methods: This observational study investigated 48 ankles of 24 patients with severe haemophilia (median age of 33 years). Read More

Haemophilia 2020 Jan 9;26(1):142-150. Epub 2019 Dec 9.

Hemophilia-Thalassaemia Center of Mashhad (Sarvar Clinic), Mashhad University of Medical Sciences, Mashhad, Iran.

Introduction: Radiocolloids labelled with less costly and more accessible radionuclides such as rhenium-188 are of interest to developing countries compared with those labelled with rhenium-186 and yttrium-90.

Aim: This study was aimed to evaluate the efficacy and safety of radiosynovectomy using rhenium-188 in patients with chronic haemophilic synovitis and recurrent hemarthrosis.

Methods: In this quasi-experimental prospective study, 20 haemophilic patients were evaluated at preinjection, and at 1, 3, 6 and 12 months after injection. Read More

Haemophilia 2020 Jan 27;26(1):129-135. Epub 2019 Oct 27.

Department of Ultrasound Medicine, Nanjing First Hospital, Nanjing Medical University, Nanjing, China.

Introduction And Aims: Age-related severity and distribution of haemophilic arthropathy (HA) among Chinese patients with haemophilia using the Haemophilia Early Detection with Ultrasound (HEAD-US) system have not been extensively studied.

Methods: In our study, 89 patients with moderate and severe haemophilia were recruited. A total of 534 joints (knees, ankles and elbows on both sides included) were evaluated using musculoskeletal ultrasound (MSKUS) and scored using the HEAD-US system. Read More

World Neurosurg 2020 Jan 25;133:112-120. Epub 2019 Sep 25.

Addis Ababa University College of Heath Science, Addis Ababa, Ethiopia.

Background: Retroclival hematomas are a rare entity and may occur in 3 compartments, namely the epidural, subdural, and subarachnoid spaces. They are frequently secondary to trauma. Hemophilia is a clinical syndrome affecting usually men and characterized by the inherited tendency to bleed excessively after slight injury. Read More

Medicina (Kaunas) 2019 Sep 24;55(10). Epub 2019 Sep 24.

Department of Ergonomics and Physiology of Physical Exercise, Faculty of Health Sciences, Jagiellonian University Collegium Medicum, 31-531 Cracow, Poland.

Concern about weight gain among people has been high due to negative health consequences in addition to the increasing prevalence of the problem. Overweight and obesity also occur in patients with hemophilia. Analysis of literature shows that increased body weight might have a biomechanical effect on the spatial orientation of the pelvis and the lumbar spine. Read More

BMJ Case Rep 2019 Sep 6;12(9). Epub 2019 Sep 6.

Department of Orthopaedic Surgery, Brigham and Women's Hospital, Boston, Massachusetts, USA.

A 55-year-old man with a history of haemophilia A and bilateral haemophilic arthropathy of the elbows presented with an enlarging left elbow mass and worsening paresthesias in the ulnar distribution of the left hand. The mass, originally thought to be olecranon bursitis and treated as such, was found to be due to a haemophilic pseudotumour. The patient underwent successful excision of the haemophilic pseudotumour with concomitant ulnar nerve decompression and had sustained resolution of the pseudotumour and symptoms at 10 years of follow-up. Read More