Characterization of hypertrophic cardiomyopathy according to global,regional, and multi-layer longitudinal strain analysis,and prediction of sudden cardiac death

To evaluate the severity of hypertrophic cardiomyopathy (HCM) according to global, regional, and multi-layer longitudinal strain (LS) analysis using speckle-tracking echocardiography. From February 2007 to November 2014, we prospectively evaluated 375 consecutive HCM patients referred to our specialized cardiomyopathy center. Demographics, clinical, and rest and exercise echocardiographic parameters were collected according to a completely standardized protocol. Global, regional, and multilayer strain analyses were performed. Correlations between LS and other characteristics were evaluated, and we assessed their prognostic value to predict sudden cardiac death (SCD) or appropriate implantable cardioverter defibrillator (ICD) shocks during follow-up, using Cox proportional hazards analyses. We finally included 217 patients (50.1?±?15.6 years, 67% male) but only 179 (82%) had LS analysis of sufficient quality. An inverse relation was observed between the mean basal left ventricular (LV) LS and diastolic parameters [E/Ea (r?=???0.30) and left atrium indexed volume (r?=???0.23)], as well as between the resting LV outflow-tract maximal gradient (r?=???0.26) or during peak exercise (r?=???0.20). Mean LS in the LV hypertrophic area was particularly related with maximal wall thickness (r?=???0.47) and transmural global LS with the degree of myocardial fibrosis in cardiac magnetic resonance (r?=???0.32). During a median follow-up of 2.8?±?1.5 years, mean transmural LS in the hypertrophic area was predictor of SCD and appropriate ICD shock (10 events/179 patients, hazard ratio?=?0.83 [95% CI 0.72–0.95], p?=?0.01). Basal LS and hypertrophic area LS are valuable parameters to evaluate HCM severity. Mean hypertrophic area LS particularly seems predictive of SCD occurrence and appropriate ICD shocks.     

Neonatal haemoglobinopathy screening: review of a 10-year programme in Brussels

Since 1994, a neonatal screening programme for major haemoglobinopathies has been conducted in Brussels. We performed a 10-year re-evaluation of the incidence of haemoglobinopathies in Brussels and found that of the 118,366 newborns screened, 64 were diagnosed with a sickle cell syndrome, six had beta-thalassaemia major, four had a haemoglobin C disease and three had a haemoglobin H disease. Of the 64 babies with a sickle cell disease, two died before the age of two years and two did not present at the first neonatal visit. Of the six babies suffering from beta-thalassaemia major, all are alive and two have undergone a haematopoietic stem cell transplantation. The universal neonatal screening programme for haemoglobinopathies should be maintained in Brussels.     

A 10-year follow-up study of pulmonary function in symptomatic elite cross-country skiers--athletes and bronchial dysfunctions

A high prevalence of asthma-like symptoms and abnormal bronchial reactivity to various stimuli has been reported among endurance athletes. This report presents the cases of three cross-country skiers who have shown a progressive development of airway obstruction during their sport careers. The observed decline in FEV1, FEV1/FVC and/or FVC (FEV, forced expiratory volume; FVC, forced expiratory vital capacity) was greater than expected from longitudinal regression equations describing the ageing effect on pulmonary function. The three subjects encountered abnormal respiratory discomfort during their sport activity which was not systematically associated with abnormal bronchial reactivity (either to methacholine or exercise hyperventilation). At the end of the follow-up periods (9-12 years), all three skiers presented objective signs of airflow limitations during intense exercise. This report suggests that repeated chronic hyperventilation of cold dry air in cross-country skiers for several years can induce permanent bronchial disorders which may include remodeling processes and induce ventilatory limitations during intense exercise.     

Recording injuries only during winter competitive season underestimates injury incidence in elite alpine skiers

Current epidemiological studies in elite alpine skiers are mostly limited to retrospective surveys on in-season injury. The aim of this study was to determine the risk and pattern of injury in elite alpine skiers during the winter competitive season (WCS) and the summer off-season (SOS). European Cup skiers were prospectively followed during 5 complete years. A total of 133 skier-seasons (79 males and 54 females) completed the study. All acute and overuse injuries that required medical attention were registered, representing a total of 166 injuries. Absolute injury incidence was 124.8 (95% CI [106-145]) injuries per 100 athletes per complete season and was higher during the WCS compared with the SOS, albeit in a smaller magnitude than expected (relative risk ratio (RR) 1.44 [1.06-1.96]). The absolute incidence of severe injuries (ie, time-loss >28 days) was twofold higher during WCS compared with SOS (RR 2.19 [1.21-3.95]). Most common injuries during the 2013-2015 seasons were knee followed by back (absolute incidences 56 [38-80] and 20 [10-36], respectively; RR 2.82 [1.42-5.61]), but this difference disappeared after 2015 (absolute incidences 25.6 [15.7-39.6] and 23.1 [13.7-36.5], respectively; RR 1.11 [0.59-2.10]). In summary, we found that 98 (59%) injuries occurred during WCS and 68 (41%) during SOS over 5 years. These results demonstrate the necessity to record injuries during the entire year to avoid a significant underestimation of the injury incidence. In addition, the current data showed an evolution of injury pattern over time and since previous reports, with back injuries being the main concern along knee joint injuries.     

Characterisation of a non-pathogenic and non-protective infectious rabbit lagovirus related to RHDV

The existence of non-pathogenic RHDV strains was established when a non-lethal virus named rabbit calicivirus (RCV) was characterised in 1996 in Italy. Since then, different RNA sequences related to RHDV have been detected in apparently healthy domestic and wild rabbits, and recently a new lagovirus was identified in Australia. We have characterised from seropositive healthy domestic rabbits a non-lethal lagovirus that differs from RHDV in terms of pathogenicity, tissue tropism and capsid protein sequence. Phylogenetic analyses have revealed that it is close to the Ashington strain and to the RCV, but distinct. We proved experimentally that it is infectious but non-pathogenic and demonstrated that, contrary to the other described non-pathogenic lagoviruses, it induces antibodies that do not protect against RHDV. Our results indicate the existence of a gradient of cross-protection between circulating strains, from non-protective, partially protective to protective strains, and highlight the extent of diversity within the genus Lagovirus.