Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database of Systematic Reviews, (1), [ARTN CD002011.pub2].

Joint damage from bleeding episodes leads to physical or functional limitations in people with haemophilia. Various factors may influence the frequency and severity of joint damage. This study examined whether age, prophylaxis, history of high-titre inhibitors (HTI) and bleeding events influenced the Haemophilia Joint Health Score (HJHS) in children. Medical and physiotherapy notes of boys with severe haemophilia, aged 4-18 years, were reviewed to identify factors associated with increased HJHS. The HJHS of 83 boys (median age: 11) ranged from 0 to 25, with 44/83 (53%) having a score of zero. The median HJHS was 0 (mean 2.6). In the non-HTI group, the HJHS for boys on late prophylaxis was 2.68 times higher than those who started early and the HJHS was on average 10% higher for every additional recent bleed. In this group the odds of having a zero score fell by 30% for every year increase in age. Boys with a history of HTI had higher HJHS scores than the non-HTI group, and age, number of recent bleeds and tolerized status were positively associated with HJHS. The score rose on average by 28% for every year of age and by 76% for non-tolerized boys. This study provides further evidence supporting early prophylaxis use and the importance of immune tolerance therapy. The HJHS is a useful tool for identifying and tracking changes in joint health with respect to therapy or disease progression. With improvements in haemophilia treatment, the disproportionate number of zero scores will continue to make interpretation of the HJHS challenging.

Development of haemophilic arthropathy has long-term implications for functional mobility in people with haemophilia, but early manifestations are often asymptomatic and difficult to identify. Earlier identification of joint damage may improve outcomes. The aim of this case note review was to determine whether the GAITRite system (electronic pressure sensitive walkway) could identify early changes in gait patterns in boys with haemophilia compared with their peers. Clinic data from medical and physiotherapy notes of boys with severe haemophilia were compared with data from age and leg length-matched controls. Data from two consecutive walks at preferred speed were collected on all participants using the GAITRite system. Clinic assessment notes from 26 boys (aged 7-17 years) with severe haemophilia were identified. Of these, 20 boys had no evidence of joint pathology on assessment and six boys had radiographic evidence of arthropathy. When these data were compared with normal controls, there were statistically significant increases in swing time, stance time, single support and double support in the asymptomatic group (P < 0.01) suggesting subtle early compensatory changes in gait pattern. The children with arthropathy had additional significant differences in their gait compared with matched controls. These differences included normalized velocity, step length, stride length, step time and base of support (P < 0.01). The GAITRite system appears sensitive enough to identify early subtle changes in gait and differentiate between asymptomatic boys with haemophilia and those with arthropathy in comparison with a matched control group. The electronic walkway is an accessible and portable means of providing quantitative gait analysis in the clinical environment. This is an important finding as early identification of gait changes may provide clinicians with the opportunity to intervene with the aim of arresting progression of joint damage.

Leaks larger than 20% result in inconsistent tidal volume delivery and gross overestimation of C(rs) and R(rs) irrespective of ventilation mode. The magnitude of tracheal tube leak needs to be accurately displayed on all ventilatory equipment to verify reliable measures of respiratory function so that appropriate clinical decisions can be made and ventilatory management optimised.