Fröhlich E, Harzer K, Heller T, Rühl U
Medizinische Klinik I, Krankenanstalten des Landkreises Ludwigsburg.
Ultraschall Med. 1990 Jun;11(3):119-22. doi: 10.1055/s-2007-1011543.
A female patient is described with highly echogenic splenic nodes found in routine diagnosis. After CT and angiography a splenectomy was carried out. The histological finding of sea blue histiocytes and the extended family history suggested a subtype of Niemann-Pick disease. Biochemical lipid analysis of the spleen confirmed Type-C Niemann-Pick disease. Niemann-Pick disease and its subtypes are discussed briefly.
本文描述了一名女性患者,在常规诊断中发现脾脏有高回声结节。经CT和血管造影检查后,实施了脾切除术。海蓝色组织细胞的组织学发现以及家族病史提示为尼曼-匹克病的一种亚型。脾脏的生化脂质分析确诊为C型尼曼-匹克病。本文简要讨论了尼曼-匹克病及其亚型。