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syndróm modrých histiocytov

  1. Subject h.syndróm modrých histiocytov
    Subject h.Sea-Blue Histiocyte Syndrome
    Entry termshistiocytóza s histiocytmi farby morskej modrej
    syndróm sea-blue histiocytov
    English X referencesHistiocytosis, Sea-Blue
    Scope note in EnglishA congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.
    See also reference (FX) in Slovak Niemannove-Pickove choroby
    See also reference (FX) in English Niemann-Pick Diseases
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    (1) - MeSH descriptor
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Number of the records: 1  

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