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尼曼一匹克氏病(Niemann—Pick’s disease):为先天性家族性疾病。由细胞内的类脂质代谢异常而使酯与辅酶的作用失调,所引起的类脂质在体内过多沉积所致的一种常染色体隐性遗传性疾病。此病病变为全身网状内皮系统有大量含有神经磷脂的泡沫样细胞。尤其是脑神经核细胞、肝细胞内,积贮着大量神经磷脂。镜检可见大量泡沫样细胞。称为尼曼一匹克氏细胞。 本病较为罕见。国外病例40％见于犹太人,有家族史的占32％。多数病历无神经系统损害,近年来根据发病年龄、种族和有无神经系统症状,而分甲、乙、丙、丁四种类型。甲型发病时间较早,相当于婴儿
Niemann-Pick’s disease: a congenital familial disease. An abnormal autosomal recessive disease caused by undue deposition of lipids in the body caused by dysregulation of lipids in the cell and dysfunction of esters and coenzymes. The disease becomes systemic reticuloendothelial system contains a large number of neurospheres containing foamy cells. In particular, neurons, brain cells, hepatocytes, accumulate a large number of neurophospholipids. Microscopic examination revealed a large number of foam-like cells. Called Niemann pike cells. The disease is more rare. 40% of foreign cases seen in Jews, family history accounted for 32%. Most medical records without neurological damage, in recent years, according to age of onset, race and with or without neurological symptoms, and points A, B, C, D four types. A type of disease earlier, the equivalent of infants