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Haemophilic Arthropathy (haemophilic + arthropathy)

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Medical Sciences	100%

Selected Abstracts

Co-morbidity in the ageing haemophilia patient: the down side of increased life expectancy

HAEMOPHILIA, Issue 4 2009
E. P. MAUSER-BUNSCHOTEN
Summary., Because of an increased life expectancy, (age-related) co-morbidity is becoming a common occurrence in haemophilia patients. In this review, haemophilia-related and non-haemophilia-related medical problems, treatment recommendations and psychosocial consequences in ageing haemophilia patients are discussed. Haemophilic arthropathy is an important cause of pain and disability, and a frequent indication for surgery in haemophilia patients. In addition, many adult patients are infected with hepatitis C or HIV, the consequences and treatment of which can add to physical and mental discomfort. Moreover, inhibitors against factor VIII can also develop in adulthood, especially in patients with mild haemophilia. Hypertension is reported to occur more often in haemophilia patients than in the general population. Other internal problems, like renal abnormalities, overweight, diabetes mellitus and hypercholesterolemia are discussed. Haemophilia seems to protect against cardiovascular disease, although the incidence is increasing. Recommendations are given on dealing with tooth extractions, surgical interventions and sexuality problems in patients with haemophilia. In addition to haemophilia in itself, co-morbidity has a major psychological impact, and an important effect on quality of life. It can also result in complex treatment regimens, in which coordination between health care workers is essential. [source]

Force fluctuations during the Maximum Isometric Voluntary Contraction of the quadriceps femoris in haemophilic patients

HAEMOPHILIA, Issue 1 2007
L.-M. GONZÁLEZ
Summary., In the general population, the degenerative processes in joints are directly related to adult age, and osteoarthrosis represents the most frequent musculoskeletal alteration. In the haemophilic patient, the degenerative processes in the joint begin at very early ages, and are directly related to musculoskeletal bleeding episodes, which are occasionally subclinical and constitute haemophilic arthropathy. In the haemophilic patient, arthropathy constitutes the most frequent, severe and disabling pathology, and its assessment includes muscular force-related parameters. We have studied the value of Maximum Isometric Voluntary Contraction in the quadriceps femoris of 46 subjects, 28 haemophiliacs (16 severe, eight moderate and four mild) and 18 healthy individuals with a view to establishing appropriate values of force and to restoring physical therapy recommendations. The maximum force values were significantly greater (P < 0.001) in the healthy individuals group. The mild haemophiliacs group also presented significant differences of force (P < 0.05) in relation to the severe and moderate haemophilic patient groups. The mild and severe haemophilia patients presented greater fluctuations of force (P < 0.001) than the control group, the haemophilia group have a minor skill to produce constant force. The seriousness of the arthropathy in the knee is directly related to diminished values of maximum force. Our work evidences that patients with severe haemophilia present a greater degree of arthropathy in relation to moderate and mild haemophilia patients. Haemophilic arthropathy is associated with muscular atrophy and strength deficit. In haemophilic patients, the deficit of maximum force and the presence of fluctuations may suggest an increased risk of bleeding during physical activities and the need to programme specific physical therapy guidelines which increase muscular power through resistance training. [source]

Clinical and radiographic scores in haemophilic arthropathies: how well do these correlate to subjective pain status and daily activities?

HAEMOPHILIA, Issue 6 2002
T. Wallny
Summary. Haemophilic patients who reached adulthood before the establishment of prophylactic treatment frequently show multiple and substantial arthropathies. The aim of this study was to determine to what extent haemophiliac's subjective impairment due to arthropathies correlates with objective clinical and radiographic parameters. By means of a questionnaire and a visual analogue scale, we consulted 79 haemophiliacs concerning their joint-pain status, how these were treated and to what extent their daily activities had been affected. Using a scoring system suggested by the Advisory Committee of the World Federation of Haemophilia, clinical evaluation was performed. Radiographs of 60 patients were assessed by means of the Petterson scale. The results were statistically compared. We found a significant correlation between pain intensity and clinical pathology as well as between pain intensity and radiographic joint damage for both knees and for the right ankle. The number of painful joints correlated well with the number of clinically/radiographically affected joints. The more pronounced the objective damage to joints, the more frequently patients claimed to have constant pain, depressive episodes and a dependency on pain-relieving medication. The more pronounced the objectively assessed damage to the knee and ankle joint, the higher the likelihood that the patient suffers from severe joint pain and reduction of activity. Treatment of painful symptoms from arthropathies is often insufficient. Scores and questionnaires may help to define the haemophiliacs pain status more clearly, thereby offering a possibility of assessment and long-term observation. [source]

Current approaches in haemophilic arthropathy of the hip

HAEMOPHILIA, Issue 3 2009
H. A. MANN
Summary., The hip is considered to be one of the main load bearing joints of the body. In the haemophilic patient joint bleeds can be catastrophic, leading to long-term joint degeneration and accompanying arthritis. In this review we explore the mechanisms of joint destruction, with particular consideration of the anatomy of the hip and how it may influence disease progression. We also review current strategies for treatment including hip replacement in the haemophilic patient and describe our experiences as a unit. Finally we evaluate future prospects in the management of hip disease in haemophilia. [source]

The benefits of prophylactic treatment with APCC in patients with haemophilia and high-titre inhibitors: a retrospective case series

HAEMOPHILIA, Issue 3 2009
L. A. VALENTINO
Summary., Prophylactic infusion of factor concentrates is a safe, effective intervention for preventing arthropathy in patients with haemophilia; on-demand treatment is insufficient to prevent the orthopaedic complications and subsequent haemophilic arthropathy that stem from recurrent joint haemorrhages. The usefulness of prophylaxis in haemophilia patients without inhibitors suggests that patients with haemophilia and inhibitors could derive similar benefits. In patients with haemophilia and high-titre (>5 BU mL,1) inhibitors, bleeding episodes are treated with bypassing agents such as activated prothrombin complex concentrates (APCCs) and recombinant activated factor VII (rFVIIa, NovoSeven®; Novo Nordisk A/S, Bagsvaerd, Denmark). It is possible to administer bypassing therapy regularly to prevent haemorrhages, with the goal of limiting arthropathy and serious life- and limb-threatening bleeding. The data evaluating the efficacy and safety of this approach in patients with inhibitors are limited, consisting of results from one prospective trial and retrospective case reports. This report describes our experience with the prophylactic use of the APCC Factor Eight Inhibitor Bypassing Activity, Anti-Inhibitor Coagulant Complex, Vapor Heated (FEIBAÔ; Baxter AG, Vienna, Austria). Data from patients at one treatment centre were retrospectively evaluated. Case records of six patients with haemophilia A or B and high-titre inhibitors were identified. When APCC was administered regularly, most patients exhibited a reduction in the numbers of haemorrhages, an improvement in orthopaedic status, and an improvement in quality of life. Prophylaxis with APCC can reduce haemorrhages and halt further joint deterioration in patients with haemophilia and inhibitors. [source]

Inter- and intra-observer reliability of radiographic scores commonly used for the evaluation of haemophilic arthropathy

HAEMOPHILIA, Issue 3 2008
M. SILVA
Summary., Although the severity of haemophilic arthropathy is commonly assessed using established radiographic scoring systems, there is limited available information about their inter- and intra-observer reliability. The purpose of the present study was to establish the inter-observer reliability (IEOR) and intra-observer reliability (IAOR) of three different methods available for the classification of haemophilic arthropathy, including the Arnold and Hilgartner classification, a modification to the Arnold and Hilgartner system described by Luck et al., and the classification described by Pettersson et al. Antero-posterior and lateral radiographs of 54 haemophilic joints were included for the analysis. To determine the IEOR for each one of the three radiographic systems, the radiographs were randomly evaluated by four observers, including two orthopaedic surgeons, one orthopaedic resident and one haematologist. For the determination of IAOR, all four reviewers repeated the assessment in a similar fashion, after a period of at least 2 weeks. IEOR and IAOR for the three classification systems was established using kappa (,) statistics. A Spearman rank correlation was used to determine the similarities between each reviewer's own interpretative scales. The IEOR was low for the Arnold and Hilgartner system (, = 0.35, P , 0.001) and the Luck system (, = 0.38, P , 0.001), but even lower for the Pettersson system (, = 0.06, P = 0.1). For the Pettersson system, particularly low , values were observed for the presence or absence of osteoporosis (, = 0.11, P = 0.0027), enlarged epiphysis (, = 0.10, P = 0.0039), erosion of joint margins (, = 0.11, P = 0.0018), and joint deformity (, = 0.16, P = 0.00001). However, a relatively high Spearman rank correlation for all three scales [rs = 0.75 (P < 0.001) for Arnold and Hilgartner system, rs = 0.74 (P < 0.001) for the Luck system and rs = 0.81 (P < 0.001) for Pettersson system] indicated an overall, general agreement among the reviewers with regard to the severity of the haemophilic arthropathy. There was a moderate IAOR value for both, the Arnold and Hilgartner system (, = 0.57, P = 0.00001) and the Luck system (, = 0.62, P = 0.00001) with a low IAOR value for the Pettersson system [, = 0.22, P = 0.00001). Currently available radiographic scoring systems for haemophilic arthropathy have low inter- and intra-observer reliability rates. Improvements, either through education or modification of the scoring systems, are critical in an era where correlations between clinical and radiographic scores have received significant attention. [source]

The co effect of prophylaxis and radiosynovectomy on bleeding episodes in haemophilic synovitis

HAEMOPHILIA, Issue 3 2008
J. BRECELJ
Summary., Prophylactic substitution treatment and radiosynoviorthosis have a leading role in preventing irreversible haemophilic arthropathy. The aim of the study was to evaluate the effects of prophylaxis treatment and radiosynovectomy on the length of intervals between subsequent haemorrhages in haemophilic patients. Thirty-three joints were treated with radiosynovectomy in 28 patients with bleeding disorders. 90Y colloid was used in knees and 186Re colloid for elbows, shoulders and ankles. Twenty patients were on prophylaxis. Joint X-rays were evaluated on the Pettersson scale between 0 (normal) and 13 (severe joint destruction). During an observation period (range 6,44 months) bleeding episodes were recorded and data statistically analysed. Before radiosynovectomy, increasing intensity of the prophylaxis 10% lengthens intervals between two haemorrhages by 1% (P < 0.05). In patients with a Pettersson score higher than nine, intervals between bleedings are shorter by 73% (P < 0.05), in comparison with patients with lower Pettersson scores of 0,5. After radiosynovectomy, the length of the first non-bleeding interval increased by 120% (to 60 days) in comparison with the intervals before the procedure (P < 0.001). But, in the following year and half, every subsequent non-bleeding interval was 8% shorter (P < 0.1). In that period, prophylaxis shortened the non-bleeding interval by 1.7% (P < 0.05) per 10% increase of its intensity. Radiosynovectomy is more efficient in patients with less affected joints and is less efficient in younger patients. Prophylaxis reduced time between the bleedings episodes after isotope application. Before radiosynovectomy, prophylaxis reduces the number of haemorrhages. Our findings support data previously published by Rodriguez-Merchan et al. [J Thromb Haemost, 5 (2007) P-W-126]. [source]

An outline of the current orthopaedic management of haemophilic disease of the upper limb

HAEMOPHILIA, Issue 5 2007
M. Z. B. CHOUDHURY
Summary., There remains a relative paucity in the literature regarding upper limb manifestations of haemophilic arthropathy. Haemophilia has a wide range of clinical manifestations, often presenting with orthopaedic complications. These arise from multiple haemarthroses which exact a cumulative toll on the fabric of the joints. Although the lower limbs are predominantly affected due to their load-bearing nature, upper limb disease is common. This arises from the mechanical demands on the upper limb as the elbow and shoulder become partially weight bearing on use of walking aids such as elbow crutches. [source]

Force fluctuations during the Maximum Isometric Voluntary Contraction of the quadriceps femoris in haemophilic patients

The Colorado Haemophilia Paediatric Joint Physical Examination Scale: normal values and interrater reliability

HAEMOPHILIA, Issue 1 2007
M. R. HACKER
Summary., ,Persons with haemophilia often experience their first joint haemorrhage in early childhood. Recurrent bleeding into a joint may lead to significant morbidity, specifically haemophilic arthropathy. Early identification of the onset and progression of joint damage is critical to preserving joint structure and function. Physical examination is the most feasible approach to monitor joint health. Our group developed the Colorado Haemophilia Paediatric Joint Physical Examination Scale to identify earlier signs of joint degeneration and incorporate developmentally appropriate tasks for assessing joint function in young children. This study's objectives were to establish normal ranges for this scale and assess interrater reliability. The ankles, knees and elbows of 72 healthy boys aged 1 through 7 years were evaluated by a physical therapist to establish normal ranges. Exactly 10 boys in each age category from 2 to 7 years were evaluated by a second physical therapist to determine interrater reliability. The original scale was modified to account for the finding that mild angulation in the weight-bearing joints is developmentally normal. The interrater reliability of the scale ranged from fair to good, underscoring the need for physical therapists to have specific training in the orthopaedic assessment of very young children and the measurement error inherent in the goniometer. Modifications to axial alignment scoring will allow the scale to distinguish healthy joints from those suffering frequent haemarthroses. [source]

A new magnetic resonance imaging scoring method for assessment of haemophilic arthropathy

HAEMOPHILIA, Issue 4 2004
B. Lundin
Summary., In a European multicentre study, 39 ankles in 28 haemophilic boys were investigated by magnetic resonance imaging (MRI). A new MRI score was developed in the format A(e:s:h) for evaluating haemophilic arthropathy. This scheme provides high resolution and allows separation of different pathological components. The factor A is calculated as the sum of scores for subchondral cysts (maximum value 6), irregularity/erosion of subchondral cortex (maximum 4) and chondral destruction (maximum 6); e, s and h, respectively, represent effusion/haemarthrosis, synovial hypertrophy and haemosiderin deposition, and they are separately evaluated on a scale of 0,4. Working independently, two radiologists scored the 39 ankles twice using both this new ,European' scoring method and a previously published ,Denver' scoring scheme. Final classification was achieved by consensus. The reproducibility of the readings was assessed, and for both scoring methods the results indicated good or moderate intraobserver agreement, and good, moderate or fair interobserver agreement. These findings suggest that MRI can be useful for semiquantitative evaluation of haemophilic arthropathy, providing the examination is performed according to an appropriate protocol, and the images are evaluated by specially trained radiologists. [source]

Experimental haemophilic synovitis: rationale and development of a murine model of human factor VIII deficiency

HAEMOPHILIA, Issue 3 2004
L. A. Valentino
Summary., Haemophilia is a genetic disease as a result of the deficiency of blood coagulation factor VIII or IX. Bleeding is common, especially into joints where an inflammatory, proliferative synovitis develops resulting in a debilitating arthritis, haemophilic arthropathy. The pathogenesis of blood-induced haemophilic synovitis (HS) is poorly understood. The gross, microscopic and ultrastructural changes that occur in the synovial membrane following human and experimental hemarthrosis have been described. Repeated episodes of bleeding induce synoviocyte hypertrophy and hyperplasia, an intense neovascular response and inflammation of the synovial membrane. The component(s) in blood that initiates these changes is(are) not known, although iron is often proposed as one possibility. Here, we describe a novel murine model of human haemophilia A, which facilitates the examination of large number of animals and tissue specimens. The effects of hemarthrosis on the physical, gross and microscopic changes evoked following joint bleeding are described. Controlled, blunt trauma to the knee joint consistently resulted in joint swelling because of a combination of bleeding and inflammation. Hemosiderin was found in the synovial membrane. Similar to hemarthrosis in human haemophilia, joint bleeding resulted in acute morbidity evidenced by inactivity, weight loss and immobility. With time the animals recovered. The model of experimental murine HS described here has utility in the study of the pathogenesis of HS. This is the first of a series of articles, which will discuss the pathophysiology and characterize the model, with comparison of his model to others which have been published previously. It should provide a useful model to test potential therapeutic interventions. [source]

Prophylactic therapy for haemophilia: early experience

HAEMOPHILIA, Issue 2003
E. Berntorp
Summary., During the 1960s, it was reported from Sweden that haemophiliacs with factor levels above 1% rarely develop arthropathy. This observation suggested that severe haemophilia could be converted to a milder form by regular infusions with factor concentrate. After several earlier publications, a report was published in 1992 that detailed 25 years' experience with prophylaxis in 60 patients from the Malmö centre. The results showed that starting prophylaxis early in life with a dose regimen that would prevent factor VIII or IX plasma levels from falling below 1% could prevent the development of haemophilic arthropathy. Also, older age groups who had received less intensive treatment, and who started prophylaxis later in life, were still in a much better condition than historic controls. In the 1970s several small but well-controlled studies from the USA, Germany and Italy clearly showed the benefit of prophylaxis in reducing bleeding frequency. Early experience from the Netherlands was published in 1971. Since these early studies, the results have been corroborated from many countries and in a large multinational study. Although the benefits of prophylaxis seem unquestionable, several research questions remain to be better elucidated, such as when to start and when to stop, dosing and dose interval, and how to assess the long-term treatment effects. These issues are of great economic importance, and the need for health economical studies is obvious. [source]

Changes in treatment strategies for severe haemophilia over the last 3 decades: effects on clotting factor consumption and arthropathy

HAEMOPHILIA, Issue 5 2001
K. Fischer
A cohort study was performed among 214 patients with severe haemophilia, born 1944,1994, to describe changes in treatment over the last 3 decades and its effects on clotting factor consumption and haemophilic arthropathy. Data on treatment strategy, clotting factor consumption, and outcome were collected for 3567 patient years (from 1972 to 1998), and 493 Pettersson scores were analysed. Median follow up was 17 years (range 6,27 years), and median age in 1998 was 27.6 years. Since 1965, replacement therapy, prophylaxis, and home treatment have been used and treatment intensified. Over the last 3 decades, annual clotting factor consumption increased by 260%, for both prophylactic and on-demand treatment. Annual clotting factor consumption kg,1 increased during childhood and appeared to stabilize in early adulthood for patients born 1965,79, who were treated with early replacement therapy or early prophylaxis. In contrast, clotting factor consumption increased continuously for patients born before 1965, who had had no access to replacement therapy during the early years of their life. The annual number of joint bleeds decreased over the years. Arthropathy as measured by the Pettersson score generally became apparent around the age of 15 years and was lowest in patients treated with primary prophylaxis. In conclusion, clotting factor consumption has increased and haemophilic arthropathy has decreased due to the intensification of treatment for severe haemophilia over the last 3 decades. Annual clotting factor consumption stabilizes in adulthood for patients who receive early intensive treatment. [source]

Total joint arthroplasty in haemophilia

HAEMOPHILIA, Issue 5 2000
Beeton
In severely affected haemophilic patients arthropathy is a common problem which can lead to considerable pain and functional deficit. Surgical management, including total joint arthroplasty, can be undertaken if conservative management fails. A search of the literature showed that a number of studies describing the use of total knee arthroplasty (TKA) and total hip arthroplasty (THA) in haemophilia have been published, whereas shoulder, elbow and ankle arthroplasties are confined to case reports. This paper reviews the functional outcome of arthroplasty in the different joints, the postoperative and long-term complications, and the impact of HIV. Although complications are commonly described and the surgery is technically demanding, the results suggest that arthroplasty, particularly of the hip and knee, can be a valuable option in the management of severe haemophilic arthropathy. [source]

Imaging of the haemorrhagic complications of the haemophilias

JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 1 2009
S Constantine
Summary Haemorrhage is the main manifestation of the haemophilias. Although acute haemorrhages can be life threatening, especially if involving the central nervous system, repeated haemorrhages involving the musculoskeletal system lead to two conditions unique to patients with haemophilias. This pictorial essay reviews the typical imaging appearances of haemorrhages and consequences in those with haemophilias, with particular emphasis on haemophilic arthropathy and pseudotumours. [source]