尼曼匹克病 (Ｎｉｅｍａｎｎ Ｐｉｃｋｄｉｓｅａｓｅ ,ＮＰＤ)是一种常染色体隐性遗传性疾病 ,至今以完整的尸检进行病理临床分析者很少 ,现将我科 2例ＮＰＤ尸检报告如下。病例 1,女 ,生后 10个月死亡。因间歇性发热 ,腹泻伴腹部包块 3个月余就诊。体检 :体温 40°Ｃ ,脾肋下 Niemann Pick disease (NPD) is an autosomal recessive disease, so far the pathological clinical analysis of a complete autopsy are few, now our department 2 NPD autopsy report is as follows. Case 1, female, 10 months after birth, died. Due to intermittent fever, diarrhea with abdominal mass more than 3 months treatment. Physical examination: body temperature 40 ° C, spleen ribs