Gaois

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  1. SOCIAL QUESTIONS|health|illness

    #559092

    IATE #1489534
    histicíotóis liopóideach Reference Faomhadh an téarma seo mar chuid de Thionscadal Lex
    ga
    galar Niemann-Pick Reference Faomhadh an téarma seo mar chuid de Thionscadal Lex
    ga
    Phosphatidlipoidose | generalisierte retikulohistiozytäre Sphingomyelinlipoidose | lipoidzellige Hepatosplenomegalie | Niemann-Pick Krankheit | großzellige Drüsenmetamorphose
    de
    Definition im Säuglingsalter beginnende familiäre, einfach-rezessiv erbliche Krankheit mit Speicherung von Sphingomyelin im retikulohistiozytären System, auch in Mesenchym und Epithel, präfinal in nahezu allen Organen Reference Reallex.Med.
    essential lipoid histiocytosis | sphingomyelin lipidosis | lipoid histiocytosis (classical phosphatide) | sphingomyelinase deficiency | DAF syndrome | lipid histiocytosis | Niemann-Pick disease
    en
    Definition group of inherited metabolic disorders known as the leukodystrophies or lipid storage diseases in which harmful quantities of a fatty substance (lipids) accumulate in the spleen, liver, lungs, bone marrow, and the brain Reference NINDS (National Institute of Neurological Disorders and Stroke) > Niemann-Pick Disease Information Page, http://www.ninds.nih.gov/disorders/niemann/niemann.htm (8.7.2009)
    Comment See also:- lysosomal storage disease (broader) [ IATE:261782 ]- Niemann-Pick Disease type C (narrower) [ IATE:3550112 ]
    maladie de Niemann-Pick | histiocytose lipoïdique essentielle | sphingomyélinose
    fr
    Definition génopathie humaine caractérisée par une accumulation anormale de sphingomyélines dans les tissus, liée à un déficit enzymatique de la sphingomyélinase qui entraîne une dégénérescence nerveuse progressive et la mort en quelques années Reference Dictionnaire de l'Académie de Médecine - version 2013, Niemann-Pick (maladie de), http://dictionnaire.academie-medecine.fr/?q=iemann-Pick%20(maladie%20de) [13.12.2013]