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1.
Haemophilia ; 30 Suppl 3: 120-127, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38578694

RESUMEN

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. Point-of-care ultrasound is emerging as a safe, cost-effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed-synovitis-rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non-surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important.


Asunto(s)
Artritis , Hemofilia A , Sinovitis , Humanos , Hemofilia A/complicaciones , Hemofilia A/terapia , Hemofilia A/diagnóstico , Calidad de Vida , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Sinovitis/diagnóstico , Sinovitis/etiología , Sinovitis/terapia , Envejecimiento , Artrodesis
2.
Artículo en Inglés | MEDLINE | ID: mdl-36987770

RESUMEN

Introduction: Avascular necrosis or osteonecrosis of the femoral head is defined as a pathological process that results in a critical reduction in the blood supply to the hip head with increased intraosseous pressure. Avascular necrosis is a multifactorial disease. The treatment used so far consists of transosseous decompression of the femoral head and is the most commonly used method in the early stages of osteonecrosis. Aim: The aim of this study was to evaluate the results after application of bone marrow stem cells obtained from the pelvic ridge, which was applied to the necrotic zone after previous decompression. Material and methods. The study is of prospective character and included 30 patients with first, second, and third degrees of AVN according to the Ficat classification, determined with X-ray. The range of motion in the hip was examined preoperatively by using a goniometer, a radiological evaluation of the degree of AVN was performed, according to the Ficat classification, and the VAS scale and the Harris Hip Score were examined preoperatively and postoperatively. Results: The average value for the Harris Hip Score (HHS) preoperatively was: for Ficat I -66.32±5.1, for Ficat II - 49.19±3.4 and for Ficat III - 33.71±2.1. At the 3-month postoperative follow-up, the average HHS values were: 87.92±4.3 for Ficat I, 78.64±6.6 for Ficat II and 76.48±2.6 for Ficat III. The same values for HHS were obtained in the control examinations at the 6th and 12th month postoperatively, indicating the fact that good bone regeneration was achieved and the progression of the condition was prevented. A decrease in the HHS value was observed at the control examination at 1 year after the surgical treatment, in 3 patients according to the Ficat classification of grade III, in whom a total hip replacement surgery was performed. Conclusion: The use of stem cells in the treatment of avascular necrosis of the femoral head has achieved good functional results and reduced pain in operated patients. Radiographically, good bone regeneration was achieved and the progression of necrosis to a higher degree was prevented.


Asunto(s)
Necrosis de la Cabeza Femoral , Humanos , Resultado del Tratamiento , Estudios de Seguimiento , Necrosis de la Cabeza Femoral/diagnóstico por imagen , Necrosis de la Cabeza Femoral/cirugía , Cabeza Femoral/cirugía , Estudios Prospectivos , Médula Ósea/cirugía , Descompresión Quirúrgica/métodos , Células Madre
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