Niemann—Pick氏病是全身脏器特别是网状内皮系统和中枢神经系统发生神经鞘髓磷脂蓄积的先天性代谢性疾病。以常染色体隐性方式遗传。本病由Niematan(1914年)和Pick(1920年)形成体系。此后由Klenk(1934年)证实蓄积物质是神经鞘髓磷脂。有关本病,在放射界讨论较少。本文拟作一综述,以期抛砖引玉。一、发病情况一般认为在犹太人多见。日本发病率为十万分之一。 Niemann-Pick’s disease is a congenital metabolic disorder in which the whole body organs, especially the reticuloendothelial system and the central nervous system, undergo neurosphere myelin accumulation. An autosomal recessive manner genetic. The disease is formed by Niematan (1914) and Pick (1920). Since then Klenk (1934) confirmed the accumulation of material is sphingomyelin. The disease, less discussion in the radiation sector. This article intends to make a review, with a view to initiate. First, the incidence is generally believed to be more common in Jews. The incidence in Japan is one in 100,000.