Handwriting Analysis in Children and Adolescents with Hemophilia: A Pilot Study.

BACKGROUND: Handwriting is a complex task that requires the integrity of different sensorimotor components to be performed successfully. Patients with hemophilia suffer from recurrent joint bleeds that may occur in the elbow, causing elbow dysfunction with handwriting performance impairment. In our study, we described instrumental dysgraphia that is related to functional disturbances. This pilot study aims to evaluate the handwriting performance in a group of patients with hemophilia. METHODS: The study was performed at the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center in Milan. Boys with severe and moderate hemophilia A and B regularly followed-up at that Center, with age between 6-19 years, were eligible. Patients were invited to the Center for one multidisciplinary evaluation of the upper limbs that included: Clinical examination, surface electromyography, and handwriting assessment. RESULTS: All patients, but one, completed handwriting assessment. Overall, 14/19 (74%) had abnormal handwriting, which was overt instrumental dysgraphia in six (32%). There was no difference in Hemophilia Joint Health Score (HJHS) between dysgraphic and non-dysgraphic boys, while surface electromyography (sEMG) revealed a prevalence of flexor muscles of the upper limb in dysgraphic as compared with non-dysgraphic boys. CONCLUSIONS: The rather high prevalence of instrumental dysgraphia found in this pilot study deserves a further development of this preliminary experience by increasing the number of examined patients and comparing them with a control group, including quality of life and psychological assessment.

A Systematic Review on the Value of Infrared Thermography in the Early Detection of Periprosthetic Joint Infections. / Infrarotthermografische Untersuchungen nach endoprothetischem Gelenkersatz ­ ein systematisches Review.

BACKGROUND: Variations in the temperature of body and skin are symptoms of many pathological changes. Although joint replacement surgery of hip and knee has been very successful in recent decades, periprosthetic infection is a growing problem and the number one reason for revision. While many studies have investigated changes in blood levels, investigation of temperature has not been performed on a regular basis. The objective of this work is to determine whether reference literature exists for the infrared thermographic examination in knee and hip arthroplasty and if reference values can be derived for the methodology or if there is a peri- and postoperative benefit. MATERIAL UND METHODS: By means of a systematic online database search and based on the Cochrane, PICOT and PRISMA guidelines, this systematic review retrieved 254 studies. All publications with thermographic examination in arthroplasty of the hip and knee were imbedded. 249 studies were excluded due to the defined inclusion and exclusion criteria and five studies with 251 patients have finally been included in the evaluation process. This was followed by an analysis and discussion of the methodology. RESULTS AND CONCLUSION: Infrared thermography is a useful tool in the perioperative care of patients after arthroplasty of the knee and hip joint. The technology is portable, easy to use and non-invasive. Based only on these few publications, values can be derived, which provide a guidance for the thermographic aftercare in arthroplasty surgery.

Physical activity improved by adherence to prophylaxis in an Italian population of children, adolescents and adults with severe haemophilia A: the SHAPE Study.

BACKGROUND: Physical activity in people with haemophilia (PWH) reduces the development of severe arthropathy, but it must be performed after regular, proper prophylaxis. Strict adherence to treatment is crucial to achieving effectiveness and established outcomes. The primary aim of this study was to collect prospective data on adherence to prophylaxis for over 36 months. A secondary aim was to verify whether adherence correlates with physical activity. MATERIALS AND METHODS: Italian patients with severe haemophilia A treated on prophylaxis with octocog alfa were included in the study. Physical findings were assessed by the Haemophilia and Exercise Project (HEP)-Test-Q and the Early Prophylaxis Immunologic Challenge (EPIC)-Norfolk Physical Activity Questionnaire; orthopaedic status was assessed by the Hemophilia Joint Health Score (HJHS). Adherence was measured as percentage of empty vials returned with respect to the prescribed amount. RESULTS: Forty-two PWH were enrolled: 31% children, 21.4% adolescents, and 47.6% adults. Type, frequency and impact of physical activities differed among the three groups. The HEP-Test-Q showed the highest impairments in the domains "endurance" and "strength/co-ordination". Eight percent of patients were classified as adherent to prophylaxis. Among them, 50% had at least one bleeding episode in the year before enrolment; this percentage dropped during the three years of the study. While remaining stable in the "non-adherent" group, the HJHS score decreased in the "adherent" patients. The mean number of school/work days lost was lower in adherent patients (from 3.4±6.8 to 0.2±0.9) than in non-adherent ones. DISCUSSION: PWH with better orthopaedic scores reported better physical performance. Adherence to long-term prophylaxis proved to be high and correlated with a reduction in bleeds, target joints, school/work days lost, and with a performance improvement in endurance sports activities over time.

Establishing an online physical exercise program for people with hemophilia.

BACKGROUND: Hemophilia is a congenital bleeding disorder with an estimated frequency of 1:10,000 births. Repeated joint bleeding is a hallmark of the disorder and leads to painful hemophilic arthropathy. Regular exercise can help improve joint stability and function, reduce the risk of injury and bleeding and improve physical fitness and quality of life. This method paper describes an online training concept aiming to offer access to appropriate exercise instructions for people with hemophilia who are not able to attend regular training at a hemophilia center. METHODS: The online exercise program is accessible through the homepage of the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University Vienna as well as through scanning a QR code printed on information material using a smart phone or tablet. RESULTS: The program contains exercises to improve mobility, coordination, muscular strength and flexibility. A brief introduction is given by a hematologist, a pediatrician and a physiatrist. An introductory video informs about contraindications and essential precautions, such as medical attendance and sufficient factor therapy to consider before starting the training. Another video gives advice on the exercise composition. The demonstrated exercises are explained by a physician and are available for adults and children. To individualize training recommendations and offer further diagnostic tools and physical treatment options as necessary, the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University of Vienna will establish consultation hours for people with hemophilia. CONCLUSION: As hemophilia is an orphan disease, patients are mainly treated in specialized centers. For patients who live far from these centers or have limited access to a training there for other reasons, the physical medicine consultation hour and the implementation of online exercise instructions offer individually adapted exercise information for a regular home-based training to benefit from increased physical fitness and joint stability.

Evaluation of early musculoskeletal disease in patients with haemophilia: results from an expert consensus.

: Early joint damage in patients with haemarthrosis often escapes diagnosis because of insufficient investigation of biomechanical changes. Arthropathy in haemophilia requires complex assessment with several tools. Considering the increased emphasis on an integrated approach to musculoskeletal (MSK) outcomes, re-evaluation of MSK assessment to address individual patient needs is warranted. To advise on the optimal use of current assessment tools and strategies for tailored MSK evaluation in patients with haemophilia. A panel of experts in haemophilic arthropathy evaluated internationally recognized assessment tools through published literature and personal expertise. Each tool was considered, scored and ranked for their utility in the clinical assessment of MSK damage. Subsequently, a patient evaluation table detailing advice on type and frequency of assessments for different patient populations was constructed. To obtain a complete MSK assessment, multiple tools must be used to ensure each criterion is evaluated. For patients with haemophilia, clinical examination of the joint, disease-specific structure/function scores, and activity/participation scores including quality of life are important, and should be performed on a regular basis according to age and clinical condition. Joint imaging is recommended in the prevention, diagnosis and follow-up of haemophilic arthropathy and should be used in conjunction with joint structure and function scores. An integrated approach to MSK assessment using combinations of tools will allow earlier management of dysfunction and may improve long-term outcomes. This approach could be used in long-term follow-up of all patients independent of age and disease stage, especially in children to prevent arthropathy.

Infrared Thermography as a Non-Invasive Tool to Explore Differences in the Musculoskeletal System of Children with Hemophilia Compared to an Age-Matched Healthy Group.

Recurrent joint bleeds and silent bleeds are the most common clinical feature in patients with hemophilia. Every bleed causes an immediate inflammatory response and is the leading cause of chronic crippling arthropathy. With the help of infrared thermography we wanted to detect early differences between a group of clinical non-symptomatic children with hemophilia (CWH) with no history of clinically detected joint bleeds and a healthy age-matched group of children. This could help to discover early inflammation and help implement early treatment and preventative strategies. It could be demonstrated that infrared thermography is sensitive enough to detect more signs of early inflammatory response in the CWH than in healthy children. It seems to detect more side differences in temperature than clinical examination of silent symptoms detects tender points. Silent symptoms/tender points seem to be combined with early local inflammation. Using such a non-invasive and sensor-based early detection, prevention of overloading and bleeding might be achieved.

Early orthopaedic challenges in haemophilia patients and therapeutic approach.

INTRODUCTION: There is growing evidence that subclinical joint bleeding early in life may lead to subtle changes in joint function, gait, and muscle development in young children with haemophilia. The early identification of such changes provides an opportunity for clinicians to intervene before irreversible structural changes occur. MATERIALS AND METHODS: We have undertaken motion analysis of the knee in 273 children (mean age 9.8 years) with haemophilia A, haemophilia B, or von Willebrand disease and compared the results with those from 200 healthy age- and sex-matched controls (mean age 10.3 years). We have also completed detailed orthopaedic assessments and evaluated overall fitness in most of these children. RESULTS: There was a clear correlation between total motion score and age: r(2)=0.47 for the healthy children and r(2)=0.69 for the haemophilic children. The differences were most striking in children aged 3-4 years. Significant between-group differences were also observed in the comparison of knee bends, gait scores, and rhythmicity. Orthopaedic examinations revealed previously clinically silent pressure pains in the ligaments of the knee joints of 38% of children and in the ligaments and capsule of the ankle joints of 60% of children. The five-item fitness check showed significant deficits relative to controls in overall fitness, endurance, coordination, and flexibility. CONCLUSIONS: Children and adolescents with haemophilia show significant functional impairments relative to normal controls. Early comprehensive assessments of the musculoskeletal system should be made so that individualized physical, physiotherapy, and sports therapy programmes can be developed.

Orthopaedic issues in sports for persons with haemophilia.

Persons with haemophilia should not feel limited in their ability to participate in sports. After consultation with a physician or other health-care provider and with a proper understanding of the risks involved and the strategies for managing these risks, patients with haemophilia can - and should - enjoy physical activities. We have analysed numerous reports of sports injury statistics and used them to rank a variety of sports according to their degree of injury risk. In addition, we have developed a brief orthopaedic examination and a five-item fitness check that evaluates the level of physical fitness of patients with haemophilia. Using these tools, we can appropriately recommend specific sports activities best suited for each patient. In addition, we recommend that patients who regularly participate in sports maintain adequate levels of clotting factor through the use of regular prophylaxis. With proper physical evaluation and preparation, patients with haemophilia can realize the physical and emotional benefits of participation in sports.