尼曼皮克(Niemam-Pick)氏病,又名鞘磷脂沉积病(Sphingomyelin lipidoses),其特点是全身网状内皮系统有大量的含有神经鞘磷脂的泡沫细胞。为常染色体隐性遗传,可见于世界各种族,但文献报告以犹太人为最多,我国也有报道,本病罕见,现报告一例。 Niemam-Pick’s disease, also known as Sphingomyelin lipidoses, is characterized by a large number of sphingomyelin-containing foam cells throughout the reticuloendothelial system. For the autosomal recessive inheritance, can be seen in all ethnic groups in the world, but the literature to the largest number of Jews, our country has also been reported, the disease is rare, a report is now reported.