107 research outputs found

    Hydrothermal synthesis, structure and thermal stability of diamine templated layered uranyl-vanadates

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    International audienceSix new layered uranyl vanadates (NH4)2[(UO2)2V2O8] (1), (H2EN)[(UO2)2V2O8] (2), (H2DAP)[(UO2)2V2O8] (3), (H2PIP)[(UO2)2(VO4)2].0,8H2O (4), (H2DMPIP)[(UO2)2V2O8] (5), (H2DABCO)[(UO2)2(VO4)2] (6) were prepared from mild-hydrothermal reactions using 1,2-ethylenediamine (EN); 1,3-diaminopropane (DAP); piperazine (PIP); 1-methylpiperazine (MPIP); 1,4-diazabicyclo[2,2,2]octane (DABCO). The structures of 1, 4, 5 and 6 were solved using single-crystal X-ray diffraction data while the structural models of 2 and 3 were established from powder X-ray diffraction data. In compounds 1, 2, 3 and 5, the uranyl-vanadate layers are built from dimers of edge-shared UO7 pentagonal bipyramids and dimers of edge-shared VO5 square pyramids further connected through edge-sharing. In 1 and 3, the layers are identical to that occurring in the carnotite group of uranyl-vanadates. In 2 and 5, the V2O8 dimers differ in orientation leading to a new type of layer. The layers of compound 4 and 6 are built from chains of edge-shared UO7 pentagonal bipyramids connected by VO4 tetrahedra and are of uranophane-type anion topology. For the six compounds, the ammonium or organoammonium cation resides in the space between the inorganic layers

    Light chain amyloidosis

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    Light chain amyloidosis (AL) is the most common subtype of amyloidosis except wild type tranthyretine amyloidosis and is caused by the deposition of misfolded monoclonal light chains of immunoglobulins produced by a monoclonal B cell, mainly of plasma cell origin. Affected patients may present with amyloidosis alone or in association with other plasma cell or lymphoid dyscrasias (multiple myeloma, Waldenström macroglobulinemia or other B lymphoma). Diagnosis of amyloidosis is histological. Cardiac and renal involvement are the most frequent and present in nearly two thirds of patients as hypertrophic heart disease and/or nephrotic syndrome, respectively. AL amyloidosis is a clonal plasma cell disorder and is treated by chemotherapy dedicated to eradicate the underlying clone. Assessment of the severity of the disease with the Mayo Clinic score is used to guide the choice of treatment. First-line treatment combine bortezomib, cyclophosphamide or melphalan and dexamethasone for severe cases, plus or minus daratumumab, an anti-CD-38 monoclonal antibody, following the excellent results of the ANDROMEDA phase 3 study; mild cases can still benefit from melphalan and dexamethasone

    New genetic loci link adipose and insulin biology to body fat distribution.

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    Body fat distribution is a heritable trait and a well-established predictor of adverse metabolic outcomes, independent of overall adiposity. To increase our understanding of the genetic basis of body fat distribution and its molecular links to cardiometabolic traits, here we conduct genome-wide association meta-analyses of traits related to waist and hip circumferences in up to 224,459 individuals. We identify 49 loci (33 new) associated with waist-to-hip ratio adjusted for body mass index (BMI), and an additional 19 loci newly associated with related waist and hip circumference measures (P < 5 × 10(-8)). In total, 20 of the 49 waist-to-hip ratio adjusted for BMI loci show significant sexual dimorphism, 19 of which display a stronger effect in women. The identified loci were enriched for genes expressed in adipose tissue and for putative regulatory elements in adipocytes. Pathway analyses implicated adipogenesis, angiogenesis, transcriptional regulation and insulin resistance as processes affecting fat distribution, providing insight into potential pathophysiological mechanisms

    Lymphome non Hodgkinien après cancer du sein (étude rétrospective de 46 patientes suivies à l'Institut Curie)

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    Le cancer du sein est le cancer féminin le plus fréquent. En France, il atteint environ une femme sur douze. L'amélioration du dépistage et de la prise en charge thérapeutique a permis d'obtenir de nombreuses survivantes à long terme et ce, quel que soit le stade initial de la maladie. Ces femmes peuvent secondairement développer un second cancer. Aucun facteur de risque génétique et/ou lié aux traitements n'a été démontré. Parmi plus de 30000 patientes suivies à l'institut Curie pour un cancer du sein depuis plus de 30 ans, nous avons recensé 57 femmes ayant développé secondairement un lymphome non Hodgkinien. Quarante six d'entre elles ont été analysées dans cette étude rétrospective. L'âge médian au diagnostic de cancer du sein était de 57 ans. Plus de 90% des patientes ont reçu un traitement par radiothérapie, en association pour 13% (6) d'entre elles à de la chimiothérapie. Avec un suivi médian de près de 12 ans, 76 % (35) des patientes étaient en rémission complète. Seuls 14 évènements relatifs au cancer du sein ont été observés. Le delai médian d'apparition du lymphome était de 7,8 ans. L'âge médian était de 63 ans. Les lymphomes de phénotype B étaient majoritairement représentés avec 50 % de lymphomes folliculaires et 30,4% de lymphomes à grandes cellules. Il n'existait aucune anomalie cytogénétique particulière. Toutes ces patientes ont pu recevoir un traitement adapté. avec un suivi médian de 4,3 ans, 32,6% (15) étaient en rémission complète. Deux patientes ont été perdues de vue et 22 (47,8%) sont décédées du lymphome. Comme dans les lymphomes de novo, le pronostic était lié au grade histologique, avec un avantage de survie pour les lymphomes de bas grade...PARIS7-Villemin (751102101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

    Aggressive FLC Escape in a Patient with IgD Myeloma

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    Background. Some patients who are stable or in remission from a myeloma secreting intact monoclonal immunoglobulin (+/− associated free light-chains (FLCs)) relapse with production of FLC. This FLC escape is one of the illustrations of the intraclonal heterogeneity of multiple myeloma. Results. We report FLC escape in a patient with IgD myeloma characterized by a severe outcome. We discuss parameters that negatively impacted prognosis in this patient, including bone lesions, biochemical parameters, and genomic abnormalities. Conclusion. This case illustrates the selective pressure exerted by therapeutic drugs and the variable sensitivity of subclones to these drugs; it also highlights the importance of FLC monitoring in treated MM patients
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