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  • 1
    ISSN: 1432-0533
    Keywords: Multicore disease ; Central core disease ; Nemaline myopathy ; Mixed myopathies ; Electron microscope
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report on the ultrastructural study of a muscle biopsy carried out in a child 6 years after the first biopsy which had led to diagnosis of “multicore disease”. Clinical evolution following a few years of muscular involvement had been favorable. The lesions observed in the second biopsy were again characterized by the presence of multicores, but in addition to cores and rods. All of these abnormalities could sometimes be noted in a single muscle fiber. Although rare cases of an association of two types of lesions have been reported, no study has hitherto shown multicores, cores, and rods in the same biopsy. This observation might confirm the possibility of common pathogenic mechanisms producing these lesions.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1279-8509
    Keywords: Acute myeloblastic leukemia ; Child ; Bone marrow transplantation ; Chemotherapy ; Cytarabine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the LAME89/91 protocol, children with acute myeloid leukemia (AML) who achieved complete remission (CR) after induction chemotherapy, were treated either with allogeneic bone marrow transplantation (BMT) if they had an HLA-compatible related donor or with chemotherapy including high-dose cytarabine. The objectives of this study were to describe the overall results of this strategy and to compare the two post-remission arms. Two hundred and thirty-one children were enrolled in the protocol. Induction chemotherapy consisted of a combination of cytarabine and mitoxantrone. A CR was achieved in 204 children (88%). Fifty-one of them had an HLA-identical sibling donor and were eligible for BMT. These 51 patients, as well as two additional children who had a one antigen HLA-mismatched father, received BMT during first CR. Consequently, 53 patients were analysed in the BMT group and 151 in the chemotherapy group. With a mean follow up duration in the study of 38 ± 2 months, overall event-free survival (EFS) was 47 ± 7% at 4 years for the 231 patients entered into the protocol. The 4-year disease-free survival (DFS) was 53 ± 8% for the 204 patients who achieved complete remission after induction therapy. The 4-year probability of relapse was 28 ± 14% in the BMT group and 47 ± 9% in the chemotherapy group (p = 0.02). The risk of therapy-related death was 6.2% for BMT and 8.1% for chemotherapy. DFS was 68 ± 14% in the BMT group and 48 ± 9% in the chemotherapy group (p = 0.02). We conclude that allogeneic BMT from a matched sibling donor is the treatment of choice for reducing the relapse risk and for increasing DFS in children with AML in first CR.
    Type of Medium: Electronic Resource
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