22 research outputs found

    Targeted therapy monitoring of BRAF-V600-mutant Erdheim-Chester disease by fast quantitative whole-body bone CZT-tomoscintigraphies

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    Abstract Erdheim-Chester disease (ECD) is a rare histiocytosis due to proto-oncogene mutations, primarily affecting the long bones and possibly being treated by novel targeted therapies. 18F-FDG PET is a reference technique for ECD assessment. However, we present a case where easier and more objective monitoring of the ECD-related bone metabolism abnormalities under treatment was obtained with the standardized uptake value-based information provided by fast whole-body [Tc-99 m]-HDP bone tomoscintigraphies (QWBT) recorded with a high-sensitivity CZT-camera/computed tomography (CT) hybrid system

    Radicals of Free and Zinc(II)-Coordinated α-Azophenols

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    The tridentate 2-tert-butyl-4-methoxy-6-(quinolin-8-ylazo)phenol ligand HL has been synthesized and structurally characterized. Its redox activity has been investigated by electrochemical measurements and DFT calculations. Oxidation of HL (irreversible process) affords primarily a hydrogen-bonded phenoxyl radical, whereas its reduction affords an iminosemiquinonate radical species. The reaction of two equivalents of HL with Zn(OAc)2 affords the zinc complex Zn(L)2, which has been structurally characterized. Its oxidation is easier than that of HL and is reversible. The EPR spectrum of [Zn(L)(L·)]+ shows an unresolved (S = 1/2) signal at g = 2.005, while [Zn(L·)]2+ exhibits spin triplet resonances (|D| = 0.0118 cm–1 and E/D = 0). The extra delocalization of spin density on the azo group contributes to a lowering of the D value relative to those of Schiff base derivatives. The temperature dependence of the EPR signal and DFT calculations reveal an excited state for the spin triplet and a weak exchange coupling constant J = –2.73 cm–1

    Altered distribution and function of splenic innate lymphoid cells in adult chronic immune thrombocytopenia

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    IF 7.607International audienceInnate lymphoid cells (ILCs) have been characterized as innate immune cells capable to modulate the immune response in the mucosae. Human ILCs have been rarely described in secondary lymphoid organs except in tonsils. Moreover, their function and phenotype in human secondary lymphoid organs during autoimmune diseases have never been studied. We took advantage of splenectomy as a treatment of immune thrombocytopenia (ITP) to describe and compare splenic ILC from 18 ITP patients to 11 controls. We first confirmed that ILC3 represented the most abundant ILC subset in human non-inflamed spleens, accounting for 90% of total ILC, and that they were mostly constituted of NKp44- cells. On the contrary, proportions of ILC1 and ILC2 in spleens were lower than in blood. Splenic IL-2- and IFN-Îł-producing ILC1 were increased in ITP. While the frequencies of total splenic ILC3 were similar in the two groups, splenic GM-CSF-producing ILC3 were increased in ITP. This is the first description of human ILC in a major secondary lymphoid organ during an autoimmune disease, ITP. We observed an expansion of splenic ILC1 that could participate to the Th1 skewing, while the increased production of GM-CSF by splenic ILC3 could stimulate splenic macrophages which play a key role in ITP pathophysiology

    Supplemental Material - Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis

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    Supplemental Material for Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis by Pierre Loiseau, Thomas Foret, Ersilia M DeFilippis, Jessie Risse, Anais D Etienne, Virginie Dufrost, Thomas Moulinet, Doruk Erkan, Hervé Devilliers, Denis Wahl, and Stéphane Zuily in Lupus</p

    Supplemental Material - Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis

    No full text
    Supplemental Material for Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis by Pierre Loiseau, Thomas Foret, Ersilia M DeFilippis, Jessie Risse, Anais D Etienne, Virginie Dufrost, Thomas Moulinet, Doruk Erkan, Hervé Devilliers, Denis Wahl, and Stéphane Zuily in Lupus</p
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