ETUDE MORPHO-GRANULOMETRIQUE ET STRUCTURALE DES SEMOULES DE BLE DUR PROPRIETES D'HYDRATATION ET D'AGGLOMERATION

International audienceLes propriétés d'hydratation et d'agglomération de la semoule de blé dur dépendent des caractéristiques de la matière à granuler, du liquide de granulation et de l'outil de granulation. Dans le procédé de fabrication des graines de couscous, la formation, la croissance et la densification des grains de semoule sont effectuées par addition d'eau, mélange et roulage. Une étude de caractérisation de la semoule de blé dur a été entreprise à différentes échelles d'observation : macroscopique, mésoscopique et moléculaire afin de comprendre les mécanismes d'hydratation et d'agglomération. La semoule de blé dur constitue une population de particules hétérogènes en granulométrie et en composition biochimique. Si cette hétérogénéité se traduit par des modifications des propriétés d'hydratation, d'autres études sont nécessaires pour mieux appréhender son influence sur les propriétés d'agglomération

Remission of steroid- and CyA-resistant nephrotic syndrome using multiple drug immunosuppression.

International audienceNephrotic proteinuria in minimal change disease (MCD) is supposed to be due to a circulating factor of immunologic origin. End-stage renal failure occurs if both steroids and immunosuppressive drugs remain ineffective. Three children (2 years, 3 years, and 6 years of age) with secondary steroid-resistant nephrotic syndrome (NS) were included, as they remained resistant to 30 days of treatment with prednisone (60 mg/m(2) per day), three pulses of methylprednisolone (1 g/1.73 m(2)) followed by oral administration of CyA 7.5 mg/kg per day over 2 months, and 1 month of intravenous (i.v.) administration of cyclosporine (blood level 500-600 ng/ml). All three patients were partially responsive to methylprednisolone pulses, with an increase of serum albumin by 100%. They were treated with plasma exchanges, cyclophosphamide and cyclosporine A, both given orally, pefloxacin and methylprednisolone pulses followed by orally administered prednisone. All three patients went into remission within 2 to 5 weeks. The character of their NS changed to a steroid-sensitive one. There were no significant side effects from the therapy. They had normal renal function, normal blood pressure and no residual proteinuria. A combination of plasmapheresis and multiple immunosuppressive medications was effective in producing remission of minimal change NS in three children who were previously resistant to glucocorticoids and cyclosporine

ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso‐occlusive crisis of sickle cell disease

International audienceBackground: Sickle cell disease (SCD) is characterized by vaso‐occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD‐related endothelial injury.Objectives: To decipher the role of VWF and its specific‐cleaving metalloprotease, ADAMTS13, in the vaso‐occlusive and thrombotic process of SCD.Patients/Methods: We investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20‐month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF.Results and Conclusions: VWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 ‐ 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134‐305; P = .04), and positively correlated with inflammatory markers (P < .02). Median ADAMTS13 activity was normal (70 IU/dL; IQR, 60‐80), but 7 patients exhibited a partial deficiency between 25 and 45 IU/dL. ADAMTS13 activity/VWF:Ag ratio, however, did not change during VOC. Median ADAMTS13:Ag was slightly decreased (611 ng/mL; IQR, 504‐703) with no significant difference between groups. Surprisingly, ADAMTS13 IgGs were detected in 33 (51%) of our patients. We conclude that, in SCD, VWF:Ag and nonrelevant ADAMTS13 IgGs may reflect the severity of the inflammatory vasculopathy enhancing vaso‐occlusive and thrombotic complications

TIP60 Inhibitor TH1834 Reduces Breast Cancer Progression in Xenografts in Mice

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