Surgical interventions in patients suffering from haemophilia A require infusions of large doses of factor VIII (F VIII) concentrates. These are expensive and may pose a burden on the immune system, which is already compromised in many haemophiliacs. F VIII is usually given as bolus injections, although there are reports on fixed dose continuous infusion. We have developed a continuous infusion programme with dosage adjusted according to daily calculation of the clearance of F VIII. Twenty-four haemophiliacs received a total of 205 d of continuous infusion with F VIII (168 d in hospital, 37 d home therapy). Eighteen patients underwent surgeries (11 elective, seven emergency) and six were treated for serious haemorrhages. We observed a significant, progressive decrease of the clearance of F VIII over the first 5 d from 3.2 (2.8-3.5) to 1.7 (1.3-1.9) ml/kg/h (median and interquartile range). The decrease of the clearance together with the fact that a continuous infusion requires less drug than bolus injections to keep the same minimal concentration, contributed to much lower doses of F VIII than reported in the literature or than required by historical controls, matched for the type of operation. Other advantages of our method are improved safety with stable activities of F VIII, lack of hazardous troughs below the haemostatic range, and the convenience, which permits ambulant therapy even when high doses of F VIII are required.

Dancers and teachers should realize that passive joint range of motion is unlikely to improve with age. Therefore, the major goal of a dancing program should focus on exercises that retain the natural flexibility of the dancers' joints rather than trying to improve them.

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International audienceThe transfer functions and penetrations of five differential mobility analyzers (DMAs) for sub-2 nm particle classification were evaluated in this study. These DMAs include the TSI nanoDMA, the Caltech radial DMA (RDMA) and nanoRDMA, the Grimm nanoDMA, and the Karlsruhe-Vienna DMA. Measurements were done using tetra-alkyl ammonium ion standards with mobility diameters of 1.16, 1.47, and 1.70 nm. These monomobile ions were generated by electrospray followed by high resolution mobility classification. Measurements were focused at an aerosol-to-sheath flow ratio of 0.1. A data inversion routine was developed to obtain the true transfer function for each test DMA, and these measured transfer functions were compared with theory. DMA penetration efficiencies were also measured. An approximate model for diffusional deposition, based on the modified Gormley and Kennedy equation using an effective length, is given for each test DMA. These results quantitatively characterize the performance of the test DMAs in classifying sub-2 nm particles and can be readily used for DMA data inversion

Sonographic examination of the knee has been proposed by several authors in the past as a simple and reliable method to diagnose Osgood-Schlatter disease (OSD). Ultrasound was used to compare the knees of 25 boys and 10 girls with typical OSD with 35 symptom-free knees of an aged-matched group of children. Based on recorded data, patients were categorized (one affected knee in each individual) according to the classification system proposed by De Flaviis et al. in 1989. The results included the following pathological findings: pretibial swelling, fragmentation of the ossification center, insertional thickening of the patellar tendon, and excessive fluid collection in the infrapatellar bursa. Of our patients, 26% fell into the type 1 category, 43% were type 2, 20% type 3, and 11% type 4. This distribution of cases was found to be statistically similar to the initial findings reported by De Flaviis and colleagues. This study therefore supports the validity and reproducibility of their classification method for the ultrasonographic evaluation of children with OSD. This is only the first step, and further assessment of this classification is still required to elucidate its clinical as well as its prognostic value.

The benefits and feasibility of progressive resistance training on muscle strength and bleeding profile were studied prospectively in two patients with severe haemophilia. Additionally, retrospective data were collected from three patients who had been training for 11-21 years (one patient for 21 years and two patients for 11 years). Muscle strength increased, especially in muscle groups surrounding the target joints (elbow and knee). Bleeding frequency decreased from 2-3 times per week to 1-2 times per week. Patients who had been training for > 11 years reported bleeding episodes of 2-4 times per month prior to training, but after > 11 years of progressive training a marked decrease in bleeding occurred, as well as a decrease in severity. These data support the importance of resistance training for haemophilia patients, not only for increasing muscle strength, but also for decreasing the frequency and severity of bleeding episodes and the associated pain. A controlled study, with a greater number of patients, is needed to confirm the suggested benefits of resistance training in haemophilia patients.

No significant affect of the amputation level except for trans-wrist amputation with 100% prosthesis use. No significant difference was found between prosthesis type and the correlation to stump problems. The above-elbow amputees, with dominant hand amputation, who used functional prosthesis (body-powered), achieved the best functional outcome and result.

Patients with haemophilia are now widely advised to participate in sport activities. However, no extensive data are available about their actual participation. The aim of this study was to describe the type; intensity and duration of leisure time physical activity (PA) among young patients with severe hemophilia and to assess whether there are differences in bleeding profile and muscle strength in related to activity intensity. Forty-four boys (ages 12-25 years) with severe haemophilia were studied. PA was assessed by the Godin and Shephard (G&S) questionnaire. Bleeding profile was determined based on a one month diary filled by each patient. Muscle strength of the lower limbs muscles was assessed using a hand held dynamometer. Only three subjects did not perform any PA. Twenty-five of the participants performed strenuous PA at least once a week. An inverse, moderate association (r(p) =-0.45, P < 0.002) was found between the G&S score and age. There were no significant differences in bleeding frequency or pain but a significant difference in the cause of bleed was found: those who exercised strenuously showed a higher proportion of bleeds due to traumatic reasons (P < 0.01). No differences in muscle strength values were noted in related to activity intensity also no linear association was noted between muscle strength and bleeding profile. Further investigation, including prospective studies, is needed in order to assess the temporal sequencing between training and the occurrence of bleeds and bleeds cause.