Management of the elbow joint.

The elbow is a complex joint that is prone to bleeding episodes. These features as well as the close proximity of the ulnar nerve and the need to use the elbow in many activities of daily living can lead to a range of symptoms including recurrent bleeds, pain, instability or loss of range of movement and nerve compression. Conservative management includes splinting and proprioceptive retraining monitored by a physiotherapist who is a musculoskeletal expert in hemophilia care. In the event that conservative measures are not successful a range of surgical options may be indicated including elbow replacement. These approaches continue to be evaluated in both the short and long term in order to determine the most effective treatment for the symptomatic elbow.

Parents of children with haemophilia--a transforming experience.

Owing to the improvements in the management of haemophilia, children with severe haemophilia in the United Kingdom have very different experiences of their condition compared with many adults' early experiences of haemophilia. However, haemophilia can still have physical and social effects which can impact on the quality of life, not only for a child who has the condition, but also for their parents. The purpose of this study was to undertake a qualitative exploration of the impact of haemophilia on parents. The participants included 12 parents of children with severe haemophilia who took part in interviews or focus groups. Four major themes emerged from the data which were initial experiences, managing the condition, engaging with others and developing mastery. The findings highlighted the importance of evaluating parents' experiences. Parents are deeply affected by their child's condition and their lives are transformed by the experiences of living with a child with haemophilia. Parents' responses are influenced by how well the child manages the disorder and the difficulties they experience. Further studies are required in order to develop a more complete understanding of the impact of haemophilia on parent's lives.

Recent developments in clinimetric instruments.

Assessment of impairment and function is essential in order to monitor joint status and evaluate therapeutic interventions in patients with haemophilia. The improvements in the treatment of haemophilia have required the development of more sensitive tools to detect the more minor dysfunctions that may now be apparent. This paper outlines some of the recent developments in this field. The Haemophilia Joint Health Score (HJHS) provides a systematic and robust measure of joint impairment. The MRI Scoring System has been designed to provide a comprehensive scoring system combining both progressive and additive scales. The Functional Independence Score for Haemophilia (FISH) has been developed to assess performance of functional activities and can be used in conjunction with the Haemophilia Activities List (HAL) which provides a self report measure of function. It is recommended that both measures are evaluated as these tools measure different constructs. Further refinement and testing of the psychometric properties of all of these tools is in progress. More widespread use of these tools will enable the sharing of data across the world so promoting best practice and ultimately enhancing patient care.

An exploration of health-related quality of life in adults with haemophilia--a qualitative perspective.

Musculoskeletal dysfunction is a common feature of haemophilia and along with other manifestations of this condition, there is a general perception that health-related quality of life (QoL) will be affected. Previous research using standardized questionnaires has demonstrated that QoL is lower in haemophilia groups compared with normal populations. However, disability studies and interviews with disabled people suggest that many disabled people experience positive life changes as a result of their illness and an affirmative model of disability has been proposed. A qualitative study involving focus groups and interviews was undertaken to explore these issues in a group of 19 severely affected adults with haemophilia. The focus groups and interviews were tape recorded and fully transcribed and the results subjected to thematic analysis. This paper focuses specifically on key issues that impacted on perceptions of QoL. The findings suggest that the participants' perceptions of their QoL were very positive. Possible reasons for this are proposed including the benefits of factor replacement, participants' recollections of their lifestyle before factor replacement, that having haemophilia was integral to the 'self' and finding a niche where they could be successful. A more positive affirmation of haemophilia may need to be considered when developing QoL measures.

Application of Azoxystrobin for Control of Benomyl-Resistant Guignardia citricarpa on 'Valencia' Oranges in South Africa.

Azoxystrobin was evaluated in replicated small-plot trials from 1995 to 1999 for control of citrus black spot (CBS) on 'Valencia' oranges caused by Guignardia citricarpa. Applications of different rates of tank mixes of azoxystrobin and mancozeb during the susceptible period from October to January were compared with an untreated control as well as the standard four applications of mancozeb with or without mineral oil (1.20 g a.i./liter + 0.5% [vol/vol]/liter and 1.60 g a.i./liter of water, respectively). Two applications of azoxystrobin in tank mixtures with mancozeb and mineral oil (0.5% [vol/vol]/liter) in mid-November and mid-January at rates of 0.10, 0.15, and 0.20 g a.i./liter controlled CBS by more than 98 to 99%, 99 to 100% and 95 to 98%, respectively. Concomitantly, where mineral oil was not added to the fungicide mixture, azoxystrobin and mancozeb resulted only in 73 to 95%, 74 to 93% and 92.2 to 92.3% CBS control, respectively. Tank mixtures of benomyl, mancozeb, and mineral oil reduced CBS by only 29%, which could be attributed to the presence of benomyl-resistant pathogen isolates in the experimental orchard. Azoxystrobin applied at rates of 0.05, 0.075, and 0.10 g a.i./liter in tank mixtures with mancozeb (1.2 g a.i./liter) and mineral oil (0.5% [vol/vol]/liter of water) or Agral 90 (0.5% [vol/vol]/liter of water) were equally effective, reducing CBS by more than 99%. When mineral oil was compared to different adjuvants in tank mixtures with azoxystrobin and mancozeb, only mineral oil resulted in 100% clean exportable fruit. There was no difference between Sunspray 6E and Bac oil when mixed with azoxystrobin and mancozeb on the degree of disease control. Furthermore, the concentration of mineral oil in water can be lowered from 0.5% (vol/vol)/liter of water to 0.3% (vol/vol)/liter of water without a loss in efficacy against CBS. It is therefore, recommended that azoxystrobin (0.075 g a.i./liter) must be applied in tank mixtures with mancozeb (1.2 g a.i./liter) and mineral oil, which can be applied at either 0.5% (vol/vol)/liter of water or 0.3% (vol/vol)/liter of water.

Evaluation of outcome of care in patients with haemophilia.

Haemophilia is a potentially disabling condition associated with high financial costs, and so the need for robust measures to evaluate outcome of care is essential. This paper is a review of some of the outcome measures commonly used to evaluate treatment in haemophilia and includes quality of life measures, evaluation of the musculoskeletal system and orthopaedic surgical procedures. Quality-of-life questionnaires are discussed with particular reference to the SF36 and AIMS2. Results of published studies to date have demonstrated variable results. Overall, haemophilia appears to reduce quality of life compared to normal population figures. Several factors are perceived to reduce quality of life, and these include being human immunodeficiency virus (HIV) positive, having impairments, and a history of orthopaedic surgery. The evaluation of the musculoskeletal system is important in order to detect any deterioration over time. Various standardized measurement tools are described. Orthopaedic surgical procedures have also been evaluated in patients with haemophilia. To date, the Hospital for Special Surgery Knee rating scale has been the most commonly used to evaluate the outcome of total knee replacement. The limitations of this system for patients with haemophilia are outlined. Overall, these measures provide useful tools for evaluating outcome, but none have been specifically developed for patients with haemophilia. Further studies would be useful to evaluate these tools and others in more depth.

Experience of prophylaxis treatment in children with severe haemophilia.

The practice of prophylactic treatment of boys with severe haemophilia has been evaluated in our centre. Prophylaxis was started at the median age of 3.7 years (range 0.4-12.7 years) in 38/41 children (93%) under 17 years of age. Median follow-up was 4.1 years (range 0.4-12.7 years). The criteria of primary prophylaxis according to the definition by the European Paediatric Network of Haemophilia Management was fulfilled by 9/38 (24%). Although a majority [76%, 29/38] of the children started prophylaxis after a median number of joint bleeds of 3.5, 70% of the children in this group had clinical joint scores of 0. Intravenous catheter insertion was required at a median age of 15.5 months (range 5-36 months) in 21% of the children, resulting in a catheter infection rate of 1.74 per 1000 catheter days. None developed an inhibitor on prophylaxis and three patients who had low-titre inhibitors (< 5 Bethesda units) prior to prophylaxis had undetectable inhibitors after prophylaxis. The home-treatment training programme required considerable time and cost. As a result, 87% of the children used peripheral venous access and hospital visits declined as prophylaxis became established. Parents' incentives for prophylaxis were that the children undertook many physical activities and sports previously not recommended, there was less parental anxiety and an overall improvement in the quality of life for the whole family.

Total joint arthroplasty in haemophilia.

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.

Muscle rehabilitation in haemophilia.

Musculoskeletal dysfunction is a common manifestation of haemophilia. This dysfunction may be associated with imbalances between muscle groups. Evidence emerging from the literature suggests that the rehabilitation of this dysfunction is very relevant for the patient with musculoskeletal problems. Treatment of muscle imbalance may be linked with a reduction in recurrence of symptoms. Further research is needed to establish the relevance of this area in patients with haemophilia but the clinical evidence supports the developing work in this field.

Conservative treatment of hemarthrosis for prevention of hemophilic synovitis.

Acute hemarthroses are probably the most frequent type of bleeding in the patient with hemophilia. Delayed and/or inadequate treatment can trigger a series of pathologic changes within the joint leading to a painful and disabling arthropathy. Despite the advent of prophylactic treatment with factor concentrates, the majority of patients in the world have no access to even on demand factor replacement. Care for all patients involves a team approach led by the hematologist but including input from orthopaedic surgeons and physiotherapists. Optimal treatment involves a combination of factor replacement, rest, ice, and supervised rehabilitation. In certain cases, joint aspiration may be considered. In developing countries, where factor concentrates are in short supply, such bleeding episodes usually are treated by physical means alone or with the addition of cryoprecipitate or fresh frozen plasma. After successful resolution of such episodes by whatever means, the events leading to the bleeding episode and its subsequent management should be considered within the setting of the treating unit. Such debriefings should aim to provide counsel regarding any appropriate lifestyle modifications and, where necessary, treatment should be arranged to minimize the risk of additional episodes.

Rind Stippling on Valencia Oranges by Copper Fungicides Used for Control of Citrus Black Spot in South Africa.

Four copper sprays and copper mixtures with dithiocarbamates aggravated stippling of the fruit rind of Valencia oranges if sprayed in succession at registered rates during the recommended protection period from October to January for control of citrus black spot in South Africa. Copper stippling was more severe on treatments in which copper oxychloride was sprayed in succession, individually, or in combination with mancozeb or maneb/ZnO. On the other hand, less copper stippling was observed on treatments in which three mancozeb applications were altered with a single copper oxychloride as tank mixtures with or without mancozeb, which was sprayed during midsummer (December and January). Cupric hydroxide resulted in more general copper stippling lesions than any other copper oxychloride spray program. Four successive applications of the wettable powder copper oxychloride formulation resulted in more copper stippling when compared with the suspension concentrate formulation. Stippling was calculated to be more severe with late applications of copper fungicides during December and January. In another experiment, all contact fungicides tested were effective in controlling citrus black spot.

Counselling guidelines for managing musculoskeletal problems in haemophilia in the 1990s.

Complex medical, psychological and social factors affect how patients with haemophilia and their families respond to bleeding episodes. Prompt, adequate treatment for bleeds remains the best way to preserve optimal musculoskeletal health. Untreated and under treated bleeding episodes into muscles and joints can cause pain, deformity and severely limit the daily activities of patients with haemophilia. Viral infections over the last decade have, to some extent, diverted attention away from musculoskeletal problems. The orthopaedic contribution to haemophilia care remains important and can be enhanced through regular review clinics and developing focused approaches to identifying and addressing musculoskeletal problems.

Pathogenesis of upper cervical instability.

SUMMARY. The purpose of this article is to review the pathogenesis of upper cervical instability. Instability can arise from inflammatory, congenital and traumatic causes. The commonest causes of atlanto-axial dislocation are rheumatoid arthritis and Down's syndrome. The review revealed much less information about the relatively minor instabilities that are probably responsible for a number of chronic complaints of the type seen by manual therapists. The potential involvement of passive (ligamentous), active (musculotendinous) and neural control subsystems in maintaining the stability of the spine may go some way to explaining the spectrum of conditions included under the term clinical instability. Copyright 1996 Harcourt Publishers Ltd.