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Experienced peer support groups (EPSG) are expected to improve self-care and complement professional health care for haemophilic patients, even those living in inconvenient clinical setting. However, these benefits have not been verified quantitatively. The structural equation modelling (SEM) was used to evaluate the effects of contact with EPSG on self-care for haemophilic patients in the Japanese clinical settings. Factors affecting self-care were compared between groups with and without EPSG contact. Self-reported questionnaires were mailed to 652 haemophilic patients with HIV in Japan (September 2005-January 2006). SEM demonstrated significant associations between EPSG contact, self-care scores and other social and individual factors. The total effect of EPSG contact on self-care was calculated. The structural differences between models were analysed in a multi-group analysis. Of the 257 respondents (response rate, 39.4%), 109 reported having contact with an EPSG (EPSG+ group) and 139 reported no contact (EPSG- group). EPSG contact was significantly associated with better self-care. In the multi-group analysis, the total effect of inconvenient access to medical services on self-care in the EPSG+ group was 10% of that in the EPSG- group and was significantly associated with poor illness-related knowledge and high anxiety level only in the EPSG- group. In the EPSG+ group, patient age was strongly associated with self-care than in the EPSG- group. These findings suggest that EPSG contact may alleviate inconvenience in medical services. Factors associated with self-care differed between groups. Health care professionals must carefully assess self-care behaviours and service accessibility based on these results., (© 2013 John Wiley & Sons Ltd.)

Haemophilic arthropathy causes pain and a severely restricted range of motion, and results in a significant reduction in quality of life. When conservative treatments have failed, orthopaedic surgery is recommended for these patients with severe haemophilic arthropathy. However, surgery for haemophilia patients is challenging due to high complication rate such as infection, delayed wound healing and mortality. The aim of this study was to evaluate the incidence of early complications and identify preoperative risk factors of surgery for haemophilia patients. We report a series of haemophilia patients undergoing elective orthopaedic surgery between 2006 and 2012. During this period, 119 surgeries in 81 patients were prepared and 118 surgeries in 80 patients were actually performed. Four deep bacterial infections and four delayed wound healings occurred within 6 months postoperatively. One patient died preoperatively and four patients died postoperatively. Only the presence of inhibitor was a significant risk factor for infection. We found no risk factor related to delayed wound healing. Our data revealed alkaline phosphatase, albumin, platelet, alpha-fetoprotein, presence of ascites and child classification C as predictors of perioperative mortality following elective orthopaedic surgery. Our role is to identify potential patients who present with risk factors for complications and attempt to seek the best determination of treatment strategy for these people., (© 2013 John Wiley & Sons Ltd.)

Few data are available on the clinical course of Japanese patients deficient in natural anticoagulants (antithrombin (AT), protein C, and protein S). We conducted a nationwide survey to reveal the clinical course of these patients. Questionnaires were sent to 321 council members of the Japanese Society on Thrombosis and Hemostasis, Japanese Society for Vascular Surgery, and Japanese Society of Phlebology. A total of 103 responses were obtained and data of 183 patients were collected. Of 183 patients, 142 (78%) experienced at least one episode of venous thromboembolism (VTE). The first VTE occurred before the age of 40 years in 71 patients (45%). Venous thromboembolism recurred in 15 (39%) patients with AT deficiency and 19 (18%) patients with other deficiencies. These findings suggest that half of the first episodes of VTE in patients deficient in natural anticoagulants occur before middle age and the risk of VTE recurrence is high in patients with AT deficiency.