BackgroundOsteonecrosis of the femoral head is a frequent complication in adult patients with sickle cell disease. However, little is known about the natural history of asymptomatic ...lesions.MethodsOne hundred and twenty-one patients (121 hips) with sickle cell disease and asymptomatic osteonecrosis of the femoral head that was contralateral to a hip with symptomatic osteonecrosis were identified with magnetic resonance imaging between 1985 and 1995. The lesions were graded with use of the Steinberg classification system. The patients were followed with annual plain radiographs. The mean duration of follow-up was fourteen years.ResultsAt the time of the initial evaluation, fifty-six hips were classified as Steinberg stage 0, forty-two hips were classified as Steinberg stage I, and twenty-three hips were classified as Steinberg stage II. At the time of the most recent follow-up, pain had developed in 110 previously asymptomatic hips (91%) and collapse had occurred in ninety-three hips (77%). Symptoms always preceded collapse. Of the fifty-six hips that were classified as Steinberg stage 0 at the time of the initial evaluation, forty-seven (84%) had symptomatic osteonecrosis and thirty-four (61%) had collapse at the time of the most recent follow-up. Of the forty-two asymptomatic stage-I hips, forty (95%) became symptomatic within three years and thirty-six (86%) had collapse of the femoral head. Of the twenty-three asymptomatic stage-II hips, all became symptomatic within two years and all collapsed; the mean interval between the onset of pain and collapse was eleven months. At the time of the final follow-up, ninety-one hips (75%) had intractable pain and required surgery.ConclusionsUntreated asymptomatic osteonecrosis of the femoral head in patients with sickle cell disease has a high likelihood of progression to pain and collapse. Because of the high prevalence of complications after total hip arthroplasty in patients with this disease, consideration should be given to early surgical intervention with other procedures in an attempt to retard progression of the disease.Level of EvidencePrognostic Level II. See Instructions to Authors for a complete description of levels of evidence.
Background and purpose
Epilepsy is most common in lower‐income settings where access to electroencephalography (EEG) is generally poor. A low‐cost tablet‐based EEG device may be valuable, but the ...quality and reproducibility of the EEG output are not established.
Methods
Tablet‐based EEG was deployed in a heterogeneous epilepsy cohort in the Republic of Guinea (2018–2019), consisting of a tablet wirelessly connected to a 14‐electrode cap. Participants underwent EEG twice (EEG1 and EEG2), separated by a variable time interval. Recordings were scored remotely by experts in clinical neurophysiology as to data quality and clinical utility.
Results
There were 149 participants (41% female; median age 17.9 years; 66.6% ≤21 years of age; mean seizures per month 5.7 ± SD 15.5). The mean duration of EEG1 was 53 ± 12.3 min and that of EEG2 was 29.6 ± 12.8 min. The mean quality scores of EEG1 and EEG2 were 6.4 range, 1 (low) to 10 (high); both medians 7.0. A total of 44 (29.5%) participants had epileptiform discharges (EDs) at EEG1 and 25 (16.8%) had EDs at EEG2. EDs were focal/multifocal (rather than generalized) in 70.1% of EEG1 and 72.5% of EEG2 interpretations. A total of 39 (26.2%) were recommended for neuroimaging after EEG1 and 22 (14.8%) after EEG2. Of participants without EDs at EEG1 (n = 53, 55.8%), seven (13.2%) had EDs at EEG2. Of participants with detectable EDs on EEG1 (n = 23, 24.2%), 12 (52.1%) did not have EDs at EEG2.
Conclusions
Tablet‐based EEG had a reproducible quality level on repeat testing and was useful for the detection of EDs. The incremental yield of a second EEG in this setting was ~13%. The need for neuroimaging access was evident.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
In France, reporting of adverse events related, or likely to be related, to transfusion is mandatory. Since its creation in 1993, the French hemovigilance system has contributed to a better ...recognition of unappreciated risks like delayed hemolytic transfusion reactions (DHTR) in sickle-cell disease (SCD) patients. Long under-reported or misclassified, reports of this serious complication of transfusion have improved, particularly through the dissemination of information within the hemovigilance network. To our knowledge, the French hemovigilance system has one of the largest series of DHTR in SCD patients. Guidelines for diagnosis and reporting to hemovigilance system as well as a specific reporting form are being developed, which should contribute to the quality of data essential for epidemiological studies.
En France, la déclaration de tout effet indésirable, dû ou susceptible d’être dû à la transfusion, est obligatoire. Depuis sa création en 1993, le système d’hémovigilance a contribué à une meilleure reconnaissance de certains risques comme les hémolyses post-transfusionnelles chez les patients drépanocytaires. Longtemps sous-déclarée ou classée sous un autre diagnostic, les déclarations de cette complication grave de la transfusion se sont améliorées, en particulier grâce à la diffusion d’informations au sein du réseau d’hémovigilance. Actuellement, le système d’hémovigilance français possède une des plus grandes séries d’hémolyses post-transfusionnelles chez les patients drépanocytaires. Un guide de déclaration et un recueil de données spécifiques sont en cours et devraient permettre d’améliorer la qualité des données et de réaliser des études épidémiologiques.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
1 Etablissement Français du Sang, Ile de France, Hôpital Henri Mondor, Créteil;
2 Centre de Référence des Cytopénies Auto-Immunes, Hôpital Henri Mondor, Créteil;
3 Service des Maladies Génétiques du ...Globule Rouge, Hôpital Henri Mondor Créteil;
4 Service de Médecine Interne, Hôpital Henri Mondor, Créteil;
5 Laboratoire dImmunologie, Hôpital Henri Mondor, Créteil;
6 Université de Poitiers, EA 3806, CHU de Poitiers
Correspondence: France Noizat-Pirenne, MD, PhD, Etablissement Français du Sang dIle de France, 51 Avenue du Maréchal de Lattre de Tassigny, 94000, Créteil, France. Tel: 331 56 72 76 37; Fax: 331 56 72 76 01, E-mail: france.noizat-pirenne{at}efs.sante.fr
Delayed hemolytic transfusion reaction (DHTR), a life-threatening transfusion complication in sickle cell disease (SCD), is characterized by a marked hemoglobin drop with destruction of both transfused and autologous red blood cells (RBCs) and exacerbation of SCD symptoms. One mechanism of RBCs destruction is auto-antibody production secondary to transfusion. As rituximab specifically targets circulating B cells, we thought that it could be beneficial in preventing this immune-mediated transfusion complication. We report the case of a SCD patient who previously experienced DHTR with auto-antibodies and who needed a new transfusion. DHTR recurrence was successfully prevented by rituximab administration prior transfusion, supporting the safe use of rituximab to prevent DHTR in SCD patients as a second line approach when other measures failed.
Key words: Sickle cell disease, rituximab, transfusion, auto-antibodies.
La prévention des hémolyses post-transfusionnelles chez les drépanocytaires est conditionnée par notre capacité à prévenir l’allo-immunisation et les conflits antigène–anticorps. Cette étude montre ...l’importance de mettre en place une organisation transfusionnelle adaptée au profil immunohématologique particulier des drépanocytaires pour leur garantir une sécurité transfusionnelle optimale.
Les données immunohématologiques de 206 adultes drépanocytaires transfusés en dehors de l’urgence ont été analysées, ainsi que le phénotype des concentrés globulaires (CGR) délivrés. Cinquante-quatre pour cent des patients ont un phénotype D+C−E−c+e+. Pour prévenir l’allo-immunisation anti-C et anti-E, 26 % de CGR D−C−E−c+e+ ont été transfusés à ces patients D+. Quarante-sept pour cent des patients ont un historique d’allo-immunisation, alors que seulement 15 % ont une recherche d’agglutinines irrégulières positive le jour de l’inclusion, les anticorps non décelables étant souvent dangereux. Enfin, cette étude montre la fréquence importante d’anti-D chez les sujets D+ et d’anti-C chez les sujets C+, posant la question du caractère partiel des antigènes D et C.
Pour une sécurité transfusionnelle optimale des patients drépanocytaires et une meilleure adéquation entre phénotypes des receveurs et phénotypes des CGR disponibles, trois points dans l’organisation transfusionnelle doivent être améliorés : la promotion du don au sein des populations afro-antillaises doit être renforcée pour disposer de CGR D+C−E−c+e+ et éviter d’utiliser la ressource limitée de CGR D−C−E−c+e+ ; les données immunohématologiques des patients doivent être accessibles aux prescripteurs et transfuseurs, afin d’éviter les accidents immuno-hémolytiques par restimulation ; enfin, la recherche par biologie moléculaire des antigènes variants d’intérêt transfusionnel doit être mise en place chez les donneurs et receveurs afro-antillais.
Prevention of hemolytic transfusion reactions depends upon our capacity to prevent allo-immunization and conflicts between antigens of transfused red blood cells and antibodies produced by the recipient. In this study, we show that to secure transfusion of sickle cell disease patients, it is necessary to take into account their immunohematologic characteristics in the organization of transfusion.
Immunohematological data of 206 chronically transfused patients have been collected as well as phenotypes of transfused units. In order to prevent allo-immunization against C and E antigens for patients typed D+C−E−c+e+ (56%), 26% of the transfused units were D−C−E−c+e+. We found that 47% of the patients had a history of allo-immunization, whereas only 15% produced an antibody the day of inclusion in the study. The non-detectable antibodies were frequently known as dangerous for transfusion. Finally, this study shows the frequency of anti-D in D+ patients and anti-C in C+ patients, pointing out the question of partial antigens.
To insure optimal transfusion safety for sickle cell disease patients, three points have to be improved: blood donation within the Afro-Caribbean community living in France, access to history of immuno-hematological data, detection of variant antigens, especially within the RH blood system.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
•We process two months mobile network trajectories from the Greater Paris region.•The transport mode is inferred from all trajectories, using few labeled data.•Total road and rail OD flows are ...estimated over time at different resolutions.•The estimates are validated against survey and travel cards flows.
Fast urbanization generates increasing amounts of travel flows, urging the need for efficient transport planning policies. In parallel, mobile phone data have emerged as the largest mobility data source, but are not yet integrated to transport planning models. Currently, transport authorities are lacking a global picture of daily passenger flows on multimodal transport networks. In this work, we propose the first methodology to infer dynamic Origin-Destination flows by transport modes using mobile network data e.g., Call Detail Records. For this study, we pre-process 360 million trajectories for more than 2 million devices from the Greater Paris as our case study region. The model combines mobile network geolocation with transport network geospatial data, travel survey, census and travel card data. The transport modes of mobile network trajectories are identified through a two-steps semi-supervised learning algorithm. The later involves clustering of mobile network areas and Bayesian inference to generate transport probabilities for trajectories. After attributing the mode with highest probability to each trajectory, we construct Origin-Destination matrices by transport mode. Flows are up-scaled to the total population using state-of-the-art expansion factors. The model generates time variant road and rail passenger flows for the complete region. From our results, we observe different mobility patterns for road and rail modes and between Paris and its suburbs. The resulting transport flows are extensively validated against the travel survey and the travel card data for different spatial scales.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
BackgroundAdult patients with sickle-cell disease are at risk for the development of osteonecrosis of the hip. However, there is little information in the literature about the rate of progression of ...osteonecrosis once symptoms begin. The purpose of this study was to evaluate the natural history of the symptomatic hip in adult patients with osteonecrosis and sickle-cell disease.MethodsNinety-two symptomatic hips in sixty-four consecutive adult patients with sickle-cell disease were initially evaluated between 1980 and 1987. Sixty symptomatic hips had radiographic evidence of osteonecrosis at the initial evaluationforty-three were classified as stage II; two, as stage III; and fifteen, as stage IV, according to the system of Steinberg et al. The other thirty-two hips had lesions (stage I) that were evident only on magnetic resonance imaging. All patients were evaluated after a mean duration of follow-up of seventeen years.ResultsOf the seventy-five hips without collapse of the femoral head at the initial evaluation, sixty-five demonstrated collapse within five years after the diagnosis. The average time between the diagnosis and collapse was forty-two months for stage-I hips and thirty months for stage-II hips. At the most recent follow-up examination, ninety hips had had collapse of the femoral head and eighty-eight of the ninety-two hips had had surgery because of intractable pain.ConclusionsSymptomatic osteonecrosis of the hip in sickle-cell disease has a high likelihood of leading to femoral head collapse, necessitating surgical intervention. When osteonecrosis develops, the deterioration is rapid and, in most patients, operative intervention is necessary because of intractable pain.Level of EvidencePrognostic study, Level II-1 (retrospective study). See Instructions to Authors for a complete description of levels of evidence.
Objectives To analyze the main characteristics of adults with sickle cell disease (SCD) and concurrent connective tissue disease (CTD). Methods A retrospective investigational study was performed. ...CTD was diagnosed according to standard international criteria. Severity of SCD was assessed by a clinical severity score. Results Thirty patients, 23 women (76%) and 7 men, with hemoglobin S/S (n = 25) or S/C (n = 5) SCD were included. The subtypes of CTD were rheumatoid arthritis (RA) (n = 15), definite systemic lupus erythematosus or “incomplete lupus” requiring treatment (n = 13), primary Sjögren's syndrome with central nervous system involvement (n = 1), and systemic sclerosis (n = 1). Twenty-five of the 30 patients (83%) received steroid treatment, and 15 (50%) received at least 1 immunosuppressive agent (methotrexate in 14 cases) to control CTD. Four RA patients were given antitumor necrosis factor (TNF)α and 1 was treated with rituximab without SCD exacerbation. After a median follow-up of 4.5 years range: 6 months to 30 years from CTD diagnosis, 11 of the 25 (44%) patients receiving steroids had at least 1 episode of severe infection (mostly due to Staphylococcus aureus or Escherichia coli ). SCD exacerbated in 13 of the 30 (43%) patients after CTD onset; 12 of these patients were receiving prednisone and/or methotrexate. Six patients (20%) had died from sepsis (n = 2), stroke (n = 2), or acute chest syndrome (n = 2). Conclusions CTD-related clinical manifestations and outcome were not particularly severe in patients with SCD. However, those with active CTD and undergoing steroid ± methotrexate treatment had more serious SCD-related manifestations, a higher rate of severe infections, and an overall patient mortality rate of 20%. Thus, the management of patients with CTD and underlying SCD should consider the risk/benefit ratio of each treatment and steroid-sparing strategies should be implemented.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
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