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Biomedical subjects

C Brachet

Publications and source records attributed to C Brachet.

6 recordsLinked to original sources
PubMed2005

[Hematopoietic stem cell transplantation for severe sickle cell disease].

Hematopoietic stem cell transplantation is the sole curative therapy for sickle cell anemia (SCA). This treatment is restricted to severe forms of disease. Lack of HLA-identical sibling donor is the major limiting factor for delivering this therapy. Conditioning regimen should be myeloablative. Post transplantation immunosuppressive medication is necessary for both graft tolerance and graft-versus-host disease (GvHD) control. For the majority of patients, bone marrow is source of stem cells. Alternative sources including related cord blood stem cells are under evaluation and promising. Outcome for 250 grafted patients worldwide is excellent. More than 85 % survive free of SCA and have a good quality of life although GvHD remains the main complication.

Anemia, Sickle Cell↗
PubMed2004

Endothelial and platelet microparticles in vasculitis of the young.

OBJECTIVE: Microparticles are released from endothelial cells in response to a variety of injurious stimuli and recently have been shown to be increased in a number of diseases associated with endothelial dysfunction. This study examined endothelial microparticle (EMP) and platelet microparticle (PMP) profiles in children with systemic vasculitis to test the hypothesis that EMPs may provide a noninvasive means of examining endothelial activation or injury. METHODS: The study cohort comprised 39 children with systemic vasculitis at various stages of disease activity, 24 control children with febrile disease, and a control group of 43 healthy subjects. Plasma was ultracentrifuged at 17,000g for 60 minutes, and the microparticle pellets were examined using flow cytometry. RESULTS: Plasma from patients with active systemic vasculitis contained significantly higher numbers of E-selectin-positive EMPs compared with that from patients in remission, healthy controls, or febrile disease controls (P = 0.000 for each). A similar result was obtained for the numbers of EMPs expressing the marker CD105. There was also a significant increase in PMPs expressing CD42a in the active vasculitis group as compared with the other groups, but this difference was not significant for PMPs expressing P-selectin. The EMP counts correlated with the Birmingham Vasculitis Activity Score and the acute-phase reactant levels in the patients with systemic vasculitis, but there was a poor correlation overall between EMP counts and the acute-phase reactant levels in the febrile disease controls. CONCLUSION: EMPs may provide a window to the activated endothelium and could provide important pathophysiologic insights into the vascular injury associated with vasculitis of the young.

Acute-Phase Proteins↗
PubMed2004

Hydroxyurea treatment for sickle cell disease: impact on haematopoietic stem cell transplantation's outcome.

Since 1988, 24 children have undergone haematopoietic stem cell transplantation (HSCT) for severe sickle cell disease (SCD) in our unit, 13 being grafted after having been exposed to hydroxyurea (HU) to control SCD-related complications. Different pre-transplant conditioning regimens were given over time: Bu14/Cy200 in six patients (group 1), Bu16/Cy200/antithymocyte globulin (ATG) in five (group 2) and Bu16/Cy200/ATG with HU prior to HSCT in 13 (group 3). The aim of this study is to compare the outcome after HSCT of these groups of patients, which differ according to pre-transplant drug exposure. Overall, 20 of the 24 transplanted children had stable engraftment and have remained free of SCD-related symptoms after HSCT; 19 of them are currently alive and cured of SCD. In group 1 (HU-, ATG-), we observed one unexplainable late death, one absent engraftment, one late rejection and one mixed stable chimerism. In group 2 (HU-, ATG+), we observed the absence of engraftment in two patients and one early rejection. In group 3 (HU+, ATG+), we observed no cases of either absent engraftment, mixed stable chimerism or late rejection. In our experience, pre-transplant treatment with HU seems to be associated with a lower incidence of rejection/absent engraftment in severe SCD patients. These results need to be confirmed with a larger number of patients.

Anemia, Sickle Cell↗
PubMed2000

[Bilateral breast masses].

A 76 year old woman is hospitalized for bilateral breast masses and neurological impairment. Her medical history is marked by rheumatoid arthritis treated with gold salts and methylprednisolone. Blood tests reveal pancytopenia; the MRI scan of the brain is suggestive of a CNS lymphoma. The pathologic examination of a breast mass specimen confirms the lymphoid nature of the neoplasm. This case report highlights the multifocal or systemic nature of non hodgkin's lymphoma and the diagnostic pitfalls of breast lymphomas. Rheumatoid arthritis and its medical management are reviewed for their possible roles in oncogenesis.

Aged↗