Haemophilic arthropathy; a case report (original) (raw)
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Study of arthropathy in haemophilia a
International journal of applied research, 2016
Introduction: Haemophilia is the most common inherited X linked severe bleeding disorder. Screening and early detection of haemophilic arthropathy along with prophylactic factor and physiotherapy decreases complication and disability. Hence this study was conducted to detect arthropathic changes and its severity in haemophilic children. Aims and Objectives: To study cases of arthropathy and to assess the severity of arthropathy in Haemophilia A patients. Methodology: Diagnosed cases of Haemophilia A Patients who came to Dr. D.Y. Patil Medical College, Pune were enrolled during study period i.e. October 2013 to September 2015 who fulfilled the inclusion criteria. A standard proforma was used to get the detailed history and the examination findings along with X ray Knee and MRI Knee were evaluated and statistically analysed. Results: Out of 50 subjects majority 84% were symptomatic & 16% were asymptomatic. Pain was the major complaint in 84%, followed by swelling 80% and restriction o...
Journal of Orthopaedics, 2019
Background: Haemophilia is characterized by a partial or complete deficiency of clotting factor VIII or IX. The repeated bleeding episodes into the joints contributes to subsequent chronic arthropathy which debilitates the patient and affects the quality of life. There are lot of hurdles in successful management of such patients. We present our experience in dealing such patients. Materials and Methods: 24 patients (27 knees) with haemophilic knee arthropathy were included. The preoperative VAS (Visual Analogue Scale) and KSS (Knee Society Score) were assessed. All underwent total knee arthroplasty with stemmed constrained prosthesis. Postoperatively VAS and KSS were analysed at the end of 12 months. Results: The mean preoperative VAS was 8.2 (Range: 7-10), mean KSS clinical score was 39.9 (Range: 12-61) and mean KSS functional score was 51.4(Range: 20-70). The mean postoperative VAS was 2.7(Range: 2-4), mean KSS clinical score was 70.5 (Range: 61-80) and mean KSS functional score was 74.2(Range: 60-80). There was statistical significant difference between preoperative and post-operative scores. At the end of 12 months, 17 patients (63%) had good outcome, seven patients (26%) had fair and three patients (11%) had excellent outcome as per the KSS scores. Conclusions: Total knee arthroplasty has good outcome with respect to clinical as well as functional outcome in haemophilic arthropathy. Although the results and outcome of total knee arthroplasty in haemophilic knee arthropathy may still be inferior to the results seen in a normal individual, a multidisciplinary approach yields a better functional and quality of living in such haemophilic patients.
Journal of Orthopaedics, 2019
Background: Haemophilia is characterized by a partial or complete deficiency of clotting factor VIII or IX. The repeated bleeding episodes into the joints contributes to subsequent chronic arthropathy which debilitates the patient and affects the quality of life. There are lot of hurdles in successful management of such patients. We present our experience in dealing such patients. Materials and Methods: 24 patients (27 knees) with haemophilic knee arthropathy were included. The preoperative VAS (Visual Analogue Scale) and KSS (Knee Society Score) were assessed. All underwent total knee arthroplasty with stemmed constrained prosthesis. Postoperatively VAS and KSS were analysed at the end of 12 months. Results: The mean preoperative VAS was 8.2 (Range: 7-10), mean KSS clinical score was 39.9 (Range: 12-61) and mean KSS functional score was 51.4(Range: 20-70). The mean postoperative VAS was 2.7(Range: 2-4), mean KSS clinical score was 70.5 (Range: 61-80) and mean KSS functional score was 74.2(Range: 60-80). There was statistical significant difference between preoperative and post-operative scores. At the end of 12 months, 17 patients (63%) had good outcome, seven patients (26%) had fair and three patients (11%) had excellent outcome as per the KSS scores. Conclusions: Total knee arthroplasty has good outcome with respect to clinical as well as functional outcome in haemophilic arthropathy. Although the results and outcome of total knee arthroplasty in haemophilic knee arthropathy may still be inferior to the results seen in a normal individual, a multidisciplinary approach yields a better functional and quality of living in such haemophilic patients.
Comparison of the clinical and ultrasound evaluation of haemophilic arthropathy in children
Gaceta Médica de México
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 2.1 scale and ultrasound with a linear transducer of 8 to 12 MHz. Elbows, knees and ankles joints were evaluated bilaterally, with HEAD-US protocol. Results: 69 paediatric patients were included of which 48 with severe haemophilia A (weight: 40.1 kg). On the HJHS scale, a greater involvement was observed in the left knee (0.49), and less in the right ankle (0.05). With the HEAD-US scale, the most affected was the right knee (0.78). There is a significant relationship in the involvement of the right knee evaluated with the HEAD-US scale in the presence of inhibitor. Conclusions: Weight above the 50 th percentile is an independent risk factor for joint bleeding complications, while age and type of haemophilia do not appear to be related. The HEAD-US method is a useful and accessible tool for early detection of arthropathy and hemarthrosis.
Assessment of the progression of haemophilic arthropathy in children
Haemophilia, 2010
Arthropathy is considered as an irreversible and progressive complication in patients with haemophilia, even in children on prophylaxis. To estimate the progression of haemophilic arthropathy, 85 joints of 24 boys with severe (n = 18) and moderate (n = 6) haemophilia (A: 22, B: 2) were investigated with clinical examination, X-rays and magnetic resonance imaging (MRI) at two time periods (time 0 and 1). Patients' age at time 0 was 10.5 ± 3.6 years and time elapsed to time 1 was 3.8 ± 1.4 years. At time 0: all investigated joints had more than three bleeds. Sixteen boys were on secondary prophylaxis for 5.4 ± 2.8 years. Clinical score (a modification of World Federation of Haemophilia's scale): 2.0 ± 3.6, X-ray score (Pettersson): 2.1 ± 2.8, MRI score (Denver): 4.5 ± 3.8. After the first evaluation, prophylaxis was intensified in 11 children and initiated in four. At time 1: clinical score: 1.5 ± 3.1, X-ray: 1.7 ± 2.7, MRI score: 5.1 ± 4.1. On average, the clinical and X-ray scores showed a significant improvement (26% and 40% of the joints respectively, P < 0.01) and the number of haemarthroses evidenced a threefold reduction from time 0 to 1 (P < 0.01), findings that could be associated with the modification of prophylaxis after time 0. MRI findings showed deterioration in 34% of the joints. Conversely, 14 joints (16.5%) with mild or moderate synovitis without cartilage degradation at time 0 showed an improvement at time 1. The information carried by the three scales could be divided into information shared by the three scores and information specific to each score, thus giving a more complete picture of joint damage caused by bleedings.
Progression of haemophilic arthropathy in children: a Lithuanian - Danish comparative study
Haemophilia, 2013
Recurrent bleeding into joints initiates a sequence of events leading to a progressive joint damage in people with severe haemophilia. This is a continuous process during childhood and adolescence, therefore joint abnormalities may be minimal on physical examination in very young childreneven those receiving on-demand treatment. The aim of our study was to quantify the burden of arthropathy in Lithuanian patients who had been treated exclusively by on-demand substitution and compare their physical joint health with age-matched Danish patients who received prophylaxis from an early age. Boys, aged 4-17 years, with severe haemophilia and no signs of inhibitors were included in the study. Joint outcome based on the Haemophilia Joint Health Score (HJHS) was analysed in two different treatment groups and compared within the matched pairs. In total, 32 (16 in each treatment group) patients were enroled. A total of 192 joints were evaluated. Joint status according to treatment strategy was strikingly different: 27.4 for on-demand vs. 3.3 for prophylaxis (<0.001) group. Significance of the difference in joint status comparing different treatment strategies was equally strong both in younger (4-9 years) and older (10-17 years) patient groups: 2.2 vs. 12.5 (P = 0.0002) and 3.9 vs. 36.3 (P < 0.0001) respectively. The results further demonstrate the unequivocal effect of prophylaxis on joint status and give an insight into early and late manifestations of joint impairment based on the HJHS in haemophilia patients with treatment on-demand compared with joint changes that may develop over the time with the preventative treatment.
Haemophilia and joint disease: pathophysiology, evaluation, and management
In patients with haemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of haemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and infl ammation (haemophilic synovitis) that contribute to end-stage degeneration (haemophilic arthropathy); with pain and limitation of motion severely affecting patients' quality of life. If joint bleeding is not treated adequately, it tends to recur, resulting in a vicious cycle that must be broken to prevent the development of chronic synovitis and degenerative arthritis. Effective prevention and management of haemophilic arthropathy includes the use of early, aggressive prophylaxis with factor replacement therapies, as well as elective procedures, including restorative physical therapy, analgesia, aspiration, synovectomy, and orthopaedic surgery. Optimal treatment of patients with haemophilia requires a multidisciplinary team comprising a haematologist, physiotherapist, orthopaedic practitioner, rehabilitation physician, occupational therapist, psychologist, social workers, and nurses.
Hemophilic Arthropathy: Barriers to Early Diagnosis and Management
Journal of Blood Medicine
Hemophilia is a congenital coagulopathy characterized by a deficiency of one of the clotting factors. It is characterized by the development of hematomas and hemarthrosis, either spontaneously or after minor trauma. The recurrence of hemarthroses leads to progressive and degenerative joint damage from childhood (hemophilic arthropathy). This arthropathy is characterized by disabling physical effects that limit the functionality and quality of life of these patients. Medical progress achieved over the last decade in the drug treatment of hemophilia has improved the medium and long-term prospects of patients with more effective and long-lasting drugs. The universal use of safer, more effective and prolonged prophylactic treatments may promote the prevention of bleeding, and also therefore, of the development of hemarthrosis and joint damage. A number of imaging instruments have been developed for the assessment of hemarthrosis and hemophilic arthropathy, using ultrasound, magnetic resonance imaging and simple radiology. Different physical examination scores and questionnaires allow the assessment of joint health, self-perceived activity and functionality of patients with hemophilia. The approach to these patients should be interdisciplinary. Assessment of the processes that affect pain in these patients and the development of pain education models should be implemented. Expert advice and information to patients with hemophilia should be based on individual functional prevention diagnoses, advice on available therapies and sports practice, as well as health recommendations.
Management of haemophilic arthropathy
…, 2008
Despite the tremendous benefit offered by primary prophylaxis, recurrent joint bleeding with progression to chronic synovitis and haemophilic arthropathy is still a daily concern for the multidisciplinary health care teams managing patients with severe haemophilia or haemophilia complicated by inhibitor development. Advanced stages of arthropathy could be prevented by regular assessment of musculoskeletal status and thus early detection of symptoms, daily rehabilitation exercises at home, and implementation of appropriate physiotherapy and medical training. PatientÕs education and psychological counselling are crucial. New tools such as magnetic resonance imaging are promising for the monitoring of these patients and might promote early detection of arthropathy and thus appropriate preventive measures to avoid further joint deterioration can be implemented. Medical synovectomy such as radionucleide synoviorthesis is a simple and noninvasive procedure that often delays the need for surgery which despite considerable improvement in techniques and postoperative rehabilitation remains a high-risk strategy in patients with severe haemophilia, especially those with inhibitors. In these high risk patients, availability of specific clotting factors such as activated prothrombin complex concentrate (FEIBA Ò , Baxter, Vienna, Austria) and more recently, recombinant factor VIIa (rFVIIa, NovoSeven Ò , Bagsvaerd, Denmark) has allowed to perform effective and safe orthopaedic procedures. The ongoing EUREKA study will undoubtedly provide additional information about the optimal use of rFVIIa in this context.