Elleder M, Smid F
Cesk Patol. 1975 Aug;11(3):122-6.
On the basis of a bioptic examination of the appendix, skin and a liver specimen, the diagnosis of phospholipidosis was made in a girl aged 27 months. In contrast to the Niemann-Pick's complex of sphingomyelinoses, phosphoglycerides were stored in larger amounts than spingomyelin. The disease should be undoubtedly included under one heading with the so-called "kephalinosis" [11] and with the cases described by Wiedemann et al. [10]. The terms "Type II phospholipidosis [Baar-Wiedemann's disease]" or, briefly, phosphoglyceridosis, appear to be most adequeate for designating the diseases in question. The disorder can be diagnosed on the basis of iron hematoxylin staining, visualizing all phospholipids. In Niemann-Pick's sfingomyelinosis, alkaline hydrolysis does not alter the colour, whereas in the phosphoglyceridosis under discussion the colour is substantially reduced or even desappears after alkaline hydrolysis.
通过对阑尾、皮肤和肝脏标本进行活检，对一名27个月大的女童做出了磷脂沉积症的诊断。与鞘磷脂沉积症的尼曼-皮克综合征不同，磷酸甘油酯的储存量比鞘磷脂更多。这种疾病无疑应与所谓的“脑磷脂沉积症”[11]以及维德曼等人描述的病例[10]归为同一类。术语“II型磷脂沉积症[巴尔-维德曼病]”或简称为磷酸甘油酯沉积症，似乎最适合用于命名上述疾病。这种病症可通过铁苏木精染色来诊断，该染色能使所有磷脂可视化。在尼曼-皮克鞘磷脂沉积症中，碱性水解不会改变颜色，而在本文讨论的磷酸甘油酯沉积症中，碱性水解后颜色会大幅变淡甚至消失。
