Joint morbidity in haemophilia has traditionally been measured using clinical and radiological scores. There have been no reliable, validated tools for the assessment of functional independence in persons with haemophilia till recently. The Functional Independence Score in Haemophilia (FISH) has been developed as a performance based assessment tool to address this need. The FISH is designed to measure the patient's independence in performing activities of daily living (grooming and eating, bathing and dressing), transfers (chair and floor), and mobility (walking, step climbing and running). On assessment of its psychometric properties in 63 patients with haemophilia (mean age 14 years), FISH was found to have good internal consistency (Cronbach's alpha of 0.85). It had moderate correlation with the World Federation of Hemophilia clinical score (r = -0.61), and a correlation with the Pettersson score of -0.38. It had good correlation with other self-rated functional scores, such as the Stanford Health Assessment Questionnaire (r = -0.75); the Western Ontario and McMaster Universities Osteoarthritis Index (r = -0.66) and the Haemophilia Activities List (HAL) (r = -0.66). It had good reliability with a pooled intra class correlation of 0.98. On assessing responsiveness following treatment of flexion deformities of the knee in 12 patients, the FISH showed significant changes in the score with a standardized responsiveness mean of -1.93. In conclusion, the FISH was found to be a reliable and valid tool with good internal consistency and responsiveness to therapy, for the assessment of functional independence in persons with haemophilia.

Three patients referred for MRI of the foot were found to have imaging features characteristic of mycetoma. Two patients presented with recurrent soft tissue masses, which were operated on several times and not suspected to be of infective aetiology. The third patient had typical clinical features with a history of blackish granule discharge. In all three patients, MRI showed conglomerate areas of small round discrete T(2) weighted hyperintense lesions, representing granulation tissue surrounded by a low-signal-intensity rim representing intervening fibrous septa. Within many of these hyperintense lesions, there was a central low-signal-intensity dot, which gives rise to the "dot-in-circle" sign that has been very rarely described in the literature. This sign is an easily recognisable and unique appearance that is highly suggestive of mycetoma.

The basis for 10-15% of patients with severe haemophilia having clinically mild disease is not fully understood. We hypothesized that polymorphisms in various coagulant factors may affect frequency of bleeding while functionally significant polymorphisms in inflammatory and immunoregulatory genes may also contribute to variations in the extent of joint damage. These variables were studied in patients with severe haemophilia, who were categorized as 'mild' (<5 bleeds in the preceding year, <10 World Federation of Haemophilia clinical and <10 Pettersson scores, n = 14) or 'severe' (all others, n = 100). A total of 53 parameters were studied in each individual for their association with the clinical severity. Age, F8:c activity and the incidence of thrombotic markers were comparable between the groups while the median number of bleeds, number of affected joints, clinical, radiological and functional joint scores (P < or = 0.001) and life-time clotting factor use (P < or = 0.007) were different. Patients with severe molecular defects had a 4.1-fold increased risk for a severe phenotype (95% CI: 1.18-14.42, P = 0.026) compared with other mutations. Of the polymorphisms studied, the FVII353Q (RR = 3.5, 95% CI: 1.04-12.05, P = 0.044) allele was associated with a severe phenotype. This data shows that apart from the F8/F9 genotype, functional polymorphisms in FVII gene affect the phenotype of patients with severe haemophilia.

ABSTRACT. We report a rare case of dual arterial supply to an otherwise normal left lower lobe. In addition to normal pulmonary arterial supply, the lower lobe of the left lung also received systemic arterial supply from the coeliac axis. The relevant anatomy and literature are reviewed. To the best of our knowledge, there are no other reported cases of anomalous systemic arterial supply from the coeliac axis to the basal segments of the left lower lobe with normal bronchial branching and pulmonary arterial supply. Systemic arterial supply to the lungs can be congenital or acquired. In the commonly reported congenital form of systemic arterial supply to the basal segments of the left lower lobe, there is abrupt tapering of the left lower lobar pulmonary artery distal to the origin of the superior segmental artery and the aberrant systemic artery originates from the descending thoracic aorta [1,2]. We report a rare case of dual arterial supply from the pulmonary and systemic arteries to the basal segments of the left lower lobe with a normal bronchial tree. Case reportA 47-year-old woman presented with history of occasional blood-streaked sputum for the past 9 years. There was no history of excessive sputum production or past history of tuberculosis. Clinical examination was unremarkable, as were routine laboratory investigations.The chest radiograph showed a few linear opacities in the left retrocardiac region (Figure 1). Bronchoscopy was normal. High-resolution computed tomography (HRCT) of the thorax was carried out to look for underlying bronchiectasis as the cause of haemoptysis. HRCT revealed a few asymmetrical dilated vessels in the left lower lobe; there was minimal adjacent ground-glass opacity and few linear opacities, suggesting postinflammatory changes (Figure 2). In view of the presence of dilated vessels, a vascular malformation was suspected and CT angiography was performed. The CT angiogram showed aberrant systemic arterial supply to the basal segments of the left lower lobe; this arterial supply arose from the coeliac axis. This anomalous artery entered the thorax via the oesophageal hiatus, traversed parallel and to the left of the oesophagus and entered the left lower lobe via the pulmonary ligament, supplying the basal segments. There was no arteriovenous malformation. Pulmonary arterial supply to the left lower lobe was also present with normal arborisation. Although the descending pulmonary artery appeared slightly smaller than on the right side, all the basal segmental branches were identified. The normal pattern of venous drainage into the inferior pulmonary vein was seen. The lung parenchyma showed mild linear and ground-glass opacity with normal branching of the bronchial tree (Figures 3, 4, 5, 6, 7 and 8). The patient was not keen on surgery or endovascular treatment and is on follow-up.

Computed tomography (CT) of the abdomen contributes a substantial amount of man-made radiation dose to patients and use of this modality is on the increase. This study intends to compare radiation dose and image quality using dose modulation techniques and weight- based protocol exposure parameters for biphasic abdominal CT. Using a six-slice CT scanner, a prospective study of 426 patients who underwent abdominal CT examinations was performed. Constant tube potentials of 90 kV and 120 kV were used for all arterial and portal venous phase respectively. The tube current-time product for weight-based protocol was optimized according to patient's body weight; this was automatically selected in dose modulations. The effective dose using weight-based protocol, angular and z-axis dose modulation was 11.3 mSv, 9.5 mSv and 8.2 mSv respectively for the patient's body weight ranging from 40 to 60 kg. For patients of body weights ranging 60 to 80 kg, the effective doses were 13.2 mSv, 11.2 mSv and 10.6 mSv respectively. The use of dose modulation technique resulted in a reduction of 16 to 28% in radiation dose with acceptable diagnostic accuracy in comparison to the use of weight-based protocol settings.