9 research outputs found

    3D articulated growth model of the fetus skeleton, envelope and soft tissues

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    International audience<p>Fetal dosimetry studies require thedevelopment of accurate 3D models of the fe-tus. This paper proposes a 3D articulated fe-tal growth model including skeleton, body en-velope, brain and lungs based on medical im-ages of 10 dierent fetuses acquired in clinicalroutine. The structures of interest were semi-manually segmented from the images and sur-face meshes were generated. A generic mesh ofeach structure has been deformed towards thesegmented ones. By interpolating linearly be-tween the subjects of the database, each struc-ture can be estimated at any age and in anyposition. This process results in an automatedmodel, the operator being only required tospecify the age and position of the desired es-timated fetus.</p

    Occurrence and severity of adverse events after autologous hematopoietic progenitor cell infusion are related to the amount of granulocytes in the apheresis product.

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    International audienceBACKGROUND: Adverse events (AEs) after hematopoietic progenitor cell (HPC) infusion are rare but might be life-threatening. These reactions have traditionally been associated with the amount of infused cryoprotectant, but persistence of such events after dimethyl sulfoxide (DMSO) depletion has questioned this assumption. STUDY DESIGN AND METHODS: The incidence of AEs on a cohort of 460 patients (490 HPC infusions) undergoing autologous DMSO-reduced HPC transplantation was prospectively evaluated. HPCs were collected from adult patients with various hematologic or solid malignancies. After quality control (QC) on fresh apheresis products and subsequent cryopreservation, HPC grafts were thawed and washed at the cell therapy facility. QC was performed on each graft after washing, and clinical data were collected for each infusion. RESULTS: AEs were reported in 66 cases (13.5%) and were graded according to the NCI-CTC scale from 1 to 4. Although none of the factors associated with patient characteristics or infusion procedure were different between the two groups (no AE vs. occurrence of AE), it was found that the absolute number of granulocytes measured before freezing was considerably higher in the AE group. Furthermore, within this group, there was a strong correlation between the amount of granulocytes and the grading of the reaction. CONCLUSION: This survey demonstrates that AEs occurring in the setting of DMSO-reduced HPC grafts are directly related to the amount of granulocytes and thus emphasizes the need for high-quality apheresis products so as to improve the safety of HPC infusion

    Modalités et impact de la cryopréservation des greffons allogéniques en contexte pandémique : recommandations de la Société Francophone de Greffe de Moelle et de Thérapie Cellulaire (SFGM-TC)

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    peer reviewedLa pandémie COVID-19 a bouleversé les activités de greffe et a nécessité le recours à la cryopréservation des greffons de cellules souches hématopoïétiques allogéniques pour sécuriser la procédure, tant au niveau du patient que du donneur. La cryopréservation en situation allogénique, habituellement anecdotique, a été utilisée par tous les centres francophones. Les données collectées auprès de 24 centres ont été analysées afin d'évaluer l'impact de la cryopréservation sur la qualité du greffon. Cette analyse démontre le rôle délétère du transit prolongé (plus de 48 heures) sur la récupération des progéniteurs CD34+, l'importance de l'augmentation de la dose de progéniteurs CD34+ demandée, justifiant la nécessité d'un contrôle de qualité après décongélation.Method and impact of allografts cryopreservation during the Covid-19 pandemic: guidelines from the Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) The COVID-19 pandemic disorganized the allogeneic stem cell transplantation activities all over the world, with the necessity to cryopreserve allografts to secure the procedure for both the recipient and the donor. Cryopreservation, usually anecdotal, has been used by all the French speaking centers; data collected from 24 centers were assessed in order to determine the impact of cryopreservation on the quality of allografts. Our analysis clearly demonstrates that increasing transit time (more than 48 hours) is deleterious for CD34+ recovery, legitimates the slight increase of the requested CD34+ cell dose with respect to the average recovery rate as well as the importance of the quality control on the infused product

    Affected female carriers of MTM1 mutations display a wide spectrum of clinical and pathological involvement: delineating diagnostic clues.

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    X-linked myotubular myopathy (XLMTM), a severe congenital myopathy, is caused by mutations in the MTM1 gene located on the X chromosome. A majority of affected males die in the early postnatal period, whereas female carriers are believed to be usually asymptomatic. Nevertheless, several affected females have been reported. To assess the phenotypic and pathological spectra of carrier females and to delineate diagnostic clues, we characterized 17 new unrelated affected females and performed a detailed comparison with previously reported cases at the clinical, muscle imaging, histological, ultrastructural and molecular levels. Taken together, the analysis of this large cohort of 43 cases highlights a wide spectrum of clinical severity ranging from severe neonatal and generalized weakness, similar to XLMTM male, to milder adult forms. Several females show a decline in respiratory function. Asymmetric weakness is a noteworthy frequent specific feature potentially correlated to an increased prevalence of highly skewed X inactivation. Asymmetry of growth was also noted. Other diagnostic clues include facial weakness, ptosis and ophthalmoplegia, skeletal and joint abnormalities, and histopathological signs that are hallmarks of centronuclear myopathy such as centralized nuclei and necklace fibers. The histopathological findings also demonstrate a general disorganization of muscle structure in addition to these specific hallmarks. Thus, MTM1 mutations in carrier females define a specific myopathy, which may be independent of the presence of an XLMTM male in the family. As several of the reported affected females carry large heterozygous MTM1 deletions not detectable by Sanger sequencing, and as milder phenotypes present as adult-onset limb-girdle myopathy, the prevalence of this myopathy is likely to be greatly underestimated. This report should aid diagnosis and thus the clinical management and genetic counseling of MTM1 carrier females. Furthermore, the clinical and pathological history of this cohort may be useful for therapeutic projects in males with XLMTM, as it illustrates the spectrum of possible evolution of the disease in patients surviving long term

    Le site de référence du Partenariat européen d’innovation pour un vieillissement actif et en bonne santé MACVIA-LR (contre les maladies chroniques pour un vieillissement en bonne santé en Languedoc-Roussillon)

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    International audienceLe site de référence du Partenariat européen d'innovation pour un vieillissement actif et en bonne santé MACVIA-LR (contre les maladies chroniques pour un vieillissement en bonne santé en Languedoc-Roussillon

    Le site de référence du Partenariat européen d’innovation pour un vieillissement actif et en bonne santé MACVIA-LR (contre les maladies chroniques pour un vieillissement en bonne santé en Languedoc-Roussillon)

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