Atteinte osseuse de la maladie de Fabry

La maladie de Fabry (MF) est une pathologie héréditairedu métabolisme des glycosphingolipides resultantdu déficit en α-galactosidase A, une enzyme lysosomale. Le déficit enzymatique conduit à l’accumulationde glycosphingolipides dans le plasma et les lysosomes de nombreux types cellulaires. Bien que la maladie de Fabry soit un modèle de maladiemulti-systémique, aucune donnée n’est disponible concernant l’atteinte osseuse. Dans ce travail, une ostéodensitométrie fut mesurée en deux sites, lacolonne vertébrale lombaire et la tête fémorale chez 23 hommes hémizygotes, âgés en moyenne de 31 ans et atteints de la forme classique de la maladie de Fabry. Selon la classification OMS de la perte de densité minérale osseuse, 20 des 23 patients (88 %) présentaient soit une ostéopénie (n = 11) soit uneostéoporose (n = 9) à l’un et/ou l’autre site

Postoperative meniscus: assessment at dual-detector row spiral CT arthrography of the knee.

PURPOSE: To assess the effectiveness of dual-detector row spiral computed tomographic (CT) arthrography of the knee in the evaluation of the postoperative meniscus for recurrent or residual meniscal tear. MATERIALS AND METHODS: Spiral CT arthrography was performed in 20 patients who presented with pain after partial meniscectomy. Findings at the initial reading of the images and at two retrospective independent readings were compared with those at second-look arthroscopy performed in all patients. At initial interpretation, conventional criteria for meniscal tear were used, including partial- or full-thickness tear of any size and meniscal separation. At retrospective interpretation, criteria for meniscal tear included large partial- and full-thickness tear and meniscal separation but not small partial-thickness tear. Sensitivity and specificity for the detection of tear of the postoperative menisci were calculated for initial and retrospective readings. RESULTS: At initial interpretation, the sensitivity and specificity for the detection of tear of the postoperative menisci were 100% and 78%, respectively. At retrospective interpretation, the sensitivity and specificity for the detection of tear of the postoperative menisci were 79% and 89% at reading 1 and 93% and 89% at reading 2, respectively. CONCLUSION: Spiral CT arthrography is valuable for the assessment of postoperative menisci, but the application of conventional definitions of meniscal tear to arthrographic findings in postoperative menisci can lead to overestimation of the clinical importance of meniscal lesions

On the Infectivity of Bacteriophages in Polyelectrolyte Multilayer Films: Inhibition or Preservation of Their Bacteriolytic Activity?

International audienc

Multifunctional polymeric implant coatings based on gelatin, hyaluronic acid derivative and chain length-controlled poly(arginine)

International audienc

The Asymmetric Profile of the Acetabulum

Despite the curvaceous profile of the acetabulum, orthopaedic surgeons have continued to implant hemispheric cups since the introduction of total hip arthroplasty. The geometric discrepancies between the natural acetabulum and implant can result in painful iliopsoas impingement attributable to prosthetic overlap at the anterior acetabular ridge over which the iliopsoas tendon extends to leave the pelvis. We expanded on previous in vitro observations of acetabular morphology using a large in vivo sample and quantified the dimensions of the psoas valley. We studied computed tomographic scans of 200 healthy hips from 50 men and 50 women. The acetabular ridges were digitized on three-dimensional bone reconstructions and their coordinates were manipulated in spreadsheets to deduce acetabular diameter, anteversion, and inclination and to plot the rim profile. Our results confirm the acetabular rim is an asymmetric succession of three peaks and three troughs. The psoas valley has the following shape distribution: 79% curved, 11% angular, 10% irregular, and 0% straight. The mean depth of the psoas valley is 5 mm and the latitude of its trough is on average 6 mm below the acetabular equator. The use of side-specific cups that replicate the curvaceous acetabular profile could prevent prosthetic overlap and reduce the incidence of iliopsoas impingement

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

International audienceBackgroundBrain MRI parenchymal signal abnormalities have been associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).PurposeTo describe the neuroimaging findings (excluding ischemic infarcts) in patients with severe coronavirus disease 2019 (COVID-19) infection.Materials and MethodsThis was a retrospective study of patients evaluated from March 23, 2020, to April 27, 2020, at 16 hospitals. Inclusion criteria were (a) positive nasopharyngeal or lower respiratory tract reverse transcriptase polymerase chain reaction assays, (b) severe COVID-19 infection defined as a requirement for hospitalization and oxygen therapy, (c) neurologic manifestations, and (d) abnormal brain MRI findings. Exclusion criteria were patients with missing or noncontributory data regarding brain MRI or brain MRI showing ischemic infarcts, cerebral venous thrombosis, or chronic lesions unrelated to the current event. Categorical data were compared using the Fisher exact test. Quantitative data were compared using the Student t test or Wilcoxon test. P < .05 represented a significant difference.ResultsThirty men (81%) and seven women (19%) met the inclusion criteria, with a mean age of 61 years ± 12 (standard deviation) (age range, 8–78 years). The most common neurologic manifestations were alteration of consciousness (27 of 37, 73%), abnormal wakefulness when sedation was stopped (15 of 37, 41%), confusion (12 of 37, 32%), and agitation (seven of 37, 19%). The most frequent MRI findings were signal abnormalities located in the medial temporal lobe in 16 of 37 patients (43%; 95% confidence interval [CI]: 27%, 59%), nonconfluent multifocal white matter hyperintense lesions seen with fluid-attenuated inversion recovery and diffusion-weighted sequences with variable enhancement, with associated hemorrhagic lesions in 11 of 37 patients (30%; 95% CI: 15%, 45%), and extensive and isolated white matter microhemorrhages in nine of 37 patients (24%; 95% CI: 10%, 38%). A majority of patients (20 of 37, 54%) had intracerebral hemorrhagic lesions with a more severe clinical presentation and a higher admission rate in intensive care units (20 of 20 patients [100%] vs 12 of 17 patients without hemorrhage [71%], P = .01) and development of the acute respiratory distress syndrome (20 of 20 patients [100%] vs 11 of 17 patients [65%], P = .005). Only one patient had SARS-CoV-2 RNA in the cerebrospinal fluid.ConclusionPatients with severe coronavirus disease 2019 and without ischemic infarcts had a wide range of neurologic manifestations that were associated with abnormal brain MRI scans. Eight distinctive neuroradiologic patterns were described

Cerebral perfusion using ASL in patients with COVID-19 and neurological manifestations: A retrospective multicenter observational study

Background and purpose: Cerebral hypoperfusion has been reported in patients with COVID-19 and neurological manifestations in small cohorts. We aimed to systematically assess changes in cerebral perfusion in a cohort of 59 of these patients, with or without abnormalities on morphological MRI sequences.Methods: Patients with biologically-confirmed COVID-19 and neurological manifestations undergoing a brain MRI with technically adequate arterial spin labeling (ASL) perfusion were included in this retrospective multicenter study. ASL maps were jointly reviewed by two readers blinded to clinical data. They assessed abnormal perfusion in four regions of interest in each brain hemisphere: frontal lobe, parietal lobe, posterior temporal lobe, and temporal pole extended to the amygdalo-hippocampal complex.Results: Fifty-nine patients (44 men (75%), mean age 61.2 years) were included. Most patients had a severe COVID-19, 57 (97%) needed oxygen therapy and 43 (73%) were hospitalized in intensive care unit at the time of MRI. Morphological brain MRI was abnormal in 44 (75%) patients. ASL perfusion was abnormal in 53 (90%) patients, and particularly in all patients with normal morphological MRI. Hypoperfusion occurred in 48 (81%) patients, mostly in temporal poles (52 (44%)) and frontal lobes (40 (34%)). Hyperperfusion occurred in 9 (15%) patients and was closely associated with post-contrast FLAIR leptomeningeal enhancement (100% [66.4%-100%] of hyperperfusion with enhancement versus 28.6% [16.6%-43.2%] without, p=0.002). Studied clinical parameters (especially sedation) and other morphological MRI anomalies had no significant impact on perfusion anomalies.Conclusion: Brain ASL perfusion showed hypoperfusion in more than 80% of patients with severe COVID-19, with or without visible lesion on conventional MRI abnormalities

Cerebral perfusion using ASL in patients with COVID-19 and neurological manifestations: A retrospective multicenter observational study

Background and purpose: Cerebral hypoperfusion has been reported in patients with COVID-19 and neurological manifestations in small cohorts. We aimed to systematically assess changes in cerebral perfusion in a cohort of 59 of these patients, with or without abnormalities on morphological MRI sequences.Methods: Patients with biologically-confirmed COVID-19 and neurological manifestations undergoing a brain MRI with technically adequate arterial spin labeling (ASL) perfusion were included in this retrospective multicenter study. ASL maps were jointly reviewed by two readers blinded to clinical data. They assessed abnormal perfusion in four regions of interest in each brain hemisphere: frontal lobe, parietal lobe, posterior temporal lobe, and temporal pole extended to the amygdalo-hippocampal complex.Results: Fifty-nine patients (44 men (75%), mean age 61.2 years) were included. Most patients had a severe COVID-19, 57 (97%) needed oxygen therapy and 43 (73%) were hospitalized in intensive care unit at the time of MRI. Morphological brain MRI was abnormal in 44 (75%) patients. ASL perfusion was abnormal in 53 (90%) patients, and particularly in all patients with normal morphological MRI. Hypoperfusion occurred in 48 (81%) patients, mostly in temporal poles (52 (44%)) and frontal lobes (40 (34%)). Hyperperfusion occurred in 9 (15%) patients and was closely associated with post-contrast FLAIR leptomeningeal enhancement (100% [66.4%-100%] of hyperperfusion with enhancement versus 28.6% [16.6%-43.2%] without, p=0.002). Studied clinical parameters (especially sedation) and other morphological MRI anomalies had no significant impact on perfusion anomalies.Conclusion: Brain ASL perfusion showed hypoperfusion in more than 80% of patients with severe COVID-19, with or without visible lesion on conventional MRI abnormalities