The majority of individuals in the UK with severe haemophilia treat themselves at home with clotting factor. The aim of this study was to examine patient's beliefs about their haemophilia, their beliefs regarding treatment with clotting factor and to assess whether relationships existed between these factors and adherence to home treatment. Patient beliefs were assessed by posting individuals with severe haemophilia A and B (n ¼ 104) the Illness Perceptions Questionnaire (IPQ) and the Beliefs about Medicines Questionnaire (BMQ). The final analysis was based on 65 questionnaires yielding a response rate of 63%. Adherence to the frequency and to the amount of each clotting factor dose was assessed for individuals treating prophylactically and the amount of each clotting factor dose for those only treating 'on-demand'. Adherence scores were calculated by comparing individual patient treatment records with recommended regimens. Regression analysis indicated that perceptions of illness identity and treatment necessity explained a significant amount of the variance in adherence to frequency of prophylactic infusions and that illness identity was associated with the adherence to the amount of each prophylactic dose. Further regression analysis also showed that the amount of each dose of clotting factor administered on-demand was associated with perceptions regarding the necessity of treatment. Analysis with a combined measure of adherence to dose amount indicated that patients treating on-demand or with prophylaxis who under treated, recorded significantly lower perceptions of illness consequence than individuals who were fully adherent or who over-treated. The results suggest that assessing illness perceptions, especially beliefs regarding identity, consequence and necessity of treatment, may play a valuable role in identifying which individuals are least likely to adhere to treatment.
Fifty adults aged 35.12±14.7 with mild (n = 12), moderate (n = 10), or severe (n = 28) haemophilia A (70%) or B (30%) from four haemophilia centres across the United Kingdom participated in the study. A total of 64% were overweight/obese according to their BMI; median orthopaedic joint scores using the WFH Orthopaedic Joint Score (OJS) were 6 (range 0-48). On a VAS pain scale (range of 0-10), patients reported mean score of 5.66 ± 2.4. 36% of participants reported not doing any sport, mainly due to their physical condition. However, 64% of participants reported undertaking sporting activity including contact sports, mostly twice per week in average 4 h week(-1) . Participating in sport did not have a statistically significant impact on HRQoL; except in the domain 'sport and leisure' of the Haem-A-QoL. Patients doing more sport reported significantly better HRQoL than those doing less sport (P < 0.005). Those doing sport for more than 4 h week(-1) had a significantly better physical performance than patients doing less sport (assessed with Hep-Test-Q). Encouraging physical activity and sport in older patients with haemophilia may have a direct impact on their HRQoL; thus, education about sport activity should be incorporated into routine haemophilia care.
Aggressive osteoblastoma is a rare primary bone neoplasm with the potential for local invasion and recurrence. While the vertebrae or long bones are most commonly affected, few well-documented cases have been reported in the jaws. A 25-year-old man presented with a palatal mass of several months' duration. He reported the lesion had undergone gradual enlargement and, while generally asymptomatic, had recently become increasingly painful. An incisional biopsy was interpreted as "osteoblastic neoplasm" most suggestive of osteoblastoma. However, final diagnosis was deferred until the resection specimen could be evaluated. Following partial maxillectomy, histopathologic examination revealed a proliferation of large epithelioid cells with eccentric nuclei and prominent nucleoli associated with broad, irregular deposits of osteoid and trabeculae of bone. The lesional cells exhibited minimal pleomorphism with infrequent, normal-appearing mitotic figures and numerous osteoclast-like giant cells were observed within an associated loose fibrovascular stroma. Transformation of "blue bone" to more organized eosinophilic trabeculae of woven bone was noted at the periphery of the lesion and there was no evidence of invasion. A diagnosis of aggressive osteoblastoma was made. Previous reports of gnathic aggressive osteoblastoma are reviewed and the features that distinguish this process from conventional osteoblastoma or osteoblastoma-like osteosarcoma are presented.
Although the syllabus is one of the most widely used documents in higher education, research investigating the effectiveness of this tool is minimal. The current study investigates student perception of the course and professor based on the syllabus. A total of 149 students were randomly assigned to one of six syllabus conditions focusing on syllabus length (i.e., short, medium, and long) and the inclusion of images. Participants completed questionnaires and participated in a focus group regarding their perceptions of the course and the professor. Results revealed that students reviewing the medium or long syllabi, as compared to the short syllabus, had a more positive impression of the course and professor. No significant differences were found for images versus no images. The majority of students (i.e., 66.6%) indicated a preference for a long syllabus with all assignment details versus a shorter syllabus with assignment details being provided later in the semester. The implications of these findings are discussed.
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